Tell me all you know about Heamochromatic NH liver Disease?

Answer 1

Here is a link that may be of some help to you:
http://www.neonatalhemochromatosis.org/

Here is information from a well respected site that doctors use, known as e medicine
http://www.emedicine.com/ped/topic954.htm
It will tell you the causes, treatment, and also the medications used to treat it.

This is a very rare disease. It is a iron storage disorder that is present at birth.
It has a different molecule origin than the adult kind of hemochromatosis. The symptoms can occur as early as 48 hours after birth because the liver cells are dying.
One of them being jaundice which is yellowing of the whites of the eyes, skin, and mucus membranes.
Many other babies are jaundice because the liver has not developed fully to handle the bilirubin at birth. Bilirubin is made by the spleen from the dead red blood cells. It is transported by a protein (Albumin)to the liver where the liver breaks it down into a soluble form that will flow with the bile to the intestines to help in the digestion of fats we eat. If the liver is damaged, this bilirubin goes into the blood instead (it is a greenish brown liquid) and causes this yellow color of the skin.
In this case, it isn't the fact that the liver is
not formed completely at birth, it is that the cells of the liver are dying and cannot convert this bilirubin into a soluble form or it is staying in the blood.

The baby may also have ascites: which is the swelling of the abdominal area cause by the build up of fluid. The protein that transport the bilirubin to the liver, is made by the liver. It is known as Albumin. Since the liver cells are dying, it cannot make an appropriate amount of this protein. Albumin is a known protein that keeps the fluid in our vascular system.
When there isn't enough, this fluid seeps out and gathers in the abdomen. In adults
they can remove this fluid through a procedure known as paracentesis, but it will continue to return because of the lack of this protein.

The iron overload can effect the salivary glands, liver, pancreas and other organs.
The physicians are not sure what is the cause of the iron overload in this case but think it may have to do with an abnormality of placental iron transfer or an alloimmune problem. A liver transplant is highly
recommended as soon as possible for a patient that has this.

There isn't much available to explain all this..
you can look under the heading of Rare
diseases, Idiopathic Neonatal Hemochromatosis, Liver disease, and others. I hope what I posted is of some
help.

Answer 2

An inherited disease (also known as "bronze diabetes") in which too much dietary iron is absorbed. Excess iron gradually accumulates in the liver, pancreas, heart, testes and to a lesser extent in other organs.

Causes:

The disease occurs almost exclusively in men. Women are very rarely affected because they regularly lose iron in their menstrual blood. Although Haemochromatosis is known to be genetic in origin, the exact method of inheritance is unclear. Male relatives of an effected person are more likely than average to develop the disease.

Symptoms and Complications:

Haemochromatosis rarely causes problems until middle age. A loss of sexual drive and a reduction in the size of the testicles are often the first signs. Eventually, excess iron causes liver enlargement and Cirrhosis (chronic liver damage), deficient insulin production by the pancreas leading to diabetes mellitus, bronzed skin colouration due to iron pigment deposition under the skin (hence the alternative name), cardiac arrhythmia and other heart disorders, and, during the late stages of the disease, liver failure and liver cancer.

Diagnosis and Treatment:

The diagnosis is based on blood tests, which reveal a high level of iron in the blood, and a liver biopsy (removal of a small sample of tissue for analysis), which shows the abnormal presence of iron.
Haemochromatosis is treated by withdrawel of blood from a vein (venesection). Initially, the procedure is performed once or twice a week. After the iron level has returned to normal, venesection is required only three or four times a year.
In young men, treatment can prevent the development of complications; in people who have fully developed haemochromatosis, regular venesections can prolong life. Drug treatment with chelating agents, such as desferrioxamine, has been investigated as an alternative to venesection.

Hope this helps.

Answer 3

My brother has this, and I had tbe tested too. Luckily for me, it turns out his haemochromatosis is the non-genetic version.

It is an inability of the liver to process iron, an excess of which is a poison. It most affects men, since bleeding controls iron levels, and women of childbearing age should naturally bleed at least once a month. It takes around 30 years of iron build up before it really becomes an issue. Therefore, female sufferers should see no problems until they are long into their menopausal years.

A patient can control the condition by regular blood letting, and drugs are not commonly needed, though they are availabe.

Answer 4

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Answer 8

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