In any body, iron, like many other trace elements, occupies a significant place. But because of the excess of this substance in the body, it has the exact opposite effect on health. A condition in which the amount of iron in the body significantly exceeds the norm is called liver hemochromatosis.
Pathology Description
What is this disease? Liver hemochromatosis is a genetic recessive pathological condition that is characterized by excessive absorption and deposition of iron in the liver and other organs. This is a hereditary disease that can lead to the development of concomitant problems such as cirrhosis, endocrine disruptions, skin hyperpigmentation. Despite the systemic pathological anatomy in liver hemochromatosis, it is this organ that suffers the most from changes in the body.
Risks and Possible Consequences
Against the background of this disease, diabetes can develop, severe pain in the joints occurs, the condition as a whole deteriorates significantly. Excess iron is associated with excessive absorption in the digestive tract. After some time, the trace element accumulates in other tissues, causing overload. The first signs of this condition are joint pain, general weakness, abnormal change in skin tone, various disorders of sexual functions, heart failure and trauma to the liver itself. In some cases, all this leads to the manifestation of problems with the thyroid gland and even to cancer. Early identification of the causes and treatment of liver hemochromatosis can prevent the development of all sorts of complications.
By the way, the name of this pathology is not the only one. Many doctors call this disease also pigmented cirrhosis, bronze diabetes and siderophilia. In the scientific literature, hemochromatosis of the liver is often called Troisier-Ano-Shoffar syndrome.
Prevalence
Most often, bronze diabetes is a hereditary disease. So, if any of your relatives encountered this disease or a mutating gene was detected in one of the tests in your family, it makes sense to get tested. In many people, hemochromatosis can be easily detected using a simple blood test or special testing. By the way, the mutating gene, which is the cause of the development of the disease, is recessive, which means that for the appearance of a clinical pathology, both parents must have it.
Recent statistical information indicates that approximately one million people in the United States are predisposed to this disease. Of these, only 150 thousand people have a confirmed diagnosis. As a result, we can say that the risk of developing hemochromatosis of the liver is only 0.33%. By the way, now about 10% of the world's population have pathological genes in their DNA. Most often, the disease occurs in representatives of the light race. And the stronger sex turned out to be more susceptible to him: their disease develops about 10 times more often.
Causes of liver hemochromatosis
The most common prerequisite for the disease is precisely heredity. The pathological gene ceases to control iron metabolism in the body and is passed on to children from parents. But in addition to this, it should be said about several more causes of hemochromatosis of the liver, which are much less likely to provoke the development of the disease.
- Infectious forms of hepatitis B and C of a chronic nature.
- Non-alcoholic steatohepatitis, which leads to fat in the liver.
- Refusal of work of the pancreatic ducts.
- All kinds of malignant formations, of which cancer and leukemia are most often manifested.
- Surgery for cirrhosis can also lead to impaired normal metabolism. Most often, such manipulations are performed to resume blood circulation in the main vessel of the liver with an advanced form of the disease.
Due to the characteristics of the pathology, most often it occurs in the family. But nevertheless, the diagnosis of liver hemochromatosis should begin with the determination of the cause that led to the onset of the disease. If the pathological condition is not caused by the mutating gene at all, treatment should be started with the elimination of the factor that provokes interruptions in the metabolism of iron.
Varieties
Today, only two types of bronze diabetes are known, slightly different from each other:
- Primary liver hemochromatosis, which is a pathology that is inherited in the form of a mutating gene. And due to the fact that it is he who controls the metabolic processes of iron in the body, in the future all sorts of violations appear.
- Neonatal hemochromatosis of the liver is a rather rare disease that occurs in infants almost immediately after birth. This pathology is characterized by rapid progression. Possible causes of this phenomenon have not yet been identified.
Clinical picture forms
There are also several different stages of liver hemochromatosis.
- The first is characterized by an allowable amount of iron in the body, despite metabolic disorders.
- The second - an excess of trace elements in this case does not yet lead to distinct manifestations of the symptoms of the disease.
- The third - is characterized by a clear clinical picture characteristic of this disease.
Any prognosis without further treatment is one of the most unfavorable situations for any patient. In this case, the patient is unlikely to live more than 5 years from the time of diagnosis. Typically, the cause of death is acute failure of the liver, kidneys, or even the heart.
In the absence of proper therapy, the patient is likely to even have bleeding in the digestive tract. Older adults with this diagnosis are more likely to develop liver cancer.
Symptomatology
All kinds of signs of liver hemochromatosis are more or less pronounced, depending on the stage of the disease. The more time passes from the moment of illness, the more expressive the clinical picture becomes. The main symptoms of liver hemochromatosis, as a rule, are more noticeable in the third stage:
- Palpable weakness and marked fatigue.
- Constant low blood pressure.
- Rapid weight loss.
- Hyperpigmentation, which consists in a strong darkening of the skin, mucous membranes and eye sclera. This symptom manifests itself in 70% of all cases of the disease.
- The development of diabetes.
- Swelling and pain in the joints, due to which movements gradually become stiff or even limited.
- Complete absence or slow decrease in sexual functions.
- The development of various diseases: especially often renal, heart, liver failure and cirrhosis.
Due to the fact that hemochromatosis can provoke a large number of other pathologies, we can talk about the likelihood of secondary symptoms of the disease.
Establishing diagnosis
In order to make sure that the patient has precisely this disease, it is necessary to make a thorough comprehensive diagnosis. Such a diagnosis can be established on the basis of certain examinations:
- Diagnosis of liver hemochromatosis should begin with the collection and analysis of the patient's history. The most important period of the onset of the first symptomatology.
- It is also necessary to collect and analyze the history of the next of kin to identify the likelihood of developing hereditary hemochromatosis of the liver.
- Genetic testing for the detection of a mutating gene.
- A study of metabolic parameters in the body, which provides general information about the amount of iron.
- Puncture liver biopsy to detect excess trace elements.
Often, the patient also needs to consult other specialists, since bronze diabetes provokes the development of many concomitant diseases. Initially, a person who suspects the occurrence of this disease should resort to the services of a hepatologist or gastroenterologist - these doctors will decide the need for further examination and consultation with other doctors.
Treatment of liver hemochromatosis
Due to the fact that this pathology is genetic, truly effective methods of therapy do not exist. Treatment is aimed at protecting internal systems from injury, as well as cleansing the entire body from harmful toxins. The quality and longevity of a patient with such a diagnosis is significantly affected by the patient's nutrition and his habits.
Therapies
Therapy for this disease is aimed primarily at stopping the process of iron accumulation in the body, as well as its conclusion. In addition, it is very important to maintain the full functionality of all systems, preventing the development of complications.
- Compliance with a special diet, which is aimed at reducing the amount of food high in iron and protein.
- Taking medications necessary for the forced binding of excess trace elements for their subsequent withdrawal from the body.
- Bloodletting, in which approximately 0.5 liters of fluid is released from the surface vessels to improve the general condition of the body. Thanks to this procedure, the appearance of pigmentation is significantly reduced.
- Symptomatic treatment necessary to prevent the severe consequences of pathology and maintain the body in the condition that was characteristic of him at the time of diagnosis.
Treatment is carried out exclusively in a hospital, including many procedures, such as bloodletting. But the patient monitors the administration of prescribed medications and compliance with a certain diet on his own.
Diet
Compliance with a special menu in the treatment of liver hemochromatosis is a prerequisite for recovery. This technique is primarily aimed at reducing the amount of iron-containing foods in the diet. Patients with a confirmed diagnosis must adhere to several rules regarding nutrition:
- First of all, the patient should abandon the use of all kinds of alcoholic beverages. After all, alcohol contributes to the fact that the digestive tract absorbs more iron. In addition, alcohol is toxic and sends the whole body.
- It is necessary to abandon cigarettes, as well as to avoid secondhand smoke, as this causes metabolic disturbances, and this complicates the course of therapy.
- You should also reduce the number of flour products on the menu. It is very important first to abandon the black varieties of bread.
- It is necessary to stop eating delicacies made from animal kidneys and liver.
- It is necessary to reduce foods containing a large amount of vitamin C in your diet. This element also contributes to increased absorption of iron.
- It is very important to exclude seafood from the menu, of which the most dangerous for patients with a diagnosis of "hemochromatosis" are lobsters, algae, shrimp and crab.
- But the use of coffee and strong black tea should be intensified. After all, these drinks include tannins, which successfully slow down the absorption of iron by the body.
Harmful and healthy products
In the case of establishing hemochromatosis of the liver, the patient must necessarily review his own diet and adjust his diet.
It should be borne in mind that most of the iron is contained in such products:
- egg yolk;
- chicken hearts;
- rabbit meat;
- beef and pork tongue;
- quail eggs;
- syrup;
- pumpkin seeds;
- black caviar;
- red meat;
- White mushrooms;
- Brewer's yeast;
- apples
- sesame;
- halva;
- black currant;
- bananas
- all kinds of legumes;
- buckwheat grain.
Such people should pay special attention to:
- on potatoes;
- beets;
- cabbage;
- carrots;
- cucumbers
- pumpkin;
- onions and chives;
- Tomatoes
- cranberries;
- celery;
- honey;
- Cherries
- grapes;
- a variety of dairy products;
- citrus fruit.
Conclusion
Liver hemochromatosis needs immediate comprehensive treatment and proper diagnosis. Only in this way can the patient significantly increase the period of his own life, as well as prevent the development and exacerbation of all kinds of problems. And they usually arise in the absence of further complex therapy.