Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction
Clinical Outcomes of Pediatric Chronic Intestinal Pseudo-Obstruction
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DOI：
10.3390/jcm10112376
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发表时间：
2021-06-01
影响因子：
3.9
通讯作者：
Kim, Hyun-Young
中科院分区：
文献类型：
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作者：
Ko, Dayoung;Yang, Hee-Beom;Kim, Hyun-Young
慢性假性肠梗阻（CIPO）是一种非常罕见的疾病，其症状为复发性肠梗阻而无任何病变。小儿CIPO的结局和结局的预测因素尚未得到很好的确定。我们分析了儿童CIPO的临床结果和相关因素。我们回顾性分析了1985年1月至2017年12月诊断的66例原发性CIPO患者。我们评估肠外营养（PN）因素，如PN持续时间、超过6个月的PN使用、家庭PN和死亡率作为结果。我们选择发病年龄、泌尿系统症状、病理类型和受累程度作为预测因素。63.6%的患者出现早发性CIPO， 21.2%的患者出现泌尿系统症状。66例患者中，分别有47例神经病变和11例肌病。广义受累型占83.3%。在最后一次随访时，24.2%的患者需要家庭PN管理。PN的平均持续时间为11.8±21.0个月。原发性CIPO总死亡率为18.2%。泌尿系统症状和受累程度可以预测PN因子。死亡率以病理类型预测。发病年龄与预后无显著相关性。伴有泌尿系统症状的CIPO和广泛性CIPO的PN预后较差。肌病被认为是原发性CIPO患儿死亡率的预测因子。
Chronic intestinal pseudo-obstruction (CIPO) is an extremely rare condition with symptoms of recurrent intestinal obstruction without any lesions. The outcomes of pediatric CIPO and predictors for the outcomes have not yet been well established. We analyzed the clinical outcomes and associated factors for the outcomes of pediatric CIPO. We retrospectively reviewed 66 primary CIPO patients diagnosed between January 1985 and December 2017. We evaluated parenteral nutrition (PN) factors such as PN duration, PN use over 6 months, home PN, and mortality as outcomes. We selected onset age, presence of urologic symptoms, pathologic type, and involvement extent as predictors. The early-onset CIPO was found in 63.6%, and 21.2% of the patients presenting with urologic symptoms. Of the 66 patients, 47 and 11 had neuropathy and myopathy, respectively. The generalized involvement type accounted for 83.3% of the cases. At the last follow-up, 24.2% of the patients required home PN management. The mean duration of PN was 11.8 +/- 21.0 months. The overall mortality rate of primary CIPO was 18.2%. PN factors were predicted by the urologic symptoms and extent of involvement. However, mortality was predicted by pathologic type. The onset age was not significantly associated with the outcomes. CIPO with urologic symptoms and generalized CIPO had poor PN outcomes. Myopathy is suggested as a predictor of mortality in children with primary CIPO.