Long-term Growth of Pediatric Patients Following Living-Donor Liver Transplantation

In order to determine the influence of living donor liver transplantation (LDLT) on long-term growth, we studied the progress of 36 children who had survived more than 5 yr after LDLT from 1994 to 1999. The median age at the transplantation was 1.5 yr (range: 6 months-15 yr) and the median follow-up period was 6.5 yr (range: 5-9 yr). A height standard deviation score (zH) was analyzed for each patient according to medical records. Significant catch-up growth occurred within 2 yr after LDLT with a mean zH changing from -1.2 to 0.0 and was maintained for up to 7 yr post-transplantation (zH-0.1). Younger children (<2 yr) were more growth-retarded at the time of LDLT, but showed higher catch-up growth rates and their final zH was greater than that of older children. Children with liver cirrhosis were more growth-retarded at the time of LDLT, but showed significant catch-up growth and their final height was similar to children with fulminant hepatitis. Growth in children who experienced significant hepatic dysfunction after LDLT was not significantly different from those without graft dysfunction. There was no difference between the types of immunosuppressants used. Our finding suggests that LDLT can result in adequate catchup linear growth, and this effect can persist even after 7 yr post-transplantation.

Analysis of Linear Growth in Children after Living-related Liver Transplantation / 대한소아소화기영양학회지

PURPOSE: The aim of this study is to evaluate the effective role of living-related liver transplantation (LRLT) on posttransplant linear growth in children. METHODS: Thirty six children were enrolled who received LRLT at Asan Medical Center from December, 1994 to February, 1999 and showed more than one-year postoperative survival. Mean height standard deviation score (zH) was analyzed according to medical records including heights during pretransplant and posttransplant follow-up periods. RESULTS: zH of total children showed significant linear growth after LRLT from -1.58 to 0.33 at 24 posttransplant month (p<0.05). zH in children under 6 years of age, to exclude the effect of adolescent linear growth spurt, showed increment in height (p<0.05). Linear growth of children with liver cirrhosis improved and that with fulminant hepatitis was matained same. While stunted children (mean zH=-2.30) achieved good catch-up growth after transplantation, children with normal growth remained same. Children with significant hepatic dysfunction after LRLT such as chronic rejection or posttransplant lymphoproliferative disorder showed retarded posttrasplant linear growth. There was no statistical difference according to the type of immunosuppressants. CONCLUSION: LRLT resulted in adequate or catch-up linear growth in children with acute, chronic and metabolic liver disease. Successful LRLT suggested to be a promising option not only in long term survival but also in normal linear growth.

Fanconi-Bickel Syndrome Presented with Diabetes Mellitus and Galactosemia : Identification of a Novel Mutation in the GLUT2 Gene

Fanconi-Bickel syndrome is a rare autosomal recessive disorder of the carbohydrate metabolism recently demonstrated to be caused by mutations in GLUT2, the gene for the glucose transporter protein 2 expressed in the liver, pancreatic beta islet-cells, intestine and kidney. Typical clinical and laboratory findings of Fanconi-Bickel syndrome are hepatomegaly secondary to glycogen accumulation, glucose and galactose intolerance, fasting hypoglycemia, a characteristic proximal tubular nephropathy and severe short stature. Several cases have been reported in other countries after Fanconi and Bickel in Switzerland first reported this syndrome in 1949. We experienced the first Korean case of Fanconi-Bickel syndrome in a neonate presented with hyperglycemia and hypergalactosemia that was initially diagnosed as transient neonatal diabetes mellitus and galactosemia. We also identified a novel mutation(K5X) in the GLUT2 gene.

The Usefulness of the Head-up Tilt Test for Diagnosis of Syncope in Pediatric Patients

PURPOSE: The purpose of this study is to examine the usefulness of the head-up tilt test for diagnosis of unexplained syncope in children. METHODS: Head-up tilt test results and clinical features of 41 children with unexplained syncope, presyncope, dizziness and seizure were studied from January, 1997 through January, 2001 at Asan Medical Center. Medical records of children were reviewed retrospectively. The children were evaluated with an 80 degrees head-up tilt test for 15 minutes with or without intravenous infusion of isoproterenol(0.05-0.1 ng/kg/min). RESULTS: 41 children made up the study population, of whom 23(56%) had a positive head-up tilt test and 21(60%) of 35 patients with a history of syncope or presyncope had a positive head-up tilt test. Isoproterenol infusion provoked the more positive head-up tilt test. The patients with positive test results showed three patterns of response to tilting. 16 patients had a predominantly vasodepressor response; three patients had a cardioinhibitory response; and four patients had a mixed response. The patients had an average of five studies performed per patient, including chest radiograph, electrocardiogram, 24 hour Holter monitoring, treadmil test, head computed tomographic scan or magnetic resonance imaging, and echocardiography. The head-up tilt test was most effective for evaluation of unexplained syncope in children. CONCLUSION: Head-up tilt testing performed early in the evaluation will increase the probability of a diagnosis, and will often prevent the need for further extensive, expensive, anxiety-producing tests in children. More controlled studies and a standardization of degree and duration of tilting are necessary to validate the head-up tilt test as a useful diagnostic tool in children with unexplained syncope.