Are there any blue Fugates alive?
Benjamin lost his blue coloring within a few weeks. Many Fugates were in good health despite having methemoglobinemia, and some reportedly lived into their 90’s. There are no known blue-skinned Fugates today.
What is a methemoglobinemia?
A condition in which a higher-than-normal amount of methemoglobin is found in the blood. Methemoglobin is a form of hemoglobin that cannot carry oxygen. In methemoglobinemia, tissues cannot get enough oxygen.
What is the reason that methemoglobinemia was isolated to Troublesome Creek area of KY?
The genetic form of methemoglobinemia is caused by one of several genetic defects, according to Tefferi. The Fugates probably had a deficiency in the enzyme called cytochrome-b5 methemoglobin reductase, which is responsible for recessive congenital methemoglobinemia.
Why the Fugate family has blue skin?
For the Fugate family, the excessive amount of blue methemoglobin in their blood turned their skin color blue. This blood disorder is the result of a recessive gene, and so requires that both parents of a child have the recessive gene for the disorder to appear in their offspring.
What is another name for methemoglobinemia?
Other names. Hemoglobin M disease. Chocolate-brown blood due to methemoglobinemia. Specialty. Toxicology.
How is methemoglobinemia treated?
Methylene blue is used to treat severe cases of MetHb, and doctors may prescribe ascorbic acid to reduce the level of methemoglobin in the blood. In severe cases, a person may require a blood transfusion or exchange transfusion. Oxygen therapy will also be provided, if required.
Does methemoglobinemia go away?
The condition is benign. There is no effective treatment for people with a congenital form who develop an acquired form. This means that they should not take drugs such as benzocaine and lidocaine. People who acquire methemoglobinemia from medications can completely recover with proper treatment.
What drugs can cause methemoglobinemia?
Drugs that may cause Methemoglobinemia include nitrates derivatives (nitrates salt, nitroglycerin), nitrites derivatives (nitroprusside, amyl nitrite, nitric oxide), sulfonamides, dapsone, phenacetin, phenazopyridine, some local anesthetics such as prilocaine, topical anesthetics such as emla cream, benzocaine.
What is the antidote for methemoglobinemia?
Methylene blue is the most effective antidote for acquired methemoglobinemia. When methylene blue is not available, alternative treatments such as ascorbic acid and hyperbaric oxygen can be useful. In this paper we presented a case of methomoglobinemia due to dapsone overdose.
What causes methemoglobinemia?
Most cases of methemoglobinemia are acquired and result from exposure to certain drugs or toxins. One of the more common causes of acquired methemoglobinemia is exposure to topical benzocaine during medical procedures.
How is methemoglobinemia diagnosed?
The diagnosis is confirmed by direct measurement of methemoglobin by a multiple wavelength co-oximeter. On a blood gas, normal PaO2 concentrations are usually found on analysis. Clinical cyanosis in the presence of normal arterial oxygen tensions is highly suggestive of methemoglobinemia.
How long does methemoglobinemia take to develop?
The onset of methemoglobinemia is usually within 20 to 60 minutes of drug administration.
What enzyme are people with methemoglobinemia missing?
Congenital methemoglobinemia is a rare underreported hemoglobin disease and often clinically missed. Upon extensive review of English literature for cases of congenital methemoglobinemia due to deficiency of cytochrome b5 reductase, we found 23 cases diagnosed as type I (including the case reported here).
Who is at risk for methemoglobinemia?
Acquired methemoglobinemia is more frequent in premature infants and infants younger than 4 months, and the following factors may have a role in the higher incidence in this age group: Fetal hemoglobin may oxidize more easily than adult hemoglobin.
What are the chances of getting methemoglobinemia?
Conclusions and relevance: The overall prevalence of methemoglobinemia is low at 0.035%; however, an increased risk was seen in hospitalized patients and with benzocaine-based anesthetics.
How many cases of methemoglobinemia are there?
Of the 319 cases, 7 were deaths, 32 cases were categorized as life threatening, and 216 were categorized as serious. A review of the 319 cases indicated that the development of methemoglobin emia after treatment with benzocaine sprays may not be related to the amount applied.
What does methemoglobinemia do to the body?
Hemoglobin is the protein in red blood cells (RBCs) that carries and distributes oxygen to the body. Methemoglobin is a form of hemoglobin. With methemoglobinemia, the hemoglobin can carry oxygen, but is not able to release it effectively to body tissues.
How does vitamin C treat methemoglobinemia?
Vitamin C is recommended for treatment of methemoglobinemia. It has been demonstrated that the reduction of the methemoglobin formation occurs at low vitamin C concentration in mice erythrocytes.
Is methemoglobinemia a genetic disorder?
Autosomal recessive congenital methemoglobinemia is an inherited condition that mainly affects the function of red blood cells. Specifically, it alters a molecule within these cells called hemoglobin. Hemoglobin carries oxygen to cells and tissues throughout the body….
What is a blue baby birth?
Infant methemoglobinemia is also called “blue baby syndrome.” It is a condition where a baby’s skin turns blue. This happens when there is not enough oxygen in the blood. Methemoglobinemia is a condition that some babies are born with (congenital) or some develop early in life (acquired).
Why does methemoglobin cause a left shift?
Methemoglobin (MetHb) is altered state of hemoglobin (Hb) in which the ferrous (Fe2+) irons of heme are oxidized to the ferric (Fe3+) state. The ferric hemes of MetHb are UNABLE to bind oxygen (O2). Thus, oxygen dissociation curve is left-shifted, making it more difficult to release O2.
Can methemoglobin bind oxygen?
Methemoglobin does not bind oxygen, thus effectively leading to a functional anemia. In addition, methemoglobin causes a leftward shift of the oxygen-hemoglobin dissociation curve, resulting in decreased release of oxygen to the tissues.
When was methemoglobinemia discovered?
It was first suggested by Hitzenberger3 in 1932 that this was an inherited metabolic disease. Gibson 4 in 1948 identified idiopathic methemoglobinemia as a disease due to an inborn error of metabolism.
Does benzocaine cause methemoglobinemia?
Benzocaine and other local anesthetics can cause methemoglobinemia, a serious condition in which the amount of oxygen carried through the blood is greatly reduced. This condition is life-threatening and can result in death.
What happens if you use too much Benzocaine?
An overdose of benzocaine topical applied to the skin can cause life-threatening side effects such as uneven heartbeats, seizure (convulsions), coma, slowed breathing, or respiratory failure (breathing stops). Avoid eating within 1 hour after using benzocaine topical on your gums or inside your mouth.
What’s better benzocaine or lidocaine?
Based on the results of our study, the following conclusions can be drawn: Two percent lidocaine gel is equally effective to 20% benzocaine gel in reducing the pain intensity of needle insertion. Both 2% lidocaine gel and 20% benzocaine gel are better than placebo in reducing pain of needle insertion.
Is it illegal to buy benzocaine?
Although benzocaine is a legal substance, it is illegal to supply it to the underground drugs trade. The drug is used legitimately in the UK as a dental anaesthetic and as an ingredient in first aid ointments, throat sprays and sunburn remedies.
How safe is Benzocaine?
FDA further advises that benzocaine oral drug products should only be used in adults and children 2 years and older if they contain certain warnings on the drug label because the local anesthetic benzocaine can cause the life-threatening condition, methemoglobinemia.
What class drug is Benzocaine?
But drug dealers like to use it in powder form to dilute pure class A and class B drugs such as naphyrone and mephedrone because of the numbing sensation it offers when placed on the skin.
How long does benzocaine gel last?
Benzocaine is a water-insoluble ester-type local anesthetic agent that is mainly useful for topical application. The onset of action is rapid (<1 minute), and the effective duration is 5 to 10 minutes.