• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.1
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• pp.49-62
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• 2020

Purpose: We aimed to investigate the disease phenotype of Korean pediatric Crohn's disease (CD) patients at diagnosis according to the Paris classification by comparison with patients from the European multicenter 5-years recruitment of children with newly developed IBD (EUROKIDS registry). Methods: Korean children and adolescents who had been newly diagnosed with CD at the age of <18 years during 2013-2016 were included in this multicenter retrospective study. Disease phenotype at diagnosis was classified according to the Paris classification, and compared with the published data from the EUROKIDS study. Results: A total of 255 patients were included. The median diagnosis age was 14.7 years (range, 0.8-17.9 years). No significant difference was observed in male-to-female ratio with EUROKIDS (1.9:1 vs. 1.45:1, p=0.062). The proportion of children aged <10 years was significantly lower in Koreans (7.1% vs. 19.6%, p<0.001). Colonic disease was less prominent (10.0% vs. 27.3%, p<0.001), while upper GI involvement was more prominent in Korean children (59.3% vs. 46.2%, p<0.001). The proportion with perianal fistulizing disease at diagnosis was significantly higher in Korean patients (44.8% vs. 8.2%, p<0.001). A separate analysis of Korean patients revealed that perianal fistulizing disease at diagnosis was positively associated with male sex and body mass index z-score (odds ratio [OR]=2.12, 95% confidence interval [CI]=1.20-3.76, p=0.010; and OR=1.29, 95% CI=1.05-1.58, p=0.015, respectively). Conclusion: Approximately half of pediatric CD patients in Korea present with perianal fistulas and/or abscesses at diagnosis, which is a distinct feature of CD in Korean children and adolescents compared to their European counterparts. An underlying genetic difference between ethnicities may play a role in this expression of different phenotypes in pediatric CD.

• Clinical and Experimental Pediatrics
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• v.59 no.10
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• pp.421-424
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• 2016

Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3. We were unable to identify the cause for the HLH. He remained symptom-free until the development of back pain and right ankle joint pain with swelling at 15 years of age. He was diagnosed with HLA B27-positive AS with bilateral active sacroiliitis. He showed symptom aggravation despite taking naproxen and methotrexate, and the symptoms improved with etanercept. On admission, his laboratory data showed leukopenia with high ferritin and triglyceride levels. Bone marrow biopsy examination showed histiocytic hyperplasia with hemophagocytosis. There was no evidence of infection. He received naproxen alone, and his symptoms and laboratory data improved without any other immunomodulatory medications. Genetic study revealed no primary HLH or inflammasome abnormalities. In this case, underlying autoimmune disease should have been considered as the cause of recurrent MAS in the young patient once primary HLH was excluded.

• Journal of Chest Surgery
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• v.42 no.6
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• pp.719-724
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• 2009

Background: As the patients who undergo heart transplantation have achieved better survival in recent years, growing number of recipients are at a risk for experiencing surgical complications in addition to rejection and infection. In this paper, we report on our experience with the surgical complications that occurred in heart transplant recipients. Material and Method: From April 1994 to September 2003, 37 heart transplantations were performed at our center by a single surgeon. The indications for transplantation were dilated cardiomyopathy, ischemic cardiomyopathy, valvular cardiomyopathy and familial hypertrophic cardiomyopathy. Result: Twenty postoperative complications required surgeries in 15 patients (41%). The types of operations required were; redo-sternotomy for bleeding (5), pericardiostomy for effusion (4), implantation of a permanent pacemaker (1), right lower lobe lobectomy for aspergilloma (1), removal of urinary stone (1), cholecystectomy for gall bladder stone (1), drainage of a perianal abscess (1), paranasal sinus drainage (1), total hip replacement (1), partial gingivectomy due to gingival hypertrophy (1), urethrostomy (1), herniated intervertebral disc operation (1) and total hysterectomy for myoma uteri (1). The locations of the complications were mediastinal in 10 (27%) cases and extramediastihalin 10 (27%) cases. Conclusion: The relatively high incidence of extrathoracic complications associated with heart transplantation emphasizes the importance of a multidisciplinary approach to the improve long-term survival when managing those complex patients.