Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children.
OBJECTIVE
Frequently relapsing and steroid-dependent minimal-change nephrotic syndrome (MCNS) that originates in childhood can persist after puberty in >20% of patients. These patients require immunosuppressive treatment during several decades of their life. We examined long-term adverse effects of persistent nephrotic syndrome and immunosuppressive medications, focusing on renal function, growth, obesity, osteoporosis, hypertension, ocular complications, and fertility in adult patients with biopsy-proven childhood-onset MCNS. Molecular analysis was performed to evaluate a possible association of a complicated course of MCNS with podocyte gene mutations.


METHODS
We performed a prospective clinical examination of 15 adult patients that included serum and urine analysis; dual-energy x-ray absorptiometry; ophthalmologic examination; semen examination; and molecular analysis of NPHS1, NPHS2, CD2AP, and ACTN4 genes.


RESULTS
All patients had normal GFR. Most frequent long-term complications were hypertension (in seven of 15 patients) and osteoporosis in one third of patients. Oligozoospermia was found in one patient, reduced sperm motility in four of eight patients, and teratozoospermia in six of eight patients. Ophthalmologic examination revealed myopia in 10 of 15 patients and cataract in three of 15 patients.


CONCLUSIONS
Children with MCNS that persists after puberty are at risk for complications such as osteoporosis, hypertension, cataract, and sperm abnormalities. Our study underscores a need for more effective and less toxic therapies for relapsing MCNS.
Kyrieleis
Henriette A C
HA
Department of Pediatric Nephrology, Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands.
Löwik
Marije M
MM
Pronk
Ilse
I
Cruysberg
Hans R M
HR
Kremer
Jan A M
JA
Oyen
Wim J G
WJ
van den Heuvel
Bert L P
BL
Wetzels
Jack F M
JF
Levtchenko
Elena N
EN
D015502:Absorptiometry, Photon; D000293:Adolescent; D000328:Adult; D001706:Biopsy; D015519:Bone Density; D002648:Child; D002675:Child, Preschool; D005128:Eye Diseases / Q000209:etiology; D005260:Female; D005298:Fertility; D006801:Humans; D007223:Infant; D047908:Intracellular Signaling Peptides and Proteins / Q000235:genetics; D007668:Kidney / Q000503:physiopathology; D008297:Male; D008565:Membrane Proteins / Q000235:genetics; D009402:Nephrosis, Lipoid / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D012008:Recurrence
D047908:Intracellular Signaling Peptides and Proteins; D008565:Membrane Proteins; C407223:NPHS2 protein; C113267:nephrin