Sabtu, 06 November 2010
Blue Skin Disorder: the blue people
Kentucky, human skin is generally olivaceous, brown, or dark. But one family in Kentucky have blue skin for a blood disorder called methemoglobinemia, earning the nickname “Blue People of Kentucky ‘.
National Institutes of Health reported no data or how many people are experiencing methemoglobinemia, but the most famous incident was the Blue People of Kentucky and Lurgan family from Ireland. As quoted from eHow, actually the nickname ‘The Blue People of Kentucky’ derived from the Fugate family who lived in Hazard, Kentucky.
During the 1800s, Martin Fugate who married a blue-skinned member of the Smith family who also carry the recessive gene. This caused Smith to be carrying the gene family methemoglobinemia and lowering blood disorders are on the next descendants.
Fugate and the Smith family lived in the area and continue to produce children and further descendants of blue-skinned. From the latest documentation, these cases occurred until the 1970s.
‘Blue People of Kentucky’ was first diagnosed with methemoglobinemia in 1960 by a hematologis, Dr. Madison Cawein.
According to folklore, Fugate experienced blue skin occurs because of heart disease or lung problems. However, examination of Dr. Martin Fugate Cawein find descendants in good health, and he suspected methmoglobinemia form of derivative.
According to Dr. Scott’s research in the Journal of Clinical Investigation, which examined methmoglobinemia Eskimos in Alaska, methmoglobinemia occurred because the loss of enzyme in red blood cells.
The absence of an important enzyme called this diaphorase, allowing components deoxygenated red blood cells (the process of reduction of dissolved oxygen) to develop inside the body, thus giving a blue bandage visible on the skin surface.
Methmoglobinemia Fugates case is genetic, recessive genes derived from both parents, causing chaos in the offspring. Besides genetics, methmoglobinemia also can be caused by exposure to chemicals, antibiotics and anesthesia.
Methmoglobinemia symptoms may include blue skin, shortness of breath, fatigue, dizziness, and fainting. More extreme cases can cause cardiac arrhythmia, convulsions, coma and even death. However, the ‘Blue People of Kentucky’ can survive longer and healthier.
According to Dr Cawein, methmoglobenemia can be treated with methylene blue, which causes the body to compensate for lack of diaphorase. These medications are excreted through urine, so drug methylene blue is required every day to maintain a normal skin color.
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