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Mutation Analysis of Korean Patients with Glycogen Storage Disease Type Ia.

Jong Won KIM ; Ji Yeon PARK ; Jeong Kee SEO

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):213-217.

PURPOSE: Glycogen storage disease type Ia (GSD Ia) is an autosomal recessive disorder of glycogen metabolism caused by glucose-6-phosphatase (G6Pase) deficiency. The clinical manifestations of G6Pase deficiency include growth retardation, hepatomegaly, hypoglycemia, lactic acidemia, hyperlipidemia and hyperuricemia. Many mutations of this gene have been found worldwide in various ethnic groups, establishing the molecular basis of GSD Ia. To elucidate a spectrum of the G6Pase gene mutations in Korean, we analyzed mutations in Korean patients with GSD Ia. METHODS: Both alleles of 9 unrelated GSD 1a patients were studied by PCR and direct DNA sequencing methods. In all patients, GSD 1a was diagnosed by the enzyme assay for the liver biopsy specimen. RESULTS: In Korean, the most prevalent mutation was g727t substitution in exon 5, which has been reported to cause abnormal mRNA splicing: Sixteen out of 18 alleles were found to have this mutation. In addition, we identified one novel mutation, a c611g, converting a proline to an alanine at codon 178. CONCLUSION: Our findings suggest that a screening for the g727t mutation by noninvasive molecular method can detect most cases of GSD Ia in Korean patients.
Alanine ; Alleles ; Biopsy ; Codon ; Enzyme Assays ; Ethnic Groups ; Exons ; Glucose-6-Phosphatase ; Glycogen Storage Disease* ; Glycogen* ; Hepatomegaly ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Liver ; Mass Screening ; Metabolism ; Polymerase Chain Reaction ; Proline ; RNA, Messenger ; Sequence Analysis, DNA

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Hepatitis Complicated with Mycoplasma pneumoniae Infection.

Jin Tae LEE ; Hee Sup KIM ; Hann TCHAH

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):207-212.

PURPOSE: Mycoplasma pneumoniae infection is relatively common in childhood. Its extrapulmonary manifestations have been reported so much, but hepatitis associated with it has not been studied yet until now in Korea. METHODS: We performed the prospective study of 19 cases that had AST and ALT>50 IU/L respectively without evidence of hepatitis A, B, C, cytomegalovirus, and Ebstein-Barr virus infections amongst 143 patients with M. pneumoniae pneumonia who were admitted to the Department of Pediatrics, Seoul Red Cross Hospital from Jan. 1999 to Dec. 2000. RESULTS: 1) Hepatitis occurred in 13.3% of Mycoplasma pneumoniae infection, especially in fall and winter times. The average age was 4.86 years and male to female ratio was 2.2 : 1. 2) Vomiting was developed in 21.1%, diarrhea in 36.8%, and hepatomegaly in 21.1%, respectively. And leukocytosis was noted in 21.1%, eosinophilia in 15.4%, anemia in 10.5%, and thrombocytosis in 5.3%, respectively. The average level of C-reactive protein was 6.34+/-4.82 mg/dl. 3) There was no hyperbilirubinemia, but hypoalbuminemia was detected in 42.1%. The average serum levels of AST and ALT were 214.05+/-183.22 IU/L and 284.16+/-286.84 IU/L, respectively. 4) Chest radiology showed lobar or lobular consolidation in 73.7%, bronchial infiltration in 26.3%, and pleural effusion in 31.6%. 5) The average length of hospitalization was 7.9 days, and the average length of normalization of serum transaminases was 8.8 days. All patients recovered within 2 weeks completely. CONCLUSION: The prognosis of M. pneumoniae hepatitis is good. However, its incidence is not so low that liver function should be considerately checked in case of M. pneumoniae infection.
Anemia ; C-Reactive Protein ; Child ; Cytomegalovirus ; Diarrhea ; Eosinophilia ; Female ; Hepatitis A ; Hepatitis* ; Hepatomegaly ; Hospitalization ; Humans ; Hyperbilirubinemia ; Hypoalbuminemia ; Incidence ; Korea ; Leukocytosis ; Liver ; Male ; Mycoplasma pneumoniae* ; Mycoplasma* ; Pediatrics ; Pleural Effusion ; Pneumonia ; Pneumonia, Mycoplasma* ; Prognosis ; Prospective Studies ; Red Cross ; Seoul ; Thorax ; Thrombocytosis ; Transaminases ; Vomiting

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The Study of the Initial Presentations of Wilson Disease at Diagonosis.

Tae Jin YANG ; Geun Ha JI ; Min Seop SONG ; Tae Gyu HWANG

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):199-206.

PURPOSE: To review the clinical and laboratory features of patients with Wilson disease at diagnosis. METHODS: In this retrospective study, records of all 20 patients, who were diagnosed as having Wilson disease at the Paik hospital in Busan from 1990 to 2000, were reviewed. RESULTS: Out of 20 patients, 12 pateints (60%) have hepatic presentation alone, 2 patients (10%) have neurologic presentation, 4 patients (20%) have hepatic and neuropsychiatric presentation, and one patient (5%) has hematologic presentation at diagnosis. One patient (5%) has neither symptom nor laboratory finding of Wilson disease except very low serum ceruloplasmin level and positive family history. Family screening test revealed 3 cases of Wilson disease. 12 patients were revealed to be combined with liver cirrhosis at diagnosis. CONCLUSION: Early diagnosis and treatment is very important in patients with Wilson disease. Children or adolescents who manifest symptoms of hepatitis, who has prolonged elevation of liver enzymes, and has family history of hapatitis of unknown origin, with mild hematologic or urinary abnormalities must be suspected to have Wilson disease. Also, in adolescents with extrapyramidal symptoms or other neuropsychiatric symptoms, liver function test should be done.
Adolescent ; Busan ; Ceruloplasmin ; Child ; Diagnosis ; Early Diagnosis ; Hepatitis ; Hepatolenticular Degeneration* ; Humans ; Liver ; Liver Cirrhosis ; Liver Function Tests ; Mass Screening ; Retrospective Studies

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Concordance of Seropositivity between Helicobacter pylori and Hepatitis A Virus IgG in Children of Gwangju and Chonnam Area.

Seon Young KIM ; Young Ok KIM ; Hae Yul CHUNG ; Byung Ju KIM ; Jae Sook MA

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):191-198.

PURPOSE: This study was undertaken to investigate the seroepidemiologic pattern of Helicobacter pylori (H. pylori) and hepatitis A virus (HAV) infections in children. METHODS: A total of 315 serum samples were obtained from healthy children, living in Gwangju and Chonnam area. All serum samples were assayed for H. pylori IgG level using enzyme immunoassay techniques. HAV IgG level in serum were tested by a competitive radio-immunoassay in 215 subjects. The age-specific seroprevalence of H. pylori and HAV was separately analysed. The concordance of seropositivity and seronegativity between H. pylori and HAV infection was examined by the kappa statistic analysis. RESULTS: Seropositivity was found in 17.5% (55/315) and 30.2% (65/215) of the subjects for H. pylori and HAV, respectively. Cross-tabulation of these data showed that 21 subjects (9.8%) were seropositive and 135 (62.8%) were seronegative for both H. pylori and HAV, 15 (7.0%) were seropositive for only H. pylori and 44 (20.5%) for only HAV. The seroprevalence of H. pylori and HAV increased significantly with age. There was a slight agreement between H. pylori and HAV seropositivity (kappa=0.26). CONCLUSION: This study shows a slight similarity in the concordance of seropositivity and seronegativity between H. pylori and HAV infection and provides evidence that H. pylori and HAV may share a common mode of transmission.
Child* ; Epidemiology ; Gwangju* ; Helicobacter pylori* ; Helicobacter* ; Hepatitis A virus* ; Hepatitis A* ; Hepatitis* ; Humans ; Immunoenzyme Techniques ; Immunoglobulin G ; Jeollanam-do* ; Seroepidemiologic Studies

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Intestinal Ultrasonographic and Endoscopic Findings in Pediatric Patients with Henoch-Schonlein Purpura and Gastrointestinal Symptoms.

Yun Il NOH ; Min Hyuk RYU ; Chul Zoo JUNG ; Dong Jin LEE ; Jung Hyeok KWON

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):181-191.

PURPOSE: The aim of this study is to investigate the usefulness of intestinal ultrasonography (US) and upper gastrointestinal endoscopy in the early diagnosis of Henoch-Schonlein purpura (HSP) with the gastrointestinal (GI) symptoms preceding the emergence of the skin lesion. METHODS: The clinical, intestinal US and upper gastrointestinal endoscopic records of 85 patients (88 cases) with GI symptoms relating to HSP presenting between January 1999 and April 2001 were reviewed. RESULTS: 1) GI symptoms were observed in 52 cases (59%) and skin, joint, renal and scrotal manifestations were observed in 88 (100%), 64 (73%), 15 (17%), 3 cases (3%) respectively. 2) Out of 52 cases with GI symptoms, abdominal pain was observed in all cases (100%). Positive stool occult blood, nausea and vomiting, abdominal tenderness, melena or tarry stool, diarrhea, hematemesis, rebound tenderness and rigidity were observed in 28 (50%), 17 (33%), 17 (33%), 12 (23%), 6 (12%), 4 (8%), 1 (2%) and 1 case (2%) respectively in order of frequency. 3) Intestinal US examination was performed in 27 cases with HSP and GI symptoms (52 cases). Out of 27 sonographic examinations 22 showed abnormal findings. Thickening of the duodeno-jejunal wall was observed in 16 cases (73%). Free peritoneal fluid, enlarged mesenteric lymph node, ileus and abnormal gall bladder were seen in 8 (36%), 8 (36%), 4 (18%) and 1 case (5%) respectively. In three cases of HSP without GI symptoms, those changes were absent. 4) In all of five cases with HSP and GI symptoms, endoscopic study showed mucosal edema and multiple hemorrhagic erosions especially at the second portion of the duodenum. Biopsy specimens from the duodenum of 2 cases out of 5 endoscopic examinations showed acute inflammatory infiltrates in the mucosa with hemorrhage. 5) Both intestinal US and endoscopic studies were performed in 4 cases with HSP and GI symptoms simultaneously. Out of 4 those cases, 3 cases showed the thickened duodeno-jejunal wall on the intestinal US, which suggested erosive hemorrhagic duodenitis by endoscopic findings. CONCLUSION: The typical but nonpathognomonic intestinal US findings including the thickening of the duodeno-jejunal wall and upper gastrointestinal endoscopic findings including hemorrhagicerosive duodenitis, in children with GI symptoms, should be considered a manifestation of HSP, even in the absence of skin lesion.
Abdominal Pain ; Ascitic Fluid ; Biopsy ; Child ; Diarrhea ; Duodenitis ; Duodenum ; Early Diagnosis ; Edema ; Endoscopy ; Endoscopy, Gastrointestinal ; Hematemesis ; Hemorrhage ; Humans ; Ileus ; Joints ; Lymph Nodes ; Melena ; Mucous Membrane ; Nausea ; Occult Blood ; Purpura, Schoenlein-Henoch* ; Skin ; Ultrasonography ; Urinary Bladder ; Vomiting

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Clinical Significance of Repeated Delayed Air Reduction in Unsuccessful Initial Reduction of Intussusception.

Song Heui SHIN ; Young Il RHO ; Young Bong PARK ; Kyung Rye MOON

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):175-180.

PURPOSE: The advantages of air reduction are the ease of performing the procedure, reduced radiation time and lower morbidity rate if perforation occurs. But, patients who fail air reduction undergo a laparotomy at which 10% have spontaneously reduced. The first enema decreases the edema and venous congestion of bowel wall, thus repeated reduction may succeed. The aim of this study is to evaluate the efficacy of delayed repeated pneumatic reduction of intussusception in patients with failure of an initial attempt. METHODS: Between January 1998 and December 1999, 21 patients with proven intussusception received repeated delayed reduction 1 to 3 hours following the first failed attempt. These patients were in stable condition and did not have peritonitis, shock or toxic sign. RESULTS: Before reduction, the patients had symptoms and signs for 6 to 48 hours (median 24 hr). The interval from the first reduction to the repeated was 1 to 3 hours (median 2.2 hr). The success rate of repeated reductions was 76.2% (16 of 21 patients), and 23.8% (5 of 21 patients) required surgery. Four of 5 patients requiring surgery were manually reduced and 1 spontaneously reduced in the operating room. CONCLUSION: We recommended a repeated reduction in patients with intussusception who are in stable clinical condition after an unsuccessful initial reduction attempt.
Edema ; Enema ; Humans ; Hyperemia ; Intussusception* ; Laparotomy ; Operating Rooms ; Peritonitis ; Shock

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Relationship between Intussusception and Vitamin C Concentrations of Whole Blood and Plasma.

Ji Hyoun SEO ; Jeum Su KIM ; Ji Young HWANG ; Jin Su JUN ; Ji Hoe PARK ; Eun A KIM ; Jae Young LIM ; Myoung Bum CHOI ; Chan Hoo PARK ; Hyang Ok WOO ; Hee Shang YOUN

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):167-174.

PURPOSE: Adenovirus infection with swollen Peyer patches in the ileum, known as one of the causes of intussusception, may cause vitamin C depletion in human body because vitamin C is a first line antioxidant. Or low vitamin C status in human body makes the man more susceptible to infection of adenovirus in the ileum with a markedly swollen lymph node. In this study, we tried to find out the relationship between pediatric intussusceptions and vitamin C concentrations of whole blood and plasma. METHODS: Whole blood and plasma vitamin C concentrations of fifty-seven patients with intussusceptions from May 1995 to December 1998 at the Gyeongsang National University Hospital were compared with those of 256 normal healthy children. Vitamin C was measured by the 2,4-dinitrophenylhydrazine methods. RESULTS: The average age of patients (male=39, female=18) with intussusceptions was fifteen months of age. Whole blood vitamin C concentrations of patients and healthy children were 1.49+/-0.64 mg/dL, and 2.18+/-0.49 mg/dL, respectively. Plasma vitamin C concentrations of patients and healthy children were 0.59+/-0.36 mg/dL, 1.47+/-0.56 mg/dL, respectively. But no differences in the vitamin C concentrations of whole blood and plasma according to age, degree of leukocytosis, fever, interval from onset, hematochezia, and need for operation were found. CONCLUSION: Whole blood and plasma vitamin C concentrations of patients with intussusceptions were lower than those of healthy children (P=0.0001). Prospective studies are needed to elucidate whether these results were consequences or causes of intussusceptions.
Adenoviridae ; Adenoviridae Infections ; Ascorbic Acid* ; Child ; Fever ; Gastrointestinal Hemorrhage ; Human Body ; Humans ; Ileum ; Intussusception* ; Leukocytosis ; Lymph Nodes ; Peyer's Patches ; Plasma* ; Vitamins*

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High Seroprevalence of Helicobacter pylori Infection in Korean Adolescent Athletes.

Heung Ryel KIM ; Yeon Hwa AHN ; Yon Ho CHOE ; Ji Eun LEE ; Yun Chul HONG

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):161-166.

PURPOSE: Poor living conditions such as overcrowding and sharing a bed are reported to be significant risk factors for H. pylori infection throughout childhood. We investigated the prevalence of Helicobacter pylori infection in Korean athletes who are obliged to live together in training camps over six months a year. METHODS: Blood sampling and surveying through questionnaire were performed on 440 regular high school students (228 boys and 212 girls) and 220 athletes (148 boys and 72 girls) of a physical education high school. We measured serum IgG Ab to H. pylori in order to compare the prevalence of H. pylori infection between normal adolescents and athletes. In addition, we conducted a nutritional analysis and questionnaire survey for socioeconomic status in order to compare other risk factors that might influence H. pylori infection between groups. RESULTS: The prevalence (43.2%) of H. pylori infection in athletes was higher than that (22.7%) in the controls. No significant differences in the nutritional factors or socioeconomic status, such as Hollingshead index, type of house, and crowding index, were found between the groups. Multivariate analysis showed that crowding and the difference in school type were the significant predictors of H. pylori seropositivity after controlling for dietary and socioeconomic factors. CONCLUSION: Living together in training camps might be a significant risk factor for H. pylori infection in athletes.
Adolescent* ; Athletes* ; Crowding ; Helicobacter pylori* ; Helicobacter* ; Humans ; Immunoglobulin G ; Multivariate Analysis ; Physical Education and Training ; Prevalence ; Surveys and Questionnaires ; Risk Factors ; Seroepidemiologic Studies* ; Social Class ; Social Conditions ; Socioeconomic Factors

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Helicobacter pylori vacA Mosaicism and New Primers for vacA Signal Sequence Indigenous to Korea.

Yeon Hwa AHN ; Heung Ryel KIM ; Ji Eun LEE ; Tae Sook HWANG ; Yon Ho CHOE

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):155-160.

PURPOSE: Helicobacter pylori has been known to have diverse vacA allelic types. The purpose of the study was to identify vacA diversity in Korea and design new primers for signal sequence alleles indigenous to Korea. METHODS: Fifty antral biopsy specimens, which had been proven to be H. pylori-positive, were examined for vacA status; signal sequence and mid-region. After PCR amplification and DNA sequencing, vacA alleles of Korean H. pylori strains were compared with those from other countries. RESULTS: Among Korean H. pylori strains vacA alleles with all combinations of signal sequence and mid-region were found, with the exception of s1b or s2. vacA genotype s1c/m1 was predominant in Korea. We found that GGGAGCGTTR in s1a and GGGGYTATTG in s1c were the indigenous sequences to Korea and constructed the new Korean specific primers for the vacA signal sequence; VASK-F, VASK-R, S1AK-F, and S1CK-F. CONCLUSION: This study showed that s1c/m1 is the predominant type of vacA allele in Korea. We designed new primers for the vacA signal sequence.
Alleles ; Biopsy ; Genotype ; Helicobacter pylori* ; Helicobacter* ; Korea* ; Mosaicism* ; Polymerase Chain Reaction ; Protein Sorting Signals* ; Sequence Analysis, DNA

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Gastrointestinal Foreign Bodies in Children: Experiences of 60 Cases in Kangwon, Korea.

Young Sub LEE ; Kae Wool KANG ; Won Kyu CHOI

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(2):148-154.

PURPOSE: Ingested foreign bodies present a common clinical problem. It is well known that most of them pass uninterrupted through the gastrointestinal tract. We evaluated the role of endoscopy and Foley catheter for removal of foreign bodies in the gastrointestinal tract. METHODS: We investigated retrospectively 60 cases with foreign bodies in the gastrointestinal tract. They had been treated at Wonju Christian Hospital, Yonsei University of Korea, from January, 1996 through December, 1999. RESULTS: The age of the patients ranged from 7 months to 13 years. Patients under 5 years were 57 cases (97%) and there was no significant difference in sex (M : F=1.07 : 1). 45 cases of the patients had no symptom. The most common foreign bodies were coins (43 cases). The most common location was esophagus (31 cases). The number of foreign body removal using flexible endoscopy and Foley catheter was 22 (36.7%) and 18 (30.0%) cases, respectively. In 18 cases (30.0%), foreign bodies passed spontaneously. Only 1 case (1.7%), curtain pin impaction at ileocecal region, required surgery. CONCLUSION: Early foreign body removal from esophagus and stomach is recommended to lessen the morbidity and complication. Fluoroscopic foley catheter technique and flexible endoscopy for removal of esophageal foreign bodies in children is safe and effective.
Catheters ; Child* ; Endoscopy ; Esophagus ; Foreign Bodies* ; Gangwon-do* ; Gastrointestinal Tract ; Humans ; Korea* ; Numismatics ; Retrospective Studies ; Stomach

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