Thymoglobuline use in pediatric heart transplantation.

The literature has few data regarding the use of polyclonal anti-thymocyte globulin in pediatric cardiac transplantation. We describe our single-center, retrospective study of the use of Thymoglobuline in a pediatric population. We included in the study 31 consecutive heart transplant recipients (mean age, 7.8 years; median age, 9 years; range, 4 months-17 years), who all survived surgery. To induce immunosuppression, all patients received Thymoglobuline therapy at age-dependent doses (1-1.5 mg/kg/day between 0 and 1 year; 1.5-2 mg/kg/day from 1 year to 8 years; and 2.5 mg/kg/day >8 years). Duration of treatment was 1 to 7 days. In patients <1 year, the total number of lymphocytes was maintained at >500/mm(3). Thirty of 31 patients are alive at the end of follow-up. During the first 3 months, 3 Grade 3A and 10 Grade 1A (Working Formulation grading system) rejection episodes occurred. All reversed after steroid treatment. Eleven viral infections, 2 bacterial infections, and 1 fungal infection occurred. Not all patients with infection were symptomatic but all responded successfully to treatment. One episode of post-transplantation lymphoproliferative disease regressed after decreasing immunosuppression therapy and after acyclovir therapy. At the end of follow-up, 19 patients are without steroids. Immunosuppression therapy with Thymoglobuline is safe in the pediatric age group if the number of lymphocytes is monitored strictly.

Usefulness of endoscopic small intestinal biopsies in children with coeliac disease.

Small intestinal biopsy is the most important diagnostic method in the routine evaluation of children with chronic diarrhoea and malabsorption. At present morphological alterations are considered essential in the diagnosis of coeliac disease (CD) and the presence of a normal small bowel biopsy specimen, observed in patients eating a diet containing gluten, rules out the diagnosis of CD. The small intestinal biopsy can be carried out either by blind suction capsule or by endoscopic forceps. In everyday clinical practice endoscopic duodenal biopsies, if taken and handled suitably, are accepted as equivalent to capsule biopsies from the proximal jejunum. In the study we reported some patients in whom has been possible to demonstrate the presence of total villous atrophy in one biopsy, while other duodenal samples taken in different duodenal portions were normal or showed mild lymphocytes and plasmacells infiltrations of the lamina propria. In patients with this type of biopsy pathology, wherein flat mucosa has been found even close to normal mucosa, the possible explanation is mucosal patchiness. The occurrence of patchly distributed intestinal atrophy in children suffering of CD raises the question of the validity of using the peroral capsule, widely believed to be the best standard for the diagnosis of CD. In our opinion, small intestinal biopsies obtained via endoscopy are more reliable than the peroral capsule biopsies in order to identify patchy mucosal atrophy and could be very useful for a correct diagnosis in CD patients.

Prevalence and clinical picture of celiac disease in italian down syndrome patients: a multicenter study.

BACKGROUND: A multicenter research study of Down syndrome patients was carried out to estimate the prevalence of celiac disease in patients with Down syndrome and to show clinical characteristics and laboratory data of Down syndrome patients. METHODS: The authors studied 1,202 Down syndrome patients. Fifty-five celiac disease patients (group 1) were compared with 55 immunoglobulin A antigliadin-positive antiendomysium antibodies-negative patients (group 2) and with 57 immunoglobulin A antigliadin-negative antiendomysium antibodies-negative patients (group 3). RESULTS: Celiac disease was diagnosed in 55 of 1,202 Down syndrome patients (4.6%). In group 1, weight and height percentiles were shifted to the left, whereas these parameters were normally distributed in groups 2 and 3. In celiac patients, diarrhea, vomiting, failure to thrive, anorexia, constipation, and abdominal distension were higher than in the other two groups. Low levels of hemoglobinemia, serum iron, and calcium were observed more frequently in group 1. The diagnosis of celiac disease was made after a mean period of 3.8 years from the initiation of symptoms. Sixty-nine percent of patients showed a classic presentation, 11% had atypical symptoms, and 20% had silent celiac disease. Autoimmune disorders were more frequent (30.9%) in group 1 than in the other two groups examined (15%; P < 0.05). CONCLUSIONS: This study reconfirms a high prevalence of celiac disease in Down syndrome. However, the diagnostic delay, the detection of atypical symptoms or silent form in one third of the cases, and the increased incidence of autoimmune disorders suggest the need for the screening of celiac disease in all Down syndrome patients.