Cricopharyngeal incoordination of infancy is a rare disease, characterized by difficult swallowing soon after birth. regurgitation, frequent choking and aspiration with deglutition, recurrent aspiration pneumonia, and to-and-fro movement of the contrast medium in the posterior pharynx on upper esophageal cineroentgenography. Clinical investigation was performed in the infants who were admitted due to difficult swallowing, regurgitation, and recurrent pneumonia from March 1, 1989 to June 30, 1992. The results were as follows: 1) Male to female sex ratio was great, and major symptoms such as difficult swallowing, regurgitation, choking and aspiration with deglutition, and those of pneumonia developed soon after birth in most cases. 2) The typical findings of this disease were noted on the cineroentgenography of upper esophagus in all cases and those of aspiration pneumonia in 10 cases. 3) The infants had been fed via gavage tube until they were able to swallow without difficulties before and after 6 months after birth. 4) Cricopharyngeal incoordination is a rare disease, but we suggest this disease should be considered in differential diagnosis in the infants with difficult swallowing soon after birth and recurrent episodes of aspiration pneumonia.
Airway Obstruction ; Ataxia* ; Deglutition ; Diagnosis, Differential ; Esophagus ; Female ; Humans ; Infant ; Male ; Parturition ; Pharynx ; Pneumonia ; Pneumonia, Aspiration ; Rare Diseases ; Sex Ratio

No abstract available.
Child* ; Hepatitis* ; Humans

Fifty five children with endoscopically proven esophageal varices from Jul. 1987 to Dec. 1992 were analyzed for endoscopic and clinical characteristics. The results were as follows: 1) According to etiological classification of portal hypertension in 55 cases, 35 cases showed intrahepatic causes 964%) and 20 cases had extrahepatic causes (36%). The most common clinical manifestation on admission was hematemesis (42%) and abdominal mass (36%). 2) Portal vein thrombosis was the most common cause of portal hypertension. Patients with portal vein thrombosis showed more frequent bleeding than with intrahepatic portal hypertension. Most of them had the first bleeding episode before 7 years old of age. Progression of portal hypertension in portal vein thrombosis was more rapid and bleeding episode was earlier than that in most of intrahepatic causes of the portal hypertension. 3) Twenty patients showed gastritis (10 cases), duodenitis (4 cases), gastric ulcer (4 cases) and esophagitis (2 cases) besides varix on the endoscopic examination. In 4 cases, the cause of upper GI bleeding was found as duodenal ulcer (3 cases) or hemorrhagic errosive gastritis (1 case) with no variceal bleeding. 4) Among 36 cases with variceal bleeding, 20 cases had predisposing factors for bleeding such as medication for URI including Aspirin. 5) More than one episode of bleeding were noted in 36 cases (65%) of the patients with varix. Thirty-one cases were treated conservatively with success. The remaining 5 cases with severe bleeding were managed with IV pitressin, SB-tube insertion and emergency shunt operation. High mortality rate (40%) was found in these patients group. In conclusion, in patients with clinically suspected portal hypertension, regular endoscopic examination might be recommended for the earlier recognition and effective prevention of variceal bleeding. for bleeding varices, in addition to conservative management, active treatment such as pitressin, SB-tube, sclerotherapy, endoscopic variceal ligation and emergency shunt operation should be considered with the emergency endoscopy which can identify bleeding focus and predict the chance of rebleeding.
Aspirin ; Causality ; Child* ; Classification ; Duodenal Ulcer ; Duodenitis ; Emergencies ; Endoscopy ; Esophageal and Gastric Varices* ; Esophagitis ; Gastritis ; Hematemesis ; Hemorrhage ; Humans ; Hypertension, Portal ; Ligation ; Mortality ; Sclerotherapy ; Stomach Ulcer ; Varicose Veins ; Vasopressins ; Venous Thrombosis

Nineteen children found to have gallstones at Seoul National University of Children's Hospital from Jun. 1982 to Jun. 1992 were reviewed. The results were as follows: 1) The mean age was 10 years, with 9 boys and 10girls. Most of the cases(63%) were over 10 years of age. 2) Clinical manifestations were abdominal pain(14 cases), nausea and/or vomiting (5 cases), fever (2 cases), jaundice (2 cases) and fatty food intolerance (1 case). Five cases were asymptomatic. Of 14 cases with abdominal pain, only 5 cases showed typical biliary colic which suggests cholelithiasis. 3) Associated conditions in cholelithiasis were Wilson disease (5 cases), congenital hereditary spherocytosis (2 cases), choledochal cyst (2 cases), total parenteral nutrition following ileal resection (1 case). And the other conditions which had association with cholelithiasis are not certain, and they were nephrotic syndrome, histiocytic cytophathic panniculitis, meningococcal meningitis, portal vein thrombosis and cardiac cirrhosis. The remaining 4 cases showed no associated conditions or diseases. 4) Eleven of 19 patients with cholelithiasis (58%) showed cholecystitis. 5) Cholecystectomy was performed in 4 cases with frequently recurred abdominal pain or associated diseases which needed operative correction. Symptoms completely subsided after operation. In conclusion, cholelithiasis in children has a variety of associated conditions and nonspecific abdominal symptoms were nore common than classic biliary colic. Although cholelithiasis in pediatric population are uncommon problems, the possibility of gallstones should be considered in the evaluation of abdominal pain in children with associated conditions.
Abdominal Pain ; Child ; Cholecystectomy ; Cholecystitis ; Choledochal Cyst ; Cholelithiasis* ; Colic ; Fever ; Fibrosis ; Gallstones ; Hepatolenticular Degeneration ; Humans ; Jaundice ; Meningitis, Meningococcal ; Nausea ; Nephrotic Syndrome ; Panniculitis ; Parenteral Nutrition, Total ; Seoul ; Venous Thrombosis ; Vomiting

Wilson disease gene (WND) locus is presumed to be located in chromosome 13q. There are studies on the clinical heterogeneities and variations between ethnic groups in Europe, North America, and the Middle East and it requires the study of another ethnic group, especially Asian population for the confirmation. This study was an anlysis of restriction fragment length polymorphism of the Wilson's disease with the probes on D13S26, D13S31, and D13S59, The subject was 34 persons of seven families. The serum ceruloplasmin and the serum copper were also measured. The results were as follows: 1) The Wilson disease gene was also presumed to be located in chromosome 13q area in Korean patients and D13S25, D13S26, D13S31 and D13S59 gene loci were also linked to Wilson disease of Koreans. The lod score of D13S25 was 1.45 (theta =0: D13S59, 1.13 (theta =0): D13S26, 247 (theta =0). 2) Three siblings of Wilson disease patients were diagnosed as carriers by the analysis of restriction fragment length polymorphism. 3) In Wilson disease patients, the serum ceruloplasmin and copper was 6.8+/1.8dl, and 64.7+/-38.6microg/dl respectively and it is 18.8+/-6.1 mg/dl and 65.3+/-10.6microg/dl respectively in heterozygotes.
Asian Continental Ancestry Group ; Ceruloplasmin ; Copper ; Ethnic Groups ; Europe ; Genetic Linkage* ; Hepatolenticular Degeneration* ; Heterozygote ; Humans ; Lod Score ; Middle East ; North America ; Polymorphism, Restriction Fragment Length ; Siblings

No abstract available.
Child* ; Humans ; Tuberculosis*

No abstract available.
Abdominal Pain* ; Child* ; Gastritis* ; Helicobacter pylori* ; Helicobacter* ; Humans

For the detection of Helicobacter pylori infection, endoscopic biopsy related tests auch as urease test, culture, and histology with special staining of bacteria are most widely used standard methods and most accurate. (continue...)
Bacteria ; Biopsy ; Child* ; Diagnosis* ; Gastritis* ; Helicobacter pylori* ; Helicobacter* ; Humans ; Immunoglobulin G* ; Prevalence ; Urease

PURPOSE: For the early diagnosis of Henoch-Schonlein purpura(HSP) presenting with acute abdominal pain preceding skin rash. METHODS: The clinical, endoscopic and radiological records of 23 cases of HSP, presenting with gastrointestinal symptoms preceding skin rash were reviewed. RESULTS: The intervals from the onset of abdominal pain to the development of the skin rash were one day to 30 days(median five days), most of them were within two weeks. The presenting abdominal symptoms were abdominal pain(23 cases), vomiting(16 cases), hematochezia or melena(eight cases) and hematemesis(three cases). The abnormal endoscopic findings include coalescing erythematous lesions, areas of submucosal hemorrhage and superficial erosions and ulcers. The upper gastro intestinal endoscopy showed the abnormalities in 21 of 23 cases, which were observed in the duodenum(21 cases), the stomach(12 cases) and the esophagus(one case). Duodenitis with hemorrhage and/or erosions in the descending duodenum was the sole endoscopic abnormality in two cases and was the most marked finding in three cases. Sigmoidoscopy showed the abnormalities in six of eight cases. The abdominal ultrasonogram showed abnormalities in 12 of 17 cases, which included small bowel wall thickening(eight cases) and intramural hemorrhage(three cases). Recurrences after three months of symptom free intervals developed in four cases; three of them had persistent nephritis beyond one year. CONCLUSION: The erosive hemorrhagic duodenitis in the descending duodenum in the upper endoscopy and the small bowel wall thickening in the abdominal ultrasonogram can be useful findings in the diagnosis of HSP presenting with acute abdomen.
Abdomen, Acute ; Abdominal Pain* ; Diagnosis ; Duodenitis ; Duodenum ; Early Diagnosis ; Endoscopy ; Exanthema* ; Gastrointestinal Hemorrhage ; Hemorrhage ; Nephritis ; Purpura* ; Recurrence ; Sigmoidoscopy ; Skin* ; Ulcer ; Ultrasonography

This study is based on 155 patients of congenital me colon. For the diagnosis, 93 cases were histologically proven and the remaining 63 cases were diagnosed on clinical basis including barium enema or surgical gross findings. On histologic examination, 80 cases(86%) showed typical features of absence of ganglion cell in the myenteric plexus and the 13 cases(14%) had atypical features which were segmental absence Of ganglion cell in one case. There we 127 males(82%) and 28 females(18%). The age at diagnosis was younger than 30 days in 87 cases(56%), I month to 1 year in 39 cases(25%) and older than I year in 29 cases(18%). The levels of aganglionosis were variable: short segment (rectosigmoid) in 134 cases(86%), intermediate segment (more proximal colon) in 14 cases(100%). and 6 cases(4%) had total aganglionosis. Common clinical presentations were abdominal distention, delayed meconium passage or bilious vomiting in neonate, and chronic constipation in infancy or childhood. Following initial colostomy or ileostomy, a definitive procedure was performed in 151 cases(Duhamel type in 150 cases; Soave type in 2 cases; Swenson type in 3 cases). Frequently associated problems after definitive procedure were persistent constipation(ll%) due to septum formation, fecaloma, remnant aganglionic segment and rectal stenosis. Overall mortality rate was 4%, and increased mortality was associated with enterocolitis(14%) which was the most frequent cause of death. The follow-up study longer than 3 months was available in 138 patients who underwent a definitive procedure(mean 2 year 11 months). Seventy-three cases(53%) had normal bowel function, 38cases(27.5%) had occasionally used enema or stool softners, and 27 cases(19.5%) had severe constipation or soiling. The bowel habit improved with time, and were considered normal in 60% of patients after follow-up more than 3 years. The results of definitive procedures for congenital megacolon including Duhamel operation was satisfactory, and long-term follow-up appeared an important and critical component of patients'care.
Child ; Male ; Female ; Infant, Newborn ; Humans ; Mortality ; Follow-Up Studies