M. Bharadwaj, Aashima Dabas, M. Mantan, V. Batra, S. Yadav
{"title":"Secondary Amyloidosis with Fatal Outcome in a Child with Meningomyelocele: Need for a Closer Renal Surveillance","authors":"M. Bharadwaj, Aashima Dabas, M. Mantan, V. Batra, S. Yadav","doi":"10.4103/mamcjms.mamcjms_60_21","DOIUrl":null,"url":null,"abstract":"Amyloidosis constitutes a group of diseases characterized by extracellular deposition of fibrils composed of low molecular weight subunits of a variety of proteins. Secondary amyloidosis may result from chronic inflammatory conditions such as familial Mediterranean fever, juvenile idiopathic arthritis, tuberculosis, and bronchiectasis. Myelomeningocele is reported rarely in literature as a cause of amyloidosis. We report here a case of 6-year-old boy with operated congenital lumbar myelomeningocele with paraparesis and neurogenic bladder, who was diagnosed with nephrotic syndrome with hypertension. He underwent a renal biopsy which showed amyloidosis. During the course of treatment, he acquired a perigenital infection and later succumbed to it. A closer follow-up of patients with neurogenic bladder can help identify and manage renal complications early.","PeriodicalId":32900,"journal":{"name":"MAMC Journal of Medical Sciences","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2022-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"MAMC Journal of Medical Sciences","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/mamcjms.mamcjms_60_21","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
Abstract
Amyloidosis constitutes a group of diseases characterized by extracellular deposition of fibrils composed of low molecular weight subunits of a variety of proteins. Secondary amyloidosis may result from chronic inflammatory conditions such as familial Mediterranean fever, juvenile idiopathic arthritis, tuberculosis, and bronchiectasis. Myelomeningocele is reported rarely in literature as a cause of amyloidosis. We report here a case of 6-year-old boy with operated congenital lumbar myelomeningocele with paraparesis and neurogenic bladder, who was diagnosed with nephrotic syndrome with hypertension. He underwent a renal biopsy which showed amyloidosis. During the course of treatment, he acquired a perigenital infection and later succumbed to it. A closer follow-up of patients with neurogenic bladder can help identify and manage renal complications early.