期刊
AMERICAN JOURNAL OF KIDNEY DISEASES
卷 58, 期 3, 页码 453-455出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.ajkd.2011.05.012
关键词
Primary hyperoxaluria; infantile oxalosis; treatment; Oxalobacter formigenes
The spectrum of primary hyperoxaluria type I is extremely heterogeneous, ranging from singular to recurrent urolithiasis and early end-stage renal disease (ESRD). In infantile oxalosis, the most devastating form, ESRD occurs as early as within the first weeks of life. No kidney replacement therapy sufficiently removes endogenously overproduced oxalate. However, curative combined liver-kidney transplant often is impracticable in small infants. Oxalobacter formigenes (O formigenes), an anaerobic oxalate-degrading bacterium, is a colonizer of the healthy human colon. Oral administration of O formigenes has been shown to significantly decrease urine and plasma oxalate levels in patients with primary hyperoxaluria. We report compassionate use of O formigenes in two 11-month-old girls with infantile oxalosis and ESRD. They received O formigenes twice a day for 4 weeks (or until transplant). Dialysis regimens were unchanged. Plasma oxalate levels decreased from > 110 mu mol/L before to 71.53 mu mol/L under treatment in patient 1 and from > 90 to 68.56 mu mol/L (first treatment period) and 50.05 mu mol/L (second treatment period) in patient 2. O formigenes was well tolerated. No serious side effects were reported. Extremely increased plasma oxalate levels in patients with infantile oxalosis may enable intestinal elimination of endogenous oxalate in the presence of O formigenes. Therefore, O formigenes therapy may be helpful as a bridging procedure until transplant in such patients. Am J Kidney Dis. 58(3): 453-455. (C) 2011 by the National Kidney Foundation, Inc.
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