Open AccessNail–Patella syndrome: A rare cause of nephrotic syndrome in pregnancy
Author(s) -
IN Aboobacker,
AS Krishnakumar,
Sajith Narayanan,
B Hafeeque,
JC Gopinathan,
Fahad Aziz
Publication year - 2018
Publication title -
indian journal of nephrology/indian journal of nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.317
H-Index - 24
eISSN - 1998-3662
pISSN - 0971-4065
DOI - 10.4103/ijn.ijn_362_16
Subject(s) - medicine , nephrotic syndrome , renal vein thrombosis , proteinuria , pregnancy , asymptomatic , gastroenterology , surgery , urology , kidney , biology , genetics
Nail-Patella syndrome (NPS) is an inherited disease with characteristic nail, limb, and renal anomalies. While almost all patients manifest classical skeletal and nail abnormalities, renal involvement is seen in only 40% of patients. Asymptomatic proteinuria is the most common renal presentation. Although a substantial number of patients can progress to nephrotic range proteinuria, development of end-stage kidney disease is rare. Pathological abnormalities are appreciable only by electron microscopy. Our patient who had not been diagnosed with NPS previously presented with new-onset proteinuria during the second trimester of her first pregnancy. The characteristic physical and radiological features confirmed the diagnosis of NPS. Her clinical course was complicated by nephrotic syndrome with fetal loss at 22 weeks and postpartum renal vein thrombosis. Nephrotic state improved after termination of pregnancy. She was managed with angiotensin receptor blocker and anticoagulation. At the end of 2 years of follow-up, she has normal glomerular filtration rate with minimal proteinuria.