Hybrid method with explorative laparoscopy and anterior open approach for re-recurrent inguinal hernia

Abstract

The new guidelines of the HerniaSurge group recommend that only an expert hernia surgeon should repair a re-recurrent inguinal hernia. We report the efficacy of the hybrid method with explorative laparoscopy and anterior open approach for re-recurrent inguinal hernia repair. A 61-year-old man underwent anterior open preperitoneal mesh repair for right inguinal direct hernia and laparoscopic transabdominal preperitoneal repair for recurrence. Two years after the second surgery, re-recurrent inguinal hernia was confirmed. We carried out explorative laparoscopy for the re-recurrent inguinal hernia, which revealed a re-recurrent hernia orifice. We performed the anterior open approach while observing from the abdominal cavity. Explorative laparoscopy can help in accurately determining the orifice of the re-recurrent inguinal hernia. Based on that information, the hernia sac can be reached through the shortest route using the anterior open approach.

https://ift.tt/2qLIIdD

Right external iliac artery thrombus following the use of resuscitative endovascular balloon occlusion of the aorta for placenta accreta

Abstract

A 33-year-old female, 32 weeks and 1 day gestation, with known placenta accreta who presented to the emergency department with 2 h of severe abdominal pain, nausea and vomiting. She became hypotensive and underwent emergency cesarean section. Emergency general surgery was consulted for placement of a resuscitative endovascular balloon for aortic occlusion (REBOA). After successful delivery, the balloon was inflated in zone 3 and systolic blood pressure rose from 70 to 170 mmHg. The patient underwent hysterectomy for ongoing hemorrhage. The patient was taken to the surgical intensive care unit. The patient was noted to have pulses following removal of the sheath. Arterial brachial indices and arterial duplex was performed 48 h after sheath removal. The patient was found to have complete occlusion of the right external iliac artery. Vascular surgery was consulted and cut-down performed with thrombus removal via fogarty catheter. The patient was discharged 2 days later without further complication.

https://ift.tt/2FlevfU

Additional unloading of the left ventricle using the Impella LP 2.5 during extracorporeal life support in cases of pulmonary congestion

Abstract

Extracorporeal life support (ECLS) is used for the treatment of severe cardiogenic shock. However, pulmonary congestion can progress to a severe problem with ECLS therapy. We report our experience with the Impella system for severe pulmonary congestion with ECLS therapy. We used the Impella system for two patients, which led to successful unloading of the left ventricle. Impella implantation during ECLS support appears to be a promising concept. However, more evidence is required for further evaluation.

https://ift.tt/2qLIDXn

Necrotizing fasciitis caused by Kocuria rosea in an immunocompromised patient

Abstract

This case report describes an immunocompromised patient with a skin boil that progressed to necrotizing fasciitis. The aim of this brief report is to raise awareness regarding necrotizing soft tissue infections caused by an unusual organism, Kocuria rosea, a typically non-pathogenic organism, and outline the course of treatment currently considered to be the standard of care.

https://ift.tt/2Fw6Ds7

Colonic perforation due to amebiasis, a rare and lethal complication

Abstract

Amebiasis is still a major healthcare concern, especially in developing countries like Ecuador. The lack of sanitary control and hygiene measures make parasites infections still a burden for patients and physicians. Despite infections due to this parasites are usually mild, severe infections and fatal outcomes although rare still occur. Bowel perforation is a rare complication of amebiasis, and unfortunately, it continues to be almost fatal. We present a case of an Ecuadorian patient who presented to the emergency room with an acute abdomen, despite adequate surgery and critical care, the patient regrettably died. Bowel perforation due to Entamoeba histolytica was the final diagnosis.

https://ift.tt/2qRdmmh

Two cases of intestinal injuries due to seat belt without seat belt sign

Abstract

Although seat belts save lives after motor vehicle accidents, they may cause different types of injuries such as abdominal wall, intra-abdominal, neck and spine or vascular injuries. Seat belt sign indicates the severity of injuries, and usually the risk of intra-abdominal injuries is high when the seat belt sign exists. Here, we present two cases of intra-abdominal injuries caused by seat belts without seat belt sign.

https://ift.tt/2FhyGew

Compound volvulus: a case report and literature review

Abstract

Compound volvulus is an uncommon cause of acute abdomen and involves the twisting of the loops of ileum around the base of the sigmoid or vice versa. It is a surgical emergency that is often missed pre-operatively. We are reporting this case and reviewing the relevant literature to increase the awareness of this condition. A 37-year-old female presented at the Emergency Unit of our hospital with features thought to be acute peritonitis secondary to typhoid perforation and a differential of rupture appendix. She had laparotomy with the finding of a gangrenous twisted terminal ileum around the base of the sigmoid for which a right hemicolectomy and sigmoidopexy were done. Compound volvulus, though rare, can still be encountered in our environment. Prompt identification, adequate resuscitation and expedient intervention will reduce morbidity and mortality.

https://ift.tt/2qRdhyZ

A nutrition problem solved by a two-step endoscopic removal of a non-adjustable gastric band

Abstract

Bariatric surgery is an effective approach for weight loss and short-term improvement in metabolic disorders. Stenosis is a common complication of gastric banding. Balloon dilatation or gastrotomy has been employed in this setting. Few studies have indicated endoscopic removal of the band a feasible procedure. We present a 60-year-old female who underwent gastric banding in 1985 and suffered from late stenosis and malnutrition. Endoscopy revealed a severe stenosis. A two-step procedure was performed. Initially a coated stent was placed into the stenosis to achieve pressure necrosis. Two weeks later, the stent was removed and the band was removed endoscopically. The procedure was performed without any complications. The patient was discharged the next day. A follow-up after 2 months revealed no problems with stenosis or malnutrition. The procedure was a safe, efficient and convenient way of handling late stenosis after gastric banding.

https://ift.tt/2FhyCvi

Elective cholecystectomy as a rare presentation of metastatic breast cancer

Abstract

Cholecystectomy leads, occasionally, to the diagnosis of subclinical primary carcinomas and rarely metastatic tumors. The authors report the diagnosis of gallbladder metastasis after an elective cholecystectomy for symptomatic cholelithiasis. Histological examination disclosed a metastasis from a lobular breast carcinoma. Authors found no more than 25 case reports of breast cancer metastasis in English literature. Of those, only one reports first diagnosis after elective cholecystectomy with no radiological or macroscopic signs of malignancy. Rare clinical presentations, as in this case, should always be kept in mind specially in high volume centers. These rare presentations lack good quality evidence and challenge the best standard treatment regimen available. Multidisciplinary assessment is the key to maximize the risk/benefit of different treatment modalities. In this case report, although curative surgery was attempted, progression disease was typical of an advance staged disease.

https://ift.tt/2qIfxbH

Objective demonstration of improvement of neurogenic bowel dysfunction in a case of spinal cord injury following stem cell therapy

Abstract

Constipation in spinal cord injury patients is a frequent complication that leads to a reduction of quality of life, extensive psychological and economic strain on patients and healthcare systems. We report a 58-year-old man with an incomplete SCI secondary to L1 vertebral fracture, presented gait disorder with neurogenic bowel and bladder dysfunction. He received 300 million autologous mesenchymal stromal cells (MSC) in the subarachnoid space by lumbar puncture. After the third administration of MSC the patient had an important improvement in almost every functional scale of spinal cord injury, especially in the Krogh's Neurogenic Bowel Dysfunction scale. Our present observation supports recent clinical findings about the benefit of autologous stem cell therapy for the improvement of bowel dysfunction in patients suffering spinal cord injury.

https://ift.tt/2FhypZ2

Hemorrhagic necrosis of small bowel following small bowel obstruction as a late complication of sex reassignment surgery—a gap in transgender care

Abstract

For decades, Thailand has been at the forefront of sex reassignment surgeries. The neovagina created by recto-sigmoid vaginoplasty is much more complex procedure when compared with penile skin version technique, but yielded more satisfactory functional outcomes. We reported an unusual case of life-threatening small bowel obstruction following previously successful recto-sigmoid vaginoplasty in a transgender woman. Transgender individuals have unique healthcare needs. However, various gaps still exist to provide multi-disciplinary care for these patients. The rate of adhesive small bowel obstruction is highest in the early period of any intra-abdominal post-operative surgeries, but the risk remains life-long. Transgender women receiving complicated vaginoplasty should be instructed to continue long-term follow-up to ensure early detection and management of post-operative complications.

https://ift.tt/2qJHEaw

Synchronous volvulus of the sigmoid and transverse colon in a 26-year-old male

Abstract

We present a 26-year-old male patient who presented with complete bowel obstruction and peritonitis. His abdominal X-rays showed grossly distended large bowels with air–fluid levels. A differential diagnosis of sigmoid volvulus was entertained and the patient was taken for an exploratory laparotomy. Intraoperatively, we found a sigmoid volvulus and a concurrent transverse colon volvulus. A subtotal colectomy and colostomy was performed. The histopathology results showed mucosal and submucosal congestion, chronic inflammation, and no malignancy or dysplasia. Synchronous volvulus of the sigmoid and transverse colon is an extremely rare. Management includes endoscopic derotation and decompression followed by semi-elective surgery in non-complicated cases. When endoscopic decompression has failed or there is suspicion of necrosis or perforation, surgery is mandatory.

https://ift.tt/2FiG3lZ

Large intra-abdominal desmoid tumour posing diagnostic and therapeutic challenges, a case report

Abstract

We present the case of a 46-year-old gentleman originally from China who presented to the acute surgical assessment unit complaining of upper abdominal discomfort, dyspepsia and early satiety ongoing for the previous 6 months. On exam he had a palpable mass in the left upper quadrant. He underwent an esophagogastroduodenoscopy which was normal and later received a CT abdomen which identified a well-circumscribed soft tissue mass in the mesenteric fat and lying adjacent to the transverse colon with no obvious cleavage plane between them. Colonoscopy was then performed which was normal. After discussion at MDT he was taken for laparotomy. At laparotomy the mass was found to be adherent to major vessels, small bowel and large bowel necessitating an extended right hemicolectomy and small bowel resection. The mass itself could not be completely excised. Histology from the resected specimen confirmed desmoid tumour.

https://ift.tt/2qGgzos

Effects of oral ısotretinoin on normal and wounded nasal mucosa: an experimental study

Abstract

Background

We aimed to investigate the effect of systemic isotretinoin therapy on normal and wounded nasal septal mucosa histopathologically in an experimental rabbit model.

Methods

Circular mucosal defect with a 7 mm diameter was made in the left septum of 12 New Zealand white rabbits. The rabbits were divided into two groups (six rabbits in each group) oral isotretinoin was given with olive oil at the operation day to the first group. The control group was only oil given group. The harvested septum mucosas were divided into four groups (1—wounded-drug given side, 2—unwounded and drug-given side, 3—wounded-control and 4—unwounded-control side). The diameter of the defect, mucosal thickness, epithelial thickness, ciliated cell level, goblet cell level and inflammation were evaluated every week for 4 weeks.

Results

At both wounded and normal side, thinning of normal respiratory ciliated epithelium was observed in the postoperative period. In study group at the wounded side; mean mucosal thickness was measured 139.66 µ (± 26.24), and in the control group, mean mucosal thickness was 238.33 µ (± 39.7) at the wounded side. (p < 0.001). The difference between the groups in thickness of normal septal mucosa was also statistically significant (p = 0.016) [190 µ (± 14.6) and 256.66 µ (± 44.66)]. The average cilia level was observed 1.16 in the wounded study group, while the average level was 2.33 in the wounded control group (p = 0.012). Average score measurements of the regenerated mucosa suggested that isotretinoin–given wounded animals have reduced goblet cell recovery, compared to the control both on the regenerated and unwounded mucosas (p = 0.007, p = 0.002, respectively). Inflammation was significantly higher in the wounded isotretinoin group (p = 0.018).

Conclusion

Oral isotretinoin has negative effects on epithelial and ciliary regeneration, significantly reduces mucosal thickness and goblet cell counts of the normal and regenerated mucosa, causes severe inflammation and significant reactive changes.

https://ift.tt/2DgCJoS

Cervical inlet patch: an important cause of Globus pharyngeus

https://ift.tt/2PScnQS

Reply to: Angulation of the ossified stylohyoid complex and Eagle syndrome

https://ift.tt/2Di9xh7

Commentary to: Predictable prosthesis length on a high-resolution CT scan before a stapedotomy

https://ift.tt/2PScmfM

Angulation of the ossified stylohyoid complex and Eagle syndrome

https://ift.tt/2DgYK6Y

Evaluation of saccular and inferior vestibular nerve function in children with auditory neuropathy spectrum disorder

Abstract

Purpose

The aim of the current work was to evaluate the function of the saccule and inferior vestibular nerve in children with auditory neuropathy spectrum disorder (ANSD) by recording the cervical-evoked myogenic potentials (C-VEMP) on those children and to compare C-VEMP results in ANSD children of pre-lingual onset to those in ANSD children of post-lingual onset.

Methods

The study included 38 ANSD children of pre-lingual onset, 16 ANSD children of post-lingual onset, and 20 control children. All participant children were subjected to C-VEMP testing using 500 Hz tone burst stimuli.

Results

The vast majority of ANSD children of pre-lingual onset (35 out of 38; 92.1%) had bilateral intact C-VEMP response with C-VEMP parameters (amplitude, asymmetric ratio, latency, and inter-aural latency difference) that were not statistically different than those in the control children. Only three children had bilateral absent C-VEMP response. On the other hand, the majority of ANSD children of post-lingual onset (11 out of 16; 68.75%) had bilateral absent C-VEMP response. The remaining five children had bilateral intact C-VEMP response with C-VEMP parameters that were not statistically different than those in the control children.

Conclusions

The pathology of ANSD spares the saccule and inferior vestibular nerve in the vast majority of ANSD children of pre-lingual onset, while it involves them in the majority of ANSD of post-lingual onset reflecting different site(s) of lesion between the two ANSD categories. Such results have important clinical implications as regards to the management of ANSD in children.

https://ift.tt/2PSchZw

Authors’ response to Commentary on “Role of barium swallow pharyngoesophagography in the management of globus pharyngeus”

https://ift.tt/2DisYq9

Role of barium swallow pharyngoesophagography in the management of globus pharyngeus

https://ift.tt/2PQd7G7

Clinical classification of cervical necrotizing fasciitis

Abstract

Purpose

Necrotizing fasciitis of the craniofacial region is a rare and potentially life-threatening bacterial infectious disease. Odontogenic infections primarily spread along facial fascia and subcutaneous tissues, resulting in upper chest skin or thoracic necrosis. The purpose of this clinical classification was to demonstrate clinically important guidelines for early diagnosis and prompt management of CNF.

Methods

Although the incidence of cervical necrotizing fasciitis (CNF) is very rare in many developed countries, prompt management with appropriate initial diagnosis is essential, especially in tropical low-economic rural regions of African countries. Over the last 12 years, our charitable team in West Africa made clinical classifications of CNF according to onset time and spreading pattern to thoracic extension.

Results

CNF patients could be divided into two primary types, limited to neck type and extended to upper chest type. We also further categorized from each type into three different groups according to the CNF onset and clinical characteristics, including acute type with hematogenous spread within 2 weeks, subacute type with suppuration over 2 to 4 weeks, chronic type without suppuration over 4 weeks, multiple type with partial skin necrosis, island type with necrotic skin coverage, and broad type with whole skin necrosis.

Conclusions

These classifications will help decrease the mortality rate in severely infected patients.

https://ift.tt/2DhSNXm

Activations of group 2 innate lymphoid cells depend on endotypes of chronic rhinosinusitis

Abstract

Objective

Chronic rhinosinusitis (CRS) is a complicated disease with several variants caused by different cellular and molecular mechanisms. The characterization of this heterogeneity supports the definition that the disease consists of many endotypes, such as eosinophilic and neutrophilic CRS, and so on. This study aimed to explore group 2 innate lymphoid cells (ILC2s) in neutrophilic CRS without nasal polyps (CRSsNP) and with nasal polyps (CRSwNP), and evaluate ILC2s across characteristics of the disease.

Methods

Nasal biopsy samples were obtained from normal subjects or subjects with CRSsNP or CRSwNP during surgery. ILC2s were sorted and purified as CD45⁺Lin⁻CD127⁺CD4⁻CD8⁻CRTH2⁺CD161⁺ cells through flow cytometry, and were compared among three groups of subjects. Then, these samples were cultured in vitro, and inflammatory factors were assessed in tissue cultures. After that, human recombinant (rm) interleukin (IL)-33 or IL-17 were administered into the cultures, and we again examined relevant inflammatory substances.

Results

ILC2s were upregulated in neutrophilic CRSsNP and CRSwNP patients, and there were no statistical differences between them. Eosinophil cation protein (ECP), myeloperoxidase (MPO), IL-25, IL-33, IL-5, IL-13, interferon (IFN)-γ and IL-17 were increased in the cultures, however, only concentrations of MPO, IFN-γ and IL-17 were enhanced in CRSwNP tissues compared to CRSsNP ones. After administration of rmIL-33, ECP, IL-5 and IL-13 were all increased in tissues from CRSsNP and CRSwNP patients, however, there were no significant differences between them. Finally, we evaluated concentrations of several above inflammatory factors after the treatment of rmIL-17, and found that MPO and IFN-γ were enhanced in these two phenotypes of patients, and were elevated significantly in CRSwNP tissue cultures.

Conclusion

These findings show that ILC2s might be inactivated in neutrophilic CRSsNP and CRSwNP based on this pilot study.

https://ift.tt/2PXluiU

Expression of matrix metalloproteinases (MMPs-2, -7, -9, and -26) and tissue inhibitors of metalloproteinases (TIMPs-1 and -2) in pleomorphic adenomas and adenoid cystic carcinomas

Abstract

Purpose

To compare the immunohistochemical expression of matrix metalloproteinases-2, -7, -9 and -26 and tissue inhibitors of metalloproteinases-1 and -2 in pleomorphic adenomas and adenoid cystic carcinomas of the minor salivary glands.

Methods

Twenty cases of pleomorphic adenomas and 20 cases of adenoid cystic carcinomas were evaluated for the immunohistochemical expression of matrix metalloproteinases-2, -7, -9, and -26 and tissue inhibitors-1 and -2 in tumor parenchyma.

Results

Most pleomorphic adenomas and adenoid cystic carcinomas showed high expression of matrix metalloproteinases and tissue inhibitors, predominantly located in the tumor cells. There was no statistically significant difference in the expression of the metalloproteinases-2 (p = 0.359), -7 (p = 0.081), and -26 (p = 0 553), as well as the tissue inhibitors-1 (p = 0.657), and -2 (p = 0.248) between the parenchyma of the studied tumors. Only matrix metalloproteinase-9 showed a significant difference in expression between the two tumors, with adenoid cystic carcinoma showing a more intense staining for this gelatinase (p = 0.041).

Conclusions

The expression of the studied metalloproteinases suggests the involvement of these enzymes in the tissue remodeling process in pleomorphic adenomas and adenoid cystic carcinomas, but only MMP-9, significantly expressed in the adenoid cystic carcinomas, appears to be involved in the process of invasiveness and more aggressive behavior of these tumors. Additionally, results point that TIMPs-1 and -2 may have more complex functions besides metalloproteinase inhibition, which may be related to the pathogenesis and biological behavior of salivary gland tumors.

https://ift.tt/2DhSD2c

The effect of ectodermal dysplasia on volume and surface area of maxillary sinus

Abstract

Purpose

Ectodermal dysplasia (ED) is a congenital syndrome characterized by abnormal development of ectodermal structures, such as skin, hair, nails, teeth, or salivary glands. Patients with ED demonstrate craniofacial dysmorphology, midfacial hypoplasia and hypodontia. The aim of this study is to evaluate volume and surface area of maxillary sinus and craniofacial structures in patients with ectodermal dysplasia (ED) using cone-beam computed tomography (CBCT) images.

Methods

The CBCT images of 20 patients with ED (study group) and 20 healthy individuals (control) which are equal in terms of age and gender were evaluated. After a three-dimensional analysis and segmentation of each maxillary sinus, the volume and surface area were calculated. Craniofacial dimensions were measured on the sagittal planes of the CBCT images. The results of the ED and control groups were statistically compared.

Results

The right and left sinus volumes and surface areas were significantly lower in the ED group than in the control group (p < 0.05) while the SNA was significantly higher (p < 0.05). ANB and SN-GoMe were also lower in patients with ED than in controls (p < 0.05). The difference in SNB values between the two groups was not significant (p > 0.05).

Conclusions

The volumes and surface areas of both maxillary sinuses were found to be smaller in patients with ED compared to healthy individuals. Class III malocclusion with maxillary retrusion is a characteristic craniofacial feature of these patients.

https://ift.tt/2PX46uN

Evaluation of upper esophageal sphincter in benign vocal lesions

Abstract

Purpose

To evaluate upper esophageal sphincter (UES) activity in patients with benign glottic lesions.

Methods

Twenty-three patients with benign vocal fold (VF) lesions scheduled for manometric evaluation were enrolled as the study group (SG); 20 healthy subjects without vocal pathology or dysphagia were included as the control group (CG). UES residual, basal, relaxation time and peak pharyngeal pressures were evaluated by manometry. The reflux symptom index (RSI) and reflux finding score (RFS) were used to estimate the clinical findings of laryngopharyngeal reflux, and the voice handicap index (VHI) was measured in both groups. The patients' data were compared using the Mann–Whitney test and t test.

Results

The diagnoses in the SG were vocal nodules, vocal polyps, vocal cysts, sulcus vocalis, or Reinke's edema. The evaluation scores (RSI, RFS, and VHI) were significantly higher in the SG than in the CG (P < 0.05). UES basal and relaxation pressures, relaxation time, and peak pharyngeal pressures did not differ between the SG and the CG (P > 0.05).

Conclusion

UES manometric pressure was similar in patients with VF mucosal lesions and controls. Studies involving larger populations are necessary to confirm the manometric changes in the UES and to elucidate the pathogenesis of benign VF lesions.

https://ift.tt/2Dj65mv

Brain-Derived Neurotrophic Factor and Its Associations with Metabolism and Physical Activity in a Latino Sample

Metabolic Syndrome and Related Disorders, Ahead of Print.


https://ift.tt/2qKG7ku

Removal of Pb(II) from aqueous solutions by adsorption on magnetic bentonite

Abstract

Bentonite is a porous clay material that shows good performance for adsorbing heavy metals and other pollutants for wastewater remediation. In our previous study, magnetic bentonite (M-B) was prepared to solve the separation problem and improve the operability. In this study, we investigated the influence of various parameters on the Pb(II) adsorption of M-B, and it showed effective performance. About 98.9% adsorption removal rate was achieved within 90 min at adsorbent dose of 10 g/L for initial Pb(II) concentration of 200 mg/L at 40 °C and pH 5. The adsorption kinetic fit well by the pseudo-second-order model, and also followed the intra-particle diffusion model up to 90 min. Moreover, adsorption data were successfully reproduced by the Langmuir isotherm; the maximum adsorption capacity was calculated as 80.40 mg/g. The mechanism of interaction between Pb(II) ions and M-B was ionic exchange, surface complexation, and electro-static interactions. Thermodynamics study indicated that the reaction of Pb(II) adsorption on M-B was endothermic and spontaneous; increasing the temperature promoted adsorption. This study was expected to provide a reference and theoretical basis for the treatment of Pb-containing wastewater using bentonite materials.

https://ift.tt/2QDGlVM

An ultra-fast non-conventional waste management protocol to recycle of industrial fly ash into zeolite X

Abstract

An ultra-fast non-conventional waste management protocol was being designed and applied to recycle and reuse industrial coal fly ash (CFA) waste to generate highly pure nanozeolite X. Both microwave heating–assisted hydrothermal treatment method and ultrasonic waves–assisted hydrothermal method have been successfully used for the fast valorization of power plant CFA waste, and the results were compared with conversional valorization method for CFA conversion to zeolite. While conventional methods like hydrothermal treatment using sodium hydroxide took up to 4 days in valorization of the solid CFA waste into a useful zeolitic X material; the non-conventional methods using microwave irradiation of frequency 2.45 GHz and ultrasound irradiation of frequency 20 kHz took 90 min and 20 min respectively to fast-valorize the solid waste into highly pure zeolite material with high cation–exchanged capacity. The unconventional techniques, therefore, can be used in the large-scale valorization of solid waste recycling and reuse to yield highly pure zeolite.

https://ift.tt/2Ps20nm

Rituximab and Omalizumab for the Treatment of Bullous Pemphigoid: A Systematic Review of the Literature

Abstract

Background

Bullous pemphigoid (BP) is the most common autoimmune blistering skin disease worldwide. Systemic corticosteroids are considered the mainstay of therapy; however, they may cause significant adverse effects and treatment failures, so additional therapeutic modalities with better safety profiles are required. Rituximab and omalizumab are novel biologic agents administered in recent years for the treatment of BP, yet data regarding their use in the disease are limited.

Objective

Our objective was to systematically review the current literature regarding the use of rituximab and omalizumab for the treatment of BP to evaluate their safety and efficacy.

Methods

A systematic review of all publications evaluating patients with BP treated with rituximab or omalizumab was performed. The primary outcome was clinical response; secondary outcomes were adverse events and recurrence rate.

Results

The systematic review included 35 publications (84 patients: 62 receiving rituximab and 22 receiving omalizumab). In total, 61 of 63 patients had not experienced disease control with systemic corticosteroids before receiving the biologic treatment. Complete response rates were 85% and 84% for rituximab and omalizumab, respectively. The recurrence rate was considerably lower with rituximab (29%) than with omalizumab (80%). Mean time to recurrence was 10.2 and 3.4 months, and adverse effects occurred in 24% and 20% of the patients, respectively.

Conclusions

Available data, although potentially limited because of publication bias, suggest that rituximab and omalizumab have similar safety profiles and provide clinical benefit for patients with BP. The reviewed data indicated that rituximab resulted in lower recurrence rates and a longer time until recurrence than omalizumab.

https://ift.tt/2zLPB2U

Annual Congress of the European Society for Medical Oncology (ESMO): Munich, Germany, 19–23 October 2018

https://ift.tt/2T8ztS0

Acknowledgement to Referees

https://ift.tt/2zPNefD

Both Bupivacaine and Levobupivacaine inhibit colon cancer cell growth but not melanoma cells in vitro

Abstract

Background

Retrospective studies indicate that the use of regional anaesthesia causes a reduction in cancer recurrence after oncological surgery, which could be due to anaesthetic's negating effect on immunosuppression related to the surgical stress response. Local anaesthetics may also exert direct suppressive effects on malignant cells, an area where further investigation is urgently needed.

Methods

Human colon cancer cells and human melanoma cells were cultured and then treated with 1 mM bupivacaine or levobupivacaine for up to 24 or 48 h. Their migratory ability was measured by scratch assay, proliferation determined with Ki67 immunofluorescence staining, and apoptosis accessed with annexin V and PI staining on flow cytometry. The effects of bupivacaine and levobupivacaine on cellular signaling and molecular response, specifically, on endoplasmic reticulum stress (ERS), were studied with immunostaining and western blot.

Results

In colon cancer cells, treatment with bupivacaine and levobupivacaine significantly inhibited cell migration (**p < 0.01, ***p < 0.001; n = 4) and proliferation (**p < 0.01; n = 4), while increasing the expression of CHOP (***p < 0.001; n = 4) and decreased the expression of Grp78 (*p < 0.05; n = 4). These effects were not mirrored by melanoma cells, such that no significant increase in apoptosis was seen in either melanoma cell lines following treatment.

Conclusion

These in vitro data suggested that both bupivacaine and levobupivacaine suppress colorectal adenocarcinoma cell proliferation and migration, which are concurrent with increased endoplasmic reticulum stress. Conversely, melanoma cells are more resilient to these two commonly used local anaesthetics. Further in vivo studies or clinical trials are needed.

https://ift.tt/2QJ2br6

Mutated Thyroid Hormone Transporter OATP1C1 Associates with Severe Brain Hypometabolism and Juvenile Neurodegeneration

Thyroid, Volume 28, Issue 11, Page 1406-1415, November 2018.


https://ift.tt/2T8ZekT

Future Meetings

Thyroid, Volume 28, Issue 11, Page 1559-1560, November 2018.


https://ift.tt/2zLSLDV

Physician Choice of Hypothyroidism Therapy: Influence of Patient Characteristics

Thyroid, Volume 28, Issue 11, Page 1416-1424, November 2018.


https://ift.tt/2TaLNkq

Metal Coordinated Poly-Zinc-Liothyronine Provides Stable Circulating Triiodothyronine Levels in Hypothyroid Rats

Thyroid, Volume 28, Issue 11, Page 1425-1433, November 2018.


https://ift.tt/2zQggLM

Regional Influences on Nutritional Iodine Status of Pregnant Women in Pakistan

Thyroid, Volume 28, Issue 11, Page 1538-1546, November 2018.


https://ift.tt/2T6BUEw

Diagnostic Performance of Ultrasound-Based Risk-Stratification Systems for Thyroid Nodules: Comparison of the 2015 American Thyroid Association Guidelines with the 2016 Korean Thyroid Association/Korean Society of Thyroid Radiology and 2017 American Congress of Radiology Guidelines

Thyroid, Volume 28, Issue 11, Page 1532-1537, November 2018.


https://ift.tt/2zNRc8n

Patients with Oncocytic Variant Papillary Thyroid Carcinoma Have a Similar Prognosis to Matched Classical Papillary Thyroid Carcinoma Controls

Thyroid, Volume 28, Issue 11, Page 1462-1467, November 2018.


https://ift.tt/2TaLCFM

Feasibility of Intraoperative Neuromonitoring During Thyroid Surgery Using Transcartilage Surface Recording Electrodes

Thyroid, Volume 28, Issue 11, Page 1508-1516, November 2018.


https://ift.tt/2zNR2xN

Food OIT is Superior to Food Avoidance

Over the last decade much attention has focused on the possibility of using oral immunotherapy (OIT) as a therapeutic approach for treatment of IgE-mediated food allergies, a disease without a Food and Drug Administration (FDA)-approved disease-modifying therapy. Peanut allergy has been a particular concern because of its persistent nature and association with potentially life-threatening reactions. The OIT literature has been criticized for heterogeneity in trial design with disparities in identification of allergic individuals by double blind placebo controlled food challenges (DBPCFC), differences in desensitization approaches and maintenance doses of allergen, absence of a standardized product, inconsistency in the primary endpoint (e.g.

https://ift.tt/2qI49fL

An Ounce of Caution: Superparamagnetic Iron Oxide Nanoparticle Based MRI Contrast Associated Anaphylaxis

Superparamagnetic iron oxide nanoparticles (SPIONs) are increasingly utilized alternatives to gadolinium-based Magnetic Resonance Imaging (MRI) contrast agents in the diagnosis of osteomyelitis in diabetic patients with renal failure [1]. Ferumoxytol is approved by the United States Food and Drug Administration (FDA), the European Medicines Agency, for intravenous (IV) use for iron replacement therapy [1-3]. Ferumoxytol is composed of an iron-carbohydrate complex that minimizes dissociation and appearance of free iron into the serum.

https://ift.tt/2QEECiY

The Effect of Delayed and Early Diagnosis in Siblings, and Importance of Newborn Screening for SCID

Severe combined immunodeficiency (SCID) is a form of primary immunodeficiency that affects both cellular and humoral immunity, caused by genetic defects in lymphocyte development and function. There are over 20 known genes mutations that lead to SCID including defects that cause radiosensitivity due to impairment in DNA repair (1). Mutations in DCLREC1 resulting in absent or dysfunctional Artemis protein are the most common cause of SCID associated with increased radiosensitivity and were identified as the etiology of the unusually high incidence of T-B-NK+ SCID in Athabascan-speaking tribes, Apache and Navajo (2,3).

https://ift.tt/2qJc1xO

CORRECTION

https://ift.tt/2DAyGoh

Local Sleep

The historic sleep regulatory paradigm invokes "top-down" imposition of sleep on the brain by sleep regulatory circuits. While remaining conceptually useful, many sleep phenomena are difficult to explain using that paradigm, including, unilateral sleep, sleep-walking, and poor performance after sleep deprivation. Further, all animals sleep after non-lethal brain lesions, regardless of whether the lesion includes sleep regulatory circuits, suggesting that sleep is a fundamental property of small viable neuronal/glial networks.

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What are the determinants of children’s sleep behavior? A systematic review of longitudinal studies

To develop evidence-based healthy sleep interventions for children, this review provides insight into the behavioral determinants of sleep behavior. Hence the objective of this review is to systematically review the longitudinal evidence on determinants of children's sleep behavior.Studies were identified from searches in PubMed, PsycINFO, and Web of Science, until January 2017. Longitudinal studies investigating the association between potential determinants and sleep behavior (duration, quality and timing) in healthy children aged 4-12 years were included.

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Serum levels of oxidative stress indicators and antioxidant enzymes in Bell palsy

Nazim Bozan, MD; Ömer Faruk Kocak, MD; Canser Y&inodot;lmaz Demir, MD; Mehmet Emre Dinc, MD; Koray Avc&inodot;, MD; Halit Demir, PhD; Ahmet Faruk K&inodot;roglu, MD

Abstract

We conducted a prospective study to comparatively evaluate serum levels of malondialdehyde, an oxidative stress indicator, and the antioxidant enzymes glutathione, catalase, and superoxide dismutase in patients with Bell palsy. Our study population was made up of 30 patients with Bell palsy-15 men and 15 women, aged 25 to 68 years (mean: 50.4)-who were seen in the Department of Otorhinolaryngology at a tertiary care medical center. For comparison purposes, we recruited 26 healthy age- and sex-matched controls-16 men and 10 women, aged 40 to 67 years (mean: 54.3). Serum samples were obtained from all participants before the initiation of steroid treatment to the Bell palsy patients. A correlation was sought between demographic data and serum levels of malondialdehyde, glutathione, catalase, and superoxide dismutase. We found that the serum level of malondialdehyde was significantly higher in the Bell palsy group and that the levels of glutathione, catalase, and superoxide dismutase were all significantly lower (p < 0.001 for all comparisons). Correlation analysis revealed that only superoxide dismutase levels were positively correlated with age (r = 0.347, p = 0.009). We suggest that oxidative stress and antioxidant mechanisms may be involved in the pathogenesis of Bell palsy. In this context, serum levels of malondialdehyde, glutathione, catalase, and superoxide dismutase may serve as biomarkers in the diagnosis and follow-up of Bell palsy. Confirmation of the validity, reliability, and reproducibility of these findings necessitates further prospective, randomized clinical trials in larger populations.

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Desmoid tumors of the head and neck: Two decades in a single tertiary care unit and review of the literature

Aleksi Schrey, MD, PhD; Maria Gardberg, MD, PhD; Riitta Parkkola, MD, PhD; Ilpo Kinnunen, MD, PhD

Abstract

Desmoid tumors (DTs) of the head and neck have typically been classified as extra-abdominal, although the anatomic challenges of the head and neck warrant consideration of these DTs as a special entity. We present a review of DTs and describe our series of five patients with DTs of the head and neck treated within 2 decades. Altogether, 53 patients with DTs treated surgically at a tertiary care center over a 20-year period were retrospectively reviewed. Outcomes of the treatment of DTs of the head and neck (n = 5) were analyzed as a case series. DTs are rare, histologically benign, but locally aggressive tumors. In our series of 5 patients with head and neck DTs, no patient experienced a recurrence during a median follow-up of 47.5 months (range 13 to 150), although all had positive histologic margins. The functional integrity of vital structures over meticulous radicality of the tumor resection must be considered, especially in the head and neck.

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Long-term use of Le Fort I osteotomy for the management of nasopharyngeal rhinosporidiosis: A case series

Vikram Shetty, DNB; Akshaya Kulkarni, MDS; Suman Banerjee, MDS

Abstract

Rhinosporidiosis is a rare, chronic, granulomatous infection of the mucous membranes that mainly involves the nose and nasopharynx; it occasionally involves the pharynx, conjunctiva, larynx, trachea and, rarely, the skin. The characteristic clinical features of this disease include the formation of painless polyps in the nasal mucosa or the nasopharynx that bleed easily on touch. At our center, excision of the lesion with a Le Fort I osteotomy is carried out in patients (1) in whom two or more previous attempts at excision of biopsy-proven rhinosporidiosis arising from the nasal mucosa was carried out or (2) in whom the rhinosporidiosis arises from the nasophayrngeal mucosa and/or extranasal sites. In this article we retrospectively present 7 cases in which, according to our inclusion criteria, complete excision of the lesion was carried out with a Le Fort I osteotomy. Excellent visualization of the entire maxillary and ethmoidal air cells after the down-fracture of the maxilla helped in the total removal of the lesions. Most of these lesions had multiple points of origin through the nasal, maxillary, and ethmoidal mucosa; the excellent visualization enabled direct cauterization of all these points of origin. The mean follow-up period was 7.96 years, and all patients were disease-free by the time the study was prepared. This article presents details of the treatment protocol and technique followed at our center for the treatment of nasopharyngeal rhinosporidiosis and the details of long-term follow-up. Through this study we hope to prove the efficacy of Le Fort I osteotomy in the definitive management of nasopharyngeal rhinosporidiosis.

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A rare case of coexisting lacrimal sac adenocarcinoma and transitional cell carcinoma

Tsutomu Nomura, MD, DDS, PhD; Daisuke Maki, MD; Fumihiko Matsumoto, MD, PhD; Taisuke Mori, DDS, PhD; Seichi Yoshimoto, MD, PhD

Abstract

Lacrimal sac tumors are rare and difficult to diagnose. We present a case of coexisting lacrimal sac adenocarcinoma and transitional cell carcinoma in a 73-year-old woman who presented with swelling of the inner canthus. Biopsy identified the growth as an adenocarcinoma. After dissection of the lacrimal carcinoma via a lateral rhinotomy, histopathologic examination confirmed the adenocarcinoma and identified a coexisting transitional cell carcinoma. A recurrence developed, but only of the adenocarcinoma component. For the recurrence, the patient was treated with wide resection, including the orbital contents, and subsequent irradiation. At 49 months postoperatively, the patient remained alive but with a lung metastasis. Wide resection was the key to treatment.

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Oropharyngeal histoplasmosis in an HIV-negative patient

Mohamedkazim Alwani, MD; Todd J. Wannemuehler, MD; Don-John Summerlin, DMD, MS; Marion E. Couch, MD, PhD, FACS

The occurrence of histoplasmosis in immunocompetent patients presents a unique diagnostic challenge due to its rarity and mimicry of other disease processes.

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Endoscopic view of a posterior nasal and nasopharyngeal vascular plexus

Dewey A. Christmas, MD; Joseph P. Mirante, MD, MBA, FACS; Eiji Yanagisawa, MD, FACS

Woodruff described a predominantly venous plexus in the posterior nasal mucosa located in the posteroinferior meatus beneath and posterior to the inferior turbinate.

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Eagle syndrome: Transient ischemic attack and subsequent carotid dissection

Thomas Sullivan, MD; Jordan Rosenblum, MD

Prophylactic contralateral styloidectomy was considered for this patient, but he was deemed a poor surgical candidate given his underlying cardiomyopathy and anticoagulation.

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Risk of developing sudden sensorineural hearing loss in patients with hepatitis B virus infection: A population-based study

Yao-Te Tsai, MD; Ku-Hao Fang, MD; Yao-Hsu Yang, MD, MSc; Meng-Hung Lin, PhD; Pau-Chung Chen, MD, PhD; Ming-Shao Tsai, MD; Cheng-Ming Hsu, MD

Abstract

Sudden sensorineural hearing loss (SSNHL) has significant impact on quality of life. It may result from viral infection, but the relationship between hepatitis B virus (HBV) infection and SSNHL remains uncertain. To investigate the risk of developing SSNHL in patients with HBV, we conducted a nationwide, population-based, retrospective cohort study from the Taiwan National Health Insurance Research Database. A total of 33,234 patients diagnosed with HBV infection and 132,936 control subjects without viral hepatitis were selected from claims made from 2000 to 2008. Each patient was followed for at least 5 years to identify new-onset SSNHL. Among the 166,170 patients, 279 patients (303,793 person-years) from the HBV cohort and 845 patients (1,225,622 person-years) from the control cohort were diagnosed with SSNHL. The incidence of SSNHL was 1.33-fold higher in the HBV group than in the control group (0.92 vs. 0.69 per 10,000 person-years), with an adjusted hazard ratio (HR) of 1.315 (95% confidence interval [CI] = 1.148 to 1.506) calculated using a Cox proportional hazard regression model. We also observed that HBV patients in the 50 to 64 years of age subgroup showed the highest incidence of SSNHL and the highest adjusted hazard ratio (HR = 2.367; 95% CI = 1.958 to 2.861). Patients with HBV infection had a higher risk of acquiring SSNHL than patients without viral hepatitis. For the early detection and timely treatment of SSNHL, clinicians should be aware of the increased risk of SSNHL in HBV patients and arrange auditory examinations for those complaining about acute hearing change.

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Iatrogenic external auditory canal cholesteatoma with mastoid erosion

Yen-Hui Lee, MD; Chih-Yu Hu, MD; Wen-Yu Chuang, MD; Kai-Chieh Chan, MD

Large, complicated iatrogenic EAC cholesteatomas usually require surgical management, with the selected technique generally based on the extent of disease and the surgeon's preference.

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Primary cutaneous histoplasmosis

Blake Raggio, MD

Diagnosis of cutaneous histoplasmosis hinges on evidence of fungus in the wound and absence of systemic disease.

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Effects of l-carnitine administration on health-related quality of life during cisplatin-based chemoradiotherapy in patients with head and neck squamous cell carcinoma

Cancer-related fatigue impairs daily functioning and negatively impacts health-related quality of life (HRQoL). Our previous study revealed that cisplatin-based chemoradiotherapy (CRT) impairs the carnitine system and carnitine deficiency leads to poor physical functioning. This open label, randomized, controlled prospective study investigated the effects of l-carnitine administration on plasma carnitine concentration, CRT-induced fatigue, and decline in HRQoL in patients with head and neck squamous cell carcinoma (HNSCC).

https://ift.tt/2DBFvGe

Efficacy of anti-PD-1 therapy in a patient with brain metastasis of parotid carcinoma: A case report

CheckMate 141, an open-label, randomized phase III trial of nivolumab, indicated that treatment with nivolumab prolonged overall survival of patients with platinum-refractory, recurrent head and neck squamous cell carcinoma. Herein, we describe a case of brain metastasis of parotid carcinoma in which a good response was achieved after nivolumab treatment. The patient was a 67-year-old woman with parotid carcinoma (cT4bN0M0) who received induction chemotherapy followed by chemoradiation. Computed tomography and magnetic resonance imaging performed 10 weeks after the primary treatment revealed a residual tumor and brain and lung metastases.

https://ift.tt/2RS2U9A

Cochlear implantation after canal wall down mastoidectomy — Outcomes after partial mastoid obliteration

To describe and evaluate the partial mastoid obliteration of the so-called radical mastoid cavity after canal-wall down mastoidectomy (CWD) for cochlear implantation (CI) compared to overclosure of the external ear canal as two stage procedures.

https://ift.tt/2DBdSx2

Atopic dermatitis: the relationship to temperature and seasonality in the United States

Abstract

Background

Climatologic factors may affect atopic dermatitis (AD), and there have been few explorations into this topic. Because of its size, the United States (US) has a broad range of climates. The objective of this study is to assess the relationship between ambulatory office visits physicians for AD with temperature and season.

Methods

US region‐specific satellite‐derived data air temperature from the North America Land Data Assimilation System from 1993 to 2011 were studied in relation with ambulatory AD visits to all physicians from the National Ambulatory Medical Care Survey.

Results

Over the 19‐year study period, in no regional location in the US is there clear evidence of a winter flare of AD. The greatest numbers of visits occur in May and June, and smaller peaks also occur in January and October. As the ambient air temperature increases, the likelihood of an AD visit rises (P < .0001). In the South, the hottest US climate, summer is responsible for substantially more AD visits. In every other region and the US overall, the largest number of AD visits occur in spring. AD visits appear to display regional variation in comparing the Northeast, South, Midwest, and West.

Conclusions

Increased temperatures predict increased likelihood of AD office visits.

https://ift.tt/2z5qWqq

Health care costs and resource utilization for different asthma severity stages in Colombia: a claims data analysis

Asthma is one of the most common chronic respiratory conditions worldwide. Asthma-related economic burden has been reported in Latin America, but knowledge about its economic impact to the Colombian health car...

https://ift.tt/2OC0xWg

Increase of global DNA methylation patterns in beauty salon workers exposed to low levels of formaldehyde

Abstract

Formaldehyde (FA) is a carcinogenic aldehyde illegally added to creams as a hair straightening agent for the Brazilian blowout (BB). This study aimed to investigate the possible effects of occupational exposure to FA on global DNA methylation in salon workers with different exposure levels. FA exposure was monitored using environmental and biological measurements. The study included 49 salon workers divided by FA levels in the workplace into group A (FA < 0.01 ppm; n = 8), group B (0.03 ppm < FA < 0.06 ppm; n = 15), and group C (0.08 ppm < FA < 0.24 ppm; n = 26). The global DNA methylation levels were 3.12%, 4.55%, and 4.29% for groups A, B, and C, respectively, with statistically higher values for groups B and C compared to group A (p = 0.002). A correlation was found between FA in passive samplers and global DNA methylation (r_s = 0.307, p = 0.032). Additionally, when only taking into account the hairdressers that performed the BB on clients instead of the whole group, a stronger correlation was observed between FA in personal passive samplers and global DNA methylation (r_s = 0.764, p = 0.006). For the first time, an increase in DNA methylation was observed in subjects occupationally exposed to FA. In conclusion, our results indicated that even low levels of FA exposure could cause a disturbance in DNA methylation, leading to epigenetic changes, which is associated with cancer development. These data suggest a possible contribution of FA to cancer development through occupational exposure.

https://ift.tt/2JVIDNk

Summary of the Third World Congress on Thyroid Cancer

Thyroid, Volume 28, Issue 11, Page 1401-1405, November 2018.


https://ift.tt/2DhCfPf

Vestibular Evoked Myographic Correlation

Abstract

This work started from the hypothesis that the physiological processes giving rise to the vestibular evoked myogenic potential (VEMP) can be induced not only by transient sounds but also by a continuous stimulation with a stochastic signal. The hypothesis is based on the idea that the number of motor unit action potentials (MUAPs) decreases after a momentary amplitude increase of the effective stimulus, whereas a momentary amplitude decrease has the opposite effect. This concept was theoretically analyzed by assuming that the effective stimulus is closely related to the envelope of the stimulus actually presented. The analysis led to the prediction that the cross-correlation function of the effective stimulus and the measured electromyogram (EMG) has VEMP-like properties. Experiments confirmed this prediction, thus providing evidence of a novel electrophysiological response: the vestibular evoked myographic correlation (VEMCorr). The methodological approach corresponded to a conventional VEMP study, except that the stimulus (delivered with a hand-held minishaker) comprised not only a series of 500-Hz tone pulses (classical VEMP measurement, for comparison) but also sequences of narrow-band noise with a center frequency of 500 Hz (VEMCorr measurement). Each of the 12 test persons showed a clear VEMCorr. Moreover, VEMP and VEMCorr largely resembled each other, as predicted. Apparently they are two different expressions of a more general mechanism that leads to a roughly linear relationship between stimulus envelope and expectation of the EMG. Future applications of the VEMCorr could exploit that a continuous-stimulation paradigm allows for varying the center frequency of the stimulus without changing the relative bandwidth.

https://ift.tt/2PoiWed

Performance and emission characteristics of CNG-fueled compression ignition engine with Ricinus communis methyl ester as pilot fuel

Abstract

Surge in petroleum prices, its drying sources and degradation in air quality focused interest on renewable energy sources as substitute for existing fuels for internal combustion engines. This study highlights the combustion, performance, and emission characteristics of diesel engines fueled with compressed natural gas (CNG) as primary fuel and castor (Ricinus communis) oil methyl ester (COME) as pilot fuel. COME was produced from non-edible grade Ricinus communis oil. The biodiesel fuel properties and characterization was done as per ASTM D6751 specifications. The CNG was inducted through inlet manifold fumigation at a consistent flow rate of 15 l/min under dual-fuel mode. It is evident from the test results that B20-CNG yields brake thermal efficiency of 23.6% when compared to 25 and 27% for D-CNG and diesel fuel, respectively. The peak cylinder gas pressure was lower in dual-fuel mode when compared to conventional diesel. The emission results show increase in NO_x emission by 24.5 and 28.4% for D-CNG and B20-CNG, respectively when compared to baseline diesel fuel at full engine load. There was increase in HC emission by 6.7 and 11% whereas CO emissions decreased by 31.6 and 37.4% for B20-CNG and D-CNG, respectively at similar operating conditions. Reduction in smoke opacity by 49.4 and 59.6% was achieved respectively for D-CNG and B20-CNG under dual-fuel mode. On the whole, COME exhibits a better pilot fuel choice for dual-fuel combustion mode in comparison to conventional fossil petroleum diesel in terms of combustion, performance, and emissions characteristics.

https://ift.tt/2qHUtlm

A study on the evaluation of water-bloom using image processing

Abstract

This study utilized remote sensing techniques using an unmanned aerial vehicle (UAV) with an attached multispectral sensor to monitor the Nakdong River. In this study, chlorophyll-a, an indicator of water quality and the normalization difference vegetation index (NDVI), which indicates the vitality of plant growth was employed. NDVI images were generated using georeferenced and Orthomosaic images. The data (field samples) used to conduct the study was collected in September 2017. The relationship between the chlorophyll-a concentrations and NDVI was then examined. The results of the relationship can be used in monitoring of green algae for water quality management.

https://ift.tt/2FjpRB3

Quality of life assessment in patients with HNF1A-MODY and GCK-MODY

Abstract

Aim

The impact of maturity onset diabetes of the young (MODY) on quality of life (QoL) has never been examined. We assessed disease impact on QoL among patients with HNF1A-MODY and GCK mutation carrier status.

Methods

The study included 80 patients with HNF1A-MODY and 89 GCK gene mutation carriers. We also examined 128 type 1 diabetes (T1DM) patients for comparison. Diabetes-specific QoL was assessed using the Audit of Diabetes Dependent Quality of Life questionnaire.

Results

HNF1A-MODY and GCK-MODY groups had similar mean age (41.7 vs. 38.0 years, respectively) and BMI (24.1 vs. 24.3 kg/m²), whereas T1DM patients were on average younger (34.2 years) with similar BMI (25.0 kg/m²). Less than a third of GCK mutation carriers were on pharmacotherapy (n = 20, 31%), while the majority of HNF1A mutation carriers used oral drugs or insulin (n = 66, 82.5%). While current QoL was similar across the three groups (p = 0.66), two other major indices—the impact of diabetes on QoL and the average weighted impact (AWI)—differed among them (p < 0.001 for both comparisons). The impact of diabetes on patient QoL and AWI observed in both MODY groups was smaller than in T1DM. Etiological diagnosis of diabetes and a diagnosis of retinopathy were the only independent factors influencing the impact of diabetes on QoL and AWI in regression analysis. In HNF1A-MODY, all three major indices of QoL were more heavily influenced for patients on insulin in comparison to other treatment sub-groups.

Conclusion

MODY has a smaller negative impact on QoL compared to T1DM. Mode of treatment further stratifies QoL decline for HNF1A-MODY subjects.

https://ift.tt/2JXXhUj

Using Shannon entropy to model turbulence-induced flocculation of cohesive sediment in water

Abstract

Turbulence-induced flocculation of cohesive fine-grained sediment plays an important role in the transport characteristics of pollutants and nutrients absorbed on the surface of sediment in estuarine and coastal waters via the complex processes of sediment transport, deposition, resuspension and consolidation. In this study, the concept of Shannon entropy based on probability is applied to modelling turbulence-induced flocculation of cohesive sediment in water. Using the hypothesis regarding the cumulative distribution function, the function of floc size with flocculation time is derived by assuming a characteristic floc size as a random variable and maximizing the Shannon entropy, subject to certain constraints. The Shannon entropy-based model is capable of modelling the variation in floc size as the flocculation time progresses from zero to infinity. The model is tested against some existing experimental data from the literature and against a few deterministic mathematical models. The model yields good agreement with the observed data and yields better prediction accuracy than the other models. The parameter that has been incorporated into the model exhibits an empirical power-law relationship with the flow shear rate. An empirical model formulation is proposed, and it exhibits high prediction accuracy when applied to existing experimental data.

https://ift.tt/2QxikzA

Quality of life assessment in patients with HNF1A-MODY and GCK-MODY

Abstract

Aim

The impact of maturity onset diabetes of the young (MODY) on quality of life (QoL) has never been examined. We assessed disease impact on QoL among patients with HNF1A-MODY and GCK mutation carrier status.

Methods

The study included 80 patients with HNF1A-MODY and 89 GCK gene mutation carriers. We also examined 128 type 1 diabetes (T1DM) patients for comparison. Diabetes-specific QoL was assessed using the Audit of Diabetes Dependent Quality of Life questionnaire.

Results

HNF1A-MODY and GCK-MODY groups had similar mean age (41.7 vs. 38.0 years, respectively) and BMI (24.1 vs. 24.3 kg/m²), whereas T1DM patients were on average younger (34.2 years) with similar BMI (25.0 kg/m²). Less than a third of GCK mutation carriers were on pharmacotherapy (n = 20, 31%), while the majority of HNF1A mutation carriers used oral drugs or insulin (n = 66, 82.5%). While current QoL was similar across the three groups (p = 0.66), two other major indices—the impact of diabetes on QoL and the average weighted impact (AWI)—differed among them (p < 0.001 for both comparisons). The impact of diabetes on patient QoL and AWI observed in both MODY groups was smaller than in T1DM. Etiological diagnosis of diabetes and a diagnosis of retinopathy were the only independent factors influencing the impact of diabetes on QoL and AWI in regression analysis. In HNF1A-MODY, all three major indices of QoL were more heavily influenced for patients on insulin in comparison to other treatment sub-groups.

Conclusion

MODY has a smaller negative impact on QoL compared to T1DM. Mode of treatment further stratifies QoL decline for HNF1A-MODY subjects.

https://ift.tt/2JXXhUj

Peak airway pressure is lower during pressure-controlled than during manual facemask ventilation for induction of anesthesia in pediatric patients—a randomized, clinical crossover trial

Abstract

Purpose

Facemask ventilation during the induction of general anesthesia in paediatric patients remains a challenge as it may result in hypoxic conditions and gastric insufflation with subsequent regurgitation and aspiration. So far, it is unclear if pressure-controlled or manual facemask ventilation is preferable in children. We hypothesized that pressure-controlled ventilation in apnoeic children results in lower peak airway pressure and flow rates compared to manual ventilation at comparable respiratory rates and tidal volumes.

Methods

Sixty-two lung-healthy children undergoing scheduled ear-nose-throat surgery were included in the study. After the induction of anesthesia, the patient's lungs were consecutively ventilated via a facemask in either manual or pressure-controlled mode, in randomized order. The primary outcome measure was peak airway pressure. Secondary outcome measures included positive end-expiratory pressure, airway compliance, tidal volume and airway flow.

Results

Data of 52 patients could be analyzed. Pressure-controlled ventilation resulted in a lower mean and peak inspiratory pressure (both p < 0.001), airway pressure amplitude (p = 0.01) and inspiratory peak flow rate (p = 0.005) compared to manual ventilation. The ratio of inspiration to expiration time was lower in pressure-controlled ventilation compared to manual ventilation (p < 0.001).

Conclusion

Pressure-controlled facemask ventilation during induction of anesthesia in pediatric patients results in lower airway pressure, and lower flow rates compared to manual ventilation, at comparable tidal and minute volumes.

https://ift.tt/2qJFeIW

Vestibular Evoked Myographic Correlation

Abstract

This work started from the hypothesis that the physiological processes giving rise to the vestibular evoked myogenic potential (VEMP) can be induced not only by transient sounds but also by a continuous stimulation with a stochastic signal. The hypothesis is based on the idea that the number of motor unit action potentials (MUAPs) decreases after a momentary amplitude increase of the effective stimulus, whereas a momentary amplitude decrease has the opposite effect. This concept was theoretically analyzed by assuming that the effective stimulus is closely related to the envelope of the stimulus actually presented. The analysis led to the prediction that the cross-correlation function of the effective stimulus and the measured electromyogram (EMG) has VEMP-like properties. Experiments confirmed this prediction, thus providing evidence of a novel electrophysiological response: the vestibular evoked myographic correlation (VEMCorr). The methodological approach corresponded to a conventional VEMP study, except that the stimulus (delivered with a hand-held minishaker) comprised not only a series of 500-Hz tone pulses (classical VEMP measurement, for comparison) but also sequences of narrow-band noise with a center frequency of 500 Hz (VEMCorr measurement). Each of the 12 test persons showed a clear VEMCorr. Moreover, VEMP and VEMCorr largely resembled each other, as predicted. Apparently they are two different expressions of a more general mechanism that leads to a roughly linear relationship between stimulus envelope and expectation of the EMG. Future applications of the VEMCorr could exploit that a continuous-stimulation paradigm allows for varying the center frequency of the stimulus without changing the relative bandwidth.

https://ift.tt/2PoiWed

Extracorporeal shockwave treatment impedes tooth movement in rats

Abstract

Background

Accelerated tooth movement has been a topic of interest for orthodontic research recently. Surgically facilitated orthodontic treatment has been shown to be an effective approach to accelerate tooth movement; however, it remains invasive, requires additional surgery, and may increase post-operative complications. In this study, we evaluate the effects of extracorporeal shockwave treatment (ESWT), a non-invasive approach to regenerate alveolar bone, on orthodontic tooth movement in rats.

Materials and methods

Seventy-two male rats, aged 10 weeks old, were subjected to 10-cN closed-coil nickel-titanium springs for unilateral maxillary first molar tooth movement. One group of rats received a single treatment of extracorporeal shockwave treatment at 500 impulses at energy flux density 0.1 mJ/mm², with a pulse rate of 5 pulses per second immediately after spring installation while the non-ESWT-treated group served as a control group. The rats were sacrificed at day 3, 7, 14, 21 and 28 for tooth movement evaluation and sample analyses. Faxitron radiography, histological, double bone labeling and gene expression analyses were performed. Serum biochemistry was evaluated at day 3, 7 and 28 of the study. Kruskal-Wallis analysis of variance was used to determine the mean difference among groups, and multiple comparisons were analyzed by Mann-Whitney-U tests with a significance level = 0.05.

Results

The results demonstrated that tooth movement in the ESWT-treated rats (0.11 ± 0.07 mm) was impeded compared to the tooth movement in the non-ESWT-treated rats (0.44 ± 0.09 mm). ESWT up-regulated several osteoblastic and osteoclastic gene markers and cytokines; however, the effects on osteoclasts were only transient. Double-fluorescence bone labeling demonstrated that osteoblastic activity increased after ESWT treatment. There was no difference in systemic RANKL/OPG ratio between groups.

Conclusions

ESWT at 500 impulse at energy flux density 0.1 mJ/mm² increased osteoblast and osteoclast activities and imbalanced bone remodeling resulting in impeded tooth movement in rats.

https://ift.tt/2qHnFsM

Brain metastases as first manifestation of advanced cancer: exploratory analysis of 459 patients at a tertiary care center

Abstract

Symptomatic brain metastases (BM) are a frequent and late complication in cancer patients. However, a subgroup of cancer patients presents with BM as the first symptom of metastatic cancer. Here we aimed to analyze the clinical course and prognostic factors of this particular BM patient population. Patients presenting with newly diagnosed BM without a history of metastatic cancer were identified from the Vienna Brain Metastasis Registry. Clinical characteristics and overall survival were retrieved by chart review. 459/2419 (19.0%) BM patients presented with BM as first symptom of advanced cancer. In 374/459 (81.5%) patients, an extracranial primary tumor, most commonly lung cancer, could be identified within 3 months after BM diagnosis. In 85/459 (18.5%) patients no extracranial primary tumor could be identified despite comprehensive diagnostic workup within the first 3 months after diagnosis of BM. Survival of patients with identified extracranial tumor differed only numerically from patients with cancer of unknown primary (CUP), however patients receiving targeted therapy after molecular workup showed significantly enhanced survival (20 months vs. 7 months; p = 0.003; log rank test). The GPA score showed a statistically significant association with median overall survival times in the CUP BM patients (class I: 46 months; class II: 7 months; class III: 4 months; class IV: 2 months; p < 0.001; log rank test). The GPA score has a strong prognostic value in patients with CUP BM and may be useful for patient stratification in the clinical setting. Comprehensive diagnostic workup including advanced imaging techniques and molecular tissue analyses appears to benefit patients by directing specific molecular targeted therapies.

https://ift.tt/2z4CU3s

Photobiomodulation therapy (PBMT) on acute pain and inflammation in patients who underwent total hip arthroplasty—a randomized, triple-blind, placebo-controlled clinical trial

Abstract

When conservative treatments fail, hip osteoarthritis (OA), a chronic degenerative disease characterized by cartilage wear, progressive joint deformity, and loss of function, can result in the need for a total hip arthroplasty (THA). Surgical procedures induced tissue trauma and incite an immune response. Photobiomodulation therapy (PBMt) using low-level laser therapy (LLLT) and/or light-emitting diode therapy (LEDT) has proven effective in tissue repair by modulating the inflammatory process and promoting pain relief. Therefore, the aim of this study was to analyze the immediate effect of PBMt on inflammation and pain of patients undergoing total hip arthroplasty. The study consisted of 18 post-surgical hip arthroplasty patients divided into two groups (n = 9 each) placebo and active PBMt who received one of the treatments in a period from 8 to 12 h following THA surgery. PBMt (active or placebo) was applied using a device consisting of nine diodes (one super-pulsed laser of 905 nm, four infrared LEDs of 875 nm, and four red LEDs 640 nm, 40.3 J per point) applied to 5 points along the incision. Visual analog scale (VAS) and blood samples for analysis of the levels of the cytokines TNF-α, IL-6, and IL-8 were recorded before and after PBMt application. The values for the visual analog scale as well as those in the analysis of TNF-α and IL-8 serum levels decreased in the active PBMt group compared to placebo-control group (p < 0.05). No decrease was observed for IL-6 levels. We conclude that PBMt is effective in decreasing pain intensity and post-surgery inflammation in patients receiving total hip arthroplasty.

https://ift.tt/2zexdQX

Glomuvenous malformations: dual PDL-Nd:YAG laser approach

Abstract

Glomuvenous malformations are uncommon simple vascular malformations that might be present at birth or appear during childhood that have been classically classified as a subtype of venous malformations. Sclerotherapy and surgery have been used in the past as treatments for this condition although with disappointing results in large glomangiomas. The treatment of these lesions has still not been standardized. We conducted a retrospective study of 17 patients treated with dual wavelength PDL-Nd:YAG. The majority of the patients experience a reduction of at least a 60% in their glomuvenous malformations. Treatment was well-tolerated, and adverse effects were rare.

https://ift.tt/2OCXYTL

Antimicrobial photodynamic therapy as a new approach for the treatment of vulvovaginal candidiasis: preliminary results

Abstract

In this work, we present the efficacy of photodynamic therapy against yeast cells in an animal model. We tested two photosensitizers, methylene blue and protoporphyrin IX. Thirty-seven female BALB-c mice with a body mass of 20–25 g were used. To achieve persistent vaginitis, estrus was induced by subcutaneous injection of 0.1 mg/mL estradiol valerate applied weekly. Three days after pseudo-estrus, intravaginal inoculation with Candida albicans was performed. Mice were anesthetized with ketamine (80 mg/kg) and xylazine (10 mg/kg) by intraperitoneal injection before inoculation, and antimicrobial photodynamic therapy (aPDT) was performed 5 days after fungal inoculation. Two photosensitizers were tested, methylene blue (MB; 100 μM) and protoporphyrin IX (PpNetNI; 10 μM). Two custom-made LEDs emitting light at 660 and 630 nm at approximately 800 mW each were used for irradiation. The aPDT treatment reduced the fungal colony-forming units (CFUs) by one order of magnitude for the MB (p = 0.020) and PpNetNI (p = 0.018) photosensitizers. Seven days after the treatment, there were significantly fewer CFUs compared to the control group (p = 0.041 and p = 0.035 for MB and PpNetNI, respectively), but this was not increased compared to the initial number immediately after aPDT. Using aPDT as a therapeutic option to decrease fungal infection in a vaginal candidiasis model resulted in a significant reduction in the C. albicans population. Both photosensitizers were effective for preventing reinfection within 7 days. The aPDT also had no effect on the vaginal mucosa at the ultrastructural level. In addition to the fungicide effect, we observed reduced swelling and lack of the formation of abscesses, microabscesses coating the cornified epithelial layer, and the accumulation of neutrophils in the submucosa.

https://ift.tt/2OCXU6t

Efficacy assessment of UVA1 and narrowband UVB for treatment of scalp psoriasis

Abstract

To compare the efficacy and safety of UVA1 and narrowband UVB (NB-UVB) therapy in the treatment of scalp psoriasis. Patients with scalp psoriasis were randomly assigned to either UVA1 or NB-UVB therapy. Both treatments were performed three times weekly for 6 weeks. Clinical efficacy was evaluated by using Psoriasis Scalp Severity Index (PSSI), and patient-reported quality of life (QoL) was assessed by Dermatology Life Quality Index (DLQI). Totally 68 patients completed the study. Both UVA1 and NB-UVB phototherapy achieved a statistically significant reduction of PSSI and DLQI scores at the end of the treatment period. Compared with the NB-UVB group, the significantly greater improvements occurred in UVA1 treatment group at week 3, although differences declined thereafter through week 10. Both UVA1 and NB-UVB therapy were well-tolerated in this study, and the occurrence of adverse events (AEs) was uncommon. Both UVA1 and NB-UVB phototherapy could offer relief of scalp symptoms in patients with scalp involvement. Furthermore, UVA1 treatment could improve the clinical manifestations and QoL more quickly than NB-UVB therapy.

https://ift.tt/2z5JRkV

Germination of pollen grains in the esophagus of individuals with eosinophilic esophagitis

Abstract

Background

Eosinophilic esophagitis (EoE) is characterized by esophageal dysfunction and, histologically, by eosinophilic inflammation. There is no a clear etiologic treatment. EoE exacerbations are often seasonal. We hypothesized that the inflammatory response of the esophageal mucosa in patients with high levels of antibodies to pollen allergens and worsened seasonal EoE might be due to swallowing airborne pollen and the intrusion into the esophageal mucosa of pollen allergens and pollen tubes, which encounter a pH and humidity resembling the stigma at pollination.

Objective

The aim of our study was to demonstrate the possible pathogenic role of environmental allergens in EoE through molecular and anatomopathological studies.

Methods

129 patients with EoE were tested for environmental and food allergens. Component resolved diagnosis (CRD), histological and botanical analysis was performed. Microscopic examination of esophageal biopsies of 129 adults patients with EoE, 82 of them with seasonal exacerbation, and 100 controls, with gastroesophageal reflux without eosinophilic infiltrate, were made to verify the presence of callose (polysaccharide abundant in pollen tubes but absent in animal tissues) in the esophagus.

Results

CRD detected pollen allergens in 87.6% of patients with EoE. The predominant allergens were group 1 grass (55%), Art v 3 (11.3%) and lipid transfer proteins (LTPs) (19.4%) of common Mediterranean foods such as peach, hazelnuts, walnuts and wheat. Callose from pollen tubes was found in 65.6% of biopsies.

Conclusion

Alteration of the mucosal barrier in EoE might cause the penetration of pollen grains into the esophageal tissues. In EoE patients, anatomopathological studies searching for intrusion to plant foods and pollen, and specific‐guided diet and immunotherapy after plant structures detection in biopsies, might be effective.

This article is protected by copyright. All rights reserved.

https://ift.tt/2zICK1e

Dermatological manifestations in Noonan syndrome: A prospective multicentric study of 129 mutation‐positive patients

Summary

Background

Data on dermatological manifestations of Noonan syndrome (NS) remain heterogeneous and based on little dermatological expertise.

Objectives

To describe the dermatological manifestations of NS, compare them with the literature findings, and test for dermatological phenotype‐genotype correlations with or without the presence of PTPN11 mutations.

Methods

We performed a large, 4‐year, prospective, multicentric, collaborative dermatological and genetic study.

Results

One hundred and twenty‐nine patients were enrolled, including 65 with PTPN11‐NS, 34 with PTPN11‐NS with multiple lentigines (NSML), and 30 without PTPN11‐NS. Easy bruising was the most frequent dermatological finding in PTPN11‐NS, present in 53.8%. Multiple lentigines and café‐au‐lait macules (≥3) were present in, respectively, 94% and 80% of NSML linked to specific mutations of PTPN11. Atypical forms of NSML could be associated with NS with RAF1 or NRAS mutations. In univariate analysis, patients without a PTPN11 mutation showed (i) a significantly higher frequency of keratinization disorders (P=0.001), including keratosis pilaris (P=0.005), ulerythema ophryogenes (P=0.0001) and palmar and/or plantar hyperkeratosis (P=0.06, trend association), and (ii) a significantly higher frequency of scarce scalp hair (P=0.035) and scarce or absent eyelashes (P=0.06, trend association) than those with PTPN11 mutations.

Conclusions

Cutaneous phenotype of NS with a PTPN11 mutation is generally mild and non‐specific, whereas the absence of a PTPN11 mutation is associated with a high frequency of keratinization disorders and hair abnormalities.

This article is protected by copyright. All rights reserved.

https://ift.tt/2z0QBAH

Dermatological manifestations in Noonan syndrome: A prospective multicentric study of 129 mutation‐positive patients

Summary

Background

Data on dermatological manifestations of Noonan syndrome (NS) remain heterogeneous and based on little dermatological expertise.

Objectives

To describe the dermatological manifestations of NS, compare them with the literature findings, and test for dermatological phenotype‐genotype correlations with or without the presence of PTPN11 mutations.

Methods

We performed a large, 4‐year, prospective, multicentric, collaborative dermatological and genetic study.

Results

One hundred and twenty‐nine patients were enrolled, including 65 with PTPN11‐NS, 34 with PTPN11‐NS with multiple lentigines (NSML), and 30 without PTPN11‐NS. Easy bruising was the most frequent dermatological finding in PTPN11‐NS, present in 53.8%. Multiple lentigines and café‐au‐lait macules (≥3) were present in, respectively, 94% and 80% of NSML linked to specific mutations of PTPN11. Atypical forms of NSML could be associated with NS with RAF1 or NRAS mutations. In univariate analysis, patients without a PTPN11 mutation showed (i) a significantly higher frequency of keratinization disorders (P=0.001), including keratosis pilaris (P=0.005), ulerythema ophryogenes (P=0.0001) and palmar and/or plantar hyperkeratosis (P=0.06, trend association), and (ii) a significantly higher frequency of scarce scalp hair (P=0.035) and scarce or absent eyelashes (P=0.06, trend association) than those with PTPN11 mutations.

Conclusions

Cutaneous phenotype of NS with a PTPN11 mutation is generally mild and non‐specific, whereas the absence of a PTPN11 mutation is associated with a high frequency of keratinization disorders and hair abnormalities.

This article is protected by copyright. All rights reserved.

https://ift.tt/2z0QBAH

Bilateral central retinal artery occlusion from catastrophic antiphospholipid syndrome

A 23-year-old woman with history of systemic lupus erythematous presented with dizziness and headache and was admitted for the stroke workup. During her stay, she had sudden painless loss of vision in her right eye consistent with central retinal artery occlusion (CRAO). Ocular massage and paracentesis were attempted without success to resume the flow. She was started on oral high-dose steroids (1 mg/kg) for lupus flare and therapeutic anticoagulation for antiphospholipid syndrome (positive for anticardiolipin and beta-2 microglobulin antibodies). On day 4, she started having painful bluish discoloration of her left index finger and right fifth toe, and on day 5 she had acute onset of left blurry vision with findings consistent with CRAO. She fulfilled the criteria of catastrophic antiphospholipid syndrome and was started on intravenous pulse steroids, plasmapheresis and higher international normalised ratio goal of 3–3.5 with improvement in her left eye vision from 20/200 to 20/20 on near card test by the end of treatment.

https://ift.tt/2PUq5CH

Renal pseudomass: be aware of splenorenal fusion

Splenosis is the heterotopic implantation of splenic tissue that usually occurs in a previous major abdominal trauma or splenectomy setting. However, splenorenal fusion is an uncommon entity, categorised as a developmental anomaly. There have been several confirmed cases in the literature. Despite some helpful imaging features, it can be easily misdiagnosed as a neoplastic renal mass, resulting in unnecessary nephrectomy. Here we presented a case of splenorenal fusion in an elderly female patient, which was initially misdiagnosed as renal cell carcinoma in sonography. More specific imaging modalities and biopsy are helpful in suggesting the correct diagnosis.

https://ift.tt/2DeOE6q

Anti-HMGCR antibody-associated necrotising myopathy and its association with statin use

A 66-year-old man presented with chest pain and a 1-year history of generalised weakness, accompanied with generalised aches and pains. Symptoms worsened when he was initiated on statins. Investigations yielded high creatine kinase, high HMG-coenzymeA reductase (HMGCR) antibody titre, myopathic features on electromyography and muscle biopsy, and muscle atrophy on MRI. These results were in keeping with anti-HMGCR antibody myopathy. The patient responded well to immunosuppressive therapy.

https://ift.tt/2Q19OvX

Cerebral tuberculomas in a 6-year-old girl causing central diabetes insipidus

A 6-year-old girl presented acutely with worsening frontal headaches. She had a 3-month history of lethargy, reduced appetite, weight loss, cough and intermittent fevers. A chest X-ray showed a left upper lobe consolidation, and a CT head showed multiple enhancing lesions with significant surrounding oedema in both cerebral hemispheres. Due to the strong suspicion of tuberculosis (TB), she was admitted and treated with anti-TB therapy and steroids. Following this, pulmonary infection with Mycobacterium tuberculosis was confirmed by a positive PCR from induced sputum. Cerebral spinal fluid (CSF) analysis was normal and tested negative for M. tuberculosis on PCR. During her first week of treatment, she developed polyuria, nocturia and polydipsia and was diagnosed with central diabetes insipidus. She was started on desmopressin which rapidly improved her symptoms, and she was continued on desmopressin for 3 months. Currently, she remains well and has shown a good response to TB treatment.

https://ift.tt/2DeOwns

Clinical and radiological findings in a severe case of cleidocranial dysplasia

Cleidocranial dysplasia (CCD) is a rare congenital autosomal dominant condition, causing hypoplasia of the clavicle, abnormal formation of teeth, skeletal and craniofacial bones. CCD is caused by the mutation of RUNX2/CBFA1 present in the short arm of chromosome 6 at position 21.1, a transcription factor essential for the formation of teeth, cartilage and bone. Patients with CCD show the classical features of excessive mobility of the shoulder bone, lack of resorption of the deciduous teeth, failure to erupt permanent teeth, multiple impacted and supernumerary teeth, and open fontanelle and sutures of the skull. In this article we report a case of CCD in a 16-year-old male patient, with an aim to highlight the clinical, radiological and recommended treatment guidelines.

https://ift.tt/2PUpXmH

Secretory cervical schwannoma: first of its kind

Schwannomas arising from cervical sympathetic chain are rare benign neoplasms which are slow growing, usually asymptomatic, biochemically non-secretory and functionally inactive tumours. We present a case of secretory schwannoma arising from the cervical sympathetic chain, causing hypertension and associated with raised urinary catecholamine degradation by-products. Transcervical excision of the tumour was followed by normalisation of blood pressure and urinary vanillylmandelic acid levels and pathologically the tumour was proved to be a schwannoma.

https://ift.tt/2Dj591D

Massive release of the histamine‐degrading enzyme diamine oxidase during severe anaphylaxis in mastocytosis patients

Abstract

Background

Histaminolytic activity mediated by diamine oxidase (DAO) is present in plasma after induction of severe anaphylaxis in rats, guinea pigs and rabbits. Heparin released during mast cell degranulation in the gastrointestinal tract might liberate DAO from heparin‐sensitive storage sites. DAO release during anaphylaxis has not been demonstrated in humans.

Methods

Plasma DAO, tryptase and histamine concentrations of four severe anaphylaxis events were determined at multiple serial time points in two patients with systemic mastocytosis. The histamine degradation rates were measured in anaphylaxis samples and in pregnancy sera and plasma with comparable DAO concentrations.

Results

Mean DAO (132 ng/ml) and tryptase (304 ng/ml) concentrations increased 187‐ and 4.0‐fold respectively over baseline values (DAO 0.7 ng/ml, tryptase 76 ng/ml) during severe anaphylaxis. Under non‐anaphylaxis conditions DAO concentrations were not elevated in 29 mastocytosis patients compared to healthy volunteers and there was no correlation between DAO and tryptase levels in mastocytosis patients. The histamine degradation rate of DAO in plasma from mastocytosis patients during anaphylaxis is severely compromised compared to DAO from pregnancy samples.

Conclusions

During severe anaphylaxis in mastocytosis patients DAO is likely released from heparin‐sensitive gastrointestinal storage sites. The measured concentrations can degrade histamine, but DAO activity is compromised compared to pregnancy samples. For accurate histamine measurements during anaphylaxis DAO inhibition is essential to inhibit further histamine degradation after blood withdrawal. Determination of DAO antigen levels might be of clinical value to improve the diagnosis of mast cell activation.

This article is protected by copyright. All rights reserved.

https://ift.tt/2T3rupc

Functional Connectivity Changes of Key Regions for Motor Initiation in Parkinson’s Disease

Abstract

Akinesia, a cardinal symptom of Parkinson's disease, has been linked to abnormal activation in putamen and posterior medial frontal cortex (pMFC). However, little is known whether clinical severity of akinesia is linked to dysfunctional connectivity of these regions. Using a seed-based approach, we here investigated resting-state functional connectivity (RSFC) of putamen, pMFC and primary motor cortex (M1) in 60 patients with Parkinson's disease on regular medication and 72 healthy controls. We found that in patients putamen featured decreases of connectivity for a number of cortical and subcortical areas engaged in sensorimotor and cognitive processing. In contrast, the pMFC showed reduced connectivity with a more focal cortical network involved in higher-level motor-cognition. Finally, M1 featured a selective disruption of connectivity in a network specifically connected with M1. Correlating clinical impairment with connectivity changes revealed a relationship between akinesia and reduced RSFC between pMFC and left intraparietal lobule (IPL). Together, the present study demonstrated RSFC decreases in networks for motor initiation and execution in Parkinson's disease. Moreover, results suggest a relationship between pMFC-IPL decoupling and the manifestation of akinetic symptoms.

https://ift.tt/2Oz8b3H

Emergent Functional Network Effects in Parkinson Disease

https://ift.tt/2z6Bnu2

Functional Connectivity of the Anterior Cingulate Cortex in Depression and in Health

Abstract

The first voxel-level resting-state functional connectivity (FC) neuroimaging analysis of depression of the anterior cingulate cortex (ACC) showed in 282 patients with major depressive disorder compared with 254 controls, some higher, and some lower FCs. However, in 125 unmedicated patients, primarily increases of FC were found: of the subcallosal anterior cingulate with the lateral orbitofrontal cortex, of the pregenual/supracallosal anterior cingulate with the medial orbitofrontal cortex, and of parts of the anterior cingulate with the inferior frontal gyrus, superior parietal lobule, and with early cortical visual areas. In the 157 medicated patients, these and other FCs were lower than in the unmedicated group. Parcellation was performed based on the FC of individual ACC voxels in healthy controls. A pregenual subdivision had high FC with medial orbitofrontal cortex areas, and a supracallosal subdivision had high FC with lateral orbitofrontal cortex and inferior frontal gyrus. The high FC in depression between the lateral orbitofrontal cortex and the subcallosal parts of the ACC provides a mechanism for more non-reward information transmission to the ACC, contributing to depression. The high FC between the medial orbitofrontal cortex and supracallosal ACC in depression may also contribute to depressive symptoms.

https://ift.tt/2OEanac

Sensitive Period for Cognitive Repurposing of Human Visual Cortex

Abstract

Studies of sensory loss are a model for understanding the functional flexibility of human cortex. In congenital blindness, subsets of visual cortex are recruited during higher-cognitive tasks, such as language and math tasks. Is such dramatic functional repurposing possible throughout the lifespan or restricted to sensitive periods in development? We compared visual cortex function in individuals who lost their vision as adults (after age 17) to congenitally blind and sighted blindfolded adults. Participants took part in resting-state and task-based fMRI scans during which they solved math equations of varying difficulty and judged the meanings of sentences. Blindness at any age caused "visual" cortices to synchronize with specific frontoparietal networks at rest. However, in task-based data, visual cortices showed regional specialization for math and language and load-dependent activity only in congenital blindness. Thus, despite the presence of long-range functional connectivity, cognitive repurposing of human cortex is limited by sensitive periods.

https://ift.tt/2z8OR8m

Microcystic Calcifying Epithelial Odontogenic Tumor

Abstract

Microcystic variant of calcifying epithelial odontogenic tumor is rare. We herein describe an additional well-documented case of microcystic CEOT. The affected patient is a Guatemalan 42-year-old female with an expansile well-defined mixed radiolucent–radiopaque lesion located in the right posterior mandible. The lesion was associated to an unerupted third molar. Histopathologic examination revealed nests and cords of moderately pleomorphic, eosinophilic polyhedral epithelial cells surrounded by a fibromyxoid stroma. The neoplastic cells showed microcystic pattern made of pseudo-glandular spaces with variable diameter. Occasional amyloid deposits and calcified acellular material were observed. Tumor cells were positive for AE1/AE3, CK14, CK19, p63, CD138, and beta-catenin. Conservative surgical resection was performed with an uneventful immediate post-surgical follow-up. After 1 year follow-up there is no evidence of recurrence. Pathologists should be aware of this unusual microcystic presentation of CEOT, which may pose a diagnostic challenge and potential diagnostic dilemma.

https://ift.tt/2PXUKii

Dentoalveolar Abscesses Not Associated with Caries or Trauma: A Diagnostic Hallmark of Hypophosphatemic Rickets Initially Misdiagnosed as Hypochondroplasia

Abstract

Hypophosphatemic rickets is a rare genetic disorder involving the regulation of fibroblast growth factor 23 (FGF23), a phosphaturic agent, clinically showing bowing of the legs, short stature and dentoalveolar abscesses. A 7-year-old boy, with previous hypochondroplasia diagnosis, was referred to our pediatric dentistry clinic presenting short stature, bone deformities and sinus tracts at deciduous teeth apex levels not related with trauma, restorations or dental caries. After deciduous teeth extraction, due to root resorption and mobility, light microscopy exhibited typical hypophosphatemic dentin, and micro-computed tomography revealed tubular clefts and porosities throughout the teeth. Laboratory tests confirmed the HR diagnosis, after which the treatment was initiated.

https://ift.tt/2Dj9GBc

Outcome differences between recanalized malignant central airway obstruction from endoluminal disease versus extrinsic compression

Abstract

We compared the outcome of Nd:YAG laser therapy with stent placement for malignant central airway obstruction (CAO) at our center over a 10-year period. This is a retrospective review of patients undergoing Nd:YAG laser therapy or self-expanding metal stent (SEMS) placement for malignant CAO between November 2007 and October 2017. Seventy-two patients were recanalized for malignant CAO. The median (range) age was 63 (23–86) years, with 49 (68%) males. Patients underwent either laser therapy alone (N = 36), stent placement alone (N = 30), or both (N = 6). The wavelength of Nd:YAG laser used was 1064 nm, and median (range) laser energy used was 25 (15–35) W, in 377 (115–1107) pulses. Fifty-one (71%) patients died with median survival of 7.2 months. In subgroup analysis, 21 (58.3%) vs. 25 (83.3%), p = 0.03 patients died in the "laser resection" vs. "stent placement" group with longer median survival of 12.4 months in the former vs. 4.5 months, p = 0.0004 in the later. Esophageal cancer and left main bronchus involvement were significantly more common (10 (33.3%) vs. 0, p = 0.0001, and 16 (53.3%) vs. 8 (22.2%), p = 0.01), in the stent placement vs. laser resection group, respectively. Trachea or main bronchi involvement and respiratory failure on presentation requiring mechanical ventilation correlated with poorer survival. The immediate restoration of luminal patency, complication rate, and 30-day mortality was similar among the two groups. The median (range) energy used for laser therapy was 25 (15–35) W. Median of 377 pulses was used for the duration of 287.5 s. The results were compared using a Wilcoxon two-sample test, and Fischer exact test with p values considered indicative of a significant difference if less than 0.05. In patients requiring recanalization of malignant CAO, the extrinsic compression from esophageal cancer, trachea or main bronchi involvement, respiratory failure on presentation requiring mechanical ventilation, and stent placement correlated with poorer survival. Interventional pulmonology training program should emphasize on dedicated training in laser therapy as it is associated with improved survival.

https://ift.tt/2AZL3Yy