• 제목/요약/키워드: azathioprine

검색결과 57건 처리시간 0.019초

점막 유천포창의 진단 및 치료 (The Diagnosis and Treatment of Mucous Membrane Pemphigoid)

  • 민숙진;박준상;고명연
    • Journal of Oral Medicine and Pain
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    • 제26권2호
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    • pp.121-126
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    • 2001
  • Mucous membrane pemphigoid is uncommom disease in oral cavity and synonymous with cicatricial pemphigoid. This disease is caused by autoimmune reaction that autoantibody reacts antigen located in basement membrane and epithelium is separated from underlying connective tissue. It affects female over sixth decade, commonly. Oral mucosa, especially gingiva is common site but conjunctival, nasal, pharyngeal, laryngeal, esophageal, varginal mucosa and skin are involved. Intraoral findings show Nikolsky sign, irregular erythema, erosion, vesicle, and ulceration at mucous membrane. To differentiate from diseases of positive Nikolsky sign, should perform histologic, immunologic test. Histologic features show subbasilar cleft and direct immunologic features show IgG, C3 deposits at basement membrane in linear pattern. Mucous membrane pemphigoid is incurable disease because symptoms are repetitively improved or worsed for several years. Patiens are commonly managed with topical and systemic steroid. To avoid side effects of prolonged steroid therapy and to maintain immunosupressive effects, combination therapy of azathioprine with steroid is effective. This case reports that mucous membrane pemphigoid is diagnosed based on clinical and histologic features, is treated with topical, systemic steroid and azathioprine therapy.

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신증후군을 동반한 HSP 신염에서 Azathioprine의 치료 효과 (Azathioprine Therapy in $Henoch-Sch\"{o}nlein$ Purpura Nephritis Accompanied by Nephrotic syndrome)

  • 손진태;김지홍;김병길;정현주
    • Childhood Kidney Diseases
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    • 제2권1호
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    • pp.41-49
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    • 1998
  • 목적 : 신증후군을 동반한 Henoch-Scholein purpura(HSP) 신염은 예후가 매우 불량한 것으로 알려져 있으며, 스테로이드와 여러 가지 면역억제제가 치료제로 사용되어 왔으나 아직 효과적인 치료 방법에 대해서는 알려져 있지 않다. 이에 저자들은 신증후군을 동반한 HSP신염에서의 azathioprine(AZA)의 치료 효과를 살펴보고자 본 연구를 시작 하였다. 방법 : 신증을 동반한 HSP 신염으로 진단 받은 15명을 대상으로 prednisolone과 AZA를 8개월간 투여하여 치료 효과를 관찰하였다. AZA는 초기용량으로 2 mg/kg/day을 매일 2회 분복하였으며, 같은 기간동안 prednisolone을 0.5-1.0 mg/kg씩 격일로 투여하였다. 치료 전후에 신조직검사를 시행하여 조직 변화를 관찰하였고 AZA의 부작용의 여부를 관찰하였다. 결과 : AZA 치료전 임상상태는 12례가 Meadow(1973)분류등급에 따라 C였으며, 3례는 B였다. 이중 12례(80.0%)에서 치료후 임상상태등급이 호전되었고, 2례(13.2%)는 변화를 보이지 않았으며, 1례(1.7%)는 악화된 소견을 보였다. 치료시작후 단백뇨의 완전관해는 8례(53.3%)에서 있었으며 이중 4례에서는 혈뇨가 지속되었으며, 부분관해는 4례(26.7%)에서 보였고, 단백뇨 및 혈뇨소실을 보이지 않은 경우는 3례(20.0%)였다. 치료시작후 평균 3개월(1개월-7개월)에서 단백뇨소실을 보였고, 혈뇨 소실은 10례(66.7%)에서 있었으며 치료시작후 평균 4.3개월 (2.5개월-8.7개월)에 혈뇨소실을 보였다. 추적 신장조직검사상 4례에서 조직병리학적 및 면역조직학적인 호전을 보였다. AZA 치료중 합병증으로 나타날 수 있는 골수억제, 백혈구감소증, 간독성, 위장관 장애, 피부반점, 감염의 소견은 전례에서 나타나지 않았다. 결론 : 신증을 동반한 HSP 신염의 치료에서 AZA의 치료효과는 임상적 뿐만 아니라 조직병리학적으로도 호전시키는 효과가 있는 것을 보여주었다. 그러나 조직학적 호전은 일부 예에서만 관찰되었고 또한 치료후 신염이 재발되는 경우가 있으므로 더 많은 환자를 대상으로 한 장기간의 추적관찰 및 다른 약제와의 비교연구가 필요할 것으로 사료된다.

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Prevention of thiopurine-induced early leukopenia in a Korean pediatric patient with Crohn's disease who turned out to possess homozygous mutations in NUDT15 R139C

  • Bae, Jaewoan;Choe, Byung-Ho;Kang, Ben
    • Journal of Yeungnam Medical Science
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    • 제37권4호
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    • pp.332-336
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    • 2020
  • Homozygous mutations in NUDT15 R139C are known as the major factor associated with thiopurine-induced early leukopenia, particularly in Asian patients. Therefore, NUDT15 genotyping is currently recommended before thiopurine treatment to identify patients who are NUDT15 poor metabolizers and consider the use of an alternative immunomodulatory therapy. We report a case of a 12-year-old Korean girl with Crohn's disease (CD), in whom thiopurine-induced leukopenia was prevented by initiation of azathioprine (AZA) therapy at a low dose (0.5 mg/kg/day) and early detection of significant hair loss and white blood cell (WBC) count decrease at 17 days from the start of AZA treatment. The WBC count dropped from 8,970/μL to 3,370/μL in 2 weeks, and AZA treatment was stopped because of concerns of potential leukopenia in the near future. Her WBC count recovered to 5,120/μL after 3 weeks. Gene analysis later revealed that she had a homozygous mutation in NUDT15 R139C, resulting in a poor metabolizing activity of NUDT15. In situations when NUDT15 genotyping is unavailable, initiation of AZA therapy at 0.5 mg/kg/day with close observation of hair loss and WBC counts within 2 weeks may be an alternative way to prevent thiopurine-induced early leukopenia in Asian children with CD.

신이식 후 면역반응의 이해 2부 이식면역검사와 면역억제제 (Allograft Immune Reaction of Kidney Transp lantation Part 2. Immunosuppression and Methods to Assess Alloimmunity)

  • 강희경
    • Childhood Kidney Diseases
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    • 제12권2호
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    • pp.133-142
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    • 2008
  • 이식을 위해서는 수여자와 공여자의 혈액형과 HLA type을 알아야 한다. 통상 ABO 혈액형이 적합한 경우 이식할 수 있으며 HLA 부적합은 근래 큰 문제가 되지 않으나 HLA 부적합이 없는 경우 이식장기의 장기생존률이 높다. PRA(panel reactive antibody)는 수여자가 HLA에 감작되었는지 검사하는 방법이며 이식 전에는 반드시 교차반응 검사를 하여 음성인 경우에만 이식을 진행한다. 이식 전후에 donor specific antibody(DSA)를 검사하여 이식장기에 대한 수여자의 면역반응을 예측 할 수 있다. 근래에는 스테로이드, calcineurin inhibitor(cyclosporine, tacrolimus), azathioprine 또는 mycophenolate mofetil (MMF)의 삼제요법을 주로 사용하며 항림프구 항체 (Thymoglobulin 또는 항IL-2 receptor 항체 basiliximab/daclizumab)을 이용하여 이식 초기에 면역억제상태를 induction하는 경우도 많다.

개에서 발생한 전신성 낙엽상 천포창에 사람 면역글로불린의 임상적 적용 (Clinical Trial of Human Intravenous Immunoglobulin in a Dog with Generalized Pemphigus Foliaceus)

  • 박성준
    • 한국임상수의학회지
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    • 제30권1호
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    • pp.61-65
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    • 2013
  • An American Cocker Spaniel (3-year-old, intact female, 6.0 kg) was referred to the Veterinary Medical Teaching Hospital of Chungnam National University for evaluation of pustules and crusts in the periocular region, dorsal and ventral region of the trunk, and digits. Complete blood count (CBC) revealed leukocytosis with mature neutrophilia, and a serum biochemistry profile revealed hypoalbuminemia. Tape strip tests identified numerous neutrophils and acatholytic cells. Histopathology identified intraepithelial pustules with neutrophils and acantholytic keratinocytes. Definitive diagnosis of pemphigus foliaceus (PF) was made by direct immunofluorescence (DIF) test with goat anti-canine IgG antibody. The human intravenous immunoglobulin (IVIG) was administered at a rate of 15 ml/h over 6 hours for 4 days. After that, the dog was maintained on prednisolone (2.2 mg/kg, PO, SID) and azathioprine (2.0 m/kg, PO, SID). An infusion of IVIG (0.5 g/kg) was repeated 3 days after 4 weeks. After 10 weeks, the dog showed the remarkable regression of lesions.

Fecal Microbiota Transplantation to Patients with Refractory Very Early Onset Ulcerative Colitis

  • Yodoshi, Toshifumi;Hurt, Thomas L.
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제21권4호
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    • pp.355-360
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    • 2018
  • Recently, fecal microbiota transplantation (FMT) has been attracting attention as a possible medical treatment of ulcerative colitis (UC). A randomized controlled trial of FMT for children with UC is currently underway. Therapeutic effects of FMT for adults with UC remain controversial. We report two cases of early-onset UC in children. A patient was diagnosed with UC at age 1-year 9-month and underwent FMT at age 2-year 3-month. He attained clinical remission for three weeks after FMT, but then relapsed at four weeks, ultimately undergoing a total colectomy. Another child was diagnosed with UC at 2-year 10-month and she underwent FMT at age 5 years. She has remained in clinical remission following FMT for 24 months and her UC has been maintained without complications with tacrolimus and azathioprine. We report that FMT for early-onset UC appears to be safe and potentially effective.

말초신경병증에 대한 정맥내 면역글로불린 요법 (Intravenous Immunoglobulin Therapy in Peripheral Neuropathy)

  • 김남희;박경석
    • Annals of Clinical Neurophysiology
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    • 제8권1호
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    • pp.6-15
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    • 2006
  • Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has been used in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

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