• Advances in pediatric surgery
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• v.9 no.1
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• pp.35-40
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• 2003

Inguinal hernia is the most frequent problem requiring surgery in children. Moreover, subsequent contralateral occurrence after repair of the symptomatic unilateral inguinal hernia(UIH) is not rare, This study is to evaluate the diagnostic value of inguinal ultrasonography (IUS) for potential bilateral inguinal hernia (BIH). A prospective study was performed for preschool children less than 6 years of age who were diagnosed as UIH from July 1999 to December 2000. We selected 58 cases with potential BIH, based on the past history, such as prematurity, ventriculo peritoneal shunt, family history of BIH. hernia on the left side (LIH), age below 2, female, and contralateral positive silk glove sign on the physical examination, Screening with IUS and bilateral surgical exploration were applied on these cases. Forty-seven cases were males (81.0%) and 11 cases were females (19.0%). Thirty four were infants. Symptomatic right inguinal hernia (RIH) were 28 (48.3%), and LIH were 30 cases (51.7%). Six cases had no evidence of contralateral patent process vaginalis (PPV) by IUS but showed contralateral PPV by operation. Two cases were suspicious to contralateral PPV under IUS, but operative findings were negative. Fifty cases showed contralateral PPV by IUS as well as operation. The detection rate of contralateral PPV under IUS was 86.2%. The preoperative IUS may reduce contralateral exploration.

• Advances in pediatric surgery
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• v.14 no.1
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• pp.1-11
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• 2008

The aim of this study is to analyze the outcomes of the esophageal atresia with tracheoesophageal fistula over the last 2 decades. The records of 51 patients born between 1987 and 2006 were reviewed. Twenty-seven patients were male. Mean values of the age, gestational age and birth weight were 2.9 days, 296 days and 2.7kg, respectively. All patients had Gross type C anomalies. Thirty-one patients (60.7 %) had one or more associated congenital anomalies and the most common anomaly was cardiac malformation. In 48 cases, primary anastomosis was done and staged operation was done in one case. Circular myotomies in the proximal esophagus were performed in 9 cases. Postoperative complication developed in 26 cases (54 %): pulmonary complication in 12 cases, anastomotic leakage in 10 and anastomotic stricture in 10, recurrent trachoesophageal fistula in one and tracheomalasia in 2 cases. Reoperation was carried out in 2 patients with anastomotic leaks, the remaining leaks were managed non operatively. Three of the strictures were reoperated upon and the others were successfully managed by balloon dilatations. Overall mortality rate was 15.6 %. Mortality rate of the second 10 years (8 %) period decreased significantly compared to that of the first 10 years (23 %) period.

• Child Health Nursing Research
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• v.3 no.2
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• pp.131-141
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• 1997

The purpose of this study was to propose for protection of childhood injury or accident and to provide safety measures. Data was collected from 2,052 who visited emergency room of E University Hospital during 1 year period from January to December, 1996. The results were as follows : 1. The most common type of accident was fall down (53.4%). 2. Male patients outnumbered female by the ratio of 1.68 : 1. 3. The highest incidence rate of accidents were observed in children between 1-3 years age group(34.9%). 4. Accidents were seasonally more frequent in spring(28.2%). 5. Accidents mainly took place at home (63%). 6. The time of accidental occurrence appeared to be predominantly high during a day 8 : 00 PM to 0 : 00 AM (35.l%). 7. The most frequent involved part of body was head and face (74%). 8. Most patients discharged from the hospital in good condition(85.4%). Through clinical analysis, in the relation to accident, there were statistically significant difference in sex, age, season, injury place(all p=0.000).

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.19 no.1
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• pp.71-75
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• 2016

Esophageal candidiasis is commonly seen in immunocompromised patients; however, candida esophagitis induced stricture is a very rare complication. We report the first case of esophageal stricture secondary to candidiasis in a glycogen storage disease (GSD) 1b child. The patient was diagnosed with GSD type 1b by liver biopsy. No mutation was found in the G6PC gene, but SLC37A4 gene sequencing revealed a compound heterozygous mutation (p.R28H and p.W107X, which was a novel mutation). The patient's absolute neutrophil count was continuously under $1,000/{\mu}L$ when he was over 6 years of age. He was admitted frequently for recurrent fever and infection, and frequently received intravenous antibiotics, antifungal agents. He complained of persistent dysphagia beginning at age 7 years. Esophageal stricture and multiple whitish patches were observed by endoscopy and endoscopic biopsy revealed numerous fungal hyphae consistent with candida esophagitis. He received esophageal balloon dilatation four times, and his symptoms improved.

• Korean Journal of Clinical Laboratory Science
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• v.46 no.3
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• pp.79-84
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• 2014

Children and adolescents normally have higher Alkaline phosphatase levels than adults. This study was conducted to provide basic data about pediatric reference intervals for ALP. The data from the 2011 Korean National Health and Nutrition Examination Survey was used. Analysis was done for 847 Children and Adolescents (443 boys and 404 girls) aged 10 to 19. The method of p-NPP & EAE buffer was used to get the data. The highest ALP levels were found in boys aged 12 to 13, the reference interval being 653~1,518 IU/L; the levels decreased for those over 13 years old. In the case of girls, the highest ALP levels were found with those aged between 11 and 12, the reference interval being 463~1,598 IU/L; the levels decreased after 12 years of age. The mean difference in all age groups was statistically significant, except for those aged 10 to 11 and 11 to 12. Therefore, it is suggested that pediatric reference intervals should be divided into three groups according to gender. Complementing data about children and adolescents below 10 years old were not included in this study and will be much needed in the next trial.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.6
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• pp.608-612
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• 2019

Gastric volvulus (GV) is an uncommon pathology, with 10-20% of cases occurring in children, typically before one year of age. It often occurs in people with congenital diaphragmatic hernias, intestinal malrotation, eventration of the diaphragm, paraesophageal hernias, wandering spleens, asplenism, or intra-abdominal adhesions. We report a rare case of chronic GV after left hemihepatectomy for hepatoblastoma in a child. The patient was a 9-year-old boy who complained of upper abdominal pain and postprandial upper abdominal distension for one year. At the age of 4 months, he was diagnosed with hepatoblastoma and had undergone left hemihepatectomy. The upper gastrointestinal contrast study revealed chronic organoaxial gastric volvulus. After a surgical procedure involving adhesiolysis and an anterior wall gastropexy, the patient improved and the symptoms resolved. Although GV is a rare disease, it should be suspected in a patient with a previous abdominal surgical history who is complaining of abdominal distension and pain.

• Annals of Pediatric Endocrinology and Metabolism
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• v.23 no.4
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• pp.229-234
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• 2018

X-linked hypophosphatemic rickets is caused by loss-of-function mutations in PHEX, which encodes a phosphate-regulating endopeptidase homolog. We report a 26-year-old man with X-linked hypophosphatemic rickets who showed decreased serum phosphate accompanied by bilateral genu valgum and short stature. He had received medical treatment with vitamin D (alfacalcidol) and phosphate from the age of 3 to 20 years. He underwent surgery due to valgus deformity at the age of 14 and 15. Targeted gene panel sequencing for Mendelian genes identified a nonsense mutation in PHEX (c.589C>T; p.Gln197Ter) and a mosaic pattern where only 38% of sequence reads showed the variant allele. This mutation was not found in his mother, who had a normal phenotype. This is a case of a sporadic nonsense mutation in PHEX and up to date, this is the first case of a mosaic mutation in PHEX in Korea.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.305-310
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• 2009

Background: The Ross procedure is known as a good surgical option for a young age group with aortic valve problems, but few reports on the Ross procedure are available in the Korean literature. This study is a review of our midterm results of 10 year experience with the pediatric Ross operation in Asan Medical Center. Material and Method: From March 1997 to October 2008, eighteen patients who were aged less than 16 years underwent the Ross procedure. There were 11 males and 7 females. The patients median age was 8.5 years (range: $0.5\sim14.0$). The aortic valve pathophysiology was 6 patients with aortic insufficiency, 4 patients with aortic stenosis, 7 patients with mixed aortic stenoinsufficiencey and 1 patient with infective endocarditis. The valve morphology was bicuspid in 11 and tricuspid in 7. All the patients were operated on with the root replacement technique. All the pumonic valves were replaced with an allograft except for one pericardial monocusp valve. The mean follow up duration was 52.8 months (range: 5.8$\sim$138.2 months). We reviewed the echocardiographic data with focusing on the, auto-graft dysfunction and reoperation. Result: There was no hospital mortality and late mortality. According to the last echocardiographic data, 2 autografts showed aortic regurgitation grade 2, 4 autografts showed aortic regurgitation grade 1 and the others were less than trivial. Reoperation of the pulmonic position conduit was performed 4 times in three patients. The rate of freedom from reoperation at 5 years was 72.2%. On the serial follow up, the Z-values of the aortic annulus/aortic sinus were changed from $1.6{\pm}1.7/0.9{\pm}1.7$ at preoperation to $1.8{\pm}1.6$(p=0.64)/$2.2{\pm}0.9$ (p=0.01) at the last follow-up. There was no significant relation between the growth of the neoaortic root and neoaortic insufficiency. Conclusion: Our midterm results of the Ross procedure in pediatric patients showed good autograft function and growth potential. Vet reoperation due to allograft dysfunction was a major concern.

• Advances in pediatric surgery
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• v.8 no.2
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• pp.143-155
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• 2002

A survey on biliary atresia was made among 26 members of the Korean Association of Pediatric Surgeons. The members were required to complete a questionnaire and a case registration form for each patient during the twentyone-year period of 1980-2000. Three hundred and eighty patients were registered from 18 institutions. The average number of patients per surgeon was one to two every year. The male to female ratio was 1:1.3. The age of patients on diagnosis with biliary atresia was on average $65.4{\pm} 36.2$ days old. The national distribution was 32.8% in Seoul, 25.3% in Gyoungki-Do, 21.6% in Gyoungsang-Do, 9.27% in Choongchung-Do, etc. in order. The most common clinical presentation was jaundice (98.4%) and change of stool color (86.2%) was second. Two hundred eighty (74.7%) of 375 patients were operated by 80 days of age. Three hundred thirty six (9 1.9%) of 366 patients were operated on by the original Kasai procedure, and 305 (84.3%) of 362 patients were observed by bile-drainage postoperatively. The overall postoperative complication rate was 18.5% and the overall postoperative mortality rate was 6.8%. The associated anomalies were observed in 72 cases (22.5%). One hundred ninty five (64.7%) of 302 patients have been alive in follow-up and 49 (25.1%) have survived over 5 years without problem after operation. Ascending cholangitis, varices and ascites affected survival significantly, and the important long-term prognostic factor was the occurrence of complications.

• Advances in pediatric surgery
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• v.4 no.2
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• pp.93-99
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• 1998

Anal endosonography has recently been popularized in adult patients in order to access staging of rectal cancer and other lesions of the anorectum. This study is not familiar to pediatric surgeons. We performed anal endosonography in 30 children without anorectal disease in order to determine the standard morphology of the anorectum. The internal anal sphincter(IAS) was clearly identified as a homogeneous hypoechoic circular band, extending caudally to a level just proximal to the anal verge. The external anal sphincter(EAS) showed mixed echogenicity and different architecture along the anal canal; the EAS was U-shape in the upper canal and it had a circular pattern in the lower canal. In the upper canal of girls, the perineal body and the vagina were found just anterior of the anal canal, which made the U shape of the EAS. In the male, the sphincter tapered anteriorly into two arcs that met in the midline. The perineal body was prominent in the female. Posteriorly, the anococcygeal ligament was represented by a triangular shadow in both sex. The thickness of the IAS was measured in 3 directions, left, right and posterior, at 3 levels, upper, middle and lower areas of the anal canal. The average thickness was 0.86-2.40 mm between 6 and 18 months of age(Group 1), 0.88-3.20 mm between 19 and 36(Group 2), 1.07-2.20 mm between 37 and 54(Group 3) and 1.18-2.42 mm more than 54-month-old(Group 4). The thickness was correlated with the age of the children only in the right upper(p=0.008) and the left middle portion(p=0.015). We were able to obtain standard morphologic features of the normal anal canal in children with anal endosonography. We believe that this technique is a safe and an effective procedure to evaluate anorectal lesions in children.