Nutritional Status and Growth in Korean Children with Crohn's Disease: A Single-Center Study

BACKGROUND/AIMS: Malnutrition and growth retardation are important issues in treating pediatric Crohn's disease (CD). Thus, we aimed to investigate the prevalence of various nutritional and growth parameters at the time of diagnosis in Korean children with CD. METHODS: Seventy-one children (<18 years) were enrolled. We analyzed the Z-scores of height-for-age (HAZ), weight-for-height (WHZ), body mass index for age (BMIZ), bone mineral density for age (BMDZ), and the biochemical markers measured at the time of diagnosis. RESULTS: At diagnosis, HAZ <-2 was observed in three patients (4%), WHZ <-2 in 20 patients (28%), BMIZ <-2 in 19 patients (27%), and BMDZ <-2 in 11 patients (18%). The HAZ was significantly lower in females and patients with extraintestinal manifestations, and the WHZ and BMIZ were significantly lower in patients with stricturing and penetrating disease. Subnormal serum levels were highly prevalent for hemoglobin, albumin, iron, ferritin, calcium, magnesium, folate, vitamin B12, and zinc. There was a significant correlation between nutritional status, growth retardation, and disease activity. CONCLUSIONS: Abnormal nutritional status was highly prevalent in Korean children with CD at the time of diagnosis and was associated with the extent, behavior, and activity of the disease.

Liver transplantation in a child with acute liver failure resulting from drug rash with eosinophilia and systemic symptoms syndrome / 소아과

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is characterized by a severe idiosyncratic reaction including rash and fever, often with associated hepatitis, arthralgias, lymph node enlargement, or hematologic abnormalities. The mortality rate is approximately 10%, primarily owing to liver failure with massive or multiple disseminated focal necrosis. Here, we report a case of a 14-year-old girl treated with vancomycin because of a wound infection by methicillin-resistant Staphylococcus aureus, who presented with non-specific symptoms, which progressed to acute liver failure, displaying the hallmarks of DRESS syndrome. With the presence of aggravated hepatic encephalopathy and azotemia, the patient was refractory to medical treatments, she received a living-donor liver transplantation, and a cure was achieved without any sign of recurrence. Vancomycin can be a cause of DRESS syndrome. A high index of suspicion and rapid diagnosis are necessary not to miss this potentially lethal disease.

Two Cases of Pediatric Collagenous Gastritis Each Presenting with Refractory Iron Deficiency Anemia and Chronic Diarrhea / 대한소아소화기영양학회지

Collagenous gastritis (CG) is a rare disorder that is characterized by the presence of a thick subepithelial collagen band with multiple infiltrated inflammatory cells of the gastric mucosa. CG is divided into two major subsets: first, in children and young adults presenting with severe anemia and abdominal colic pain (pediatric-type CG); and second, in adult patients with chronic watery diarrhea associated with collagenous colitis (adult-type CG). We report two cases of pediatric-type CG, each presenting with refractory anemia and chronic diarrhea.

Tumor Necrosis Factor-alpha Gene Polymorphisms in Korean Children with Inflammatory Bowel Disease / 대한소아소화기영양학회지

PURPOSE: The aim of this study was to investigate the existence of TNF-alpha polymorphisms in Korean children with Crohn's disease (CD), ulcerative colitis (UC), as compared to healthy controls. METHODS: Blood samples were obtained from 40 patients with CD, 14 patients with UC, and 30 healthy controls. Genotyping for 5 TNF-alpha polymorphisms (G238A, G308A, C857T, C863A, and T1031C) was performed. The allele frequencies for the inflammatory bowel diseases, CD and UC, were measured in patients with these disease and in healthy controls, and these allele frequencies were compared between the 3 groups. We examined the significant association between polymorphism and disease phenotype, such as location, behavior, perianal disease, and pediatric Crohn's activity index (PCDAI) in CD. RESULTS: Based on our findings, the TNF-alpha allele frequencies of G238A, G308A, C857T, C863A, and T1031C were 3.3, 8.3, 16.7, 16.7, 21.7% in healthy control, 2.5%, 7.5%, 18.8%, 20.0%, 22.5% in CD, 7.1%, 7.1%, 7.1%, 21.4%, 28.6% in UC. They were no statistically significant differences between the 3 groups. There were no associations between genotypes and phenotypes in CD, except a statistically significant higher allele frequency of G238A in ileal type (L1) disease (p=0.010). CONCLUSION: Our results indicate that 5 TNF-alpha polymorphisms do not seem to be associated with susceptibility to inflammatory bowel disease in Korean pediatric patients even though young patients were anticipated to have a stronger genetic affiliation for these diseases than adult patients. We think that further studies are needed to find those genes associated with susceptibility to CD and UC in Korean pediatric patients with inflammatory bowel disease.

A case of anemia caused by combined vitamin B12 and iron deficiency manifesting as short stature and delayed puberty / 소아과

Anemia caused by vitamin B12 deficiency resulting from inadequate dietary intake is rare in children in the modern era because of improvements in nutritional status. However, such anemia can be caused by decreased ingestion or impaired absorption and/or utilization of vitamin B12. We report the case of an 18-year-old man with short stature, prepubertal sexual maturation, exertional dyspnea, and severe anemia with a hemoglobin level of 3.3 g/dL. He had a history of small bowel resection from 50 cm below the Treitz ligament to 5 cm above the ileocecal valve necessitated by midgut volvulus in the neonatal period. Laboratory tests showed deficiencies of both vitamin B12 and iron. A bone marrow examination revealed dyserythropoiesis and low levels of hemosiderin particles, and a cytogenetic study disclosed a normal karyotype. After treatment with parenteral vitamin B12 and elemental iron, both anemia and growth showed gradual improvement. This is a rare case that presented with short stature and delayed puberty caused by nutritional deficiency anemia in Korea.

The natural history and prognostic factors of Graves' disease in Korean children and adolescents / 소아과

PURPOSE: Graves' disease is the most common cause of hyperthyroidism in children and adolescents. In this study, we investigated the natural course and the prognostic factors of Graves' disease in Korean children and adolescents. METHODS: One-hundred thirteen (88 girls and 25 boys) patients were included in this study. A retrospective analysis was made of all patients who were diagnosed with Graves' disease. The following parameters were recorded and analyzed: patient's sex, age at diagnosis, duration of disease, laboratory findings, symptoms and signs, and family history of autoimmune thyroid disease. RESULTS: All patients were initially treated with antithyroid drugs, either methimazole (93.8%) or propylthiouracil (6.2%). Antithyroid drugs had been discontinued in 75 (66.4%) of 113 patients. Of these 75 patients, 23 (20.4%) relapsed after 25.5+/-33.7 months. Thirteen (11.5%) of 23 patients, who experienced the first relapse, showed a second remission. However, 2 (1.8%) of 13 patients relapsed again. Euthyroid state could not be achieved by antithyroid drugs in 1 patient, and radioactive iodine therapy was performed. The older the patient at diagnosis, the greater the likelihood of remission (P=0.034). CONCLUSION: Age at diagnosis seems to be a prognostic factor in Korean children and adolescents with Graves' disease, and should be taken into account in treatment plan determination.

Analysis of Overall Clinical Manifestations of Tuberous Sclerosis Complex Patients in the Childhood : Updated Diagnositic Criteria / 대한소아신경학회지

PURPOSE: The aim of this study is to analyze the patterns of presentation and progress of tuberous sclerosis complex (TSC) and the prevelance of items in diagrnostic criteria of TSC in the pediatric population group. METHODS: We reviewed 74 patients diagnosed with TSC by clinical diagnostic criteria, who visited Asan Medical Center from April, 1991 to August, 2008. RESULTS: Forty-four males and 30 females were enrolled with 2.3 years(0 days-15.4 years) of mean age at the diagnosis. Mean follow-up period was 3.2(0-12.6) years. Remarkable initial symptoms or signs at the first hospital visit were seizure(n=48, 64.9%), cardiac rhabdomyoma(n=15, 20.2%) and skin lesions(n=7, 9.5%). Seven of 74 patients(9.5%) had familial history of TSC. Seventy-one patients(95.9%) showed one or more brain lesions on the neuroimaging studies, including cortical tubers(n=69, 93.2%), subependymal nodules(n=71, 95.9%), subependymal giant cell astrocytomas(SEGA, n=5, 6.8%). Retinal astrocytic hamartomas were confirmed in 14 patients and suspicious in 4 patients. Skin lesions were detected in 58 patients(78%). Sixty-one patients(82%) had experienced one or more seizures and 58 were confirmed as epilepsy. Twenty-eight patients(38%) had experienced infantile spasm. Thirty-two of 52 patients evaluated(61.5%) had mental retardation. Gene studies were done in 13 patients and revealed two TSC1 gene mutations and seven TSC2 gene mutations. CONCLUSION: Seizure attacks and skin lesions were two most common causes to visit hospital in pediatric TSC patients. Considering the high incidence of epilepsy and its correlation with poor neurodevelopemental outcome, early diagnosis and adequate treatment will lead to the better quality of life.