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A Case of Secondary Sclerosing Cholangitis in Langerhans Cell Histiocytosis.

Ja Hyung KIM ; Bo Hwa CHOI ; Kyung Mo KIM ; Hyung Nam MOON

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):120-124.

Sclerosing cholangitis is a chronic cholestatic disease characterized by inflammation and obliterative fibrosis of the bile ducts, leading to biliary cirrhosis and ultimately to liver failure. In children, sclerosing cholangitis is known to be associated with Langerhans cell histiocytosis, as well as with congenital immunodeficiencies and cystic fibrosis. Secondary sclerosing cholangitis is suspected in Langerhans cell histiocytosis with chronic cholestasis, liver dysfunction and portal hypertension. Unlike primary sclerosing cholangitis, the cholangitis associated Langerhans cell histiocytosis is destructive in nature and progresses more rapidly to biliary cirrhosis, therefore uniformly the prognosis is poor. In this setting, liver transplantation should be considered early in children with sclerosing cholangitis complicating Langerhans cell histiocytosis before end-stage liver failure and variceal bleeding. We experienced a case of secondary sclerosing cholangitis in Langerhans cell histiocytosis in a 2-year-old boy. We report this case with brief review of the related literatures.
Bile Ducts ; Child ; Child, Preschool ; Cholangitis ; Cholangitis, Sclerosing* ; Cholestasis ; Cystic Fibrosis ; Esophageal and Gastric Varices ; Fibrosis ; Histiocytosis, Langerhans-Cell* ; Humans ; Hypertension, Portal ; Inflammation ; Liver Cirrhosis, Biliary ; Liver Diseases ; Liver Failure ; Liver Transplantation ; Male ; Prognosis

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A Case of Mesenchymal Hamartoma Associated with Biliary Atresia.

Kyung Dan CHOI ; Jeong Kee SEO ; Eon Woo SHIN ; Jae Sung KO ; Woo Sun KIM ; Young Su PARK ; Kyung Hoon KANG ; Je Geun CHI

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):113-119.

Mesenchymal hamartoma is an uncommon benign tumor usually found in childhood, especially during the first two years of life. The tumor consists of a solid component and multiple cyst. Without treatment, these lesions can grow to an enormous size. We experienced a case of mesenchymal hamartoma which was found incidentally, in a 8 month old girl who had ascending cholangitis after Kasai operation due to biliary atresia. On abdominal ultrasonography and CT, there was a well-defined homogeneous small echogenic mass in the right lobe of the liver with cirrhosis. Five months later, she underwent liver transplantation. Gross picture of the resected liver showed a dark greenish pigmented solid mass in the right lobe of the cirrhotic liver. Microscopic findings showed reactive mesenchyma and epithelial overgrowth.
Biliary Atresia* ; Cholangitis ; Female ; Fibrosis ; Hamartoma* ; Humans ; Infant ; Liver ; Liver Transplantation ; Mesoderm ; Ultrasonography

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A Case of Nonsyndromic Intrahepatic Bile Duct Paucity with Congenital Bilateral Vocal Cord Paralysis and 13q Deletion.

Ju Young CHUNG ; Jeong Soo LEE ; Byung Eoi KIM ; Myung Jai CHOI ; Dong Chul PARK ; Sang Woo KIM ; Kyung Hoon KANG

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):108-112.

Nonsyndromic intrahepatic bile duct paucity is known to be associated with several kinds of etiology such as infection, chromosomal anomaly, metabolic disease and idiopathic. We report a rare case of intrahepatic bile duct paucity with congenital bilateral vocal cord paralysis and 13q deletion.
Bile Ducts, Intrahepatic* ; Metabolic Diseases ; Vocal Cord Paralysis* ; Vocal Cords*

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Intussusception due to Diverticulum of Ileocecal Area: A case report.

In Cheol KIM ; Dae Won SHIN ; Yong Jae CHOI ; En Joo BAE ; Kyeung Ja LEE ; Boung Yoon RYU ; Ji Woong CHO ; Kyung Chan CHOI

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):104-107.

Intussusception is a frequent cause of bowel obstruction in the first five years of life and it is one of the most common surgical emergencies in infancy and early childhood. The age of five months child was administered in Department of Pediatrics of Chunchon Sacred Heart Hospital. His main symptoms were vomiting and high fever for three days. Abdominal sonography, air reduction and abdominal computerized tomography (CT) were performed and the conclusion of these study was intussusception due to cyst mass lesion; duplication cyst, mesenteric cyst or Meckel's diverticulum. He was transferred for operation. We had performed laparotomy for reduction of the intussusception. Operative findings revealed ileocolic type of intussusception due to cystic tumor on ileocecal valve that was invaginated into the cecum, and hyperplasia of the Peyer's patch were seen. But we failed manual reduction because of the tumor in the ileocecal area. So we had performed partial resection of the ileocecum. Diverticulum of the ileum was confirmed by pathologic examination. We experienced unusual cause of the intussusception. So we report this case with a review of the literatures.
Cecum ; Child ; Diverticulum* ; Emergencies ; Fever ; Gangwon-do ; Heart ; Humans ; Hyperplasia ; Ileocecal Valve ; Ileum ; Intussusception* ; Laparotomy ; Meckel Diverticulum ; Mesenteric Cyst ; Pediatrics ; Vomiting

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A Case of Adenomyoma of the Stomach in a Child Presenting Epigastric Pain.

Mi Young HEO ; Ji A JUNG ; Kum Ja CHOI ; Sun Hee SUNG ; Jeong Wan SEO

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):99-103.

Adenomyomas of the stomach are rare tumors characterised by duct/gland-like structures embedded within a smooth muscle stroma. A 5-year-old female patient was admitted to the department of Pediatrics, Ewha Womans University MokDong Hospital with the history of severe epigastric pain and vomiting for 1 day. Esophagogastroduodenoscopy showed bridging fold with central dimpling on posterior wall of prepyloric antrum. Endoscopic biopsy was nondiagnostic. The patient complained epigastric pain continuously and underwent wedge resection. Pathologic examination showed an adenomyoma of the prepyloric antrum. After wedge resection, the patient did not complain epigastric pain during the postoperative follow-up. We report an unusual case of an adenomyoma of stomach.
Adenomyoma* ; Biopsy ; Child* ; Child, Preschool ; Endoscopy, Digestive System ; Female ; Follow-Up Studies ; Humans ; Muscle, Smooth ; Pediatrics ; Stomach* ; Vomiting

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A Case of Dumping Syndrome Following Nissen Fundoplication in an Infant.

Jin Soo MOON ; Hye Ran YANG ; Sun Hwan BAE ; Jae Young KIM ; Jae Sung KO ; Jeong Kee SEO

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):92-98.

The dumping syndrome has been a known complication of gastric surgery in adults, but it is recognized as a very rare disease in the pediatric population, especially in Korea. We report a case of dumping syndrome in a 10-month-old infant, who underwent Nissen fundoplication for the treatment of gastroesophageal reflux(GER). He was admitted because of aspiration pneumonia, and diagnosed as GER by 24-hour ambulatory esophageal pH monitoring test. For the treatment of GER, Nissen fundoplication was performed. After the operation, symptoms occurred within 30 minutes of meals, such as diaphoresis, palpitation, weakness, abdominal fullness, nausea, and diarrhea. The gastric emptying scan showed very rapid gastric emptying. His oral glucose tolerance tests revealed early-onset hyperglycema followed by delayed-onset hypoglycemia, which was the characteristic finding of the dumping syndrome. We introduced uncooked cornstarch to resolve symptoms and maintain the serum glucose level. After the feeding of uncooked cornstarch, his symptoms subsided and normal oral glucose test was restored. After the six months of treatment, his weight and height were increased dramatically from below 3 percentiles up to the normal range. The dumping syndrome should be considered when an infant suffers from the feeding difficulties after the gastric surgery like Nissen fundoplication, and the diet therapy including uncooked cornstarch could be applied as an effective measure.
Adult ; Blood Glucose ; Diarrhea ; Diet Therapy ; Dumping Syndrome* ; Esophageal pH Monitoring ; Fundoplication* ; Gastric Emptying ; Gastroesophageal Reflux ; Glucose ; Glucose Tolerance Test ; Humans ; Hypoglycemia ; Infant* ; Korea ; Meals ; Nausea ; Pneumonia, Aspiration ; Rare Diseases ; Reference Values ; Starch

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Assessment of Nutritional Status in Hospitalized Pediatric Patients.

Dong Gon LEE ; Young Ill RHO ; Kyung Rye MOON

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):83-91.

PURPOSE: The aim of this study was to investigate the current prevalence of protein-energy malnutrition (PEM) and the nutritional status of hospitalized pediatric patients. METHODS: We evaluated the nutritional status of the 200 patients from February to July 1994 and the 233 patients from February to July 1999 admitted to Pediatric Department of Chosun University Hospital. Nutritional status was assessed by anthropometric and laboratory data. The nutritional status was classified according to based on the Waterlow criteria and using the laboratory data obtained between 3 days to 5 days after admission. RESULTS: 1) The prevalence of acute PEM (weight for height) was as follows: severe, 0.5%; moderate, 7%; mild, 18%; and none, 74.5% in 1994 and severe, 2.24%; moderate, 3.59%; mild, 19.73%; and none, 74.4% in 1999. 2) The prevalence of chronic PEM (height for age) was as follows: severe, 5%; moderate, 5.5%; mild, 25.5%; and none, 64% in 1994 and severe, 2.24%; moderate, 4.04%; mild, 22.87%; and none, 70.85% in 1999. There was not a statistically significant difference between 1994 and 1999. 3) The prevalence of PEM according to age group, all age group had in general higher prevalence of mild PEM. 4) Values for hemoglobin and albumin were below than total lymphocyte values in PEM. CONCLUSION: The prevalence of acute or chronic PEM was common in hospitalized children. Therefore, the assessment of nutritional status may an important role to establish effective nutritional support and to improve their subsequent hospital course in hospitalized pediatric patient
Child ; Child, Hospitalized ; Humans ; Lymphocytes ; Nutritional Status* ; Nutritional Support ; Prevalence ; Protein-Energy Malnutrition

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Evaluation of Acid Steatocrit in Korean Infants.

Kyo Sun KIM ; Ji He PARK ; Jae Geon SIM

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):77-82.

PURPOSE: The fecal acid steatocrit is an improved steatocrit method for the evaluation of fecal fat. The present study was set up in order to define the normal range of acid steatocrit values during the first 3 months of life. METHODS: Fecal acid steatocrit values were determined in 78 healthy full term and in 21 healthy prematurely born infants between May 1998 and April 2000. The acid steatocrit method was performed in these babies during the first 3 months of life. RESULTS: Steatorrhea occurs during the first month and then decreases, as shown by the fall in the acid steatocrit curve from 1st to 3rd month in our subject. Very high acid steatocrit results (above 90%) were found in all full term and premature infants during the first month of age. Acid steatocrit results of human milk-fed infants were significantly lower than those of formula-fed infants (p=0.0018). CONCLUSION: We conclude that high acid steatocrit results during the first 1 month of age can be due to physiologic steatorrhea. The acid steatocrit micromethod can be used for the evaluation of milk fat absorption in infants and monitoring steatorrhea instead of other more cumbersome methods.
Absorption ; Humans ; Infant* ; Infant, Newborn ; Infant, Premature ; Milk ; Reference Values ; Steatorrhea

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Making Differential Diagnosis of Biliary Atresia Using Endoscopy.

Nam Seon BECK ; I Seok KANG ; Hann TCHAH

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):71-76.

PURPOSE: Many diagnostic modalities for neonatal cholestasis have shown features that are helpful, however until recently none of them are not pathognomonic of biliary atresia (BA). We carried out a prospective study of infants with cholestatic jaundice with the aim of establishing an efficient method of diagnosing BA. METHODS: Twenty-seven consecutive infants with cholestatic jaundice were enrolled in this study and ranged from 7 to 152 days in age (mean; 51.2+/-34.2 days). Gastroduodenoscopy was carried out using a fiberscope (Olympus N30). All the babies were fasted for at least 4 hours before the procedure and 20 ml of 10% dextrose solution was given at the time of endoscopy. The endoscopic examination focused on the 5 minutes observation of the evidence of biliary secretion. If there was lack of the evidence of the biliary secretion, endoscopy was removed and repeated the examination with some pause. RESULTS: There are lack of the evidence of biliary secretion in all infants with BA. In non-BA group, 8 out of the 10 infants showed biliary secretion on the first trial, however one (Alagille syndrome) of the two infants without evidence of biliary secretion, finally exhibited biliary secretion on the second trial. The above observations resulted in the diagnostic accuracy of 96.3% with 100.0% sensitivity and 90.0% specificity. CONCLUSION: In light of the results from our relatively small study, endoscopy is a convenient, and relative inexpensive procedure. we strongly support the use of endoscopy for the diagnosis of BA in the screening and evaluation of infantile cholestasis.
Biliary Atresia* ; Cholestasis ; Diagnosis ; Diagnosis, Differential* ; Endoscopy* ; Glucose ; Humans ; Infant ; Jaundice, Obstructive ; Mass Screening ; Prospective Studies ; Sensitivity and Specificity

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

Sun Hee RIM ; Hye Jin YUN ; Young Mee SUH ; Bo Hwa CHOI ; Kyung Mo KIM ; Young Joo LEE ; Sung Gyu LEE

Korean Journal of Pediatric Gastroenterology and Nutrition.2001;4(1):63-70.

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.
Adolescent ; Child* ; Chungcheongnam-do ; Follow-Up Studies ; Hepatitis ; Humans ; Immunosuppressive Agents ; Liver Cirrhosis ; Liver Diseases ; Liver Transplantation* ; Liver* ; Lymphoproliferative Disorders ; Medical Records

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