• Title/Summary/Keyword: Gastroenterology

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Successful Treatment with Partial Splenic Embolization in a Case of Post-transplantations Lymphoproliferative Disease with Persistent Hypersplenism (림프세포증식성 질환에서 효과를 보인 부분적 비동맥 색전술 1례)

  • Kim, Mi Jin;Rho, Young Il;Yang, Eun Seok;Moon, Kyung Rye
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.1
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    • pp.92-97
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    • 2004
  • Posttranplantations lymphoproliferative disease (PTLD) is a common and life-threatening complication for soid organ transplantation associated with the use of chronic immunosuppression and Epstein-Barr virus. There is no standardized treatment algorithm, but numerous management strategies are vaiable. Partial splenic embolization (PSE) had been demonstrated to be an effetive alternatie to splenectomy for patients hypersplenism and portal hypertension. PSE has the advantages of non-invasive intervention and resolution of the complications of hypersplenism. We report the effect of the PSE in a 6-year old male liver transplantation recepient with PTLD who has undergone persistent hypersplenism post-transplant. We reduced immunosuppression agent, started antiviral agent. We started with interferon and IV globulin one month after admission. Hepatosplenomegaly and cervical lymphadenopathy were improved. But fever was not subside. We selectively embolized the lower pole of the spleen to achieve a 50~60% reduction in flow as determined by angiography. After embolization, fever subside and peripheral blood findings were improved. Follow up abdominal CT revealed reduced volume of spleen due to ischemic change and there was no multiple enlarged mesenteric lymphnode compared to preembolization state. We thick that PSE is a safe an effetive treatment modality of PTLD with persistent hypersplenism in patients twho failed to medical treatment.

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Treatment of Severe Small Bowel Involvement in Henoch-Schönlein Purpura: Two Cases Report (심한 소장침범을 보인 Henoch-Schönlein Purpura의 치료 2례)

  • Kim, Hyung Tae;Moon, Jin Soo;Jang, Hyun Oh;Jo, Heui Seung;Lee, Jong Guk;Kim, Ki Hong;Seo, Jung Wook;Kim, Min Kyung
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.1
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    • pp.78-82
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    • 2004
  • Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a vasculitis of the small vessels in skin, joints, gastrointestinal (GI) tract and kidney. GI symptoms occur in up to 85% of patients and may lead to severe problems such as intussusception, obstruction, and perforation. GI symptoms may not be easily controlled, showing refractoriness to the conventional corticosteroid therapy. Although GI involvements of HSP are acute, and self-limited in most instances, they may cause fatal results in some unusual cases. In such conditions all the possible therapeutic modalities should be considered. We report two cases of severe small bowel involvement of HSP. One case presented with severe abdominal pain showing refractoriness to corticosteroid, but improved with IV immunoglobulin therapy. In the second case, HSP with transmural infarction in the small bowel could be cured with surgical intervention.

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Long-term Follow-up of a Case of Cowden Syndrome (Cowden 증후군 1례의 장기 추적 관찰)

  • Choi, Seon Yoon;Kim, Heung Sik;Park, Kyung Sik;Lee, Hee Jung;Oh, Hoon Kyu;Hwang, Jin Bok
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.1
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    • pp.112-118
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    • 2004
  • Cowden syndrome is very rare clinical condition with characteristic mucocutaneous lesions associated with abnormalities of the breast, thyroid, and gastrointestinal tract. Here, the case of a 16-year-old girl with diverse gastrointestinal polyposis with breast and thyroid lesions is reported as a definite case of Cowden syndrome. During follow up for 4 years, changes in the characteristic lesions were observed; Esophageal acanthosis and oropharyngeal polyps were newly developed. Gastric, duodenal, rectal and ileal polyps were noted at the first visit when she was 12 years of age and revealed histologically hyperplastic polyps. Mucocutaneous lesions, the pathognomonic finding of Cowden syndrome, were not noted at the first visit or during the follow up period. Breast and thyroid masses were noted at the first visit. Breast aspiration showed highly cellular ductal epithelial fragments and fibroblastic stromal cell fragments, and a thyroid biopsy shows proliferation of irregular sized follicles, with variable colloid contents. Macrocephaly was also noted at the first visit. The characteristic lesions of Cowden syndrome change with the patient's age, and the long-term follow up is recommended in cases suspected with this syndrome.

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Anaphylactic Shock in a Breast Milk-Fed Infant due to Skin Contact with Egg White (모유 수유아에서 발생한 계란 흰자 피부접촉에 의한 아나필락시스 쇽 1례)

  • Kim, Eo Jin;Yoon, Young Ran;Yeom, Jung Sook;Kim, Jum Su;Seo, Ji Hyun;Lim, Jae Young;Choi, Myoung Bum;Park, Chan Hoo;Woo, Hyang Ok;Youn, Hee Shang
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.7 no.1
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    • pp.83-86
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    • 2004
  • Food allergy is not uncommon among small children. Cow milk and eggs are most frequently incriminated as the major cause of food allergy. A 4-month-old female infant who did not have a previous history of contact with the egg developed anaphylactic shock when an emulsion of raw egg white was rubbed on the buttock by her mother to relieve erosive diaper dermatitis. She had been fed on breast milk. She had no past medical history of any other allergy and no family history of atopy, asthma or allergic rhinitis. Her IgE PRIST was 29.46 IU/ml and multiple antigen simultaneous testing chemiluminescent assay for food specific IgE antibody showed a level 4 positive value only to egg white.

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A Case of Solid and Papillary Epithelial Neoplasm of the Pancreas (고형 및 유두상 췌장 종양 1례)

  • Kim, Jong-Keun;Lee, Hyung-Shin;Kim, Sang-Yong;Han, Ji-Whan;Lee, Kyung-Yil;Whang, Kyung-Tai
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.3 no.2
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    • pp.217-221
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    • 2000
  • Solid and papillary epithelial neoplasm of the pancreas is an uncommon low-grade malignant tumor found predominantly in young females. It is rare in childhood. The origin of the tumor is probably from a multipotential stem cell of the pancreas. Neoplasm usually behave like a very low grade malignancy, so that complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. We report a case of solid and papillary epithelial neoplasm of the pancreas in a 14 year -old girl who presented with intermittent abdominal pain and abdominal mass. CT scan shows a large, about $7{\times}6\;cm$ sized, well-marginated hypodense round mass in the head of the pancreas with some ill defined enhancing solid internal portion. She had taken complete excision of the pancereatic mass and Roux-en-Y pancreaticojejunostomy and histologically comfirmed solid and papillary epithelial neoplasm of the pancreas. A brief review of literature was made.

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A Case of Autoimmune Enteropathy Treated with Corticosteroid and FK506 (Corticosteroid와 FK506으로 치료된 자가면역성 장병증 1례)

  • Lee, Mee-Jeong;Jun, Nu-Lee;Choi, Bo-Hwa;Park, Sung-Hye;Kim, Kyung-Mo
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.3 no.2
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    • pp.199-205
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    • 2000
  • Autoimmune enteropathy is a rare chronic diarrheal disease of infancy. Clinicopathologically, this entity is characterized by chronic secretory diarrhea, villous atrophy with crypt hypoplasia of a small intestine and/or associated autoimmune disorders, and absence of severe immunodeficiency. For the confirmation of diagnosis, antienterocyte autoantibody should be delineated. The treatment of choice of this disorder is immunosuppression. We has been experienced a case of autoimmune enteropathy without autoimmune disorders in a 10-month-old male infant. He developed protracted diarrhea from 5 months of his age and has been appeared to be failure to thrive. Antienterocyte autoantibody was demonstrated by immunohistochemistry and western blotting. He was successfully treated with corticosteroid and FK506. This is the first case report of autoimmune enteropathy without autoimmune disorders in Korea.

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Asymptomatic Bile Duct Dilatation in Children: Is It a Disease?

  • Son, Yeo Ju;Lee, Mi Jung;Koh, Hong;Kim, Seung
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.18 no.3
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    • pp.180-186
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    • 2015
  • Purpose: Bile duct dilatation is a relatively common sonographic finding; nevertheless, its clinical significance in children is controversial because little research has been done in the area. Therefore, we investigated the natural course and clinical significance of biliary duct dilatation in children. Methods: We performed a retrospective study of 181 children (range, 1-day-old to 17-year-old) in whom dilatation of the intrahepatic duct and/or common hepatic duct and/or common bile duct was detected by abdominal ultrasonography at the Severance Children's Hospital between November 2005 and March 2014. We reviewed and analyzed laboratory test results, clinical manifestations, and clinical course in these patients. Results: Pediatric patients (n=181) were enrolled in the study and divided into two groups. The first group included 59 subjects, without definitive cause of bile duct dilatation, who did not require treatment; the second group included 122 subjects, with definitive cause of bile duct dilatation or underlying biliary disease, who did require treatment. In the first group, 24 patients (40.7%) showed spontaneous resolution of bile duct dilatation, 20 patients (33.9%) showed no change, and 15 patients (25.4%) were lost to follow-up. In the second group, 31 patients were diagnosed with choledochal cysts, and 91 patients presented with biliary tract dilatations due to secondary causes, such as gallbladder or liver disease, post-operative complications, or malignancy. Conclusion: Biliary dilatation in pediatric patients without symptoms, and without laboratory and other sonographic abnormalities, showed a benign clinical course. No pathologic conditions were noted on follow-up ultrasonography.

Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study

  • Lee, Hwa Pyung;Kang, Ben;Choi, So Yoon;Lee, Sanghoon;Lee, Suk-Koo;Choe, Yon Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.18 no.3
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    • pp.175-179
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    • 2015
  • Purpose: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy. Methods: This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group). Results: Among the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077). Conclusion: Although overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.

Low Serum Potassium Levels Associated with Disease Severity in Children with Nonalcoholic Fatty Liver Disease

  • Tabbaa, Adam;Shaker, Mina;Lopez, Rocio;Hoshemand, Kazem;Nobili, Valerio;Alkhouri, Naim
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.18 no.3
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    • pp.168-174
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    • 2015
  • Purpose: Recent studies have suggested that decreased serum potassium level may contribute to various metabolic disorders in adult patients including nonalcoholic fatty liver disease (NAFLD). We aimed to study the correlation between serum potassium levels and the histologic severity of NAFLD in children. Methods: Pediatric patients with biopsy-proven NAFLD were included in this study. Demographic, clinical, and histopathological data were obtained. Multivariable logistic regression analysis was used to assess whether potassium levels are associated with the presence of nonalcoholic steatohepatitis (NASH) or fibrosis after adjusting for possible confounders. A p-value <0.05 was considered statistically significant. Results: Among 125 biopsies, 49.6% (62) had evidence of NASH while 66.4% (83) had some degree of fibrosis (stage 1-3). Mean serum potassium was significantly lower in NASH group as compared to non-NASH group ($4.4{\pm}0.42mmoL/L$ vs. $4.8{\pm}0.21$, p<0.001). Higher potassium level had negative correlation with presence of steatosis, ballooning, lobular inflammation, fibrosis and NAFLD activity score (p<0.05). On multivariable analysis and after adjusting for the metabolic syndrome and insulin resistance, higher potassium level was significantly associated with lower likelihood of having a histological diagnosis of NASH on biopsy (odds ratio [OR], 0.12; 95% confidence interval [95% CI], 0.05-0.28; p<0.001). Similarly, the likelihood of having fibrosis decreases by 76% for every 0.5 mmoL/L increase in potassium (OR, 0.24; 95% CI, 0.11-0.54; p<0.001). Conclusion: Our study shows an inverse relationship between serum potassium levels and the presence of aggressive disease (NASH and fibrosis) in children with NAFLD.

Associations among the Degree of Nonalcoholic Fatty Liver Disease, Metabolic Syndrome, Degree of Obesity in Children, and Parental Obesity

  • Oh, Min-Su;Kim, Sorina;Jang, Joon-Hyuck;Park, Jong Yoon;Kang, Hyun-Sik;Lee, Mu Sook;Kang, Ki Soo
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.19 no.3
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    • pp.199-206
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    • 2016
  • Purpose: To analyze the associations among the degrees of nonalcoholic fatty liver disease (NAFLD) by ultrasonography and metabolic syndrome, degrees of obesity in children, and degrees of parental obesity. Methods: A total of 198 children with obesity who visited a pediatric obesity clinic were prospectively enrolled in this study. The severity of NAFLD based on ultrasonography was classified into no, mild, moderate, or severe NAFLD group. The degree of obesity based on the percentage over standard weight for height per sex was classified into mild, moderate, or severe. Results: Of 132 patients evaluated for the degree of NAFLD and metabolic syndrome, the p-value of correlation between the two factors was 0.009. Therefore, metabolic syndrome might significantly affect the degree of NAFLD. Of 158 patients evaluated for the degree of NAFLD and the degree of obesity, the p-value of correlation between the two factors was 0.122. Of 154 patients evaluated for the degree of obesity and father's obesity, the p-value was 0.076. Of 159 patients evaluated for the degree of obesity and mother's obesity, the p-value was 0.000, indicating that mother's obesity could significantly affect the degree of obesity in children. Of 142 patients evaluated for the degree of obesity and metabolic syndrome, the p-value was 0.288. Conclusion: Metabolic syndrome might significantly affect the degree of nonalcoholic fatty liver in children. In addition, mother's obesity might be a significant factor that affects the degree of obesity in children.