• 제목/요약/키워드: Hemolytic disease

검색결과 113건 처리시간 0.03초

Perioperative red blood cell transfusion in orofacial surgery

  • Park, So-Young;Seo, Kwang-Suk;Karm, Myong-Hwan
    • Journal of Dental Anesthesia and Pain Medicine
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    • 제17권3호
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    • pp.163-181
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    • 2017
  • In the field of orofacial surgery, a red blood cell transfusion (RBCT) is occasionally required during double jaw and oral cancer surgery. However, the question remains whether the effect of RBCT during the perioperative period is beneficial or harmful. The answer to this question remains challenging. In the field of orofacial surgery, transfusion is performed for the purpose of oxygen transfer to hypoxic tissues and plasma volume expansion when there is bleeding. However, there are various risks, such as infectious complications (viral and bacterial), transfusion-related acute lung injury, ABO and non-ABO associated hemolytic transfusion reactions, febrile non-hemolytic transfusion reactions, transfusion associated graft-versus-host disease, transfusion associated circulatory overload, and hypersensitivity transfusion reaction including anaphylaxis and transfusion-related immune-modulation. Many studies and guidelines have suggested RBCT is considered when hemoglobin levels recorded are 7 g/dL for general patients and 8-9 g/dL for patients with cardiovascular disease or hemodynamically unstable patients. However, RBCT is occasionally an essential treatment during surgeries and it is often required in emergency cases. We need to comprehensively consider postoperative bleeding, different clinical situations, the level of intra- and postoperative patient monitoring, and various problems that may arise from a transfusion, in the perspective of patient safety. Since orofacial surgery has an especially high risk of bleeding due to the complex structures involved and the extensive vascular distribution, measures to prevent bleeding should be taken and the conditions for a transfusion should be optimized and appropriate in order to promote patient safety.

포도당-6-인산탈수소효소 결핍증 환아의 치과적 관리 (Dental Management in a Child Patient with Glucose-6-phosphate Dehydrogenase Deficiency : A Case Report)

  • 전지윤;양선미;최남기;김선미
    • 대한소아치과학회지
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    • 제48권3호
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    • pp.359-366
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    • 2021
  • 포도당-6-인산탈수소효소 결핍증은 X 염색체 열성으로 유전되는 세계에서 가장 흔한 효소 결핍증이다. 이 질환은 우리나라에서는 드물게 나타나지만, 다문화 가정의 증가로 유병률이 높아질 가능성이 있다. 이 결핍증의 주된 문제는 소아치과에서 일반적으로 사용되거나 처방되는 일부 약물에 의해 용혈성 빈혈이 유발될 수 있다는 점이다. 소아치과 의사는 환자의 병력에 대한 정확한 지식을 갖고 용혈을 유발할 수 있는 산화 스트레스를 피하기 위해 소아 혈액 전문의와 상담해야 한다. 가장 효과적인 치료는 빈혈을 유발할 수 있는 인자에 노출되지 않도록 예방하는 것이다. 이 질환으로 진단받은 환자들에게 위험인자에 대한 적절한 교육은 필수적이다. 이 증례보고는 G6PD 결핍증 환아에서 주의해야 할 약물과 치과적 관리 방법에 대해 논의하고자 하였다.

Coexistence of Gilbert Syndrome and Hereditary Spherocytosis in a Child Presenting with Extreme Jaundice

  • Lee, Jae Hee;Moon, Kyung Rye
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제17권4호
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    • pp.266-269
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    • 2014
  • Gilbert syndrome is the most common inherited disorder of bilirubin glucuronidation. It is characterized by intermittent episodes of jaundice in the absence of hepatocellular disease or hemolysis. Hereditary spherocytosis is the most common inherited hemolytic anemia and is characterized by spherical, osmotically fragile erythrocytes that are selectively trapped by the spleen. The patients have variable degrees of anemia, jaundice, and splenomegaly. Hereditary spherocytosis usually leads to mild-to-moderate elevation of serum bilirubin levels. Severe hyperbilirubinemia compared with the degree of hemolysis should be lead to suspicion of additional clinical conditions such as Gilbert syndrome or thalassemia. We present the case of a 12-year-old boy with extreme jaundice and nausea. The diagnosis of hereditary spherocytosis was confirmed by osmotic fragility test results and that of Gilbert syndrome by genetic analysis findings.

Complement regulation: physiology and disease relevance

  • Cho, Heeyeon
    • Clinical and Experimental Pediatrics
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    • 제58권7호
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    • pp.239-244
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    • 2015
  • The complement system is part of the innate immune response and as such defends against invading pathogens, removes immune complexes and damaged self-cells, aids organ regeneration, confers neuroprotection, and engages with the adaptive immune response via T and B cells. Complement activation can either benefit or harm the host organism; thus, the complement system must maintain a balance between activation on foreign or modified self surfaces and inhibition on intact host cells. Complement regulators are essential for maintaining this balance and are classified as soluble regulators, such as factor H, and membrane-bound regulators. Defective complement regulators can damage the host cell and result in the accumulation of immunological debris. Moreover, defective regulators are associated with several autoimmune diseases such as atypical hemolytic uremic syndrome, dense deposit disease, age-related macular degeneration, and systemic lupus erythematosus. Therefore, understanding the molecular mechanisms by which the complement system is regulated is important for the development of novel therapies for complement-associated diseases.

남매에서 비슷한 시기에 발생한 Henoch-$Sch{\ddot{o}}nlein$ 자반증 2례 (Two Cases of Henoch-$Sch{\ddot{o}}nlein$ Purpura in one Family at Similar Period)

  • 김소영;임인석;유병훈
    • Childhood Kidney Diseases
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    • 제4권2호
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    • pp.161-165
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    • 2000
  • 저자들은 남매에서 3주 정도의 차이를 둔 비슷한 시기에 피부의 자반성 구진, 관절의 부종 및 관절통을 주소로 내원하여 HS 자반증으로 진단받은 두명의 환아를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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A clinical case of bovine anemia due to Theileria orientalis group in a non-grazed dairy cow in the upper part of South Korea

  • Espiritu, Hector M.;Lee, Hee-Woon;Lee, Sang-Suk;Cho, Yong-Il
    • 대한수의학회지
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    • 제61권4호
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    • pp.33.1-33.5
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    • 2021
  • This study reports the detection and genotyping of the first clinical case of bovine anemia due to Theileria orientalis group (BATOG) in non-grazed dairy cow in upper South Korea. Blood and serum tests revealed anemia and hyperbilirubinemia from animal showing clinical symptoms, and later confirmed as piroplasmosis-positive. Follow-up surveillance on the herd revealed 2 asymptomatic cows with anemia. The three animals were confirmed theileriosis-positive and genotyping revealed the clinical and one of the asymptomatic cases have Chitose, while the other has Ikeda genotype. Clinical BATOG cases were rarely reported worldwide, and asymptomatic animals left untreated could serve as parasite reservoir.

First report of tetracycline-resistant Aeromonas veronii infection in Amur catfish (Silurus asotus) cultured in Korea

  • Kim, Hyo Yeon;Jun, Jin Woo;Lim, Se Ra;Park, Seon Young;Han, Jee Eun;Park, Se Chang;Kim, Ji Hyung
    • 대한수의학회지
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    • 제59권4호
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    • pp.207-211
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    • 2019
  • Mass mortality in commercially cultured Amur catfish (Silurus asotus), showing symptoms of dermal ulcerations, occurred on a private farm in Mar 2019 in Korea. β-hemolytic bacteria were isolated from the ulcers and kidneys of the fish and identified as Aeromonas veronii. The isolate was resistant to tetracycline and possessed cytotoxic heat-labile enterotoxin (aerolysin/hemolysin). We investigated the genetic determinants associated with tetracycline resistance, and the isolate has been confirmed to simultaneously possess tetA and tetE genes. This is the first report on the occurrence of tetracycline-resistant A. veronii infection related to mass mortality in commercially cultured Amur catfish in Korea.

Characterization of Phage-Resistant Strains Derived from Pseudomonas tolaasii 6264, which Causes Brown Blotch Disease

  • Yun, Yeong-Bae;Han, Ji-Hye;Kim, Young-Kee
    • Journal of Microbiology and Biotechnology
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    • 제28권12호
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    • pp.2064-2070
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    • 2018
  • Pseudomonas tolaasii 6264 is a representative strain that causes bacterial blotch disease on the cultivated oyster mushroom, Pleurotus ostreatus. Bacteriophages are able to sterilize the pathogenic P. tolaasii strains, and therefore, they can be applied in creating disease-free mushroom cultivation farms, through a method known as "phage therapy". For successful phage therapy, the characterization of phage-resistant strains is necessary, since they are frequently induced from the original pathogenic bacteria in the presence of phages. When 10 different phages were incubated with P. tolaasii 6264, their corresponding phage-resistant strains were obtained. In this study, changes in pathogenic, genetic, and biochemical characteristics as well as the acquired phage resistance of these strains were investigated. In the phylogenetic analyses, all phage-resistant strains were identical to the original parent strain based on the sequence comparison of 16S rRNA genes. When various phage-resistant strains were examined by three different methods, pitting test, white line test, and hemolytic activity, they were divided into three groups: strains showing all positive results in three tests, two positive in the first two tests, and all negative. Nevertheless, all phage-resistant strains showed that their pathogenic activities were reduced or completely lost.

톨라신의 용혈활성에 대한 Ni2+의 저해효과 (Inhibitory Effect of Ni2+ on the Tolaasin-induced Hemolysis)

  • 최태근;왕희성;김영기
    • Journal of Applied Biological Chemistry
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    • 제52권1호
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    • pp.28-32
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    • 2009
  • 세균독소인 톨라신은 재배버섯에서 버섯균사 및 자실체의 구조를 파괴하여 갈반병을 일으킨다. 톨라신의 세포독성은 톨라신이 적혈구의 세포막에 pore를 형성하여 세포구조를 파괴하기 때문에 용혈활성을 측정하여 평가한다. 저자들은 $Zn^{2+}$$Cd^{2+}$에 의한 톨라신의 용혈활성 저해효과를 측정하는 중에 $Ni^{2+}$이 또 다른 저해제로 톨라신의 독성을 억제하는 것을 발견하였다. $Ni^{2+}$에 의한 톨라신 용혈활성의 저해는 농도의존적이었으며, Ki 값은 대략 10mM이었고, 이것은 $Ni^{2+}$$Cd^{2+}$에 비하여 저해효과가 높음을 의미한다. 용혈활성은 50mM 이상의 $Ni^{2+}$농도에서 완전히 제거되었으며, $Ni^{2+}$의 효과는 EDTA 첨가에 의해 가역적임을 확인하였다. 톨라신에 의한 용혈활성이 20mM $Ni^{2+}$에 의해서 완전히 억제된 상태에서 EDTA를 가하면 즉각 용혈활성이 나타났다. $Ni^{2+}$에 의한 톨라신 독성의 저해기작은 알 수 없지만, $Ni^{2+}$은 톨라신의 독성과정인 막결합, 분자중합체 형성, pore 형성, pore를 통한 막대한 양의 이온이동 등에 작용할 수 있을 것이다. 된 연구의 결과는 $Ni^{2+}$이 독성과정의 마지막 단계인 pore를 통한 이온이동 과정을 저해함을 보여준다.

구개편도염과 편도주위농양의 임상적 고찰 (A Clinical Study of Tonsillitis and Peritonsillar Abscess)

  • 최창만;이병화;오대식;양철민;채규학
    • 대한기관식도과학회지
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    • 제3권2호
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    • pp.293-301
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    • 1997
  • The tonsillitis has long been one of the most common disease in the otolaryngologic field. Peritonsillar abscess occurs when bacterial infection of the tonsil spreads to the potential peritonsillar space deep behind the tonsil, and it usually occurs in patients with recurrent tonsillitis or in those with tonsillitis who have been inadequately treated. We studied retrospectively 71 patients who had been diagnosed as acute tonsillitis and 82 patients who had been diagnosed as peritonsillar abscess and had admitted in our department of the Ulsan Dong Kang Hospital from January, 1995 to September, 1997. Especially in the bacteriologic studies, we compared acute tonsillitis and peritonsillar abscess with chronic tonsillitis. The following results were obtained: 1) The sex distributions of acute tonsillitis were 47 males(66%) and 24 females(34%) cases, but 57 males(70%) and 25 females(30%) in cases of peritonsillar abscess. There were predominant in male and frequently affected in second and third decades in 53 cases(76%) of acute tonsillitis and 56 cases(68%) of peritonsillar abscess. 2) It was same found in each season. 3) The duration from onset of symptom to visit in our department was 3.92 days in cases of acute tonsillitis and 5.95 days in cases of peritonsillar abscess in average 4) The major symptoms were sore throat, swallowing difficult. And others were fever, fatigability, dysarthria, trismus, headache, otalgia. 5) Among the 71 cases of acute tonsillitis and 82 cases of peritonsillar abscess, most temperature of patients at visit were 36.6-37.5 $^{\circ}C$ in each 36 cases(51%), 57 cases(70%). 6) In each disease, 35 cases(47%), 45 cases(75%) consisted of single infection and 39 cases(53%), 15 cases(25%) consisted of mixed infection. In acute tonsillitis, 111 strains were isolated from 74 cases, the most common strain was 69 strains(62.2%) of $\alpha$-hemolytic streptococci. In the peritonsillar abscess, 77 strains were isolated from 60 cases, the most common strain was 49 strains(63.6%) of $\alpha$-hemolytic streptococci. In chronic tonsillitis, 563 strains were isolated from 382 cases, the most common strain was 334 strains(50.3%) of $\alpha$-hemolytic streptococci. 7) In acute tonsillitis and peritonsillar abscess, the most common leukocyte levels were reported with 10, 000-15, 000/$\mu$L in 23 cases(32%). The CRP levels were reported with abnormal findings in 61 cases(97%), 63 cases(95%) above 0.3 in each cases.

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