We report a female infant with Galloway-Mowat syndrome. In addition to the characteristic dysmorphic appearance, neurological anomalies and early-onset nephrotic syndrome, she had arachnodactyly, an observation thus far reported uniquely in Taiwan. Also, her elder sister had the same condition. Renal pathology on light microscopy showed cystic dilatation of the renal tubules. Electron microscopy showed an irregular glomerular basement membrane and effacement of foot processes. This observation suggests that malformation of the glomerular basement membrane may cause the glomerulopathy in Galloway-Mowat syndrome.

No abstract

We retrospectively reviewed our experience of Epstein-Barr virus (EBV)-associated acute renal failure. Of 165 previously healthy children hospitalized with serologically proven primary EBV infection, 8 had acute renal failure, of whom 5 (group A) did not have virusassociated hemophagocytic syndrome (VAHS), while 3 (group B) did have VAHS. All had complications in four or more organ systems. Two patients in group A had renal biopsies showing acute tubulointerstitial nephritis, and the clinical and laboratory findings in the other 3 group A patients were consistent with acute tubulointerstitial nephritis. Acyclovir was used in 1 patient, but she died of hepatic failure and pulmonary hemorrhage. The other 4 spontaneously recovered renal function after supportive care, including hemodialysis in 1 patient. Our experience does not support the routine use of corticosteroids or antiviral agents in these patients. Children in group B had a relatively normal urinalysis. Renal biopsies were not performed, but their presentations were compatible with acute tubular necrosis. We conclude that EBV should be considered as a possible etiological agent in all children presenting with acute renal failure of unknown cause. The diagnosis depends on a high index of suspicion and careful serological evaluation in atypical cases.