Meconium ileus equivalent (MIE) complicating cystic fibrosis of the pancreas (CF) increases in frequently with increasing age of patients. In the present paper the course of 11 children and adolescents with MIE diagnosed and treated at the University Dept. of Paediatrics in Zürich during the last 15 years, i.e. 9% of 120 CF patients, is analyzed. 9 were successfully managed by medical treatment alone, 1 three months old infant was treated surgically with no later relapse and a 26 years old patient with a chronic deleterious course leading to opiate dependency could be relieved only by ileostomy. 5 patients experienced only 1 episode of MIE, 2- two to three and in 4 a chronic refractory course with multiple episodes was probably due to an inadequate medical management. The evaluation of the events in our patients and of the available data from the literature allows the following conclusions: MIE is a preventable condition in CF patients; a rigorous medical treatment of exocrine pancreatic insufficiency supplemented with mucolytic agents orally can usually prevent MIE and relieve an established MIE; surgical treatments is indicated only in desperate situations.

Adolescent↗

PubMedDate not supplied

Childhood celiac disease: a long-term analysis of relapses in 91 patients.

The diagnosis of celiac disease (CD) was uniformly established between 1960 and 1983 in 314 infants and children according to the following criteria: a flat proximal small bowel mucosa in untreated patients and an unequivocal response and remission on a gluten-free diet (GFD). In 91 patients, one or more interruptions of the GFD (challenges) were documented by repeated intestinal biopsies. Sixty-eight percent of the challenges were initiated by the patients themselves. Seventy-one patients (Group A, 81.4%) had a flat mucosa after 0.25-14.67 years off diet. In 11 (Group B, 12%), a successive deterioration of the mucosa occurred during 0.5-6.67 years after interruption of GFD without becoming flat. Six patients (Group C, 6.6%) had a normal intestinal mucosa after 2.24-6.92 years off diet. Three patients are still under study, being off diet for less than 2 years. Median age at diagnosis was similar in all three groups (1.16, 1.16, and 1.12 years). Median duration of GFD prior to challenge was 4.54 years in Group A, 12.16 years in Group B (p less than 0.001), and 6.38 years in Group C. A mucosal relapse within 2 years after the beginning of challenge was observed in 21 of 24 patients (87.5%) studied longitudinally during planned challenges. It is concluded that only a minority (6.6%) of CD patients, when diagnosed as above, will not relapse after an interruption of GFD and that another small group (12%) will deteriorate very slowly. Routine gluten challenges in all CD patients, interrupting established life and dietary habits and including repeated biopsies in the majority of patients who deteriorate and relapse, seem therefore not justifiable.

Adolescent↗