• Clinical and Experimental Pediatrics
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• 제54권1호
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• pp.36-39
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• 2011

Bartter syndrome (BS) is a clinically and genetically heterogeneous inherited renal tubular disorder characterized by renal salt wasting, hypokalemic metabolic alkalosis and normotensive hyperreninemic hyperaldosteronism. There have been several case reports of BS complicated by focal segmental glomerulosclerosis (FSGS). Here, we have reported the case of a BS patient who developed FSGS and subsequent end-stage renal disease (ESRD) and provided a brief literature review. The patient presented with classic BS at 3 months of age and developed proteinuria at 7 years. Renal biopsy performed at 11 years of age revealed a FSGS perihilar variant. Hemodialysis was initiated at 11 years of age, and kidney transplantation was performed at 16 years of age. The post-transplantation course has been uneventful for more than 3 years with complete disappearance of BS without the recurrence of FSGS. Genetic study revealed a homozygous p.Trp(TGG)610Stop(TGA) mutation in the CLCNKB gene. In summary, BS may be complicated by secondary FSGS due to the adaptive response to chronic salt-losing nephropathy, and FSGS may progress to ESRD in some patients. Renal transplantation in patients with BS and ESRD results in complete remission of BS.

• Pediatric Infection and Vaccine
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• 제24권1호
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• pp.60-64
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• 2017

급성 음낭증은 갑작스런 음낭의 부종과 통증을 주소로 하는 음낭의 병적 상태를 말하는 것으로 가와사키의 드문 합병증으로 알려져 있다. 저자들은 급성 음낭증을 동반한 가와사키병으로 진단된 2개월 남자 환아를 경험하였으며, 환아는 정맥 면역글로불린 및 아스피린 투여 후 발열과 가와사키병의 임상 증상들이 호전되었고 2달째에 고환 초음파검사에서 급성 음낭증의 호전을 확인하고 심장 초음파 검사에서 관상동맥 합병증이 보이지 않아 치료를 종료하였다.

• Advances in pediatric surgery
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• 제11권2호
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• pp.141-149
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• 2005

Ulcerative colitis, an inflammatory bowel disease, is primarily managed medically with a combination of 5-ASA and steroids. However, this chronic disease requires surgical management if symptoms persist or complications develop despite medical management. The clinical course, indications and outcome of surgical management of 21 patients under the age of 15 who were endoscopically diagnosed with ulcerative colitis at the Seoul National University Children's Hospital between January, 1988 and January, 2003 were reviewed. Mean follow up period was 3 years and 10 months. The mean age was 10.3 years old. All patients received medical management after diagnosis and 8 patients (38 %) eventually required surgical management. Of 13 patients who received medical management only, 7 patients (53 %) showed remission, 4 patients are still on medical management, and 2 patients expired due to congenital immune deficiency and hepatic failure as a result of sclerosing cholangitis. In 8 patients who received surgical management, the indications for operation were, 1 patient sigmoid colon perforation and 7 patients intractability despite medical management. The perforated case had a segmental colon resection and the other 7 patients underwent total colectomy with ileal pouch-anal anastomosis. One patient expired postoperatively due to pneumonia and sepsis. and 1 is still on medical management because of mild persistent hematochezia after surgery. Six other operated patients are doing well without medical therapy. Pediatric ulcerative colitis patients can be surgically managed if the patient is intractable to medical management or if complications such as perforation are present. Total colectomy & ileal pouch-anal anastomosis is thought to be the adequate surgical method.

• Clinical and Experimental Pediatrics
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• 제53권3호
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• pp.437-441
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• 2010

독성표피괴사용해(TEN)와 스티븐-존슨 증후군(SJS)은 약물이나 감염에 의해 발생하는 피부점막을 침범하는 드물지만 치명적인 질병이다. 스테로이드는 TEN의 치료에 많이 이용되어왔지만, 아직까지도 논쟁중이다. 스테로이드에 의한 정신과적 영향은 두통, 불면증, 우울증, 심리질환 등이 있다. 스테로이드에 의한 정신질환에서 치료는 스테로이드의 감량 또는 중단이고, 항정신성 약물을 투여한다. 신증후군으로 진단된 11세 남아에서 스테로이드 치료 후 관해상태에서 유지치료를 시행하고 있던 중에 TEN이 발생하였다. 저자들은 이 환아에서 치료목적으로 투여한 고용량 스테로이드 정맥주사로 인해 정신질환이 동반되었고, 이후 스테로이드 감량과 항정신성 약물과 면역글로불린으로 증상이 호전되는 것을 경험하여 이를 보고하는 바이다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제33권5호
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• pp.554-558
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• 2007

저자 등은 좌측 이하부에 발생한 기무라씨병을 가진 19세의 남환에 대하여 표층 이하선 절제술을 동반한 종양 절제술과 함께 방사선 치료를 시행하였다. 수술 후 약 6년 9개월 경과 관찰 한 결과 현재까지 특기할만한 후유증 및 합병증과 재발 소견 혹은 증상은 보이지 않았기에 문헌 고찰과 함께 보고하는 바이며, 이후에도 지속적인 경과관찰을 요하리라 사료된다.

• 대한기관식도과학회지
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• 제6권1호
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• pp.102-107
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• 2000

Monocytoid B-cell lymphoma is uncommon, low grade lymphoma originating from monocytoid B lymphocytes. Monocytoid B-cell lymphoma usually presents as a localized lymphadenopathy. Peripheral lymph nodes are most often involved, particularly those in the frequent in the head and neck area. A distinctive feature is the association of monocytoid B-cell lymphoma with autoimmune diseases. Sjogren Syndrome had been present in 22% of patient with monocytoid lymphoma. Extranodal involvement by monocytoid lymphoma was reported in the salivary gland, breast, thyroid, and stomach. There were also occasional extensions to the liver and retroperitoneum. The bone marrow and peripheral blood involvement by monocytoid lymphoma is very rare, which is frequently seen in hairly cell leukemia. Fever, weight loss, and other constitutional signs are usually absent. Most patients have no symptoms, and the only sign is enlarged lymph nodes. The clinical course remains indolent; most patients are in complete remission and recurrence with progression to a high-grade lymphoma of large cell type was recorded only in a few cases. Authors experienced a case of monocytoid B-cell lymphoma associated with Sjogren Syndrome mistaken to simple cervical lymphadenitis in a 60-year-old female. We report this case with a review of literatures.

• The Korean Journal of Physiology and Pharmacology
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• 제5권1호
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• pp.79-86
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• 2001

Recent clinical studies have shown that a high proportion of patients with acute promyelocytic leukemia (APL) achieve complete remission after treatment with all-trans retinoic acid (ATRA). However, most patients who receive continuous treatment with ATRA relapse and develop ATRA-resistant leukemia. Dendritic cells (DCs) are important antigen-presenting cells in the development of antileukemic T-cell responses. In this study, we investigated the strategies to overcome ATRA resistance of APL cells by inducing the differentiation of DCs from human leukemic cell lines for the developtment of adoptive immunotherapy. CD83 was used as a mature DC marker in this study and the expression of CD83 mRNA was determined by RT-PCR method. The promyelocytic leukemic cell line HL-60, B lymphoblast cell lines RPMI 7666 and NC-37 could be induced to dendritic cells in vitro. Treatment of HL-60 with phorbol 12-myristate 13-acetate (PMA) resulted in the expression of myeloid-related DC phenotypes, while treatment of RPMI 7666 with fms-like tyrosine kinase 3 ligand (Flt3-ligand, FL) and treatment of NC-37 with PMA and FL led to the expression of lymphoid-related DC phenotypes. In conclusion, myeloid-related DC phenotypes and lymphoid-related DC phenotypes could be generated from HL-60, NC-37 and RPMI 7666 cell lines, respectively. These DC phenotypes can potentially be used to generate antileukemic T cells in vitro for adoptive immunotherapy.

• Radiation Oncology Journal
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• 제34권2호
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• pp.96-105
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• 2016

Purpose: The standard radiation dose for patients with locally rectal cancer treated with preoperative chemoradiotherapy is 45-50 Gy in 25-28 fractions. We aimed to assess whether a difference exists within this dose fractionation range. Materials and Methods: A retrospective analysis was performed to compare three dose fractionation schedules. Patients received 50 Gy in 25 fractions (group A), 50.4 Gy in 28 fractions (group B), or 45 Gy in 25 fractions (group C) to the whole pelvis, as well as concurrent 5-fluorouracil. Radical resection was scheduled for 8 weeks after concurrent chemoradiotherapy. Results: Between September 2010 and August 2013, 175 patients were treated with preoperative chemoradiotherapy at our institution. Among those patients, 154 were eligible for analysis (55, 50, and 49 patients in groups A, B, and C, respectively). After the median follow-up period of 29 months (range, 5 to 48 months), no differences were found between the 3 groups regarding pathologic complete remission rate, tumor regression grade, treatment-related toxicity, 2-year locoregional recurrence-free survival, distant metastasis-free survival, disease-free survival, or overall survival. The circumferential resection margin width was a prognostic factor for 2-year locoregional recurrence-free survival, whereas ypN category was associated with distant metastasis-free survival, disease-free survival, and overall survival. High tumor regression grading score was correlated with 2-year distant metastasis-free survival and disease-free survival in univariate analysis. Conclusion: Three different radiation dose fractionation schedules, within the dose range recommended by the National Comprehensive Cancer Network, had no impact on pathologic tumor regression and early clinical outcome for locally advanced rectal cancer.

• Childhood Kidney Diseases
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• 제7권2호
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• pp.245-252
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• 2003

Posttransplant lymphoproliferative disease(PTLD)는 이식 후 발생하는 림프증식성 질환으로 이식장기의 거부반응을 억제하기 위한 면역억제제의 사용 및 이에 따른 EBV 감염과 연관이 있다고 알려져 있다. 소아 PTLD의 경우 성인에 비해 EBV의 초감염 또는 재활성이 더 많은 것으로 보고되고 있으며 최근 더 강력한 면역억제제들의 개발 및 사용에 따라 발생이 증가하고 있다. 본 증례는 14세 여아로 신이식 44개월 후에 EBV 감염의 증거 없이 지발성 PTLD가 발생하였으며 골수 검사상 B-세포 급성 림프구성 백혈병으로 진단되어 항암화학요법 치료를 시작하였고, 치료 후 완전 관해는 이루어졌으나 심한 중성구 감소증에 따른 패혈성 쇼크로 입원 77일만에 사망하였다.

• 대한한방내과학회지
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• 제43권4호
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• pp.751-760
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• 2022

Objective: To show a decrease in tumor recurrence and improvement in quality of life in patients with recurrent cervical cancer. Method: A 58-year-old female patient diagnosed with recurrent cervical cancer in February 2021 was treated for 14 months with integrative cancer treatment (ICT) to decrease the tumor size and improve chemotherapy-induced peripheral neuropathy (CIPN) and nausea. The patient underwent chemotherapy or concomitant chemoradiation therapy (CCRT) with ICT. Radiologic outcomes were assessed by abdomen & pelvis computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography-computed tomography (PET-CT) based on the Response Evaluation Criteria in Solid Tumors (RECIST) protocol. Clinical outcomes were assessed by the National Cancer Institute Common Terminology Criteria for Adverse Event (NCI-CTCAE), Eastern Cooperative Oncology Group (ECOG), and a numeric rating scale (NRS). Result: During the 14 months of treatment, the size and metabolic activity of the recurrent tumor decreased and underwent partial remission based on RECIST. The NRS scores for CIPN and nausea were improved, and the ECOG score improved from grade 2 to 1. No serious side effects of grade 3 or higher were noted on the NCI-CTCAE test. Conclusion: This case suggests that ICT may have a synergetic effect with chemotherapy or radiotherapy for recurrent cervical cancer.