To understand the genetics of steroid-sensitive nephrotic syndrome (SSNS), we conducted a genome-wide association study in 987 childhood SSNS patients and 3,206 healthy controls with Japanese ancestry. Beyond known associations in the HLA-DR/DQ region, common variants in NPHS1-KIRREL2 (rs56117924, P[4.94E-20, odds ratio (OR) [1.90)

Nebulized HS therapy did not significantly reduce LOS among infants with bronchiolitis due to RSV.

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Background: The prognosis of steroid resistant nephrotic syndrome (SRNS) in children is poorer than steroid-sensitive cases. The diagnosis is made following the response to the initial 4-week corticosteroid therapy, which might be accompanied by side effects. However, predictive indicators at initial diagnosis remain unknown. We aimed to investigate whether selectivity index (SI) and other indicators at initial diagnosis, for example, serum immunoglobulin M (IgM) and total serum protein albumin ratio (TA ratio, total serum protein level over albumin level), can predict SRNS. Methods: Eighty children were enrolled from seven hospitals in Japan between January 2008 and December 2019 (mean age 4.7; male 65.0 %). Of the enrolled 80 children, 13 (16.3 %, M:F = 5:8) had been diagnosed as steroid-resistant after the initial treatment with steroids. The association between serum IgM (tertile categories; low: 24-133; middle: 134-169; and high: 169.1-510 mg/dl), SI (<0.2 or ≥0.2), and TA ratio (tertile categories; low: 1.8-2.6; middle: 2.62-3.75; and high: 3.8-15.3) at initial diagnosis and steroid resistance was evaluated with logistic regression adjusting for age and sex. Results: Low levels of serum IgM were significantly associated with steroid resistance (adjusted odds ratio (aOR) = 6.94; 95% confidence interval (CI): 1.12-43.11). TA ratio and SI were not significantly associated with steroid resistance. Conclusions: Low levels of serum IgM at initial diagnosis might predict steroid resistance among Japanese children with idiopathic nephrotic syndrome.

Determining the cause of focal segmental glomerulosclerosis (FSGS) has crucial implications for evaluating the risk of posttransplant recurrence. The degree of foot process effacement (FPE) on electron micrographs (EM) of native kidney biopsies can reportedly differentiate primary FSGS from secondary FSGS. However, no systematic evaluation of FPE in genetic FSGS has been performed. In this study, percentage of FPE and foot process width (FPW) in native kidney biopsies were analyzed in eight genetic FSGS patients and nine primary FSGS patients. All genetic FSGS patients showed segmental FPE up to 38% and FPW below 2000 nm, while all primary FSGS patients showed diffuse FPE above 88% and FPW above 3000 nm. We reviewed the literature which described the degree of FPE in genetic FSGS patients and identified 38 patients with a description of the degree of FPE. The degree of FPE in patients with mutations in the genes encoding proteins associated with slit diaphragm and cytoskeletal proteins was varied, while almost all patients with mutations in other FSGS genes showed segmental FPE. In conclusion, the present study suggests that the degree of FPE in native kidney biopsies may be useful for differentiating some genetic FSGS patients from primary FSGS patients.