Primary hyperoxaluria type 2 and systemic oxalosis. A case report

Primary hyperoxaluria type 2 is a rare disease characterized by over production of oxalate due to a deficiency of an intra hepatic enzyme leading to renal lithiasis, nephrocalcinosis and chronic kidney damage. We report the case of 17-year-old male patients with history of urinary tract infections a...

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Detalhes bibliográficos
Autores: Loza Munarriz, Reyner, Ponce Gambini, Jenny, Ynguil Muñoz, Angélica, Sarmiento Barrientos, Tatiana, Cok García, Jaime
Tipo de documento: artigo
Estado:Versão publicada
Data de publicação:2019
País:Perú
Recursos:Universidad Peruana Cayetano Heredia
Repositório:Revistas - Universidad Peruana Cayetano Heredia
Idioma:espanhol
OAI Identifier:oai:revistas.upch.edu.pe:article/3585
Acesso em linha:https://revistas.upch.edu.pe/index.php/RMH/article/view/3585
Access Level:Acceso aberto
Palavra-chave:Hiperoxaluria
nefrocalcinosis
urolitiasis
insuficiencia renal crónica
Hyperoxaluria, nephrocalcinosis, urolithiasis, renal insufficiency, chronic.
Descrição
Resumo:Primary hyperoxaluria type 2 is a rare disease characterized by over production of oxalate due to a deficiency of an intra hepatic enzyme leading to renal lithiasis, nephrocalcinosis and chronic kidney damage. We report the case of 17-year-old male patients with history of urinary tract infections and renal lithiasis since the age of 6 years. The patient developed end-stage kidney disease at the age of 11 years receiving chronic ambulatory peritoneal dialysis. He developed back pain and polyarthralgia of the ankles, knees, shoulders and progressive deformity of the hands. The magnetic resonance of the spine revealed flattening of D9-D9. Bone biopsy of the affected area showed presence of calcium oxalate. A genetic study confirmed the diagnosis of primary hyperoxaluria type 2. This entity should be suspected in children with renal stones at an early age, this may be the first case in Peru.