• Title/Summary/Keyword: Still disease, adult-onset

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The Clinical Study on 1 Case of Patient with Adult-onset Still's Disease who had a Diffuse Cerebral Dysfunction developed after Hypoxia. (A case of Adult-onset Still's Disease and Diffuse Cerebral Dysfunction) (저산소증으로 미만성 뇌피질 손상이 유발된 성인형 스틸병 환아(患兒) 1례(例) 보고(報告))

  • Song, In-Sun;Shin, Ji-Na;Song, Mi-Jin;Lee, Jeong-Lim
    • The Journal of Pediatrics of Korean Medicine
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    • v.17 no.2
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    • pp.15-26
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    • 2003
  • Objective : There were few reports on the treatment of Adult-onset Still's disease and Diffuse cerebral dysfunction. This oriental medical treatment shows the possibility of healing Adult-onset Still's disease and Diffuse cerebral dysfunction, therefore we announce this. Methods : The acupuncture, herbal medicine, herbal acupucture(Jahageo, Nockyong) therapies were applied for treating this patient's chief symptom(chest discomfort, palpitation, rigidity, mentality change, global aphasia, dysphagia, coughing and sputum) Results : 1. Adult-onset Still's disease is a rare systemic inflammatory disorder of unknown etiology, characterised by salmon-colored rash, intermittent spiking high fever, arthralgia and variety of systemic features. This is one type of Juvenile arthritis. 2. Diffuse cerebral dysfunction is an anoxic-ischemic encephalopathy, and most cause of this is the hypoxia caused by hypotention or respiratory distress. 3. After oriental medical treatments, his chief symptoms were improved. Conclusion : The more study about oriental medical treatment on Adult-onset Still's disease and Diffuse cerebral dysfunction is needed.

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A Case of Systemic Lupus Erythematosus Misdiagnosed as Adult-onset Still's Disease (성인 발병성 스틸병(Adult-onset Still's disease)으로 오인된 전신홍반루푸스(Systemic Lupus Erythematosus) 1예)

  • Oh, Myung-Jin;Kim, Hyun-Je;Lee, Han-Sol;Hur, Ji-An;Hong, Young-Hoon;Lee, Choong-Ki
    • Journal of Yeungnam Medical Science
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    • v.27 no.1
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    • pp.78-84
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    • 2010
  • Adult-onset Still's disease (AOSD) is an inflammatory disorder that's characterized by daily, spiking high fever, arthritis and an evanescent, salmon-pink rash. AOSD is diagnosed purely on the basis of the typical clinical features of the illness. The symptoms commonly include swelling of the lymph nodes, enlargement of the spleen and liver, and a sore throat. AOSD is difficult to differentiate from systemic lupus erythematosus (SLE) due to the similar clinical manifestations. We report here on a case of a 16-year-old female patient with autism and epilepsy and who complained of daily spiking fever for 20 days. The patient had maculopapular skin rashes on the face and whole body and lymphadenopathy. The liver function tests were elevated mildly. The initial rheumatoid factor (RF) and antinuclear antibody (ANA) tests were negative. We diagnosed her as having adult-onset Still's disease according to the criteria of Yamaguchi. We successfully treated her with oral prednisolone. But her antinuclear antibody test was changed to positive after discharge. So we finally diagnosed her as having SLE.

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Red blood cell distribution width is useful in discriminating adult onset Still's disease and sepsis within 24 hours after hospitalization

  • Park, Hee-Jin;Song, Jungsik;Park, Yong-Beom;Lee, Soo-Kon;Lee, Sang-Won
    • The Korean journal of internal medicine
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    • v.33 no.6
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    • pp.1234-1240
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    • 2018
  • Background/Aims: Red blood cell distribution width (RDW) is a value representing the heterogeneity in the size of red blood cell, and it is usually used in distinguishing types of anaemia. Recently, it was reported that it could reflect the burden of inflammation in diverse diseases and their prognosis. Hence, in this study, we investigated whether RDW may contribute to discriminating adult onset Still's disease (AOSD) from sepsis in serious febrile patients within 24 hours after hospitalization. Methods: We reviewed the medical records and enrolled 21 AOSD patients, 27 sepsis patients and 30 matched healthy controls. We collected at least two laboratory results of variables including RDW within 24 hours after hospitalization, and we calculated their mean values. Results: Sepsis patients showed the significantly increased median white blood cell count, compared to AOSD patients ($14,390.0/mm^3$ vs. $12,390.0/mm^3$, p = 0.010). The median RDW in sepsis patients was higher than that in AOSD patients (15.0% vs. 13.3%, p = 0.001), and furthermore, the median RDW in both patient-groups was significantly higher than that in healthy controls. In contrast, the median ferritin level in sepsis patients was lower than that in AOSD patients (544.0 mg/dL vs. 3,756.6 mg/dL, p = 0.001). In multivariate analysis, RDW ${\geq}14.8%$ (odds ratio, 17.549) and ferritin < 2,251.0 mg/dL (odds ratio, 32.414) independently suggested sepsis more than AOSD in patients initially presenting with fever requiring hospitalization. Conclusions: RDW might be a rapid and helpful marker for a differential diagnosis between AOSD from sepsis at an early phase.

Reatment of Oral Erosive Lichen Planus with Griseofulvin (미란성 구강편평태선(oral erosive lichen planus)에 대한 griseofulvin의 치료효과)

  • Chung, Sung-Chang;Kim, Ki-Suk;Im, Dong-Woo
    • The Journal of the Korean dental association
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    • v.25 no.7 s.218
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    • pp.673-678
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    • 1987
  • Oral lichen planus is a chronic inflammatory disease of adult onset. Current therapeutic modalities for severs oral erosive lichen planus are still generally unsatisfactory. A case of 53 year-old female patient with severe oral erosive lichen planus is reported in which the lesion showed the dramatic improvement without serious adverse effects upon using griseofulvin.

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Korean Experiences of Citrin Deficiency: Seven cases of citrin deficiency and nine major mutation screening in newborns in Korea (한국인에서의 사이트린 결핍증의 경험)

  • Kim, Ju-Hyun;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.6 no.1
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    • pp.96-107
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    • 2006
  • Citrin deficiency resulting from mutations of SLC25A13is associated with two major clinical phenotypes; neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) and adult-onset type 2 citrullinemia (CTLN2). In Korea, 7 cases of citrin deficiency have been diagnosed based on biochemical and molecular findings. Four NICCD cases were identified by newborn screening using MS/MS or presenting symptoms like cholestatic jaundice. They are all males, presenting with conjugated hyperbilirubinemia, elevated liver enzymes, hypoalbuminemia, mild hyperammonemia, elevated citrullin, methionine and threonine. All of them have been spontaneously recovered from hepatic manifestation by the age of 6-8 months. Mutation analysis has been performed using their genomic & cDNAs obtained from skin fibroblasts. They turned out to be compound heterozygotes carrying each of 851del4, IVS11+1G>A, and IVS13+1G>A. Three CTLN2 patients were identified. Two adult male patients presented with a sudden loss of consciousness, seizure, vomiting, hyperammonemia and citrullinemia in their twenties. They carried an IVS13+1G>A, 851del4, and IVS11+1G>A mutant alleles. The other CTLN2 patient was 52 year old female patient, manifesting lethargy, altered consciousness, irritability and hyperammonemia. Similar clinical symptoms had recurred at the delivery of first and second babies in her past medical history. She was managed by hemodialysis and survived with neurological sequellae. Also, we screened the presence of 9 common mutations in 500 Korean newborns using dried blood spot of filter papers. Only a allele carried 854del4 mutation. In conclusion, the entire picture of citrin deficiency in Korea including incidence, genotype, clinical features and natural courses, is still vague at the present time.

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A Case Report and Literature Review : Treatment of Nasopharyngeal Cancer(NPC) Patient with AOSD(Adult Onset Still's Disease) (성인형 스틸씨 병에서 병발된 비인두암의 치료 및 문헌 고찰)

  • Kim, Jun Young;Kim, Jin Dong;Lee, Kyung Bin;Lee, Won Jae;Na, Gun Wung;Park, Wonil;Park, Gi Cheol;Kim, Tae Gyu;Ji, Jun Ho
    • Korean Journal of Head & Neck Oncology
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    • v.30 no.1
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    • pp.15-19
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    • 2014
  • 비인두암에서 시행되는 방사선 치료 이후에 구강건조증과 피부 변화는 흔하게 볼 수 있는 합병증 중 하나이다. 비인두암의 좋은 예후를 고려할 때, 그러한 치료와 관련된 독성들은 상대적으로 오랫동안 문제를 야기하며 삶의 질 저하를 불러온다. 특히 류마티스 관절염, 루푸스와 같은 결합조직 질환을 가진 환자들에게서 방사선 치료를 시행하였을 때, 빈번하게 심각한 독성이 관찰된다. 본 증례는 성인형 스틸씨 병에서 병발된 비인두암의 치료로 항암방사선 동시치료를 실시한 결과, 비교적 경미한 구강 건조, 점막염, 불면증 등의 합병증이 관찰되었으나, 완전 관해가 획득되었다. 이에 저자들은 과거에 보고된 적 없는 성인형 스틸씨 병에 병발한 비인두암의 치료 경험을 다른 결합조직질환들에서 방사선 치료의 문헌들과 함께 보고하는 바이다.