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

Pediatric endocrine hypertension

Endocrine causes of hypertension are rare in children and screening for endocrine hypertension in children should be carried out only after ruling out renal and renovascular causes. Excess levels and/or action of mineralocorticoids associated with low renin levels lead to childhood hypertension and this can be caused by various conditions which are discussed in detail in the article. Childhood pheochromocytomas are being increasingly diagnosed because of the improved application of genetic testing for familial syndromes associated with pheochromocytomas. Adolescents with polycystic ovarian syndrome (PCOS) can also have hypertension associated with their obese phenotype

Transient congenital hypothyroidism

Transient thyroid function abnormalities in the new born which revert back to normal after varying periods of time are mostly identified in the neonatal screening tests for thyroid and are becoming more common because of the survival of many more premature infants. It can be due to factors primarily affecting the thyroid-like iodine deficiency or excess, maternal thyroid-stimulating hormone receptor (TSHR) antibodies, maternal use of antithyroid drugs, DUOX 2 (dual oxidase 2) mutations, and prematurity or those that affect the pituitary-like untreated maternal hyperthyroidism, prematurity, and drugs. Most of these require only observation, whereas some, such as those due to maternal TSHR antibodies may last for upto three-to-six months and may necessitate treatment. Isolated hyperthyrotropinemia (normal Tetraiodothyronine (T4) and high Thyroid Stimulating hormone (TSH)) may persist as subclinical hypothyroidism in childhood. Transient hypothyroxinemia (low T4 and normal TSH) is very common in premature babies. The recognition of these conditions will obviate the risks associated with unnecessary thyroxine supplementation in childhood and parental concerns of a life long illness in their offspring

Conn's syndrome: A rare cause of acute flaccid paralysis in an adolescent

Background: Acute flaccid paralysis is a medical emergency. Hypokalemia secondary to an aldosterone-secreting adrenal adenoma or Conn's syndrome is a rare cause of hypokalemic paralysis. There are very few case reports from the pediatric population of the same. Clinical Description: We report the case of a 17-year-old girl, previously asymptomatic, who presented with sudden-onset, progressive weakness of all four limbs. There was no history of altered sensorium, cranial nerve involvement, or abdominal complaints. On examination, she was found to be hypertensive. Preliminary investigations revealed severe hypokalemia and metabolic alkalosis. There was no history suggestive of gastrointestinal potassium losses. Hence, a possibility of renal losses was considered and she was found to have kaliuresis. Management and Outcome: In view of hypokalemia, with hypertension with increased renal potassium loss, a possibility of hyperaldosteronism was considered. Plasma aldosterone concentration was elevated with levels of 20.3 ng/dl (normal <15 ng/dl). The direct renin concentration was <0.5 μIU/l (normal 5–14 μIU/l). This confirmed a diagnosis of primary hyperaldosteronism. Contrast-enhanced CT of the abdomen showed an adrenal adenoma. She electively underwent a laparoscopic adrenalectomy after her motor power improved with potassium replacement. Currently, she remains normotensive, asymptomatic, and off medications. Conclusion: The case highlights that Conn's syndrome though rare is an important cause that a high index of suspicion is necessary in young hypertensive patients to make an early diagnosis of this potentially treatable condition

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

A comparative study of zoledronic acid and once weekly Alendronate in the management of acute Charcot arthropathy of foot in patients with diabetes mellitus

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 <1oF. 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

Technetium uptake predicts remission and relapse in Grave's disease patients on antithyroid drugs for at least 1 year in South Indian subjects

Context: Most of the information on remission related factors in Grave's disease are derived from Western literature. It is likely that there may be additional prognostic factors and differences in the postdrug treatment course of Grave's disease in India. Aim: To study factors which predict remission/relapse in Grave's disease patients from South India. Also to establish if technetium (Tc) uptake has a role in predicting remission. Subjects and Methods: Records of 174 patients with clinical, biochemical, and scintigraphic criteria consistent with Grave's disease, seen in our Institution between January 2006 and 2014 were analyzed. Patient factors, drug-related factors, Tc-99m uptake and other clinical factors were compared between the remission and nonremission groups. Statistical Analysis Used: Mann–Whitney U-test and Chi-square tests were used when appropriate to compare the groups. Results: Fifty-seven (32.7%) patients attained remission after at least 1 year of thionamide therapy. Of these, 11 (19.2%) patients relapsed within 1 year. Age, gender, goiter, and presence of extrathyroidal manifestations were not associated with remission. Higher values of Tc uptake were positively associated with remission (P- 0.02). Time to achievement of normal thyroid function and composite dose: Time scores were significantly associated with remission (P - 0.05 and P - 0.01, respectively). Patients with lower FT4 at presentation had a higher chance of remission (P - 0.01). The relapse rates were lower than previously reported in the literature. A higher Tc uptake was found to be significantly associated with relapse also (P - 0.009). Conclusion: The prognostic factors associated with remission in Graves's disease in this South Indian study are not the same as that reported in Western literature. Tc scintigraphy may have an additional role in identifying people who are likely to undergo remission and thus predict the outcome of Grave's disease