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Association of Paediatric Surgeons of Bangladesh
Journal of Paediatric Surgeons of Bangladesh
Review Articles · Vol. 6 No. 2 (2015)

Hemophilia in Children

Cover
JPSB
Journal of
Paediatric Surgeons of Bangladesh
Published : July 2015
CONTENTSVolume 06Number 2July 2015
Editorial
Counselling of Parents, attendants, guardians in dealing of Paediatric Surgical Patients
Kazi Habibur Rahman
32
Review Articles
Hemophilia in Children
Shahnoor Islam, AKM Amirul Morshed, Zohora Jameela Khan
54
Original Articles
Three Years Experience of Inguinal Hernia in Children in BSMMU
Shoheli Alam, KM Didarul Islam, Abu Saleh Md Oli Ullah, Md Tosaddeque Hossain Siddiqui, Md Ruhul Amin
34
Outcome of Partial Splenectomy for Hypersplenism in Children with Thalassemia
Mizanur Rahman, Susankar Kumar Mondal, Amin Lutful Kabir, Md Ruhul Amin, Matiur Rahman
39
Abdominal Ultrasonography and Radiography Diagnosis in Preterm Necrotizing Enterocolitis
S Tuya, Ch Battulga, TS Tsogtsolmaa, M Tumennasan, N Davaatseren, Kaniz Hasina
47
Case Reports
Spinal Primitive Neuroectodermal Tumor in Children A rare case report and literature review
AKM Amirul Morshed, Shahnoor Islam, Sayeeda Anwar
64
The Official Journal of the
ASSOCIATION OF PAEDIATRIC SURGEONS OF BANGLADESH (APSB)
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Authors

  • Shahnoor IslamAssociate Professor, Department of Pediatric Surgery, Dhaka Medical College Hospital. Dhaka-1000
  • AKM Amirul MorshedAssociate Professor and Head, Department of Pediatric Hematology and Oncology, Dhaka Medical College Hospital. Dhaka-1000
  • Zohora Jameela KhanAssociate Professor, Department of Pediatric Hematology and Oncology, Dhaka Medical College Hospital. Dhaka-1000

Keywords

Hemophilia Children Clotting factor Inhibitors Prophylaxis Comprehensive care

Abstract

Hemophilia is the most common serious congenital coagulation factor deficiencies. The prevalence of hemophilia is estimated to be about 1:10,000 birth and that of the severe form of the disease to be about 6% per 1,00,000 population. The most pathetic part of this disease is that even medical personnel are sometimes not familiar with its diagnosis and management. There is obviously a need to establish facilities and treatment options that will help the patient with hemophilia to manage their life with ease. As this is a genetic disorder no complete cure is possible as of now. The only available treatment option is the infusion of factors and some adjuvant therapies depending upon the bleeding conditions. Hemophilia, if not properly managed, can lead to chronic disease and lifelong disabilities. The challenges and issues in infants/young children are different from those in older children and adults although episodes of bleeding still predominate as the diagnostic trigger. Currently, inhibitor development is a challenging complication of pediatric hemophilia and prophylaxis is emerging as the optimal preventive care strategy. In this section we will review some important aspects of hemophilia in children including early prophylaxis, current evidence relating to inhibitor development.

Journal of Paediatric Surgeons of Bangladesh (2015) Vol. 6 (2): 54–63

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