SICKLE CELL (ANEMIA) DISEASE

Pathophysiology of Sickle Cell (Anemia) Disease

Abstract

Sickle cell anemia (SCD) is a genetic disease called "autosomal recessive". Autosomal because the gene involved in the disease is carried by a non-sex chromosome. Recessive, as it is expressed only when both copies of the gene, the alleles are carriers of the anomaly. In other words, parents, healthy carriers, have sent each assigned an allele of the mutation to their child, in fact, is ill. Now there is a type of anemia characterized by an alteration in the normal hemoglobin (whose figure resembles a disk), which changes shape when the amount of oxygen in the blood is reduced for any reason, leading to the cells that containing also change their structure and adopt a sickle or scythe, the anomaly is named disease (or anemia), sickle cell, which in his figure interfere with normal blood flow, and that they block small blood vessels. Also known as sickle cell anemia, is usually made evident at six months old with a swollen abdomen, swollen hands and feet and yellow skin and eyes over time will give rise to poor oxygenation of the heart growth, ulcers in the legs, frequent respiratory infections, changes in the gums, and can even delay sexual maturity in adolescence.

Table of Contents

Background of the Study4

Sickle cell disease (SCD)6

Discussion and Analysis6

Pathophysiology6

Symptoms of sickle cell disease and therapeutic approaches6

Prevalence of the disease and protection against malaria6

Etiology6

Treatment6

Complications6

Anesthetic management6

Conclusion6

References6

Pathophysiology of Sickle Cell (Anemia) Disease

Background of the Study

The origin of the sickle cell gene is located, altered, which encodes a protein chain in the composition of hemoglobin. The latter is found in red blood cells (or erythrocytes), which carry oxygen in the blood. In case of sickle cell disease, poor conformation of hemoglobin gives red blood cells a sickle shape characteristic. This particular red blood cell, sickle drepanos meaning in Greek, makes their movement. Few quick to deform, unlike their normal counterparts perfectly round blood cells sickle cling to the walls of blood vessels. In adulthood there will be chronic fatigue, abdominal and joint pain, irritability, blindness in the last years of the life and times of prolonged erections (priapism)-and sometimes painful-in men.

Anemia, one of the most common blood disorders, occurs when the concentration of red blood cell is too low. This can lead to health problems because RBCs contain hemoglobin, which transports oxygen to body tissues. Anemia can cause various complications, including fatigue and exhaustion from overuse of many organs of the body. (Pemberton, 2006)

Anemia can be caused by many factors, but the three main bodily mechanisms that produce it are:

excessive destruction of red blood cells

blood loss

inadequate production of red blood cells

Among many other causes, anemia can be the result of inherited disorders, nutritional problems (such as iron deficiency or vitamin), infections, some types of cancer or exposure to drugs or toxins.

Anemia caused by destruction of red blood cells

Hemolytic anemia occurs when red blood cells are destroyed prematurely (the normal half-life of erythrocytes is 120 days, but ...