About dubin johnson syndrome

What is dubin johnson syndrome?

Dubin Johnson Syndrome is a rare genetic liver disorder that tends to affect people of Middle Eastern Jewish heritage disproportionately to other groups. It appears to be associated with clotting factor VII in this population. Symptoms may include a yellowish color to the skin (jaundice), and a liver that is sometimes enlarged and tender.

What are the symptoms for dubin johnson syndrome?

Improper functioning of the liver symptom was found in the dubin johnson syndrome condition

Approximately 80% to 99% of people with DJS have intermittent jaundice caused by excess bilirubin (bile pigment) that cannot be excreted normally. It builds up in the liver cells and then goes into the blood and is deposited in the eyes and skin. The same pigment can cause an abnormal urine color. The liver functions normally aside from the loss of an important transporter protein needed to move bilirubin out of the liver. Other less common symptoms include Fatigue and Fever. Rarely, bilirubin levels can become so high that organ damage is possible.

What are the causes for dubin johnson syndrome?

DJS is caused by changes (mutations) in the ABCC2 gene. This gene codes for a protein called multidrug resistance protein 2 (MRP2). This protein moves substances out of the cell and is found mainly in the liver but is also present in the kidneys, the intestine, and the placenta. The normal functioning protein works to secrete bilirubin into the bile, which is then transported to the gallbladder where it is stored. When the gall bladder is contracted during digestion, the bile is secreted into the intestine and then passes into the feces. Several different mutations have been identified that alter the function of the carrier protein. This process requires energy in the form of ATP and a common site of mutation is the part of the carrier that coordinates this aspect of the process.

Pregnancy or use of oral contraceptives may cause the disease to become apparent in women when no symptoms appeared previously.

DJS is inherited in an autosomal recessive pattern. Recessive genetic disorders occur when an individual inherits a non-working gene from each parent. If an individual receives one working gene and one non-working gene for the disease, the person will be a carrier for the disease, but usually will not show symptoms. The risk for two carrier parents to both pass the non-working gene and, therefore, have an affected child is 25% with each pregnancy. The risk to have a child who is a carrier, like the parents, is 50% with each pregnancy. The chance for a child to receive working genes from both parents is 25%. The risk is the same for males and females.

What are the treatments for dubin johnson syndrome?

Treatment of DJS is symptomatic and supportive. Many patients never require any treatment even though they have recurrent mild jaundice. However, metabolism of certain drugs may be affected in patients with DJS as many pharmaceutical products are metabolized in the liver. Therefore, a physician should carefully supervise medications.

Genetic counseling is recommended for patients and their families.

What are the risk factors for dubin johnson syndrome?

Dubin-Jhonson syndrome is a rare genetic liver disorder that is inherited by the autosomal recessive pattern ( inherit one mutated gene from each parent ). It is characterized by the breakdown of red blood cells into the yellowish pigment in the blood that results in bilirubin buildup. Moreover, this buildup is released into the bile, a digestive juice secreted by the liver. Moreover, this pigment gives stool a deep yellowish color, and the liver turns .

The risk factor associated with the Dubin-Jhonson syndrome:

  • Use of alcohol: Dubin johnson syndrome is associated with the liver, and alcohol leads to improper functioning of the liver. Hence, you should avoid alcohol during the treatment and even after recovery.
  • Birth control pills: the salt present in birth control pills like Valium, Indocin, and Dilantin increases the bilirubin production that directly affects the liver and makes the condition worse.
  • Pregnancy: During pregnancy, estrogen and progesterone are on the hike, which may affect the liver, and slower bile flows out of the liver.
  • Environmental factor: Contaminated air and water can affect the lungs, liver, and digestive systems resulting in the improper working of the liver.
  • Nausea: Usually, a person with Dubin-Jhonson experiences frequent vomiting and dizziness.


Conditions
Jaundice,Excess bilirubin in the blood,Improper functioning of the liver
Drugs
This benign disorder doesn’t require any specific therapy
Symptoms
Fatigue,Abnormal urinary color,Abnormal gastric mucus,Fever,Biliary tract abnormality,Conjugated bilirubinemia

Is there a cure/medications for dubin johnson syndrome?

Dubin-Jhonson syndrome is an inherited genetic disorder that causes yellowish pigment in the blood, which results in bilirubin. It passes into the child through the autosomal recessive pattern ( inherit one mutated gene from each parent ). However, this bilirubin buildup is released into the bile( a digestive juice secreted by the liver), giving the stool a deep yellowish color, the eyes turn white, and the liver turns .

Dubin- johnson syndrome is a benign disorder that doesn’t require any specific therapy. However, the patients should be warned that oral contraceptive use, pregnancy, and intercurrent illness can exacerbate the associated jaundice.

Conditions
Jaundice,Excess bilirubin in the blood,Improper functioning of the liver
Drugs
This benign disorder doesn’t require any specific therapy
Symptoms
Fatigue,Abnormal urinary color,Abnormal gastric mucus,Fever,Biliary tract abnormality,Conjugated bilirubinemia

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