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Sir, Familial hypocalciuric hypercalcemia (FHH) is a rare genetic disorder with autosomal dominant inheritance and high penetrance that manifests with mild parathyroid hormone (PTH)-dependent hypercalcemia and hypocalciuria.[[1]],[[2]] The disorder results from an inherited defect in calcium sensing mechanism at the level of parathyroid gland and the thick ascending limb of loop of Henle of kidney.[[3]] FHH is genetically heterogenous and three subtypes have been described, namely, FHH1, FHH2, and FHH3, related to inactivating mutations in the genes encoding calcium sensing receptor (CaSR), G-protein subunit alpha-11 (GNA11), and adaptor-related protein complex 2 sigma 1 subunit (AP2S1) proteins, respectively.[[3]] Most patients with FHH are asymptomatic.[[4]] The disorder runs a benign course in most patients; parathyroidectomy is neither required, nor it is curative.[[5]] Symptoms of neonatal hypocalcemia have been described in unaffected children of women affected with this rare disorder.[[6]] Here, we describe an Indian kindred with genetically confirmed type 1 FHH where the presentation in the index case was delayed till the age of 48 years, and confounded by secondary hyperparathyroidism related to vitamin D deficiency. Given the biochemical picture of mild PTH-dependent hypercalcemia, hypocalciuria, and absence of any end-organ manifestations of PTH excess, we considered a possibility of FHH and requested for family biochemical screening. A further work-up of hypercalcemia was not done and the patient was administered calcium (1 gram per day elemental calcium) and injectable (cholecalciferol 600,000 IU intramuscular once a week for 2 weeks), followed by oral vitamin D (cholecalciferol 60,000 IU sachet once a week for 8 weeks) preparations. [Extracted from the article]

Background: Pituitary apoplexy results from hemorrhage, infarction, or hemorrhagic infarction within a pituitary tumor. Subclinical or clinical apoplexy is not uncommon in acromegaly, owing to the large size of the tumor at initial detection. Growth hormone excess in acromegaly often persists following surgery. However, in rare instances, pituitary apoplexy may present a spontaneous cure to growth hormone excess. Case Report: A 40-year-old male presented with holocranial headache for the past 16 years that had worsened in severity during the prior year. Two months before presentation, he experienced a severe headache that he described as the worst headache of his life. The patient had prominent acromegaloid features that he ignored, as they seemed to cause no harm. The patient had no signs of clinically active disease. Magnetic resonance imaging of the brain revealed a pituitary macroadenoma with evidence of hemorrhage. Serum insulin-like growth factor 1 and oral glucose-suppressed serum growth hormone levels were normal, suggestive of inactive or silent disease. Pituitary apoplexy causing spontaneous remission of acromegaly was diagnosed, and close follow-up was planned for the evolution of hypopituitarism. Conclusion: This case highlights a rare presentation of acromegaly in which an episode of symptomatic pituitary apoplexy revealed the diagnosis of pituitary adenoma and led to the cure of growth hormone hypersecretion. [ABSTRACT FROM AUTHOR]

Background: Despite being the most common cause of secondary hypertension, prevalence of primary aldosteronism (PA) among patients with young‐onset hypertension (YH ‐ age of hypertension onset <40 years) remains poorly studied. Objective: We assessed the prevalence of PA in patients with YH referred for evaluation of secondary hypertension. Design and Patients: In this prospective, cross‐sectional study, 202 patients with YH, visiting endocrine and cardiology clinics of All India Institute of Medical Sciences, India, were evaluated. Measurements: Primary aldosteronism was screened by measuring plasma aldosterone concentration (PAC) and direct renin concentration (DRC) and calculating aldosterone‐to‐renin ratio (ARR), followed by confirmatory saline infusion test (SIT) according to Endocrine Society Guideline. Those confirmed with post‐SIT PAC >5 ng/dl underwent adrenal computed tomography (CT), followed by adrenal venous sampling (AVS). Results: Of 202 YH patients, 38 (18.8%) screened positive, and PA was confirmed in 36 (17.8%). The mean age was 43.9 ± 10.9 years, and median duration of hypertension was 10.5 (3.5–18) years. The prevalence of PA increased with grade of hypertension (8.1% in grade 1 to 37.1% in grade 3), number of antihypertensive medications (2.5% in those taking ≤1 to 50% in those taking ≥4 medications) and severity of hypokalaemia (0% in potassium >5 to 85.7% in potassium <3.5 mmol/L). The prevalence of PA by age of hypertension onset was highest in age group 30–39 years (31.3%). Conclusions: There is a high prevalence and a long delay in diagnosis of PA among patients with YH, and YH should be considered as a separate high‐risk category in PA screening algorithm. [ABSTRACT FROM AUTHOR]

Motherhood is tough and life-changing for every woman and even more so for females doing residency training. Pregnancy in itself was challenging and took a physical and mental toll on the body. Long and demanding work-hours with unrealistic expectations from oneself added to severe stress. If pregnancy part was difficult, then juggling the responsibilities of a child as well maintaining work-life balance was like walking the tight-rope with insecurities and self-doubts creeping in at several occasions. It required a great deal of motivation to carry on. It was the support of my 'village,' which made me finally achieve things that seemed impossible in the beginning. [ABSTRACT FROM AUTHOR]

Objective: Endogenous Cushing's syndrome (CS) is a known cause of secondary osteoporosis. Vertebral fractures (VFs) in endogenous CS may occur despite normal bone mineral density (BMD). Trabecular bone score (TBS) is a relatively new, non‐invasive technique to assess bone microarchitecture. The objective of our study was to analyse the BMD and bone microarchitecture using TBS in endogenous CS and compare it with a group of age and sex‐matched healthy controls, and also analyse the factors predicting BMD and TBS. Design: Cross‐sectional study of cases and controls. Patients and measurements: We included 40 female patients with overt endogenous CS, out of which 32 were adrenocorticotropic hormone (ACTH)‐dependent CS and 8 were ACTH‐independent. We also included 40 healthy, female controls. Both patients and controls were subjected to an assessment of biochemical parameters and BMD and TBS. Results: Patients with endogenous CS had significantly lower BMD at the lumbar spine, femoral neck, and total hip and significantly lower TBS than healthy controls (all p <.001), while no significant difference was noted in the distal radius BMD (p =.055). In endogenous CS, a large proportion of patients, n = 13 (32.5%) had normal BMD for age (BMD Z‐score ≥ −2.0) with low TBS (L1‐L4 TBS ≤ 1.34). TBS correlated negatively with HbA1c (p =.006), and positively with serum T4 (p =.027). Conclusion: TBS should be considered an important complementary tool in addition to BMD for the routine assessment of skeletal health in CS. [ABSTRACT FROM AUTHOR]

Introduction: Addison disease refers to primary hypoadrenalism, most commonly caused by autoimmineadrenalitis and infections of the adrenal glands. It is a rare condition associated with high morbidity and mortality rates. However it is easily treatable once a correct diagnosis is made. Usual symptoms are weakness, tiredness, nausea, vomiting, postural dizziness and anorexia. Sometimes Addison disease may present with unusual symptoms so that making a correct diagnosis may require a high index of suspicion. Materials and Methods: We describe a case of a 19 year old male who presented to the emergency with complaints of abnormal behaviour in the form of inability to recognize family members and 2-3 episodes of seizure for past 3 days. Results: He was found to have hypotension, hyponatremia and hypoglycemia and was diagnosed as a case of Addison Disease. CT scan of abdomen showed bilateral adrenal calcification. Conclusion: Physicians must be aware of the varied presentation of Addison disease and basic investigations may give important clue to the correct diagnosis. Addison disease must be kept in the differential diagnosis of such cases. [ABSTRACT FROM AUTHOR]

Background: Acute febrile encephalopathy (AFE) is a clinical term used to an altered mental state that either accompanies or follows a short febrile illness and is characterized by a diffuse and nonspecific brain insult manifested by a combination of coma, seizures, and decerebration. Objective: To identify the clinical and etiological profile of acute febrile encephalopathy in adult patients presenting to JNMCH, Aligarh from January-December, 2014. Patients and Methods: 238 adults (>14 yrs.) presenting to the emergency with complains of fever (>38°C) of less than 2 weeks duration with altered sensorium with/or without seizure were prospectively investigated for etiological cause. The investigations included routine blood investigations, chest X-rays, cerebrospinal fl uid (CSF) microscopy, bacterial and fungal cultures, CSF serology, peripheral smear and serology for malarial parasite. Other investigations included were CT or MRI wherever indicated. Non-infectious causes were ruled out by various investigations mentioned above. Outcome was evaluated by the report of investigations and response to treatment over during the period of inpatient treatment. Results: Out of all the patients admitted, acute pyogenic meningitis was the most common cause accounting for 34.5% of cases followed by cerebral malaria 20.2%, sepsis associated encephalopathy 14.3%, acute meningoencephalitis 9.2%, tubercular meningitis 6.7%, enteric encephalopathy 2.5% and fungal meningitis in 1.3% cases. Conclusion: Acute pyogenic meningitis is the most common cause of AFE in the patients who reported to JNMCH in the past one year. Prompt and specific treatment can mimimise morbidity and mortality in many cases presenting with AFE. [ABSTRACT FROM AUTHOR]

It is now well established that non enzymatic glycation leads to formation of Amadori products which over course of time leads to formation of advanced glycation end products (AGEs). Accumulation of AGEs is very toxic and can have direct and indirect effects in the pathogenesis of various diseases including diabetes, alzheimer's disease, rheumatoid arthritis etc. Hence, to curb these harmful effects, a number of natural and synthetic compounds are being investigated so as to reduce clinically the impact of AGEs. Here, various AGE inhibitors and breakers published till date are being reviewed. Also, potential novel therapies are also being explored. [ABSTRACT FROM AUTHOR]

With the onset of the COVID-19 pandemic, researchers and policymakers began to pay attention to the expected number of out-of-school children and emerging learning crises in developed countries, where self-isolation, social distancing, and access to education through technology are possible on a wide scale. Within the region of South Asia, and among the impoverished communities of Bangladesh, India and Pakistan, this situation is quite the opposite, where millions of people still live below the poverty line, and technology-driven education is not yet a privilege. Despite the utmost commitment to provide free and quality education to all, the three nation-states prioritised healthcare over other businesses, delaying the policy response to the educational and learning crises. In this paper, the authors examine the policy responses from each country and conclude that inequities are not being adequately addressed, and that remote learning initiatives are not producing comparable results to prepandemic learning. The authors suggest that to make education accessible, equitable, and to improve student learning outcomes, the countries need to invest in school leader capacity building, and strengthening of the technology infrastructure and resources [ABSTRACT FROM AUTHOR]

Parathyroid carcinoma is a rare tumor associated with significantly high levels of parathyroid hormone (PTH) and primary hyperparathyroidism (HPT) and is difficult to distinguish from parathyroid adenoma, which is a much more common cause of HPT. Parathyromatosis is a rare condition in which several nodules of hyperfunctioning parathyroid tissue form in the neck and mediastinum, and it presents as recurrent hyperparathyroidism. We describe the case of a 44-year-old woman with recurrent hyperparathyroidism who presented with hypercalcemic crisis and biochemical investigations showed a severely raised serum intact parathyroid hormone (iPTH) level of 2513 pg/ml and serum calcium level of 17 mg/dl. A four-dimensional computed tomography (4D CT) of the neck was done, which showed few arterially enhancing nodular lesions in the right paratracheal and retrotracheal location at the level of thoracic inlet showing washout in delayed images suggestive of recurrent/residual parathyroid lesions. A technetium-99 m methoxyisobutylisonitrile (Tc-99 m MIBI) scan was done, and the planar neck anterior early images showed an area of increased radiotracer uptake in the right lower neck region with persistent uptake in the delayed images. Single photon emission computed tomography/ computed tomography (SPECT/CT) images showed a soft-tissue lesion in the right paratracheal region at the level of C7 vertebra showing increased radiotracer uptake suggestive of a recurrent/residual parathyroid lesion. Exploration and excision of the recurrent parathyroid mass was planned, and the right paratracheal mass with nodes above the aortic arch along with the subcutaneous and intramuscular lesions were excised. Postoperative serum calcium was 7.8 mg/dl, and serum iPTH was 135 pg/ml. Histopathological examination of the excised tissue showed the features of parathyroid carcinoma. Thus, Tc-99 m MIBI scan and 4D CT were critical in evaluating this case of parathyroid carcinoma with recurrent hyperparathyroidism and planning its management. [ABSTRACT FROM AUTHOR]