Hemolytic crisis

Some deaths. Yellow discoloration of sclera of eye passing of red-colored urine. Copper concentrations, in mg/kg DW, from sheep fed nonheliotrope diets were 1394 in liver (824 in controls) and 132 in kidney (20 in controls). Sheep on heliotrope diet had 2783 mg/kg DW in liver and 321 mg Cu/kg DW in kidney Severe morphological changes in liver, kidney, and brain tissue damage continued after cessation of copper and was sufficiently severe to lead to repeated hemolytic crises. Maximum copper concentrations at day 83 were 3289 mg/kg DW in liver (138 in controls), and 683 in kidney (15 in controls)... 

In the few cases that have been described, mild chronic hemolytic anemia is associated with deficiency of GSH-S/ The disorder is inherited in an autosomal recessive mode. Such people may also have more severe acute hemolytic crises when exposed to redox substances, such as 8-amino-quinolines or other oxidant drugs. 

Occasionally, hemolytic crises are described in patients with deficiency of glu-cose-6-phosphate dehydrogenase under therapy with nalidixic acid. In rare cases, thrombocytopenic purpura or hemorrhagic enteritis has been observed (Traut-MANN and Opitz 1973). Arthralgia is sometimes described after nalidixic acid, and is thought to be of allergic origin. The joint involvement is occasionally associated with exanthema or fever (Bailey et al. 1972). Rubinstein (1979) describes a case of drug-induced lupus erythematosus after nalidixic acid. 

Patients who are deficient in y-glutamylcysteine synthetase or glutathione synthetase should avoid drugs that can induce hemolytic crises in patients with glucose-6-phosphate dehydrogenase deficiency, e.g. phenobar-bital, acetylsalicylic acid, sulfonamides. 

Fukuoka was found to be homozygous for the 1615 G to A (539 Asp to Asn) mutation. This mutation occurred at relatively conserved amino acid residues and caused an alteration in hydrophobicity. Recently, we examined the structure-function relationship of these variants using the recombinant protein (F14). Although all of the four variants were found to be heat labile, the residual GPI activity seems to reflect clinical severity, such as the degree of anemia and episodes of hemolytic crisis. GPI Matsumoto, associated with severe anemia and hemolytic crisis, was extremely unstable, and GPI Iwate, which is associated with compensated hemolytic anemia, showed moderate heat instability. Affinity for substrate, fructose-6-phosphate, was slightly decreased in GPI Narita and GPI Fukuoka, which were associated with moderate anemia and hemolytic crisis. 

This distribution suggests a race linkage of the trait. Further studies on families and relatives of affected subjects revealed the deficiency to be related to a dominant gene which is sex linked and sex modified, since the full symptoms of hemolytic crisis occur only in males (G19, L3, M7, S21, S22, S24). 

Hemolytic crisis may be confirmed by kwr hrmfigltibin and elevated reticulocyte counts. 

Exposure to naphthalene can cause a severe hemolytic crisis in some individuals with a genetically linked metabolic defect associated with insufficient activity of the glucose-6-phosphate dehydrogenase enzyme in red blood cells.10 Effects include anemia and marked reductions in red cell count, hemoglobin, and hematocrit. Contact of naphthalene with skin can result in skin irritation or severe dermatitis in sensitized individuals. In addition to the hemolytic effects just noted, both inhalation and ingestion of naphthalene can cause headaches, confusion, and vomiting. Kidney failure is usually the ultimate cause of death in cases of fatal poisonings. 

De Andrade, D.R., Fujito Neto, F.G., Vieira, G.S., Tiberio, I.F., Worth, M.R, Carlich, L Acute hemolytic crisis followed by fulminant hepatic failure with fatal outcome as a first clinical manifestation of Wilson s disease. Rev. Hosp. Clin. Fac. Med. Sao Paulo 1994 49 69-75... 

An interesting role of acid phosphatase alleles on an unusual condition in man has recently been reported by Bottini et al. (B27). It has long been appreciated that subjects with erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) deficiency may have a severe hemolytic crisis after ingestion of fava beans. Although this deficiency is a necessary condition for the occurrence of hemolytic episodes, not all, indeed only... 

A hemolytic crisis has been recorded in a patient with glucose-6-phosphate dehydrogenase deficiency (18). 

Conen D, Blumberg A, Weber S, Schubothe H. Hamolytische Krise und akutes Nierenversagen unter Rifampicin. [Hemolytic crisis and acute kidney failure from rifampicin.] Schweiz Med Wochenschr 1979 109(15) 558-62. 

Djerassi L, Hemolytic crisis in G6PD-deficient individuals in the occupational setting, Int. Arch. Occup. Environ. Health, 71, S26, 1998. 

Shibuya A, Hirono A, Ishii S, Fujii, Miwa S. Hemolytic crisis after excessive ingestion of fava beans in a male infant with G6PD Canton. Int J Hematol 1999 233-235. 

Hemolytic crisis in a non-ketotic and euglycemic child with glucose-6-phosphate dehydrogenase deficiency and onset of type 1 diabetes meUitus. 

Web site http //www.nlm.nih.gov/medlineplus/ency/article/003088.htm Hemolytic crisis... 

Kunzmann S, Thomas W, Mayer B, Kuhn S, Hebestreit H. Lmnune-mediated severe hemolytic crisis with a hemoglobin level of 1.6 g/dl caused by anti-piperadllin antibodies in a patient with cystic fibrosis. Infection 2010 38(2) 131-A. 

Transfusion reaction Hemolytic crisis Hemorrhagic shock Eclampsia Liver disease Leukemia... 

Elevated levels of certain hepatic enzymes [e.g., aspartate aminotransferase (AST), lactate dehydrogenase (LDH), sorbitol dehydrogenase (SDH), arxl arginase] indicate hepatic necrosis [see Chapter 8 III C 2 a (4) (b). Table 8-1)]. Serum or plasma liver enzymes may be elet ed 3-6 weeks before a hemolytic crisis and may be used to prerict animals at risk. 

The answer is 3 [III B 4 c IV E 3 a V F 3 VIIIC 3J. Of the choices presented, only arsenic toxicosis is not readily confirmed by detection of high concentrations In the blood. Arsenic in domestic animals has a high affinity for many epithelial tissues and is excreted rapidly in the urine. Copper accumulates in erythrocytes just prior to and during the acute hemolytic crisis. Iron is transported in the blood bound to transferrin, and both bound iron and total iron increase as dosage Increases. Selenium is present in both erythrocytes and serum or plasma and responds quickly to dietary or parenteral dosing. Lead is primarily associated with erythrocytes and is consistently elevated in lead poisoning. 

Icterus, dark urine and kidneys (from either hemoglobinuria or myoglobinuria), and anemia are signs of an acute hemolytic crisis, which is characteristic of ovine copper tc (icosis. 

Ammonium molybdate, sheep, acute hemolytic crisis... 

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