• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.17 no.3
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• pp.147-154
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• 2014

Purpose: Gender differences in paediatric patients with inflammatory bowel disease (IBD) are frequently reported as a secondary outcome and the results are divergent. To assess gender differences by analysing data collected within the Swiss IBD cohort study database since 2008, related to children with IBD, using the Montreal classification for a systematic approach. Methods: Data on gender, age, anthropometrics, disease location at diagnosis, disease behaviour, and therapy of 196 patients, 105 with Crohn's disease (CD) and 91 with ulcerative or indeterminate colitis (UC/IC) were retrieved and analysed. Results: The crude gender ratio (male : female) of patients with CD diagnosed at <10 years of age was 2.57, the adjusted ratio was 2.42, and in patients with UC/IC it was 0.68 and 0.64 respectively. The non-adjusted gender ratio of patients diagnosed at ${\geq}10$ years was 1.58 for CD and 0.88 for UC/IC. Boys with UC/IC diagnosed <10 years of age had a longer diagnostic delay, and in girls diagnosed with UC/IC >10 years a more important use of azathioprine was observed. No other gender difference was found after analysis of age, disease location and behaviour at diagnosis, duration of disease, familial occurrence of IBD, prevalence of extra-intestinal manifestations, complications, and requirement for surgery. Conclusion: CD in children <10 years affects predominantly boys with a sex ratio of 2.57; the impact of sex-hormones on the development of CD in pre-pubertal male patients should be investigated.

• Child Health Nursing Research
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• v.23 no.4
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• pp.459-469
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• 2017

Purpose: Nurses experience burnout related to various factors. For this descriptive research job stress, compassion satisfaction, and compassion fatigue were examined as to their relationship to burnout in nurses from children's hospital. Methods: The participants were 305 nurses working in children's hospital. Self-report questionnaires were used to measure job stress, compassion satisfaction, compassion fatigue and burnout. Results: Nurses in children's hospital experienced a greater than moderate degree of job stress, compassion satisfaction, compassion fatigue and burnout, whereas differences existed according to general characteristics. Job stress, compassion fatigue and burnout showed a significant positive correlation and results of compassion fatigue and burnout were similar. Also, job stress, compassion satisfaction and compassion fatigue were associated with burnout in nurses working in children's hospital. Conclusion: Findings indicate that as longer work experience is accompanied by higher job stress and burnout, it is necessary to develop intervention programs to reduce burnout among career nurses exposed to greater job stress in children's hospital.

• Kidney Research and Clinical Practice
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• v.36 no.3
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• pp.257-263
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• 2017

Background Rituximab (RTX) can be used as a rescue therapy for steroid-dependent nephrotic syndrome (SDNS). However, the efficacy and safety of long-term, repeated use of RTX are not established. This study was conducted to assess the efficacy and safety of long-term, repeated RTX treatment in children. Methods Eighteen consecutive child patients with SDNS who were treated with three or more cycles of RTX for one year or longer were recruited, and their medical records were retrospectively reviewed. Results The patients were followed for $4.7{\pm}1.9years$ and received $5.2{\pm}2.3cycles$ of RTX over $2.8{\pm}1.1years$. Approximately 70% of the additional RTX cycles were administered due to recovery of B-cells without relapse. The relapse rate decreased from $3.4{\pm}2.0per$ year initially to $0.4{\pm}0.8per$ year at the third year after RTX treatment. Approximately 10% of the RTX infusions were accompanied by mild infusion reactions. Eight patients showed sustained remission without any oral medication after the last cycle of RTX, while 10 patients had one or more episodes of relapse after the last cycle of RTX. The relapse rate in the latter group decreased from $2.8{\pm}1.5per$ year before RTX treatment to $1.3{\pm}0.8per$ year after cessation of RTX treatment. No significant differences in clinical parameters were found between the two groups. Conclusion This retrospective study showed that pre-emptive and long-term, repeated RTX treatment is relatively effective and safe in children with SDNS. However, well-designed prospective studies are needed to confirm these findings.

• Clinical and Experimental Pediatrics
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• v.64 no.6
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• pp.310-312
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• 2021

• Advances in pediatric surgery
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• v.3 no.1
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• pp.47-53
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• 1997

Accidental ingestion of caustic substance is one of the common problems among children around the world. Acid intake accounts for a mere 5% of all reported cases of corrosive ingestion in the West. Because of the esophageal sparing effect of acid, clinically significant esophageal involvement after acid ingestion occurs in only 6 to 20 percent of the instances. Despite effort of prevention, 7% to 15% of children sustaining caustic esophageal burns develop esophageal strictures. If balloon dilatation or bougie dilatation fails to resolve the esophageal strictures, successful outcome following replacement by colon or stomach has been reported in children. But the complications and morbidity following these operations are still relatively high. Seven patients with corrosive-acid induced esophageal strictures who were operated upon at the Department of Pediatric Surgery, Seoul National University Children's Hospital from 1991 to 1995 were reviewed. Primary resection and anastomosis was performed in all of 7 patients. The stricture involved short segments of the esophagus at the level of the lower cervical and the upper thoracic vertebra. The operations were approached through a left cervical incision or a left thoracotomy. In one patient, operative repair of anastomotic leakage was done, and three patients required re-resection of anastomotic strictures postoperatively, and one patient required a third operation(reversed gastric tube) due to an anastomotic stricture. The other anastomotic leaks, strictures or pulmonary complications were resolved with conservative treatment. In conclusion, primary resection and anastomosis of the esophagus was performed successfully on the 6 of 7 children with acid induced corrosive esophageal strictures. This approach is physiologic, especially in children who should have a long life expectancy, and recommended for the treatment of short-segment acid induced corrosive esophageal strictures.

• Clinical and Experimental Pediatrics
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• v.62 no.4
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• pp.144-147
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• 2019

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.

• Journal of Chest Surgery
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• v.51 no.1
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• pp.81-84
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• 2018

• Childhood Kidney Diseases
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• v.21 no.1
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• pp.35-39
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• 2017

Azathioprine is commonly used as immunosuppressive therapy for various inflammatory diseases including chronic glomerulonephritis. Myelosuppression is a common side effect of azathioprine, resulting in the need for dose reduction. However, severe pancytopenia or alopecia is not often encountered. Here, we report a case of severe myelosuppression, and alopecia totalis that occurred after azathioprine treatment in a patient with IgA nephropathy. A 10-year-old boy with IgA nephropathy was treated with oral deflazacort and later with azathioprine. After 4 weeks, the patient complained of hair loss, and despite a dose reduction in azathioprine, he developed bone marrow suppression and alopecia totalis in two weeks. The blood indices and alopecia of the patient had returned to normal after azathioprine withdrawal and 3 consecutive doses of granulocyte colony-stimulating factor. We suggest that physicians remain vigilant to the side effects of azathioprine. Unusual hair loss after azathioprine treatment might suggest a defect in the metabolism of the drug, warranting the discontinuation of azathioprine to prevent more severe side effects.

• Journal of Genetic Medicine
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• v.10 no.1
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• pp.47-51
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• 2013

Loeys-Dietz syndrome (LDS) is an autosomal dominant disorder caused by heterozygous mutations in the genes encoding transforming growth factor-${\beta}$ receptor type 1 or 2. It is typically characterized by a triad of hypertelorism, cleft palate or bifid uvula, and arterial tortuosity with aneurysm or dissection. Characteristic vascular abnormalities such as tortuosity, aneurysms, dissections, and stenosis are the most severe complications of LDS and can occur in the neurovascular system. We report a 5-year-old boy who presented with headaches and neurovascular abnormalities and was diagnosed with LDS with a novel mutation of the TGFBR1 gene. It is the first Korean report of neurovascular abnormalities in LDS.

• Advances in pediatric surgery
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• v.3 no.1
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• pp.54-60
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• 1997

Eight pediatric patients with Peutz-Jeghers syndrome were treated from 1984 to 1994 at the Department of Surgery, Seoul National University Children's Hospital. We reviewed the clinical features of our own 8 cases as well as 56 cases reported in Korean literatures. The results were compared to those of western reports. Gastrointestinal complications in our series were more frequent than in the Korean series, but the distribution of polyps was similar. The Korean series showed the following characteristics compared to the western reports; demographic backgrounds were similar; gastrointestinal symptoms were more common; location of the polyp was more frequent in colon (2 times); and the most prevalent site of malignant change was the colon in Korean cases.