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Background@#Routine practice of antibiotic skin test (AST) before administering antibiotics is with little scientific basis. However, AST is frequently performed in real-world practice. We aimed to explore the current practice status of AST. @*Methods@#All admission cases in Severance Hospital between January 2019 and December 2020 of patients at less than 19 years of age were collected retrospectively. AST results for 4 penicillins (ampicillin, ampicillin-sulbactam, amoxicillin-clavulanate, and piperacillin-tazobactam) and 6 cephalosporins (cefazolin, cefotaxime, cefotiam, cefpiramide, ceftriaxone, and flomoxef) as well as adverse drug reaction (ADR) reports were collected. @*Results@#Among 36,381 hospitalization cases, 7,589 and 16,468 were administered penicillins and cephalosporins, respectively. Penicillins were administered without AST in 2,622 cases (35%), 2 (0.08%) of which showed ADR. Of the remaining 4,967 cases who received AST, 57 (1.1%) showed a positive reaction. For cephalosporins, 15,473 cases (94%) received antibiotics without AST. Among them, 17 cases (0.1%) showed ADRs. For 995 cases that rceived AST, 22 (2.2%) revealed a positive reaction. Among 79 cases who were AST positive, 10 took the original medication. @*Conclusion@#The current practice of AST reveals heterogeneous and inconsistent patterns. This brings the need for a standardized guideline for the safe and effective use of antibiotics.
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Purpose@#The radiographic assessment of lung edema (RALE) score enables objective quantification of lung edema and is a valuable prognostic marker of adult acute respiratory distress syndrome (ARDS). We aimed to evaluate the validity of RALE score in children with ARDS. @*Materials and Methods@#The RALE score was measured for its reliability and correlation to other ARDS severity indices. ARDSspecific mortality was defined as death from severe pulmonary dysfunction or the need for extracorporeal membrane oxygenation therapy. The C-index of the RALE score and other ARDS severity indices were compared via survival analyses. @*Results@#Among 296 children with ARDS, 88 did not survive, and there were 70 ARDS-specific non-survivors. The RALE score showed good reliability with an intraclass correlation coefficient of 0.809 [95% confidence interval (CI), 0.760–0.848]. In univariable analysis, the RALE score had a hazard ratio (HR) of 1.19 (95% CI, 1.18–3.11), and the significance was maintained in multivariable analysis adjusting with age, ARDS etiology, and comorbidity, with an HR of 1.77 (95% CI, 1.05–2.91). The RALE score was a good predictor of ARDS-specific mortality, with a C-index of 0.607 (95% CI, 0.519–0.695). @*Conclusion@#The RALE score is a reliable measure for ARDS severity and a useful prognostic marker of mortality in children, especially for ARDS-specific mortality. This score provides information that clinicians can use to decide the proper time of aggressive therapy targeting severe lung injury and to appropriately manage the fluid balance of children with ARDS.
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The combination of central nervous system abnormalities and renal impairment is a notable characteristic of GallowayMowat syndrome (GAMOS), a disease which often accompanies microcephaly, developmental delay, and nephrotic syndrome. Many subtypes exist having various phenotypes and genotypes, and many genetic causes are still being identified.An 18-month-old boy first visited our clinic for seizure, delayed development, and microcephaly. During follow-up visits he developed proteinuria and nephrotic syndrome at the age of 6. Nephrotic syndrome became refractory to treatment. These phenotypes were suggestive of GAMOS. Next generation sequencing was performed for genetic analysis and revealed novel compound heterozygous variants in the WDR4 gene: c.494G>A (p.Arg165Gln) and c.540C>G (p.Ile180Met). This is the first case in Korea of GAMOS involving the WDR4 gene.
RESUMEN
The combination of central nervous system abnormalities and renal impairment is a notable characteristic of GallowayMowat syndrome (GAMOS), a disease which often accompanies microcephaly, developmental delay, and nephrotic syndrome. Many subtypes exist having various phenotypes and genotypes, and many genetic causes are still being identified.An 18-month-old boy first visited our clinic for seizure, delayed development, and microcephaly. During follow-up visits he developed proteinuria and nephrotic syndrome at the age of 6. Nephrotic syndrome became refractory to treatment. These phenotypes were suggestive of GAMOS. Next generation sequencing was performed for genetic analysis and revealed novel compound heterozygous variants in the WDR4 gene: c.494G>A (p.Arg165Gln) and c.540C>G (p.Ile180Met). This is the first case in Korea of GAMOS involving the WDR4 gene.