Failure of isolated kidney transplantation in a pediatric patient with primary hyperoxaluria type 2

PH type 2 is caused by decreased activity of GRHPR enzyme that eventually leads to ESRD and systemic oxalosis. Here, we describe an Iranian pediatric patient with PH2 and early ESRD development who received recommended treatment by undergoing isolated kidney transplantation. Diagnosis criteria inclu...

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Bibliographic Details
Published in:Pediatric transplantation 2014-05, Vol.18 (3), p.E69-E73
Main Authors: Naderi, GholamHossein, Latif, AmirHossein, Tabassomi, Firouzeh, Esfahani, Seyed Taher
Format: Article
Language:English
Subjects:
Alcohol Oxidoreductases - metabolism
Biopsy
Calcium Oxalate - chemistry
Child
Creatinine - blood
graft loss
Graft Rejection
Humans
Hyperoxaluria, Primary - complications
Hyperoxaluria, Primary - therapy
Iran
Kidney Calculi - complications
Kidney Failure, Chronic - complications
Kidney Failure, Chronic - therapy
Kidney Transplantation
Liver - enzymology
Liver Failure
Male
Nephrectomy
oxalosis
primary hyperoxaluria type 2
treatment
Treatment Outcome
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Summary:PH type 2 is caused by decreased activity of GRHPR enzyme that eventually leads to ESRD and systemic oxalosis. Here, we describe an Iranian pediatric patient with PH2 and early ESRD development who received recommended treatment by undergoing isolated kidney transplantation. Diagnosis criteria included a history of reoccurring calcium oxalate renal stones and elevated oxalate levels combined with liver biopsy and decreased enzymatic activity at age five. ESRD prompted transplantation and was performed at age nine. On Day 12 post‐op, his serum creatinine level increased. A graft biopsy showed calcium oxalate crystal deposits in renal tubes with no evidence of acute rejection, which resolved with intensive hydration and administration of a potassium citrate solution. Subsequent biopsies confirmed results found in first biopsy. Despite the immunosuppressive therapy, his serum creatinine level increased again after 11 months. Renal tubular obstruction then led to graft nephrectomy. Pathological analysis of tissue confirmed findings of past biopsies. This was a very rare case of early ESRD in PH2 resulting in a failed isolated kidney transplant. As the GRHPR enzyme is predominantly expressed in liver, we suggest a combined liver‐kidney transplant may be beneficial in patients with PH2.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.12240