The occult rheumatic scourge: A clinicopathological analysis of missed rheumatic heart disease.

Aims: To study the clinical and pathological manifestations of missed cases of rheumatic heart disease (RHD) and postulate possible reasons behind a missed diagnosis. Materials and Methods: Retrospective 20-year (2000-2019) autopsy data of chronic RHD were reviewed and patients, in whom the valvular deformities had been incidental autopsy findings, were selected. The clinical details of these patients were correlated with the morphology of the affected valves. On this pathological analysis, the patients were assigned to a category of subtle or significant valvular deformity. By clinically correlating, the latter group was subdivided into clinically misdiagnosed, clinically undiagnosed, and sudden cardiac death. Statistical Analysis: Nil. Results: Among the 475 cases of chronic RHD identified at autopsy in the study period, the disease was diagnosed incidentally in 69 patients (14.5%). Significant valvular deformity was noted in 61 cases while the other 8 cases had subtle valvular deformity. The most common cause of death was cardiac failure in 39 out of 69 patients (56%). Eleven (16%) patients had experienced sudden cardiac death. Among the undiagnosed cases, 5 (7%) of them had a diagnosis of non-rheumatic cardiac disease, while the other 14 (20.5%) patients had overwhelming non-cardiac diseases. Conclusions: Our study indicates that mortality and morbidity due to RHD are underdetermined. The patients remain undiagnosed due to either insignificant valvular involvement, clinically silent in the presence of significant valvular deformity, presence of other overwhelming diseases or misdiagnosis partly due to the resemblance with the other pathologies.

Acute rheumatic fever - A pathological analysis of clinically missed cases.

BACKGROUND: Acute rheumatic fever (ARF) and its post-inflammatory sequel chronic rheumatic heart disease (RHD) are endemic in the Indian setting. Despite the updated Jones criteria, many cases of ARF remain undiagnosed or are missed. AIMS: This study aims to analyze pathological profiles of such cases and their importance in context of clinical presentation and Jones criteria. MATERIALS AND METHODS: A 22-year retrospective observational study of ARF was conducted in the Department of Pathology in a tertiary care institute. The cases were categorized as 1. Those fulfilling and 2. those partially or not fulfilling the Jones Criteria. Based on the autopsy findings, the lesions were classified as categorized mitral stenosis and/or regurgitation (MS ± MR) and pure mitral regurgitation (MR). STATISTICAL ANALYSIS: Nil. RESULTS: In 22 years, among 697 cases of autopsied cases of RHD, there were 59 cases (8.5%) of ARF. Among them, seven cases fulfilled the Jones criteria; five of them were clinically diagnosed. The remaining 52 cases (88.1%, 34 with MS ± MR and 18 with MR) did not fulfill or partially fulfilled the Jones criteria and were not diagnosed. A total of 18 patients (30.5%) had the first attack of ARF, whereas recurrences were noted in the remaining patients (69.5%). CONCLUSIONS: The study indicates that the typical manifestations of ARF under Jones criteria may not be present, especially in cases with recurrence. Hence, a presumptive or possible diagnosis of ARF can be made with presence of minor criteria or strong clinical suspicion in such cases.

COVID-19-Related Giant Coronary Aneurysms in an Infant with Multisystem Inflammatory Disorder in Children: The First Case Report from the United Arab Emirates and the Arab Region.

BACKGROUND: Multisystem inflammatory disorder in children and adolescents is a relatively new and rare complication of COVID-19. This complication seems to develop after the infection rather than during the acute phase of COVID-19. The clinical features are similar to a well-known inflammatory syndrome in children, Kawasaki disease, and it can lead to collapse and multiple organ failure requiring intensive care. The COVID-19-associated multisystem inflammatory syndrome in children and adolescents is referred to mutually as pediatric inflammatory multisystem syndrome temporally linked with SARS-CoV-2 (PIMS-TS) or multisystem inflammatory syndrome in children (MIS-C) correlated with COVID-19, and here, it is referred to as MIS-C. Case Presentation. This report describes a nine-month-old Asian infant presented with a two-week history of fever with nonspecific signs of viral illness and erythematous rash. The clinical and biochemical findings were compatible with complicated typical Kawasaki disease (KD). The infant fulfilled the World Health Organization criteria for MIS-C and was treated with intravenous immunoglobulin and anticoagulation, which he responded well to. He was discharged home in good condition after almost 3 weeks of treatment. CONCLUSION: This case highlights a rare but new phenomenon attributed to severe acute respiratory syndrome coronavirus 2 infection. We report the first case report of MIS-C in the United Arab Emirates and Arab region. Among KD's complications, massive aneurysm with thrombosis is rare and usually will have deleterious results if not diagnosed and managed promptly.

Study of serum insulin levels in patients of euglycemic essential hypertension: a cross sectional study in North Indian population.

OBJECTIVE: Essential hypertension is associated with multiple metabolic abnormalities, among them one of the most important is hyperinsulinemia. Hyperinsulinemia has been suggested as being responsible for the increased arterial pressure in patients with essential hypertension. But this is contradicted by the finding that all patients of essential hypertension are not hyperinsulinemic. The present study was conducted to explore the hypothesis that hyperinsulinemia plays a pathogenic role in hypertension in euglycemic North Indian population. METHODS AND MATERIALS: 120 subjects were studied (60 hypertensive and 60 normotensive). Blood pressure, fasting insulin levels, lipid profile and BMI were calculated for both the groups. Statistical analysis was done using online statistical software freely at www.openepi.com. RESULTS: Hypertensive subjects were characterized by increased fasting insulin levels (16.77±7.62 vs. 8.84±2.04µIU/ml, p<0.01), increased BMI (p<0.01) and dyslipidemia, i.e. increased total cholesterol, high serum triglycerides, high LDL-C and low HDL-C with p<0.01. There was a positive correlation of fasting insulin levels with BMI, total cholesterol and LDL-C (p<0.01) and a negative correlation with HDL-C (p<0.05). However, serum insulin levels showed a non significant correlation with mean systolic and mean diastolic blood pressure (p>0.05). CONCLUSIONS: Our study showed a significant increase in serum insulin levels in hypertensive patients thereby supporting a possible pathogenic role of insulin resistance in onset of hypertension even when the fasting blood sugar is within normal limits.

An interesting case of pituitary adenoma presenting as an invasive nasopharyngeal tumor.

Pituitary masses usually present as sellar masses with suprasellar or parasellar extension. However, in unusual cases pituitary tumors especially macroprolactinomas and nonfunctional adenomas can present with intranasal extension which can be misdiagnosed as nasal polyp or a primary invasive nasopharyngeal malignant tumor. The otolaryngologists should be familiar with this rare presentation of pituitary masses. Measurement of prolactin (PRL) is essential in cases of recalcitrant nasal polyps or rhinorrhea as it may change the management in such cases. Here we describe case of a patient with an invasive pituitary adenoma who had presented in the otorhinolaryngology department with a nasal obstruction and epistaxis. We have also reviewed 30 cases of pituitary adenoma with nasopharyngeal invasion published in past.

Parathyroid carcinoma masquerading as morning sickness in pregnancy.

Incidence of primary hyperparathyroidism (PHP) in pregnancy is 8/100,000 population/year with less than 200 cases reported. Physiological changes associated with pregnancy make a diagnosis of PHP difficult and 80% are asymptomatic. High index of suspicion is required as physiological hypocalcemia related to hemodilution, increased glomerular filtration rate resulting in maternal hypercalciuria and gestational hypoalbuminemia can mask hypercalcemia of PHP. Maternal and fetal complication rates are high. Early recognition followed by appropriate management and treatment significantly reduces complications. Here, we present a rare case of parathyroid carcinoma in pregnancy and highlight the difficulties in diagnosis given the non-specific symptoms related to hypercalcemia. We have also discussed the management of PHP during the pregnancy. PHP is a preventable cause of fetal and maternal morbidity and mortality.

Primary hyperparathyroidism in India: A cocktail of contemporary and classical presentations: Lesson from 47 cases.

Primary hyperparathyroidism (PHPT) is now the contemporary form of previously existing classical disease, which is increasingly diagnosed due to hypercalcemia with asymptomatic form predominant in developed countries, whereas symptomatic form predominant in developing countries. So, we highlighted important subtle features of PHPT, problems in diagnosis in day to day practice in patients with PHPT at our center.

A case of androgen-secreting adrenal carcinoma with non-classical congenital adrenal hyperplasia.

Androgen excess is one of the most common and disturbing endocrine disorder of reproductive-aged women, affecting approximately 7% of this population Androgen excess results in the development of androgenic features in the women affected, with the development of hirsutism, androgenic alopecia, ovulatory dysfunction, and, if extreme, even virilization and masculinization. Adrenocortical carcinoma (ACC) is a rare malignancy accounting for 0.02% of all annual cancers reported. About 60% are functional tumors secreting hormones, with its consequent clinical manifestations, the Cushing's syndrome due to cortisone, virilization due to androgens, feminization due to estrogens, or hypertension due to aldosterone. Adrenal tumors that secrete androgens exclusively are extremely rare. Here, we present a rare case of androgen-secreting adrenocortical carcinoma with non-classical congenital adrenal hyperplasia.

Adrenal incidentaloma: A case of pheochromocytoma with sub-clinical Cushing's syndrome.

Adrenal incidentalomas (AIs) are a cluster of different pathologies, but AIs with dual functional aspects are very rare. We report a case of AI with the evidence of both pheochromocytoma and sub-clinical Cushing's syndrome. A 42-year-old female patient presented with the history of abdominal pain. Abdominal computed tomography revealed right adrenal mass suggestive of pheochromocytoma. On endocrine evaluation, she admitted history of intermittent headache and palpitations for 4 years and was on treatment for hypertension and diabetes. There were no signs and symptoms suggestive of Cushing's syndrome. The laboratory data demonstrated 10 times raised 24-h urinary fractionated metanephrines with non-suppressible serum cortisol after 2-day low-dose dexamethasone suppression test. She underwent right-sided adrenalectomy with subsequent resolution of both pheochromocytoma and hypercortisolism. Patient was discharged in good clinical condition.

A change for good may not be always good! An interesting case of recurrent hypoglycaemia.

Good control of plasma glucose levels always remains a desired goal for both diabetic patients and their physicians. However, unintended tight glucose control should always be alarming to the treating physician, although, it may seem to be a blessing to the patient. A downward trend in blood glucose profile in an elderly male with long standing diabetes generally points to developing renal insufficiency. Apart from that, other causes responsible for lowering of blood glucose have to be considered at times when clinical scenario is slightly different. Here, we present a case of an elderly man who presented with an unintended tight control of blood glucose over a period of few months, which upon neglecting culminated into recurrent hypoglycaemia. The etiology of recurrent hypoglycaemia in this patient was non-islet cell tumor hypoglycaemia (NICTH). The causes, pathophysiology, and management of NICTH are discussed briefly.

Adrenal incidentalomas: A collection of six interesting cases and brief review of literature.

INTRODUCTION: Adrenal incidentalomas (AI) are detected in approximately 4-7% of patients in imaging studies. Majority are benign, but careful evaluation is warranted to rule out carcinoma and functional adenomas. AIM: The purpose of presenting these cases is to highlight the approach to management of AI in terms of diagnosis, follow-up, and treatment. MATERIALS AND METHODS: Seven patients presenting in the endocrine clinic with AI were evaluated for their presenting clinical features and investigated. RESULTS: Case 1 was a 49-year-old female, with adrenal androgen secreting adrenocortical carcinoma with amenorrhoea which was mistaken as menopause. She had minimal hirsutism, which was mistaken as postmenopausal hirsutism. Case 2 was a 39-year-old male, presenting with hyperglycemia found to have Conns' syndrome with aldosterone producing adenoma on routine ultrasound. Case 3 was a 32-year-old male, presenting with gastritis and bloating, where ultrasound showed bilateral large adrenal masses revealed as diffuse large B cell lymphoma on biopsy. Case 4 was a 21-year-old boy, who had pheochromocytoma misdiagnosed as benign intracranial hypertension (HTN). Case 5 was a 59-year-old hypertensive male, presenting with fever had pheochromocytoma with catecholamine excess, producing fever. Case 6 was isolated adrenal tuberculosis who presented with chronic diarrhea. CONCLUSION: AI are common, though prevalence varies depending on the reason for scanning, the characteristics of the population studied, and the radiological techniques used. Most are non-secreting cortical adenomas. AI should be evaluated both biochemically and radiologically. When a hormonal disorder is suspected clinically, targeted, diagnostic testing for autonomous cortisol secretion, pheochromocytoma, and hyperaldosteronism is indicated.

A challenging case of an ectopic parathyroid adenoma.

UNLABELLED: The occurrence of ectopic parathyroid adenomas is not uncommon (3-4% of all parathyroid adenomas). A 42-year-old female diagnosed as having GH secreting pituitary adenoma presented with an ectopic mediastinal parathyroid adenoma located between left (Lt) pulmonary artery and Lt main bronchus. The aim of presenting this case is not to appreciate the rarity of the condition but to rather discuss some of the vital practical problems faced during its management. Patient presenting in endocrine OPD with nausea, vomiting, drowsiness and chronic constipation was investigated biochemically and with various imaging modalities and accordingly managed. Patient was also investigated from the perspective of MEN 1 syndrome. RESULTS: Baseline routine investigations revealed hypercalcemia (corrected S. Ca- 16.9 mg/dl) due to primary hyperparathyroidism (PHP, PTH-1190 ng/L) with adenoma located between Lt main bronchus and Lt pulmonary artery. Patient was medically managed and after proper preoperative preparation, surgical excision by open thoracotomy was planned but two days before surgery she developed pulmonary embolism and was shifted to ICU where she died after 20 days. An accurate preoperative localization by various imaging procedures plays a decisive role in case of ectopic adenomas in the chest. Ectopic parathyroid adenomas are frequent cause of failed initial surgery. The best surgical approach to these ectopic adenomas is still controversial. Equally effective newer medical treatment modalities are also required in patients who are awaiting or are unfit for surgery. Lastly combination of MEN 1 with ectopic parathyroid adenoma is rare.