Tooba Fateen, Saima Irem, Aleena Hussain, Nazish Saqlain, Nisar Ahmed.
Frequency of Rare Bleeding Disorders: Three Years Experience at the Hematology Unit of a Tertiary Care Hospital, The Children's Hospital Lahore.
Pak Paed J Jan ;41(4):239-42.

Objective: To determine the frequency of rare bleeding disorders among all patients diagnosed with bleeding disorders. Introduction: The rare bleeding disorders are inherited as autosomal recessive disorders. They represent 3 to 5% of all the inherited deficiencies of coagulation factors. Their frequency in the general population ranges from 1:500000 to 1:2 millions. They are more common in countries where there is high rate of consanguineous marriages. Settings: This study was carried out in the Coagulation Division of Hematology & Transfusion Medicine Department of The Children?s Hospital & Institute of Child Health Lahore from July 2013 to Dec 2015. Samples from suspected bleeding disorders children aged 0-18 years were collected. Coagulation profile including Specific Factor assays were carried out on them. Results: Out of 204 patients having clinical features suggestive of bleeding disorders, thirty one (15%) patients had rare bleeding disorders. This group comprised of ten patients with Factor X deficiency, another ten patients with Factor VII deficiency, eight patients with Factor V deficiency, two patients with Factor I deficiency and one patient with combined VKDFD (Vitamin K dependent factors deficiency). No patient was diagnosed as factor XIII, Factor II or factor XI deficient. 159 (77.9%) of all patients were male and 45 (22%) were female. Age-wise, thirty patients were less than one year of age, one hundred and sixty eight were between one year and ten years of age and six patients were between eleven and eighteen years of age (14.7%, 82.3% and 2.9% respectively). Conclusion: Rare bleeding disorders are not so rare in our society and early diagnosis and treatment can save patients from developing complications.

PakMediNet -Pakistan's largest Database of Pakistani Medical Journals - http://www.pakmedinet.com