• Advances in pediatric surgery
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• 제19권2호
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• pp.122-129
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• 2013

Immature ganglion cell (IGC) is known for its relationship with intestinal motility and its impact on postoperative functional outcomes of Hirschsprung's disease (HD). There are few studies on the relationship between intestinal dysmotility and IGC in HD patients. 67 patients pathologically diagnosed with HD and who received definitive operation in Seoul National University Children's Hospital from 2010 to 2011 were included. 10 patients were excluded due to inadequate immunohistochemical staining results. The proximal end of resected ganglionic segment was evaluated with immunohistochemistry examination with MAP-2, a marker of ganglionic cells and bcl-2, a marker of IGCs The median age at operation was 155 (15-4678) day-old. 55 (96.5%) patients positive for bcl-2, were regarded as having IGC, and 2 (3.5%) patients positive for MAP-2 but negative for bcl-2, were regarded as having only mature ganglion cells. In the bcl-2 positive group, there were 7 patients (12.7%) with constipation, 15 patients (27.3%) with soiling, 3 patients (5.5%) with perianal excoriation and 6 patients (10.9%) with medication use. In bcl-2 negative group, intestinal dysmotility was not seen. There was no statistical significance in the two groups. Considering that HD is diagnosed at a young age, the rate of IGC present is very high and it might be inappropriate to relate IGC to functional outcome at young ages.

• Advances in pediatric surgery
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• 제5권1호
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• pp.69-74
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• 1999

Condyloma acuminata, an uncommon disease m the pediatric patients, is being reported with increasing frequency in both adults and children. During the last 10 years, we managed 11 children with condyloma acuminata of the anogenital area. The patients' age at presentation varied from 9 months to 11 years, and boys outnumbered girls by a ratio of 2 : 1. The lesions were found in the perianal area in 9 patients and the genital area in 2. No history of sexual contact or abuse could be elicited. Although the exact mode of transmission of human papillomavirus(HPV) couldn't be defined, maternal-infant transmission at birth and close nonsexual family contact were the main possible causes of HPV infection in our patients. Our experience suggest that fulguration of the warts and concomitant excision with scissors of the larger lesions are the most satisfactory method of treatment in children. However, condyloma acuminata have high recurrence rate despite these treatments. Viral typing and careful epidemiological investigation on a larger series of patients may clarify the causative factors and effective modalities of treatment in childhood anogenital condyloma acuminata.

• Advances in pediatric surgery
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• 제15권2호
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• pp.103-112
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• 2009

Catheter related and perianal problems are common surgical complications encountered during the treatment of pediatric malignancies. However acute surgical abdominal emergencies are rare. The aim of this study is to review acute surgical abdominal complications that occur during the treatment of childhood malignancies. Out of a total of 1,222 patients who were newly diagnosed with malignant disease, between January 2003 and May 2008, there were 10 patients who required surgery because of acute abdominal emergencies. Their medical records were reviewed retrospectively. Hematologic malignancies were present in 7 patients (4 leukemia, 2 lymphoma, 1 Langerhans cell histiocytosis) and solid tumors in 3 patients (1 adrenocortical carcinoma, 1 desmoplastic small round cell tumor, 1 rhabdomyosarcoma). Seven patients had intestinal obstruction, two had gastrointestinal perforation and one, typhlitis. Intestinal obstructions were treated with resection of the involved segment with (N=2) or without (N=3) enterostomy. Two patients had enterostomy alone when resection could not be performed. Intestinal perforation was treated with primary repair. Typhlitis of the ascending colon was treated with ileostomy. Right hemicolectomy was necessary the next day because of the rapidly progressing sepsis. Three patients are now alive on chemotherapy and one patient was lost to followed-up. Among six patients who died, five died of their original disease progression and one of uncontrolled sepsis after intestinal perforation. Although rare, acute surgical abdominal complications can occur in childhood malignancies. Rapid and accurate diagnosis and appropriate operation are required for effective treatment of the complications.

• 대한소아치과학회지
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• 제41권1호
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• pp.80-84
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• 2014

난치성 궤양성 소장결장염은 영아에게서 드물게 나타나는 만성 염증성 장 질환으로 1991년 처음으로 보고되었다. 난치성 궤양성 소장결장염은 주로 영아기에 구강 내 궤양에서 시작되어 항문주위 및 장 질환으로 진행되는 특징을 갖는다. 생후 18일 된 환아가 구강 내 궤양으로 감별할 수 있는 전신질환에 대해 알아보고자 본과로 의뢰 되었다. 당시 구강 내 궤양을 제외한 특이 사항은 없었으며 3주 뒤 혈변이 발생하였다. 대장 내시경 상에서 대장 내 거대 궤양이 발견되었으며 여러검사 결과 난치성 궤양성 소장결장염으로 진단되었다. 치과의사가 난치성 궤양성 소장결장염을 조기에 진단하는 것은 면역 억제제에 반응이 없는 본 질환에 대해 결장절제술을 신속하게 시행함으로써 증상의 완화와 질병의 진행을 막는데 효과적으로 대처할 수 있으므로 매우 중요하다. 또한 대부분의 난치성 궤양성 소장결장염 환아에게서 구강 내 궤양이 가장 먼저 발현되므로 이 질환의 증상과 특징에 대해 아는 것은 매우 의미 있을 것으로 사료되어 보고하는 바이다.

• Archives of Plastic Surgery
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• 제44권3호
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• pp.228-233
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• 2017

Background Hidradenitis suppurativa (HS) is a chronic skin condition that can affect any area with apocrine sweat glands and has the potential to involve multiple sites concurrently. Commonly affected sites include the axilla, groin, perineum and perianal areas. In this study we performed a literature review on the surgical methods for HS and describe an innovative technique for reconstructing axilla HS using an inner-arm transposition flap. Methods We reviewed all cases (5 cases from 4 patients) of transposition flap reconstruction performed by the senior author at a single London tertiary hospital from 2008-2013. Patient related outcome measures were collected using the Derriford appearance scale (DAS 24) and a study specific questionnaire. Results All patients were satisfied with their final result. One out of five cases had a complication but did not result in flap failure. There is no disease recurrence to date. DAS 24 scores collected demonstrated acceptable postoperative distress that did not deviate far from the norm tables while study specific questionnaire reveal desirable outcomes. Conclusions We have managed to achieve our aim through the use of the innovative inner-arm transposition flap. Our study hopes to provide an additional technique for axillary reconstruction. This technique offers the effective concealment of scars with the benefit of tightening of the arm tissue producing 'brachioplasty like' effects. All things considered it would be reasonable to conclude the innovative flap technique is a reliable, effective, and simple method that results in multiple benefits.

• Clinical and Experimental Pediatrics
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• 제59권10호
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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.

• Radiation Oncology Journal
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• 제28권4호
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• pp.205-210
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• 2010

목적: 항문암으로 근치적 목적의 동시 항암화학 방사선치료를 받은 환자들을 대상으로 치료반응의 예측인자, 생존율과 관련된 예후인자를 알아보고자 하였다. 대상 및 방법: 전남대학교병원에서 1993년부터 2008년까지 병리조직학적으로 항문의 편평상피세포암으로 진단받고 근치적 목적의 동시 항암화학 방사선치료를 받은 42명의 환자를 대상으로 후향적으로 분석하였다. 환자들 나이의 중앙값은 61.5세 (39~89에)였고 방사선량의 중앙값은 50.4 Gy (30.0~64.0 Gy)였다. T병기 상 2기 이하였던 환자는 총 36명 (85.7%)이었다. 영역림프절전이는 14명(33.3%)에서 있었다. 항암화학요법으로는 5-fluorouracil, mltomycin 병행요법으로 치료받은 환자가 36명 (85.7%)이었고 나머지는 5-fluorouracil, cisplatin 병행요법을 받았다. 결과: 전체 환자의 추적관찰기간의 중앙값은 62개월 (2~202개월)이었다. 5년 전체생존율, 국소영역제어율, 무병생존율, 무장루생존율은 각각 86.0%. 71.7%. 71.7%. 78.2%였다. 전체생존율과 관련된 인자로 단변량분석에서는 Eastem Cooperative Oncology Group (ECOG) 수행능력과 완전관해여부가 유의하였고 다변량분석에서는 ECOG 수행능력만이 유의하였다. 국소영역제어율, 무병생존율과 무장루생존률에 관련된 인자는 단변량이나 다변량분석 모두 유의한 인자는 없었다. 치료 후 완전관해 여부와 관련하여 유의하게 예측할 수 있는 인자는 없었다. 국소영역재발은 7명에서, 원격전이는 1명에서 발생하였다. 전체 환자에서 평가가 가능하였던 급성 부작용으로 3도의 피부염이 4명(9.5%), 2도는 22명(52.4%)에서 발생하였다. 결론: 본 연구에서 항문의 편평상피세포암에 대한 근치적 목적의 동시 항암화학 방사선치료로 양호한 생존율을 얻을 수 있었고, 특히 ECOG 수행능력이 1 이하인 환자의 생존율이 유의하게 높았다. 그러나 치료 후 종양반응을 예측할 수 있는 유의한 인자는 없었다.