• 제목/요약/키워드: Steroid resistant

검색결과 53건 처리시간 0.026초

Brassinosteroid의 대사공학 (Metabolic Engineering of the Brassinosteroid Biosynthetic Pathways)

  • 이미옥;송기홍;이현경;정지윤;최빛나리;최성화
    • Journal of Plant Biotechnology
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    • 제29권2호
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    • pp.139-144
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    • 2002
  • Sterols play two major roles in plants: a bulk component in biological membranes and precursors of plant steroid hormones. Physiological effects of plant steroids, brassinosteroids (BRs), include cell elongation, cell division, stress tolerance, and senescence acceleration. Arabidopsis mutants that carry genetic defects in BR biosynthesis or its signaling display characteristic phenotypes, such as short robust inflorescences, dark-green round leaves, and sterility. Currently there are more than 100 dwarf mutants representing 7 genetic loci in Arabidopsis. Mutants of 6 loci, dwf1/dim1/cbb1, cpd/dwf3, dwf4, dwf5, det2/dwf6, dwf7 are rescued by exogenous application of BRs, whereas bri1/dwf2 shares phenotypes with the above 6 loci but are resistant to BRs. These suggest that the 6 loci are defective in BR biosynthesis, and the one locus is in BR signaling. Biochemical analyses, such as intermediate feeding tests, examining the levels of endogenous BR, and molecular cloning of the genes revealed that dwf7, dwf5, and dwf1 are defective in the three consecutive steps of sterol biosynthesis, from episterol to campesterol via 5-dehydroepisterol. Similarly, det2/dwf6, dwf4, and cpd /dwf3 were shown to be blocked in D$^4$reduction, 22a-hydroxylation, and 23 a-hydroxylation, respectively. A signaling mutant bril/dwf2 carries mutations in a Leucine-rich repeat receptor kinase. Interestingly, the bri1 mutant was shown to accumulate significant amount of BRs, suggesting that signaling and biosynthesis are dynamically coupled in Arabidopsis. Thus it is likely that transgenic plants over-expressing the rate-limiting step enzyme DWF4 as well as blocking its use by BRIl could dramatically increase the biosynthetic yield of BRs. When applied industrially, BRs will boost new sector of plant biotechnology because of its potential use as a precursor of human steroid hormones, a novel lead compound for cholesterol-lowering effects, and a various application in plant protection.

한국 신증후군 환아에서 NR3C1 유전자 다형성 분석 (Polymorphisms of the NR3C1 gene in Korean children with nephrotic syndrome)

  • 조희연;최현진;이소희;이현경;강희경;하일수;최용;정해일
    • Clinical and Experimental Pediatrics
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    • 제52권11호
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    • pp.1260-1266
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    • 2009
  • 목 적 : 특발성 신증후군은 소아의 가장 흔한 일차성 사구체 질환 중의 하나이다. 신증후군은 초기 경구 스테로이드 치료에 대한 반응에 따라서 임상적으로 스테로이드 반응성 신증후군과 스테로이드 저항성 신증후군으로 분류될 수 있다. 그러나 현재까지 신증후군에서 스테로이드의 정확한 작용 기전은 알려져 있지 않다. 신증후군 환자를 대상으로 여러 가지 유전자 다형성을 분석함으로써 스테로이드 치료에 대한 반응의 차이를 설명하려는 여러 시도들이 있어왔다. 방 법 : 본 연구에서는 190명의 신증후군 환자를 대상으로 NR3C1 유전자 다형성(ER22/23EK, N363S, BclI)을 확인하여 유전형과 임상-병리 양상의 연관성에 대해서 분석하였다. 결 과 : 신증후군 환자의 평균 연령은 4.95세였고 남아가 134명이었다. 11명의 환자는 신증후군의 가족력이 있었다. 그러나 이 환자들을 대상으로 NPHS2, WT1, ACTN4, TRPC6 유전자 분석을 시행한 결과 이상 소견은 발견되지 않았다. 80명의 환자(42.1%)는 초기 스테로이드 저항성이었고 그 중 31명의 환자는 말기 신질환으로 진행하였다. 신장 조직 검사는 113명의 환자를 대상으로 시행되었고 그 중 36명의 환자(31.9%)는 미세변화 신증후군이었고 77명의 환자(68.1%)는 초점성 분절성 사구체 경화증이었다. BclI 유전형을 비교하였을 때 G allele 빈도는 환자군과 대조군에서 차이가 없었다. ER22/23EK과 N363S 유전형은 각각 ER/ER과 NN으로 환자군과 대조군에서 동일한 양상을 보였다. BclI 유전형은 신증후군의 발병 나이, 초기 스테로이드 반응 여부, 신장의 병리학적 소견, 말기 신질환으로의 진행여부와 연관성을 보이지 않았다. 결 론 : 한국 신증후군 환아를 대상으로 한 이 연구 결과는 NR3C1 유전자의 ER22/23EK, N363S 및 BclI 유전자 다형성이 신증후군의 발병, 초기 스테로이드 치료에 대한 반응, 신장의 조직학적 소견 및 신 기능의 저하에 영향을 미치지 않음을 보여준다.

소아 특발성 신증후군에서의 cyclosporine A(Cipol-$N^{(R)}$)의 치료 효과 (Cyclosporine A (Cipol-$N^{(R)}$) Therapy in Children with Idiopathic Nephrotic Syndrome)

  • 홍인희;고철우;구자훈;김지홍;김병길;조병수
    • Childhood Kidney Diseases
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    • 제3권1호
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    • pp.48-56
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    • 1999
  • 목 적 : 소아 특발성 신증후군에게 cyclosporine A(Cipol-$N^{(R)}$,종근당)를 투여하여 이의 효과와 부작용을 관찰하고 또한 기존의 cyclosporine A 제제를 사용한 치료 효과와 비교하고자 본 연구를 시행하였다. 방 법 : 대상 환아는 특발성 신증후군 환아로서 스테로이드 의존형(SD) 또는 빈발 재발형(FR) 39명과 스테로이드 저항형(SR) 3명으로 하였으며 이들에게 cyclosporine A를 1일 체중당 4-5mg을 경구 투여하고 스테로이드 제제 (prednisolone 혹은 같은 역가의 deflazacort)는 격일 요법으로 감량 투여하였다. 4개월의 조사 기간중 2, 4, 8, 12, 16주에 이들 환아에 대한 문진, 이학적 검사 및 각종 검사를 시행하였다. 결 과 : 39명의 SD/FR NS 환아중 35명 ($89.7\%$)에서 16주간 관해가 지속되었으며 혈청 단백, 알부민, 콜레스테롤 및 24시간 뇨 단백은 치료 개시후 4주째에 정상 수치를 보였고 BUN, creatinine 및 24시간 뇨를 이용한 사구체 여과율은 16주간 변동 사항이 없었다. 3명의 SR NS 환아에서는 2명에서 완전 관해가 유도되고 지속되었으며 1명에서는 반응이 없었다. Cyclosporine A에 대한 부작용은 다모증이 8명, 고뇨산혈증이 5명에서 관찰되었으나 이로 인해 투약을 중지할 필요가 없었으며 기타 CBC, 간기능 검사, 혈압 등은 16주간 정상 소견을 보였다. 결 론 : 스테로이드 의존형, 빈발 재발형 및 스테로이드 저항형의 특발성 신증후군 환아에게 cyclosporine A는 유용하게 사용할 수 있을 약제로 생각되며 본 연구의 다음 과제는 cyclosporine A의 장기 투여(1-2년)로서 얻을 수 있는 효과 및 약제의 안전성 (특히 신독성)여부를 확인하여야 할 것으로 생각한다.

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Complications of nephrotic syndrome

  • Park, Se-Jin;Shin, Jae-Il
    • Clinical and Experimental Pediatrics
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    • 제54권8호
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    • pp.322-328
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    • 2011
  • Nephrotic syndrome (NS) is one of the most common glomerular diseases that affect children. Renal histology reveals the presence of minimal change nephrotic syndrome (MCNS) in more than 80% of these patients. Most patients with MCNS have favorable outcomes without complications. However, a few of these children have lesions of focal segmental glomerulosclerosis, suffer from severe and prolonged proteinuria, and are at high risk for complications. Complications of NS are divided into two categories: disease-associated and drug-related complications. Disease-associated complications include infections (e.g., peritonitis, sepsis, cellulitis, and chicken pox), thromboembolism (e.g., venous thromboembolism and pulmonary embolism), hypovolemic crisis (e.g., abdominal pain, tachycardia, and hypotension), cardiovascular problems (e.g., hyperlipidemia), acute renal failure, anemia, and others (e.g., hypothyroidism, hypocalcemia, bone disease, and intussusception). The main pathomechanism of disease-associated complications originates from the large loss of plasma proteins in the urine of nephrotic children. The majority of children with MCNS who respond to treatment with corticosteroids or cytotoxic agents have smaller and milder complications than those with steroid-resistant NS. Corticosteroids, alkylating agents, cyclosporin A, and mycophenolate mofetil have often been used to treat NS, and these drugs have treatment-related complications. Early detection and appropriate treatment of these complications will improve outcomes for patients with NS.

Risk Assessment of Growth Hormones and Antimicrobial Residues in Meat

  • Jeong, Sang-Hee;Kang, Dae-Jin;Lim, Myung-Woon;Kang, Chang-Soo;Sung, Ha-Jung
    • Toxicological Research
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    • 제26권4호
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    • pp.301-313
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    • 2010
  • Growth promoters including hormonal substances and antibiotics are used legally and illegally in food producing animals for the growth promotion of livestock animals. Hormonal substances still under debate in terms of their human health impacts are estradiol-$17\beta$, progesterone, testosterone, zeranol, trenbolone, and melengestrol acetate (MGA). Many of the risk assessment results of natural steroid hormones have presented negligible impacts when they are used under good veterinary practices. For synthetic hormonelike substances, ADIs and MRLs have been established for food safety along with the approval of animal treatment. Small amounts of antibiotics added to feedstuff present growth promotion effects via the prevention of infectious diseases at doses lower than therapeutic dose. The induction of antimicrobial resistant bacteria and the disruption of normal human intestinal flora are major concerns in terms of human health impact. Regulatory guidance such as ADIs and MRLs fully reflect the impact on human gastrointestinal microflora. However, before deciding on any risk management options, risk assessments of antimicrobial resistance require large-scale evidence regarding the relationship between antimicrobial use in food-producing animals and the occurrence of antimicrobial resistance in human pathogens. In this article, the risk profiles of hormonal and antibacterial growth promoters are provided based on recent toxicity and human exposure information, and recommendations for risk management to prevent human health impacts by the use of growth promoters are also presented.

Bilateral iliac and popliteal arterial thrombosis in a child with focal segmental glomerulosclerosis

  • Han, Kyoung Hee;Park, Ji Youn;Min, Seung-Kee;Ha, Il-Soo;Cheong, Hae Il;Kang, Hee Gyung
    • Clinical and Experimental Pediatrics
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    • 제59권5호
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    • pp.242-245
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    • 2016
  • Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS.

Treatment of the Mycobacterium chelonae Infection after Fat Injection

  • Kim, Seok-Kwun;Choi, Ji-An;Kim, Myung-Hoon;Kim, Min-Su;Lee, Keun-Cheol
    • Archives of Plastic Surgery
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    • 제42권1호
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    • pp.68-72
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    • 2015
  • For recent years, use of autologous fat injection has increased significantly in facial contouring surgery. Along with such increase in use, complications like atypical mycoplasma infection have been also on the increasing trend. The authors report two cases of Mycobacterium chelonae infection that occurred after autologous fat injection. Patients were treated as infection that resistant to common antibiotics and results were negative to routine culture and Gram staining. Acid-fast bacillus stain, polymerase chain reaction (PCR) test and mycobacterial cultures were conducted for diagnosis under suspicion of atypical mycoplasma infection. Then, combination antibiotics therapy, surgical treatment, and steroid injection were performed for treatment. Both patients were diagnosed with Mycobacterium chelonae in PCR test. They were positive to mycobacterial cultures. Combination antibiotics therapy was repeated to improvement of symptom. However, they could not be free from side effects such as deformation in facial contour, scar and pigmentation even after full recovery. When chronic wound infections after autologous fat injection, we must suspect atypical or mycobacterial infection and conduct examinations for a early diagnosis and proper antibiotic therapy that is effective to the nontuberculous mycobacteria.

Tacrolimus 연고제로 호전을 보인 항문 주위 크론병 3례 (Topical Tacrolimus Resulting in a Marked Resolution of Perianal Crohn's Disease)

  • 박문호;최원정;황진복
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제8권1호
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    • pp.70-75
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    • 2005
  • 저자들은 기존의 크론병 치료에 호전이 없었던 항문 주위 병변에 0.3 mg/g의 저농도 tacrolimus 연고제를 1일 2회 환부에 도포하는 방법으로 약물에 의한 부작용 발생 없이 병변의 현저한 호전을 보인 3례를 경험하였기에 보고하는 바이다.

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Treatment of Henoch-Sch$\ddot{o}$nlein Purpura with Intravenous Immunoglobulin

  • ;;;;박용훈
    • Journal of Yeungnam Medical Science
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    • 제18권2호
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    • pp.246-252
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    • 2001
  • 고용량 스테로이드 충격 요법에 반응하지 않는 심한 복통과 신생검에서 50% 이상 반월체 형성이 되는 알레르기성 자반증 산염 환자의 치료를 위하여 고용량 정맥용 면역 글로불린을 투여하여 복부 증상이 조기 회복되었으며 이후 혈뇨와 단백뇨도 호전되었다. 그러므로 복부 증상과 선장 침범이 스테로이드 치료야 반응하지 않는 알레르기성 자반증 환자의 경우에 정맥용 면역 글로블린 치료가 고려되어야 할 것으로 사료된다.

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방사성 활막절제술 (Radiosynovectomy)

  • 김덕윤
    • Nuclear Medicine and Molecular Imaging
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    • 제40권2호
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    • pp.127-131
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    • 2006
  • Radiosynovectomy has been used as an effective treatment in patients with resistant synovitis after failure of long-term medication and intraarticular steroid injection. Although $^{90}Y$ silicate/citrate, $^{186}Re$ sulfide, and $^{169}Er$ citrate were approved in Europe for the appropriate radiopharmaceuticals for radiosynovectomy other radionuclides such as $^{32}P$-chromic phosphate, $^{165}Dy$-ferric hydroxide macroaggregate, $^{188}Rh$-microspheres, $^{153}Sm-particulate, and $^{166}Ho$-ferric hydroxide macroaggregate have been used in many countries. Reported success rates range from 40% to 90% for the different joints and underlying disease. In Korea, $^{188}Re$-tin-colloid and $^{166}Ho$-chitosan complex are now using as the major radionuclides in radiosynovectomy with good clinical results. A study on radiation synovectomy using $^{188}Re$-tin-colloid for patients with Korean rheumatoid arthritis shows the treatment resulted in the improvement of arthritis and well tolerated in our study, the radiosynovectomy with $^{166}Ho$-chitosan complex in 53 hemophilic patients markedly decreased intra-articular bleeding frequency and need for coagulation factor. This review inculdes general priciples in the application of radiosynovectomy and the clinical experience in Korea.