Dubin-Johnson syndrome
Dubin-Johnson syndrome Causes
Dubin-Johnson syndrome is a hereditary condition which is very rare, usually recessive, which means that a child must have gotten a copy of the defective recessive gene from both parents who are also both carriers of the disease.
Dubin-Johnson syndrome Definition
Dubin-Johnson syndrome is a disorder usually autosomal and recessive. This condition causes an increase of bilirubin without elevation of the liver enzymes ALT and AST. This condition is often associated with the inability of hepatocytes to secrete produced conjugated bilirubin to the bile in the liver. It can be diagnosed in early infancy. Building up of biliburin in the bloodstrean cause yellowish color of the eye and skin.
Dubin-Johnson syndrome Diagnosis
There are plenty of protein that is resistant to canalicular multi-drug, which also causes the transfer of bilirubin to the bile canaliculi. An isoform of this very protein is applied locally to the lateral hepatocyte membrane, thus allowing the transport of glutathione conjugates and glucuronide back to the blood. Analysis of some urine porphyrinsa also show a normal level of the coproporphyrin but I isomer accounts for a large rate of 80% compared to the normally 25% only. Liver will have a color of dark pink or black because of the accumulation of pigments. The conjugated hyperbilirubinemia is a result of defective endogenous and exogenous transfer of anionic conjugates from hepatocytes into the bile.[1] Pigment deposition in lysosomes causes the liver to turn black.
Dubin-Johnson syndrome Symptoms and Signs
People with this disease have mild jaundice which will last for a lifetime. People also manifest a yellowish color of their skin and the white part of the eye.
Dubin-Johnson syndrome Treatment
Treatment of Dubin-Johnson sysndrome is usually not necessary because most patients lead a normal life and have normal life spans.