Di Meglio Lavinia, Grimaldi Giusi, Esposito Francesco, Gelzo Monica, Esposito Maria Valeria, Castaldo Giuseppe, Canani Roberto Berni
Department of Translational Medical Science, University Federico II, Naples, Italy.
Diagnostica Ecografica e Prenatale Aniello Di Meglio, Naples, Italy.
Front Pediatr. 2022 Jan 20;9:810765. doi: 10.3389/fped.2021.810765. eCollection 2021.
Oral salt substitutive therapy is pivotal for the survival of patients with congenital chloride diarrhea (CLD), however this therapy is unable to influence the symptoms severity. Butyrate has been proposed to limit diarrhea severity in CLD. Unfortunately, the optimal dose schedule is still largely undefined. In addition, butyrate seems not to be well-tolerated by all patients, with some subjects reporting diarrhea worsening. We investigated the efficacy of a step-up therapeutic approach with sodium butyrate in patients who experienced a diarrhea worsening or an absent improvement after the direct administration of 100 mg/kg/day of sodium butyrate.
The efficacy of a step-up therapeutic approach starting from 50 mg/Kg/day with a subsequent 25 mg/kg/day weekly increase up to 100 mg/kg/day of oral sodium butyrate was investigated in previously three unresponsive CLD children.
The step-up therapeutic approach resulted effective in limiting diarrhea severity in all our three previously unresponsive CLD patients.
Our results suggest the efficacy of the step-up therapeutic approach in CLD children.
口服盐替代疗法对先天性氯腹泻（CLD）患者的生存至关重要，然而这种疗法无法影响症状的严重程度。有人提出丁酸盐可减轻CLD患者的腹泻严重程度。遗憾的是，最佳剂量方案仍很大程度上未明确。此外，并非所有患者对丁酸盐的耐受性都良好，一些患者报告腹泻加重。我们研究了对直接给予100mg/kg/天丁酸钠后腹泻加重或无改善的患者采用递增式丁酸钠治疗方法的疗效。
在之前三名无反应的CLD儿童中，研究了从50mg/Kg/天开始，随后每周增加25mg/kg/天直至100mg/kg/天口服丁酸钠的递增式治疗方法的疗效。
递增式治疗方法在我们所有三名之前无反应的CLD患者中均有效减轻了腹泻严重程度。
我们的结果表明递增式治疗方法对CLD儿童有效。
