Complete remission in the nephrotic syndrome study network

Background and objectives This analysis from the Nephrotic Syndrome Study Network (NEPTUNE) assessed the phenotypic and pathology characteristics of proteinuric patients undergoing kidney biopsy and defined the frequency and factors associated with complete proteinuria remission (CRever). Design, setting, participants, & measurements We enrolled adults and children with proteinuria ≥0.5 g/d at the time of first clinically indicated renal biopsy at 21 sites in North America from April 2010 to June 2014 into a prospective cohort study. NEPTUNE central pathologists assigned participants to minimal-change disease (MCD), FSGS, membranous nephropathy, or other glomerulopathy cohorts. Outcome measures for this analysis were (1) CRever with urine protein-to-creatinine ratio (UPC)<0.3 g/g with preserved native kidney function and (2) ESRD. Continuous variables are reported as median and interquartile range (IQR; 25th, 75th percentile). Cox proportional hazards modeling was used to assess factors associated with CRever. Results We enrolled 441 patients: 116 (27%) had MCD, 142 (32%) had FSGS, 66 (15%) had membranous nephropathy, and 117 (27%) had other glomerulopathy. The baseline UPC was 4.1 g/g (IQR, 1.9, 7.7) and the eGFR was 81 ml/min per 1.73 m2 (IQR, 50, 105). Median duration of observation was 19 months (IQR, 11, 30). CRever occurred in 46% of patients, and 4.6% progressed to ESRD. Multivariate analysis demonstrated that higher prebiopsy proteinuria (hazard ratio, 0.3; 95% confidence interval, 0.2 to 0.5) and pathology diagnosis (FSGS versus MCD; hazard ratio, 0.2; 95% confidence interval, 0.1 to 0.5) were inversely associated with CRever. The effect of immunosuppressive therapy on remission varied by pathology diagnosis. Conclusions In NEPTUNE, the high frequency of other pathology in proteinuric patients affirms the value of the diagnostic kidney biopsy. Clinical factors, including level of proteinuria before biopsy, pathology diagnosis, and immunosuppression, are associated with complete remission

Нейроэндокринная гиперплазия младенцев: обзор литературы и серия наблюдений

Neuroendocrine hyperplasia of infancy (NEHI) is a rare disease of unknown etiology, which is observed in children of the first 2 years of life, characterized by persistent tachypnea syndrome and such nonspecific changes in lung biopsy as hyperplasia of bombesin-positive neuroendocrine cells of the peripheral respiratory tract. The article provides data on pathogenesis, clinical, morphological and image-diagnostics of this rare lung disease. It also presents data on observations of 26 patients with NEHI.Нейроэндокринная гиперплазия младенцев (НЭГМ) - редкое заболевание неизвестной этиологии, наблюдающееся у детей первых 2 лет жизни, характеризующееся наличием синдрома персистирующего тахипноэ и неспецифических изменений при биопсии легких в виде гиперплазии бомбезин-позитивных нейроэндокринных клеток периферических дыхательных путей. Приведены данные о патогенезе, клинической, морфологической и имидж-диагностике данного редкого заболевания легких. В статье представлены данные о наблюдениях за 26 пациентами с НЭГМ

Нейроэндокринная гиперплазия младенцев: обзор литературы и серия наблюдений

Neuroendocrine hyperplasia of infancy (NEHI) is a rare disease of unknown etiology, which is observed in children of the first 2 years of life, characterized by persistent tachypnea syndrome and such nonspecific changes in lung biopsy as hyperplasia of bombesin-positive neuroendocrine cells of the peripheral respiratory tract. The article provides data on pathogenesis, clinical, morphological and image-diagnostics of this rare lung disease. It also presents data on observations of 26 patients with NEHI.Нейроэндокринная гиперплазия младенцев (НЭГМ) - редкое заболевание неизвестной этиологии, наблюдающееся у детей первых 2 лет жизни, характеризующееся наличием синдрома персистирующего тахипноэ и неспецифических изменений при биопсии легких в виде гиперплазии бомбезин-позитивных нейроэндокринных клеток периферических дыхательных путей. Приведены данные о патогенезе, клинической, морфологической и имидж-диагностике данного редкого заболевания легких. В статье представлены данные о наблюдениях за 26 пациентами с НЭГМ