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Hereditary Spherocytosis

Hereditary Spherocytosis

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Published by joanna

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Published by: joanna on Sep 07, 2009
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St. Dominic College of Arts and Sciences
Emilio Aguinaldo Highway, Talaba IV, Bacoor, Cavite
College of Nursing
A Case Study
Diabetes Mellitus Type II
“Hinog na Mangga”
Presented by:
Group 1
Agcaoili, JenalynAranzaso, ChristianColumna, LiezelHierco, Rica BiancaLegayada, Mary JerahManigsaca, MelizenParaiso, JoannaRomero, JelicaTurla, Jordina
H.S or commonly known as Hereditary Spherocytosis is an inherited blood disorder where a metabolic defect causes defects in the red blood cell membranes which lead to their characteristic spherical shape, a condition that is mild and requires no specific therapy.Hereditary Spherocytosis is a disease that results in the formation of abnormal red bloodcells with fragile cell walls. Red blood cells circulate in the blood and contain hemoglobin,which carries oxygen to all parts of the body. The shape of a normal red blood cell resembles adisc. Normal red blood cells easily change shape to move effectively through the small bloodvessels between organs of the body. A person with Hereditary Spherocytosis has red blood cellsthat are very round and have difficulty changing in shape. The lack of ability to change shapesmakes moving through the small blood vessels difficult. Therefore, the red blood cells stay inthe spleen longer than normal. This lengthy stay in the spleen damages the cell membranes.Eventually, the spleen will destroy these red blood cells.Paleness or yellow color of the skin or eyes, stomach pain, shortness of breath, irritabilityin children, fever and vomiting; these are some of the signs and symptoms of HereditarySpherocytosis. Symptoms of Hereditary Spherocytosis vary depending on the severity of thedisease.2
Hereditary Spherocytosis (H.S) is an inherited disease that causes anemia. If a child hasHereditary Spherocytosis, either parent may also have the disease. Occasionally, neither parentof an affected child has the disease; this is considered a spontaneous mutation.We choose Hereditary Spherocytosis as our case study because of its relevance toimmunology. Immunology covers the study of all aspects of the immune system in allorganisms. It deals with the function of the immune system in states of both health and disease,and the inability of the immune system to function in immunological disorders such asautoimmune diseases; like Hereditary Spherocytosis, in which the immune system attacks itsown host’s body.
One of the cases that we handled in Bautista General Hospital in Cavite, City isHereditary Spherocytosis. Hereditary Spherocytosis is a rare condition that captured the interestof the researchers to further study the case.Our patient is a 10 months old baby boy. He was admitted in Bautista General Hospitalwith a yellowish color of the skin because of his present condition that has been acquired fromhis mother. This condition is passed down from parents to children. A parent with the diseasehas a 50% chance of having a child with the disease. It is more common in people whoseancestors came from Northern Europe. The incidence of Hereditary Spherocytosis is about 200-3

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