• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권2호
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• pp.174-179
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• 2020

We present an 8 years old girl who was diagnosed at 6 months of age of Progressive Familial Intrahepatic Cholestasis type 2. Although liver transplantation (LT) was classically considered curative for these patients, cholestasis recurrence with normal gamma-glutamyl transpeptidase (GGT), mediated by anti-bile salt export pump (BSEP) antibodies after LT (auto-antibody Induced BSEP Deficiency, AIBD) has been recently reported. Our patient underwent LT at 14 months. During her evolution, patient presented three episodes of acute rejection. Seven years after the LT, the patient presented pruritus with cholestasis and elevation of liver enzymes with persistent normal GGT. Liver biopsy showed intrahepatic cholestasis and giant-cell transformation with very low BSEP activity. Auto-antibodies against BSEP were detected therefore an AIBD was diagnosed. She was treated with Rituximab and immunoadsorption with resolution of the AIBD. As a complication of the treatment she developed a pneumocystis infection successfully treated with corticoids, cotrimoxazol and anidulafungin.

• 대한장애인치과학회지
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• 제7권1호
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• pp.29-32
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• 2011

상부위장관 이물질의 섭취는 대부분 위장관에서 자연적으로 배출되지만, 20% 정도에서는 내시경 또는 수술적 제거의 적응증이 된다. 특히 치과치료중 소아가 삼킬 수 있는 여러 가지 기구 중 bur 등은 예리하고 길이가 길기 때문에 생리적 협착 부위에서 걸려 자연배출이 힘들 수 있다. 또한시간이 지날수록 식도벽에 고착되고 궤양 등의 합병증을 일으켜 내시경으로 제거하기가 어렵기 때문에 소아가 삼켰을 경우 신속한 대처가 필요하겠다.

• Advances in pediatric surgery
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• 제13권2호
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• pp.105-111
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• 2007

We reviewed the records of 25 patients who were re-operated upon after primary repair of esophageal atresia with or without fistula at the Department of Pediatric Surgery, Seoul National University Children's Hospital, from January 1997 to March 2007. Types of the esophageal atresia anomalies were Gross type A in 5 patients, C in 18, and E in 2. The indications for re-operation were anastomosis stricture (n = 14), tracheo-bronchial remnant (n = 4), persistent anastomosis leakage (n = 3), recurrent tracheo-esophageal fistula (n = 2) and esophageal web (n = 2). The interval between primary and secondary surgery was from 48 days to 26 years 5 months (mean: 2 years and 4 months). Four patients required a third operation. The interval between the second and third operation was between 1 year 1 month and 3 year 10 month (mean: 2 years 5 months). Mean follow up period after last operation was 35 months (1 years-8 years 6 months). The secondary surgery was end-to-end esophageal anastomosis in 15, esophagoplasty in 5, gastric tube replacement in 5. After secondary operation, 6 patients had anastomosis stricture (4 patients were relieved of the symptoms by balloon dilatation, 2 patients underwent tertiary operation). Five patients had leakage (sealed on conservative management in all). Two patients had recurrent tracheo-esophagel fistula (1 patient received chemical cauterization and 1 patient underwent tertiary operation). Currently, only one patient has feeding problems. There were no mortalities. Secondary esophageal surgery after primary surgery for esophageal atresia was effective and safe, should be positively considered when complications do not respond to nonoperative therapy.

• 대한치과마취과학회지
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• 제13권4호
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• pp.215-220
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• 2013

The co-occurrence of congenital central hypoventilation syndrome (CCHS) and Hirschsprung's disease (HD) is termed Haddad syndrome, which is an extremely rare discorder. It was reported first by Haddad in 1978 and there are approximately 60 cases reported in the worldwide literature. Recent studies described that congenital central hypoventilation syndrome had deep relation to the mutation of the PHOX2B gene in its diagnosis and phenotype. This article presents a case report: Dental treatment of a 3-year-old male patient with Haddad syndrome under outpatient general anesthesia. The special considerations of dental care, especially caries theatment of the patient with Haddad syndrome are discussed.

• Clinical and Experimental Pediatrics
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• 제63권11호
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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.

• Journal of Dental Anesthesia and Pain Medicine
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• 제22권4호
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• pp.239-254
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• 2022

The high success rate of dental treatment is dependent on the cooperation of pediatric patients during procedures. Dental treatment often causes pain, particularly in children. The factors in providing treatment to pediatric patients include the characteristics and location of the tooth, profoundness of the anesthesia including the type of local anesthetic, and cooperation of the patient. Previous studies have examined several techniques to successfully achieve profound pulpal anesthesia in maxillary permanent teeth. The dentist should select the injection technique to be used based on patient needs. In children, either buccal with palatal injections or buccal with intra-septal injections may be used to anesthetize the permanent maxillary first molar. Buccal with palatal injections are commonly used prior to routine maxillary dental procedures. Currently, there are only a few studies on the employment of buccal with intra-septal injections to anesthetize permanent maxillary first molars in pediatric patients. This review will focus on efficacy of buccal with palatal versus buccal with intra-septal pulpal anesthesia of the permanent maxillary first molars in pediatric patients and aim to determine which technique should be used during routine dental procedures.

• Advances in pediatric surgery
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• 제13권1호
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• pp.76-80
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• 2007

The authors applied anterior sagittal transrectal apporach (ASTRA) for the repair of urethrovaginal fistula which developed after total repair of persistent cloaca. The patient had been diagnosed to have persistent cloaca, double uterus and double vagina, and received PSARP, excision of right-side uterus and vagina, and left vaginal switch operation at 22 months old. After operation, the patient admitted several times due to frequent urinary tract infection and ectopic stone formation in bladder and neovagina. Urethro-neovaginal fistula was confirmed by cystoscopy and corrected with ASTRA. Postoperative voiding cystourethrogram showed no fistula tract. ASTRA showed improved surgical field, minimized ureterocystic damage, and preserved perirectal nerve due to limited incision of rectum.

• Advances in pediatric surgery
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• 제15권1호
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• pp.64-67
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• 2009

Splenic cystic lesion is uncommon in children, and cystic lymphangioma of the spleen has not been reported in Korean pediatric patients. Here we report a case of cystic lymphangioma arising from the spleen in a 16 year-old male. The patient presented with left flank pain for 5 days after blunt trauma to the same site. On physical examination, left abdominal tenderness and a palpable spleen were noted. Abdominal ultrasound and MRI revealed multiple septated macro-cystic mass abutting to the spleen medially, suggestive of cystic lymphangioma of the spleen. Laparotomy revealed a 20 cm sized cyst in the spleen, and 2,000mL of dark-brownish fluid was aspirated from the cyst. Splenectomy was performed. Pathological examination revealed the cystic lymphangioma. Post-operative recovery was uneventful, and the patient was discharged at 7 days after surgery.

• Advances in pediatric surgery
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• 제17권1호
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• pp.58-64
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• 2011

Minimally invasive techniques for pediatric inguinal hernia repair have been evolving in recent years. We applied the laparoscopic method to repair pediatric inguinal hernia using the techniques of sac transection and intra-corporeal ligation. Between November 2008 and August 2010, 67 pediatric patients (47 boys and 20 girls) with inguinal hernias were included in this study. Postoperative activities, pain, and complication were checked prospectively at regular follow-up. One patient presented with clinically bilateral hernia, and three patients had metachronous hernias. Thirty-two cases out of 63 patients with unilateral hernias had a patent processus vaginalis on the contralateral side. Mean operation time was $35{\pm}11.4$ minutes for unilateral hernias and $43{\pm}11$ minutes for bilateral hernias. There were no intra-operative complications. One patient had a small hematoma on the groin postoperatively, which subsided spontaneously in a week. Recurrence and metachronous hernia were not found at follow up. In summary, laparoscopic inguinal repair in children is safe, easy to perform and has an additional advantage of contralateral exploration. Further studies should include long term follow-up.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제23권3호
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• pp.304-309
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• 2020

An 18-year-old woman with annular pancreas and duodenal duplication presented with recurrent acute pancreatitis and underwent a resection of duodenal duplication. However, the patient experienced recurrent abdominal pain after resection. Abdominal computed tomography and magnetic resonance imaging showed a dilatation of the peripheral pancreatic duct and stenosis and malformation of both the Wirsung's and Santorini's duct due to multiple stones. The modified puestow procedure was performed. The main pancreatic ducts in the body and tail were opened, and the intrapancreatic common bile duct was preserved. A Roux-en-Y pancreatico-jejunostomy was performed for reconstructing the pancreaticobiliary system after removing the ductal protein plug. The patient experienced no abdominal pain, no significant elevation of the serum amylase and lipase levels, and no stone formation during the 2 years of follow-up. This procedure is considered to be beneficial for pediatric patients with chronic pancreatitis due to annular pancreas and duodenal duplication.