परिभाषा
Spherocytosis is a condition that causes an abnormality in the red blood cell membrane. While healthy blood cells are shaped like flattened, indented disks, these abnormal membranes lead to sphere-shaped red blood cells, and the premature breakdown of those cells.
Red blood cells suffering from spherocytosis are smaller, rounder in shape, and more fragile than healthy red blood cells. The rounded shape causes the red blood cells to be caught in the spleen, where they break down.
Spherocytosis occurs in all races, but is most common in people of northern European descent.
Spherocytosis cases may be very mild, with minor symptoms, or very severe with symptoms that quickly surface. These symptoms may occur after certain types of infections. Severe cases may be diagnosed in childhood, while those with mild symptoms may not be diagnosed until into adulthood.
With treatment, symptoms can be controlled.
का कारण बनता है
Spherocytosis is a hereditary condition caused by genes inherited from the parents.
जोखिम कारक
जोखिम कारक वह है जो किसी बीमारी या स्थिति के होने की संभावना को बढ़ा देता है।
The following factors increase your chance of developing spherocytosis:
- Having a family member with spherocytosis
लक्षण
लक्षणों में शामिल हैं:
- पीलिया
- Pallor
- सांस लेने में कठिनाई
- थकान
- कमजोरी
- In children, irritability and moodiness
- Hemolytic anemia
- पित्ताशय की पथरी
निदान
आपका डॉक्टर आपके लक्षणों और चिकित्सा इतिहास के बारे में पूछेगा, और एक शारीरिक परीक्षण करेगा।
परीक्षण शामिल हो सकते हैं निम्नलिखित:
- Examination of the spleen
- रक्त परीक्षण
- लिवर फ़ंक्शन परीक्षण
- Osmotic and incubated fragility tests to diagnose hereditary spherocytosis
- Coombs’ Test—an antiglobulin test to examine red blood cell antibodies
उपचार
आपके लिए सर्वोत्तम उपचार योजना के बारे में अपने डॉक्टर से बात करें। उपचार के विकल्पों में शामिल हैं:
Folic Acid Supplementation
Folic acid 1 mg daily and consideration for blood transfusions are recommended during periods of severe anemia.
सर्जरी
Surgical removal of the spleen can cure the anemia. The abnormal shape of blood cells remain, but the blood cells are no longer destroyed in the spleen.
Currently, meningococcal, Haemophilus, and pneumococcal vaccines are administered several weeks before splenectomy. Lifetime penicillin prophylaxis is recommended after surgery to prevent dangerous infections. The surgery is not recommended for children under the age of five. There is a lifetime risk of serious and potentially life threatening infections.
रोकथाम
Because spherocytosis is an inherited condition, it is not possible to prevent the disease. Regular screening of individuals at high risk, however, can prevent the risk of complications of the disease with early treatment.