Disease also known as Lima beans yellow beans is due to eating beans and broad beans or smell the smell of pollen and bean induced acute anemia, jaundice and hemoglobinuria as the main clinical manifestations of the disease. More than 90% occurring in men, and most found in children under age 5. In the southwest, south, east and north China have been found all over, in the Guangdong, Sichuan, Guangxi, Hunan, Jiangxi to the most.
Erythrocyte glucose -6-- phosphate dehydrogenase (G6PD) has a genetic defect in the consumption of fresh green beans or bean pollen exposure occurs after both acute hemolytic anemia - bean disease. Pathogenic mechanisms are not yet very clear, but the genetic defects known to have sensitive red blood cells, a factor in the case of beans, caused by acute intravascular hemolysis.
[Clinical manifestations]
Early aversion to cold, fever, dizziness, fatigue, weakness, anorexia, abdominal pain, followed by jaundice, anemia, hemoglobin in urine, urine color was soy sauce, then fever, fatigue fatigue increased, sustainable 3 days or so. And hemolytic anemia occur at the same time, vomiting, diarrhea and abdominal pain increased, hepatomegaly, abnormal liver function, approximately 50% of patients with splenomegaly. Seen severe cases coma, convulsions and acute renal failure, if the aid is often less than 1 to 2 days at death.
[Diagnostic Methods]
1. Are eating beans green beans or inhalation of pollen history.
2. Clinical features:
① incubation period of several hours to 48 hours.
② performance poisoning: early aversion to cold, fever, dizziness, fatigue, weakness, anorexia, abdominal pain, followed by jaundice, anemia, hemoglobin in urine, urine color was soy sauce, then fever, fatigue fatigue increased, sustainable 3 around June.
③ The check: methemoglobin reduction test (MHb) to restore normal rates of> 75% (colorimetric method), broad bean MHb reduction rate in patients with 31% to 74% (genetic heterozygotes), with reduction rate "30% (pure zygosity); blood corpuscles with denatured globin (He Enzi bodies) can be more than 5% (normal is 0 to 0.28%).
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Friday, November 19, 2010
Von Willebrand
VWD von Willebrand referred to is second only to the most common hemophilia hereditary hemorrhagic disease. Childhood that is characterized by hemorrhagic tendency, prolonged bleeding time and factor Ⅷ content reduced. VWD is the lack of high molecular weight factor Ⅷ part, that Ⅷ R due. 1 Ⅷ R is autosomal, and Ⅷ: C is the X chromosome inherited.
Typical VWD only factor Ⅷ related antigen (Ⅷ R: Ag) that Ⅷ R antigen part of the activity decreased, and the factor Ⅷ Rui Situo enzyme cofactor (Ⅷ R: RCOF) reduced or lack of, and Ⅷ: C also reduced, but the extent as severe hemophilia. Ⅷ R: Ag and Ⅷ R: RCOF is between platelets and vascular endothelial and platelet adhesion between the important factor, is induced platelet aggregation from Rui Situo enzyme cofactor, since the lack of von Willebrand The factors that platelet adhesion and aggregation function has obstacles, prolonged bleeding time.
Von Willebrand mode of inheritance is autosomal dominant inheritance, individual subtypes of recessive inheritance. Incidence of both men and women, parents can pass, and some parents may be no bleeding patients. Von Willebrand more bleeding tendency in childhood, adulthood, only a small number of patients to clinical symptoms. Von Willebrand typical symptoms of bleeding in hemophilia similar, but lesser extent, but there are more serious. Condition can be mitigated with age.
Von Willebrand medicine is a "hyperlipidemia" Consumption "category. Its pathogenesis and hemophilia similar.
[Clinical manifestations of von Willebrand]
1. Have a family history: in line with autosomal dominant inheritance, which both men and women to the disease, both parents can be inherited. 2. Hemorrhagic tendency: a mucous membrane, subcutaneous bleeding, or menorrhagia, severe bleeding after surgery with or without history of a small number of joint, muscle bleeding, but generally no joint deformity.
[Von Willebrand diagnosis is based on]
(A) with or without family history, have a family history consistent with autosomal dominant or recessive inheritance.
(B) clinical there mucosa, skin, visceral bleeding, or menorrhagia history of trauma, surgery with or without abnormal bleeding history, a small number of patients can the relevant section of cavity, muscle or other parts of the bleeding.
(C) of the laboratory examination can help diagnose
1. Platelet morphology and count properly.
2. Prolonged bleeding or aspirin tolerance test was positive.
3. Platelet adhesion test extended or normal.
4. Activated partial thromboplastin time prolonged or normal.
5.vWF factor antigen (vWF: Ag) to reduce or normal.
6. Ⅷ coagulation factor activity (Ⅷ: C) reduced or normal.
7. Must exclude platelet dysfunction disease.
Typical VWD only factor Ⅷ related antigen (Ⅷ R: Ag) that Ⅷ R antigen part of the activity decreased, and the factor Ⅷ Rui Situo enzyme cofactor (Ⅷ R: RCOF) reduced or lack of, and Ⅷ: C also reduced, but the extent as severe hemophilia. Ⅷ R: Ag and Ⅷ R: RCOF is between platelets and vascular endothelial and platelet adhesion between the important factor, is induced platelet aggregation from Rui Situo enzyme cofactor, since the lack of von Willebrand The factors that platelet adhesion and aggregation function has obstacles, prolonged bleeding time.
Von Willebrand mode of inheritance is autosomal dominant inheritance, individual subtypes of recessive inheritance. Incidence of both men and women, parents can pass, and some parents may be no bleeding patients. Von Willebrand more bleeding tendency in childhood, adulthood, only a small number of patients to clinical symptoms. Von Willebrand typical symptoms of bleeding in hemophilia similar, but lesser extent, but there are more serious. Condition can be mitigated with age.
Von Willebrand medicine is a "hyperlipidemia" Consumption "category. Its pathogenesis and hemophilia similar.
[Clinical manifestations of von Willebrand]
1. Have a family history: in line with autosomal dominant inheritance, which both men and women to the disease, both parents can be inherited. 2. Hemorrhagic tendency: a mucous membrane, subcutaneous bleeding, or menorrhagia, severe bleeding after surgery with or without history of a small number of joint, muscle bleeding, but generally no joint deformity.
[Von Willebrand diagnosis is based on]
(A) with or without family history, have a family history consistent with autosomal dominant or recessive inheritance.
(B) clinical there mucosa, skin, visceral bleeding, or menorrhagia history of trauma, surgery with or without abnormal bleeding history, a small number of patients can the relevant section of cavity, muscle or other parts of the bleeding.
(C) of the laboratory examination can help diagnose
1. Platelet morphology and count properly.
2. Prolonged bleeding or aspirin tolerance test was positive.
3. Platelet adhesion test extended or normal.
4. Activated partial thromboplastin time prolonged or normal.
5.vWF factor antigen (vWF: Ag) to reduce or normal.
6. Ⅷ coagulation factor activity (Ⅷ: C) reduced or normal.
7. Must exclude platelet dysfunction disease.
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