The current management of thalassemia includes regular transfusion programs and chelation therapy. It is important that physicians be aware that endocrine abnormalities frequently develop mainly in those patients with significant iron overload due to poor compliance to treatment, particularly after the age of 10 years. Since the quality of life of thalassemia patients is a fundamental aim, it is vital to monitor carefully their growth and pubertal development in order to detect abnormalities and to initiate appropriate and early treatment. Abnormalities should be identified and treatment initiated in consultation with a pediatric or an adult endocrinologist and managed accordingly. Appropriate management shall put in consideration many factors such as age, severity of iron overload, presence of chronic liver disease, thrombophilia status, and the presence of psychological problems. All these issues must be discussed by the physician in charge of the patient's care, the endocrinologist and the patient himself. Because any progress in research in the field of early diagnosis and management of growth disorders and endocrine complications in thalassemia should be passed on to and applied adequately to all those suffering from the disease, on the 8 May 2009 in Ferrara, the International Network on Endocrine Complications in Thalassemia (I-CET) was founded in order to transmit the latest information on these disorders to the treating physicians. The I-CET position statement outlined in this document applies to patients with transfusion-dependent thalassemia major to help physicians to anticipate, diagnose, and manage these complications properly.

Congenital hyperinsulinism (CHI) is the result of unregulated insulin secretion from the pancreatic β-cells leading to severe hypoglycaemia. In these patients it is important to make an accurate diagnosis and initiate the appropriate management so as to avoid hypoglycemic episodes and prevent the potentially associated complications like epilepsy, neurological impairment and cerebral palsy. At a genetic level abnormalities in eight different genes (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF4A and UCP2) have been reported with CHI. Loss of function mutations in ABCC8/KCNJ11 lead to the most severe forms of CHI which are usually medically unresponsive. At a histological level there are two major subgroups, diffuse and focal, each with a different genetic etiology. The focal form is sporadic in inheritance and is localized to a small region of the pancreas whereas the diffuse form is inherited in an autosomal recessive (or dominant) manner. Imaging using a specialized positron emission tomography scan with the isotope fluroine-18 L-3, 4-dihydroxyphenyalanine (18F-DOPA-PET-CT) is used to accurately locate the focal lesion pre-operatively and if removed can cure the patient from hypoglycemia. Understanding the molecular mechanisms, the histological basis, improvements in imaging modalities and surgical techniques have all improved the management of patients with CHI.

The Indian Diabetes Risk Score was initially developed by the Madras Diabetes Research Foundation (MDRF-IDRS) to help detect undiagnosed Type 2 diabetes (T2DM) in the community. Soon it was found that the MDRF-IDRS could also help to predict incident diabetes, metabolic syndrome, coronary artery disease (CAD), non-alcoholic fatty liver disease as well as sleep disorders in the community. It helps to differentiate T2DM from non-T2DM. Finally, it also helps to identify those with CAD, peripheral vascular disease and neuropathy among those with T2DM. Thus, the MDRF-IDRS is a simple, virtually 'no cost' tool which is useful in several clinical and epidemiological settings.

Polycystic ovary syndrome affects 6 to 15% of reproductive age women worldwide. It is associated with increased risk of miscarriage, gestational diabetes mellitus, hypertensive disorders of pregnancy, preterm delivery, and birth of small for gestational age infant. Many studies on issues relating to pathophysiology and management of these complications have been published recently. These issues are being reviewed here using relevant articles retrieved from Pubmed database, especially from those published in recent past.

Luteal phase insufficiency is one of the reasons for implantation failure and has been responsible for miscarriages and unsuccessful assisted reproduction. Luteal phase defect is seen in women with polycystic ovaries, thyroid and prolactin disorder. Low progesterone environment is created iatrogenically due to interventions in assisted reproduction. Use of gonadotrophin-releasing hormone analogs to prevent the LH surge and aspiration of granulosa cells during the oocyte retrieval may impair the ability of corpus luteum to produce progesterone. Treatment of the underlying disorder and use of progestational agents like progesterone/human chorionic gonadotrophin have been found to be effective in women with a history of recurrent miscarriage. There has been no proved beneficial effect of using additional agents like ascorbic acid, estrogen, prednisolone along with progesterone. Despite their widespread use, further studies are required to establish the optimal treatment. Literature review and analysis of published studies on luteal phase support.

The myometrium must remain relatively quiescent during pregnancy to accommodate growth and development of the feto-placental unit, and then must transform into a highly coordinated, strongly contracting organ at the time of labour for successful expulsion of the new born. The control of timing of labour is complex involving interactions between mother, fetus and the placenta. The timely onset of labour and delivery is an important determinant of perinatal outcome. Both preterm birth (delivery before 37 week of gestation) and post term pregnancy (pregnancy continuing beyond 42 weeks) are both associated with a significant increase in perinatal morbidity and mortality. There are multiple paracrine/autocrine events, fetal hormonal changes and overlapping maternal/fetal control mechanisms for the triggering of parturition in women. Our current article reviews the mechanisms for uterine distension and reduced contractions during pregnancy and the parturition cascade responsible for the timely and spontaneous onset of labour at term. It also discusses the mechanisms of preterm labour and post term pregnancy and the clinical implications thereof.

Fetus exists in a complex, dynamic, and yet intriguing symbiosis with its mother as far as fuel metabolism is concerned. Though the dependence on maternal fuel is nearly complete to cater for its high requirement, the fetus is capable of some metabolism of its own. The first half of gestation is a period of maternal anabolism and storage whereas the second half results in exponential fetal growth where maternal stores are mobilized. Glucose is the primary substrate for energy production in the fetus though capable of utilizing alternate sources like lactate, ketoacids, amino acids, fatty acids, and glycogen as fuel under special circumstances. Key transporters like glucose transporters (GLUT) are responsible for preferential transfers, which are in turn regulated by complex interaction of maternal and fetal hormones. Amino acids are preferentially utilized for growth and essential fatty acids for development of brain and retina. Insulin, insulin like growth factors, glucagon, catecholamines, and letpin are the hormones implicated in this fascinating process. Hormonal regulation of metabolic substrate utilization and anabolism in the fetus is secondary to the supply of nutrient substrates. The knowledge of fuel homeostasis is crucial for a clinician caring for pregnant women and neonates to manage disorders of metabolism (diabetes), growth (intrauterine growth restriction), and transitional adaptation (hypoglycemia).

The hepatitis C virus infection represents an important public health problem and is associated with various hepatic and extrahepatic manifestations. Symptoms outside of the liver can occur in multiple organ systems, including hematologic, renal, dermatologic, endocrine, and rheumatologic systems. Among these different organ systems, special attention has focused on the endocrine system because it affects almost every organ in the body. Among the endocrine disorders, thyroid problems are the most common and the thyroid is one of the principal target organs for extrahepatic manifestations in HCV infected patients. In addition, research data suggest that interferon treatment may be associated with immune-mediated thyroid lesions. However, case reports suggest that the response of thyroid extrahepatic manifestations to interferon in patients with chronic HCV is greatly different. The objective of this study was to summarize currently available data on thyroid conditions associated with chronic HCV infection. Moreover, we investigate the incidence of the development of immune mediated thyroid disorders during interferon therapy in these patients.

Vitamin D deficiency is a preventable health problem. Vitamin D deficiency among pregnant women is frequent in many populations over the world. Research indicates that adequate vitamin D intake in pregnancy is optimal for maternal, fetal and child health. Adverse health outcomes during pregnancy are preeclampsia; gestational diabetes mellitus and caesarean section. Consequences in newborns are low birth weight, neonatal rickets, a risk of neonatal hypocalcaemia, asthma and/or type 1 diabetes. Vitamin D deficiency during pregnancy is the origin for a host of future perils for the child, especially effect on neurodevelopment and immune system. Some of this damage done by maternal Vitamin D deficiency gets evident after many years. Therefore, prevention of vitamin D deficiency among pregnant women is essential. The currently recommended supplementation amount of vitamin D is not sufficient to maintain a value of 25 hydroxy vitamin D above 30 ng/ml, during pregnancy. Studies are underway to establish the recommended daily doses of vitamin D in pregnant women. Clearly, further investigation is required into the effects of vitamin D, of vitamin D supplementation, and of vitamin D analogs for improvement in human health generally and mothers and children specifically. This review discusses vitamin D metabolism, dietary requirements and recommendations and implications of vitamin D deficiency during pregnancy and lactation.

The natriuretic system consists of the atrial natriuretic peptide (ANP) and four other similar peptides including the wrongly named brain natriuretic peptide (BNP). Chemically they are small peptide hormones predominantly secreted by the cardiac myocytes in response to stretching forces. The peptide hormones have multiple renal, hemodynamic, and antiproliferative effects through three different kinds of natriuretic receptors. Clinical interest in these peptide hormones was initially stimulated by the use of these peptides as markers to differentiate cardiac versus noncardiac causes of breathlessness. Subsequently work has been done on using these peptides to prognosticate patients with acute and chronic heart failure and those with acute myocardial infraction. Synthetic forms of both atrial- and brain-natriuretic peptides have been studied and approved for use in acute heart failure with mixed results. This review focuses on the biochemistry and physiology of this fascinating hormone system and the clinical application of these hormones.

Objective: To share our clinical experience with exenatide in obese North Indian subjects with type 2 diabetes. Materials and Methods: We share our experience with use of exenatide in 74 patients treated at Indraprastha Apollo Hospital, a tertiary care centre in New Delhi, India Subjects included obese / overweight subjects (mean weight and BMI; 97.67 ± 5.6 kg and 34.56 kg/m ² ) with known history of type 2 DM (Mean: 9 ± 5.6 years) and maintaining suboptimal glycemic control (HbA1c >7%) on oral antidiabetic agents, with or without basal insulin. Metformin and sulphonylureas were continued (with dose adjustment if indicated), as was basal insulin (glargine / detemir). TZDs and DPP4 inhibitors were discontinued. The dose of exenatide was increased to 10 mcg twice a day after 4-12 weeks. 56 patients completed minimum 3 month therapy. 42 patients completed 6 months, 32, 9 months and 25 completed 12 months. Data of patients who had completed at-least 3 months of therapy was included for analysis. Results and Discussion: 69.77, 67.44, and 13.95% of the patients were receiving metformin, secretagogues or thiazolidinediones alone or in combination; 17.76% of the patients were on basal insulin. The change in fasting and post-prandial blood glucose levels were significant at 3, 6, 9 and 12 months with p0 -value <0.05. The mean weight loss at one, three, and six months and one year was 1.7 ± 1.3, 3.8 ± 2.5, 6.3 ± 3.4, and 8.3 ± 4.3 kg, respectively (P <0.05). The mean HbA1c (baseline: 8.8 ± 1.3%) at 3, 6 months and at one year was 7.8 ± 0.9, 7.7 ± 0.8 and 7.2 ± 0.8 (P <0.05). Thirty-five percent of the patients had a 'good' A1c value (< 7%) at the end of 12 months. 13 patients discontinued exenatide (three due to lack of response, six due to cost of therapy and four due to severe nausea). Nausea was the most common side effect, occurring in 95% patients within 1 month, although the incidence declined with passage of time. Conclusions: Clinical use of Exenatide is associated with significant improvement in glycemic control and major weight loss (8.3±4.3 kg at 1 year) in obese subjects with type 2 diabetes. Nausea is the most common side effect. In conclusion, exenatide is a effective and useful option for treatment of type 2 diabetes in obese Indian subjects.

Aim: Obesity is the most common cause of insulin resistance and metabolic syndrome (MS). These are the most important risk factors for coronary heart disease (CHD). No evidence exists regarding the prevalence of the MS in children in sSrinagar city of Kashmir India. We aimed to evaluate the prevalence of MS in 8-18-year-old school-going children of Kashmir, India. Materials and Methods: In this cross-sectional study, 758 respondents in 8-18 years of age were randomly selected using a simple random sampling method. The self-designed questionnaire was individually completed after receiving a written informed consent. The weight, height, waist circumference (WC), body mass index (BMI), and blood pressure were measured using standard tools. Ten milliliters of blood was taken for measuring lipid profile and fasting blood sugar (FBS) of the school children. We determined MS according to the modified Adult Treatment Panel III (ATP III) criteria. Results: The prevalence of the MS was 3.8% (boys: 3.9%, girls: 3.8%) and the prevalence of obesity was 9.9% (boys: 9.9%, girls: 10.6%) among the studied children. Obese subjects had the highest proportion of MS compared with those at risk for overweight and those with normal weight (30.7% vs. 2.5% and 0.5%, respectively; P = 0.000). Conclusion: The MS is prevalent even in young children, so we suggest screening programs for children aged 8-18 years to control obesity and MS in the developing world.

The study was aimed to determine the association of angiotensin converting enzyme (ACE) insertion/deletion (I/D) and apolipoprotein E (Apo E) Hha I gene polymorphisms with obesity, fat mass, and blood glucose levels in Asian Indian population. A total of 350 (184 men and 166 women) adult (30 years and above) Asian Indians of Calcutta and suburb participated in the study. Anthropometric measures, fat mass, and blood glucose measures were collected. Out of 350 subjects, a sample of 139 individuals was collected randomly for genotyping (adjusted for age and sex). The ACE and Apo E genotypes were determined by agarose gel electrophoresis. It was observed that neither ACE (I/D) nor Apo E (Hha I) gene polymorphisms showed any significant association with body mass index, waist circumference, fat mass, fasting, and post meal blood glucose levels. Even synergistically (ACE + Apo E), these two polymorphisms showed no significant association with obesity, fat mass, and blood glucose level. ACE (I/D), Apo E (Hha I), as well as ACE + Apo E seem to have no significant association with obesity, fat mass, and blood glucose levels in this population.

Introduction : Notable lipid abnormalities in DM include elevated LDL-C which have been reported to be the prevalent lipid abnormality in DM and elevated total cholesterol levels. Although the Statins are widely used in the management of lipid abnormalities, their effects on the lipid abnormalities in Nigerians with DM has not been extensively evaluated. Objective: This report sets out to determine the effect of Simvor, a brand of Simvastatin in Nigerians with DM and abnormal lipid profiles. Materials and Methods : A total of 300 diabetic patients with abnormal lipid profile who were treatment naοve for lipid disorders were longitudinally recruited for the study. They were managed with Simvastatin (Simvor) in doses ranging from 20-40 mg alongside dietary counseling and exercise recommendation. Results: The mean age (SD) of the study subjects was 58.4 (10 years). The male; female ratio was 99:211. The proportions of lipid abnormalities for LDL-C, TCHOL, HDL-C and TG were 87%, 45%, 53% and 7% respectively. Following Simvastatin (Simvor) treatment, the mean LDL-C value was reduced by 16%, TCHOL by 23%, TG by 6% and HDL-C increased by 10%. Simvastatin (Simvor) was generally well tolerated and no cardiovascular events were noted in the study subjects during the period of the study. Conclusion: Simvastatin (Simvor) was effective and well tolerated in the management of lipid disorders in Nigerians with DM.

Aim: The aim of this study was to assess and compare the response to two forms of treatment-immobilization with zoledronic acid injection and immobilization with oral weekly Alendronate, in patients with diabetes mellitus and acute Charcot arthropathy (CA) of foot in terms of clinical and radiological parameters. Material and Methods: Patients attending the endocrinology and podiatry clinic with history of diabetes mellitus and Acute CA were taken for study. The patients were randomized into two treatment groups. Group Z-zoledronic acid injection along with total contact cast (TCC). Group A-Tab. Alendronate 70 mg. once a week till the complete clinical resolution of acute CA along with TCC. Forty-five patients were randomized and 40 of them completed the study. The primary end point was complete clinical resolution of acute CA-defined as temperature difference between normal and affected foot <1^oF. Results: Among the 40 patients, 30 (75%) had complete clinical resolution. The mean number of days taken for complete clinical resolution since the initiation of treatment (either Zoledronic acid or Alendronate) was approximately 122 days. There was no significant difference in a number of days required for complete clinical resolution, between the two forms of therapy. There was more than 50% reduction in the visual score between the baseline and the final scan. The target to non-target ratio in the skeletal phase also showed an average of 40% reduction from the baseline to the final skeletal scintigraphy. Conclusion: Both Intravenous Zoledronic acid and oral alendronate had comparable efficacy with respect to the time taken for attaining complete clinical resolution of acute CA of foot. However, Alendronate therapy was cost effective among the two. ^99m Tc MDP bone scan can be used as an adjuvant to the clinical parameters in assessing the response to therapy.

Aims and Objectives : To assess the prevalence of metabolic syndrome (MetS) in human immunodeficiency virus (HIV) positive patients. Prevalence of MetS was compared in patients who were not on highly active antiretroviral therapy (HAART) to patients who were on HAART. Materials and Methods: Seventy HIV positive cases were studied. Pregnant and lactating women, patients on drugs other than HAART known to cause metabolic abnormalities and those having diabetes or hypertension were excluded. Cases were evaluated for MetS by using National Cholesterol Education Program Adult Treatment Panel-III. Results: 47 cases were on HAART and 23 cases were not on HAART. Fasting Blood Glucose ≥100 mg/dl was present in 28.6% cases, out of whom 27.7% were on HAART and 30.4% were not on HAART (P = 0.8089). 12.9% cases had BP ≥130/≥85 mm Hg, out of whom 14.9% were on HAART and 8.7% were not on HAART (P = 0.4666). 42.9% cases had TG ≥150 mg/dl, out of whom 44.7% were on HAART and 39.1% were not on HAART (P = 0.6894). HDL cholesterol was low (males <40 mg/dl, females <50 mg/dl) in 50% cases, out of whom 55.3% were on HAART and 39.1% were not on HAART (P = 0.2035). Conclusions: Prevalence of MetS was 20%. Majority of patients had only one component of MetS (32.9%). Low HDL was present in 50%, followed by raised triglycerides in 42.9%. Waist circumference was not increased in any of the patients. There was no statistically significant difference between those on HAART and those not on HAART in distribution of risk factors and individual components of MetS.

Background: Congenital adrenal hyperplasia (CAH) is an inherited recessive disorder of adrenal steroidogenesis. The enzymes most commonly affected are 21-hydroxylase. Past reports suggested brain magnetic resonance imaging (MRI) abnormalities in CAH patients, affecting white matter signal, temporal lobe and amygdala structure and function. Aims: In the present study, we aimed to investigate the frequency of white matter changes and temporal lobes structures dysgenesis in a population of patients having CAH due to 21-hydroxylase deficiency. Materials and Methods: Neurological examination and brain MRI were performed in 26 patients. Results: Neurological examination revealed mental retardation in three patients, tremor in two patients, tendon reflexes asymmetry in one patient, and cerebellar syndrome in one patient. Eleven patients (42.3%) showed MRI abnormalities: Eight of them had white matter hyperintensities, one patient had moderate atrophy in the right temporal, and hippocampal dysgenesis was found in the remaining two patients. Conclusions: Brain MRI abnormalities in CAH patients include white matter hyperintensities and temporal lobe structures dysgenesis. The mechanisms involved seem related to hormonal imbalances during brain development and exposure to excess exogenous glucocorticoids. Clinical implications of such lesions remain unclear. More extensive studies are required to define better the relationships between brain involvement and different CAH phenotypes and treatment regimens.

Background: Demonstration of central: Peripheral adrenocorticotropic hormone (ACTH) gradient is important for diagnosis of Cushing's disease. Aim: The aim was to assess the utility of internal jugular vein (IJV): Peripheral vein ACTH ratio for diagnosis of Cushing's disease. Materials and Methods: Patients with ACTH-dependent Cushing's syndrome (CS) patients were the subjects for this study. One blood sample each was collected from right and left IJV following intravenous hCRH at 3 and 5 min, respectively. A simultaneous peripheral vein sample was also collected with each IJV sample for calculation of IJV: Peripheral vein ACTH ratio. IJV sample collection was done under ultrasound guidance. ACTH was assayed using electrochemiluminescence immunoassay (ECLIA). Results: Thirty-two patients participated in this study. The IJV: Peripheral vein ACTH ratio ranged from 1.07 to 6.99 ( n = 32). It was more than 1.6 in 23 patients. Cushing's disease could be confirmed in 20 of the 23 cases with IJV: Peripheral vein ratio more than 1.6. Four patients with Cushing's disease and 2 patients with ectopic ACTH syndrome had IJV: Peripheral vein ACTH ratio less than 1.6. Six cases with unknown ACTH source were excluded for calculation of sensitivity and specificity of the test. Conclusion: IJV: Peripheral vein ACTH ratio calculated from a single sample from each IJV obtained after hCRH had 83% sensitivity and 100% specificity for diagnosis of CD.

Objective: To estimate the prevalence of metabolic syndrome among adolescents attending school in the Jammu region, India. Materials and Methods: This is a cross-sectional study conducted between November 2009 and December 2010, among a total of 1160 school-going adolescents of both sexes aged 10-18 years. Relevant metabolic and anthropometric variables were analyzed and criteria suggested by National Cholesterol Education Program Adult Treatment Panel Third (NCEP-ATP III) modified for age was used to define metabolic syndrome. Results: The overall prevalence of metabolic syndrome was 2.6%. Prevalence of metabolic syndrome was higher in males (3.84%) than in females (1.6%) and slightly higher in urban area (2.80%) than in rural area (2.52%), whereas prevalence of metabolic syndrome among centrally obese subjects was as high as 33.33%. High density lipoprotein cholesterol was the most common and high blood pressure was the least common constituent of metabolic syndrome. Metabolic syndrome was most prevalent in 16-18 years age group (4.79%). Conclusion : This study demonstrates that metabolic syndrome phenotype exists in substantial number (up to 3%) of adolescent population in the Jammu region, India, and particularly 33% of obese adolescents are at risk to develop metabolic syndrome. These findings pose a serious threat to the current and future health of these young people.

Background: Polycystic ovary syndrome (PCOS) is common diagnosis in women presenting with infertility. All the dimensions of PCOS have not been completely explored. Many studies have tried to characterize the exact presentation of the disease. In this study we studied clinical features of PCOS in Indian women to characterize different phenotypes of this syndrome. Prevalence of acanthosis nigricans (AN) as surrogate marker of insulin resistance, obesity, hirsutism and hypothyroidism in PCOS women have been simultaneously studied. Materials and Methods: Present work is a non comparative cross-sectional open label study carried out over a period of 18 months in an endocrinology hospital in western Maharashtra, India. Results and Conclusion: Authors conclude that PCOS occurs both in obese and non-obese women; AN and hirsutism occur in equal proportion of patients. AN is correlated with obesity. Hormonal dysfunctions in PCOS manifested together or independently. PCOS women can be sub grouped based on clinical features suggestive of endocrinological malfunctions and can be investigated accordingly for selection of appropriate treatment modalities.

Background: Gestational diabetes mellitus (GDM) is a perfect window of opportunity for the prevention of DM in two generations, and its incidence is increasing in our country. Awareness of the condition among antenatal women will translate into prevention and early diagnosis of the disease. This study was done to determine the awareness of GDM among all the antenatal women who attend a Primary Health Center (PHC) for antenatal care. Materials and Methods: A pretested questionnaire consisting of details on background characteristics, 12 questions focusing on Type 2 DM and GDM, and a question on the source of knowledge was administered to all women attending the antenatal clinic. Their responses were scored and the women were graded as having good, fair, or poor knowledge about GDM. Results: One hundred and twenty antenatal women participated in the study. Mean age of the women was 23.8 years (SD: 2.94). Overall, 17.5% women had good knowledge, 56.7% had fair knowledge, and 25.8% women had poor knowledge about GDM. The major sources of awareness of GDM were reported to be television/radio, neighbors/friends, and family members. Discussion: Only a small proportion of rural antenatal women had good knowledge about GDM. The awareness that untreated GDM may pose a risk to the unborn child was high among the study women. Health care workers have to play a greater role in bringing about awareness about GDM among antenatal women.

The prevalence of Diabetes is projected to increase to 80 million by 2030, placing an immense burden on the health care resources of our country. Thus, diabetes poses a challenge to health systems and the individual. Hence, it is necessary to look at adjuncts to effective management of Diabetes; adjuncts which are not resource intensive and are nearer to the community that people live in. Yoga holds promise as a therapeutic intervention and health promotion measure. This brief communication explores the studies done to date on the beneficial effects of Yoga on Diabetes.

Adrenal incidentaloma was detected in an 81-year-old male patient and a 37-year-old female patient who had been diagnosed with essential thrombocytosis. Each patient's Janus Kinase 2 (JAK2) V617F mutation was positive, and they were evaluated as having non-functional adrenal incidentaloma. The JAK2 activates the signal transducers and activators of transcription (STAT) proteins which then activate the phosphoinositol-3 kinases, Ras, mitogen-activated protein (MAP) kinases, and transcription. Constitutive activation causes cell proliferation and dysregulation of apoptosis. It is thought that STAT3 activation-mediated JAK family kinases have a central role in the solid tumor cell series. Permanent activation of STAT3 and STAT5 causes tumor cell proliferation, survival, metastasis, and an increase in tumor-mediated inflammation in solid and hematologic tumors. According to our literature screening, irregular JAK signaling, seen at the pathogenesis of many solid and hematologic tumors, has not been previously evaluated with regard to adrenal tumors. As a result, our cases are the first coexistence of JAK V617F mutation with adrenal incidentaloma in the literature. Because of this, we think that JAK2 mutation must be evaluated to clarify the etiology of adrenal incidentalomas.

The differential diagnosis for precocious puberty in a young female includes peripheral causes. This case report documents a rare cause of isosexual precocious puberty, a juvenile granulosa cell tumor of the ovary-and a brief literature review. A 7-year-old girl presented with rapid onset of pubertal development and elevated estradiol levels. Abdominal ultrasound revealed a mass in the right adnexa. Other causes of precocious puberty were excluded. Elective surgery was planned, but the patient presented to the emergency room with torsion of ovary. She underwent an exploratory laparotomy for tumor resection and right salpingo oophorectomy. Pathology reported a juvenile granulosa cell tumor of the ovary. Postoperatively, she experienced a cessation of vaginal bleeding and estradiol levels normalized. Early stage disease has good prognosis. Adjuvant chemotherapy is not indicated in this setting.

Peripheral vascular disease is a rare feature of pheochromocytoma. This potentially catastrophic but curable tumor should be suspected in combination of distal necrosis with hypertension and palpable pulses. We report such an unusual case of pheochromocytoma presenting as toe necrosis.

Catecholamine-induced vasculitis is a well known but rarely described entity. However, aortoarteritis as a manifestation of catecholamine-induced vasculitis is not described in the literature. We have reported two patients in whom pheochromocytoma coexisted with aortoarteritis. Both patients were young females with history of bilateral pheochromocytomas in more than one first-degree relative. Both patients also had bilateral adrenal pheochromocytomas (second patient also had paraganglioma at left renal hilum) with elevation of plasma free normetanephrine levels. We conclude that there may be an association between pheochromocytoma and aortoarteritis, and that catecholamine excess may have a role in the etiopathogenesis of aortoarteritis in these patients.

Tumours metastasizing to the pituitary gland are uncommon. Symptomatic patients with pituitary metastases can present with diabetes insipidus, headache, visual field defects and/or anterior pituitary hormonal dysfunction. Treatment options for pituitary metastases include, surgical resection, cranial or parasellar irradiation and/or chemotherapy, and hormonal replacement if indicated. The overall prognosis of pituitary metastases is poor. We present a case of hypopituitarism as the presenting feature of bronchogenic carcinoma with metastases to the pituitary gland.

McCune- Albright Syndrome (MAS) is a rare fibrosseous lesion, characterized by a classic triad of polyostotic fibrous dysplasia (PFD), cafι -au-lait macules (CALM) and underlying endocrinopathies. We present the oral findings of an interesting case of MAS with relevant review of literature. A 30-year-old male presented to us with swelling of both jaws over a period of two years. Cutaneous examination revealed cafι - au - lait macule over the back, crossing the midline. Skeletal survey showed expansile, osteolytic, mixed radiolucent- radiopaque lesions in skull and jaw bones. Serum alkaline phosphatase was elevated (388 IU/L), with normal calcium, phosphorus, parathyroid hormone and 25 hydroxy vitamin D levels. Diagnosis of McCune- Albright syndrome was made and he was treated with parenteral bisphosphonates (intravenous Zoledronate 4 mg) and is under follow up for surgical recontouring of the jaws. Early recognition facilitates better treatment and improves prognosis by reducing the morbidity.

Hypothyroidism presenting as recurrent hypokalemic paralysis is rare in the literature. This transient and episodic neurological condition is commonly associated with thyrotoxicosis. We report a case of young female admitted with recurrent paralytic attacks since last 1 year. She had no symptom of hypothyroidism. She had weakness of all four limbs, delayed relaxation of ankle jerks, and normal higher mental function. There was no enlargement of thyroid. Serum potassium level ranged from 1.6 to 3.2 meq/L during attack with high serum creatine phosphokinase level. Electromyography was normal. The patient was diagnosed having chronic thyroiditis with high thyroid-stimulating hormone and thyroid-related antibodies. Follow up shows satisfactory result with thyroxine replacement. It is an extremely rare and unusual presentation of hypothyroidism, probably the fourth reported case of hypothyroidism with hypokalemic paralysis, to the best of our knowledge.