ABSTRACT
We report the outcomes of an adult and pediatric split liver transplant from an adult male donor who died due to an unrecognized UCD, OTC deficiency. Recognizing inborn errors of metabolism can be challenging, especially in adult centers where such disorders are rarely encountered. Shortage of donors for liver transplantation has led to procedures to maximize donor utilization, such as split and live donor grafts. The cause of death should be ascertained before accepting a cadaveric donor organ.
Subject(s)
Liver Transplantation , Ornithine Carbamoyltransferase Deficiency Disease/diagnosis , Postoperative Complications/diagnosis , Adult , Child , Fatal Outcome , Female , Humans , Liver Transplantation/methods , Male , Ornithine Carbamoyltransferase Deficiency Disease/etiologyABSTRACT
Mitochondrial respiratory chain (RC) abnormalities in children can present as multiorgan disease, including liver failure, usually within the first year of life. Cardiorespiratory complications have previously been described in association with RC defects; however, to our knowledge no cases of pulmonary hypertension have been described. We discuss two patients with proven mitochondrial RC liver disease who developed severe pulmonary hypertension, one subsequent to cadaveric orthotopic liver transplantation, the second in the neonatal period. It is our contention that pulmonary hypertension should now be included as another potential manifestation of paediatric mitochondrial disease.
Subject(s)
Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/pathology , Mitochondrial Diseases/diagnosis , Adenosine Triphosphate/metabolism , Electron Transport , Fatal Outcome , Fibroblasts/metabolism , Humans , Hypertension, Pulmonary/mortality , Infant , Infant, Newborn , Liver/metabolism , Liver Diseases/metabolism , Liver Failure , Liver Transplantation , Male , Mitochondria/metabolism , Mitochondrial Diseases/mortality , Mitochondrial Diseases/pathology , Skin/metabolism , Time FactorsABSTRACT
OBJECTIVES: Differentiation of Crohn's disease (CD) from ulcerative colitis (UC) is problematic, primarily when inflammation is confined to the colon. In a historical cohort study, we evaluated the usefulness of baseline gastric antral biopsies in the differentiation of pediatric chronic colitides. METHODS: During initial investigation for suspected inflammatory bowel disease, 39 children and adolescents with colitis but normal small bowel radiography underwent pretreatment upper endoscopy concurrently with colonoscopy. Two reviewers assigned a colonoscopic diagnosis (colonic CD, UC, or indeterminate colitis) based on the macroscopic and microscopic appearances of the colonic mucosa. Antral histological findings were compared between groups using Fisher's exact test. RESULTS: Five (14%) of colonoscopic diagnoses (four indeterminate, one UC) were changed to CD by the finding of granulomatous inflammation in antral biopsies. Nonspecific antral gastritis was found in similar proportions of children and adolescents with Crohn's colitis and UC (92% vs 75%). Focal antral gastritis was more common in patients with Crohn's colitis than UC (52% vs 8%). CONCLUSIONS: Nonspecific antral gastritis is common in all forms of chronic colitis. Nevertheless, upper gastrointestinal endoscopy with biopsy is useful in the differentiation of inflammatory bowel disease confined to the colon, particularly when colonoscopic findings are indeterminate.
Subject(s)
Colitis/pathology , Pyloric Antrum/pathology , Adolescent , Biopsy , Child , Diagnosis, Differential , Female , Granuloma , Humans , Inflammation , MaleSubject(s)
Hydrops Fetalis/diagnostic imaging , Intestinal Diseases/diagnosis , Lymphatic Diseases/diagnosis , Protein-Losing Enteropathies/diagnosis , Adult , Diagnosis, Differential , Female , Humans , Hydrops Fetalis/diagnosis , Infant, Newborn , Intestinal Diseases/pathology , Lymphatic Diseases/pathology , Lymphatic System/abnormalities , Male , Pregnancy , Protein-Losing Enteropathies/pathology , UltrasonographyABSTRACT
OBJECTIVE: Cholestatic liver disease in infancy is caused by a wide range of conditions. This study reviews the pattern of diagnosis of infants with cholestasis presenting to a tertiary referral paediatric hospital in Sydney, Australia, during a 12-year period (1985-96). METHODOLOGY: Infants aged less than 6 months with cholestasis were identified retrospectively from hospital records and data retrieved from the medical records. RESULTS: There were 205 infants identified as having cholestatic liver disease. The aetiology of the cholestasis was idiopathic in 25%, metabolic/genetic in 23%, and due to obstruction in 20%, parenteral nutrition in 20%, infection in 9% and bile duct hypoplasia in 3%. CONCLUSIONS: This study highlights the changing patterns of diagnosis of cholestatic liver disease in infants at a tertiary paediatric facility, demonstrating that up to 50% of cases are now due to genetic/metabolic diseases or parenteral nutrition, and a high proportion are due to idiopathic disease.
Subject(s)
Cholestasis/etiology , Age of Onset , Cholestasis/epidemiology , Female , Humans , Infant , Infant, Newborn , Infections/complications , Male , Metabolism, Inborn Errors/complications , New South Wales/epidemiology , Parenteral Nutrition, Total/adverse effects , Retrospective StudiesABSTRACT
The objective of our study was to compare the safety and efficacy of discharging asthmatic children from hospital on three versus four hourly nebulized salbutamol. The setting was a tertiary referral paediatric hospital in Sydney, NSW, Australia. The design was a randomized controlled parallel group study. All children admitted to hospital with acute asthma and who were over 18 months of age were eligible to enter the study. Patients were excluded if they had non-English speaking parents, no telephone, or chronic cardiac or neurological disease. Children were treated according to standard asthma management but were randomly allocated to be discharged on three or four hourly nebulized salbutamol. Patients were surveyed using a telephone questionnaire 1 to 2 weeks after discharge. The primary outcome measure was re-presentation to the Emergency Department (ED) within 7 days. Other outcomes included readmission to hospital, re-presentation to the local doctor, parental satisfaction and length of hospital stay. A total of 63 children were enrolled in the study (32 in the three hourly group and 31 in the four hourly group). There were no re-presentations to the ED or hospital readmissions within 1 to 2 weeks in either group. However, re-presentations to the local doctor were common, 71.8% in the three hourly and 74.1% in the four hourly groups, respectively. These were predominantly for routine review. The mean (+/- SD) hospital length of stay was not significantly different between the three and four hourly groups, 48.94 (+/- 20.61) and 54.88 (+/- 32.59) hours, respectively (P = 0.672). Parents felt the timing of discharge was 'too early' in five (15.6%) of three hourly and five (16.1%) of four hourly patients. Three (9.7%) of the four hourly but none of the three hourly patients felt they were sent home 'later than necessary'. Five (15.1%) of the three hourly and three (9.7%) of the four hourly group parents did not feel comfortable looking after their child at home immediately after discharge. None of these differences were statistically significant. Discharge of asthmatic children from hospital on three hourly nebulized salbutamol is as safe and effective as on four hourly. Parents are generally very satisfied with timing of discharge, irrespective of frequency of nebulization. Earlier discharge benefits both the child and their family, and improves hospital bed utilization.
