염증성 근육병증에 동반된 폐침범의 임상적 특징

김현숙 ( Hyun Sook Kim ),서수홍 ( Soo Hong Seo ),허지안 ( Ji An Hur ),곽승기 ( Seung Ki Kwok ),주지현 ( Ji Hyeon Ju ),윤종현 ( Chong Hyeon Yoon ),김완욱 ( Wan Uk Kim ),홍연식 ( Yeon Sik Hong ),조철수 ( Chul Soo Cho ),김호연 ( Ho Y 대한류마티스학회 2007 대한류마티스학회지 Vol.14 No.1

Objective: To investigate the clinical manifestation and prognostic factors of interstitial lung disease (ILD) in Korean patients with idiopathic inflammatory myopathies include with polymyositis (PM) and dermatomyositis (DM). Methods: Clinical and laboratory data of 110 patients with PM/DM in our rheumatology clinic were investigated. Clinical data including history, medication, pulmonary function tests (PFT) findings, radiologic findings, and labaratory findings were obtained from medical records at the first diagnosis of ILD with PM/DM. ILD was diagnosed on the basis of the imaging abnormalities defined above on definite findings of chest X-rays and high resolution computed tomography (HRCT), restrictive changes on PFT with respiratory symptoms. During the course of treatment, we assessed chest radiograph and HRCT findings. Results: Forty-two PM/DM patients (38.2%) developed ILD. Anti-extracellular nuclear antigen (ENA) antibody, anti-Jo-1 antibody and ground glass opacity in HRCT were significantly high in PM-ILD. However honeycoomb appearance (53%:22%) and fibrosis (41%:6%) in HRCT were significantly high in DM-ILD. Interest in aspects of prognosis including initial steroid treatment response in HRCT were favorable in PM-ILD. There were statistically significant association between normal level of CPK and usual interstitial pneumonia (UIP) pattern in HRCT in DM-ILD. Such cases had resistance to steroid therapy. Overall interval between steroid and immunosuppressant therapy was significantly shorter in those with DM-ILD. Conclusion: The clinical manifestations between PM-ILD and DM-ILD in Korean patients were not significant different from those of other populations. DM-ILD is more refractory to steroid treatment, expecting in poor prognosis compared with PM-ILD. So immediate intensive immunosuppressive therapy should be considered in DM-ILD.

베체트 증후군 환자에서 혈관내피성장인자(Vascular Endothelial Growth Factor, VEGF)의 상승

홍지현 ( Ji Hyun Hong ),김현숙 ( Hyun Sook Kim ),김해림 ( Hae Rim Kim ),박미경 ( Mi Kyung Park ),윤종현 ( Chong Hyeon Yoon ),이상헌 ( Sang Heon Lee ),김호연 ( Ho Youn Kim ),박성환 ( Sung Hwan Park ) 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.3

Objective: Vascular endothelial growth factor (VEGF) is a potent endothelium-specific cytokine and stimulates inflammation and angiogenesis. Vascular endothelial dysfunction is one of the characteristic features of Behcet`s syndrome. We previously demonstrated the possible involvement of proinflammatory cytokines (TNF-α, IL-15, MIF) and chemokines (IL-8, MCP-1, MIP-1α) in the etiopathogenesis of Behcet`s syndrome and its association with disease activity. In this study, we assesed VEGF levels in patients with Behcet`s syndrome and its relation with disease activity. Methods: Serum VEGF levels of 65 patients with Behcet`s syndrome and 35 healthy control volunteers were measured by enzyme-linked immunosorbent assay. The level of MIF and IL-8 were also measured in 41 pateints. Among the 65 patients, 18 patients were in active state of disease which was classified according to clinical finding. Results: The mean serum VEGF levels were higher in patients of Behcet`s syndrome than control subjects (332.7 pg/mL vs. 234.4 pg/mL, p=0.004) and patients with active disease had significantly higher level of VEGF than inactive disease (377.8 pg/mL vs. 265.0 pg/mL, p<0.05). There is no difference in the mean serum level of VEGF between Behcet`s syndrome with vascular complication and without vascular complication. Serum level of VEGF showed strong positive correlation with MIF (r=0.57, p=0.001). Conclusion: Serum VEGF levels are elevated in patients with Behcet`s syndrome, particularly in active state and have positive correlation with MIF. These results suggest that the increased VEGF levels in serum of Behcet`s syndrome patients may participate, like other proinflammatory cytokines and chemokines, in the pathogenesis of Behcet`s syndrome.

류마티스관절염에서 초음파의 이용

김해림 ( Hae Rim Kim ),윤종현 ( Chong Hyun Yoon ) 대한류마티스학회 2009 대한류마티스학회지 Vol.16 No.2

The administration of disease-modifying antirheumatic drugs (DMARDs) in the early period of rheumatoid arthritis (RA) is critical for protecting against joint damage and inducing remission. Physicians need to identify patients at risk of progression to RA at the early stages of arthritis. Musculoskeletal ultrasonography (MSUS) allows the direct visualization of synovitis and bone erosion in the early phase, and may be useful for differentiating early rheumatoid arthritis from other inflammatory arthritis. Power Doppler sonography is a promising tool for assessing the disease activity and monitoring the effects of DMARDs. This article reviews the current status and recent advances in MSUS imaging in RA.

산전 초음파로 발견된 선천성 요로계 기형의 분류

최진호,한혜원,원혜성,김건석,윤종현,박영서,Choi Jin-Ho,Hahn Hye-Won,Won Hye-Sung,Kim Kun-Suk,Yoon Chong-Hyun,Park Young-Seo 대한소아신장학회 2002 Childhood kidney diseases Vol.6 No.2

목적 : 산전 초음파로 발견할 수 있는 요로계 기형은 매우 종류가 많고 광범위하며 예후 또한 무증상인 것에서부터 치명적인 경우까지 다양하게 보일 수 있다. 저자들은 산전 초음파로 발견되는 선천성 요로 기형의 빈도와 분포에 대해 전반적으로 알아보고자 이 조사를 실시하였다. 방법 : 1989년 6월부터 2002년 5월까지 13년간 본윈 및 타원 산부인과에서 발견되어 본원 소아과에서 선천성 요로계 기형으로 확진된 환자 558례를 대상으로 기형의 종류와 빈도를 환자들의 임상 기록을 토대로 조사하였다 결과 : 전체 대상 환자 558명중 남자가 360명, 여자 198명이었다. 요로계 기형 중 선천성 수신증이 292례에서 관찰되었다. 낭성이형성신 65례, 수뇨관신증 32례, 신장 중복 31례, 신무발육증 25례, 단순 낭종 21례, 다낭성 신 20례, 요도 게실 13례, 신형성부전 11례, 마제신 10례, 방광 요관 역류 9례, 후부요도 판막 8례, 방광 게실 7례, 거대 요관 6례, 이소성 신장 5례, 신수질낭포 1례가 발견되었다. 두 가지 이상의 요로계 기형을 가진 경우가 82례 있었다. 수신증의 경우 방광 요관 역류가 4례, 방광-질 누공이 1례, 신경인성 방광이 1례에서 동반되었다. 낭성이형성신은 방광 요관 역류가 7례, 수신증이 2례, 신발육부전증, 신장 중복, 요도 게실, 후부 요도 판막이 1례씩 동반되었다. 신장 중복은 요도 게실이 11례, 수신증과 방광 요관 역류가 각각 5례씩 동반되었다. 수뇨관신증에서는 방광 요관 역류가 1례에서 동반되었다. 신무발육증에서는 방광 요관 역류가 5례, 중복 요관이 1례, 요도 게실이 1례에서 동반되어 있었고, 단순 낭종의 경우 수신증 1례, 방광 요관 역류가 1례에서 동반되었다. 요도 게실의 경우는 수신증 3례, 수뇨관신증 5례, 방광 요관 역류 1례가 동반되었다. 결론 : 산전 초음파로 발견할 수 있는 선천성 요로계 기형에는 많은 종류가 있으며 임상 경과 및 예후도 다양하다. 각각의 기형에 대한 정확한 분포를 조사하려면 산전 진단과 산후 진단과의 비교 및 전체 인구에서의 발생 빈도를 함께 조사해야 하겠다. Purpose : A study was done to assess the incidence and classification of congenital urinary tract anomalies detected by antenatal ultrasonogram. Methods : We reviewed 558 cases of urinary tract anomaly which were detected by antenatal ultrasonogram and postnatally confirmed between June 1989 and May 2002. We investigated the incidence and classified congenital urinary tract anomalies by review of medical records, antenatal and postnatal radiologic studies retrospectively. Results : In 558 cases of congenital urinary tract anomalies, 292 cases of hydronephrosis were found and the most common. Another anomalies were composed of 65 cases of multicystic dysplastic kidney, 32 cases of hydroureteronephrosis, 31 cases of duplication of kidney, 25 cases of renal agenesis, 21 cases of simple renal cyst, 20 cases of polycystic disease, 13 cases of ureterocele, 11 cases of renal hypoplasia, 10 cases of horseshoe kidney, 9 cases of vesicoureteral reflux, 8 cases of posterior urethral valve, 7 cases of bladder diverticulum, 6 casts of megaureter, 5 cases of ectopia, 2 cases of megacystis, and 1 case of medullary cystic disease. In 82 of the 558 cases, there were two or more combined urinary tract anomalies. Associated diseases other than urinary tract were observed in 13 cases, of which the congenital heart disease was the most common. Conclusion : The congenital urinary tract anomaly is frequently found and diverse during the antenatal ultrasonography. The multicenter study is needed to investigate precise incidence and distribution of each anomalies in general population.

소아에서 둔상 후에 발생한 담도협착의 내시경적 치료 경험 1례

김경모,김성철,서동완,윤종현,Kim, Kyung-Mo,Kim, Seong-Chul,Seo, Dong-Wan,Yoon, Chong-Hyun 대한소아소화기영양학회 1998 Pediatric gastroenterology, hepatology & nutrition Vol.1 No.1

저자들은 복부 둔상 후에 발생한 담도 단독 협착을 보인 환아에서 수술적인 치료를 대신하여 내시경적 경비담도 배액술과 플라스틱 스텐트 삽입으로 증상의 호전과 2년의 추적관찰에서 재발을 보이지 않고 있는 1례를 경험하여 문헌고찰과 함께 보고하는 바이다. Isolated injury to the extrahepatic biliary tract following blunt abdominal trauma is rare, and there is little information especially in children regarding the endoscopic diagnosis and management of occult injury to the biliary tract. We experienced a 5-year-old boy who presented with jaundice 16 days after blunt abdominal trauma and was diagnosed as isolated distal common bile duct stricture by ultrasonography of abdomen. We could get more detailed information about the injury by endoscopic retrograde cholangiopancreatography. We could also manage this isolated common bile duct stricture successfully with endoscopic nasobiliary drainage and plastic stent insertion instead of surgical correction. There appeared to be no recurrence of stricture as evidenced by biochemical test and ultrasonography during 2 years of follow up.

산전 초음파로 발견된 수신증의 치료 방침

한혜원,전누리,김건석,문대혁,윤종현,박영서,Hahn Hye-Won,Jun Nu-Lee,Kim Kun-Seok,Moon Dae-Hyuk,Yoon Chong-Hyun,Park Young-Seo 대한소아신장학회 2003 Childhood kidney diseases Vol.7 No.1

목적 : 신생아 수신증은 산전 초음파 시행의 증가와 함께 그 빈도가 증가하고 있는 질환이나 그 자연 경과에 대한 이해의 부족으로 산후의 치료, 특히 수술적 중재에 대한 합의는 아직 이루어지지 않은 상태이다. 저자들은 미리 정해진 원안에 따라 추적 관찰 중인 환아들의 경과 관찰을 통해 수술 가능성을 시사하는 조기 검사 결과와 술후 신기능 저하의 위험 인자를 분석하여 수신증의 치료 지침을 정립하고자 하였다. 방법 : 1994년 4월부터 2000년 6월까지 산전초음파 검사에서 수신증이 발견되어 출생 1개월이내 시행한 신초음파 검사에서 진단이 확인된 환아들을 대상으로 하였다. 일측성 수신증을 가진 환아들만을 대상으로 하였으며 방광요관역류를 포함한 요로계 기형이 동반된 환아들은 제외하였다. 128명의 환아의 128 신단위(renal unit)가 대상이 되었으며, 이들 중 12개월 이상 추적관찰이 된 90 신단위만을 연구 대상으로 하였다. 신단위는 생후 3일 이후부터 1개월 이내에 시행한 신초음파 검사의 신우 전후경에 따라 6개의 군으로 나누었으며 경과 추적 관찰 및 수술적 중재는 미리 정해진 원안(set protocol)에 따라 시행하였다. $^{99m}Tc-MAG3$과 신장 초음파 검사를 3, 6, 12개월에 시행하였고 신초음파 검사에서 신우 전후경과 Society for Fetal Urology grade를 측정하였다. 결과 : 초기 APPD가 10 mm 미만인 관에서는 수술을 받은 예가 없었으며 대부분 완해 혹은 호전의 경과를 보였다. APPD 25 mm 이상군에서는 대부분 수술을 받았으며, 초기 APPD가 10-19 mm인 군에서는 12개월에 완해부터 수술까지 다양한 결과를 보였다. 초기 APPD가 10-19 mm인 군에서 pyeloplasty를 시행받은 환아들의 초기 검사소견을 수술을 받지 않은 환아들과 비교하였을 때, 수술을 받게 된 환아들에서는 초기의 SFU 지수가 유의하게 높았으며 신기능곡선의 모양이 폐색의 형태가 주로 보였다. 술후의 신기능을 추적하였을 때, 신기능이 저하된 군에서는 기능이 회복된 군과 비교하여 술전 신기능이 감소하여 있었고, 수술 당시 연령이 증가되어 있었다. 결론 : 초기 신초음파 검사에서 신우 전후경이 10 mm 미만인 경우는 대부분 자연 소실되므로 경과 관찰만 하면 되고, 20 mm 이상인 경우는 대부분 수술이 필요하며, 10-19 mm인 환아들에서 SFU 지수가 높거나 MAG3 스캔에서 폐색을 시사하는 소견이 있을 경우, 신기능의 저하가 진행될 수 있으므로 조기 수술이 신장 기능 보존을 위해 바람직할 것으로 생각되며, 수술 적응증을 상대 신기능 40% 이하로 상향 조정하는 것이 추천된다. Purpose : Neonatal hydronephrosis has been detected with increasing frequency with the widespread use of prenatal ultrasonography, but the consensus about its postnatal management has not yet been reached, especially about surgical intervention. We attempted to determine the guideline of follow-up study and surgical intervention of hydronephrosis by analyzing clinical outcomes of neonates with hydronephrosis. Materials and Methods : Between 1994 and 2000, 128 hydronephrotic kidneys were postnatally confirmed. Cases associated with other urologic anomalies were excluded and 90 unilateral hydronephrotic kidneys with a minimum follow-up of 12 months were enrolled in this study. We classified the patients into 6 groups according to the anterior posterior pelvic diameter(APPD) at initial ultrasonography(USG) within 1 month after birth. Renal USG and $Tc^{99m}-mercaptoacetyl$ triglycerine(MAG3) scan were done according to a set protocol, and pyeloplasty was performed when indicated according to our protocol. Results : Most cases whose APPD were below 10 mm improved or resolved. Only few cases with APPD above 20 mm showed spontaneous improvement and most(88%) had undergone operation. Those with initial APPD within 10-19 mm showed variable outcomes. When the risk factors for irreversible renal functional deterioration were analyzed, the age at pyeloplasty and pre-operative functional deficit were significant. Conclusion : We concluded that in infants with initial APPD below 10 mm, consideration of surgery is not needed, and in those with initial APPD above 20 mm, early operation is recommended. Our set protocol based on initial USG is useful, but the cut-off value of relative renal function(RRF) for operation might be increased to 40% to improve post operative RRF.

신생아 및 영아기의 허쉬슈프렁병 진단

김대연,김성철,김경모,김애란,김기수,김정선,구현우,윤종현,김진천,피수영,김인구,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Kyung-Mo,Kim, Ellen Ai-Rhan,Kim, Ki-Soo,Kim, Jung-Sun,Goo, Hyun-Woo,Yoon, Chong-Hyun,Kim, Jin-Cheon,Pil, Soo-Young,Kim, I 대한소아외과학회 2002 소아외과 Vol.8 No.1

Diagnosing Hirschsprung's disease is a clinical challenge. Hirschsprung's disease should be considered in any child who has a history of constipation dating back to the newborn period. We examined diagnostic methods and their results retrospectively in 37 neonates and infants who underwent both barium enema and anorectal manometry for the diagnosis of Hirschsprungs disease at Asan Medical Center between January 1999 and April 2001. Two radiologists and a surgeon repeatedly reviewed both of the diagnostic results. In anorectal manometry, thirty-four studies were in agreement with the definitive diagnosis, giving an overall diagnostic accuracy of 91.9 % (neonate; 100 %, infant; 85.7 %). The accuracy and specificity of barium enema was lower than those of anorectal manometry, but sensitivity was higher. There was no significant difference between the two methods. Both studies showed findings consistent with the final diagnosis. However, discordant results needed further evaluation or close observation to diagnose accurately. We conclude that Hirschsprungs disease should not be diagnosed by only one diagnostic method.

혈청음성 척추관절병증 환자의 발꿈치뼈 부착부병증에 대한 초음파 검사의 유용성

김해림 ( Hae Rim Kim ),홍지현 ( Ji Hyun Hong ),윤종현 ( Chong Hyeon Yoon ),이상헌 ( Sang Heon Lee ),박성환 ( Sung Hwan Park ),김호연 ( Ho Youn Kim ) 대한류마티스학회 2005 대한류마티스학회지 Vol.12 No.2

Objective: To determine the diagnostic value of ultrasonography (US) in detection of calcaneal enthesopathies and compare US findings with clinical examination and laboratory data in patients with seronegative spondyloarthropathy (SpA). Methods: We studied fifty six patients with SpA (ankylosing spondylitis 51; psoriatic arthritis 2; reactive arthritis 3). Gray scale US and power Doppler sonography (PDS) was performed in Achilles tendons and plantar fascia using a 40 mm, 12 MHz linear probe to detect tendon thickness, loss of normal fibrillar echogenecity, blurred tendon margin, calcification, fluid collection around tendon, bony erosion, enthesopathic spur, retrocalcaneal bursitis and increased vascularity. Clinical examination including Mander enthesis index (MEI) score, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) were examined at the same time. Results: In 112 Achilles tendons, 72.3% showed abnormal US findings, as followings, increased tendon thickness 50.9%; loss of normal fibrillar echogenecity 32.1%; blurred tendon margin 24.1%; calcification 5.4%; fluid collection around tendon 17.7%; bony erosion 16%; enthesopathic spur 8.9%; retrocalcaneal bursitis 13.4%; and increased vascularity in power Doppler sonography (PDS) 14.2%. In 112 plantar aponeurosis, 59.8% showed abnormal US withenthesopathic spur 8.9%; retrocalcaneal bursitis 13.4%; and increased vascularity in power Doppler sonography (PDS) 14.2%. In 112 plantar aponeurosis, 59.8% showed abnormal US findings, as followings, increased tendon thickness 12.5%; loss of normal fibrillar echogenecity 50%; blurred tendon margin 30.3%; bony spur 2.7%; and increased vascularity in PDS 4.5%. PDS findings well correlated with findings of gray scale US. While 46% of symptomatic patients and 41.2% of patients with tenderness have abnormal X-ray findings, 69.4% of symptomatic patients and 73.8% of patients with tenderness have abnormal US findings. Patients with clinical symptoms, elevated CRP level and >1 MEI score showed increased vascularity in PDS. Conclusion: US is a simple and useful method in the detection of enthesopathies of SpA, even in patients without clinical symptom nor abnormal radiographic finding, and PDS combined with gray scale US is more sensitive tool which reflects the clinical examination.

일차성 쇼그렌증후군 환자에서 나타나는 자가항체와 선외증상의 양상

서수홍 ( Soo Hong Seo ),김현숙 ( Hyun Sook Kim ),곽승기 ( Seung Ki Kwok ),주지현 ( Ji Hyeon Ju ),김상현 ( Sang Hyon Kim ),윤종현 ( Chong Hyeon Yoon ),김호연 ( Ho Youn Kim ),박성환 ( Sung Hwan Park ) 대한류마티스학회 2007 대한류마티스학회지 Vol.14 No.1

Objective: To investigate the extraglandular manifestations and serological features of Korean patients with primary Sjogren`s syndrome (pSS). Methods: Clinical and laboratory data of 125 patients with pSS attending rheumatology clinic of Kangnam St. Mary`s hospital, with a mean follow-up period of 64.8 months, were assessed retrospectively. Results: Arthralgia was the most common extraglandular manifestation of pSS. Other extraglandular manifestations, such as myalgia, peripheral neuropathy, Raynaud`s phenomenon, and hypothyroidism were frequently presented, but lymphoma was rare manifestation in Korean patients with pSS. Some extraglandular manifestations of pSS were associated with hypocomplementemia and antibodies against Ro, and La, and antinuclear antibody. Conclusion: The clinical manifestations and extraglandular manifestations of Korean patients with pSS patients were similar to other studies except low prevalence of lymphoma. Positivity of anti-Ro/SS-A, anti-La/SS-B antibodies and hypocomplementemia were closely associated with extraglandular manifestations in patients with pSS.

Haddad 증후군 1예

이민규,김준성,박성종,김기수,김인구,윤종현,김경모,Lee, Min Kyu,Kim, Joon Sung,Park, Seong Jong,Kim, Ki Su,Kim, In Koo,Yoon, Chong Hyun,Kim, Kyung Mo 대한소아소화기영양학회 2005 Pediatric gastroenterology, hepatology & nutrition Vol.8 No.2

저자들은 청색증, 복부 팽만을 주소로 내원한 생후 2일 된 남아에서 Haddad 증후군, 즉 결장 전체의 무신경절증 형태의 Hirschsprung병을 동반한 선천성 중추성 저환기 증후군 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다. The combination of Congenital central hypoventilation syndrome with Hirschsprung's disease, also known as Haddad syndrome, belongs to the family of diseases now designated as Neurocristopathies. We have experienced a case of Haddad syndrome in a male infant who presented with repetitive abdominal distension, bilious vomiting, and sleep apnea. Following colon study and rectal biopsy disclosed the absence of the ganglion cell. And the infant could not be weaned from mechanical ventilation since birth because of the absence of effective, spontaneous respiration during sleep. As he was diagnosed as Haddad syndrome, tracheostomy and ileostomy were performed consecutively. At the age of 4 months, he was relatively healthy but remained ventilator-dependent. We report the first Korean case of Haddad syndrome with a brief review of the related literature.