Methemoblobinemia - Blue Skin People
HUMAN SMURF
With Great Honour
Done by

Glennis Ong Yu Xuan
& Tan Li Si
2M03 / P01

Medical Biochemistry
Lecturer - Dr John Zhu CongJu
Contents
of this blog

1. The Story of the Fugates
2. Methemoglobinemia??
- The Disorder
- How did methemoglobin levels rise so high?
- Can it be treated?
3. The Biochemistry
References
& the links

[1] abc News
[2] Autosomal Dominant Conditions
[3] Autosomal Recessive Conditions
[4] Illustrated Medicine
[5] International Business Times
[6] Medicine Net - Acquired Methemoglobinemia
[7] Medscape Reference - Methemoglobinemia
[8] Medline Plus - Methemoglobinemia
[9] Methemoglobinemia, beta-globin type
[10] MeSH Descriptor Data - Methemoglobinemia
[11] The Blue People Of Troublesome Creek
[12] UCDavis ChemWiki - Pentose Phosphate Pathway






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for this blog template

Template: Elle (blog)
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Others: colour codes





Methemoglobinemia
Wednesday, 24 July 2013

[met-he-mo-glo-bi-ne-mi-a] [4]
{A Disorder}
Types: Congenital (Inherited) and Acquired methemoglobin  [4,5]
Pattern: Autosomal Recessive, but became Autosomal Dominant [5]
Autosomal recessive conditions: Both parents are carriers, 'a' is the gene with the recessive mutation. Example: Cystic fibrosis
Autosomal Recessive [3]
VS.  Autosomal dominant conditions: One parent is affected, 'B' is the gene with the dominant mutation. Example: Huntington's disease.
Autosomal Dominant [2]













Methemoglobinemia - A condition when methemoglobin level is more than 1% in Red Blood Cells (RBCs) [6,7]

 METHEMOGLOBIN
= An abnormal form of hemoglobin [1]
- Heme group is in the ferric state (Fe3+) instead of the ferrous state (Fe2+) [4]
Causing methemoglobin to not be able to release oxygen to tissue cells effectively. [4]
[4]


Methemoglobin Level & Range: [4]
The normal range of methemoglobin in RBCs: 0%-3%
With increase in level, symptoms will get more serious as well.
With methemoglobin levels of
3%-15% : There'll be slight some discoloration of the skin (e.g Pale, gray, blue),
15%-20%: Cyanosis (bluish appearance of the skin, mucous membranes, or the area underneath the fingernails due to a lack of oxygen in the blood)
and as levels further increases above 70%, death is usually resultant.

From this, it can be inferred that methemoglobin level in Blue-Skin people is below 20% 

How did methemoglobin levels rise so high?
Lack of enzyme Diaphorase - responsible for conversion of Methemoglobin to Hemaglobin [4,11]

Hemoglobin = reduced form of Methemoglobin

 In Normal, Healthy Bodies,
Diaphorases (enzymes), such as Cytochrome-b5-reductase, Nicotinamide adenine dinucleotide phosphate (NADPH) and more, are present.Therefore, any excess methemoglobins in the blood, will be converted back to hemoglobin.


 Can it be treated?
- YES!
[5,7,8]