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I read the above mentioned article
with interest(1). The authors state that prophylactic use of Factor X to
prevent recurrent ICH was initially reported by Sandler et al. We
reported in 1991, 4 neonates with Factor X deficiency and the use of
prophylactic prothrombin complex in all of them(3). One infant died at 4
months inspite of replacement therapy with PPSB (Biotest) every third
day, due to massive intracranial hemorrhage. Another baby boy who was
also given PPSB every third day remained asymtomatic till eight months
when he was taken back to Sudan.
The remaining 2 children,
who were 11 years and 9 years of age (when I left Middle East in 1998)
were on replacement therapy every 10 days, at a dosage of 40 units per
kg body weight. They were doing well without any serious mucous membrane
or closed cavity hemorrhages. We have discussed in our article how we
arrived at the dosage and frequency of administration.
Incidentally, our report
has the largest collection of neonates with congenital factor X
deficiency so far published.
N.S. Menon,
Consultant Pediatrician,
V.G. Saraf Memorial Hospital,
Sreekandath Road,
Kochi 682 016, India.
-
Ramdas J, Ole D,
Warrier RP. Factor X deficiency: An unusual cause for spontaneous
intracranial bleeding. Indian Pediatr 2000; 6: 656-659.
-
Sandler G, Gross S.
Prevention of recurrent intracranial hemorrhage in a Factor X
deficient infant. Am J Pediatr Hematol Oncol 1992; 14: 163-165.
- Kalla S, Menon NS. Neonatal
congenital Factor X deficiency. Pediatr Hematol Oncol 1991; 8:
347-354.
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