{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T19:20:59Z","timestamp":1787772059144,"version":"build-2784847793"},"reference-count":12,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023]]},"abstract":"<jats:p>\n                    Congenital nephrotic syndrome (NS) is characterized by early-onset heavy proteinuria. Most cases of congenital NS are associated with genetic mutations in the podocyte proteins. The causal relationship of perinatal infections with congenital NS has not yet been proven. Inadequate response to the treatment of such infections should prompt us to conduct genetic testing for congenital NS. The heavy proteinuria associated with congenital NS is usually difficult to control with conventional treatment. It often results in progressive kidney disease with a high risk of mortality in early life. Here, we describe an infant who developed congenital NS and was found to have a coexisting\n                    <jats:italic toggle=\"yes\">Cytomegalovirus<\/jats:italic>\n                    infection and an underlying\n                    <jats:italic toggle=\"yes\">NPSH1<\/jats:italic>\n                    mutation. Proteinuria did not respond to a standard dose of enalapril. A supramaximal dose of enalapril was tried and was effective and safe in controlling the proteinuria. It was associated with improved growth, complete resolution of edema, normal serum albumin, and normal renal function beyond 2 years of age.\n                  <\/jats:p>","DOI":"10.4103\/1319-2442.397210","type":"journal-article","created":{"date-parts":[[2024,3,9]],"date-time":"2024-03-09T00:55:51Z","timestamp":1709945751000},"page":"462-467","source":"Crossref","is-referenced-by-count":0,"title":["Effectiveness of Supramaximal Angiotensin-converting Enzyme Inhibition in Controlling Proteinuria in Congenital Nephrotic Syndrome with Cytomegalovirus Infection and an NPHS1 Mutation"],"prefix":"10.4103","volume":"34","author":[{"given":"Nimisha","family":"Dange","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Topiwala National Medical College and B.Y.L. Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiran P.","family":"Sathe","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Topiwala National Medical College and B.Y.L. Nair Charitable Hospital, Mumbai, Maharashtra, India"},{"name":"Division of Pediatric Nephrology, Sir H. N. Reliance Foundation Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alpana","family":"Kondekar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Topiwala National Medical College and B.Y.L. Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vishal","family":"Sawant","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Topiwala National Medical College and B.Y.L. Nair Charitable Hospital, Mumbai, Maharashtra, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2024,3,11]]},"reference":[{"key":"R1-20240314","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1159\/000503303","article-title":"Congenital nephrotic syndrome in India in the current era: A multicenter case series","volume":"144","author":"Sinha","year":"2020","journal-title":"Nephron"},{"key":"R2-20240314","doi-asserted-by":"crossref","first-page":"1985","DOI":"10.1007\/s00467-020-04578-4","article-title":"Congenital nephrotic syndrome: Is early aggressive treatment needed?","volume":"35","author":"H\u00f6ltt\u00e4","year":"2020","journal-title":"Yes. Pediatr Nephrol"},{"key":"R3-20240314","doi-asserted-by":"crossref","first-page":"740","DOI":"10.1007\/s00467-006-0051-4","article-title":"Cytomegalovirus-related congenital nephrotic syndrome with diffuse mesangial sclerosis","volume":"21","author":"Besbas","year":"2006","journal-title":"Pediatr Nephrol"},{"key":"R4-20240314","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1007\/s12519-016-0009-y","article-title":"The etiology of congenital nephrotic syndrome: Current status and challenges","volume":"12","author":"Wang","year":"2016","journal-title":"World J Pediatr"},{"key":"R5-20240314","doi-asserted-by":"crossref","first-page":"2121","DOI":"10.1007\/s00467-007-0633-9","article-title":"Congenital nephrotic syndrome","volume":"24","author":"Jalanko","year":"2009","journal-title":"Pediatr Nephrol"},{"key":"R6-20240314","first-page":"171","article-title":"The renin-angiotensin-aldosterone system in renal and cardiovascular disease and the effects of its pharmacological blockade","volume":"3","author":"Macia-Heras","year":"2012","journal-title":"J Diabetes Metab"},{"key":"R7-20240314","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1186\/CVM-2-4-155","article-title":"Dose response of ACE inhibitors: Implications of the SECURE trial","volume":"2","author":"Lonn","year":"2001","journal-title":"Curr Control Trials Cardiovasc Med"},{"key":"R8-20240314","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1053\/ajkd.2002.36005","article-title":"High-dose ACE inhibition: Can it improve renoprotection?","volume":"40","author":"Navis","year":"2002","journal-title":"Am J Kidney Dis"},{"key":"R9-20240314","doi-asserted-by":"crossref","first-page":"S1359","DOI":"10.1016\/j.drudis.2020.08.005","article-title":"Pharmacology of enalapril in children: A review","volume":"25","author":"Smeets","year":"2020","journal-title":"Drug Discov Today"},{"key":"R10-20240314","doi-asserted-by":"crossref","first-page":"2443","DOI":"10.1093\/ndt\/gfn239","article-title":"Maximizing inhibition of the renin-angiotensin system with high doses of converting enzyme inhibitors or angiotensin receptor blockers","volume":"23","author":"Berl","year":"2008","journal-title":"Nephrol Dial Transplant"},{"key":"R11-20240314","doi-asserted-by":"crossref","first-page":"1340","DOI":"10.1007\/s00467-005-1918-5","article-title":"Congenital nephrotic syndrome responsive to angiotensinconverting enzyme inhibition","volume":"20","author":"Sreedharan","year":"2005","journal-title":"Pediatr Nephrol"},{"key":"R12-20240314","doi-asserted-by":"crossref","first-page":"14","DOI":"10.2165\/00002018-199207010-00004","article-title":"Adverse effects of angiotensin converting enzyme (ACE) inhibitors. An update","volume":"7","author":"Parish","year":"1992","journal-title":"Drug Saf"}],"container-title":["Saudi Journal of Kidney Diseases and Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/1319-2442.397210","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T18:26:53Z","timestamp":1787768813000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/1319-2442.397210"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023]]},"references-count":12,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2023]]}},"URL":"https:\/\/doi.org\/10.4103\/1319-2442.397210","relation":{},"ISSN":["1319-2442","2320-3838"],"issn-type":[{"value":"1319-2442","type":"print"},{"value":"2320-3838","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023]]}}}