Hôpital Robert Debré, Paediatric Gastroenterology and Respiratory Department, CF Centre, University Paris 7, 75019 Paris, France.
Hôpital Robert Debré, Clinical Epidemiology Unit, 75019 Paris, France; Sorbonne Paris Cité, UMR-S1123, ECEVE, University Paris Diderot, 75019 Paris, France; Inserm U1123 and CIC-EC 1426, 75019 Paris, France.
J Cyst Fibros. 2016 Jul;15(4):531-9. doi: 10.1016/j.jcf.2016.02.002. Epub 2016 Feb 23.
Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis.
A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults.
102 patients presented 112 episodes. The incidence of DIOS was similar in children and adults. Medical treatment failed only in cases of complete DIOS (11%). Children with meconium ileus had a higher rate of surgery for DIOS (15% vs. 2%, p=0.02). Complete DIOS entailed longer hospitalisation (4 [3; 7] days vs. 3 [1; 4], p=0.002). Delayed arrival at hospital and prior weight loss had a significant impact on the time needed for DIOS resolution. Associated CF co-morbidities for DIOS included meconium ileus (40% vs. 18%, p<0.0001), exocrine pancreatic insufficiency (92% vs. 84%, p=0.03), liver disease (22% vs. 12%, p=0.004), diabetes mellitus (49% vs. 25%, p=0.0003), and Pseudomonas aeruginosa (68% vs. 52%, p=0.01); low fibre intake and insufficient hydration were frequently observed. Female gender was associated with recurrent DIOS (75% vs. 52%, p=0.04), constipation with incomplete episodes (39% vs. 11%, p=0.03), and poor patient compliance in taking pancreatic enzyme therapy during complete episodes (25% vs. 3%, p=0.02).
DIOS is a multifactorial condition having a similar incidence in children and adults. We show that delayed arrival at hospital after the initial symptoms causes significant morbidity. Early recognition and treatment would improve the prognosis.
远端肠梗阻综合征（DIOS）是囊性纤维化的一种特定并发症。
在 10 个国家进行了一项研究，前瞻性评估了儿童和成人的发病率、相关因素和治疗方式。
102 例患者出现 112 例次。DIOS 的发病率在儿童和成人中相似。仅在完全 DIOS（11%）的情况下，药物治疗才会失败。患有胎粪性肠梗阻的儿童因 DIOS 而接受手术的比例更高（15%比 2%，p=0.02）。完全 DIOS 导致住院时间延长（4 [3；7] 天比 3 [1；4]，p=0.002）。延迟就诊和体重减轻与 DIOS 缓解所需时间有显著影响。与 DIOS 相关的 CF 合并症包括胎粪性肠梗阻（40%比 18%，p<0.0001）、外分泌胰腺功能不全（92%比 84%，p=0.03）、肝病（22%比 12%，p=0.004）、糖尿病（49%比 25%，p=0.0003）和铜绿假单胞菌（68%比 52%，p=0.01）；经常观察到低纤维摄入和水分不足。女性与复发性 DIOS 相关（75%比 52%，p=0.04）、不完全发作时与便秘相关（39%比 11%，p=0.03）以及在完全发作时服用胰腺酶治疗的患者顺应性差（25%比 3%，p=0.02）。
DIOS 是一种多因素疾病，在儿童和成人中的发病率相似。我们表明，初始症状后延迟就诊会导致显著的发病率。早期识别和治疗将改善预后。
