Schmidt's Syndrome: An Uncommon Cause of Spontaneous Hypoglycemia.

Schmidt's syndrome, or autoimmune polyendocrine syndrome type 2 (APS-2), is an uncommon disorder characterized by the co-occurrence of autoimmune thyroiditis and adrenalitis. APS-2 is defined as a combination of Addison's disease, autoimmune thyroid disease, and/or type 1 diabetes mellitus. It is an autosomal dominantly inherited polygenic disorder with incomplete penetrance; the candidate genes include but are not limited to HLA-DR3, HLA-DR4, CTLA-4, PTPN22, and CD25-IL-2. Autoimmune thyroiditis, often Hashimoto's disease, results in hypothyroidism. Primary adrenal failure results in enhanced secretion of adrenocorticotrophic hormone melanocyte and co-secretion of melanocyte-stimulating hormone, contributing to hyperpigmentation. Mineralocorticoid deficiency results in salt wasting, fatigue and cramps, postural hypotension, and hyperkalemia. Cortisol, an insulin counter-regulatory hormone, plays a pivotal role in maintaining euglycemia; deficiency predisposes to the development of hypoglycemia. We here report a rare presentation of Schmidt's syndrome as hypoinsulinemic hypoglycemia in a middle-aged male patient. Management includes treatment of acute hypoglycemic episodes with glucose or glucagon, long-term glucocorticoids and mineralocorticoids for adrenal insufficiency, and thyroid hormone supplements for hypothyroidism. This case report and brief overview aim to contribute to the scientific understanding of Schmidt's syndrome/APS-2. Additionally, here we briefly outline the diagnostic challenges in hypoglycemia evaluation, including the utilization of Whipple's triad and the gold standard supervised 72-hour fast and evaluation for primary adrenal and thyroid insufficiencies.

Metabolic Dysfunction-Associated Steatohepatitis and Progression to Hepatocellular Carcinoma: A Literature Review.

The prevalence of metabolic-associated fatty liver disease (MAFLD) is increasing globally due to factors such as urbanization, obesity, poor nutrition, sedentary lifestyles, healthcare accessibility, diagnostic advancements, and genetic influences. Research on MAFLD and HCC risk factors, pathogenesis, and biomarkers has been conducted through a narrative review of relevant studies, with a focus on PubMed and Web of Science databases and exclusion criteria based on article availability and language. Steatosis marks the early stage of MASH advancement, commonly associated with factors of metabolic syndrome such as obesity and type 2 diabetes. Various mechanisms, including heightened lipolysis, hepatic lipogenesis, and consumption of high-calorie diets, contribute to the accumulation of lipids in the liver. Insulin resistance is pivotal in the development of steatosis, as it leads to the release of free fatty acids from adipose tissue. Natural compounds hold promise in regulating lipid metabolism and inflammation to combat these conditions. Liver fibrosis serves as a significant predictor of MASH progression and HCC development, underscoring the need to target fibrosis in treatment approaches. Risk factors for MASH-associated HCC encompass advanced liver fibrosis, older age, male gender, metabolic syndrome, genetic predispositions, and dietary habits, emphasizing the requirement for efficient surveillance and diagnostic measures. Considering these factors, it is important for further studies to determine the biochemical impact of these risk factors in order to establish targeted therapies that can prevent the development of HCC or reduce progression of MASH, indirectly decreasing the risk of HCC.

Cirrhosis of Liver and Diabetes Mellitus: The Diabolic Duo?

INTRODUCTION: Cirrhosis of the liver and diabetes mellitus are two chronic illnesses with significant impact on the quality of life. Studies from different part of the world have shown the combination to be associated with higher incidence of complications of cirrhosis and reduced survival. However, data on the impact of pre-cirrhotic and post-cirrhotic diabetes on cirrhosis is minimal. AIM: The aim of the study was to determine the complications of cirrhosis patients with and without co-existent DM and to compare the relation between cirrhosis patients with antecedent DM and hepatogenous DM. MATERIALS AND METHODS: The present prospective study was conducted at a tertiary care hospital in Kerala, India, over a period of three years. Cirrhosis patients with and without diabetes, along with subcategorization as antecedent and hepatogenous diabetes, were studied for various complications and outcome including death. Chi-square and Mann-whitney tests were used for comparing data. RESULTS: Patients with cirrhosis and diabetes had higher incidence of gall stones (27.6% versus 13.2%; p=0.008) and urinary infection (29.3% versus 7.5%; p=<0.001). Incidence of hepatocellular carcinoma and mortality were similar between the groups. Patients with antecedent diabetes and hepatogenous diabetes were similar with respect to complications and mortality. Child-Turcotte-Pugh (CTP) score, Model for End stage Liver Disease (MELD) score, urinary tract and respiratory infections and duration of cirrhosis were independent predictors of mortality in patients with cirrhosis. CONCLUSION: Coexistent diabetes mellitus increases the incidence of complications and hospitalizations in cirrhosis patients but without impact on mortality rates. There is no significant morbidity or mortality difference between cirrhotics with antecedent diabetes and hepatogenous diabetes.

Acute esophageal necrosis: an uncommon cause of hematemesis.

Acute esophageal necrosis or black esophagus is an uncommon clinical entity, diagnosed at the upper gastrointestinal endoscopy with the presence of strikingly black necrotic esophagus. Very often no definite etiology will be identified even though a large list of potential associations has been postulated. Upper gastrointestinal bleeding is the most common clinical presentation, others being epigastric pain, retrosternal chest discomfort and dysphagia. Only about a hundred cases of acute esophageal necrosis have been described in medical literature till this date. We report a case of acute esophageal necrosis in an elderly female who had presented with hematemesis.

Muscle hematomas: uncommon but horrendous complication of cirrhosis liver.

Bleeding manifestations, usually cutaneous or gastrointestinal - are common in liver cirrhosis. Spontaneous intracerebral and intramuscular bleeds are reported uncommonly, often associated with gross hepatic dysfunction and severely deranged hemostatic parameters, and are often markers to imminent mortality. Male sex, alcoholic etiology, significant thrombocytopenia, severe coagulopathy, and advanced liver disease are common denominators in patients with spontaneous intramuscular bleed. We here report three cases of spontaneous muscle hematomas complicating hepatic cirrhosis. To the best of our knowledge, this is the largest single center series reporting spontaneous muscle hematomas complicating cirrhosis from the Indian subcontinent.

Sweet's syndrome in human immune deficiency virus-infected patient.

Sweet's syndrome is an uncommon dermatosis and can be associated with a wide variety of illnesses including infections and malignancies. Sweet's syndrome as a dermatological manifestation in human immunedeficiency virus (HIV) infection is rarely reported. Furthermore, called acute febrile neutrophilic dermatosis is characterized by fever and skin lesions, which are often erythematous papules and pseudovesicles. Diagnosis is based on clinical features and histology. The gold standard for treatment is systemic steroids although many other medications have been tried with variable success. We here report a case of Sweet's syndrome in an HIV-infected patient.

Pseudo-Ludwig's Phenomenon: A Rare Clinical Manifestation in Liver Cirrhosis.

Pseudo-Ludwig's phenomenon, an exceedingly rare clinical entity, is characterized by non-infectious upper airway obstruction resulting from spontaneous hematoma in the sublingual space. The condition is often described in patients on anticoagulants with grossly deranged coagulation profile. We describe a case of pseudo-Ludwig's phenomenon in a cirrhotic patient with significantly deranged hemostasis profile; the first to be reported in medical literature.

Spontaneous bacterial peritonitis in extrahepatic portal venous obstruction.

Spontaneous bacterial peritonitis is defined by a positive ascitic fluid bacterial culture and an elevated ascitic fluid absolute polymorphonuclear count (≥250 cells/mm3) without an evident intra-abdominal, surgically treatable source of infection. Transient ascites is well documented in patients with extrahepatic portal venous obstruction but spontaneous bacterial peritonitis complicating extrahepatic portal venous obstruction is extremely uncommon. The postulated reasons for the low incidence of spontaneous bacterial peritonitis in extrahepatic portal venous obstruction includes: lower incidence of ascites; intact hepatic reticuloendothelial system; and a relatively high ascitic fluid protein content. Here we report two cases of spontaneous bacterial peritonitis complicating extrahepatic portal venous obstruction.