RESUMO
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.
RESUMO
The role of the liver in drug metabolism makes individuals with hepatic dysfunction more susceptible to adverse drug reactions, necessitating careful consideration in analgesic selection and dosing. Acetaminophen, despite being a common cause of liver failure, is considered safe within recommended dosages. Nonsteroidal anti-inflammatory drugs (NSAIDs), while effective, pose risks in cirrhosis due to complications like renal failure and gastrointestinal bleeding. Cyclooxygenase-2 inhibitors have limited data, and their use is discouraged due to cardiovascular concerns. Opiates, though potent, require cautious use in cirrhosis due to altered metabolism, potential adverse effects, and the risk of addiction. Tricyclic antidepressants like nortriptyline and desipramine can be utilized for neuropathic pain, while SSRIs and SNRIs are not recommended. Anticonvulsants such as gabapentin and pregabalin are preferred for neuropathic pain, with gabapentin being the first-line choice. Topical analgesics, including NSAIDs, lidocaine, and rubefacients, are deemed safe for use in cirrhosis, offering localized relief with minimal systemic effects. Nonpharmacological approaches addressing medical, psychological, and socio-economic factors are crucial adjuvants to analgesic therapy in advanced liver diseases. Physiotherapy, psychotherapy, behavioral therapy, relaxation techniques, acupuncture, and traditional practices like yoga and massage, as well as novel modalities, contribute to a holistic pain management strategy. This review provides healthcare professionals with valuable insights into the complex landscape of analgesic therapy in cirrhosis. Meticulous consideration of drug metabolism, hepatic safety, and individual patient factors is paramount in optimizing pain management strategies for this challenging patient population.
RESUMO
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.
RESUMO
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.