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Additionally, [Ca2+]i levels in HEK293 cells with CaSR-WT and CaSR-Y825F were determined at 340 nm/380 nm wavelength ratios using Fura-2 AM. At [Ca2+]e concentrations of 2.5 mM and 3 mM, the ratios of CaSR-Y825F cells were higher (2.6 and 3.5, respectively) than those of CaSR-WT cells, which were 1.04 and 1.40, respectively. This cell function study proved that the CaSR-Y825F, which is in HEK293 cells transformed with a activating CaSR variant, elevated MAP kinase signaling(pERK, pJNK, pp38) and increased [Ca2 +] i to induce hypocalcemia. This cell function study proved that the CaSR-Y825F, which is in HEK293 cells transformed with a activating CaSR variant, elevated MAP kinase signaling(pERK, pJNK, pp38) and increased [Ca2 +] i to induce hypocalcemia.Cushing syndrome (CS) is rare in children. The clinical presentation of CS varies according to extent and duration of glucocorticoid excess, and urolithiasis is a common complication. We report the first case of a patient with CS associated with acute kidney injury (AKI) due to urolithiasis. A 6-year-old boy presented to the Emergency Department with seizure. On physical examination, he had clinical features of CS and high blood pressure. Brain computed tomography (CT) suggested posterior reversible encephalopathy syndrome due to hypertension. On evaluation of hypertension, laboratory tests suggested adrenocortical tumor, but abdominal CT suggested pheochromocytoma. During further evaluation, his condition deteriorated with AKI due to bilateral ureteral stones, for which the patient underwent continuous renal replacement therapy in the intensive care unit. After controlling hypercortisolism with etomidate and performing ureteral stent indwelling, an adrenal mass was resected and histologically confirmed as an adrenocortical adenoma. We review the clinical manifestations, diagnosis, and management of CS associated with urolithiasis and AKI. Early recognition and careful monitoring of urolithiasis in CS patients are important to avoid severe complications of urolithiasis.Type 1 diabetes mellitus (T1DM) requires life-long insulin therapy because of diminished insulin-secretion capability. Glycemic control and glucose monitoring are important to prevent T1DM complications. Continuous glucose monitoring (CGM) measures glucose level, every one to five minutes, in the interstitial fluid from a subcutaneous sensor and facilitates better glycemic control, reduces hypoglycemia, and is safely used in the pediatric population. CGM can be categorized as retrospective, real-time, or intermittently scanned CGM, and all forms are available in Korea. The CGM device has 3 components sensor, transmitter, and monitor/receiver. Key metrics of CGM include days of CGM application, percentage of time with CGM, mean glucose, glucose management indicator, glycemic variability, and use of Ambulatory Glucose Profile for CGM reports. CGM sensors and transmitters have been partly reimbursed by the Korean National Health Insurance Service (NHIS) since 2019, and 1,434 T1DM patients (male, 40.8%; age less then 20 years, 52.4%) in Korea were prescribed CGM as of December 2019. In Korea, the number of CGM users will increase due to reimbursement for CGM sensors and transmitters by the NHIS. Successful CGM use requires long-term policies to establish diabetes education and financial assistance. Clinicians should become well-acquainted with interpretation of CGM data and information updates to facilitate integration of CGM data into clinical practice among pediatric T1DM patients.Congenital hyperinsulinism (CHI) is a rare glucose metabolism disorder characterized by unregulated secretion of insulin leading to hyperinsulinemic hypoglycemia (HH). Most cases are caused by mutations in the KATP-channel genes ABCC8 and KCNJ11. We report two patients with severe HH from the first day of life. Patient 1 developed midgut volvulus after initiating diazoxide and required intestinal resection. He was subsequently managed with high-dose octreotide and glucose enriched diet. Consistent with diffuse type CHI by 18F-DOPA-PET/CT, genetic testing revealed a homozygous ABCC8 variant, c.1801G>A, p.(Val601Ile). The rare variant was previously reported to be diazoxide-responsive and the patient responded well to diazoxide monotherapy with clinical remission at two years of age. Patient 2 responded to diazoxide with spontaneous clinical remission at 15 months of age. However, an oral glucose tolerance test at seven years of age revealed hyperinsulinism. Genetic testing revealed the proband and several seemingly healthy family members harbored a novel, heterozygous ABCC8 variant, c.1780T>C, p.(Ser594Pro). Genetic findings identified previously unrecognized HH in the proband.We report the outputs of a satellite event in Ho Chi Minh City, Vietnam, organized as part of the "2 nd Global Grand Challenges of Engineering Summit". The event considered challenges and potential solutions for improving low- and middle-income country (LMIC) healthcare systems, with particular reference to critical care. Participants from key regional and local stakeholders in healthcare and engineering discussed how new advances in technology, especially in the field of Artificial Intelligence, could be of potential benefit. This article summarizes the perspectives and conclusions of a group of key stakeholders from LMICs across South and South East Asia.Background Monoclonal antibodies are a relatively new therapeutic option for patients with severe refractory asthma, which can be used as an add-on to maintenance therapy, reducing the need for systemic corticosteroid usage, improving asthma symptom control and reducing exacerbations. We report a case of a patient with severe refractory eosinophilic asthma, reluctant to take systemic steroids, who was successfully treated with benralizumab alone during an acute asthma attack. Case presentation A 59-year-old Caucasian woman with a history of allergic asthma since childhood showed a progressive decline in lung function with difficult to control symptoms and an increased number of hospitalizations despite maximal maintenance treatment, and was diagnosed with severe refractory asthma. https://www.selleckchem.com/products/bmh-21.html She was reluctant to take systemic corticosteroids during exacerbations due to severe urinary retention; therefore, she started omalizumab with a partial reduction of symptoms and exacerbations over time. During a follow-up visit, she showed signs of acute exacerbation and she was switched to benralizumab during her acute phase with a rapid, dramatic amelioration of respiratory symptoms and pulmonary function, without concomitant systemic corticosteroid administration.
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