증례 : 대장 샘암종 환자에서 이시성으로 발생한 간의 샘편평세포암종 1예

방병욱 ( Byoung Wook Bang ),임미진 ( Mie Jin Lim ),임주한 ( Joo Han Lim ),김은주 ( Eun Joo Kim ),정석 ( Seok Jeong ),최석진 ( Suk Jin Choi ),박인서 ( In Suh Park ) 대한내과학회 2007 대한내과학회지 Vol.72 No.1

간의 원발성 샘편평세포암종은 드물게 발생하며, 간의 다른 원발성 종양보다 불량한 예후를 가지고 있다. 저자들은 상행결장암으로 수술 후 항암화학요법 중 우연히 발견된 간의 원발성 샘편평세포암종을 경험하였기에 보고한다. A primary adenosquamous carcinoma of the liver is a rare subtype of a cholangiocarcinoma. Adenosquamous carcinoma is known to have an aggressive biologic behavior and is associated with a poor prognosis. Recently, we experienced a case of a primary adenosquamous carcinoma of the liver. A 69-year-old man was subjected to a right hemicolectomy five months ago due to an ascending colon cancer. At a periodic check-up, abdominal ultrasonography and magnetic resonance imaging showed a ~1.1 cm-sized hepatic mass which was highly suspicious of being a hepatic metastasis from the ascending colon cancer. A hepatic segmentectomy was performed. The tumor was microscopically composed of an adenocarcinoma and a squamous cell carcinoma, and it was different from the previous colon cancer as determined by immunohistochemistry. Three months after the hepatic segmentectomy, multiple hepatic metastasis was found and the patient has been treated as an outpatient with capecitabine. (Korean J Med 72:74-78, 2007)

S 단백 활성도 감소가 베체트병의 질병 활동도를 반영한다

권성렬 ( Seong Ryul Kwon ),임미진 ( Mie Jin Lim ),박신구 ( Shin Goo Park ),하기철 ( Ki Cheol Ha ),기성호 ( Sung Ho Ki ),박보형 ( Bo Hyoung Park ),문연숙 ( Yeon Sook Moon ),박원 ( Won Park ) 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.4

Objective: To see whether the active inflammation in Behcet`s disease (BD) can cause thrombotic disorders by decreasing the protein S activity (PSA), we evaluated the relationship between PSA decrease and disease activity of BD. Methods: We included 122 patients with BD whose PSAs were measured between August 1998 and April 2005. In 51 BD patients, the PSA was measured again when there were changes in the number of items of The Behcet`s Disease Current Activity Form (BDCAF). Results: Thrombosis rate was 2.5% (3/122), and the PSA was low in all 3 of the patients. The incidence of low PSA in total 122 BD patients was 27% (33/122). The incidence of low PSA in active BD patients was 33.7% (31/92), and it was significantly more frequent in active BD patients than in inactive patients, 6.7% (2/30) (x2-test, p value=0.0038). The decrease of PSA had good correlation with the number of BDCAF items (r=-0.351, p=0.012). Conclusion: The PSA decrease is related to the disease activity of BD. PSA could be not only the risk factor for thrombotic disorder but also the activity marker for the BD and other inflammatory diseases.

전신경화증 환자에서 발생한 다발성 골관절 결핵

김상구 ( Sang Gu Kim ),임미진 ( Mie Jin Lim ),권성렬 ( Seong Ryul Kwon ),정재천 ( Jae Cheon Jeong ),최석진 ( Suk Jin Choi ),김영욱 ( Young Wook Kim ),박원 ( Won Park ) 대한류마티스학회 2008 대한류마티스학회지 Vol.15 No.4

Multifocal skeletal tuberculosis is defined as osteoarticular lesions that occur simultaneously at two or more locations. Cystic lesions of multiple bones in tuberculosis are encountered less frequently in adults than in children. The multifocal cystic skeletal tuberculosis involving hands and feet is rare, particularly. We report a case of systemic sclerosis that was treated with glucocorticoid and antirheumatic drug for many years and complicated with multifocal cystic skeletal tuberculosis. This case was misled to the diagnosis of staphylococcal pyogenic abscess by the first microbial culture of the draining pus. The sonographic finding suggested rather metastatic bone lesion or systemic inflammatory arthritis such as rheumatoid arthritis. Although it is rare, tuberculosis should be included in the differential diagnosis of multiple cystic systemic skeletal lesions in immunocompromised patients.

항 Ro/SS-A 항체 양성인 일차성 쇼그렌증후군 환자에서 침샘 초음파의 유용성

박원 ( Won Park ),김영욱 ( Young Wook Kim ),권성렬 ( Seong Ryul Kwon ),임미진 ( Mie Jin Lim ),김환철 ( Hwan Cheol Kim ) 대한내과학회 2009 대한내과학회지 Vol.77 No.4

Background/Aims: This study sought to evaluate the diagnostic value of salivary gland ultrasonography in primary Sjogren`s syndrome with anti-Ro/SS-A antibody. The secondary goal was to assess the relationship between the grade of parenchymal inhomogeneity and the anti-Ro/SS-A antibody titer or clinical manifestations. Methods: The parotid and submandibular glands were examined ultrasonographically in 30 patients having primary Sjogren`s syndrome with anti-Ro/SS-A antibody and 30 control subjects with no evidence of Sjogren`s syndrome. The ultrasonographic definition of parenchymal inhomogeneity of the salivary glands was blurred glandular borders, multiple hyperechoic bands, and hypoechoic areas. The parenchymal inhomogeneity of the glands was categorized into four grades. Results: Parenchymal inhomogeneity of the parotid gland was seen in 25 (83.3%) patients with primary Sjogren`s syndrome and 2 (6.7%) control subjects. Of these cases, the submandibular gland showed parenchymal inhomogeneity in 24 (80%) patients with primary Sjogren`s syndrome and 2 (6.7%) control subjects. The sensitivity and specificity of parenchymal inhomogeneity of the parotid gland were 83.3% and 93.3%, respectively, and 80% and 93.3% for the submandibular gland. The grade of ultrasonographic parenchymal inhomogeneity was related to a diagnosis of Sjogren`s syndrome (p<0.001) and the time of dissolution of the wafer, but had no relationship with the anti-Ro/SS-A antibody titer. A high degree of interobserver agreement was found in the assessment of parenchymal abnormalities of the salivary gland (parotid gland: κ=0.859; submandibular gland: κ=0.837). Conclusions: Salivary gland ultrasonography is a useful method for visualizing glandular structural changes and making a diagnosis of primary Sjogren`s syndrome. (Korean J Med 77:480-487, 2009)

류마티스관절염 환자에서 파골세포의 분화 및 비스포스포네이트의 영향

김성수 ( Sung Soo Kim ),권성렬 ( Seong Ryul Kwon ),임미진 ( Mie Jin Lim ),박원 ( Won Park ) 대한류마티스학회 2007 대한류마티스학회지 Vol.14 No.1

목적: 첫 번째, 류마티스관절염과 골다공증 환자들의 말초혈액단핵구에서 파골세포의 분화 정도를 분석하였다. 두 번째, 류마티스관절염 환자와 나이가 비슷한 골다공증 환자, 그리고 젊은 정상인을 대상으로 비스포스포네이트의 단기간 처리가 파골 세포 생성 및 관련된 사이토카인의 발현에 미치는 영향을 비교하고자 하였다. 방법: 미국류마티스협회의 기준에 의해 진단된 류마티스관절염 12명과 그들과 나이가 비슷한 일차성 저골밀도 환자 12명을 대상으로 하였다. 이들에서 사이토카인 발현과 파골세포의 분화의 정도를 관찰하였다. 두 번째 실험에서는 혈청양성 저골밀도 류마티스관절염 환자와 나이가 비슷한 일차성 골다공증, 젊은 정상성인, 각각 6명씩에서 말초혈액단핵구를 얻었다. 이들 세포에서 사이토카인의 발현 정도는 real time RT-PCR로 확인하였고, tartrate-resistant acid phosphatase (TRAP) 양성거대세포의 형성과 칼슘판의 흡수구멍형성을 측정하여 파골 세포의 발현과 그 기능을 확인하였다. 각각의 실험은 24시간 비스포스포네이트 처리 전후로 시행하여 그 효과를 관찰하였다. 결과: 첫 번째 실험의 경우 기본적인 사이토카인 mRNA의 발현과 TRAP 양성 거대세포의 형성과 흡수구멍의 형성 정도는 류마티스관절염 환자와 일차성 골다공증 환자 사이에 차이가 없었다. 두 번째 실험은 비스포스포네이트 단기처리 후 macrophage colony-stimulating factor (M-CSF), receptor activator of nuclear factor-κB ligand (RANKL), tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), interferon-γ (IFN-γ) 등의 mRNA 발현은 증가하였고 receptor activator of nuclear factor-κB (RANK), cathepsin K, TRAP 등의 발현은 세 그룹 모두에서 감소하였다. 그 중 IL-6와 IFN-γ의 증가가 가장 저명하였다. IL-1 mRNA 의 발현은 변함이 없었다. 그들의 비스포스포네이트에 의한 mRNA 변화는 증가와 감소 모두 IL-6를 제외하면 류마티스관절염 환자에서 전반적으로 그 변화폭이 적었다. 세 그룹 모두에서 TRAP 양성 거대세포의 발현과 흡수구멍의 생성은 현저히 저해되었다. 결론: 비스포스포네이트는 말초혈액단핵구에서 염증성사이토카인의 발현을 증가시켰다. 사이토카인의 변화 중 가장 두드러진 것은 INF-γ의 증가였다. 이 결과는 IFN-γ가 T세포를 통한 파골세포 생성저해의 매개체일 수 있다는 것을 시사한다. 이들 사이토카인의 반응은 다른 그룹에 비해 류마티스 관절염 환자에서 의미 있게 감소하였다. 비스포스포네이트의 단기 처리는 파골세포의 발달을 전구세포 단계에서 저해하여 후속되는 배양에서 파골세포의 발현을 억제하였다. 그러나 이 반응도 다른 그룹에 비해 류마티스관절염 환자에서 의미 있게 감소하였다. 그러므로 류마티스관절염 환자에서 발생하는 골다공증과 골미란의 감소에 비스포스포네이트가 도움을 주며, 특히 골다공증이 동반된 경우를 치료할 때는 그 용량이나 투여방법을 파제트병이나 전이성종양의 경우처럼 더욱 강력하여야 할 필요가 있다.

지주막하 출혈로 내원한 호산구육아종증다발혈관염 환자 1예

오세환 ( Se Hwan Oh ),박원 ( Won Park ),권성렬 ( Seong Ryul Kwon ),임미진 ( Mie Jin Lim ),주고운 ( Ko Woon Joo ),이오현 ( Oh Hyun Lee ),이하영 ( Ha Young Lee ),오세양 ( Se Yang Oh ),정경희 ( Kyong Hee Jung ) 대한류마티스학회 2015 대한류마티스학회지 Vol.22 No.3

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss syndrome, is an anti-neutrophil cytoplasmic antibody associated vasculitis, accompanied by asthma, hypereosinophilia, nonfixed pulmonary infiltrates, and sinusitis. Peripheral neuropathy is common in patients with EGPA; however, a few cases of EGPA with central nervous system (CNS) involvement have been reported. A 45-year-old female referred for right side weakness and posterior neck pain was diagnosed as EGPA with subarachnoid hemorrhage and mononeuritis multiplex. She was effectively treated with a high dose glucocorticoid, cyclophosphamide, and intravenous immunoglobulin. EGPA with CNS involvement is uncommon and causes significant morbidity and mortality. Therefore more rapid and accurate diagnostic evaluation may be required. EGPA should be considered in patients with neurological symptoms and hypereosinophilia. (J Rheum Dis 2015;22:190-194)

증례 : 류마티스 ; 레이노 병을 동반한 수부의 사구 종양(Glomus tumor)의 1예

이승호 ( Seung Ho Lee ),박원 ( Won Park ),권성렬 ( Seong Ryul Kwon ),임미진 ( Mie Jin Lim ),최석진 ( Suk Jin Choi ),김여주 ( Yeo Ju Kim ),정경희 ( Kyong Hee Jung ) 대한내과학회 2014 대한내과학회지 Vol.87 No.6

본 증례는 레이노 현상으로 내원한 환자에서 네 번째 손가락 끝의 국소 통증이 지속되어 수부 자기공명영상 검사를 이용해 사구 종양을 진단하고 수술적 치료로 호전된 경우이다. 레이노병 환자에서 손의 국소적 통증이 지속된다면, 매우 드물긴 하나 사구 종양과 같은 국소 병변의 동반에 대해서도 고려할 필요가 있다. Although it is difficult to reach a diagnosis in patients who complain of pain or sensitivity to cold in their hands, Raynaud`s phenomenon is most often suspected in such cases. Symptoms of Raynaud`s phenomenon include pallor, cyanosis, and redness following cold exposure. Glomus tumors can also increase patients` sensitivity to cold. In this case, our patient complained of symptoms indicative of Raynaud`s phenomenon. Although treatment of Raynaud`s phenomenon improved the symptoms, pain persisted in the fourth finger of the left hand. We diagnosed the patient with a glomus tumor and, after surgical treatment, the patient`s symptoms improved. Here, we have also reviewed and discussed a number of reports of glomus tumors associated with Raynaud`s disease. Diagnosing a glomus tumor in the hand may take some time, and the diagnosis could be further delayed if accompanied by Raynaud`s phenomenon. Although glomus tumors are rare, we suggest that clinical awareness is important for early diagnosis and treatment. (Korean J Med 2014,87:765-770)

골다공증 치료를 받지 않는 류마티스 관절염 환자의 골밀도 검사 간격

조민재 ( Min-jae Jo ),정경희 ( Kyong-hee Jung ),권성렬 ( Seong-ryul Kwon ),임미진 ( Mie-jin Lim ),박신구 ( Shin-goo Park ),박원 ( Won Park ) 대한내과학회 2016 대한내과학회지 Vol.91 No.2

목적: 저자들은 골다공증 치료를 받지 않고 있는 류마티스관절염 환자들에서 골다공증의 위험 요인과 골밀도 검사의 적절한 간격을 알아보고자 하였다. 방법: BMD 측정을 두 번 이상 실시받고, 골다공증을 진단받지 않은 40세 이상의 류마티스 관절염 환자 511명을 후향적으로 조사하였다. 이들을 기저치 BMD가 정상(T-score >-1), 경증 골감소(-1 ≥ T-score > -1.5), 중등도 골감소(-1.5 ≥T-score > -2), 중증 골감소(-2 ≥ T-score > -2.5)의 4군으로 분류하였다. 각 군에서 10%의 환자가 골다공증 또는 골절이 발생되는 기간을 추정하였다. 결과: 총 관찰 기간은 2,214인-년이었고 평균 4.3인-년이었다. 각 군에서 10%의 환자가 골다공증 또는 골절이 발생되는기간은 정상군은 10년 이상, 경증군은 4.3년, 중등도군은 2.5년, 중증군은 1.5년이었다. 결론: 초기 BMD 측정의 T-score가 추후 골다공증이 발생의 가장 강력한 인자였다. 그러므로 류마티스 관절염 환자에서 초기 BMD의 결과에 따른 다음 검사의 적절한 간격 조정이 필요하다. Background/Aims: Osteoporosis occurs more frequently in rheumatoid arthritis (RA) patients than in healthy individuals. This study investigated the appropriate bone mineral density (BMD) measurement interval and risk factors associated with osteoporosis for RA patients. Methods: A retrospective study was performed on 511 RA patients aged more than 40 years old who had undergone BMD testing more than once and who had normal BMD or osteopenia at the baseline BMD test and no history of any fracture of the spine or femur. The subjects were categorized into four subgroups: normal BMD (T-score > -1), mild (-1 ≥ T-score > -1.5), moderate (-1.5 ≥ T-score > -2), and advanced (-2 ≥ T-score > -2.5) osteopenia. The BMD testing interval was defined as the estimated time for 10% of the RA patients to make the transition to osteoporosis without osteoporotic fracture or the administration of any osteoporosis drug. Results: The observation period was 2,214 patient-years, with an average of 4.3 years. The estimated BMD testing interval was more than 10 years for normal, 4.3 years for mild, 2.5 years for moderate, and 1.5 years for advanced osteopenia in each of the RA patient groups. Conclusions: Our study indicated that in normal or osteopenic RA groups, a baseline BMD T-score is the most important factor in estimating the interval in which osteoporosis is predicted to occur. In addition, we recommend that the BMD measuring interval should be greater than 10 years in normal BMD RA patients, 4 years in mild, 2 years in moderate, and 1 year in advanced osteopenic RA patients on the basis of L-spine BMD. (Korean J Med 2016;91:166-173)

통풍 환자를 위한 웹기반 자가-관리 중재 프로그램이 통풍 관련 지식, 치료제 복용 순응도 및 자가-관리에 미치는 효과

오현수(Oh, Hyun Soo),박원(Park, Won),권성렬(Kwon, Seong Ryul),임미진(Lim, Mie Jin),서연옥(Suh, Yeon Ok),서화숙(Seo, Wha Sook),박종숙(Park, Jong Suk) 한국간호과학회 2013 Journal of Korean Academy of Nursing Vol.43 No.4

Purpose: This study was conducted to examine the changing patterns of knowledge related to disease, medication adherence, and self-management and to determine if outcomes were more favorable in the experimental group than in the comparison group through 6 months after providing a web-based self-management intervention. Methods: A non-equivalent control group quasi-experimental design was used and 65 patients with gout, 34 in experimental group and 31 in comparison group, were selected from the rheumatic clinics of two university hospitals. Data were collected four times, at baseline, at 1 month, 3 months, and 6 months after the intervention. Results: According to the study results, the changing patterns of knowledge and self-management were more positive in the experimental group than in the control group, whereas difference in the changing pattern of medication adherence between two groups was not significant. Conclusion: The results indicate that the webbased self-management program has significant effect on improving knowledge and self-management for middle aged male patients with gout. However, in order to enhance medication adherence, the web-based intervention might not be sufficient and other strategies need to be added.

SAPHO 증후군에서 경구 Alendronate로 치료한 1예

심현익 ( Hyun Ik Shim ),박원 ( Won Park ),김여주 ( Yeo Ju Kim ),정경희 ( Kyong Hee Jung ),백지현 ( Ji Hyeon Baek ),임미진 ( Mie Jin Lim ),주고운 ( Kowoon Joo ),권성렬 ( Seong Ryul Kwon ) 대한류마티스학회 2015 대한류마티스학회지 Vol.22 No.5

본 저자들은 손가락의 골절과 감염이 동반된 SAPHO 증후군에서 경구 bisphosphonate로 성공적으로 치료하였기에 이를 보고하는 바이다. SAPHO syndrome, characterized by synovitis, acne, pustulosis, hyperostosis, and osteitis is rare compared to other spondyloarthropathies. It is also difficult to diagnose, and treatment methods have not yet been fully identified. Approximately 72% of patients are diagnosed with at least one other disease before a final diagnosis of SAPHO syndrome. In addition, SAPHO syndrome is subject to a delayed diagnosis period of 4.5 to 9.1 years. Medications such as non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, and tumor necrosis factor inhibitors are used in treatment of SAPHO syndrome. Bisphosphonate is also used for refractory SAPHO syndrome; however, most reports on this relate to intravenous injection of medication. The authors experienced and subsequently reported on a case involving a patient with SAPHO syndrome accompanied by fracture and infection of the left second finger who was treated with the oral biphosphonate, alendronate. (J Rheum Dis 2015;22:313-316)