Primary hiperoxaluria diagnosed after kidney transplantation: report of 2 cases and literature review [documento electrónico] / John Fredy Nieto Ríos, ; Catalina Ocampo Kohn, ; Gustavo Adolfo Zuluaga Valencia, ; Arbey Aristizabal Álzate,  . - 2017.
Obra : Brazilian Journal of Nephrology

Idioma : Inglés (eng)

Palabras clave :	Hyperoxaluria  primary  kidney failure  chronic  kidney transplantation  lithiasis  nephrocalcinosis
Resumen :	Primary hyperoxaluria (PH) is a very rare genetic disorder; it is characterized by total or partial deficiency of the enzymes related to the metabolism of glyoxylate, with an overproduction of calcium oxalate that is deposited in different organs, mainly the kidney, leading to recurrent lithiasis, nephrocalcinosis and end stage renal disease (ESRD). In patients with ESRD that receive kidney transplantation alone, the disease has a relapse of 100%, with graft loss in a high percentage of patients in the first 5 years of transplantation. Three molecular disorders have been described in PH: mutation of the gene alanin glioxalate aminotransferase (AGXT); glyoxalate reductase/hydroxy pyruvate reductase (GRHPR) and 4-OH-2-oxoglutarate aldolase (HOGA1). We present two cases of patients with a history of renal lithiasis who were diagnosed with primary hyperoxaluria in the post-transplant period, manifested by early graft failure, with evidence of calcium oxalate crystals in renal biopsy, hyperoxaluria, hyperoxalemia, and genetic test compatible; they were managed with proper diet, abundant oral liquids, pyridoxine, hydrochlorothiazide and potassium citrate; however, they had slow but progressive deterioration of their grafts function until they reached end-stage chronic renal disease.
Mención de responsabilidad :	John Fredy Nieto Rios, Monica Zuluaga, Lina Maria Serna Higuita, Adriana Florez, Diana Carolina Bello-Marquez, Arbey Aristizábal, Catalina Ocampo Kohn, Gustavo Adolfo Zuluaga
Referencia :	J Bras Nefrol. 2017 Oct-Dec;39(4):462-466.
DOI (Digital Object Identifier) :	10.5935/0101-2800.20170081
PMID :	29319775
Derechos de uso :	CC BY
En línea :	http://bjn.org.br/about-the-authors/1996/en-US
Enlace permanente :	https://hospitalpablotobon.cloudbiteca.com/pmb/opac_css/index.php?lvl=notice_display&id=4072