Secondary renal Fanconi syndrome caused by valproate therapy.
Although renal Fanconi syndrome resulting from valproate (VPA) has occasionally been reported, the detailed clinical characteristics of this disease remain unclear. To clarify the clinical features of patients with VPA-induced Fanconi syndrome, we analyzed the clinical and laboratory data of seven affected patients. All patients were children, were severely disabled and required tube feeding. Five patients required treatment with multiple anticonvulsant agents. Hypophosphatemia and hypouricemia were found in all patients. Mild proteinuria, increased excretion of urinary beta2-microglobulin (beta2MG) and generalized hyperaminoaciduria were present in all patients. The renal biopsy of one patient exhibited tubulointerstitial nephritis without any structural abnormalities of the mitochondria in proximal renal tubular cells. All patients recovered from the Fanconi syndrome after the cessation of VPA therapy without any long-term renal sequellae. These results indicate that young age and being severely disabled with tube feeding and anticonvulsant polytherapy are contributory factors to the development of VPA-induced Fanconi syndrome. Serum phosphate and uric acid concentrations and urinary beta2MG levels in addition to serum electrolytes and urinalysis should be examined regularly in patients receiving VPA therapy, especially in those with the contributory factors outlined above. Patients with Fanconi syndrome caused by VPA have a favorable renal outcome.
Watanabe
Toru
T
Department of Pediatrics, Niigata City General Hospital, Japan. twata@hosp.niigata.niigata.jp
Yoshikawa
Hideto
H
Yamazaki
Sawako
S
Abe
Yuki
Y
Abe
Tokinari
T
D000293:Adolescent; D000927:Anticonvulsants / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D005198:Fanconi Syndrome / Q000097:blood / Q000139:chemically induced* / Q000652:urine; D005260:Female; D006801:Humans; D007674:Kidney Diseases / Q000097:blood / Q000139:chemically induced* / Q000652:urine; D008297:Male; D009395:Nephritis, Interstitial / Q000139:chemically induced; D014635:Valproic Acid / Q000009:adverse effects*
D000927:Anticonvulsants; D014635:Valproic Acid