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What is Methemoglobinemia?
Methemoglobin is a type of Hb . Methemoglobinemia is a blood term where the patient role has abnormal quantity of methemoglobin . Hemoglobin is the molecule that is present in there d roue cellsand helps in distributing the O all over the torso ; whereas , methemoglobin does not have the ability to release atomic number 8 . When a patient suffers from methemoglobinemia , the Hb in the physical structure is not capable to release O in an in force manner to the body tissue .
Methemoglobinemia is also known as erythrocyte reductase deficiency , Hemoglobin M disease or generalized reductase deficiency .
Pathophysiology of Methemoglobinemia
In human consistency , the normal level of methemoglobin are less than 1 % . Increased methemoglobin level in the blood pass off when the body mechanics , which is responsible for for fight down itself against oxidative stress inside the RBCs , gets swamped and the ferrous ion in the oxygen gets oxidized to the ferrous state ; thus converting hemoglobin to methemoglobin . This results in fall ability of the body to release the O to its tissues and leads to hypoxia . Because of this , the blood takes on a chocolate - brown or bluish color . The methemoglobin that is spring spontaneously gets boil down ordinarily , i.e. normal hemoglobin gets regenerated . This hap with the assistance of protective enzyme systems and also glutathione enzyme and ascorbic Zen . Any disruption in these enzyme system cause methemoglobinemia . Hypoxia results from the decrease oxygen - binding capacity of methemoglobin along with increase oxygen - binding affinity in the subunit present in the same particle of haemoglobin . This prevents oxygen expiration at normal tissue atomic number 8 levels .
Causes & Types of Methemoglobinemia
Methemoglobinemia can be inherited or congenital , i.e. it can be pass from the parents / syndicate . Methemoglobinemia can also pass off from vulnerability to certain chemical , drugs , or foods ; where it is referred to as acquired methemoglobinemia .
There are two types of inherited methemoglobinemia . Methemoglobinemia which is inherited by both parents is the first type . The parents often do not have this condition themselves ; however , they pack the cistron which cause methemoglobinemia . This happen when there is a problem with an enzyme do it as cytochrome b5 reductase .
This frame of methemoglobinemia has two types :
Type 1 : Erythrocyte Reductase Deficiency Methemoglobinemia : This appears when there is deficiency of this enzyme in the ruby-red ancestry cell .
Type 2 : Generalized Reductase Deficiency Methemoglobinemia : This come about when the enzyme is not able to work anywhere in the body .
Hemoglobin M disease is the second form of inherited methemoglobinemia that is caused due to flaw present in the haemoglobin atom . The abnormal factor can be passed to the child through one parent only for this disease to be inherited .
Acquired methemoglobinemia is more unremarkably found than inherited methemoglobinemia . Acquired methemoglobinemia develops in some somebody after they get exposed to certain drug and chemical substance , such as anesthetics ( benzocaine ) , benzol , certain antibiotics ( chloroquine , dapsone ) and nitrites .
Signs & Symptoms of Methemoglobinemia
symptom of Hemoglobin M disease which is another type of inherited methemoglobinemia admit : blueish colouring of the peel .
The symptom of character 1 methemoglobinemia , i.e. erythrocyte reductase deficiency methemoglobinemia are : There is bluish colouring of the tegument .
The symptom of type 2 methemoglobinemia , i.e. generalised reductase deficiency methemoglobinemia are :
symptom of Acquired Methemoglobinemia are :
Diagnosis of Methemoglobinemia
A lineage test helps with the diagnosing of methemoglobinemia . If a babe is conduct with this stipulation , then he / she will have cyanosis , i.e. blue skin color at birthing or after sometime . Other mental test , such as pulse oximetry tryout and arterial rip gases , can be done .
Treatment of Methemoglobinemia
intervention of methemoglobinemia consists of methylene blue , which is a medicine used for treating austere methemoglobinemia . It is authoritative to note that methylene blue can be grave and should not be used in someone who are suffering from or who are at risk of exposure for G6PD deficiency , which is a blood disease .
Ascorbic superman can also be used for decreasing the level of methemoglobin . Hyperbaric oxygen therapy and exchange transfusion can be done as substitute treatment for methemoglobinemia .
No treatment is want in bulk of the cases of mild acquired methemoglobinemia ; but patient role should avoid the specific chemical substance or medicine which has caused methemoglobinemia . intervention may be needed in dangerous cases of acquired methemoglobinemia including blood transfusion .
Prognosis of Methemoglobinemia
The forecast of haemoglobin M disease and case 1 methemoglobinemia is very salutary . The prognosis of case 2 methemoglobinemia is not good , as it is more serious and patient usually go within the first few years of his / her living .
The forecast of acquired methemoglobinemia commonly is also good and patients meliorate once the queasy intellectual nourishment , drug , chemical is identified and stop .
Potential Complications of methemoglobinemia include : seizures , shock and death .
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