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

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

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

No abstract available.
Child* ; Humans ; Tuberculosis*

The functional and morphologic cardiac developments are determined by the morphogenesis, growth and remodeling of the heart resulted from the cell proliferation and apoptosis. We studied the distribution of the proliferation and apoptotic activity of myocardial cells according to the developmental stages in embryos of C57bl/6 mice. Serial histologic sections were stained with PCNA and TUNEL method and were analyzed with image analyzer (BMI, Seoul). The ventricular myocardium of an embryonic heart could be divided into trabecular, inner compact and outer compact layers. Proliferation indices at layers of the left ventricular myocardium on embryonal days (ED) 13, 14, 16, 17 and 18 were 19.9%/47.4%/60.4%, 16.1%/45.8%/60.3%, 24.6%/45.6%/38.1%, 23.3%/17.7%/18.3% and 31.2%/28.0%/19.4% (trabecular/ inner compact/ outer compact) and the right ventricle, 11.0%/34.4%/60.5%, 23.0%/44.0%/69.0%, 29.2%/42.9%/35.1%, 30.4%/30.5%/22.3% and 32.4%/28.4%/16.3%. The apoptotic indices of the left ventricle/VIF were 0.23%/3.66% on ED 13-14, 0.42%/1.31% on ED 16 and 0.05%/0.60% on ED 17-18. The results show that the proliferation of the myocytes was maximal at the outer compact layer on ED 13 and 14 but lowest on ED 17 and 18. This decrease was more pronounced at the left ventricle. The innermost trabecular layer showed a constant proliferation activity of 11.0-32.4%. The presence of spatiotemporal differences in the cell proliferation reveals regional regulation in the developmental timing of cardiac development. Functional maturation is considered to be responsible for the change of proliferation activity. The apoptosis was most frequent and intense in the VIF and crux throughout the periods of each embryonal day where as rarely seen in the ventricular myocardium, especially in the trabecular layer of myocardium. These findings suggest that the apoptosis plays the role in the development of atrioventricular, ventriculoarterial septation and valve formation. Our results also reveal that the participation of apoptosis in formation of the trabeculation can be denied.
Animals ; Apoptosis* ; Cell Proliferation* ; Embryonic Structures ; Heart ; Heart Ventricles ; In Situ Nick-End Labeling ; Mice ; Morphogenesis ; Muscle Cells ; Myocardium ; Proliferating Cell Nuclear Antigen

The pathologic features of acute corrosive esophago-gastritis by ingestion of "Trapunc", a common commercial drain cleansing liquid, is presented. A 37-year-old woman ingested abut 30 ml of Trapunc (3 gm NaOH/100 ml) to commit suicide and received piecemeal esophagectomy and total gastrectomy 9 days after the episode. The esophagus and stomach were extremely friable and necrotic. The most part of the stomach showed acute toxic necrotizing gastritis which was manifested by extensive greenish brown discoloration due to liquefaction necrosis of the mucosa except for a few rugae along the greater curvature. The antrum and distal body revealed severe mucosal detachment and even transmural necrosis. The tissue reaction was basically the same as those of NaOH-induced corrosive esophago-gastritis of acute stage, although it appeared to be severer probably due to sodium hypochlorite, and additive constituent of the ingested cleanser. A unique distribution pattenr of mucosal involvement is discussed.
Female ; Humans

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.
Biopsy* ; Child* ; Humans

The authors developed a computer program for use in report printing as well as data storage and retrieval system at the surgical pathology and its efficacy was evaluated at the Department of Pathology, Seoul National University Hospital. This program used IBM PC XT and was written in DBASE III plus language. The main features of the program included an automatic coding and decoding of the diagnosis, automatic searching of the previous biopsy during gross dictation, powerful word processing function and flexibility of the program. The data storage was carried out during the typewriting of the report, so that the typist's workload became markedly reduced. Two kinds of data files wer stored in the hard disk ; the temporary file contained full informations and the permanent file contained the core data only. Searching of a specific case was performed by pathology accession number, chart number, patient's name or by SNOMED code within a second. All the cases were arranged by copied to the diskette during the daily service automatically, with which data were easily restored in case of hard disk failure. The advantages of this program using a persosnal computer were discussed with comparison to those of larger computer system. Based on the experience of 8 months usage in Seoul National University, we assume that this program gives a sufficient solution to the surgical pathology service of many institutes where a large computer system with well designed software is not available yet.
Biopsy