Spontaneous bilateral adrenal hemorrhage of pregnancy

Spontaneous adrenal hemorrhage of pregnancy is an acute hemorrhage into the adrenal gland in pregnancy in the absence of trauma, tumor or decoagulant therapy. This can have catastrophic consequences on the mother and the baby and if the hemorrhage involves both the adrenal glands the risk is aggravated because of the high incidence of resulting adrenal insufficiency. We report a case of spontaneous bilateral adrenal hemorrhage in pregnancy resulting in adrenal crisis. A 26 year old primigravida presented at 32 weeks of gestation initially with right sided infrascapular pain and one month later with similar pain in the left side associated with high blood pressure. Imaging with ultrasound and MRI was suggestive of bilateral adrenal mass probably hemorrhage; 2 days following the second episode of pain she developed drowsiness and hypotension and a diagnosis of primary adrenal insufficiency was confirmed by a low serum cortisol and high ACTH. She stabilized with hydrocortisone therapy and the fetus was closely monitored. At 37 weeks she had a normal vaginal delivery under steroid cover. Repeat MRI abdomen 3 months after delivery showed resolution of the hemorrhage but biochemically she continued to be cortisol insufficient at 1 year of follow up. Prompt diagnosis of adrenal hemorrhage in pregnancy and treatment of adrenal insufficiency along with close fetal monitoring usually results in good perinatal outcome in spontaneous adrenal hemorrhage of pregnancy

Delayed diagnosis of plasma cell disorder-related Fanconi syndrome in young adults presenting as osteomalacia: report of two cases with normokalemia and normal haematological parameters at the time of presentation

Adult-onset hypophosphatemic osteomalacia is rare and diagnosis is frequently delayed. Fanconi syndrome (FS) due to monoclonal gammopathy is a well-recognized, but rare cause of hypophosphatemia. The relatively young age of patients and normal routine hematological parameters often results in late recognition of this treatable disease entity. Low phosphorus, elevated alkaline phosphatase, mildly impaired renal function and hypokalemia are often the only abnormalities on routine evaluation. We summarize the clinico-pathological features of two cases who initially presented with fractures and proximal myopathy and were subsequently found to have FS secondary to light chain proximal tubulopathy. Atypical features like absence of hypokalemia at presentation  and elevated Fibroblast Growth Factor 23(FGF 23), a marker of oncogenic osteomalacia were noted. Marked clinical improvement and recovery of renal parameters were evident with phosphate supplements and  chemotherapy for the plasma cell disorder. FS due to monoclonal gammopathy  may present with atypical features  and diagnosis may be  challengin

Syndrome of inappropriate antidiuretic hormone secretion: Revisiting a classical endocrine disorder

Hyponatremia occurs in about 30% of hospitalized patients and syndrome of inappropriate antidiuretic hormone secretion (SIADH) is a common cause of hyponatremia. SIADH should be differentiated from other causes of hyponatremia like diuretic therapy, hypothyroidism and hypocortisolism. Where possible, all attempts should be made to identify and rectify the cause of SIADH. The main problem in SIADH is fluid excess, and hyponatremia is dilutional in nature. Fluid restriction is the main stay in the treatment of SIADH; however, cerebral salt wasting should be excluded in the clinical setting of brain surgeries, subarachnoid hemorrhage, etc. Fluid restriction in cerebral salt wasting can be hazardous. Sodium correction in chronic hyponatremia (onset >48 hours) should be done slowly to avoid deleterious effects in brain

Etiological profile, targeted levothyroxine dosing and impact of partial newborn screening in congenital hypothyroidism—A single centre experience

Background: Congenital hypothyroidism (CH) is the most common cause of preventable intellectual disability. Newborn screening (NBS) for CH has been in vogue in many parts of the world since 1970, but despite its well-known benefits, many developing countries including India have not been able to establish universal NBS for CH till date. Objective: The aim of this study was to review the clinical aspects of congenital hypothyroidism in a tertiary care university referral teaching hospital, focusing on aetiology of CH, predictors of permanence, optimal targeted dose strategies based on aetiology and the effect of newborn screening on the time to diagnosis. Material and Methods: The electronic medical records of 233 children with CH referred to our centre between January 2009 and December 2019 were analysed. A partial NBS was established in the state in 2012. Results: Dyshormonogenesis (57.5%) was the most common aetiology of CH. The incidence of transient CH in children with a gland in situ (GIS) was 35%. Levothyroxine (LT-4) dose of >2.75 μg/kg/day (sensitivity 76.5, specificity 72), >2.15 μg/kg/day (sensitivity 82.4, specificity 61.9) and >1.85 μg/kg/day (sensitivity 76.5, specificity 61.9) at years 1, 2 and 3, respectively, were predictors of permanent CH. An initial LT-4 dose ≥8 μg/kg was sufficient and very seldom led to undertreatment in children with dyshormonogenesis. On the contrary, even doses ≥13 mcg/kg/day led to frequent undertreatment in children with thyroid dysgenesis. After the introduction of newborn screening, the median age at diagnosis came down from 45 days (IQR 14–180 days) to ten days (IQR 3–12 days). Conclusion: Targeted dosing based on aetiology of CH may be more appropriate to optimise outcomes. The time to diagnosis of CH reduced significantly after the adoption of even a partial NBS program highlighting the urgent need for implementation of the same in resource poor settings

Laboratory and Imaging Profile of Primary Hyperparathyroidism: A Record Based Retrospective Study

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