A novel treatment for height growth in patients with growth hormone insensitivity syndrome by cyproheptadine hydrochloride

Abstract

Background

Cyproheptadine HCl (CyproH) is an appetite stimulating drug and while it was prescribed for a patient with growth hormone insensitivity syndrome (GHIS) for increasing appetite, his height growth was surprisingly increased. Therefore the aim of this study was to investigate the effect of CyproH on growth parameters of the patients with GHIS.

Patients and design

Twenty patients were enrolled in two prospective cohorts at two different times. Fifteen cases were observed for 1.17 ± 1.3 yr without treatment (observation period, OP). Then, CyproH was administered for 2.2 ± 2.7 yr (treatment period, TP) and growth parameters were compared within these two periods. Five patients who did not receive any treatment for 1 – 8.24 yr (4 ± 2.9) were control group.

Results

Height velocity (HV) increased from 1.88 ± 0.7 to 6.1 ± 0.8 cm/year and HV-SDS reached from -4.5 ± 0.74 to -0.21± 1.2 in OP and TP, respectively (P<0.001), whereas HV and HV-SDS were 2.2 ± 1.1 cm/yr and -4.2 ±1.2 respectively in controls (P<0.001). Height-SDS was -7.0 ± 1.7and increased to -6 ± 2.2 after treatment (P= 0.002). Gain in height was 2.3 ± 0.6 SDS in 5 patients who were treated for 5.4 ± 2.8 yrs. BMI-SDS was not significantly changed within two time periods and also in cases and controls.

Conclusion

CyproH caused height growth in the patients with GHIS and therefore this treatment can be considered as an alternative option to IGF-I injection.

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Hashimoto's thyroiditis with elevated serum IgG4 concentrations is not equivalent to IgG4 Hashimoto's thyroiditis

Abstract

Background

Hashimoto's thyroiditis (HT) with serum IgG4 concentrations greater than 135 mg/dl can be diagnosed as elevated serum IgG4 HT. HT can also be classified into IgG4 HT and non-IgG4 HT based on an immunohistochemistry analysis of IgG4. The aim of our study was to determine the relationship between elevated serum IgG4 HT and IgG4 HT.

Method

93 HT patients with both thyroid tissues and serum samples stored before pathological examination were collected. The serum levels of IgG, IgG4, TgAb IgG, TgAb IgG4, TPOAb IgG, and TPOAb IgG4 were measured by ELISAs. The expression levels of IgG4, IgG and TGF-β1 in thyroid tissues were detected by immunohistochemistry.

Results

HT patients were divided into two groups: elevated serum IgG4 HT (n = 12) and non-elevated serum IgG4 HT (n = 81). Hypothyroidism was found in 5/12 cases (41.7%) in the elevated serum IgG4 HT group and 10/81 cases (12.3%) in the non-elevated serum IgG4 HT group (P = 0.023). Serologically, there were no significant differences in the levels of TgAb IgG, TPOAb IgG, TgAb IgG4, and TPOAb IgG4 between the two groups, and the expression of TGF-β1 in thyroid tissues was not significantly different between the groups. Most importantly, the frequency of patients who satisfied the criteria for IgG4 HT diagnosis was comparable (25% vs. 20.9%, P = 0.756).

Conclusions

The measurement of serum IgG4 allows the identification of HT patients closely associated with hypothyroidism. However, our study demonstrated that elevated serum IgG4 HT is not equivalent to IgG4 HT.

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Treatment escape reduces the effectiveness of cabergoline during long-term treatment of acromegaly in monotherapy or in association with first-generation somatostatin receptor ligands

Abstract

Background

Few studies evaluated the use of cabergoline (CAB) for acromegaly treatment in monotherapy or in combination with first-generation somatostatin receptor ligands (SRLs).

Aim

Evaluate the efficacy, predictors of response and safety of CAB treatment in acromegaly both in monotherapy and in combination with SRLs.

Methods

We retrospectively collected demographic, biochemical, tumor and treatment data. Short-term disease control was defined as random GH level <1.0 μg/L and normal age-matched IGF-I level after 3-6 months of treatment with the higher dose used. Long-term disease control was defined as maintenance of normal GH and IGF-I levels at the last visit (at least nine months of treatment).

Results

82 patients were studied. The median total time of treatment in monotherapy or in combination with SRLs was 14 months (3 – 124) and 34 months (3 – 88), respectively. Short-term disease control was observed in six (21%) patients in the monotherapy group and in 20 (32%) in the combination group. Treatment escape was observed in one patient after 16 months of CAB monotherapy and in six (30%) patients with combination therapy (after a median of 38 months), resulting in long-term disease control of 18% and 23%, respectively. Hyperprolactinemia was a predictor of response to monotherapy and pre-treatment GH level to combination treatment.

Conclusion

We presented the results of the largest single-center study with CAB in monotherapy and in combination with SRL. The efficacy of CAB in acromegaly seems to be lower than that of other drugs, and treatment escape may occur after a long-term follow-up.

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Health-Related Quality of Life in patients with Non-Surgical Hypoparathyroidism and Pseudohypoparathyroidism

Abstract

Objective

Non-surgical hypoparathyroidism (NS-HypoPT) and pseudohypoparathyroidism (PHP) are rare diseases, with a prevalence of 2/100.000 and 1/100.000, respectively. Only few studies on Quality of Life (QoL) among patients with Ns-HypoPT and PHP are available. We aimed to investigate the QoL among patients with Ns-HypoPT and PHP including information about education.

Design

A cohort study with patients identified from a previously epidemiological study.

Patients

57 patients with Ns-HypoPT and 30 patients with PHP.

Measurements

The well-validated questionnaires SF-36v2 and WHO-5 Well Being Index. Results compared to norm-based material, disease specific norm-based material and patients with post-surgical HypoPT

Results

SF36v2 showed a significantly reduced score in all eight subdomains in patients with NS-HypoPT compared to a norm-based population. PHP patients scored lower in five subdomains. Females were more affected than males. Compared with post-surgical HypoPT, Ns-HypoPT and PHP are compatible at most domains. At the domains Physical Function, Social Function and Mental Health, Ns-HypoPT and PHP patients scored significantly lower (p_all<0.05). At the Mental Component Score, patients with Ns-HypoPT, had a lower score compared with post-surgical HypoPT (p<0.01). The overall WHO-5 Well Being Index score was comparable between groups (p=0.45). No differences were seen comparing patients with post-surgical HypoPT and Ns-HypoPT (p=0.68) or post-surgical HypoPT and PHP (p=0.67). A WHO-5 score below 28 indicates depression (NS-HypoPT=7; PHP=3, p=0.71), whereas a score between 28-50 suggesting poor emotional well-being (NS-HypoPT=19; PHP=5, p=0.13). The remaining patients scored above 50 suggesting well-being.

Conclusion

QoL is impaired equally among patients with Ns-HypoPT and PHP.

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Intracystic papillary carcinoma of the breast: Experience of a major Chinese cancer center

Publication date: Available online 8 March 2018
Source:Pathology - Research and Practice
Author(s): Jun Zhang, Tiemei Zhang, Nan Wu, Xinhua Zhao, Qingsong Wang, Yanyang Jiang, Ming Gao, Lin Gu
BackgroundIntracystic papillary carcinoma (IPC) is a rare breast neoplasm. There are few studies focusing on its clinical features and limited data about its preoperative diagnosis,treatment and outcomes. The purpose of this study is to explore specific characteristics of patients with IPC, investigate its clinicopathological features, prognosis in China and confirm its surgery management.MethodsWe identified 111 patients with IPC from the registry of Tianjin Medical University Cancer Institute and Hospital between 2004 and 2017. Follow-up of cases dating back to January 1, 2004 was obtained from retrospective chart review and patient questionnaires. Differences in clinical features and survival of patients were assessed using the Kaplan-Meier method.ResultsThe median tumor size was 2.25 cm. Median age was 62 years. In the cases of axillary lymph node dissection(ALND), only 1.1% (1/85) of patients with axillary lymph node metastasis were found. The diagnostic accuracy of preoperative Color Doppler ultrasound and Mammograms for IPC was 62.0% (62/100) and 63.5% (54/85) respectively. The median follow-up period was 52 (range2–149) months. The overall survival rate was 98.9%, 92.2%,and 85.6% at 2,5,and 10 years and the relapse-free rate was 99.1%,97.2%,and 92.0% at 2,5, and 10 years, respectively. The disease-specific survival rate was 100%.ConclusionOverall, we report some unique features of IPC in the Chinese population. The patients of IPC of the breast in China have more excellent prognosis than in Caucasian and other races. The diagnostic accuracy of imaging was low, and was easily misinterpreted as a cyst or benign disease. Preoperative core needle biopsies are very difficult for accurate pathological diagnosis of IPC. IPC is a localized disease with a low frequency of axillary lymph node involvement, rare distant metastases and excellent survival. The low incidence of axillary lymph node metastasis suggests that quadrectomy + sentinel lymph node biopsy (SLNB) and breast-conserving surgery is recommended for IPC.



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Hypothalamic Epigenetics Driving Female Puberty

Abstract

Puberty involves a series of morphological, physiological and behavioral changes during the last part of the juvenile period that culminates in the attainment of fertility. The activation of the pituitary-gonadal axis by increased hypothalamic secretion of Gonadotropin Releasing Hormone (GnRH) is an essential step in the process. The current hypothesis postulates that a loss of transsynaptic inhibition together with a rise in excitatory inputs are responsible for the activation of GnRH release. Similarly, a shift in the balance in the expression of puberty activating (PA) and puberty inhibitory (PI) genes exists during the pubertal transition. In addition, recent evidence suggests that the epigenetic machinery controls this genetic balance, giving rise to the tantalizing possibility that epigenetics serves as a relay of environmental signals known for many years to modulate pubertal development. Here we review the contribution of epigenetics as a regulatory mechanism in the hypothalamic control of female puberty.

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Prevalence of chronic postsurgical pain after thoracotomy and total knee arthroplasty: a retrospective multicenter study in Japan (Japanese Study Group of Subacute Postoperative Pain)

Abstract

We performed a multicenter observational study to assess the prevalence and risk factors of persistent pain after lung cancer surgery and total knee arthroplasty (TKA) in the Japanese population. After receiving Ethics Committee approval, a retrospective chart review was performed for patients who underwent surgery at seven university hospitals in Japan in 2013. A total of 511 patients who underwent lung cancer surgery and 298 patients who underwent TKA were included. The prevalence of chronic postsurgical pain (CPSP) at 3 and 6 months was 18 and 12% after lung surgery and 49 and 33% after TKA, respectively. The prevalence of analgesic use at 3 and 6 months was 16 and 9% after lung surgery and 34 and 22% after TKA, respectively. In both groups, preoperative analgesic use was associated with CPSP. Anesthetic methods or techniques during both types of surgery did not significantly affect the prevalence of CPSP. This is the first study in which the prevalence of CPSP after lung surgery and TKA in Japanese population was extensively evaluated in a multicenter trial. Further prospective studies are needed to confirm the prevalence of CPSP in the Japanese population and to identify risk factors and prevention methods.

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