American Academy of Pediatrics Task Force on Pediatrics AIDS: Infants and children with acquired immunodeficiency syndrome: placement in adoption and foster care.
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A total of 87 cases of chronic intestinal pseudo-obstruction were identified as fitting the criteria for the syndrome. Five families, three with autosomal dominant inheritance, accounted for 15 cases. The remainder were single cases in families, indicating either autosomal recessive inheritance, spontaneous mutation, or acquired disease. Of the 87 patients, 47 were male; 19 patients were symptomatic at birth and 37 (43%) within the first month of life; 64% were diagnosed by the first year of age and the remainder were diagnosed by 18 years of age. Patients currently range in age from 3 months to 24 years. Abdominal distension in 70, vomiting in 50, and constipation in 50 of the 87 cases were the three commonest presenting symptoms. Diarrhea and failure to thrive were present in 20 cases. Urinary tract infection and failure to void were present in 10 cases. Diagnosis was established by clinical presentation, radiographic studies, and at exploratory laparotomy. Esophageal motility was abnormal in 14 patients. Anal manometric studies were done in 16 and showed normal recto-anal inhibitory reflex in all but one. Only 34 patients had biopsy studies as part of their evaluation and, of these, only 12 were full-thickness. Abnormal plexuses were found in eight and degeneration of smooth muscles in four. There was little or no benefit from the use of any medication to promote motility. One patient with intractable pseudo-obstruction benefited from a subtotal enterectomy. Of those patients not lost to follow-up, 31.4% died; 43% of these deaths occurred within the first 6 months from complications of total parenteral nutrition. One infant had a complete spontaneous remission by 1 year of age, with normalization of radiographic findings; another had partial remission by 6 months of age.
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This article reports on the diagnostic results of 232 patients who were examined in close collaboration by the paediatrician and the child psychiatrist at a Swiss children's hospital between 1971 and 1980. The main diagnosis was psychogenic disorders with mainly psychic symptoms in 53.9% of the cases, psychogenic disorders with mainly somatic symptoms in 37.1%, minimal brain damage in 11.2%, and disturbances of development in 8.6%. Psychosomatic symptoms as accessory findings were found in another 30%. Thus, functional somatic disorders were components of the polysymptomatic syndromes in more than 60% of the patients.
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