• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.20 no.1
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• pp.14-21
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• 2017

Pediatric onset Crohn's disease (CD) tends to have complicated behavior (stricture or penetration) than elderly onset CD at diagnosis. Considering the longer duration of the disease in pediatric patients, the accumulative chance of surgical treatment is higher than in adult onset CD patients. Possible operative indications include perianal CD, intestinal stricture or obstruction, abdominal abscess or fistula, intestinal hemorrhage, neoplastic changes and medically untreatable inflammation. Growth retardation is an operative indication only for pediatric patients. Surgery can affect a patient's clinical course, especially for pediatric CD patient who are growing physically and mentally, so the decision should be made by careful consideration of several factors. The complex and diverse clinical conditions hinder development of a systemized treatment algorithm. Therefore, timing of surgery in pediatric CD patients should be determined with individualized approach by an experienced and well organized multidisciplinary inflammatory bowel disease team. Best long-term outcomes will require proactive post-operative monitoring and therapeutic modifications according to the conditions.

• Clinical and Experimental Pediatrics
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• v.55 no.5
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• pp.171-176
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• 2012

Purpose: The aim of this study was to investigate the natural history of epilepsy and response to anti-epileptic drug treatment in patients with Angelman syndrome (AS) in Korea. Methods: We retrospectively reviewed the clinical records of 14 patients diagnosed with epilepsy out of a total of 17 patients with a genetic diagnosis of AS. These patients were seen at the Department of Pediatric Neurology at Severance Children's Hospital from March 2005 to March 2011. Results: Fourteen (9 males and 5 females) subjects (82.3%) were diagnosed with epilepsy in AS. The most common seizure types were generalized tonic-clonic (n=9, 27%) and myoclonic (n=9, 27%), followed by atonic (n=8, 24%), atypical absence (n=4, 12%) and complex partial seizure (n=3, 9%). The most commonly prescribed antiepileptic drug (AED) was valproic acid (VPA, n=12, 86%), followed by lamotrigine (LTG, n=9, 64%), and topiramate (n=8, 57%). According to questionnaires that determined whether each AED was efficacious or not, VPA had the highest response rate and LTG was associated with the highest rate of seizure exacerbation. Complete control of seizures was achieved in 6 patients. Partial control was achieved in 7 patients, while one patient was not controlled. Conclusion: Epilepsy is observed in the great majority of AS patients. It may have early onset and is often refractory to treatment. There are few reports about epilepsy in AS in Korea. This study will be helpful in understanding epilepsy in AS in Korea.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.4
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• pp.201-212
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• 2023

Purpose: The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs. Methods: The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded. Results: The study included 24 patients (58.3% male; median age [range]: 29 [0.5-204] months). Common clinical presentations included chronic diarrhea (n=8), colitis (n=6), acute hepatitis (n=4), and acute liver failure (n=2). The association of autoimmune diseases, development of malignant diseases, and severe progression of viral diseases was observed in 20.8%, 8.3%, and 16.6% of the patients, respectively. Antibody deficiency was predominantly diagnosed in 29.2% of patients, combined immunodeficiency in 20.8%, immune dysregulation in 12.5%, defects in intrinsic and innate immunity in 4.2%, autoinflammatory disorders in 8.3%, and congenital defects of phagocytes in 4.2%. Five patients remained unclassified (20.8%). Conclusion: Patients with PIDs may initially experience gastrointestinal or liver problems. It is recommended that the association of autoimmune or malignant diseases or severe progression of viral diseases provide pediatric gastroenterologists some suspicion of PIDs. After screening using basic laboratory tests, genetic analysis is mandatory for a definitive diagnosis.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.22
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• pp.9891-9898
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• 2014

Background: This study was planned in an attempt to develop scales for the assessment of fatigue in pediatric oncology patients aged 13-18 and also for their parents. Materials and Methods: In collecting the study data, we used the Child and Parent Information Form, Visual Fatigue Scale, Scale for the Assessment of Fatigue in Pediatric Oncology Patients Aged 13-18 and the Scale for the Assessment of Fatigue in Pediatric Oncology Patients Aged 13-18 for Parents. We also used Pearson correlation analysis, Cronbach alpha coefficient, factor analysis and ROC analysis for the study data. Results: In this study, the total Cronbach alpha value of the parent form was 0.99, the total factor load was 0.72-0.94 with 95% the total variance being explained. The cutoff point of the parent form is 73 points. The total Cronbach alpha value of the child form was 0.99, the total factor load was 0.82-0.95, with 89.4% of the total variance being explained. The cutoff point of the child form was 75.5 points. Conclusions: This study suggests that the Scale for the Assessment of Fatigue in Pediatric Oncology Patients Aged 13-18 and the Scale for the Assessment of Fatigue in Pediatric Oncology Patients Aged 13-18 for Parents are valid and reliable instruments in assessing the fatigue symptoms of children in Turkey.

• Clinical and Experimental Pediatrics
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• v.63 no.11
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• pp.447-450
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• 2020

Background: Esophageal stricture (ES) is an uncommon clinic entity in pediatrics that may be congenital or acquired in childhood. Acquired noncaustic ES is very rare, and clinical features of affected patients are unknown. Purpose: We aimed to evaluate the clinical findings, and outcomes of patients with acquired noncaustic ES to aid physicians in the early referral of patients to gastroenterologists. Methods: The medical data of patients with acquired noncaustic ES who were followed in our gastroenterology clinic between January 2009 and December 2019 were reviewed. Results: Acquired noncaustic ES was found in 12 of the 4,950 patients (0.24%) who underwent endoscopy during the study period. The main symptoms were dysphagia (58.3%), vomiting (33.3%), and chronic anemia (8.3%). Chronic malnutrition and underweight were found in 66.6% of the patients. The most common etiological factors were radiotherapy, peptic reflux, and achalasia (16.6%, each), while chemotherapy, squamous-cell carcinoma (SC) of the esophagus, eosinophilic esophagitis (EoE), esophageal web, epidermolysis bullosa, and esophageal diverticulum (8.2%, each) were the other etiological factors. Patients with EoE underwent endoscopic bougie dilation in addition to steroid use and elimination diet. Patients with epidermolysis bullosa and esophageal web underwent bougie dilation. Patients with peptic reflux-related ES were initially put on antireflux therapy, but during follow-up, one patient required esophageal replacement with colonic interposition. Patients with radiotherapy-related ES recovered with medical therapy. The patient with initially underwent surgical gastrostomy and tumoral mass excision. The patient then received chemotherapy and radiotherapy and underwent jejunal interposition. Patients with achalasia underwent surgical esophagomyotomy. Conclusion: The presence of solid dysphagia, malnutrition, and an associated disease may alert physicians to the presence of ES.

• Child Health Nursing Research
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• v.6 no.1
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• pp.5-17
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• 2000

The purpose of this study was to provide qualified nursing care to examine the degree of satisfaction with nursing care of pediatric patients visiting emergency center. Data were collected from March, 8 to June, 5, 1999 from 304 subjects including 2 University hospitals located in Seoul. Using the 23 item questionnaire, which made out by Davis CHECSS tool through modification and supplement by researchers. The collected data were scored by use of means and standard deviations according to the degree of satisfaction with nursing care of pediatric patients visiting emergency center and each item as an independent variable was analysed by t-test and ANOVA test. The results are as follows. 1. The degree of satisfaction with the nursing care of pediatric patients visiting emergency center showed as an average of 3.69. 2. With the resept of general characteristics related to pediatric patients visiting emergency center, there were statiscally significant difference in duration of stay at emergency center(F=2.908, p=.014), type of disease(F=1.777, p=.046). The degree of satisfaction with the nursing care of pediatric patients of examiners showed relatively high, but emotional care and information supply are required.

• Advances in pediatric surgery
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• v.18 no.2
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• pp.59-67
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• 2012

Fundoplication is a common surgical procedure for gastroesophageal reflux Disease (GERD). Recently the procedure has been performed with increased frequency laparoscopically. The aim of this study is to compare laparoscopic Nissen fundoplication (LNF) and open Nissen fundoplication (ONF) for GERD in children. We studied retrospectively the 88 pediatric patients who underwent the Nissen fundoplication for GERD as primary antireflux surgery from 1994 and 2009. ONF was performed in 34 cases and LNF was in 54 cases. 58 patients have neurologic impairment. Time to initial food intake after the surgery were reduced in the LNF group (p= 0.032). Recurrent GERD symptom occurred in one patient in LNF group and four patients in ONF group within 1 year after the surgery (p= 0.012). There were no statistically significant differences in post operative morbidity and mortality between both groups. In conclusion, our practice of Nissen fundoplication indicates that LNF takes priority in most pediatric patients.

• Advances in pediatric surgery
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• v.19 no.1
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• pp.1-13
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• 2013

The Korean Association of Pediatric Surgeons (KAPS) performed the second nationwide survey on biliary atresia in 2011. It was a follow-up study to the first survey, which was performed in 2001 for the retrospective analysis of biliary atresia between 1980 and 2000. In the second survey, the authors reviewed and analyzed the clinical data of patients who were treated for biliary atresia by the members of KAPS from 2001 to 2010. A total of 459 patients were registered. Among them, 435 patients primarily underwent the Kasai operation. The mean age of patients who underwent the Kasai operation was $66.2{\pm}28.7$ days, and 89.7% of those patients had type III biliary atresia. Only five patients (1.4%) had complications related to the Kasai operation. After the Kasai operation, 269 (61.8%) of the patients were re-admitted because of cholangitis (79.9%) and varices (20.4%). One hundred and fifty-nine (36.6%) of the patients who underwent the Kasai operation subsequently underwent liver transplantation. The most common cause of subsequent liver transplantation was persistent hyperbilirubinemia. The mean interval between the Kasai operation and liver transplantation was $1.1{\pm}1.3$ years. Overall the 10-year survival rate after the Kasai operation was 92.9% and the 10-year native liver survival rate was 59.8%. We had 23 patients for primary liver transplantation without the Kasai operation. The mean age patients who underwent primary liver transplantation was $8.6{\pm}2.9$ months. In summary, among the 458 Kasai-operation and liver-transplantation patients, 373 lived, 31 died, and 54 were unavailable for follow up. One-third of the patient who survived have had complications correlated with biliary atresia. In comparison with the first survey, this study showed a higher survival rate and a greater number of liver transplantation.

• Advances in pediatric surgery
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• v.9 no.1
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• pp.45-51
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• 2003

A nationwide survey on choledochal cyst was undertaken among 39 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and the case registration form for each patient during the five year period of 1997 to 2001. Three hundred and forty eight patients were registered from 32 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:3.4. The age of patients on diagnosis was $49.0{\pm}44.4$ months. The geographic distribution was 34.8% in Seoul and Kyoungki-do, 33.3% in Kyoungsang-do, 17.9% in Cholla-do, and 8.5% in Choongchung-do, in order of frequency. The three common clinical presentations were abdominal pain (63.8%), vomiting (35.3%), and jaundice (29.1%). Only seven patients (2%) presented with classic triad, and 25 patients were diagnosed by antenatal ultrasonographic examination. According to the Todani Classification, 238 patients (7l.3%) were type 1, 3 (0.9%) type 11, and 93 (27.8%) type IV. At the time of the operation, three important associated conditions were choledocholithiasis in 45 patients (15.1%), liver fibrosis (Grade 1-4) in 35, and previous operative procedure for biliary diseases in 10. Associated anomalies were observed in 13 patients (3.8%). Three hundred thirty nine (98.8%) of 343 lesions were treated by Cyst excision and Roux-Y hepaticoiejunostomy. One hundred seventy-six patients had an anomalous arrangement of the pancreatobiliary ductal system (APBD): APBD was not in 92 patients, biliary duct joined to the pancreatic duct in 51, and pancreatic duct joined to the biliary duct in 26. There were 8.5% early, and 7.7% late phase operative complications. The major complications were bleeding, anastomotic leakage, and acute pancreatitis. The combination of acute abdomen and choledochal cyst may suggest spontaneous rupture. Because of the development of late intrahepatic bile duct stones, long term follow up after cyst excision and hepaticojejunostomy is required. The optimal time of surgical intervention should also be considered in the situation of routine use of antenatal ultrasonographic examination. This is the first review of the choledochal cyst in Korea and provides baseline data for future comparisons.

• Advances in pediatric surgery
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• v.15 no.2
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• pp.121-131
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• 2009

Intestinal malrotation presents with different clinical characteristics, depending upon the age of the patient. The medical records of 44 patients treated for intestinal malrotation with/without midgut volvuls between January 2002 and August 2009 at Seoul National University Children's Hospital were reviewed retrospectively. Patients were grouped by the age criteria of 1 and 12 months. Fourteen patients were under 1 month of age (31.8 %), 9 patients between 1 month and 12 months (20.5 %) and 21 patients over 12 months (47.7 %). Twenty patients (45.5 %) presented with volvulus. Vomiting (66 %) and abdominal pain (20 %) were the most common symptoms. UGIS (68.2 %) was the most frequent diagnostic tool. Mean postoperative hospital stay was 13.7 days. There were 7 mild postoperative complications. Volvulus was more frequent in patients under 1 month (p=0.025) than over 1 month. The interval between diagnosis and operation was shorter in patients under 1 month (p=0.003) than in patients over one month of age. In the age between under and over 12 months, volvulus was more common in those under 12 months of age, but the difference was not significant. The interval from diagnosis to operation was shorter in patients under 12 months than over 12 months of age (p=0.001). Vomiting was the most frequent symptom in patients under 12 months. On the other hand, abdominal pain was the most frequent symptom in patients over 12 months. In conclusion, patients with intestinal malrotation had age-related differences in the presence of midgut volvulus, the interval between diagnosis and operation, and clinical symptoms. The age of the patient should be considered in order to determine adequate treatment of malrotation.