From the Department of Pediatrics, University of Rochester School of Medicine, Rochester, NY.
the Department of Biostatistics and Computational Biology, University of Rochester School of Medicine, Rochester, NY.
J Pediatr Gastroenterol Nutr. 2022 Jul 1;75(1):42-47. doi: 10.1097/MPG.0000000000003459. Epub 2022 Apr 20.
Alteration of the airway microbiota is a hallmark of cystic fibrosis (CF) pulmonary disease. Dysfunction of cystic fibrosis transmembrane regulator (CFTR) in the intestine also promotes changes in local microbiota such as small intestinal bacterial overgrowth (SIBO), which is common in CF. We evaluated whether therapy with the CFTR modulator combination lumacaftor/ivacaftor (luma/iva) has a beneficial impact on SIBO as measured by breath testing (BT).
A multicenter longitudinal study of CFTR-dependent disease profiling (NCT02477319) included a prospective evaluation for SIBO by BT. Tidal breath samples were collected after fasting and 15, 30, 45, 60, 90, and 120 minutes after ingestion of glucose, before and 1 month after subjects initiated luma + iva.
Forty-two subjects enrolled in the sub-study (mean age = 23.3 years; 51% female; 9.5% Latinx); 38 completed a hydrogen BT at both time points, of which 73.7% had a positive BT before luma/iva (baseline) and 65.8% had a positive test after luma/iva ( P = 0.44); shifts from negative to positive were also seen. Use of azithromycin (63.1%) and inhaled antibiotics (60.5%) were not associated with positive BT. Acid-blocking medications were taken by 73% of those with a negative BT at baseline and by 35% with a positive baseline BT ( P = 0.04).
We found a high rate of positive hydrogen breath tests in individuals with CF, confirming that SIBO is common. One month of luma/iva did not significantly change the proportion of those with positive breath hydrogen measurements.
气道微生物群的改变是囊性纤维化（CF）肺部疾病的标志。肠道中囊性纤维化跨膜转导调节因子（CFTR）的功能障碍也会促进局部微生物群的变化，例如小肠细菌过度生长（SIBO），这在 CF 中很常见。我们评估了 CFTR 调节剂组合 lumacaftor/ivacaftor（luma/iva）治疗是否通过呼吸测试（BT）对 SIBO 有有益的影响。
一项囊性纤维化跨膜转导调节因子依赖性疾病分析的多中心纵向研究（NCT02477319）包括通过 BT 对 SIBO 进行前瞻性评估。空腹后采集潮式呼吸样本，并在摄入葡萄糖后 15、30、45、60、90 和 120 分钟以及受试者开始使用 luma+iva 前后 1 个月进行采集。
42 名受试者参加了子研究（平均年龄=23.3 岁；51%为女性；9.5%为拉丁裔）；38 名受试者在两个时间点均完成了氢 BT，其中 73.7%在 luma/iva 之前（基线）有阳性 BT，65.8%在 luma/iva 之后（P=0.44）有阳性测试；也观察到从阴性到阳性的转变。使用阿奇霉素（63.1%）和吸入抗生素（60.5%）与阳性 BT 无关。在基线 BT 阴性的受试者中，有 73%的人服用了抑酸药物，而在基线 BT 阳性的受试者中，有 35%的人服用了抑酸药物（P=0.04）。
我们发现 CF 患者的氢呼吸试验阳性率很高，证实 SIBO 很常见。一个月的 luma/iva 并没有显著改变呼吸氢测量阳性的比例。
