Leng Changsen, Sun Yue, van IJzendoorn Sven C D
Department of Biomedical Sciences of Cells and Systems, Centre for Liver, Digestive and Metabolic Disease, University of Groningen, University Medical Centre Groningen, 9713 AV Groningen, The Netherlands.
Department of Thoracic Surgery, Guangdong Esophageal Cancer Institute, State Key Laboratory of Oncology in South China, Collaborative Innovation Centre for Cancer Medicine, Sun Yat-sen University Cancer Centre, Guangzhou 510060, China.
J Clin Med. 2021 Aug 31;10(17):3935. doi: 10.3390/jcm10173935.
Microvillus inclusion disease (MVID) is a rare enteropathy caused by mutations in the or gene. MVID is a disease that is difficult to manage with clinical heterogeneity. Therefore, knowledge about factors influencing MVID morbidity and mortality is urgently needed. Triggered by a recent study that reported a high percentage of preterm births in twelve cases of MVID, we have conducted a comprehensive retrospective study involving 88 cases of MVID with reported gestational ages. We found that moderate to late preterm birth occurred in more than half of all cases, and this was particularly prominent in -associated MVID. Preterm birth in MVID counterintuitively correlated with higher birth weight percentiles, and correlated with higher stool outputs and a significantly shorter average survival time. Data from this study thus demonstrate an increased risk of preterm birth in -associated MVID, with a clinical impact on morbidity and mortality. Adverse effects associated with preterm birth should be taken into account in the care of children diagnosed with MVID. Documentation of gestational age may contribute to a better prognostic risk assessment in MVID.
微绒毛包涵体病（MVID）是一种由 或 基因的突变引起的罕见肠病。MVID是一种临床异质性高、难以管理的疾病。因此，迫切需要了解影响MVID发病率和死亡率的因素。受最近一项研究的启发，该研究报告了12例MVID中有很高比例的早产病例，我们对88例报告了胎龄的MVID病例进行了全面的回顾性研究。我们发现，超过一半的病例发生了中度至晚期早产，这在与 相关的MVID中尤为突出。MVID中的早产与较高的出生体重百分位数呈反直觉的相关性，并且与较高的粪便排出量和显著缩短的平均生存时间相关。因此，本研究的数据表明，与 相关的MVID中早产风险增加，对发病率和死亡率有临床影响。在诊断为MVID的儿童护理中应考虑与早产相关的不良影响。记录胎龄可能有助于更好地评估MVID的预后风险。
