Background information by Professor Omar Hasan Kasule Sr. for Year 2 Semester 1 Medical PPSD session on 03th September 2008
ANEMIA
Background information
Anemia is a symptom of an underlying disease. It is essentially a deficiency of oxygen carrying capacity of blood. Anemia results when the amount of hemoglobin is reduced. This occurs when production falls, when cell destruction is excessive, and when there is blood loss. Blood loss may be due to trauma or diseases resulting in chronic blood loss.
Decreased production of red blood cells occurs in nutritional deficiency (iron, folic acid, and vitamin B12), infection, bone marrow damage by ionizing radiation as well as toxic chemicals, and in neoplastic infiltration of the bone marrow.
Increased destruction of red blood cells occurs in various hemolytic diseases. Hemolytic anemias are accompanied by jaundice, and reticulocytosis. They are caused by red cell abnormalities such as hereditary spherocytosis, enzyme deficiencies, drugs and toxins, autoimmunity, and incompatible transfusions.
Anemias of various causes manifest different appearances on microscopic examination of the blood film. Macrocytic anemia is due to release of immature red blood cells into the circulation due to compensatory hemopoiesis. Hypochromic microcytic anemias are due to iron deficiency or impaired globin production. Normochromic normocytic anemias are due to sudden loss of blood. Normocytic anemias are found in many chronic diseases, hormonal deficiencies (anterior pituitary, thyroid, adrenocortical, and testicular). In vitamin B12 and folic acid deficiency or malabsorption, megaloblastic anemia results accompanied by reduced reticulocytes, granulocytes, and platelets. Aplastic anemia is due to bone marrow failure caused by toxins and autoimmunity. It is characterized by low levels of leucocytes and platelets.
Ethico-legal-fiqh issues
The main interest of the Law is prevention of nutritional anemia. Anemia is common in childhood and pregnancy. It is obligatory for the mother to take hematinics as directed in the interests of the fetus. It is obligatory on parents to provide sufficient nutrition to the infant to prevent anemia. Although breast-feeding is recommended for 2 years, it is not sufficient by itself after the 6th month. The father is obliged by Law to provide sufficient nutrition to the nursing mother and the infant to prevent anemia.
Physicians must be alert to anemia because it can be suspected in most cases from the routine screening examination of any patient presenting to the clinic. The physician can suspect anemia by looking for pallor of the conjuctiva or the tongue. Anemia of slow onset may not present with any dramatic symptoms and cases have been recorded of patients who presented to hospital with extremely low hemoglobin levels that were discordant with but their general health condition.
COAGULATION DISORDERS
Background information
Coagulation failure may be caused by hemophilia, vitamin K deficiency, liver disease, Von Willebrand’s disease, disseminated intravascular coagulation (DIC), and other causes. Hemophilia is an X-linked disorder. Hemophilia A is due to absence of coagulation factor VIII. Hemophilia B is due to deficiency of factor IX. Vitamin K deficiency leads to coagulation disorders because it is needed for the synthesis of prothrombin, factor X, factor IX, protein C, and protein S. Synthesis of clotting factors is impaired n severe liver disease. Fibrinogen deficiency occurs as an inherited disorder and also as part of the syndrome of disseminated intravascular coagulation. Von Willebrand’s disease is autosomal dominant condition due to lack of a plasma protein that binds to factor VIII and promotes interaction of platelets with blood vessels.
Disseminated intravascular coagulation (DIC) is caused by bacteria of other material that induced abnormally active coagulation using up all available fibrinogen and thus causing coagulation failure.
Excessive coagulation results in thrombosis which is formation of blood clots blocks blood vessels. It starts by damage to the vascular endothelium and platelet aggregation at the site of injury and eventual formation of a thrombus. An embolus is a dislodged thrombus. Causes of thrombosis may be inherited (such as deficiency of antithrombin III, protein C, protein S, and plasminogen), pulmomary embolism, and deep vein thrombosis. Risk factors for thrombosis are: increase of platelets, abnormal platelets, oral contraceptive pills, and pregnancy. Heparin prevents clot formation. Clots are dissolved by fibrinolytic agents such as streptokinase, urokinase, and tissue plasminogen activator.
Ethico-legal-Fiqh issues
Pre-marital genetic testing and counseling may be useful for couples especially if closely related and there is a history of hereditary coagulation disorders in the family.
Patients on treatment for coagulation disorders need constant and close monitoring because they could develop thrombosis or hemorrhage. Their coagulation medication will have to be adjusted if they have other disease conditions that may cause interference. Surgery and pregnancy for patients with coagulation disorders require very careful weighing of risks and benefits.