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Varicella is a common childhood disease that can cause morbidity and mortality among immunosuppressed patients. There have been few previous studies monitoring the course of pediatric liver transplant patients with acute varicella. The aim of this study was to evaluate the treatment, outcomes, and complications of pediatric liver transplant patients admitted with acute varicella infection. A retrospective chart review was carried out based on discharge diagnoses of orthotopic liver transplant and varicella among pediatric patients (age range, birth–18 years) admitted to the UCLA Medical Center between 1985 and 2001. Five hundred fifty-six pediatric patients received liver transplantations between 1985 and 2001. Twenty-two of these patients were admitted to the UCLA Medical Center with varicella (11 females, 11 males). No patients were treated on an outpatient basis. Mean age of the patients was 6 years (range, 1–16 years). None of these patients received the varicella vaccine before hospitalization. On admission, 5 of 22 patients (23%) had received varicella zoster immunoglobulin within 96 hours of exposure. The mean length of hospitalization was 6 days (range, 2–11 days). All immunosuppression dosages were reduced during the admissions. None of the patients had been treated with high-dose corticosteroids for acute rejection before the onset of the varicella infection. Patients were treated until defervescence with intravenous acyclovir and until their varicella lesions crusted. Patients were discharged with oral acyclovir to complete a 10-day course (including the intravenous treatment). No patients had complications from the varicella infection. A complication of an elevated serum creatinine for one patient was noted with the intravenous acyclovir treatment. This patient had associated headache and nausea that resolved when the creatinine level returned to normal. There were no complications or dissemination of varicella infection among our pediatric liver transplant patients. Further prospective randomized trials are required to evaluate the management of pediatric liver transplant patients infected with varicella.

One hundred two pediatric patients received all or part of their nutritional needs parenterally at home during the past decade. All received total parenteral nutrition (TPN) at night during an 8-to 12-h infusion. Patients with short bowel syndrome (33), inflammatory bowel disease (23), chronic intractable diarrhea (15), chronic idiopathic intestinal pseudo-obstruction syndrome (10), and malignancy (10) made up the largest groups. The mean duration of parenteral support was 735 days (range, 90–3650 days); the mean number of catheters per patient was 2.1 (range, 1–8). Twenty-one patients continue to receive full or partial home TPN: four for more than 10 years and seven for more than 5 years. Fifty-one no longer require it and have had healing of mucosa or bowel adaptation. Complications related to administration of fluid and electrolytes were quite rare. Biotin deficiency was recognized once. Thirty-one have died, but only 13 deaths were related to TPN. Sepsis in nine and liver failure in two were the most common causes of death in the TPN-related group. Three of 21 still on home TPN have graduated either from high school or college. All but one of the school age children attend regular school; one attends a school for the medically disabled, another attends a school for the mentally gifted.

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 snowed 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.

Of a total of 187 consecutive liver grafts in 149 pediatric recipients, 59 episodes of steroid-resistant, biopsy-proven rejection (32 of grafts) were treated with OKT3 monoclonal antibody. After 59 OKT3-treated episodes, liver function at the end of treatment was normal in 40, improved in 35, and unchanged in 24. Of 21 partial responses, 12 episodes eventually resolved to yield an overall complete response rate of 59. CD3-positive T-cells >5 occurred during 61 of OKT3-treated rejection episodes and was associated with impaired efficacy of OKT3 (30 complete response rate). Six grafts were re-treated with OKT3 for rejection and in all CD3-positive T-cells could not be maintained <5. Of the six grafts requiring repeat OKT3, five failed-re-treatment and required retransplantation. OKT3 antibodies of low titer were found prior to a second use of OKT3 in 65 of episodes. Patients treated with OKT3 after failing more than two preceding steroid courses had a significantly increased chance of graft loss (57; p = 0.01). We conclude that this group of pediatric patients appeared less responsive to OKT3 compared to other series combining pediatric and adult recipients, possibly due to a more vigorous immune response in the child.