RESUMO
Primary hyperoxaluria is a rare hereditary disease caracterised by deposits of calcium oxalate in different tissues, essentially the kidney. The digestive localization was exceptional. We reported two childs who are aged respectively 13 and 16 years; they are admitted for end stage renal failure caused by primitive hyperoxaluria. The diagnosis was suspected because there were consanguinity, nephrolithiasis in the two cases and nephrocalcinosis in one. It is confirmed by increase oxalic and oxaluric levels. The hemodialysis was started. After some years, the two patients developed crystal deposition in her digestive wall
RESUMO
The authors reported 26 cases of osteodystrophy in witch 10 girls and 16 boys aged between 3 and 22 years. 9 patients are asymptomatic, 5 have bone pain and 6 with lower member distortion. The hypocalcaemia is recovered at 7 patient, the hyperphosphoremia at 17 and the increase of the alkalin phosphatases at 20. The PTH is constantly increased. The x-ray standard shows geodes in 8 cases, a reduction of the transparency in 9. All patient received substitutive treatment. The evolution is marked by a stabilization or improvement of lesions in 20 cases and an aggravation at 6