진단 전에 자반이 동반되지 않았던 Henoch-Schonlein 자반 위장병증의 임상적 고찰

오재민,박재홍,Oh, Jae Min,Park, Jae Hong 대한소아소화기영양학회 2004 Pediatric gastroenterology, hepatology & nutrition Vol.7 No.1

목 적: Henoch-Schonlein 자반증(HSP)은 비혈소판 감소성 피부 병변, 관절통 및 관절염, 신 질환, 산통성 복통이 특징적인 소혈관의 혈관 염증성 질환이다. 피부가 가장 흔히 침범되며 환자의 14~36%에서는 특징적 피부 자반이 나타나기 전에 복부 증상이 나타난다고 알려져 있다. 최근 들어 피부 발진이 없이 복통으로 발현되는 HSP에 대한 보고가 있으나, 아직까지 진단에 많은 어려움이 있으며 보다 체계적인 연구가 필요할 것으로 사료되어 본 연구를 시행하였다. 방 법: 1995년에서 2000년까지 HSP로 진단된 환아 85명을 대상으로 진단 전 피부 병변이 없었던 군(자반 음성군)과 있었던 군(자반 양성군)으로 구분하고 병력지를 근거로 후향적 조사 및 일부 검사에 대한 전향적 연구를 시행하였다. 결 과: 자반 양성군이 72명, 자반 음성군이 10명이었다. 자반 양성군의 경우 평균 연령 6.84세, 남녀비 1.25 : 1이었고, 자반 음성군의 경우는 평균 연령 7.07세, 남녀비 1 : 1이었다. 일반 혈액학적 검사에서 백혈구 증다증이나 혈소판 증가증의 빈도는 두 군 사이에 통계학적인 차이를 보이지 않았다(p value>0.05). C-염증성 단백의 증가는 자반 양성군에서 54.8% (34명), 자반 음성군에서 42.9% (3명)로 두 군 사이에 의미 있는 차이가 없었다(p value>0.05). 복부 증상의 경우 오심, 구토 등은 자반 음성군에서 8명(80%), 자반 양성군에서 27명(36%)으로 자반 음성군이 높은 빈도를 나타내었으나(p value=0.01), 흑색변이나 혈변과 같은 위장관 출혈을 보인 경우는 자반 양성군에서 21명(29.2%), 자반 음성군에서 4명(40%)으로 두 군 사이에 통계학적인 차이가 없었다. 관절 증상이나 신 침범의 동반 빈도도 양군에서 차이를 보이지 않아 자반을 제외한 임상 양상은 두 군이 서로 비슷했다. 자반 음성군의 경우 복부 초음파 검사 및 소장 조영술에서 소장 점막의 비후로 인한 변화가 관찰되었고, 상부 위장관 내시경을 시행한 8명 중, 6명에서 융합성의 미란성 발적, 부종, 다발성 선상 궤양, 보라빛 변색 등이 위 전정부 또는 십이지장 구부나 둘째부에서 관찰되었다. 2명에서는 대장 내시경 검사를 시행, 1명에서 직장강 내에 미란성의 병변을 확인하였다. 3명의 환아를 대상으로 정상 피부에서 조직 검사를 시행하였는데 백혈구 파괴성 혈관염에 합당한 소견을 보였다. 자반 음성군 환아 10명 중 9명에서 스테로이드를 경구 또는 정주하여 증상의 호전을 보였으며 1명에서는 단순 진통제만으로 증상 경감을 보였다. 결 론: 진단 시 자반이 없었던 경우 일반적인 검사에서는 진단적 소견이 없었으나, 위장관 내시경 검사에서 HSP 위장병증에 합당한 소견이 관찰되거나 복부 초음파 검사 또는 소장 조영술 검사에서 장벽의 변화, 피부 또는 신장 조직 검사에서 백혈구 파괴성 혈관염이 입증될 경우 진단이 가능할 것으로 사료된다. Purpose: Henoch-$Sch{\ddot{o}}nlein$ purpura (HSP) is a small-vessel vasculitic disease that most often affects the skin. Abdominal symptoms precede the typical purpuric rash of HSP in 14~36%. It is a challenge to diagnose HSP in the absence of a rash, because there are no biologic tests that can identify HSP with certainty, so we tried to find out the characteristic features of HSP gastroenteropathy without purpura before diagnosis. Methods: This study included 82 children with HSP who had been admitted or visited outward of the Department of Pediatrics, Pusan National University Hospital from 1995 to 2000. The cases that the onset of purpura preceded or coincided that of abdominal pain were defined as purpura-positive group. The cases that the onset of abdominal pain preceded purpura more than 1 week and purpura was not presented till diagnosed as HSP gastroenteropathy were defined as purpura-negative group. We compared and analyzed the clinical features of the two groups by reviewing the medical records retrospectively. To ensure the diagnosis of HSP gastroenteropathy, we conducted upper GI series, abdominal ultrasonogram, abdominal CT, endoscopy and/or skin biopsy. Results: The number of cases of purpura-positive group and purpura-negative group were 72 and 10, respectively. There is no difference between two groups in the incidence of clinical symptoms and laboratory findings. Children with HSP gastroenteropathy had characteristic erosive or ulcerative lesions in the stomach or duodenum on esophagogastroduodenoscopy, or mural thickening of the small bowel on abdominal ultrasonogram, CT or upper GI series. Skin biopsy revealed leukocytoclastic vasculitis in 3 of them, although biopsy specimen was taken from any areas of normal- appearing skin. In purpura-negative group, 9 patients improved by steroid therapy. Conclusion: In purpura-negative group, there is no diagnostic feature on the laboratory findings and clinical features. Therefore, to diagnose HSP gastroenteropathy in patients with abdominal pain in the absence of the characteristic rash, careful observation of clinical features and laboratory data, and prompt application of available diagnostic tools such as gastrointestinal endoscopy, radiologic study and skin biopsy are recommended. Early use of corticosteroid may reduce the suffering in these patients.

위장관을 침범한 hench - Schoenlein Purpura 2예 - 내시경적 소견을 중심으로 -

김원호(Won Ho Kim),박찬일(Chan Il Park),송영구(Young Goo Song),박상진(Sang Jin Park),강진경(Jin Kyoung Kang),박인서(In Seo Park),이상엽(Sang Yup Lee) 대한소화기학회 1993 대한소화기학회지 Vol.25 No.6

Henoch-Schoenlein purpura (H-S purpura) is a systemic vasculitis of unknown cause that is characterized by palpable purpura (usually on the lower extremities and over the buttocks), arthralgias, gastrointestinal symptoms, and glomerulonephritis. Diagnosis of H-S purpura is sometimes difficult, since GI symptoms precede the skin lesions in 10%. of patients with H-S purpura. It has been suggested that endoscopic and radiologic studies may be helpful in the diagnosis of H-S purpura, but there seem to be no endoscopic findings specific for H-S. purpura. We performed GI endoscopy in two patients with H-S purpura. Upper GI endoscopy showed multiple conglomerated erosions only on the fold in 2nd portion of the duodenum and intervening mucosa between folds were not remarkable in both patients, and colonoscopy showed several erosions on terminal ileum in one patient.

Allergic Purpura에 합병된 Acute Pancreatitis 1례

이성철,김화자,차한,박호진,Lee, Sung-Chul,Kim, Hwa-Ja,Tchah, Hann,Park, Ho-Jin 대한소아소화기영양학회 1999 Pediatric gastroenterology, hepatology & nutrition Vol.2 No.1

저자들은 복통, 구토, 혈변을 주소로 입원한 6세 여아에서 allergic purpura에 급성 췌장염이 병발된 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다. Allergic (Henoch-Schonlein) purpura is a nonthrombocytopenic purpura that affects small nonmuscular vessels with involvement of skin, gastrointestinal tract, joint, and kidney. Pancreatitis has rarely been reported as a complication of H-S purpura. In 1965, hemorrhagic pancreatitis as a complication of H-S purpura was reported by Toskin. We experienced one case of pancreatitis complicated with H-S purpura: pancreatic head enlargement was noted in small bowel series and abdominal sonogram with increased serum amylase level (160 U/dL) in a 6-year-old female child who complained of abdominal pain, vomiting, bloody stool, and petechiae on lower extremities.

알레르기성 자반증의 치료법에 관한 문헌고찰 -중의학 저널을 중심으로-

석연희,민상연,김장현,Suk, Yun Hee,Min, Sang Yoen,Kim, Jang Hyun 대한한방소아과학회 2013 대한한방소아과학회지 Vol.27 No.4

Objectives Allergic purpura is frequent disease in children. The purpose of this study is to analyze traditional Chinese medicine treatments of Allergic purpura in children through traditional Chinese medical journal research. Methods 22 studies were selected based on title keyword "Allergic purpura" "Child" published in CAJ (China Academic Journal). The studies were about clinical effect, herbs, injections, treatments, and nursing in Allergic purpura in children. Results & Conclusions There are so many treatments studied on purpura in children based on CAJ. A Single or combination traditional Chinese medicine treatment is effective in Allergic purpura in children. Therefore, we need to study more about Allergic purpura in children and should make more clinical cases about it.

증례 : 심한 증식성 루푸스 신염과 혈전성 혈소판 감소성 자반증이 병발한 남성 환자에서 cyclophosphamide 치료 경험 1예

정혜원 ( Hae Won Jung ),신준암 ( Jun Am Shin ),이유지 ( Yu Ji Lee ),강나리 ( Na Ree Kang ),권기영 ( Ghee Young Kwon ),한봉준 ( Bong Jun Han ),김윤구 ( Yoon Goo Kim ) 대한내과학회 2006 대한내과학회지 Vol.71 No.2

TTP가 병발한 SLE에서 조기에 신선 냉동 혈장 투여와 혈장 교환술을 적극적으로 시행해야 한다. 이러한 적극적인 초기 치료에도 불구하고 병이 진행하거나, 루푸스 신염이 동반된 경우에 cyclophosphamide의 병합 요법이 치료에 도움이 됨을 저자들은 경험하여 보고하는 바이다. Thrombotic thrombocytopenic purpura is a rare but fatal complication of systemic lupus erythematosus. The diagnosis of thrombotic thrombocytopenic purpura as a syndrome distinct from systemic lupus erythematosus may be challenging particularly when thrombotic thrombocytopenic purpura is presented concomitantly with systemic lupus erythematosus. Early diagnosis and aggressive treatment including plasmapheresis would be required. However, recent reports have suggested that the use of cyclophosphamide may have a role. We describe a patient with systemic lupus erythematosus who was first presented with severe thrombotic thrombocytopenic purpura. Diagnosis was based on typical clinical features of thrombotic thrombocytopenic purpura and laboratory findings of active lupus nephritis. Renal biopsy also confirmed the coexistence of thrombotic thrombocytopenic purpura and diffuse proliferative lupus nephritis. Although prompt extensive plasmapheresis and high dose steroid therapy were performed, oliguric renal failure and thrombocytopenia persisted. After addition of cyclophosphamide to the treatment with plasmapheresis and steroid, clinical manifestations of thrombotic thrombocytopenic purpura and lupus nephritis were markedly improved. (Korean J Med 71:214-218, 2006)

Henoch-Schonlein 자반증 환아의 임상적 고찰

하태선,구현회,Ha, Tae-Sun,Koo, Hyun-Hoe 대한소아청소년과학회 2003 Clinical and Experimental Pediatrics (CEP) Vol.46 No.11

목 적 : 지난 30년간의 연구로 병인론에 관한 중요한 정보들이 축적되어왔다. 하지만 아직도 완전히 이해된 것은 아니며, 연령이나 성별, 임상증상의 발현율에 대한 다양한 연구 결과들이 발표되고 있다. 이에 본 연구에서는 저자들이 경험한 125명의 HS 자반증 환아들의 임상적 특징을 알아보고, 이전의 다른 논문의 연구결과들과 비교해 보고자 하였다. 방 법 : 환아는 1992년 3월부터 2000년 4월까지 충북대학교병원 소아과에 방문하여 특징적인 자반과 복통이나 관절염 등의 임상증상을 통하여 HS 자반증으로 진단된 환아를 대상으로 하였으며 입원하거나 외래에 내원한 경우 모두 포함하였다. 결 과 : HS 자반증으로 진단된 환아는 남자 87명 여자 38명으로 구성되었고, 연령은 1세에서 14세에 걸쳐 분포하고 있었다. 계절에 따른 변화로 3월부터 5월까지 모두 56명(44.8%), 10월에서 12월까지 51명(32.8%)으로 나타났다. 자반은 모든 환아에서 관찰되었다. 복통은 88명(70.4%)에서 관찰되었으며, 위장관 출혈은 18명(14.4%)에서 관찰되었고, 관절염은 67명(53.6%)에서 관찰되었다. 모두 125명의 환아 중 신장 침범이 있는 환아는 48명으로 HS 자반증 환아 중 38.4%였다. 부고환염은 남아 87명 중 15명(17.2%)에서 발생하였다. 자반이 발생한 후 나타난 동반 증상 중 2명(1.6%)의 환아에서 경련이 발생하였으나 항경련제 치료없이 호전되었고, 두통이 11명(8.8%)의 환아에서 발생하였다. 결 론 : 결론적으로, HS 자반증은 전신적인 혈관염이기 때문에 자반 이외의 증상들이 선행할 수 있으며, 소아에서 설명할 수 없는 복통이나 관절염, 음낭 증상 등이 발생할 경우, 자반이 출현하기 전이라도 임상 각과에서 HS 자반증에 대해 의심해보고, 소아 신장 전문의와의 협진이 필요할 것으로 사료된다. Purpose : Henoch-$Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis, characterized by cutaneous purpura, abdominal pain, arthralgia and renal involvement. The clinical features of HSP have been reasonably well documented but there are still many gaps in our understanding of HSP. The aim of this study was to present the clinical features of 125 children with HSP and compare them with previous reports, placing particular emphasis on clinical information. Methods : We collected the clinical data of 125 patients with acute HSP who visited Chungbuk National University Hospital from March 1992 to April 2002. Data were expressed as the mean or $mean{\pm}SD$ and statistical analysis was performed using Chi-square approximation. P<0.05 was considered as significant. Results : The patient population consisted of 87 boys and 38 girls ranging in age from one to 14 years. HSP occurs throughout the year, but this study shows seasonal skewing, with most patients presenting from fall through spring and a paucity of cases in summer. All patients had non-thrombocytopenic purpura concentrated on the buttocks and lower extremities. Purpuric lesions were also scattered on the arms and occaisionally on the face and ears, but the trunk was largely spared. A recurrence of purpura was defined as the reappearance of a rash or other symptoms following resolution of disease for at least two weeks. The mean number of recurrences was 0.51. Eighty eight patients(70.4%), 18 patients(14.4%) and 67 patients(53.6%) complained of abdomianl pain, gastrointestinal bleeding and arthralgia, respectively. Nephritis occurred in 48(38.4%) patients. Fifteen boys (17.2%) developed epididymitis. Neurologic features occurred in 13(10.4%) and two(15%) of these were seizures. Conclusion : HSP all showing purpura as defined is characterized by various clinical features, including abdominal pain, arthralgia, epididymitis and nephritis which could occur before the appearance of purpura. Therefore, we suggest that the possibility of HSP should be considered in children before invasive procedures, even if the above symptoms and signs present without purpura.

Henoch-Schönlein 신염의 예측인자로서 Neutrophil-Lymphocyte Ratio의 유용성에 대한 연구

김민솔,서원석 순천향대학교 순천향의학연구소 2019 Journal of Soonchunhyang Medical Science Vol.25 No.2

Objective: Henoch–Schönlein purpura is the most widespread systemic vasculitis during childhood. This study evaluated the relationship between neutrophil-lymphocyte ratio (NLR) and Henoch–Schönlein nephritis, which is important for the long-term prognosis of Henoch–Schönlein purpura. Methods: We retrospectively reviewed patients diagnosed with Henoch–Schönlein purpura at the Department of Pediatrics of Soonchunhyang University Bucheon Hospital between January 2007 and June 2018. One hundred and twelve patients under the age of 18 who were diagnosed with Henoch–Schönlein purpura according to Henoch–Schönlein purpura diagnostic criteria, European League Against Rheumatism were included in the study. Blood and urine test, gastroscopy and colonoscopy, and abdominal computed tomography performed during the first visit were included. Results: Among 112 Henoch–Schönlein purpura patients, 25 patients were Henoch–Schönlein nephritis. NLR was significantly increased in patients with Henoch–Schönlein nephritis compared to patients without nephritis (P=0.042), which is an independent predictor of Henoch–Schönlein nephritis. Conclusion: This study showed that NLR could be a useful predictor of Henoch–Schönlein nephritis in patients with Henoch–Schönlein purpura.

Henoch-Schlein Purpura 환자 치험 2례

김창훈,연경진,노석선 대한한방안이비인후피부과학회 2005 한방안이비인후피부과학회지 Vol.18 No.3

The Henoch-Schnlein Purpura (HSP) is a systemic small vasculitis, characterized by palpable purpura, abdominal pain, hematuria or proteinuria, and arthritis. The etiology is unknown but it is manifested by allergic reaction, so it is called Anaphylactoid Purpura. The prognosis of disease is usually good, but about 50% of patients have experienced recurrences. Oral corticosteroid is treatment of choice, but it cannot prevent recurrence of Disease. We experienced two case of young patients who suffered from the Henoch-Schnlein purpura. We prescripted Mokyangbinrang-hwan, Insamyang-witang, Gwibi-tang. For both two patient, symptoms were much relieved.

위장관을 침범한 Henoch-Sch o¨nlein purpura 1예

이승곤,김채규,서종옥,이호영,이호준,정회상,박유환,정춘해 朝鮮大學校 附設 醫學硏究所 1996 The Medical Journal of Chosun University Vol.21 No.2

Henoch-Scho¨nlein purpura(H-S purpura) is a systemic necrotizing vasculitis of small vessels that is characterized by nonthrombocytopenic palpable purpura on the buttocks and extremities, arthritis of knees and ankles, glomerulonephritis, and colicky abdominal pain, histologically characterized by leukocytoclastic vasculitis. Gastrointestinal involvement with colicky abdominal pain and GI bleeding, most frequently in jejunum and ileum, occurs in above a half of all patients, but common in children. We performed GI endoscopy in a case of H-S Purpura. Gastroduodenoscopic findings showed erythemas and erosions in gastric antrum, and mucosal edema and petechiae in duodenal 2nd portion. Colonoscopic findings showed 5-10㎜ sized multiple shallow hemorrhagic ulcers in transverse and descending colons We report a case of H-S purpura involving colon with literatural review.

소아 알레르기성 자반증의 임상적 고찰 및 스테로이드 치료가 알레르기성 자반증 신장염에 미치는 영향

신성호,임인석,이동근 중앙대학교 의과대학 의과학연구소 1995 中央醫大誌 Vol.20 No.1

Anaphylactoid purpura is a generalized vasculitis characterized by cutaneous purpura, abdominal pain, gastrointestinal bleeding and nephritis. This study was performed to analyzed clinical symptoms and to determine whether corticosteroid therapy was effective in preventing delayed nephritis in children with anaphylactoid purpura.Clinical manifestations and laboratory findings were reviewed by medical records in 62 cases with anaphylactoid purpura hospitalized at Chung-Ang University Hospital during the period between March, 1985 and February, 1994. The following results were obtained. 1) The age distribution of anaphylactoid purpura showed a peak incidence between 4 to 6 years of ago and male preponderance was observed(male to femals ratio =1.3:1). 2) In seasonal incidence, the cases were frequent during autumn(34%) and Spring(29%). 3) No specific history was noted in 33 cases(53%), previous history of upper respiratory infection was noted in 23 cases(37%) and only 6 cases(10%) showed gastroenteritis. 4) The clinical manifestations showed in skin(100%), gastrointestinal tract(81%), joint(79%), kidney(34%) respectively. 5) As for the gastrointestinal symptoms, abdominal pain was observed in all cases and others were occult blood(50%), and nausea and vomiting(38%). 6) The joint swelling was observed mostly on knee joint(80%), and ankle joint(61%). 7) Hematologically, mild anemia was observed in 29% of cases, leukocytosis in 61% of cases, increased platelet count in 52% of cases, increased ESR in 58% of cases. Positive anti-streptolysin 0 titer was observed in 39% of cases and positive C-reactive protein in 67% of cases. In coagulation studies were within normal ranges. 8) skin, abdominal and joint manifestation was recovered within 1 month. As to the 21 cases with renal involvement, 4 cases(19%) normalized within 1 month, and at least 11 cases(52%) showed persistent abnormal finding in urinalysis over 1 month after initial presentations. 9) Delayed nephritis developed in 7 of 35(20%) patients who received prior treatment with corticosteroids and in 3 of 16(19%) patients who were not treated. Thus, prior corticosteroid therapy had no effect on the frequency of delayed nephritis.