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Hemoglobinuria is a condition in which the oxygen transport protein hemoglobin is found in abnormally high concentrations in the urine. [1] The condition is caused by excessive intravascular hemolysis, in which large numbers of red blood cells (RBCs) are destroyed, thereby releasing free hemoglobin into the plasma. [2]
While the urine dipstick test is able to recognize heme in red blood cells, it also identifies free hemoglobin and myoglobin. [6] Free hemoglobin may be found in the urine resulting from hemolysis, and myoglobin may be found in the urine resulting from rhabdomyolysis (muscle breakdown). [6] [5] Thus, a positive dipstick test does not ...
These components comprise hemoglobin and others. [3] At this stage, the hemoglobin is called free hemoglobin. [3] Free hemoglobin (also called naked hemoglobin) is the un-bound hemoglobin that is not enclosed in the red blood cell. The naked hemoglobin is devoid of its anti-oxidant sentries that are normally available within the RBC. [5]
In the presence of free hemoglobin/myoglobin, uniform colour ranging from a negative yellow through green to a strongly positive green-blue appears on the pad. In contrast, intact red blood cells are lysed when they come in contact with the pad, and the liberated hemoglobin produces an isolated reaction that results in a speckled pattern on the ...
Thus, a positive result for blood can represent the presence of red blood cells , free hemoglobin (hemoglobinuria), or myoglobin (myoglobinuria). [51] Red blood cells can sometimes be distinguished from free hemoglobin or myoglobin as the former causes a speckled pattern on the test pad while the latter results in a uniform color change.
Hemosiderinuria (syn. haemosiderinuria) is the presence of hemosiderin in urine. [1] It is often the result of chronic intravascular hemolysis, in which hemoglobin is released from red blood cells into the bloodstream in excess of the binding capacity of haptoglobin. The function of haptoglobin is to bind to circulating hemoglobin, thereby ...
The heme is ultimately converted to bilirubin and removed in stool and urine. [44] Hemoglobin may be cleared directly by the kidneys resulting in fast clearance of free hemoglobin but causing the continued loss of hemosiderin loaded renal tubular cells for many days. Additional effects of free hemoglobin seem to be due to specific reactions ...
Danicopan, sold under the brand name Voydeya, is a medication used for the treatment of paroxysmal nocturnal hemoglobinuria. It is a complement inhibitor which reversibly binds to factor D to prevent alternative pathway-mediated hemolysis and deposition of complement C3 proteins on red blood cells. The most common side effects include fever, headache, increased levels of liver enzymes (a sign ...