• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.257-262
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• 2005

Clinical findings in neonates and infants with cholestasis characteristically include prolonged jaundice, acholic stool, pruritus and failure to thrive. We report two cases of cholestasis presenting with spontaneous bleeding due to vitamin K deficiency. Laboratory studies on admission revealed moderate liver dysfunction and a bleeding tendency due to vitamin K deficiency. After administration of vitamin K, the bleeding tendency disappeared. Vitamin K deficiency was resulting from a combination of cholestasis-induced fat malabsorption and low vitamin K supplementation due to breast-milk feeding. Cholestasis should be considered in neonate and infant with a bleeding tendency.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.105-109
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• 2000

X-linked agammaglobulinemia (XLA) is a primary inherited B-cell immunodeficiency. The prevalence of neoplastic disease in patients with XLA is approximatedly 0.7%. The most frequent tumor is lymphoreticular malignancy. We report a case of hepatocellular carcinoma (HCC) in a 13-year-old boy with XLA, after probable maternal transmission of hepatitis B virus. The authors consider that the vertical transmission of hepatitis B virus might play an important role in the development of HCC in a child with XLA who could not eliminate hepatitis B virus effectively.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.93-97
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• 2000

We experienced a case of pathologic aerophagia in a 10-year-old girl who has mental retardation. It was observed that the abdomen was non-distended in the morning and became maximally distended in the evening. Increased passage of flatus, but normal eructation, was noted. Roentgenographic examination showed 'esophageal air sign', abnormal air shadow on proximal esophagus adjacent to the trachea, in simple chest X-ray and visible air swallowing fluoroscopically. We tried a percutaneous endoscopic gastrostomy (PEG) to decompress swallowed air in stomach. We suggest that placement of a PEG catheter in early life, especially in mentally retarded patients, that can be used as desufflator, can prevent the complications of aerophagia. 'Esophageal air sign' may be very helpful for early detection of pathologic aerophagia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.89-92
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• 2000

Acute gastric volvulus is uncommon but surgically emergent. Normally, the stomach is held in position by four ligaments: gastrophrenic, gastrohepatic, gastrosplenic, and gastrocolic. In addition, relative fixation of the pylorus and esophagus provides further anchorage. A normal diaphragm also helps to prevent abnormal displacement of abdominal viscera and development of gastric volvulus. Volvulus may be organoaxial, mesenteroaxial, or a combination of both. Organoaxial volvulus is the rotation of the stomach around an axis extending from the hiatus of the diaphragm to the pylorus. Mesenteroaxial volvulus is the rotation of the stomach around an axis transecting the lesser and greater curvatures of the stomach. The symptoms of gastric volvulus depend on its type, the extent and degree of rotation and obstruction, and associated defects. Classic clinical features of acute gastric volvulus, as by Borchardt in 1904, include unproductive retching, acute, localized epigastric distention, and the inability to pass a NG tube. The presence and severity of these features depend on the degree of gastric obstruction of both the gastroesophageal junction and pyloric outlet. It may be suspected on plain abdominal radiographs and usually confirmed by upper gastrointestinal series. Acute volvulus requires immediate surgical repair, fixation to avoid recurrence, and correction of any underlying anatomic abnormality. Any associate defect should be repaired and the stomach must be fixed. The authors report a case of an 3-year-old girl who had a mesenterioaxial gastric volvulus.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.75-83
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• 2000

Cyclic vomiting syndrome(CVS) is a disorder of unknown etiology that is characterized by its clinical pattern of intermittent episodes of severe vomiting, similar in time of onset and duration, with no symptoms during the intervening period. By definition, CVS is an idiopathic disorder that requires exclusionary laboratory testing. Not only can it be mimicked by many specific disorders, eg, surgical, neurologic, endocrine, metabolic, renal, but within idiopathic CVS there may be specific subgroups that have different mechanisms. It has been reported that CVS usually begins in toddlers and resolves during adolescence. Migraine is also self-limiting episodic condition of children and the clinical features of migraine and CVS show considerable similarity. It is proposed that CVS is a condition related to migraine. This paper reports clinical courses of long term follow-up and reversible EEG changes in three patients whose history included CVS. Clinical situations of attack interval, duration and associated symptoms had changed variablely in each patients through long term follow-up period. Cyclic vomiting subsided in two cases. Abnormal delta activity was seen during episodes and resolved at follow-up, when the patient asymptomatic. The brain wave changes support the interpretation of CVS as a migraine variant.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.47-55
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• 2000

Purpose: The aim of this research is to examine the abnormal liver function complicated with Kawasaki disease (KD) and to analyze its clinical characteristics and complications. Methods: Ninty eight cases were diagnosed as having KD, among which thirty four cases had abnormal alanine aminotransferase (ALT) (>30 IU/L). These abnormal ALT cases were evaluated in terms of age and sex distribution, major symptoms, complications, laboratory and histopathologic findings. Results: Male to female ratio was 2.4:1 and most patients (91.2%) were under 5 years of age. Cervical lymphadenopathy was observed in 3 cases (8.8%); and recurred cases were two (5.8%). Average fever duration was 8 days; average length of hospitalization was 9 days; and average recovery period was 13 days. Immediately after admission, positive CRP was observed in 31 cases (91.2%), leukocytosis (> $10,000/mm^3$) in 26 cases (76.0%), thrombocytosis (> $450,000/mm^3$) in 7 cases (20.6%), and anemia (<10 gm/dl) in 7 cases (20.6%), respectively. GB hydrops or cholecystitis were noted in 3 cases (8.8%), abnormal ECG finding in 1 case (2.9%), coronary dilatation or aneurysm in 2 cases (5.9%). Liver biopsy was done in four cases and revealed mild infiltration of lymphocytes on the portal area and mild bile duct proliferations. Conclusion: The abnormal liver function was noted in 34.7% of KD patients, and subsided all within one month. But the liver function test should be checked closely in patients of the abnormal liver function test complicated with KD despite of its good prognosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.208-214
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• 2003

Juvenile polyposis is an uncommon condition characterized by the development of multiple juvenile polyps predominantly in the colon but also in the rest of the gastrointestinal tract. Patients with juvenile polyposis commonly present with rectal bleeding, diarrhea, abdominal pain, anemia, prolapse of the polyp. We experienced a juvenile polyposis in a 7 year-old male patient with protein losing enteropathy who was diagnosed by $^{99M}Tc$-human serum albumin abdominal scintigraphy, colonoscopy, and small bowel series. Proctocolectomy with ileostomy was performed and then protein losing enteropathy was resolved.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.6 no.2
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• pp.202-207
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• 2003

Intestinal neuronal dysplasia (IND) is a disorder of abnormal intestinal innervation resulting in dysfunctional colonic motility. IND shares clinical features with Hirschsprung's disease but differentiated by histological findings such as hyperplasia of submucosal and myenteric plexuses, giant ganglia, ectopic ganglion cell and increased acetylcholinesterase activity in lamina propria. Although IND may exist as an isolated condition, more commonly, it occurs in association with Hirschsprung's disease. We report a case of twins affected with IND. Both children manifested with delayed passage of meconium and severe abdominal distention after birth. Barium enema in both patients showed microcolon. They underwent emergency ileostomy under the impression of total aganglionosis. But surgical biopsy specimens showed hyperganglionosis in submucosa with formation of giant ganglia. Both neonates suffers from several episodes of peudo-obstruction after the repair operation of colostomy.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.1
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• pp.86-90
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• 2011

Wilson's disease is an autosomal recessive disorder of copper metabolism consequence of which leads to accumulation of copper in the liver, brain, cornea and other tissues. The manifestations are more likely to be hepatic in the early childhood and neurological in the adolescents. In addition, the abnormalities that develop during disease progression may result in other manifestations such as hematologic, endocrine, or renal findings. We report a thirteen year-old girl who manifested tetany shortly after the initial diagnosis of Wilson's disease. Despite aggressive calcium, magnesium and vitamin D replacement, the hypocalcemia and hypomagnesemia did not respond to the therapy promptly. It took more than three weeks for blood levels of the minerals to be normal. We concluded that tetany occurred in our patient because of hypoparathyroidism as a rare complication of Wilson disease, vitamin D deficiency resulting from various conditions, and inconclusive hypomagnesemia.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.15 no.1
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• pp.38-43
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• 2012

Purpose: Epstein-Barr virus (EBV) hepatitis is a usually asymptomatic and self-limiting disease in immunocompetent patients. However, the range of severity is wide, and the serological diagnosis is typically difficult until the convalescent phase. Thus, we examined the value of plasma EBV DNA real-time quantitative polymerase chain reaction (RT-qPCR) in EBV hepatitis for the timely diagnosis and the relationship between EBV viral load and clinical severity. Methods: Sixty samples were confirmed as having EBV infection by RT-qPCR with the EBV BALF5 gene sequence. We examined the clinical characteristics of EBV hepatitis by reviewing medical records. Results: The median total duration of fever was 8 days (range: 0-13 days). The mean peak value of aspartate aminotransferase (AST) was $241{\pm}214$ U/L, and the mean peak value of alanine aminotransferase (ALT) was $298{\pm}312$ U/L. There was no correlation between the serum levels of liver enzyme and plasma EBV DNA titer ($p$=0.1) or between median total duration of fever and EBV DNA titer ($p$=0.056). The median age of the EBV VCA IgM-negative group was lower compared with the EBV VCA IgM-positive group in EBV hepatitis (2 years vs. 6 years, $p$=0.0009). Conclusion: The severity of EBV hepatitis does not correlate with circulating EBV DNA load according to our data. Furthermore, we suggest that plasma EBV PCR may be valuable in young infants in whom the results of serology test for EBV infection commonly are negative.