| Clinicopathological Study about Childhood Primary Nephrotic Syndrome Resistant to 4-week Daily Steroid Therapy |
|
Jung Sue Kim, Il Soo Ha, Hae Il Cheong, Yong Choi |
|
Department of Pediatrics, Seoul National University, College of Medicine, Seoul, Korea |
| 소아의 스테로이드 저항성 신증후군의 임상병리학적 고찰 |
|
김정수, 하일수, 정해일, 최용 |
|
서울대학교 의과대학 소아과학교실 |
|
|
|
|
| Abstract |
Purpose
: Steroid-resistant nephrotic syndrome in children is difficult to manage and tends to
progress to chronic renal failure. We studied clinicopathological correlations in primary nephrotic
syndrome in children resistant to 4-week daily steroid therapy.
Methods
: Among children who had been admitted to Seoul National University Children's
Hospital during the period between Oct. 1985 and Jul. 1995 and diagnosed as primary nephrotic
syndrome, 87 patients were selected for this study. They showed poor response to 4-week daily
steroid therapy either initially (initial nonresponder) or subsequently in the disease course
(subsequent nonresponder). The medical records including renal pathologic findings were
analyzed retrospectively.
Results
: The mean age at the onset of nephrotic syndrome was 7.3¡¾4.1 years and male to
female ratio was 62:25. Pathologically, 28 (32%) had minimal change lesion (MCL), 47 (54%)
had focal segmental glomerulosclerosis (FSGS) and 12 (14%) had others. There were 15 (54%)
initial nonresponders and 13 (46%) subsequent nonresponders in the MCL group, and there were
26 (55%) and 21 (45%), respectively, in the FSGS group. The incidence of hematuria was less
frequent in the MCL group. The frequencies of hypertension and azotemia were not significantly
different between in the MCL and the FSGS group. Among 10 patients with MCL in whom the
steroid therapy were extended to 6 weeks, 3 patients responded subsequently. And 1 of 3
patients among the FSGS group responded to 8-week daily steroid therapy. The 2nd line drug
therapy such as oral cyclophosphamide, intravenous pulsed methylprednisolone, enalapril,
dipyridamole, etc. was tried in 26 patients with MCL and all 47 patients with FSGS. In the
MCL group, 7 of 13 initial nonresponders and 9 of 13 subsequent nonresponders responded to
these 2nd line drug therapies. In the FSGS group, 10 of 26 initial nonresponders and 11 of 21
subsequent nonresponders responded to these therapies. While only 1 subsequent nonresponder in
the MCL group progressed to chronic renal failure, 9 initial and 4 subsequent nonresponders
progressed in the FSGS group.
Conclusion
: The FSGS group formed about a half and the MCL group formed about a third
of steroid-resistant nephrotic syndrome in children. Although the response to 2nd line drug
therapies was not different between 2 groups, the incidence of progression to chronic renal
failure was significantly higher in the FSGS group. |
| Key Words:
Steroid-resistant nephrotic syndrome, Minimal change lesion, Focal segmental glomerulosclerosis, Initial nonresponder, Subsequent nonresponder |
|
PDF Links
Download Citation
