Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome.
OBJECTIVE
Long-term outcome of steroid-sensitive idiopathic nephrotic syndrome (SSNS) in children is usually considered benign, although data on follow-up into adulthood are scarce. The aim of this study was to investigate adults who had childhood SSNS regarding their relapse rate, growth, and renal and extrarenal morbidity.


METHODS
Adult patients (n=42, 26 males) were evaluated at a median age of 28.0 (18.1 to 46.9) years and a median follow-up of 22.0 (2.9 to 39.0) years since diagnosis.


RESULTS
Fourteen of 42 (33%) patients relapsed in adulthood. The number of relapses during childhood and adolescence and a complicated course-administration of steroid-sparing medication such as cyclophosphamide, chlorambucil, and cyclosporin A-were identified as risk factors. Final adult height (median SD score -0.4, range -3.3 to +1.3) and body mass index (BMI) were normal. Renal function was normal in all patients, and overall morbidity was low. Only eight patients (three males) had children. Cytotoxic therapy was identified as a major factor contributing to childlessness.


CONCLUSIONS
Relapses in adulthood were common in pediatric patients with SSNS. Growth and renal function were normal, and overall morbidity was low. Yet, transition to an adult nephrologist is recommended for all children with relapsing SSNS.
Rüth
Eva-Maria
EM
University Children's Hospital Zurich, Department of Pediatric Nephrology, Zurich, Switzerland. eva-maria.rueth@gmx.de
Kemper
Markus J
MJ
Leumann
Ernst P
EP
Laube
Guido F
GF
Neuhaus
Thomas J
TJ
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000328:Adult; D018906:Antineoplastic Agents, Alkylating / Q000009:adverse effects*; D002675:Child, Preschool; D002699:Chlorambucil / Q000009:adverse effects*; D003520:Cyclophosphamide / Q000009:adverse effects*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007248:Infertility, Male / Q000139:chemically induced*; D007677:Kidney Function Tests; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D009765:Obesity / Q000139:chemically induced*; D011379:Prognosis; D012008:Recurrence
D000305:Adrenal Cortex Hormones; D018906:Antineoplastic Agents, Alkylating; D002699:Chlorambucil; D003520:Cyclophosphamide