Steroid-sensitive nephrotic syndrome: from childhood to adulthood.
BACKGROUND
The clinical presentation, treatment, and outcome of steroid-sensitive nephrotic syndrome (SSNS) during childhood have been extensively studied. Conversely, few data regarding the outcome in adulthood of childhood SSNS have been published previously. We undertook to conduct a retrospective study of the outcome in adulthood of a large cohort of patients diagnosed with an SSNS during childhood.


METHODS
We identified all children born between 1970 and 1975 who had been admitted to our institution for an SSNS. Data regarding the outcome in adulthood of these patients were obtained through mailed questionnaires or phone calls to patients and/or their parents or through attending physicians.


RESULTS
One hundred seventeen patients were identified. Data regarding the outcome of SSNS in adulthood were available for 102 patients (87.2%). Forty-three patients (42.2%) experienced at least one relapse of nephrotic syndrome in adulthood. By univariate analysis, young age at onset (<6 years) and more severe disease in childhood, indicated by a greater number of relapses (12.9 for adulthood relapsers versus 5.4 for adulthood nonrelapsers; P < 0.0001) and more frequent use of immunosuppressors (74.4% versus 31.6%; P < 0.0001) or cyclosporine (42.9% versus 7.3%; P < 0.0001) were predictive of the occurrence of SSNS relapse in adulthood. Conversely, relapse rate in the first 6 months of disease was not predictive of further relapses in adulthood. By multivariate analysis, only number of relapses during childhood was predictive of adulthood relapses (P < 0.0058). Long-term side effects of steroids were found in 44.2% of adulthood relapsers; the most frequent were osteoporosis and excess weight.


CONCLUSIONS
The incidence of childhood SSNS relapses in adulthood was relatively high in our study. Further studies are required to assess long-term complications in adults with relapses and a history of prolonged steroid and immunosuppressor use.
Fakhouri
Fadi
F
Department of Nephrology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, Paris, France. fakhouri@necker.fr
Bocquet
Nathalie
N
Taupin
Pierre
P
Presne
Claire
C
Gagnadoux
Marie-France
MF
Landais
Paul
P
Lesavre
Philippe
P
Chauveau
Dominique
D
Knebelmann
Bertrand
B
Broyer
Michel
M
Grünfeld
Jean-Pierre
JP
Niaudet
Patrick
P
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000453:epidemiology; D012008:Recurrence; D012189:Retrospective Studies; D017678:Sex Distribution; D013256:Steroids / Q000627:therapeutic use*; D016896:Treatment Outcome
D013256:Steroids