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성인과 소아 아토피피부염 환자에서 가려움증의 임상적 특징
심우행 ( Woo Haing Shim ),송창현 ( Chang Hyun Song ),박현제 ( Hyun Je Park ),김훈수 ( Hoon Soo Kim ),진현우 ( Hyun Woo Chin ),김수한 ( Su Han Kim ),고현창 ( Hyun Chang Ko ),김문범 ( Moon Bum Kim ),김도원 ( Do Won Kim ),김병수 ( By 대한피부과학회 2011 대한피부과학회지 Vol.49 No.4
Background: Itch is an essential feature of atopic dermatitis (AD). Active AD cannot be diagnosed without a history of itch. There has been no previous study delineating the different characteristics of itch according to age. Objective: We wanted to characterize and compare the clinical patterns and the sensory and affective dimensions of itch in adult and childhood AD patients. Methods: A face-to-face structured questionnaire based on the McGill pain questionnaire was given to 90 patients with AD. The patients were classified into two groups: adult AD patients (≥15 years old) and childhood AD patients (<15 years old). Results: Eighty-eight percent of the total AD patients experienced itch everyday. This itch was mostly aggravated during the summer and at night in both groups of AD patients. The antecubital fossae, popliteal fossae and neck were the major sites of involvement with itch. More patients perceived the characteristics of itch as crawling, burning and tickling rather than stinging, stabbing and pinching. The majority of AD patients answered that sweat and hot climate aggravated the itch and medications and cold climate alleviated the itch. However, compared with the childhood patients, more adult patients experienced the aggravation of itch by exposure to dust, and the itch was alleviated by taking medications and by concentrating on work. A burning sensation with itch was more frequently reported by the adult patients when compared to that of the childhood patients. Conclusion: This study highlights the detailed description and characteristics of itch in adult and childhood AD patients. The proper management of itch according to age may be prerequisite to improve AD patients` quality of life. (Korean J Dermatol 2011;49(4):318∼327)
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TRPS1 유전자의 새로운 과오돌연변이를 보인 제 3형 모발-비-수지 이형성 증후군
노동영 ( Dongyoung Roh ),김태욱 ( Tae-wook Kim ),박성민 ( Sung-min Park ),이현주 ( Hyun-joo Lee ),진현주 ( Hyunju Jin ),유향석 ( Hyang-suk You ),심우행 ( Woo-haing Shim ),김건욱 ( Gun-wook Kim ),김훈수 ( Hoon-soo Kim ),김병수 ( By 대한피부과학회 2018 大韓皮膚科學會誌 Vol.56 No.8
Trichorhinophalangeal syndrome (TRPS) is a rare autosomal dominant hereditary disease caused by mutations in the TRPS1 or the EXT1 gene. Patients show 3 different forms (TRPS I-TRPS III) of this condition sharing similar clinical features including sparse hair, a bulbous nose, an elongated philtrum, short stature, and shortened phalanges. A 10-year-old girl presented with sparse hair and thin hair shafts since birth. She also showed a bulbous nose, an elongated philtrum, brachydactyly of the great toes, and a short stature. Radiological examination showed cone-shaped epiphyses and shortened phalanges. Genetic analysis revealed a novel missense mutation c.2759G>C (p.Trp920Ser) in the TRPS1 gene. We diagnosed this patient with TRPS type III. To our knowledge, only 3 reports have described a genetically analyzed TRPS1 gene mutation among the 11 reported cases of TRPS in the Korean literature. Furthermore, we identified a novel missense mutation in the TRPS1 gene. (Korean J Dermatol 2018;56(8):494∼498)
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비호지킨림프종에서 골수흡인 검사 결과에 따른 전체 생존율의 분석
최정윤 ( Jung Yoon Choi ),강가원 ( Ka-won Kang ),이병현 ( Byung-hyun Lee ),유은상 ( Eun Sang Yu ),김대식 ( Dae Sik Kim ),이세련 ( Se Ryeon Lee ),성화정 ( Hwa Jung Sung ),김석진 ( Seok Jin Kim ),최철원 ( Chul Won Choi ),김병수 ( By 대한내과학회 2019 대한내과학회지 Vol.94 No.2
목적: 비호지킨림프종 환자를 위한 병기 설정 검사는 골수흡인 검사와 골수 조직 검사의 방법으로 시행하게 된다. 두 가지 검사 결과가 일치하면 골수 침범 여부가 명백하나골수흡인 검사에서만 양성이 나온 경우는 가이드라인이 없으며 임상적으로 논란의 여지가 있다. 따라서 본 연구에서는 이러한 환자군에 대하여 임상 적용에 필요한 생존율을 형태학적인 방법으로 비교 분석함으로써 임상적으로 고려해야 할 사안을 검토하는 것으로 하였다. 방법: 본 연구는 고려대학교의료원의 림프종 레지스트리에 1991년부터 2016년까지 등록된 환자를 대상으로 하여 대상군은 골수비침습군(BMA-/BMBx-), 흡인 검사에서만 양성이고 조직 검사에서는 음성이 나온 군(BMA+/BMBx-), 조직검사에서 골수 침범이 확인된 군(BMBx+)으로 구분하였으며 카플란-마이어 생존분석과 다변량 분석을 사용하였다. 결과: 환자군은 총 1,735명 중에서 불충분한 결과를 가진 409명을 제외하고 1,326명을 대상으로 하였다. 카플란-마이어 생존분석에서 BMBx+군은 BMA-/BMBx-군과 비교하여 유의하게 좋지 않은 생존율을 보였으나(p < 0.001) BMA+/BMBx-와 다른 군 간의 전체 생존율에 대한 유의한 차이는 없었다 (vs. BMA-/BMBx-, p = 0.163; BMBx+, p = 0.292). 그러나 다른 생존율과 관련된 인자들을 보정한 다변량 분석에서 BMA+/BMBx-는 BMA-/BMBx-에 비하여 경계선 상의 유의성을 보여주었으며(p = 0.081) 이는 특히 지연성 비호지킨림프종 환자의 서브그룹 분석에서는 유의한 차이를 나타냈다 (p = 0.003). 결론: 비호지킨림프종 환자에서 골수흡인 검사에서만 양성이 나오더라도 불량한 예후를 시사하고 있음을 인지하고 주의를 기울이는 것이 중요하다 하겠다. Background/Aims: The staging work-up for patients with non-Hodgkin’s lymphoma includes bone marrow aspiration and biopsy. Consistent results of both procedures can clarify the diagnosis. However, no clear guidelines have been established regarding positive results for bone marrow aspiration alone. The aim of this study was, therefore, to analyze the overall survival (OS) for the clinical diagnoses of these patients using morphological methods. Methods: We performed a retrospective analysis of patients who were consecutively enrolled in the Korea University Lymphoma Registry from 1991 to 2016. OS was compared according to the bone marrow group: without bone marrow involvement (BMA-/BMBx-), with positive results for aspiration and negative results for biopsy (BMA+/BMBx-), and with bone marrow involvement in biopsy (BMBx+). OS was assessed using the Kaplan-Meier method and multivariate analysis. Results: Of 1,735 patients, 1,326 were analyzed and 409 were excluded. In the Kaplan-Meier survival analysis, OS was significantly worse for patients in the BMBx+ group compared with those in the BMA-/BMBx- group (p < 0.001). However, there was no significant difference in OS between patients in the BMA+/BMBx- group and those in other groups (vs. BMA-/BMBx-, p = 0.163; BMBx+, p = 0.292). In multivariate analysis, by adjusting survival-related variables, the BMA+/BMBx- group showed marginal significance compared to the BMA-/BMBx- group (p = 0.081), and showed significance in the subgroup of indolent non-Hodgkin’s lymphoma patients (p = 0.003). Conclusions: This study suggested that even if there are positive results in bone marrow aspiration alone in patients with non-Hodgkin lymphoma, attention to patient characteristics, involving significance as a poor prognosis for OS, is required. (Korean J Med 2019;94:182-190)
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