ACTA ENDOCRINOLOGICA (BUC)

About 15-20% of adults with Down?s syndrome have autoimmune hypothyroidism. Among patients with Down?s syndrome, prevalence of Graves disease (1-2%) may not be increased compared with the general population. About 15% of the patients with Turner?s syndrome have autoimmune hypothyroidism. Until 2006, only 15 cases of Graves disease have been reported among patients with Turner?s syndrome. We present two adult patients with chromosomial disorders (Down?s syndrome, Turner?s syndrome) with Graves? disease without clinical Graves ophthalmopathy. Clinical picture of thyrotoxicosis was oligosymptomatic in the patient with Down?s syndrome, probably because Down?s syndrome represents a syndrome of accelerated ageing. Both were pretreated with antithyroid drugs before radioiodine therapy. Both patients developed postradioiodine hypothyroidism: after 8 months in the case with Turner?s syndrome and after 11 years in the patient with Down?s syndrome. Adults with these two chromosomial disorders should be screened annually for autoimmune thyroid dysfunction.

Context. We aimed to examine the factors affecting adverse gestational outcome in gestational diabetes (GDM) patients, who were grouped as obese and normal- weight, having only-diet, or insulin treatments. Subjects and Methods. The study included 373 patients, treated with diet or insulin. These patients were sub-grouped as obese and non-obese, and examined retrospectively. The variables affecting adverse gestational outcome in obese GDM patients having dietary and/ or insulin treatments were detected with multiple regression analysis. Results. The weight gained during pregnancy in the GDM group having insulin treatment was more than the one in only-diet treated GDM group (p=0.004). Pre-pregnancy body mass index, the weight gained during pregnancy, hemoglobin A1C levels in the second and third trimesters, caesarian rates were higher in the insulin-treated obese patients than in the other groups (p<0.001). The odds ratio for fasting blood glucose level in insulin-treated obese GDM group was 1.081 (95% CI =1.004 - 1.163) (p=0.039); and it was 0.982 (95% CI =0.924 - 1.002) (p=0.048) for the weight gained during pregnancy, in only-diet treated obese GDM patients. Conclusion. The control of weight gained during pregnancy, and of fasting blood glucose levels in obese patients having GDM, is important to decrease adverse gestational outcome.

Introduction. Antigonadotropic effect of melatonin in seasonally breeding rodents is due to the influence of this indol on the reproductive axis hypothalamicpituitary-gonads, although it is known that melatonin has a negative effect on the hormonal function of the reproductive axis, and is unknown whether it can affect the fertility of male Wistar rats.\r\nAim. The objective of this study was to investigate the effects of melatonin application on the fertility of the male Wistar rat.\r\nMaterials and Methods. Sixteen male Wistar rats were used. Rats were divided into two groups, Control and the group of animals treated with melatonin 1mg/kg (M) injected i.p. daily for two months. The evaluation of fertility was\r\ndetermined by the number of pregnant female rats. In addition, it was quantified the number of spermatozoa and Leydig cells in the seminiferous tubules. After removing\r\nthe treatment for two months, the experiments were repeated, to determine the possible recovery.\r\nResults. The results showed that rats treated with melatonin were able to impregnate only 25% of the mated female rats\r\n(p<0.05). In this group, the number of sperm decreased with a significance of p< 0.001 while the number of Leydig cells increased with respect to the control group with a significance of p< 0.001. After removing the treatment for two months, both the sperm and the Leydig cells were recovered at similar levels as the control group, while the fertility was not totally recovered in the group treated\r\nwith melatonin. These results suggest that the exogenous melatonin application (1mg/kg i.p) induces infertility.

Objective. Unilateral adrenalectomy (UA) is an alternative for treatment in bilateral adrenal incidentaloma (AI) to avoid possible long-term risks of bilateral adrenalectomy. In this study, we aimed to evaluate the effectiveness of UA in bilateral AI patients with subclinical hypercortisolemia (SH). Method. A total of 35 patients were included in this study. The patients were divided into two groups; those who underwent UA (n=27) and patients without adrenalectomy (PWA) (n=8). Hormone tests related to cortisol mechanism were reviewed to analyze results at the time of diagnosis compared to the latest available results to figure out any changes in cortisol mechanism and determine whether SH has recovered or not. Results. Median age of PWA group were higher compared to UA group (p=0.03). Median duration of followup in groups were similar (p=0.3). In the PWA group, none of the patients recovered from hypercortisolemia during their follow-up. In UA group 92.6% of the patients went into remission, whereas during follow-up 3.3% had recurred and another 3.3% were found to have post-adrenalectomy persistent SH. Patients in UA group had lower final cortisol level following dexamethasone suppression (p=0.003) and higher final adrenocorticotrophic hormone (ACTH) levels (p=0.001) than patients in PWA group. In UA group, final basal cortisol level (p=0.009) and final cortisol level after 1 mg dexamethasone suppression test (DST) (p=0.004) were lower than corresponding levels at the time of diagnosis. Discussion. Our study demonstrates unilateral adrenalectomy targeting the side with the larger lesion is an effective approach to reduce excess cortisol levels in bilateral AI patients with SH.

A number of recent studies in animals and humans have linked energy regulation and the circadian clock at the molecular, physiological and behavioural levels, concluding that disruption of clock genes results in metabolic dysregulation. The search to understand the causes of obesity and diabetes and the development of new therapeutic strategies have mostly focused on caloric intake and energy balance. In this review, we present a global overview of the circadian clock as a critical interface between nutrition and homeostasis.

Sialadenosis is an uncommon, noninflammatory condition which usually causes bilateral, diffuse enlargement of the\r\nsalivary glands, particularly of the parotid. Sialadenosis has been associated most often with alcoholism, eating disorders,\r\nmalnutrition, medications and diabetes mellitus. Herein, we describe a case report of a patient with sialadenosis precipitated by poorly controlled diabetes mellitus.

Aim. This study aimed to investigate the clinical presentation and treatment outcomes of newly diagnosed acromegaly patients treated with various combinations\r\nof treatment modalities.\r\nMaterials and Methods. Eighty-four acromegaly patients (42 female, 42 male, mean age 40.29?13.32 y), followed and\r\ntreated between 2000 and 2010 were included. Data on patient demographics, delay between onset of symptoms and the\r\ndiagnosis, details of treatments, and treatment- or disease-related morbidity and mortality were collected and analyzed. The median delay time for diagnosis was 60.71 ? 56.19 months. Patients received various combinations of surgery, gamma knife radiotherapy or conventional radiotherapy,\r\nlong acting somatostatin analogue, and dopamine agonist.\r\nResults. After ten years of treatment, median GH and IGF-1 values decreased from 13.7 ng/mL (IQR: 5.3- 34) to 1.04\r\nng/mL (IQR: 0.52-2.7) and from 600 &#956;g/L (IQR: 460.5-787.5) to 194 &#956;g/L (IQR :157-356), respectively (p<0.0001).\r\nMedian GH during 75 g oral glucose tolerance test (OGTT) decreased from 4.35 ng/mL (IQR: 1.87-13.15) to 1.40 ng/mL\r\n(IQR: 0.60-3.40) (p<0.0001). Fifty patients (59.5%) were in remission according to IGF-1 values and 52 patients (62 %) were cured according to nadir GH during OGTT respectively. The mortality rate was 5.9%.\r\nIn conclusion. Strict biochemical control following appropriate intervention significantly reduces both morbidity and mortality in acromegaly patients.

Objective. This study aims to determine the prevalence of neuropathy in the prediabetic period. Design, Subjects and Method. Informed consent was attained from the patients who volunteered to participate in the study after ethics committee approval was obtained. Patients under the age of 18, having vitamin B12 or folic acid deficiency, history of collagen tissue-rheumatological disease, chronic kidney failure, cirrhosis, ethylism, thyroid disease, autoimmune disease, malignancy, tuberculosis, type 1 or 2 diabetes mellitus and pregnant women were excluded from the study. Patients diagnosed with prediabetes were evaluated by the DN4 neuropathy complaint questionnaire. Neuropathy was diagnosed in patients having a score of four or more. For the statistical analyses Student t-test, Pearson chi-square test, and Fisher's exact test were performed using the NCSS program. Results. A total of 224 volunteers, 167 women and 57 men, were included in the study. The mean age of the participants was 51 and the mean level of hemoglobin A1C was 5.9. Neuropathy was detected in 45% of the cases. Especially in women, there was a significant increase in the frequency of neuropathy compared to men. The most common complaints found in our study were burning sensation and numbness in the extremities. Conclusions. Similar to diabetic patients, prediabetic patients also have a high rate of neuropathy. For the early diagnosis of neuropathy and to be treated promptly, screening tests such as DN4 should be performed for all prediabetic patients. According to the test results, advanced examinations such as EMG or biopsy should be performed earlier.

Background. Langerhans cell histiocytosis (LCH) is a rare group of neoplastic diseases resulting from Langerhans dendritic cells. The most common site (80%) is bones. Thyroid gland involvement is exceedingly rare and usually expected to be seen as a part of multisystemic disease. Case Report. We present a 45 year old male patient operated due to multinodular goiter and neck pain, and diagnosed with LCH in his postoperative pathologic examination. As a result of the systemic screening performed after the pathological diagnosis, the disease was interestingly localized to the thyroid gland. Systemic involvement did not develop in the two-year follow-up of the patient who did not receive additional chemotherapy treatment. Conclusion. It is difficult to diagnose LCH in the thyroid gland before surgery. Although surgical treatment with or without chemotherapy is recommended, surgery is not recommended alone since it is generally systemic involvement. However, in primary thyroid LCH cases limited to the thyroid gland, we recommend that only total thyroidectomy treatment should be kept in mind.

Proprotein convertase 1/3 (PC 1/3) deficiency is a rare, autosomal recessive disorder caused by mutations in the PCSK1 gene. The disease is characterized by earlyonset chronic diarrhea/malabsorption, followed by severe obesity and hormonal deficiencies such as hypocortisolism, hypothyroidism, diabetes insipidus, hypogonadism, growth deficiency, and diabetes mellitus. Ewing’s sarcoma is a rare tumor, usually of small dimensions of neuroectodermal origin that is difficult to distinguish pathologically from a primitive neuroectodermal tumor. A 22-year-old female patient with PC 1/3 deficiency was admitted to our clinic with recurrent urinary tract infections. Magnetic resonance imaging (MRI) revealed an 11x12 cm pelvic mass displacing the uterus. A core-needle biopsy was performed on the pelvic mass. As a result of the pathological evaluation, ıt was diagnosed with pelvic Ewing’s sarcoma. The patient was started on the VAC-IE chemotherapy protocol. We report a case of pelvic Ewing’s sarcoma in a patient with PC 1/3 deficiency. Further research is needed to assess malignancy risk in metabolic disorders including very rare disorders like PC 1/3 deficiency.