Haemosiderosis Health Dictionary

An inherited disease in which too much dietary iron is absorbed. Excess iron gradually accumulates in the liver, pancreas, heart, testes, and other organs. Men are more frequently affected because women regularly lose iron in menstrual blood.

Loss of sex drive and a reduction in the size of the testes are often the first signs. Excess iron over a period of time causes liver enlargement and cirrhosis, and can lead to diabetes mellitus, bronzed skin coloration, cardiac arrhythmia, and, eventually, liver failure and liver cancer.

Diagnosis is based on blood tests and a liver biopsy. Treatment is by regular venesection. (See also haemosiderosis.)... haemochromatosis

ACUTE LIVER DISEASE The hepatitis viruses (A– F) are of paramount importance. Hepatitis E (HEV) often produces acute hepatic failure in pregnant women; extensive epidemics – transmitted by contaminated drinking-water supplies – have been documented. HBV, especially in association with HDV, also causes acute liver failure in infected patients in several tropical countries: however, the major importance of HBV is that the infection leads to chronic liver disease (see below). Other hepatotoxic viruses include the EPSTEIN BARR VIRUS, CYTOMEGALOVIRUS (CMV), the ?avivirus causing YELLOW FEVER, Marburg/Ebola viruses, etc. Acute liver disease also occurs in the presence of several acute bacterial infections, including Salmonella typhi, brucellosis, leptospirosis, syphilis, etc. The complex type of jaundice associated with acute systemic bacterial infection – especially pneumococcal PNEUMONIA and pyomiositis – assumes a major importance in many tropical countries, especially those in Africa and in Papua New Guinea. Of protozoan infections, plasmodium falciparum malaria, LEISHMANIASIS, and TOXOPLASMOSIS should be considered. Ascaris lumbricoides (the roundworm) can produce obstruction to the biliary system. CHRONIC LIVER DISEASE Long-term disease is dominated by sequelae of HBV and HCV infections (often acquired during the neonatal period), both of which can cause chronic active hepatitis, cirrhosis, and hepatocellular carcinoma (‘hepatoma’) – one of the world’s most common malignancies. Chronic liver disease is also caused by SCHISTOSOMIASIS (usually Schistosoma mansoni and S. japonicum), and acute and chronic alcohol ingestion. Furthermore, many local herbal remedies and also orthodox chemotherapeutic compounds (e.g. those used in tuberculosis and leprosy) can result in chronic liver disease. HAEMOSIDEROSIS is a major problem in southern Africa. Hepatocytes contain excessive iron – derived primarily from an excessive intake, often present in locally brewed beer; however, a genetic predisposition seems likely. Indian childhood cirrhosis – associated with an excess of copper – is a major problem in India and surrounding countries. Epidemiological evidence shows that much of the copper is derived from copper vessels used to store milk after weaning. Veno-occlusive disease was ?rst described in Jamaica and is caused by pyrrolyzidine alkaloids (present in bush-tea). Several HIV-associated ‘opportunistic’ infections can give rise to hepatic disease (see AIDS/HIV).

A localised (focal) form of liver disease in all tropical/subtropical countries results from invasive Entamoeba histolytica infection (amoebic liver ‘abscess’); serology and imaging techniques assist in diagnosis. Hydatidosis also causes localised liver disease; one or more cysts usually involve the right lobe of the liver. Serological tests and imaging techniques are of value in diagnosis. Whilst surgery formerly constituted the sole method of management, prolonged courses of albendazole and/or praziquantel have now been shown to be e?ective; however, surgical intervention is still required in some cases.

Hepato-biliary disease is also a problem in many tropical/subtropical countries. In southeast Asia, Clonorchis sinensis and Opisthorchis viverini infections cause chronic biliary-tract infection, complicated by adenocarcinoma of the biliary system. Praziquantel is e?ective chemotherapy before advanced disease ensues. Fasciola hepatica (the liver ?uke) is a further hepato-biliary helminthic infection; treatment is with bithionol or triclabendazole, praziquantel being relatively ine?ective.... liver disease in the tropics

Analysis of a sample of blood to give information on its cells and proteins and any of the chemicals, antigens, antibodies, and gases that it carries. Such tests can be used to check on the health of major organs, as well as on respiratory function, hormonal balance, the immune system, and metabolism. Blood tests may look at numbers, shape, size, and appearance of blood cells and assess the function of clotting factors. The most important tests are blood count and blood group tests if transfusion is needed. Biochemical tests measure chemicals in the blood (see acid–base balance; kidney function tests; liver function tests). Microbiological tests (see immunoassay) look for microorganisms that are in the blood, as

in septicaemia. Microbiology also looks for antibodies in the blood, which may confirm immunity to an infection. blood transfusion The infusion of large volumes of blood or blood products directly into the bloodstream to remedy severe blood loss or to correct chronic anaemia. In an exchange transfusion, nearly all of the recipient’s blood is replaced by donor blood. Before a transfusion, a sample of the recipient’s blood is taken to identify the blood groups, and it is matched with suitable donor blood. The donor blood is transfused into an arm vein through a plastic cannula. Usually, each unit (about 500 ml) of blood is given over 1–4 hours; in an emergency, 500 ml may be given in a couple of minutes. The blood pressure, temperature, and pulse are monitored during the procedure.

If mismatched blood is accidentally introduced into the circulation, antibodies in the recipient’s blood may cause donor cells to burst, leading to shock or kidney failure. Less severe reactions can produce fever, chills, or a rash. Reactions can also occur as a result of an allergy to transfused blood components. All

blood used for transfusion is carefully screened for a number of infectious agents, including HIV (the AIDS virus) and hepatitis B and hepatitis C.

In elderly or severely anaemic patients, transfusion can overload the circulation, leading to heart failure.

In patients with chronic anaemia who need regular transfusion over many years, excess iron may accumulate (haemosiderosis) and damage organs such as the heart, liver, and pancreas.

Treatment with desferrioxamine to remove excess iron may be needed.... blood tests

Any of a variety of conditions in which there is too much iron in the body. (See also haemosiderosis.)... siderosis

A group of inherited blood disorders in which there is a fault in the production of haemoglobin. Many of the red blood cells become fragile and haemolyse (break up), leading to anaemia (see anaemia, haemolytic). Thalassaemia is prevalent in the Mediterranean, the Middle East, and Southeast Asia, and in families originating from these areas.

Normal adult haemoglobin contains 2 pairs of globins (protein chains): alpha and beta. In thalassaemia, a recessive defective gene results in reduced synthesis of 1 of the chains. Usually beta-chain production is disturbed (beta-thalassaemia). Beta-thalassaemia minor (thalassaemia

trait), which is never severe, is caused by 1 defective gene. The presence of 2 defective genes causes beta-thalassaemia major (Cooley’s anaemia). The much rarer disorder alpha-thalassaemia varies in severity; alpha-thalassaemia major usually results in fetal death.

Symptoms of beta-thalassaemia major appear 3–6 months after birth. If untreated, bone marrow cavities expand, leading to a characteristic enlargement of the skull and facial bones.

Beta-thalassaemia major is diagnosed from microscopic examination of the blood, and from other blood tests. Treatment is with blood transfusions and, sometimes, splenectomy. However, successive blood transfusions cause a buildup of iron in the body (see haemosiderosis). Chelating agents are given by continuous infusion to help the body excrete the excess iron. A bone marrow transplant offers a cure for the disease.

Genetic counselling is advised for parents or other close relatives of a child with thalassaemia, and also for any person with thalassaemia trait.... thalassaemia