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Dr. Andrew Rynne

Family Physician

Exp 50 years

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Is there a permanent treatment for Glanzmans Thrombostania ?

Dear Sir, my wife is suffering from Glanzmans Thrombostania since 1992, we married in 1994 till childless, tried IVF 8 times with Dr. Malpani, Dr. Upadhaya and Dr. SV Desai of Ghatkoper but result was negative. Pl advice me weather I should further try or not. and weather any permanent treatment is available for the decease. pl advice
Sat, 7 May 2011
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General & Family Physician 's  Response
Glanzmann thrombasthenia is a genetic platelet disorder in which the platelet glycoprotein IIb/IIIa (GP IIb/IIIa) complex is either deficient or present but dysfunctional. The genes of both of these proteins are on chromosome 17, and 50% activity of each protein is enough to support normal platelet aggregation. Defects in the GP IIb/IIIa complex leads to defective platelet aggregation and subsequent bleeding.

Glanzmann thrombasthenia is rare and it is inherited in an autosomal recessive pattern. The disorder was first described by Dr. Eduard Glanzmann in 1918.
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Pediatrician Dr. Suryakanta Baraha's  Response
hello,
Glanzmann's thrombasthenia is an autosomal recessive, inherited platelet function disorder. There is an absence of glycoprotein (GP) IIb/IIIa on the platelet membrane which causes reduced platelet aggregation with a defective platelet haemostatic plug formation.Pregnancy and delivery is rare in these patients and is associated with a high risk of severe haemorrhage
1-Once pregnancy occurs, patients
may develop bleeding during pregnancy for
obstetric causes, intrapartum or postpartum
2-Pregnancy in patients with Glanzmann's
thrombasthenia is rare,
but it is life-threatening
for both the patient and her fetus.
The fetal risk
is related to fetal immune thrombocytopenia
induced by the transplacental passage of the
maternal IgG anti-GPIIb-IIIa isoantibodies, In
case of the severe fetal thrombocytopenia, there
is a risk of fetal intracranial hemorrhage.
3-A team work, the obstetrician and the
hematologist is essential in deciding the best
option and best time to interfere in this high- risk
group of patients.
so mr pithabas better take consultation of both gynecologist and hematologist.
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Sports Medicine Specialist Dr. Abhay Dikshit's  Response
Hi,
The disease is a congenital platelate disease.
As such it has no connection with infertility.
The bleeding tendancy increases because ofthe disease.
It is a genetic disease. So genetic counselling & testing is of much help before trying for pregnancy.
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  User's Response
Dr. ajay agarwal's  Response
i think there is no relation between Glanzmans Thrombostania and childless
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Dear Sir, my wife is suffering from Glanzmans Thrombostania since 1992, we married in 1994 till childless, tried IVF 8 times with Dr. Malpani, Dr. Upadhaya and Dr. SV Desai of Ghatkoper but result was negative. Pl advice me weather I should further try or not. and weather any permanent treatment is available for the decease. pl advice