DIAGNOSIS OF CONGENITAL NEPHROTIC SYNDROME: A CLINICAL AND A PATHOLOGIC CHALLENGE |
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Authors: | Ada Rivera Suzanne Meleg-Smith Igor Yosipiv Samir El-Dahr Frank Boineau |
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Institution: |
a Department of Pathology and Laboratory Medicine, Tulane University Health Sciences Center, New Orleans, Louisiana, USA.
b Department of Pediatrics, Pediatric Nephrology Section, Tulane University Health Sciences Center, New Orleans, Louisiana, USA. |
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Abstract: | The diagnosis of congenital nephrotic syndrome (NS) is a challenge both for clinicians and for pathologists. We observed three cases in a series of 50 children with NS nonresponsive to therapy, corresponding to one case each of minimal change disease, Finnish-type glomerulopathy, and diffuse mesangial sclerosis--two histopathologic studies were performed in each case. The age at presentation did not predict the diagnosis nor the prognosis: The NS presented at 7 months of age in the patient with diffuse mesangial sclerosis, but it was present at birth in the patient with minimal change disease. In these 2 patients the final diagnosis was made with the first renal biopsy. Conversely, in the patient with Finnish-type glomerulopathy, the diagnosis was only possible in the repeat biopsy, as the early pathologic changes were nonspecific. This study shows the essential role of the renal biopsy in determining the etiologic diagnosis and prognosis in patients with congenital nephrotic syndrome. |
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