生长抑素类似物和免疫抑制疗法治疗新生儿和婴儿乳糜腹膜和乳糜胸的复杂形式

A. S. Gurskaya, M. A. Sulavko, R. R. Bayazitov, I. V. Karnuta, E. V. Ekimovskaya, O. N. Nakovkin, D. M. Akhmedova, A. A. Klepikova, R. A. Khagurov, N. V. Petrova, V. А. Skvortsova
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引用次数: 0

摘要

乳糜腹膜和乳糜胸是死亡率高的罕见疾病,其最佳治疗策略尚不清楚。该研究的目的是评价合成生长抑素类似物(奥曲肽)和西罗莫司免疫抑制治疗乳糜腹膜和乳糜胸的结果。该研究得到了俄罗斯卫生部国家儿童健康医学研究中心独立伦理委员会和科学委员会的批准。患者的父母同意使用他们孩子的数据,包括照片,用于研究目的和出版物。我们对2018年至2022年诊治的9例先天性乳糜腹膜和乳糜胸患儿进行了回顾性研究。所有患儿均接受腹腔或胸腔引流、肠外营养和药物保守治疗。第一线治疗是14-20天的奥曲肽,如果没有效果,则改用西罗莫司。观察奥曲肽保守治疗5例的疗效,剂量为5 ~ 10µg/kg/h。如果到第14天还没有效果,患者开始服用西罗莫司,剂量为0.05-0.2 mg/天,证明对所有患者都有效(n = 4)。我们的研究表明,西罗莫司对新生儿和婴儿复杂的乳糜腹膜和乳糜胸病例有效。由于这些疾病的罕见性,我们的结论是基于一个小队列的分析。为了证实我们的结果并制定统一的诊断和治疗指南,需要进一步进行更有针对性的多中心研究。在这些指导方针被采纳之前,关于乳糜腹膜和乳糜胸的治疗应根据个人情况作出决定,并由治疗中心的医学委员会批准。
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A somatostatin analogue and immunosuppressive therapy in the treatment of complex forms of chyloperitoneum and chylothorax in newborns and infants
Chyloperitoneum and chylothorax are rare conditions with high mortality rates whose optimal treatment strategy remains unclear. The aim of the study was to evaluate the results of chyloperitoneum and chylothorax treatment with a synthetic somatostatin analogue (octreotide) and immunosuppressive therapy with sirolimus. The study was approved by the Independent Ethics Committee and the Scientific Council of the National Medical Research Center for Children’s Health of Ministry of Healthсare of Russia. The patients' parents gave their consent to the use of their children's data, including photographs, for research purposes and in publications. We conducted a retrospective study of nine children diagnosed with congenital chyloperitoneum and chylothorax who had been treated from 2018 to 2022. All the children received either abdominal or pleural drainage, parenteral nutrition, and conservative therapy with drugs. The first line of therapy was octreotide for 14–20 days that was then switched to sirolimus if there had been no effect. The effectiveness of conservative therapy with octreotide at a dose of 5–10 µg/kg/hour was observed in 5 cases. If there had been no effect by day 14, the patients were started on sirolimus at a dose of 0.05–0.2 mg/day which proved to be effective in all the patients (n = 4). Our study showed that sirolimus is effective in complex cases of chyloperitoneum and chylothorax in newborns and infants. Because of the rarity of these disorders, our conclusions were based on the analysis of a small cohort. To confirm our results and develop uniform diagnostic and treatment guidelines, further, more targeted multicenter research is needed. Until such guidelines are adopted, decisions on the treatment of chyloperitoneum and chylothorax should be made on an individual basis and approved by the medical committee of a treatment center.
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来源期刊
Pediatric Hematology/Oncology and Immunopathology
Pediatric Hematology/Oncology and Immunopathology Medicine-Pediatrics, Perinatology and Child Health
CiteScore
0.40
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发文量
49
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