Intestinal obstruction syndromes in cystic fibrosis: Meconium ileus, distal intestinal obstruction syndrome, and constipation

被引：80
作者：
Van Der Doef H.P.J. [1 ]
Kokke F.T.M. [1 ]
Van Der Ent C.K. [2 ]
Houwen R.H.J. [1 ]
机构：
[1] Department of Pediatric Gastroenterology (KE.04.133.1), University Medical Center Utrecht, 3508 AB Utrecht
[2] Department of Pediatric Pulmonology (KH 01.419.0), University Medical Center Utrecht, 3508 AB Utrecht
来源：
Current Gastroenterology Reports | 2011年 / 13卷 / 3期
关键词：
Abdominal radiography; Anthropometric variables; Body mass index; Coefficient of fat absorption; Constipation; Cystic fibrosis; Cystic fibrosis transmembrane regulator; Distal intestinal obstruction syndrome; Fat malabsorption; Height; Meconium ileus; Meconium ileus equivalent; Modifier genes; Nutritional status; Pulmonary function; Spirometry; Steatorrhea; Survival; Weight;
D O I：
10.1007/s11894-011-0185-9
中图分类号：
学科分类号：
摘要：
Meconium ileus at birth, distal intestinal obstruction syndrome (DIOS), and constipation are an interrelated group of intestinal obstruction syndromes with a variable severity of obstruction that occurs in cystic fibrosis patients. Long-term follow-up studies show that today meconium ileus is not a risk factor for impaired nutritional status, pulmonary function, or survival. DIOS and constipation are frequently seen in cystic fibrosis patients, especially later in life; genetic, dietary, and other associations have been explored. Diagnosis of DIOS is based on suggestive symptoms, with a right lower quadrant mass confirmed on abdominal radiography, whereas symptoms of constipation are milder and of longer standing. In DIOS, early aggressive laxative treatment with oral laxatives (polyethylene glycol) or intestinal lavage with balanced osmotic electrolyte solution and rehydration is required, which now makes the need for surgical interventions rare. Constipation can generally be well controlled with polyethylene glycol maintenance treatment. © 2011 The Author(s).
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页码：265 / 270
页数：5
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共 47 条
  • [1] Slieker M.G., Uiterwaal C.S.P.M., Sinaasappel M., Heijerman H.G.M., Van Laag J.D., Van Der E.C.K., Birth prevalence and survival in cystic fibrosis: A national cohort study in the Netherlands, Chest, 128, 4, pp. 2309-2315, (2005)
  • [2] Bali A., Stableforth D.E., Asquith P., Prolonged small-intestinal transit time in cystic fibrosis, British Medical Journal, 287, 6398, pp. 1011-1013, (1983)
  • [3] Escobar H., Perdomo M., Vasconez F., Et al., Intestinal permeability to 51Cr-EDTA and orocecal transit time in cystic fibrosis, J Pediatr Gastroenterol Nutr, 14, pp. 204-207, (1992)
  • [4] Sinaasappel M., Relationship between intestinal function and chloride secretion in patients with cystic fibrosis, Neth J Med, 41, pp. 110-114, (1992)
  • [5] Mall M., Kreda S.M., Mengos A., Jensen T.J., Hirtz S., Seydewitz H.H., Yankaskas J., Kunzelmann K., Riordan J.R., Boucher R.C., The DeltaF508 Mutation Results in Loss of CFTR Function and Mature Protein in Native Human Colon, Gastroenterology, 126, 1 SUPPL. 1, pp. 32-41, (2004)
  • [6] Van Der Doef H.P., Kokke F.T., Beek F.J., Et al., Constipation in pediatric cystic fibrosis patients: An underestimated medical condition, J Cyst Fibros, 9, pp. 59-63, (2010)
  • [7] Kerem E., Corey M., Kerem B., Durie P., Tsui L.-C., Levison H., Clinical and genetic comparisons of patients with cystic fibrosis, with or without meconium ileus, Journal of Pediatrics, 114, 5, pp. 767-773, (1989)
  • [8] Kappler M., Feilcke M., Schroter C., Et al., Long-term pulmonary outcome after meconium ileus in cystic fibrosis, Pediatr Pulmonol, 44, pp. 1201-1206, (2009)
  • [9] Van Der Doef H.P., Slieker M.G., Staab D., Et al., Association of the CLCA1 p.S357N variant with meconium ileus in European patients with cystic fibrosis, J Pediatr Gastroenterol Nutr, 50, pp. 347-349, (2010)
  • [10] Blackman S.M., Deering-Brose R., McWilliams R., Naughton K., Coleman B., Lai T., Algire M., Beck S., Hoover-Fong J., Hamosh A., Fallin M.D., West K., Arking D.E., Chakravarti A., Cutler D.J., Cutting G.R., Relative Contribution of Genetic and Nongenetic Modifiers to Intestinal Obstruction in Cystic Fibrosis, Gastroenterology, 131, 4, pp. 1030-1039, (2006)
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