BACKGROUND Infantile oxalosis, the most devastating form of primary hyperoxaluria type 1 (PH1), often leads to end-stage renal disease (ESRD) during the first weeks to months of life. CASE-DIAGNOSIS Here, we report the outcome of the therapeutic use of Oxalobacter formigenes (Oxabact OC5; OxThera AB, Stockholm, Sweden) in a female infant with PH1 who exhibited severely elevated plasma oxalate (Pox) levels, pronounced nephrocalcinosis, anuretic end-stage renal disease, and retinal oxalate deposits. Following the diagnosis of PH1 at an age of 8 weeks, a combined regimen of daily peritoneal dialysis, daily pyridoxine treatment and hemodialysis (3 times a week) was unable to reduce the pronounced hyperoxalemia. After the addition of Oxalobacter formigenes therapy to the otherwise unchanged treatment regimen, Pox levels first stabilized and subsequently declined from 130 μmol/L to around 80 μmol/L. Nephrocalcinosis and retinal deposits stabilized. Oxalobacter formigenes treatment was well-tolerated and no related adverse events were observed. The patient showed nearly age-appropriate growth and development and received successful combined liver-kidney transplantation at the age of two years. CONCLUSIONS Treatment with O. formigenes combined with intensive dialysis led to reduction of Pox, stabilization of systemic oxalosis, and improvement in the clinical disease course. O. formigenes treatment may be an option for reduction of oxalosis in infantile patients with insufficient response to conservative treatments until combined liver-kidney transplantation can be performed.

中文翻译：

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富氧草酸杆菌治疗与深层透析相结合，可降低重症婴儿草酸血症患者的血浆草酸根水平并中止疾病进展。

背景婴儿草酸化症是原发性高草酸尿症1型（PH1）最具破坏性的形式，通常在生命的最初几周至几个月内导致终末期肾脏疾病（ESRD）。病例诊断在这里，我们报道了在母体PH1严重升高的血浆草酸盐（Pox）水平，明显的肾钙化病，尿毒症末期的女婴中，使用产氧化草酶（Oxabact OC5； OxThera AB，斯德哥尔摩，瑞典）的结果。阶段性肾脏疾病和视网膜草酸盐沉积。在8周龄诊断为PH1之后，每日腹膜透析，每日吡ido醇治疗和血液透析（每周3次）的联合治疗无法降低明显的高氧血症。在原本不变的治疗方案中增加了草酸杆菌的治疗后，痘痘水平首先稳定下来，然后从130μmol/ L降至约80μmol/ L。肾钙化和视网膜沉积稳定。变形草酸杆菌的治疗耐受性良好，未观察到相关不良事件。该患者显示出接近年龄的生长和发育，并在两岁时成功完成了肝肾联合移植。结论O.formigenes治疗与强化透析相结合可减少Pox，稳定系统性草酸中毒并改善临床疾病进程。在对保守治疗反应不足的婴儿患者中，O。formigenes治疗可能是减少草酸化的一种选择，直到可以进行肝肾联合移植。肾钙化和视网膜沉积稳定。变形草酸杆菌的治疗耐受性良好，未观察到相关不良事件。该患者显示出接近年龄的生长和发育，并在两岁时成功完成了肝肾联合移植。结论O.formigenes联合深层透析治疗可减少Pox，稳定系统性草酸中毒并改善临床疾病进程。在对保守治疗反应不足的婴儿患者中，O。formigenes治疗可能是减少草酸化的一种选择，直到可以进行肝肾联合移植。肾钙化和视网膜沉积稳定。变形草酸杆菌的治疗耐受性良好，未观察到相关不良事件。该患者显示出接近年龄的生长和发育，并在两岁时成功完成了肝肾联合移植。结论O.formigenes治疗与强化透析相结合可减少Pox，稳定系统性草酸中毒并改善临床疾病进程。在对保守治疗反应不足的婴儿患者中，O。formigenes治疗可能是减少草酸化的一种选择，直到可以进行肝肾联合移植。该患者显示出接近年龄的生长和发育，并在两岁时成功完成了肝肾联合移植。结论O.formigenes治疗与强化透析相结合可减少Pox，稳定系统性草酸中毒并改善临床疾病进程。在对保守治疗反应不足的婴儿患者中，O。formigenes治疗可能是减少草酸化的一种选择，直到可以进行肝肾联合移植。该患者显示出接近年龄的生长和发育，并在两岁时成功完成了肝肾联合移植。结论O.formigenes联合深层透析治疗可减少Pox，稳定系统性草酸中毒并改善临床疾病进程。在对保守治疗反应不足的婴儿患者中，O。formigenes治疗可能是减少草酸化的一种选择，直到可以进行肝肾联合移植。