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What are the performance and hemochromatosis diagnosed?As time and iron accumulation, HHC patient symptoms and gradually increased gradually, HHC early or initial symptoms are usually nonspecific symptoms, including weakness, joint pain, lethargy, chronic fatigue, weight loss (wasting), abdominal pain , skin color changes and lack of sexual desire, advanced the disease is the most important of the three major clinical manifestations of iron pigmentation of skin, secondary diabetes, cirrhosis of the liver.
HFE-related HHC, clinical manifestations of male usually becomes evident 40 to 50 years old; symptoms often occur in women after menopause, late age of onset often than men, caused by changes in non-HFE gene and HFE-related HHC HHC have a common Clinical features, youth HHC: a typical 10 to 30 years in the onset; common complications include: DM, hypogonadism, cardiac arrhythmia, heart failure, compared with HFE-related HHC heart failure more often.
Skin pigmentation: 90% ~ 100% of the patients had skin pigmentation, characteristic of metal or slate gray color is sometimes described as a bronze or dark brown, which is due to increased melanin (resulting in bronze) and iron deposition (resulting in gray pigment) calm in the dermis, the skin is dry, smooth, thin, poor flexibility, sparse hair loss, pigmentation is usually generalized, but the exposed parts of skin, armpits, genitals, nipples, scars and other parts may be more apparent , oral mucosa and gums have pigm


entation, the distribution of pigmentation characteristics and similar chronic adrenal insufficiency.
Secondary Diabetes: seen in 50% to 80% of patients, in patients with a family history of diabetes are more likely to occur, early mild impairment may no typical symptoms, the latter can be severe pancreatic damage with typical symptoms of , late complications of diabetes and other causes of the same may occur without prompt treatment of complications of acute metabolic disorders such as ketoacidosis, hyperosmolar non-ketotic diabetic coma, etc., in the past that the islet B cell destruction is the main cause of diabetes But in recent years that some patients with plasma insulin concentration did not significantly decreased, it was that the iron has a direct antagonistic effect of insulin, can make the peripheral tissue utilization of glucose and result in elevated blood sugar and diabetes, treatment with other types of diabetes are similar, but more often in patients with HC with marked insulin resistance.
Liver disease: is the most common clinical manifestations of HHC, hepatomegaly may first appear on the symptoms or abnormal liver function, patients with symptoms can occur in more than 90% of hepatomegaly, which some patients with impaired liver function almost no experimental room evidence, mildly elevated transaminases found in 30% to 50% of patients, bloodletting therapy, the aminotransferase returned to normal, advanced stage of liver dysfunction and portal hypertension, may occur with severe hepatic coma, gastrointestinal bleeding, portal hypertension and esophageal varices the appearance of varicose causes cirrhosis of the liver than other rare, HHC's long-term 14% of patients with liver cirrhosis (10% to 30%) of cancer, higher than any type of cirrhosis of the liver, often as a late sequela; most of the tumor multi-focal, symptoms may have been transferred, splenomegaly seen in about half of symptomatic patients.
Heart Disease: Congestive heart failure commonly seen in about 10% of young patients, especially young blood in patients with congestive heart failure symptoms may suddenly appear, if untreated can rapidly until death, the heart showed diffuse expansion, if no other obvious clinical manifestations may be misdiagnosed as idiopathic cardiomyopathy, arrhythmia, angina, myocardial infarction is less common arrhythmias include supraventricular premature beats, paroxysmal fast arrhythmia, atrial flutter, atrial fibrillation, atrioventricular block, ventricular fibrillation.
Endocrine dysfunction: The most common is hypogonadism, may be earlier than the other clinical symptoms, manifested as male impotence, libido disappeared, testicular atrophy, sperm scarce, infertility, male breast development; women have sparse menstrual disorders, amenorrhea, no pregnancy, body hair loss, etc., the change was mainly due to hypothalamic damage from iron deposition
Hemochromatosis should be treated?There is no effective curative therapy, common treatment measures include the removal of excess body iron and supportive therapy of damaged organs. Once-based disease patients should be treated immediately as soon as possible to reduce the body iron load, so that the body iron content was normal or near normal levels, which is to extend survival, so that the best way to reverse the tissue damage, commonly used treatment methods are as follows:
1. Diet in patients with HHC should be a balanced diet, eat more fruits and vegetables, legumes, grains, low-fat dairy products; to reduce intake of high iron foods, fish and poultry, meat and seafood lower iron content; drinking tea and coffee can reduce iron intake; try not to drink alcohol or less; avoid supplemental iron ions, iron complex vitamins, zinc and cough syrups do not add; avoid meals vitamin C, can be in the dining room drinking orange juice and other vitamin-rich fruit juices. Seafood contains bacteria, the HHC can cause a fatal infection, it should be carefully cleaned utensils and seafood, seafood full processing. Instead of glass or ceramic pan and stainless steel cookware.
2. Venous blood to reduce iron overload in the body the most important and effective measure is venous blood therapy. Each time blood normally 400 ~ 500ml, 1 or 2 times a week. Each time blood can remove about 200 ~ 300mg of iron, each row of iron in blood hemoglobin levels to vary for each row of discharge is equal to 1g of hemoglobin iron 3.4mg. 1 year can be bled about 100 units (per unit of 200ml). Specific programs should be considered in patients with bleeding body iron overload in varying degrees and different each time before and after bleeding, monitoring of serum iron, serum ferritin and transferrin saturation. Gradually removed the general iron will fall in serum ferritin, and transferrin saturation remains high. When the Hb dropped


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