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.

Development and validation of a gel wax phantom to evaluate geometric accuracy and measurement of a hyperechoic target diameter in diagnostic ultrasound imaging.

Diagnostic ultrasound (US) scanners are generally evaluated using proprietary quality assurance (QA) phantoms, but their prohibitively high cost may prevent organizations to perform the necessary tests. This study aimed to develop a low-cost gel wax phantom with targets to determine the lateral and axial resolution and diameter of a hyperechoic target in an US scanner. The acoustic property (AP) of gel wax, which includes the speed of sound (cus), acoustic impedance (Z), and attenuation coefficient (µ), were determined for multiple transducers operating at 2.25, 5, 10, 15, and 30 MHz. These results were compared to the AP of soft tissue. Two polytetrafluoroethylene (PTFE) rectangular frames with holes separated by 5, 10, and 20 mm were constructed. Nylon filaments and stainless-steel disc (SS disc) (diameter = 16.8 mm) were threaded through the frames and suitably placed in gel wax to obtain orthogonal targets in the phantom. The target dimensions obtained from computerized tomography (CT) and US images of the phantom were compared for phantom validation. The average cus=1431.4 m/s, mass density ρ = 0.87 g/cm3, Z = 1.24 MRayls, and µ ranged from 0.7 to 0.98 dB/cm/MHz for gel wax at 22 °C. The US image measurement exhibited a maximum error in determining the diameter of the SS disc, resulting in a value of 18 mm instead of its actual value of 16.8 mm. The phantom volume decreased by 1.8% in 62 weeks. The present phantom is affordable, stable, customizable, and can be used to evaluate diagnostic US scanners across multiple centers.

Acetaminophen: A Liver Killer or Thriller.

Acetaminophen, or paracetamol, ranks among the most extensively utilized analgesic and antipyretic medications globally. The administration of acetaminophen to individuals with underlying liver disease has long sparked concerns regarding the potential risk of hepatotoxicity. However, the available literature and recommendations consider it a safe option in all forms of liver diseases and is deemed safe when used at recommended doses. This article aims to offer a concise review of the pharmacokinetics, toxicity profile, and the intricate considerations surrounding the safety of acetaminophen in patients with liver disease. By delving into the liver-acetaminophen interactions, we seek to provide a nuanced perspective on the use of acetaminophen in this critical patient population.

Medicaid Expansion and Cancer Mortality by Race and Sex in Louisiana.

INTRODUCTION: The purpose of this study is to determine the association between Medicaid expansion in Louisiana and cancer mortality by race and sex. METHODS: Data from the National Vital Statistics System mortality files were used to quantify deaths from cancer between 2010 and 2019 for Louisiana and a sample of states that had yet to adopt the Affordable Care Act's Medicaid expansion as of December 2019. A series of population-weighted comparative interrupted time series models were estimated to determine whether Louisiana's Medicaid expansion was associated with reduced cancer mortality. Analyses were conducted in May 2021-August 2021. RESULTS: Medicaid expansion was associated with an average of 3.3 (95% CI= -6.4, -0.1; p=0.045) fewer quarterly cancer deaths per 100,000 Black female Louisiana residents and an average of 5.8 (95% CI= -10.4, -1.1; p=0.015) fewer quarterly cancer deaths per 100,000 Black male residents. There were no statistically significant changes in cancer mortality for White people in Louisiana associated with Medicaid expansion. Following expansion, the Black-White mortality gap in cancer deaths declined by approximately 57% for female individuals (4.6-2.0) and 49% for male individuals (10.1-5.2). CONCLUSIONS: Medicaid expansion in Louisiana was associated with a reduction in cancer mortality for Black female and male adults. Estimates of the association between Medicaid expansion and cancer mortality in Louisiana directly relate to the potential impacts for states that have yet to adopt Medicaid expansion under the Affordable Care Act, which are primarily located in the Southern U.S.

Design, Fabrication, and Validation of a Polymethyl Methacrylate Head Phantom for Dosimetric Verification of Cranial Radiotherapy Treatment Plans.

PURPOSE: The present study aims to design and fabricate a novel, versatile, and cost-effective Polymethyl Methacrylate (PMMA) head phantom for the dosimetric pretreatment verification of radiotherapy (RT) treatment plans. MATERIALS AND METHODS: The head phantom designing involves slice-wise modeling of an adult head using PMMA. The phantom has provisions to hold detectors such as ionization chambers of different sizes, Gafchromic films, gel dosimeter, and optically stimulated luminescence dosimeter. For the point dose verification purpose, 15 volumetric modulated arc therapy patient plans were selected, and doses were measured using a CC13 ionization chamber. The percentage gamma passing rate was calculated for acceptance criteria 3%/3 mm and 2%/2 mm using OmniPro I'mRT film QA software, and Gafchromic EBT3 films were used for 2D planar dose verification. RESULTS: Treatment planning system calculated, and the measured point doses showed a percentage deviation ranged from 0.26 to 1.92. The planar dose fluence measurements, for set acceptance criteria of 3%/3 mm and 2%/2 mm, percentages of points having gamma value <1 were in the range of 99.17 ± 0.25 to 99.88 ± 0.15 and 93.16 ± 0.38 to 98.89 ± 0.23, respectively. Measured dose verification indices were within the acceptable limit. CONCLUSIONS: The dosimetric study reveals that head phantom can be used for routine pretreatment verification for the cranial RT, especially for stereotactic radiosurgery/RT as a part of patient-specific quality assurance. The presently fabricated and validated phantom is novel, versatile, and cost-effective, and many institutes can afford it.

Estimation of dosimetric discrepancy due to use of Onyx™ embolic system in Stereotactic Radiosurgery/Radiotherapy (SRS/SRT) planning.

More often the embolic materials in the brain create artefacts in the planning CT images that could lead to a dose variation in planned and delivered dose. The aim of the study was to evaluate the dosimetric effect of artefacts generated by the Onyx™ embolization material during Stereotactic Radiosurgery/Radiotherapy (SRS/SRT) planning. An in-house made novel Polymethyl Methacrylate (PMMA) head phantom (specially designed for SRS/SRT plans) was used for this purpose. For the evaluation process, we have created concentric ring structures around the central Onyx materials on both the CT sets (with and without Onyx material). The verification plans were generated using different algorithms namely Analytical Anisotropic Algorithm (AAA), Acuros XB and Monaco based Monte Carlo on both CT sets. Mean integral dose over the region of interest were calculated in both CT sets. The dosimetric results shows, due to the presence of Onyx material, relative variation in mean integral dose to the proximal structure (Ring 1) were -4.02%, -2.98%, and -2.49% for Monte Carlo, Acuros XB, and AAA respectively. Observed variations are attributed to the presence of artefacts due to Onyx material. Artefacts influence the accuracy of dose calculation during the planning. All the calculation algorithms are not equally capable to account such variations. Special cares are to be taken while choosing the calculation algorithms as it impacts the results of treatment outcome.

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.

Isolated pancreatic tuberculosis masquerading as pancreatic cancer.

Isolated pancreatic tuberculosis (TB) remains a rarity despite the high incidence of tuberculosis in many of the African and Asian countries. Presentation as discrete pancreatic mass often masquerades as pancreatic neoplasm and diagnosis may require histology. Extra-hepatic portal hypertension due to splenic vein thrombosis complicating pancreatic TB has been reported in the literature. We report here a case of isolated pancreatic TB with pancreatic head mass mimicking neoplasm with extra-hepatic portal hypertension. The possibility of TB should be considered in the list of differential diagnoses of pancreatic mass and an endoscopic, ultrasound-guided biopsy might help to clinch the diagnosis of this potentially curable disease.

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.

Hemoglobin SO-Arab and α-thalassemia diagnosed in an adult: A case-based review of the hemoglobinopathies.

Hemoglobin SO-Arab is a rare sickling disorder with a clinical course similar to that of hemoglobin SS. Hemoglobin C-Harlem is another rare condition that produces sickling disorders in affected individuals with a disease course and electrophoretic findings similar to that of hemoglobin SO-Arab. The authors report the case of a 38-year-old African American man with hemoglobin SO-Arab and the challenges that may arise in working up a rare hemoglobinopathy.

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.