Hemochromatosis - bronze diabetes or pigment cirrhosis

Type 1
2nd type
Latent
Steroidogenic
Subcompensated
Gestational
Latent
Insipidus
Insulin-independent
Labile
Mody
Primary
Compensated
Decompensated
Acquired
Alloxan
Autoimmune
Bronze

 

HemochromatosisHemochromatosis (in other words it might be called bronze diabetes, or pigment cirrhosis of the liver) can be attached to regular diseases and is characterized by dysfunction or mistaken iron metabolism, high content of serum in blood and by accumulation of iron in the internal organs and tissues of organism. It can be met rarely, and mostly in men.

Scientists consider that hemochromatosis is hereditary disease, but not all patients have a mutation of the corresponding gene. Doctors distinguish primary bronze diabetes (that is genetically-mediated), and secondary hemochromatosis (which is developed under the influence of triggering factors). In case of this disease excess iron is accumulated in the liver, pancreas, heart muscle, pituitary gland, and skin up to a critical mass (about 20 grams), and above this threshold bronze diabetes is being manifested.

Secondary pigment cirrhosis is often seen in chronic smokers and alcoholics, because the metabolism, affects the liver and hemoglobinopathies. Usually this disease occurs in people who suffer from anemia or blood transfusion with blood diseases, and in people who take long-term or uncontrolled iron supplements.

There are no signs that will show development of this disease on early stages. There are general symptoms that are characteristic to many various diseases also as for example: fatigue, weight loss, pain in the lower back and abdomen and unstable blood pressure.

Specific symptoms of this disease are changes of skin color from normal to gray, and then some bronze shades, that usually appear later. Discoloration of the skin can be seen first of all on the face and in region of upper body, then it can be met on the back of the hands, on the genitals and legs. Quite often postoperative or posttraumatic seams and hems are darken.

Also one of the symptoms of this disease is reducing of the amount of hair, including hair that is located on the body. The patient complains on limited mobility and flexibility of joints and pain, impaired function of the pituitary gland, then partial loss of sexual function appears and certain number of secondary sexual characteristics. There also may be noticed some interruptions in the work of heart and development of diabetes mellitus.

Symptoms of this disease can be similar to symptoms in primary islet diabetes - thirst, hunger, glycosuria and polyuria, and in severe cases - acidosis, ketonuria (increasing of the number of ketone bodies that are contained in the blood).

Bronze diabetes mellitusPrimarily diagnostics may reveal enlarged spleen and liver, and that symptoms will require further laboratory and medical instrumental investigations in case of patient with this disease. Analyses will help determine the content of iron in the blood transferrin, ferritin, urine and after that deferoxamine probe should be completed. As a mandatory step of medical examination patient should complete X-ray scan, ECG, CT scan and a liver biopsy, results of which will help to finally confirm or refute the diagnosis by its typical signs.

Treatment of this disease is completed only in hospital self-treatment in case of this disease is excluded, since it takes a bloodletting. Treatment may be performed on an outpatient basis further with compulsory regular evaluation of the patient, and additional correction purposes. In this case some dietary restrictions are administered and also prophylactic treatment is prescribed to available and potential complications of this disease.

Hemochromatosis without treatment results death of patient within 5 years (statistics of mortality in this case are 100%). Timely and appropriate treatment of patient will help prevent the development of cirrhosis, cancer of liver and other disorders, so as result it can extend the life of the patient up to 20 - 30 years. Main cause of death of patients treated from hemochromatosis is delayed applying to hospitals, bleeding, liver cancer and diabetic coma.