Cardiomyopathy Information and Courses from MediaLab, Inc.
These are the MediaLab courses that cover Cardiomyopathy and links to relevant pages within the course.
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| Development of Iron Overload The amount of time needed for iron to increase to levels causing organ damage is variable and may be partially dependent on gender, dietary or other environmental factors, and unknown genetic factors. Blood loss through menstruation and pregnancy are thought to delay the onset of iron overload, and therefore symptoms of HH, in women. Similarly, regular blood donation may confer some degree of protection. The loss of hemoglobin within intact erythrocytes reduces the amount of iron available for recycling.As levels of storage iron increase, clinical features of iron overload, including hepatic dysfunction or failure, diabetes, hypogonadism, arthritis, cardiomyopathy, hyperpigmentation, and fatigue, may become evident.Symptomatic patients typically present in middle age between the ages of 30 and 60, although this is quite variable. Persons as young as 20 may show clinical signs and symptoms of HH.(6) In the US, males are more than twice as likely as females to be diagnosed with HH, and the majority of cases are found in Caucasians. | View Page |
| Prognosis and Mortality The major determinant of prognosis in cases of hereditary hemochromatosis (HH) is the degree of organ damage from iron overload at the point of diagnosis. The presence of liver cirrhosis reduces life expectancy. Damage that has occurred to tissues and organs is irreversible, but further damage can be halted with treatment. When there is no evidence of cirrhosis at time of diagnosis, life expectancy may be equal to that of persons without HH. With proper management of HH through treatment, affected individuals have good long-term outcomes. Hepatocellular carcinoma associated with cirrhosis, hepatic failure, and cardiac failure are the most common causes of death in persons with HH. Compared to the normal population, liver cancer is many times more prevalent as a cause of death in persons with HH. Cardiomyopathy, diabetes, and cirrhosis are all more common causes of death among persons with HH than among normal persons. The earlier HH is detected, before the onset of severe organ damage, the lower the risk of mortality. | View Page |
| Rationale for Treatment Treatment for hereditary hemochromatosis (HH) is typically indicated for iron overload in symptomatic patients. The goal of therapy is to reduce stored iron which may result in reversal or resolution of some symptoms and improve prognosis. Causes of death in patients with HH include serious medical conditions such as hepatocellular carcimoma, cirrhosis, cardiomyopathy, and diabetes. Ideally, treatment should begin before these conditions develop. The earlier HH is detected, before the onset of severe organ damage, the lower the risk of mortality. | View Page |
| Which of the following is NOT a cause of death in patients with hereditary hemochromatosis (HH)? | View Page |