골수 섬유증 14예에 대한 고찰

김성숙,백애란,백인기,고일향,임경호,김예회 인제대학교 1985 仁濟醫學 Vol.6 No.1

1976년 5월부터 1983년 4월까지 서울 백병원에서 골수 섬유증으로 진단된 14예에 대해 임상 및 병리학적 검색을 하며 그 결과를 보고하고자 한다. Myelofibrosis, which is a relatively rare disease, is classified into secondary and idiopathic. Idiopathic myelofibrosis is a group of myeloproliferative disorder and subdivided into chronic and acute from as different disease entity. 14 cases of myelofibrosis diagnosed at Seoul Paik Hospital from April, 1975, to April, 1983 were reviewed. The results are summarized as follows: 1.According to etiology, idiopathic forms was 7 cases and secondary form was 7 cases. The associated diseases in the secondary form were chronic myelogenous leukemia (4 cases), malignant lymphoma (1 case), acute myelomonocytic leukemia (1 case) and metastatic carcinoma (1 case). 2.According to age and sex distribution, there were 4 males and 10 females (M:F ratio was 1:6 in the idiopathic form). Cases were distributed in all age group from 2nd decade to 8th decade, 8 of 14 cases were found in 3rd and 4th decade. (4 of 7 idiopathic cases were in 3rd decade). 3.According to degree of fibrosis, mild (grade 2+) from was 7 cases, moderate (grade 3+) was 4 cases, and marked (grade 4+) was 3 cases. There was no significant correlation between the degree of fibrosis and age or type. 4.According to type, chronic idiopathic myelofibrosis was 4 cases, acute IMF was 2 cases and undetermined form was 1 case.

특발성 아나필락시스 환자에서 오말리주맙의 치료 효과

반가영 ( Ga Young Ban ),양은미 ( Eun Mi Yang ),김지혜 ( Ji Hye Kim ),신유섭 ( Yoo Seob Shin ),예영민 ( Young Min Ye ),남동호 ( Dong Ho Nahm ),박해심 ( Hae Sim Park ) 대한천식알레르기학회(구 대한알레르기학회) 2015 Allergy Asthma & Respiratory Disease Vol.3 No.5

Anaphylaxis is a severe and life-threatening systemic reaction. Despite the extensive evaluation to determine the cause, 30%-60% of cases of anaphylaxis in adults remain idiopathic. Recently, omalizumab treatment has been postulated to treat refractory idiopathic anaphylaxis. We report a case of idiopathic anaphylaxis treated with omalizumab and investigated its pharmacological mechanism. A 66-year-old female presented to our clinic with recurrent anaphylaxis. She suffered from anaphylaxis 2-3 times a month for 6 months. She had past medical history of nonallergic bronchial asthma. History was carefully undertaken and anaphylaxis was not related to any specific foods, drugs, exercise, and insect bites. Serum specific IgE antibodies to common food allergens showed negative results. Oral provocation tests to food additives revealed to be negative. To screen systemic mastocytosis and mast cell activating syndrome, baseline tryptase level was checked, and it was within normal range. From comprehensive evaluation, she was diagnosed as having idiopathic anaphylaxis. She could not tolerate oral medications due to gastrointestinal discomfort, therefore, omalizumab treatment (150 mg, monthly) was started. After 6 months of treatment, anaphylaxis did not occur with complete remission status. To evaluate the pharmacological mechanism of omalizumab treatment, basophil histamine releasability test was performed. Histamine releasability induced by anti-IgE did not change after 6 months of treatment, while that induced by calcium inophore decreased. Omalizumab treatment can induce remission or favorable effects on idiopathic anaphylaxis, which may be derived from increased threshold of mast cell degranulation. Long-term studies in a larger cohort will be needed to confirm its efficacy. (Allergy Asthma Respir Dis 2015;3:380-383)

항경련제 복용을 중단한 특발성 간질 환아 치험 1례

한재경,김윤희,김윤희,Han, Jae-Kyung,Kim, Yun-Hee,Kim, Yun-Hee 대한한방소아과학회 2004 대한한방소아과학회지 Vol.18 No.2

Objective : Epilepsy is a brain condition which causes a person to suddenly lose consciousness and sometimes to have fits. The etiologic factors of epilepsy are various, but most of cases are idiopathic. The purpose of this study is to report a pediatric patient with idiopathic epilepsy. Method : We treated him with herb medicine, acupucture and anticonvulsant. Then, we stopped administering anticonvulsant, and observed the progress of his condition. Result : We had good effect with oriental medical treatment on an epileptic who quitted taking anticonvulsant. Conclusion : We report the good results of oriental medicine on a pediatric patient with idiopathic epilepsy. And the further study is needed with more cases, longer duration and other tools.

소아에서 일차성 막성 신병증의 임상-병리학적 고찰

이범희,조희연,강주형,강희경,하일수,정해일,이현순,최용,Lee Bum-Hee,Cho Hee-Yeon,Kang Ju-Hyung,Kang Hee-Gyung,Ha Il-Soo,Cheong Hae-Il,Lee Hyun-Soon,Choi Yong 대한소아신장학회 2003 Childhood kidney diseases Vol.7 No.2

목적 : 막성 신병증은 소아에서 드문 신질환이다. 저자들은 일차성 막성 신병증의 임상 경과의 이해와 치료 방침의 결정에 도움을 주고자 일차성 환자들을 후향적으로 고찰하였다. 방법 : 1977년부터 2003년에 소아 막성 신병증 환자 58명 중 42명(72.4%)이 B형 간염 연관성이었고, 16명(27.6%)이 일차성이었다. 2000년 이후 진단된 환자는 모두 일차성이었다. 임상-병리학적 소견(성별, 연령, 단백뇨, 혈청 알부민, 콜레스테롤, 크레아티닌 청소율, 신세뇨관-간질 변화, 신사구체 경화증, 고혈압, 신정맥혈전증, ACE inhibitor 및 면역억제제 사용력)을 조사하였고, 관해군과 비관해군 간 비교를 하였다. 결과 : 남자 6명, 여자가 10명이었고, 진단 시 중앙 연령은 13세 5개월이었다. 발현 증상은 신증후군(7명, 43.8%), 육안적 혈뇨(5명, 31.3%)와 현미경적 혈뇨 및 단백뇨(3명, 18.8%)였다. 고혈압(2명, 12.5%), 신정맥 혈전증(2명, 12.5%)과 저칼슘성 테타니(1명)가 동반되었다. 조직소견 상범사구체성(6명, 37.5%) 또는 분절성 경화증(5명, 31.3%), 반월체(1명)와 경도(11명, 68.7%) 또는 중등도의 신세뇨관-간질 변화(3명, 18.8%)가 보였다. 13명(86.7%)이 스테로이드를 투여 받았고, 이 중 2명은 cyclophosphamide, 1명은 cyclosporin을 투여 받았다 10명(52.5%)은 ACE inhibitor를 투여받았다. 진단 1개월 후 누락된 1명을 제외하고, 7명(46.7%)에서 단백뇨가 소실되었고, 8명(53.3%)은 단백뇨가 지속되었으며, 그 중 2명(13.3%)은 만성신부전으로 진행하였다. 관해군과 비관해군간에 임상-조직 병리학적 소견은 유의한 차이가 없었다. 결론 : 우리나라 소아에서 막성 신병증은 일차성이 막성 신병증의 대부분을 차지하게 되었는데, 그 이유는 B형 간염 예방 접종 도입 이후 B형 간염 연관성 막성 신병증이 현저하게 감소하였기 때문이다. 이 질환의 임상 경과의 이해와 치료 방침의 확립을 위해서 다기관의 전향적 연구가 필요하다. Purpose : Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. Methods : Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed aster 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. Results : The median onset age was 13.4 years. Clinical manifestations were nephrotic syn-drome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. Conclusion : With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done.

상완부에 발생한 특발성 구획증후군

정덕환,김영준,김정석 대한수부외과학회 2015 대한수부외과학회지 Vol.20 No.2

Compartment syndrome is caused by elevated pressure in a restricted compartment. It typically occurs after fractures of the extremities and usually has an acute clinical progression. Chronic compartment syndrome is another relatively well known form, typically associated with forceful exercise. Also, there are various reports of compartment syndrome not associated with typical causes. However, reports on compartment syndrome with unknown etiology are rare and there has been none in Korean literature. We report a case of compartment syndrome with no recognizable cause, hence classified as idiopathic. 구획증후군은 제한된 구획 내 압력의 상승으로 인하여 발생하는 질환이다. 사지의 골절 후 발생하여 급성의 경과를 보이는 경우가 전형적이다. 다른 형태로 심한 운동과 관련된 만성 구획증후군이 알려져 있고, 이러한 전형적인 원인과 관련되지 않은 구획증후군의 보고가 다양하게 있어왔다. 하지만, 원인을 찾을 수 없는 구획증후군의 보고는 매우 드물며 국내의보고는 없었다. 저자들은 젊은 건강한 성인의 상완부에서 원인을 알 수 없는 구획증후군을 경험하였으며 특발성으로 분류하였다.

미숙아에게서 기계환기 중 발생한 특발성 기복증

송준환 순천향대학교 순천향의학연구소 2016 Journal of Soonchunhyang Medical Science Vol.22 No.1

Pneumoperitoneum is generally a surgical emergency that needs immediate surgical intervention to improve survival in neonates. Its main etiologies include necrotizing enterocolitis, secondary meconium ileus, intestinal obstruction, or various other causes such as gastric perforation from the insertion of a nasogastric tube. However, pneumoperitoneum can rarely present without any gastrointestinal perforation. This condition has been called nonsurgical, asymptomatic, benign, misleading, spontaneous, or idiopathic pneumoperitoneum. In this report, a rare case of pneumoperitoneum in a premature infant is presented, the definite cause of which was not found even after an explorative laparotomy.

인공 대동맥판막 기능부전을 일으킨 특발성 과호산구 증후군 -1예 보고-

박종빈,유동곤,성규완,정상식,강길현,김종욱 대한흉부외과학회 2007 Journal of Chest Surgery (J Chest Surg) Vol.40 No.4

특발성 과호산구 증후군은 호산구 매개에 의한 조직 손상으로 인해 다발성 장기부전, 특히 심장을 침범하는 드문 전신성, 백혈구증식성 질환이다. 심장침범은 특발성 과호산구 증후군 환자의 75% 이상에서 일어난다. 심장증상은 심내막 하 섬유증, 제한성 심근병증, 판막부전, 그리고 말초동맥 혈전증을 유발하는 혈전이다. 이 질환은 남자에서 여자보다 9:1로 호발하고, 20∼50대에서 주로 발현하는 경향이 있으며 소아에서는 매우 드물다. 인공 대동맥판막부전을 나타낸 특발성 과호산구 증후군 환자(58세, 남자)를 인공판막 재치환 수술 후 부신피질호르몬제와 hydroxyurea 투여로 성공적으로 치료하였기에 보고하는 바이다.

신생검증례에 의한 사구체신질환의 분석

김기현(Ki Hyun Kim),권혁찬(Hyuk Chan Kwon),김문범(Moon Beom Kim),안원석(Won Suk An),감동호(Dong Ho Gam),김상곤(Sang Gon Kim),김성은(Seong Eun Kim),김종성(Jong Seong Kim) 대한내과학회 1995 대한내과학회지 Vol.49 No.5

N/A Objectives: We intended to evaluate the incidence and clinical characteristics of various glomerular diseases (GD) in adults including the patients with asymptomatic urinary abnormalities. Methods: We conducted retrospective study with review of histologic findings and clinical records of the 4% cases diagnosed as GD during 9years since 1985. The diagnoses of all cases were confirmed by renal biopsies. The laboratory studies to differentiate secondary diseases were also performed. Results: 1) There were 382 (78.6%) cases of primary GD and 104 (21.4%) of secondary GD. Among primary GD, IgA nephropathy (IgAN) was the most common as 151 (39.5%) cases followed by 123(32.2%) of minimal change nephrotic syndrome (MCNS), 34 (8.9%) of membrananous glomerulonephritis (MGN), 23 (6.0%) of membranoproliferative GN type I (MPGNI), and 21 of focal glomerulo- sclerosis (FGS) in the order of frequency. 2) In the secondary GD group, HBsAg positive cases were 49 (47.1%), and the rest was 35 (33.7%) of lupus nephritis (LN), 7(6,7%) of diabetic nephropathy, and 3 (2.9%) of HenochShoenlein purpura. 3) Out of 162 IgAN, 151(93.2%) cases were idiopathic- focal GN in 70(48.6%), diffuse mesangial proliferative (DMsPGN) in 40(27.8%), minor glomerular abnormality in 29(20.1%), and sclerosing GN in 5 (3.5%) cases. In 11cases of systemic disease-associated IgAN, there were 7 HBV surface antigenemia, 2liver cirrhosis, 1alcoholic liver disease, 1thin glomerular basement membrane disease. The frequent events causing medical attention in patients with IgAN were gross hematuria (36.6%), urinary abnormality on routine check (26.7%), and edema (21.4%), but only 11% of patients had true edema and hypoalbuminemia. 4) In idiopathic nephrotic syndrome group (INS, total 230cases), the prevalence of each disease and the incidence of clinically overt NS on admission were 60.6%, 16.7%, 11.3%, and 10.3% and 94%, 71%, 65%, and 81% in MCNS, MGN, MPGNI, and FGS, respectively. 5) Serum HBsAg was positive in 40% of MPGNI, 27.3% of MGN, and 5.7% of IgAN. 6) According to WHO classification, the histologic findings of LN (35 of 106cases, 33.0%) were type IV 48.6%, V in 28.6%, and each of II and III in Conclusion. 1) IgAK was the most common primary GD. Thus, gross hematuria or abnormal urine findings on routine examination should be considered as highly suggestive of IgAN. 2) MCNS was the most common cause of INS in this study. 3) HRV infection seems to have a significant role in the pathogenesis of MPGNI and MGN, but not in IgAN. 4) In secondary GD, SLE is an important systemic disease requiring renal biopsy.

증례 : 순환기 ; 다량의 심낭 삼출로 발현된 POEMS 증후군 1예

오창명 ( Chang Myung Oh ),방우대 ( Woo Dae Bang ),이상국 ( Sangkook Lee ),조성수 ( Sung Soo Cho ),노송미 ( Songmi Noh ),김진석 ( Jin Seok Kim ),장혁재 ( Hyuk Jae Chang ) 대한내과학회 2011 대한내과학회지 Vol.81 No.2

심낭 삼출은 악성종양, 결핵 등 다양한 원인에 의해 유발되는 질환으로 과거와는 달리 선험적인 치료에 의존하기 보다 정확한 원인 규명의 중요성이 점차 커지고 있다. 저자들은 심낭 삼출을 주된 증상으로 하면서 말초신경병증, 비장비대, 갑상샘저하증, 단세포감마글로불린병증, 피부색소침착, 다모증, 흉막 삼출, 전신 부종, 폐동맥고혈압 등을 동반한 POEMS 증후군 1예를 경험하여 문헌고찰과 함께 보고한다. 심낭 삼출의 치료에 반응이 없으면서 원인을 찾지 못하여 특발성 심낭염으로 진단한 환자의 추가적인 감별 진단의 하나로 POEMS 증후군이 고려되어야 하겠다. Pericardial effusions can be caused by a wide variety of infectious or noninfectious diseases. After a conventional diagnostic work-up, the etiology of pericardial effusion often remains idiopathic. We report a patient with POEMS syndrome whose main clinical problem was recurrent pericardial effusions. Patients with POEMS syndrome often have generalized edema and a pleural effusion, while a pericardial effusion is a very rare complication. A 44-year-old man visited our hospital because of a recurrent pericardial effusion. He was initially diagnosed with idiopathic pericarditis five months prior. We reassessed the patient meticulously and found IgG lambda type monoclonal gammopathy, polyneuropathy, lymphadenopathy, peripheral edema, pleural effusion, hypothyroidism, pulmonary hypertension, hyperpigmentation, hypertrichosis, and papilledema, which we diagnosed as POEMS syndrome. (Korean J Med 2011;81:245-250)

특발성 진성 성조숙증으로 진단된 소아의 원인 및 임상적 분석

김기훈,신동길,김대현,Kim, Ki-Hoon,Shin, Dong-Gil,Kim, Dae-Hyun 대한한방소아과학회 2011 대한한방소아과학회지 Vol.25 No.2

Objectives: The purpose of this study is to estimate genetic and environmental factors, which can affect Idiopathic true Precocious puberty, and to evaluate the clinical and endocrinologic characteristics. Methods: Retrospective and Comparative analysis of 76 children (72 girls and 4 boys) has been diagnosed with idiopathic true precocious puberty, and treated with GnRHa from December 2008 to July 2011. Results: 1. The Average chronological age (CA. yr) of children diagnosed with idiopathic true precocious puberty was $8.40{\pm}0.81$ (girls), $9.93{\pm}0.12$ (boys). 2. The Average height & weight percentile (%ile) of the girls diagnosed with idiopathic true precocious puberty was $67.38{\pm}22.04$, $67.69{\pm}23.20$. 3. The girls' mothers have diagnosed with idiopathic true precocious puberty, and they were shorter than the average. This shows that mother's small height and idiopathic true precocious puberty are closely related to each other. 4. BMI percentile (%ile) of girls diagnosed with idiopathic true precocious puberty was $63.26{\pm}24.86$. 23.6% of children were diagnosed with overweight or obesity. This result shows that obesity and idiopathic true precocious puberty are proportionally related. 5. Birth weights (kg) of the children diagnosed with idiopathic true precocious puberty were $3.16{\pm}0.43$ (girls), $3.15{\pm}0.38$ (boys). 8.3% of children were diagnosed with Intrauterine growth retardation. 6. The Average bone ages (BA. yr) of the children diagnosed with idiopathic true precocious puberty were $10.51{\pm}0.99$ (girls), $12.10{\pm}0.97$ (boys). The Average BA-CA was $2.11{\pm}0.81$ (girls), $2.00{\pm}0.87$ (boys). 7. The Average predicted adults' height (PAH. cm) of the children diagnosed with idiopathic true precocious puberty was $151.61{\pm}4.00$ (girls), $163.50{\pm}2.15$ (boys). The Average MPH-PAH was $6.84{\pm}4.91$ (girls), $6.00{\pm}5.35$ (boys). 8. 23.6% of the children treated with GnRHa were co-treated with Growth Hormone. Conclusions: Estimated factors which cause Idiopathic true precocious puberty are mother's small height, obesity, and Intrauterine growth retardation. However, the studies of Oriental Medicine for Idiopathic true precocious puberty were lacking. Further clinical and experimental researches are needed.