Demographics, clinical profile and treatment landscape of patients with haemophilia B in China

Haemophilia ◽  
2022 ◽  
Author(s):  
Wenhui Zhang ◽  
Xuewen Song ◽  
Xueqing Dou ◽  
Man‐Chiu Poon ◽  
Wei Liu ◽  
...  
Keyword(s):  
Clinical Profile ◽  
Haemophilia B

scholarly journals Clinical Profile of Haemophilia In Children in A Tertiary Care Hospital

2013 ◽  
Vol 37 (2) ◽  
pp. 90-96 ◽  
Author(s):  
MA Karim ◽  
R Siddique ◽  
CY Jamal ◽  
A Islam
Keyword(s):  
Neonatal Period ◽  
Severe Disease ◽  
Tertiary Care ◽  
Clinical Profile ◽  
Haemophilia A ◽  
Care Hospital ◽  
Initial Manifestation ◽  
Haemophilia B ◽  
Pediatric Hematology ◽  
History Of

Introduction : Haemophilias are the most common inherited coagulation disorders transmitted by X- linked recessive fashion affecting the males and females are the carriers of the disease. Haemophilias are distributed worldwide and have heterogeneous presentation depending upon its severity starting from neonatal period. Knowledge of spectrum of presentation of haemophilia helps in early diagnosis and planning of management. Objectives : To observe the clinical presentation of haemophilia in children Methodology : This observational study was carried out in the Department of Pediatric Hematology and Oncology, Bangabandhu Sheikh Mujib Medical University for a period of one year from 1st July 2007 to 30th June 2008. Clinical profile of 50 diagnosed cases of haemophilia <15 years of age was analyzed. Result: All the 50 cases of haemophilia were male. Mean age of the patients was 6.62±3.87 years with an age range of 6 months to 14 years. Forty (80%) cases were haemophilia A and 10 (20%) cases were Haemophilia B. Only 40% cases had family history of bleeding. Among the Haemophilia A, 52.5% cases had mild, 47.5% cases had moderate disease and among the Haemophilia B, 40% cases had mild, 50% cases had moderate and 10% cases had severe disease. Heamarthrosis of knee joint was the major presentation followed by oral cavity bleeding, bleeding following tooth extraction and circumcision. Sixty two percent cases had initial bleeding episode before 1 year of age and by 5 years of age 94% of cases had produced symptoms. No patient had history of bleeding during neonatal period. Bruises and hematoma were the most common initial manifestation followed by joint bleeding, cut injury of lips and chin, scalp and facial hematoma. Conclusion: Bruises, hematoma and joint bleeding either spontaneous or after trauma were the main feature at initial presentation of haemophilia in children, so presence of these features in an otherwise normal child should be considered for evaluation of haemophilia. More vigilance is to be needed for detection of haemophilia in newborn. DOI: http://dx.doi.org/10.3329/bjch.v37i2.17266 BANGLADESH J CHILD HEALTH 2013; VOL 37 (2) : 90-96

A new strategy for prenatal diagnosis in a sporadic haemophilia B family

Haemophilia ◽  
2001 ◽  
Vol 7 (4) ◽  
pp. 416-418 ◽  
Author(s):  
M. Acquila ◽  
F. Bottini ◽  
A. Valetto ◽  
D. Caprino ◽  
P. G. Mori ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Haemophilia B ◽  
New Strategy

Impact of extraversion on the clinical profile of panic disorder

2007 ◽  
Author(s):  
Emilie Chan ◽  
Ewelina Zaremba ◽  
Jacques Bradwejn ◽  
Diana Koszycki
Keyword(s):  
Panic Disorder ◽  
Clinical Profile

Performances of an Artificial Reagent for the One-stage Factor IX Assay

1975 ◽  
Vol 33 (03) ◽  
pp. 547-552 ◽  
Author(s):  
L Meunier ◽  
J. P Allain ◽  
D Frommel
Keyword(s):  
Human Plasma ◽  
Factor Ix ◽  
Severe Haemophilia ◽  
Normal Human Plasma ◽  
Haemophilia B ◽  
One Stage ◽  
Normal Human ◽  
The One

SummaryA mixture of adsorbed normal human plasma and chicken plasma was prepared as reagent for factor IX measurement using a one-stage method. The substrate was found to be specific for factor IX. Its performances tested on samples displaying factor IX activity ranging from <l%–2,500% compared favorably with those obtained when using the plasma of severe haemophilia B patients as substrate.

Management of Haemophilia in Sweden

1976 ◽  
Vol 35 (03) ◽  
pp. 510-521 ◽  
Author(s):  
Inga Marie Nilsson
Keyword(s):  
Factor Viii ◽  
Total Population ◽  
Age Groups ◽  
Factor Ix ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Haemophilia B ◽  
Human Fraction ◽  
Mild Haemophilia

SummaryThe incidence of living haemophiliacs in Sweden (total population 8.1 millions) is about 1:15,000 males and about 1:30,000 of the entire population. The number of haemophiliacs born in Sweden in 5-year periods between 1931-1975 (June) has remained almost unchanged. The total number of haemophilia families in Sweden is 284 (77% haemophilia A, 23% haemophilia B) with altogether 557 (436 with A and 121 with B) living haemophiliacs. Of the haemophilia A patients 40 % have severe, 18 % moderate, and 42 % mild, haemophilia. The distribution of the haemophilia B patients is about the same. Inhibitors have been demonstrated in 8% of the patients with severe haemophilia A and in 10% of those with severe haemophilia B.There are 2 main Haemophilia Centres (Stockholm, Malmo) to which haemophiliacs from the whole of Sweden are admitted for diagnosis, follow-up and treatment for severe bleedings, joint defects and surgery. Minor bleedings are treated at local hospitals in cooperation with the Haemophilia Centres. The concentrates available for treatment in haemophilia A are human fraction 1-0 (AHF-Kabi), cryoprecipitate, Antihaemophilic Factor (Hyland 4) and Kryobulin (Immuno, Wien). AHF-Kabi is the most commonly used preparation. The concentrates available for treatment in haemophilia B are Preconativ (Kabi) and Prothromplex (Immuno). Sufficient amounts of concentrates are available. In Sweden 3.2 million units of factor VIII and 1.0 million units of factor IX are given per year. Treatment is free of charge.Only 5 patients receive domiciliary treatment, but since 1958 we in Sweden have practised prophylactic treatment of boys (4–18 years old) with severe haemophilia A. At about 5-10 days interval they receive AHF in amounts sufficient to raise the AHF level to 40–50%. This regimen has reduced severe haemophilia to moderate. The joint score is identical with that found in moderate haemophilia in the same age groups. For treatment of patients with haemophilia A and haemophilia B complicated by inhibitors we have used a large dose of antigen (factor VIII or factor IX) combined with cyclophosphamide. In most cases this treatment produced satisfactory haemostasis for 5 to 30 days and prevented the secondary antibody rise.

The Formation of Intermediate Product I in a Purified System The Role of Factor IX or of its Precursor and of a Hageman Factor-PTA Fraction

1961 ◽  
Vol 6 (02) ◽  
pp. 224-234 ◽  
Author(s):  
E. T Yin ◽  
F Duckert
Keyword(s):  
Intermediate Product ◽  
Factor Ix ◽  
Assay Method ◽  
Lag Phase ◽  
Factor X ◽  
Haemophilia B ◽  
Hageman Factor ◽  
One Stage ◽  
The One

Summary1. The role of two clot promoting fractions isolated from either plasma or serum is studied in a purified system for the generation of intermediate product I in which the serum is replaced by factor X and the investigated fractions.2. Optimal generation of intermediate product I is possible in the purified system utilizing fractions devoid of factor IX one-stage activity. Prothrombin and thrombin are not necessary in this system.3. The fraction containing factor IX or its precursor, no measurable activity by the one-stage assay method, controls the yield of intermediate product I. No similar fraction can be isolated from haemophilia B plasma or serum.4. The Hageman factor — PTA fraction shortens the lag phase of intermediate product I formation and has no influence on the yield. This fraction can also be prepared from haemophilia B plasma or serum.

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