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Introduction: Primary Hyperparathyroidism (PHPT) is an uncommonly diagnosed symptomatic disease that occurs in India. Unlike the Western countries where the disease is asymptomatic, the various manifestations that occur are skeletal, muscular, and renal. Studies on PHPT from developing countries like India, have shown that symptomatic PHPT is still common as compared to developed countries. Data on PHPT from Kerala (a state with health indices comparable to the Western world) are lacking. Aim: To compare the preoperative and postoperative calcium and Parathyroid Hormone (PTH) levels among the participants. Materials and Methods: A record-based retrospective study was conducted at Amrita Institute of Medical Sciences, Cochin, in the Department of Endocrinology. All 116 patients who had PHPT from January 2013 to December 2019 were included in the study. The data available in the hospital records were analysed for various laboratory values and imaging diagnoses. The preoperative and postoperative calcium and PTH levels were compared. Data were analysed by paired sample t-test. (Wilcoxon sign-rank test). The data analysis was done using coGuide. Results: The mean age was 53.42±14.89 years in the study population. Among 116 subjects, (46 (39.66%) were males and 70 (60.34%) were females) eighty-three (71.55%) had the symptomatic disease. The majority, 80 (68.97%) participants underwent single parathyroidectomy. The difference between pre-operative and post-operative calcium and PTH was statistically significant (p-value <0.001). Conclusion: PHPT largely occurs in the fifth decade of life, and the majority were symptomatic. Females were more affected compared to males in the present study. Preoperative imaging is recommended in routine practice. [ABSTRACT FROM AUTHOR]

Refractory hypothyroidism is a commonly encountered clinical challenge despite hypothyroidism being an easy condition to manage. However, truly refractory hypothyroidism accounts for only a small proportion of refractory hypothyroidism, remainder being "pseudo-refractory." The 6-dimensional tool helps guide physicians to manage poorly controlled hypothyroid individuals better. This tool highlights some important aspects of evaluating such individuals such as dialogue, diagnosis, definition of endpoint, disturbance in assay, drug intake (L-thyroxine and concomitant drug intake), and comorbid disease. Most importantly, this simple reader friendly tool can help differentiate truly refractory from pseudo-refractory hypothyroidism. The latter being easily correctable holds the promise of improving the quality of life in such individuals. [ABSTRACT FROM AUTHOR]

Monogenic forms of diabetes in children are frequently misclassified as either type 1 diabetes or young‐onset type 2 diabetes. There is a paucity of literature regarding pediatric monogenic diabetes in the Indian population. A retrospective analysis of case records of 37 children with monogenic diabetes who were diagnosed between 2008 and 2019 in a South Indian tertiary care center was performed. The write‐up describes the clinical, biochemical, and genetic characterization of these patients with the diagnoses of neonatal diabetes mellitus (15 patients), MODY (five patients), and various forms of syndromic diabetes (13 with Wolfram syndrome, two with H syndrome, one with mitochondrial diabetes, and one with thiamine responsive megaloblastic anemia). [ABSTRACT FROM AUTHOR]

Context: Medullary thyroid carcinoma (MTC), being an aggressive disease, requires meticulous follow-up and multidisciplinary management. The clinical presentation, management, outcome of MTC varies among different populations. Aims: An audit was conducted to evaluate the demography, clinical presentation, management, and outcome of MTC in a tertiary care center in South India. Settings and Design: A retrospective analysis was conducted of data from hospital records of patients with MTC treated at our center from 2004 to 2019. Statistical Analysis: All analyses were performed with SPSS software (version 16). Results: Among the 82 patients (M 42, F 40), mean age was 42.07 years (SD 14.5), 46 were operated at our center and 36, outside. Follow-up data were not available for all patients. Median duration of the disease was 36 months and median follow-up was 28 months. Lymphnode dissection was more common (37/46) in patients operated at our center than outside operated patients (17/36) (P < 0.01). At presentation, more than half of the patients had stage IV disease and 8 had distant metastases. Bone, lung, and liver were the common sites of metastases. Persistent hypercalcitoninemia >50 pg/mL was seen in 49.9%. Salvage surgeries of the neck were necessary in 29 patients (38.2%). Mean survival was 66 months and 10-year survival was 35%. Male gender (P = 0.008) and Stage IV disease at presentation (P = 0.038) were associated with poorer survival. Conclusion: MTC, in our population, presented at an advanced stage. Male gender and stage IV at presentation had poor survival. Early diagnosis, aggressive initial neck clearance, close follow-up with tumor markers, appropriate imaging, along with prompt surgical intervention will help to improve outcome. [ABSTRACT FROM AUTHOR]

Context: Insulin autoantibody syndrome (IAAS) is considered to be a rare cause of hyperinsulinaemic hypoglycaemia. Lack of familiarity with the varied clinical manifestations leads to underdiagnosis. Localization techniques aimed at insulin-secreting neoplasms and nesidioblastosis, which are expensive often are ordered when the correct diagnosis is not made. Aims: We describe the myriad of clinical manifestations associated with IAAS based on single centre experience. Settings and Design: Retrospective analysis of patients who got admitted with symptoms suggestive of hypoglycaemia and underwent mixed meal test and prolonged hypoglycaemic test from 2016 to 2019. Subjects and Methods: Retrospective data of 12 patients with IAAS who were diagnosed in the threeyear time period between 2016 and 2019 are included in this analysis. Clinical details, biochemical parameters and imaging modalities were analysed. Statistical Analysis: All analyses were performed with SPSS software (version 17). Results: Total of twelve patients 12 (5 male and 7 females) were identified as IAAS. Median age of presentation was 57 years. Median insulin levels and median C-peptide levels were 300 miu/ml and 18.5 ng/ml respectively. Only 3 (25%) patients had spontaneous resolution. Steroid induced remission occurred by 3 months in the remaining patients. Intermittent hyperglycaemia was seen in 9 (75%) patients. Implicatable drug use preceding the occurrence of the clinical symptoms was observed in five patients. Conclusion: IAAS is not uncommon in India. The diagnosis should be pursued in patients with hyperinsulinaemic hypoglycaemia especially when insulin levels are very high or when there is intermittent hyperglycaemia. [ABSTRACT FROM AUTHOR]

Context: The literature on outcomes of bilateral adrenalectomy (BADx) in Cushing's syndrome (CS) is scant. Aims: The aim of this study is to analyze the short- and long-term outcomes of patients who underwent BADx and to compare the outcomes among different etiologies of CS. Settings and Design: This is a retrospective analysis of patients who underwent BADx for CS at our center between 2005 and 2018. Materials and Methods: In all, 33 patients were studied for clinical outcomes, survival rates, and long-term complications. Statistical Analysis: All analyses were performed with SPSS software (version 21.0). Results: The mean age at surgery was 39.33 ± 15.67 years. The primary etiology for CS was Cushing's disease (CD) in 42.42%, ectopic source in 36.36%, primary pigmented nodular adrenocortical disease (PPNAD) in 12.12%, and adrenocorticotrophin hormone–independent macronodular adrenal hyperplasia (AIMAH) in 9.09% of patients. The median follow-up time was 72.77 months. Improvement in hypertension and diabetes status after surgery was seen in 78% and 76.19% of patients, respectively. Proximal myopathy improved in 68% of patients. Nelson's syndrome and adrenal crisis were seen in 21.4% of patients each on long-term follow-up. Total mortality after BADx was 33.3%. Mortality in the first 30 days after surgery was seen in five patients (15.15%). Higher cortisol levels at presentation and age more than 40 years were predictors of mortality. Among the Cushing's subtypes, PPNAD had the best prognosis followed by CD. Perioperative Infections were a major cause of mortality. Conclusion: BADx is an effective treatment for CS especially in patients with PPNAD and CD but carries a significant mortality rate too. [ABSTRACT FROM AUTHOR]

Background: The ideal management of common bile duct (CBD) stones remains controversial, whether with single-stage management using laparoscopic CBD exploration (LCBDE) during laparoscopic cholecystectomy, or with 2-stage management using preoperative or postoperative ERCP. We wished to elucidate the practice patterns within our health system, which includes both large urban referral centers and small rural critical access hospitals.Study Design: We conducted a retrospective data analysis from our 22-hospital, not-for-profit, integrated healthcare system. All patients with a diagnosis of choledocholithiasis who underwent laparoscopic cholecystectomy (LC) and either ERCP or LCBDE for duct clearance between 2008 and 2013 were included. Demographic data, along with disease-specific characteristics and outcomes, were collected and compared.Results: During the study period, 37,301 patients underwent LC. Of these, 1,961 (5.3%) met inclusion criteria. Single-stage management with LC+LCBDE was performed in 28% of patients, and the remaining 72% underwent 2-stage management with ERCP (73% postoperative ERCP, 27% preoperative). Mean total number of procedures was lowest in the LC+LCBDE group vs the post-cholecystectomy ERCP group vs the preoperative ERCP group (mean 1.4 vs 2.1 vs 2.3; p < 0.05). Hospital charges were also lower in the LC+LCBDE group vs post-cholecystectomy ERCP vs preoperative ERCP groups ($9,000 vs $10,800 vs $14,200; p < 0.05). Single-stage vs two-stage management varied greatly between hospitals (from 0% to 93%).Conclusions: Single-stage management of CBD stones resulted in the fewest procedures and lower hospital charges without an increase in complications. Single-stage management (LC+LCBDE) of CBD stones is underused and can offer better value in today's cost-constrained environment. [ABSTRACT FROM AUTHOR]