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What Is Survival Rate For Hereditary Spherocytosis?

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Posted on Wed, 24 May 2017
Question: One of my neighbours has hereditary spherocytosis and his spleen removed in 1995.
He is 33yrs old, married with wife and daughter.
Can you let me know what can be his survival criteria or what complications he may suffer in future. What complications will his children suffer
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Answered by Dr. Diptanshu Das (37 minutes later)
Brief Answer:
His children will also have the disease

Detailed Answer:
Thanks for asking on Health care magic.

Hereditary spherocytosis an autosomal dominant disorder and so his children and their progenies as well would have the disease. It does not directly pose a threat to life but the removal of spleen makes such persons vulnerable to infections (with microorganisms having capsule made of a certain substance). Apart from general health and hygiene measures there is no direct way to prevent them. However, in case an infection develops prompt treatment is advisable.

Anemia, increased blood viscosity, unconjugated jaundice and pigmented gallstones or "sludge" in gall bladder are likely to develop.

Feel free to write back.

Regards
Note: For detailed guidance on genetic screening consult a genetics specialist

Above answer was peer-reviewed by : Dr. Kampana
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Answered by
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Dr. Diptanshu Das

Pediatrician

Practicing since :2005

Answered : 3875 Questions

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What Is Survival Rate For Hereditary Spherocytosis?

Brief Answer: His children will also have the disease Detailed Answer: Thanks for asking on Health care magic. Hereditary spherocytosis an autosomal dominant disorder and so his children and their progenies as well would have the disease. It does not directly pose a threat to life but the removal of spleen makes such persons vulnerable to infections (with microorganisms having capsule made of a certain substance). Apart from general health and hygiene measures there is no direct way to prevent them. However, in case an infection develops prompt treatment is advisable. Anemia, increased blood viscosity, unconjugated jaundice and pigmented gallstones or "sludge" in gall bladder are likely to develop. Feel free to write back. Regards