The Indian Academy of Pediatrics has released consensus guidelines for the diagnosis and management of hemophilia in India. The guideline is published in the journal Indian Pediatrics.
Guideline for the management of hemophilia in Indian setting is essential as despite having standard principles of management of hemophilia, the treatment differs in various countries depending on available resources.
For developing the guidelines Indian Academy of Pediatrics conducted a consultative meeting on Hemophilia on 18th September 2016 in New Delhi, which was attended by experts in the field working across India. Scientific literature was reviewed, and guidelines were drafted. All expert committee members reviewed the final manuscript.
- Specific factor assays confirm a diagnosis and classify hemophilia according to residual factor activity (mild 5-40%, moderate 1-5%, severe <1%).
- Genetic testing helps in identifying carriers and providing genetic counseling and prenatal diagnosis.
- Patients with hemophilia should be managed by a multi-specialty team approach.
- Continuous primary prophylaxis (at least low-dose regimen of 10-20 IU/kg twice or thrice per week) is recommended in severe hemophilia with dose tailored as per response.
- Factor replacement remains the mainstay of treating acute bleeds (dose and duration depend on body weight, site, and severity of bleed).
- Factor concentrates (plasma-derived or recombinant), if available, are preferred over blood components.
- Other supportive measures (rest, ice, compression, and elevation) should be instantly initiated.
- Long-term complications include musculoskeletal problems, development of inhibitors and transfusion-transmitted infections, which need monitoring.
- Adequate vaccination of children with hemophilia (with precautions) is emphasized.
Hemophilia (A or B) is an inherited (X-linked recessive) bleeding disorder caused by the deficiency of coagulation factors VIII or IX, respectively. Males are affected and females are asymptomatic or mildly affected carriers. Deficiency of either of these factors results in defective intrinsic coagulation pathway (decreased and delayed generation of thrombin, defective clot formation, and hemorrhagic diathesis).
Globally, the prevalence is around 1 in 5000 male births for hemophilia A and 1 in 30,000 male births for hemophilia B. World Federation of Hemophilia (WFH) report on the annual global survey 2015, which covered 91% of world population, identified nearly 190,000 hemophilia patients (nearly 150,000 (80%) with hemophilia A). There were around 17,500 hemophilia patients (83% hemophilia A) identified from India in this survey, which should be an underestimate.
For further reference log on to https://doi.org/10.1007/s13312-018-1302-8