Hemoglobinuria

Hamo-chininshure, /. hemoquinic acid, -glo-binurie, /. hemoglobinuria, -lyse, /. hemolysis, haemolysis. 

Individuals with hereditary low plasma cholinesterase levels (Kalow 1956 Lehman and Ryan 1956) and those with paroxysmal nocturnal hemoglobinuria, which is related to abnormally low levels of erythrocyte acetylcholinesterase (Auditore and Hartmann 1959), would have increased susceptibility to the effects of anticholinesterase agents such as methyl parathion. Repeated measurements of plasma cholinesterase activity (in the absence of organophosphate exposure) can be used to identify individuals with genetically determined low plasma cholinesterase. 

Auditore JV, Hartmann RC. 1959. Paroxysmal nocturnal hemoglobinuria—II. Erythrocyte acetylcholinesterase defect. Am J Med 27 401-410. 

Paroxysmal nocturnal hemoglobinuria (MIM 311770) Mutation resulting in deficient attachment of the GPI anchor to certain proteins of the red cell membrane... 

Paroxysmal nocturnal hemoglobinuria (MIM 311770) Acquired defect in biosynthesis of the GPf structures of decay accelerating factor (DAF) and CD59. 

Other diseases Autoimmune diseases Amyloidosis Aplastic anemia Paroxysmal nocturnal hemoglobinuria Fanconi s anemia Thalassemia major Sickle cell anemia Severe combined immunodeficiency Inborn errors of metabolism... 

Cu excess Deserts, Uzbekistan Serozems Disturbance of Cu exchange, endemic intero-hemoglobinuria... 

Plasma Haptoglobin in Relation to Hemoglobinemia, Hemoglobinuria and Erythrocyte Turnover... 

G6. Gilles, H. M., and Ikeme, A. C., Hemoglobinuria among adult Nigerians due to glucose-6-phosphate dehydrogenase deficiency with drug sensitivity. Lancet i, 889-891 (1960). 

Hemoglobinuria and hematin casts were reported in the renal tubules of rats treated dermally with 107.7 mg/kg phenol (Conning and Hayes 1970). These observations are indicative of red blood cell hemolysis however, this was not confirmed with hematological examinations. 

Hemoglobinuria and hematin casts in the distal convoluted tubules and tubular lumens located in the medulla and papilla were reported in rats after a single dermal exposure to 107.1 mg/kg liquid phenol (Conning and Hayes 1970). These phenomena are probably related to red blood cell lysis and increased glomerular filtration of hemoglobin. Hemoglobinuria is characteristic of lethal or near-lethal exposures by the dermal route. 

It has been stated that the dark urine is a result of oxidation products of phenol (Baker et al. 1978). The dark urine may also be a result of increased hemoglobin in the urine as suggested by the Merliss (1972) case report. In this case it took 2-3 months for the urine to clear after exposure was ended, which is not consistent with pharmacokinetic data that indicate that absorbed phenol is excreted in the urine in 1 day (Piotrowski 1971). A study in rats treated dermally with phenol, which found severe hemoglobinuria and hematin casts in the tubules (Conning and Hayes 1970), suggests that hemoglobin or hemoglobin breakdown products could contribute to the dark urine observed in humans. 

Soliris is for the treatment of paroxysmal nocturnal hemoglobinuria (PNH). PNH is a chronic disease where a patient s oxygen-carrying red blood cells are missing the normally present complement inhibitors. The cells are therefore abnormally fragile and inadvertently destroyed by normal complement activation. 

Broad bears, corrmonly called fava beans, are common to diets in Mediterranean countries (Greece, Italy, Spain, Portugal, and Turkey), in which their ingestion may cause severe hemolysis in G6PDH individuals. Clinically, the condition presents as pallor, hemoglobinuria, jaundice, and severe anemia 24-48 hours after ingestion of the beans. 

Paxillus syndrome is a food allergy, not a true poisoning. As a consequence, some who eat the mushrooms will not develop symptoms. Symptoms may include colic, vomiting, diarrhea, oliguria or anuria, kidney pain, hemoglobinuria, and renal failure. A hemagglutination test has been used for confirmation (Bresinsky and Besl, 1990). 

Applied to the skin or eyes of rabbits, PNCB did not cause irritation it was absorbed, producing methemoglobinemia, Heinz bodies in erythroqnes, anemia, hematuria, and hemoglobinuria. The acute dermal LDso for rabbits was 3400mg/kg. 

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