Operational tolerance is not always permanent: A 10-year prospective study in pediatric liver transplantation recipients.

Immunosuppression withdrawal can be safely performed in select liver transplantation recipients, but the long-term outcomes and sustainability of tolerance have not been well studied. We completed a 10-year prospective, observational study of 18 pediatric liver transplantation recipients with operational tolerance to (1) assess the sustainability of tolerance over time, (2) compare the clinical characteristics of patients who maintained versus lost tolerance, (3) characterize liver histopathology findings in surveillance liver biopsies; and (4) describe immunologic markers in patients with tolerance. Comparator patients from two clinical phenotype groups termed "stable" and "nontolerant" patients were used as controls. Of the 18 patients with operational tolerance, the majority of patients were males (n = 14, 78%) who were transplanted for cholestatic liver disease (n = 12, 67%). Median age at transplantation was 1.9 (range, 0.6-8) years. Median time after transplantation that immunosuppression had been discontinued was 13.1 (range, 2.9-22.1) years. As many as 11 (61%) maintained tolerance for a median of 10.4 (range, 1.9-22.1) years, whereas 7 (39%) lost tolerance after a median of 3.2 (range, 1.5-18.6) years. Populations of T regulatory cells (%CD4+ CD25hi CD127lo ) were significantly higher in patients with tolerance (p = 0.02). Our results emphasize that spontaneous operational tolerance is a dynamic and nonpermanent state. It is therefore essential for patients who are clinically stable off immunosuppression to undergo regular follow-up and laboratory monitoring, as well as surveillance biopsies to rule out subclinical rejection.

Management of Biliary Stricture in Pediatric Liver Transplantation Patients: Long-Term Outcomes.

Postoperative biliary complications have been reported to occur in 10% to 33% of pediatric liver transplantation (LT) recipients. Percutaneous intervention has become the primary treatment method for these complications; however, the efficacy and outcomes of these patients have not been well studied. Institutional pediatric LT from 1998 to 2019 were retrospectively reviewed to determine the patients referred for percutaneous treatment of post-LT biliary strictures. Clinical parameters, percutaneous transhepatic cholangiograms (PTCs), biliary catheter placement, cholangioplasty, and long-term outcomes were analyzed. Of the 396 consecutive pediatric LT recipients during our study period, 50 (12.6%) were diagnosed with biliary strictures on PTC. LT biliary reconstructions were Roux-en-Y hepaticojejunostomy in 28 patients (56%), choledochojejunostomy in 11 patients (22%), and choledochocholedochostomy in 11 patients (22%). Median age at LT was 23.2 months (interquartile range [IQR], 10.9-90.6), and 14 patients (28%) developed hepatic artery thrombosis. A total of 44 patients (88%) were treated with internal/external biliary catheters, of whom 38 (76%) underwent balloon cholangioplasty. By 12 months, 84% of patients had complete stricture resolution and catheter removal. Median total duration of catheter drainage was 152 days (IQR, 76-308). A total of 8 patients required additional surgery (biliary reconstruction or repeat LT [re-LT]) or died with a drainage catheter in place from complications unrelated to PTC intervention. Among the 6 patients (12%) treated with unilateral external biliary drainage catheters, 2 had catheters removed for inadequate drainage but then had spontaneous biliary obstruction resolution, 1 underwent duct reconstruction, and 3 required long-term catheterization. Biliary strictures following pediatric LT can be successfully treated with internal/external biliary drainage catheters and cholangioplasty if the stricture can be crossed. However, patients with isolated strictured ducts may require long-term external catheter drainage until re-LT or percutaneous obliteration of isolated ducts.

Central venous catheter repair is highly successful in children with intestinal failure.

PURPOSE: Damaged central venous catheters (CVCs) are commonly repaired to avoid line replacement and preserve vascular access. However, limited data suggest an increased risk for central line-associated bloodstream infections (CLABSIs) associated with the repair procedure. The purpose of this study was to describe outcomes of CVC repairs among parenteral nutrition (PN) dependent children with intestinal failure (IF). METHODS: A 2-year retrospective review was performed on children with IF on home PN > 6 months. Outcomes of interest were repair success and postrepair CLABSI incidence. Descriptive statistics included medians and frequencies. RESULTS: A total of 36 pediatric IF patients underwent 96 CVC repairs during the study period. The median CVC repair count was 1.5 repairs/patient (range, 1 to 16 repairs/patient) with >1 repair in half the patients. Ninety-four broken catheters (98%) were successfully repaired with restoration of function. Of the unsuccessful repairs (2%), the two catheters eventually required surgical removal and replacement. One repair (1%) was followed by a CLABSI with Enterococcus faecalis in an immunocompromised patient. CONCLUSION: CVC repair is a highly successful procedure with a low risk for infection. Catheter repair should be considered whenever possible as it may extend the lifetime of the catheter and decrease the risk for vascular access loss. LEVEL OF EVIDENCE: Treatment study; level IV.

Epidemiology and Risk Factors for Outpatient-Acquired Catheter-Related Bloodstream Infections in Children Receiving Home Parenteral Nutrition.

BACKGROUND: Few studies have examined the epidemiology and risk factors for the development of outpatient-acquired catheter-related bloodstream infections (CRBSIs) in children receiving home parenteral nutrition. This study aimed to (1) characterize the incidence, clinical presentation, and epidemiology of CRBSIs and (2) identify risk factors for CRBSIs in children receiving home parenteral nutrition. METHODS: A longitudinal database approved by our Institutional Review Board was created to prospectively track CRBSIs in the UCLA pediatric population from January to December 2012. Eligible patients included those < 18 years old receiving home parenteral nutrition. RESULTS: Thirty of 60 patients (50%) were diagnosed with 66 CRBSIs, for an overall CRBSI rate of 3.6 per 1000 catheter days. Of the CRBSIs, 73% were due to single microorganisms and 27% were polymicrobial. There was a significant difference in median (range) time for blood cultures to turn positive depending on type of CRBSIs (p = 0.03), with polymicrobial infections detected at 13.4 (8.7-24.3) hours, gram-negative infections at 16.5 (9-30.8) hours, and gram-positive infections at 18.9 (8.4-37.1) hours. The most common presenting symptom was fever (82%), followed by gastrointestinal symptoms (42%) and chills (29%). The only significant multivariate risk factor for CRBSIs was presence of a feeding tube (2.3-fold increase in CRBSI risk, p = 0.04). DISCUSSION: Outpatient-acquired CRBSIs are common in children receiving home parenteral nutrition. CRBSIs typically present with fever, but are also associated with gastrointestinal and/or respiratory symptoms. The presence of feeding tubes may predispose children on home parenteral nutrition to developing CRBSIs.

Obliterative portal venopathy: A histopathologic finding associated with chronic antibody-mediated rejection in pediatric liver allografts.

The significance of post-transplant HLA DSA and chronic AMR in LT is an emerging field of study. Although OPV has previously been described as a histopathologic finding in DSA-positive adult LT recipients, it was not included in the recent Banff criteria for chronic AMR. Our aim was to describe the association between OPV and chronic AMR in pediatric LT recipients. A retrospective review of 67 liver biopsies performed between November 2014 and April 2016 in 45 pediatric LT recipients identified four patients with OPV. Clinical status, liver biochemistry, the presence of DSA, and available non-HLA antibody testing, as well as histopathologic features of chronic AMR, were assessed. All four patients with OPV had class II DSA and histopathologic features of chronic AMR based on the Banff criteria. Two patients were noted to have non-HLA antibodies. Three patients are undergoing treatment with IVIG but have persistent DSA. Two patients have graft failure and are awaiting retransplantation. In conclusion, OPV is a histopathologic finding associated with chronic AMR in pediatric LT recipients. Further studies are needed to elucidate whether OPV is reversible and/or amenable to medical therapy.

Nutrition Deficiencies in Children With Intestinal Failure Receiving Chronic Parenteral Nutrition.

BACKGROUND: Home parenteral nutrition (PN) is a lifesaving therapy for children with intestinal failure (IF). Our aims were to describe the prevalence of micronutrient deficiencies (vitamin D, zinc, copper, iron, selenium) in a diverse population of children with IF receiving PN and to identify and characterize risk factors associated with micronutrient deficiencies, including hematologic abnormalities. METHODS: Data were collected on 60 eligible patients through retrospective chart review between May 2012 and February 2015. Descriptive statistics included frequencies, medians, interquartile ranges (IQRs), and odds ratios (ORs). Statistical analyses included χ2 , Fisher's exact, t tests, and logistic, univariate, and multivariate regressions. RESULTS: Patients were primarily young (median age, 3.3 years; IQR, 0.7-8.4), Latino (62%), and male (56%), with short bowel syndrome (70%). Of 60 study patients, 88% had ≥1 deficiency and 90% were anemic for age. Of 51 patients who had all 5 markers checked, 59% had multiple deficiencies (defined as ≥3). Multivariate analysis shows multiple deficiencies were associated with nonwhite race (OR, 9.4; P = .012) and higher body mass index z score (OR, 2.2; P = .016). Children with severe anemia (hemoglobin <8.5 g/dL) made up 50% of the cohort. Nonwhite race (OR, 6.6; P = .037) and zinc deficiency (OR, 11; P = .003) were multivariate predictors of severe anemia. CONCLUSIONS: Micronutrient deficiency and anemia are overwhelmingly prevalent in children with IF using chronic PN. This emphasizes the importance of universal surveillance and supplementation to potentially improve quality of life and developmental outcomes. Future research should investigate how racial disparities might contribute to nutrition outcomes for children using chronic PN.

Acute antibody-mediated rejection in ABO-compatible pediatric liver transplant recipients: case series and review of the literature.

Acute AMR is well reported following ABO-incompatible LTx. However, it remains uncommon in ABO-compatible LTx. It typically presents with graft dysfunction ≤2 weeks post-LTx and is often associated with graft loss. We report the clinical presentation, treatment regimen, and outcome of six pediatric LTx recipients diagnosed with early acute AMR based on (i) clinical signs of graft dysfunction, (ii) histopathology indicative of acute injury ± C4d staining, and (iii) presence of HLA DSA. All patients developed elevated ALT and GGT ≤ 45 days post-LTx. All showed HLA class I (n=4) and/or II (n=6) DSA (peak MFI 6153-11 910). Four had de novo DSA, and two had preformed DSA. Five were initially diagnosed with ACR refractory to steroid therapy. Four exhibited resolution of graft dysfunction with AMR therapy. Two had refractory AMR-one was re-transplanted; the other was treated with eculizumab and showed improvement in graft function but later died due to a tracheostomy complication. Our case series suggests that AMR following ABO-compatible LTx may be under-diagnosed. The presentation can be variable, and treatment should be individualized. Eculizumab may be an option for refractory AMR. Ultimately, future multicenter studies are needed to better define diagnostic criteria, characterize optimal treatment, and assess long-term outcomes following liver AMR.

Donor-specific HLA Antibodies Are Associated With Late Allograft Dysfunction After Pediatric Liver Transplantation.

BACKGROUND: The role of donor-specific HLA antibodies (DSA) after pediatric liver transplantation (LTx) is not clearly established. We completed a cross-sectional study to characterize DSA in long-term survivors of pediatric LTx and assess the impact of C1q-binding DSA on allograft outcomes. METHODS: Serum samples were collected at routine clinic visits from 50 pediatric LTx recipients classified into 3 clinical phenotypes: nontolerant (n = 18) with de novo autoimmune hepatitis (DAIH) and/or late acute cellular rejection (ACR); stable (n = 25) on maintenance tacrolimus; operationally tolerant (n = 7). Samples were blinded, and antibody detection was performed using Luminex single antigen class I and II beads. Patients with positive DSA were tested for C1q-binding DSA. RESULTS: DSA were detected in 54% (n = 27) of the patients, with the majority directed at HLA class II antigens (DR, 41%; DQ, 53%). Patients with DSA were younger at the time of LTx (P = 0.016) and time of study (P = 0.024). Mean aspartate aminotransferase, alanine aminotransferase, total bilirubin, and gamma glutamyl transferase were higher in DSA-positive patients, though did not reach statistical significance. Nontolerant patients were significantly more likely to have DQ DSA (61%) compared to stable (20%) and tolerant (29%) patients (P = 0.021). The nontolerant phenotype was associated with DSA and C1q-binding DSA, with odds ratios of 13 (P = 0.015) and 8.6 (P = 0.006), respectively. The presence of DQ DSA was associated with DAIH and late ACR, with odds ratios of 12.5 (P = 0.004) and 10.8 (P = 0.006), respectively. CONCLUSIONS: Allograft dysfunction is not always evident in patients with DSA, but DQ DSA are strongly associated with DAIH, late ACR, and chronic rejection.

Endoscopy Following Pediatric Intestinal Transplant.

OBJECTIVES: Biopsies remain the criterion standard in the diagnosis of intestinal transplant (ITx) rejection, and gastrointestinal endoscopy plays a pivotal role in patient management. Herein, we describe a single-center 23-year endoscopic experience in pediatric ITx recipients. METHODS: A retrospective review of endoscopy and pathology reports of all ITx recipients <18 years old transplanted between 1991 and 2013 was performed with the aim of describing the procedural indications, findings, and complications. RESULTS: A total of 1770 endoscopic procedures within 1014 sessions were performed. A combination of esophagogastroduodenoscopy and ileoscopy was the most common procedure (36%). Increased stool output (35%) and surveillance endoscopy (32%) were the most common indications. A total of 162 episodes of biopsy-proven rejection were diagnosed. The first episode of rejection occurred at a median of 1 month after ITx. Of histology-proven rejections, 45% had normal-appearing endoscopies. The rate of procedural complications, including but not limited to bleeding and perforation, was 1.8%. CONCLUSIONS: Endoscopy with biopsy plays a significant role in the care of ITx recipients. Multiple procedures are required for graft surveillance, diagnosis of rejection, subsequent treatment, and follow-up of therapy. The gross endoscopic appearance, particularly in mild to moderate acute cellular rejection, does not correlate well with histology. Complex anatomy, complication rates that are higher than patients with non-ITx pediatric endoscopy, and timely histologic interpretation by experienced pathologists are reasons that these procedures should be performed at centers accustomed to caring for ITx recipients. The field would benefit from the development of a noninvasive biomarker to reliably and efficiently detect rejection.

Vitamin D deficiency in children with intestinal failure receiving home parenteral nutrition.

BACKGROUND: Vitamin D plays important roles in both skeletal and nonskeletal health. Limited data suggest that patients with intestinal failure (IF) receiving home parenteral nutrition (PN) are at risk for vitamin D deficiency due to inadequate oral intake, poor absorption, and chronic illness. The purpose of this study was to document vitamin D status in pediatric patients with IF receiving home PN. MATERIALS AND METHODS: We performed a 2-year retrospective review of children with IF followed at our center who had been on home PN for ≥6 months and had ≥1 serum 25-hydroxyvitamin D (25-OHD) level checked as part of routine clinical care. Patients were then categorized as deficient (<20 ng/mL), insufficient (20-29 ng/mL), or normal (≥30 ng/mL) based on their lowest vitamin D level. Demographic data and clinical characteristics were also assessed. RESULTS: Eleven of 27 children (41%) had ≥1 insufficient 25-OHD level, including one child with vitamin D deficiency. Diagnosis of short bowel syndrome (compared with dysmotility or malabsorption syndromes) was associated with decreased likelihood of suboptimal vitamin D status, with an odds ratio of 0.12 (95% confidence interval, 0.02-0.8, P = .028). Osteopenia was noted in 59% of the cohort. There was a trend toward higher risk for osteopenia in patients with low 25-OHD levels compared with those with normal 25-OHD levels (82% vs 44%, P = .109). CONCLUSION: Suboptimal 25-OHD levels are common in children with IF on home PN. This emphasizes the critical importance of routine surveillance of serum vitamin D levels and consideration of enteral supplementation when indicated.

Postinfectious gastroparesis: a case series of three adolescent females.

Postinfectious gastroparesis (PIGP) is a subgroup of idiopathic gastroparesis rarely reported in adolescents. This study describes 3 adolescent females with severe PIGP, who each underwent extensive workup prior to referral to a pediatric gastroenterologist. PIGP may be an underrecognized disorder in pediatrics, particularly in adolescents, and if untreated, can lead to significant morbidity.

Long-term nutrition and predictors of growth and weight gain following pediatric intestinal transplantation.

BACKGROUND: Advances in intestinal transplantation (ITx) have resulted in improved survival and the opportunity to examine nutritional outcomes. The aim of this study was to describe detailed, long-term nutritional results and identify positive predictors of growth and weight gain following pediatric ITx. METHODS: A single-center retrospective, Institutional Review Board-approved review of a prospective database was conducted. Inclusion criteria were ITx recipients 18 years or younger with survival of 6 months or more. Outcomes included anthropometric measurements and biochemical markers at 6, 12, 24, 36, and 48 months post-ITx. More than 25 ITx-related variables were analyzed as potential predictors of growth and weight gain. Statistical analysis was performed using chi-square test, t test, and analysis of variance. RESULTS: Between November 1991 and April 2007, 50 children received 55 ITx; 33 patients met eligibility criteria. Median age at ITx was 2.2 years, follow-up time was 3.8 years, and time from ITx to cessation of total parenteral nutrition was 31 days. The most common micronutrient deficiencies post-ITx were zinc, iron, and copper. Serum protein levels improved significantly over time. Weight gain occurred within 6 months and vertical growth within 12 months, although limited catch-up growth was seen. Early predictors of weight gain and growth included shorter hospitalization and absence of rejection. Long-term predictors were low steroid dosage, infrequent hospitalization, and the use of peptide-based formulas. CONCLUSIONS: This represents one of the largest and most comprehensive long-term studies on nutritional outcomes in pediatric ITx. Overall, positive growth and weight gain were seen as were micronutrient deficiencies. Numerous long-term nutritional challenges exist which require a multidisciplinary approach and future prospective studies.

Predictors of survival following liver transplantation in infants: a single-center analysis of more than 200 cases.

BACKGROUND: Infants (<12 months) who require liver transplantation (LTx) represent a particularly challenging and understudied group of patients. METHODS: This retrospective study aimed to describe a large single-center experience of infants who received isolated LTx, illustrate important differences in infants versus older children, and identify pretransplant factors which influence survival. More than 25 pre-LTx demographic, laboratory, and operative variables were analyzed using the Log-rank test and Cox proportional hazards model. RESULTS: Between 1984 and 2006, 216 LTx were performed in 186 infants with a mean follow-up time of 62 months. Median age at LTx was 9 months, the majority had cholestatic liver disease, were hospitalized pre-LTx, and received whole grafts. Leading indications for re-LTx (n=30) included vascular complications (43%) and graft nonfunction (40%), whereas leading causes of death were sepsis and multiorgan failure. One-, 5-, and 10-year graft and patient survivals were 75%/72%/68% and 79%/77%/75%, respectively. Relative to older pediatric recipients, infants had worse overall patient survival (P=0.05). The following were significant univariate predictors of graft loss: age less than 6 months and reduced cadaveric grafts; and of patient loss: age less than 6 months, calculated CrCl less than 90, pre-LTx hospitalization, pre-LTx mechanical ventilation, repeat LTx, infants transplanted for reasons other than cholestatic liver disease, and patients transplanted between 1984 and 1994. CONCLUSIONS: Long-term outcomes for infants undergoing LTx are excellent and have improved over time. As the largest, single-center analysis of LTx in infants, this study elucidates a unique set of predictors that can aid in medical decision making.

Isoniazid-related hepatic failure in children: a survey of liver transplantation centers.

BACKGROUND: Isoniazid (INH) therapy for tuberculosis carries a known risk for hepatoxicity, and leads to hepatic failure in a small subset of patients. This incidence has been described for adults, but is uncertain in children. Our aim was to estimate the incidence of pediatric referrals for INH-related liver failure, and to describe the characteristics and outcomes of these patients. METHODS: The 84 U.S. centers performing pediatric liver transplants between 1987 and 1997 were surveyed regarding patients with INH-induced liver failure. Additional transplant statistics were obtained from the United Network for Organ Sharing. Estimates of the number of children taking preventive INH were derived from a nationwide public health database. RESULTS: Twenty cases of INH-related liver failure were found during a 10-year period. Four patients (20%) recovered spontaneously; 10 (50%) underwent orthotopic liver transplantation (OLT), while six (30%) died awaiting OLT. Mean age at presentation was 9.8 years (range 1.3-17). Mean length of INH therapy was 3.3 months (range 0.5-9). Notably, five patients seen for symptoms of hepatitis were initially told not to stop treatment. INH-associated liver failure accounted for 0.2% (8 of 4679) of all pediatric OLTs, and 14% (8/56) of transplants for drug hepatoxicity. The estimated incidence of liver failure was up to 3.2/100,000 for children on prophylactic INH. CONCLUSIONS: While INH-associated liver failure in children is rare, discontinuation at the onset of symptoms does not assure recovery. This indicates a need for increased awareness of hepatotoxicity risk, expanded biochemical monitoring for children receiving INH, and prompt withdrawal in symptomatic patients.

Mutant neurogenin-3 in congenital malabsorptive diarrhea.

BACKGROUND: Neurogenin-3 (NEUROG3) is expressed in endocrine progenitor cells and is required for endocrine-cell development in the pancreas and intestine. The NEUROG3 gene (NEUROG3) is therefore a candidate for the cause of a newly discovered autosomal recessive disorder characterized by generalized malabsorption and a paucity of enteroendocrine cells. METHODS: We screened genomic DNA from three unrelated patients with sparse enteroendocrine cells for mutations of NEUROG3. We then tested the ability of the observed mutations to alter NEUROG3 function, using in vitro and in vivo assays. RESULTS: The patients had few intestinal enteroendocrine cells positive for chromogranin A, but they had normal numbers of Paneth's, goblet, and absorptive cells. We identified two homozygous mutations in NEUROG3, both of which rendered the NEUROG3 protein unable to activate NEUROD1, a downstream target of NEUROG3, and compromised the ability of NEUROG3 to bind to an E-box element in the NEUROD1 promoter. The injection of wild-type but not mutant NEUROG3 messenger RNA into xenopus embryos induced NEUROD1 expression. CONCLUSIONS: A newly discovered disorder characterized by malabsorptive diarrhea and a lack of intestinal enteroendocrine cells is caused by loss-of-function mutations in NEUROG3.

Resolution of hypophosphatemia is associated with recovery of hepatic function in children with fulminant hepatic failure.

Fulminant hepatic failure (FHF) is a rare but often fatal disease in children. Clinical and laboratory predictors of liver regeneration and recovery, however, have not been well established. We hypothesized that hypophosphatemia may indicate recovery of liver synthetic function in children with FHF. We retrospectively reviewed the medical records of children with FHF who were admitted to UCLA and recovered hepatic function either spontaneously or by liver transplantation (LTx). Serum phosphate (Ph) and prothrombin time or international normalized ratio (INR) were compared over the patient's clinical course. Records of 39 children who spontaneously recovered experienced profound hypophosphatemia that resolved as liver synthetic function improved. Similar patterns were seen in the 84 children who recovered after LTx. We found that hypophosphatemia precedes the recovery of liver synthetic function in children with FHF who recovered with or without transplantation, and that Ph levels return to normal as liver synthetic function improves. These data suggest that hypophosphatemia may be a useful laboratory indicator of recovering liver function in children with FHF.

Long-term parenteral nutritional support and intestinal adaptation in children with short bowel syndrome: a 25-year experience.

OBJECTIVE: To analyze the outcome of children with short bowel syndrome (SBS) who required long-term parenteral nutrition (PN). STUDY DESIGN: Retrospective analysis of children (n=78) with SBS who required PN >3 months from 1975 to 2000. STATISTICS: univariate analysis, Kaplan-Meier method, and Cox proportional regression model were used. RESULTS: We identified 78 patients. Survival was better with small bowel length (SBL) >38 cm, intact ileocecal valve (ICV), intact colon, takedown surgery after ostomy (all P <.01), and primary anastomosis (P <.001). PN-associated early persistent cholestatic jaundice (P <.001) and SBL of <15 cm (P <.01) were associated with a higher mortality. Intestinal adaptation was less likely if SBL <15 cm (P <.05), ICV was removed, colonic resection was done (both P <.001), >50% of colon was resected (P <.05), and primary anastomosis could not be accomplished (P <.01). Survival was 73% (57), and 77% (44) of survivors had intestinal adaptation. CONCLUSIONS: SBL, intact ICV, intestinal continuity, and preservation of the colon are important factors for survival and adaptation. Adaptation usually occurred within the first 3 years. Need for long-term PN does not preclude achieving productive adulthood. Patients with ICV even with <15 cm of SBL and patients with SBL >15 cm without ICV have a chance of intestinal adaptation.

Varicella in pediatric liver transplant patients: a retrospective analysis of treatment and outcome.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.