Dysgerminoma in a 15 years old phenotypically female Swyer syndrome with 46, XY pure gonadal dysgenesis: A case report

Swyer syndrome is a 46, XY karyotype, with pure gonadal dysgenesis and primary amenorrhea. These females have primordial Mullerian structures and seek medical attention as they experience primary amenorrhea. Here, we report a 15‐year‐old girl, diagnosed as Swyer syndrome associated with left ovarian dysgerminoma.     

Morning melatonin serum values do not correlate with disease activity in rheumatoid arthritis: a cross-sectional study

Rheumatoid arthritis (RA), the most prevalent autoimmune arthritis worldwide, usually presents with a circannual manner and, meanwhile, follows a circadian rhythm for symptoms like morning stiffness. Therefore, association between RA and some hormones such as melatonin (MLT) and vitamin D, whose serum values are related to body circadian rhythms or seasonal variations, has become more noticeable recently. Since some studies proposed that RA patients show altered MLT circadian rhythms, especially in concordance with symptoms, in this research, we present the correlation between MLT serum values and RA disease activity score (DAS28ESR). The current cross-sectional study was carried out on 80 volunteers (60 patients and 20 healthy controls). Fifty percent of the participants in each group were sampled in cold, and the same percentage were sampled in warm seasons at 8 a.m. Disease activity was estimated utilizing DAS28ESR. Patients with possible known confounders of MLT secretion were excluded. A commercial MLT ELISA kit was employed to measure MLT. Statistical analysis was conducted by SPSS-11 software. This study outlined higher serum values of MLT in RA patients compared with controls (P = 0.006, z = ?2.73). However, MLT did not correlate with DAS in patients (P = 0.45, r = ?0.09). GLM analysis demonstrated that DAS28ESR, age, disease duration, medications, gender, and season of sampling had no influence on serum MLT. However, newly diagnosed RA patients presented higher MLT values than established ones (P = 0.03, t = ?2.2). A cutoff point value of 23 pg/mL (63.3 % sensitivity and 90 % specificity) for MLT was computed between patients and controls. This study denoted that morning MLT serum values are higher in RA patients than in healthy volunteers. However, MLT and RA disease activity or other disease characteristics do not correlate. MLT serum values were higher in newly diagnosed RA patients than established ones.     

Histopathologic Findings of Pneumatocele in a Patient with Hyper-IgE syndrome, compatible with cystic adenomatoid malformation

Hyper-immunoglobulin E syndrome is a rare primary immunodeficiency disease characterized by recurrent abscess formation, respiratory tract infections and very high titers of serum IgE associated with peculiar face and skeletal features. We report a seven-year old girl presenting with persistent productive cough and history of chronic eczematoid facial lesions since infancy and two episodes of hospitalizations due to pneumonia and perianal abscess. Additionally, in physical examination finger tip clubbing, laxity of joints and crackles in both lungs were detected. Immunologic work up revealed markedly raised IgE level and eosinophilia. The patient was diagnosed as hyper IgE syndrome based on his clinical and laboratory findings. Chest X-ray revealed multiple large cystic lesions in left lung which were confirmed by spiral CT-scan. Pneumonectomy specimen examination showed cystic adenomatoid malformation, characterized by the presence of various cysts lined by epithelium in different sizes. There are few reports of cystic adenomatoid malformation in children. To our best known, this is the first report of cystic adenomatoid malformation in a child with hyper IgE syndrome. Early diagnosis and surgical therapy are helpful in prevention of repeated infections in these patients.     

Low Postprandial Circulating Inactive Ghrelin: Role of Early Satiety in Undernourished Children

Objective

To determine difference in the levels of circulating inactive ghrelin between undernourished and healthy children.

Methods

The present cross-sectional study was conducted in undernourished children from southwestern Iran, from July 2011 through July 2012. Postprandial inactive ghrelin levels were measured in 40 undernourished children and sex- and age-matched healthy controls by enzyme immunoassay.

Results

The levels of postprandial inactive ghrelin were considerably lower in undernourished compared to the healthy children (6.4 vs. 12.9, P?vs. 7.1, P?=?0.032).

Conclusions

Thus, the levels of inactive ghrelin was found to be low in undernourished children compared to healthy children. Early loss of appetite might be a result of low circulating inactive ghrelin levels in the postprandial state in undernourished children.     

Gender differences in non-glycemic responses to improved insulin sensitivity by pioglitazone treatment in patients with type 2 diabetes

Excess cortisol and GH induce insulin resistance, a central feature of type 2 diabetes (T2D). To study whether the insulin sensitizer pioglitazone affects basal cortisol levels and the GH–IGF-I axis in patients with T2D. Forty-eight patients with T2D (men/women = 28:20, age 61 ± 1 years, BMI 31 ± 0.6 kg/m²) were treated for 26 weeks with pioglitazone 30–45 mg daily in addition to their preexisting therapy. Insulin, proinsulin, HbA_1c, IGF-I, IGFBP-1, and basal cortisol were analyzed before and after treatment. Pioglitazone decreased proinsulin/insulin ratio and HbA_1c decreased (HbA_1c from 7.8 ± 0.2 to 6.6 ± 0.2 % in men and from 7.6 ± 0.2 to 6.1 ± 0.2 % in women, p < 0.001 in both). There was a redistribution of fat but no change in waist circumference. IGF-I and adiponectin increased (p ≤ 0.001) in both genders. IGFBP-1 increased but significantly only for the whole group (p = 0.033). Triglycerides decreased significantly in women only (p = 0.015). Before treatment, women had lower basal cortisol (p = 0.045). Basal cortisol increased in women (from 390 ± 26 to 484 ± 32 nmol/L, p = 0.020) but not in men and did not differ between genders at week 26. ΔIGFBP-1 correlated with Δcortisol (r = 0.458; p = 0.049) and Δadiponectin (r = 0.600; p = 0.005) in women only. In addition to the known effect of improving insulin sensitivity, pioglitazone increased IGF-I regardless of gender and in women also increased basal cortisol. Increased IGF-I may contribute to improved insulin sensitivity after treatment. There seems to be gender differences in treatment responses to pioglitazone on lipid metabolism and basal cortisol, perhaps correcting different mechanisms of insulin resistance between genders.     

Efficient protocol for rapid Aloe vera micropropagation

Context: Aloe vera Linn. (Liliaceae) is a medicinal plant and has a number of curative properties. Vegetative propagation has not enough potential for supplying market demand. However, via in vitro propagation makes possible the mass production of Aloe plants.

Objective: The current study was conducted to investigate growth regulators’ effects on proliferation of A. vera.

Materials and methods: In this study, for comparison of plant growth regulators’ effects on proliferation, the shoot tips and auxiliary buds of A. vera were cultured in the Murashige and Skoog (MS) medium. Rooted plantlets were transferred to garden soil, compost, and sand in the proportion of 1:1:1, respectively, after hardening.

Results: The maximum number of shoots was obtained on the medium supplemented with 1?mg/L IAA?+?4?mg/L BAP and 0.2?mg/L IAA?+?0.8?BAP mg/L. Rooting was also achieved in the same media composition proliferation of shoot. The acclimatized plants showed 100% of survival. The regenerated plants looked healthy, and they were morphologically similar to that of stock plants.

Conclusion: These results suggest that in vitro culture may be used as a technique for rapid propagation of A. vera.     

Childhood Sex-Typed Behavior and Gender Change in Individuals with 46,XY and 46,XX Disorders of Sex Development: An Iranian Multicenter Study

Disorders of sex development (DSD) are congenital conditions in which the typical genetic and hormonal profiles are affected and thereby the usual process of sexual differentiation. Most of these studies, however, have been conducted in Western countries. In the present study, preschool sex-typed activities of Iranian individuals with DSD and their age-matched non-affected male and female relatives were assessed using the Pre-School Activities Inventory (PSAI) modified for retrospective self-report. A total of 192 individuals participated in our study, including 33 46,XX individuals with congenital adrenal hyperplasia (CAH; M age?=?10.36, SD?=?5.52), 15 46,XY individuals with complete androgen insensitivity syndrome (CAIS; M age?=?19.8, SD?=?7.14), and 16 46,XY individuals with 5-alpha reductase deficiency type-2 (5α-RD-2; M age?=?17.31, SD?=?7.28), as well as one age-matched non-affected male and female relative for each patient. With regard to PSAI scores, male-identifying participants with 5α-RD-2 and male controls reported similar levels of male-typical childhood play. Female-identifying participants with 5α-RD-2 and CAH showed comparable scores: significantly less masculine and more feminine than male controls, but significantly more masculine and less feminine than females with CAIS and female controls. These findings support the role of androgens in the development of sex-typical childhood play behavior, with those being exposed to higher levels of fetal functional androgens expressing more masculine behavior at preschool ages.     

A review of pioglitazone HCL and glimepiride in the treatment of type 2 diabetes

Type 2 diabetes (T2D) is a progressive disorder with a consistent and steady increase in glycosylated hemoglobin (HbA1c) over time associated with enhanced risk of micro- and macrovascular complications and a substantial reduction in life expectancy. There are three major pathophysiologic abnormalities associated with T2D: impaired insulin secretion, excessive hepatic glucose output, and insulin resistance in skeletal muscle, liver, and adipose tissue. These defects have been treated in clinical praxis by use of oral insulin secretagogues (sulfonylureas/ glinides) or insulin, biguanides, and thiazolidinediones (TZDs) respectively. Pioglitazone HCL is an insulin sensitizer in the TZD family and glimepiride is an insulin secretagogue in the SU family. This article reviews mechanisms of action and clinical data behind the use of these two commonly used oral hypoglycemic agents with documented efficacy and good safety profile of once-daily administration, alone or in combination with insulin or metformin, in the management of T2D in terms of glycemic and non-glycemic effects, tolerability and side effects, and impact on vascular health.     

Cytomegalovirus Localization in Atherosclerotic Plaques is Associated with Acute Coronary Syndromes: Report of 105 Patients

It has been shown that cytomegalovirus (CMV) is present in coronary atherosclerotic plaques, but the clinical rele-vance of this presence remains to be elucidated. In this study we sought to examine CMV infection in atherosclerosis patients defined by different methods and to identify the clinical significance of CMV replication in the atherosclerotic plaques. The study included 105 consecutive patients who were admitted to our department and underwent coronary artery bypass grafting (CABG) surgical interventions. Coronary atherosclerotic specimens as well as 53 specimens from the mamillary artery of these same patients were analyzed. Enzyme-linked immunosorbent assay (ELISA) and poly-merase chain reaction (PCR) methods were used for evaluations. The CMV PCR test result was positive for 28 (26.7%) of patients with coronary artery atherosclerosis. After adjusting for other risk factors, coronary artery disease patients with a history of acute coronary syndrome were more likely to be positive for CMV PCR test (P=0.027; odds ratio: 4.2; 95% CI: 1.18-15.0). They were also more likely to have a positive family history for cardiovascular diseases (CVD). This study confirms previous evidence about the replication of CMV virus in the atherosclerotic plaques of coronary arteries and brings clinical significance to this observation by showing a higher prevalence of acute coronary syndromes in those patients with CMV-infected plaques. Our study also suggests a familial vulnerability to CMV replication in the coronary artery walls.