Conditions in which there is poor blood flow to the systemic circulation, such as coarctation of the aorta suggests that the body does not receive the oxygenated blood it requires with resultant cyanosis.The five most common cyanotic heart defects that may result in Blue Baby Syndrome include the following:

Methemoglobinemia
Methemoglobinemia which can be acquired or congenital, occurs when the iron in hemoglobin is oxidized from Fe2+ to Fe3+, leading to poor binding of oxygen. Additionally, the oxygen that is already bound is held more tightly to the hemoglobin due to a higher affinity, resulting in less oxygen delivery. A methemoglobin level >1.5g/dL causes cyanosis. The most common congenital cause is a deficiency in the enzyme cytochrome b5 reductase which reduces methemoglobin in the blood. However, in infants the most common cause of methemoglobinemia is acquired through the ingestion of nitrates through well water or foods. Infants younger than 4 months are at greater risk given that they drink more water per body weight, they have a lower NADH- cytochrome b5 reductase activity, and they have a higher level of fetal hemoglobin which converts more easily to methemoglobin. Additionally, infants are at an increased risk after an episode of gastroenteritis due to the production of nitrites by bacteria. The sources of nitrate can include fertilizers used in agricultural lands, waste dumps or pit latrines. For example, nitrate levels are subject to monitoring to comply with drinking water quality standards in the United States and other countries.
This mutant shows a higher sensitivity to acetylcholine and unchanged desensitization compared to wild-type. Diagnosis
Diagnosis is typically made upon patient history, although EEG recordings can be confirmatory if they occur during attacks. Management
Anti-epileptic drugs are normally used to combat ADNFLE. These drugs are discussed in the main epilepsy article. Footnotes
Further reading
GeneReviews/NCBI/NIH/UW entry on Autosomal Dominant Nocturnal Frontal Lobe Epilepsy


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