Secondary Amyloidosis with Fatal Outcome in a Child with Meningomyelocele: Need for a Closer Renal Surveillance

Abstract

Amyloidosis constitutes a group of diseases characterized by extracellular deposition of fibrils composed of low molecular weight subunits of a variety of proteins. Secondary amyloidosis may result from chronic inflammatory conditions such as familial Mediterranean fever, juvenile idiopathic arthritis, tuberculosis, and bronchiectasis. Myelomeningocele is reported rarely in literature as a cause of amyloidosis. We report here a case of 6-year-old boy with operated congenital lumbar myelomeningocele with paraparesis and neurogenic bladder, who was diagnosed with nephrotic syndrome with hypertension. He underwent a renal biopsy which showed amyloidosis. During the course of treatment, he acquired a perigenital infection and later succumbed to it. A closer follow-up of patients with neurogenic bladder can help identify and manage renal complications early.

Keywords

• infection
• neurogenic bladder
• saa
• steroid-resistant nephrotic syndrome