Linked Life Data

10570859

Source:http://linkedlifedata.com/resource/pubmed/id/10570859

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1999-12-2
pubmed:abstractText
Chronic idiopathic intestinal pseudo-obstruction (CIPS) is a rare condition in which there is a defective motility of the gastrointestinal tract of unknown cause leading to repeated bouts of intestinal obstruction without organic explanation. This syndrome groups several ill-defined varieties of motor disorders that can sometimes be classified according to the presence of familial incidence and to the presence of muscular or nervous lesions. Nevertheless, a considerable proportion of cases cannot be ascribed to either type. CIPS is a very difficult challenge for pediatric surgeons because our role is never curative and because when we are involved in it is usually as a result of a false diagnosis. We present herein the experience of 2 Pediatric Surgery Departments in this entity. In the last 30 years we have been involved in the management of 16 children with CIPS. Male-to-female ratio was 5:11 and all but 3 patients had symptoms before 6 months of life. Thirteen had abdominal distension, 10 maldevelopment, 9 recurrent bouts of intestinal obstruction, 8 chronic diarrhea, 7 vomiting, 2 dysphagia and 2 constipation. Seven out of the 16 had urinary tract involvement and in three prenatal diagnosis of megacysts was made. The mean delayed time for diagnosis was 3.08 years. Esophageal or antroduodenal manometry was performed in 8 patients and it was abnormal in 7. Histologic and histochemical samples were available in 8 patients, but only in 4 was enough to make a diagnosis of myopathy. Twelve patients underwent 41 surgical procedures. Three are currently included in a program of home parenteral nutrition. Only three have died, and the mean age of the survivors is 13.9 years. In most of the patients with CIPS surgery is only useful for nutritional purposes, for diversion procedures or for intestinal transplantation in extreme cases. Every effort should be made to avoid unnecessary explorations, misdiagnosis and delay in the identification of the syndrome.
pubmed:language
spa
pubmed:journal
pubmed:citationSubset
IM
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0214-1221
pubmed:author
pubmed:issnType
Print
pubmed:volume
12
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
71-4
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1999
pubmed:articleTitle
[Diagnosis and therapeutic options in chronic idiopathic intestinal pseudo-obstruction: review of 16 cases].
pubmed:affiliation
Departamento de Cirugía Pediátrica, Hospital Infantil La Paz, Madrid.
pubmed:publicationType
Journal Article, Comparative Study, English Abstract