Severe haemophilia A children on low‐dose tertiary prophylaxis showed less joint deterioration and better maintenance of functional independence than children on on‐demand treatment: A 6‐year follow‐up study

Haemophilia ◽  
2020 ◽  
Vol 26 (5) ◽  
pp. 779-785
Author(s):  
Ying Liu ◽  
Lixia Chen ◽  
Kuixing Li ◽  
Mingnan Shi ◽  
Man‐Chiu Poon
Keyword(s):  
Low Dose ◽  
Functional Independence ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
On Demand ◽  
Follow Up Study

scholarly journals Four Years’ Follow-Up Study of Liver Function of Haemophiliacs

1977 ◽  
Author(s):  
F. Panicucci ◽  
U. Baicchi ◽  
A. Sagripanti
Keyword(s):  
Alkaline Phosphatase ◽  
Liver Disease ◽  
Liver Function ◽  
Normal Values ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Serum Albumins ◽  
Follow Up Study ◽  
Hepatitis B Antigen

By physical and biochemical examinations 112 patients with severe haemophilia A or B and 24 with mild to moderate forms were studied to determine the incidence of liver damage, which may have resulted from commercial concentrate therapy.Data of the follow-up study during a period of 4 years showed transiently elevated values of SGOT and SGPT in 100 patients, persistently elevated values in 14 and normal values in 22 patients. Test for hepatitis B antigen was positive in 9 patients. Hepatomegaly, splenomegaly or both were found in 15 patients. Acute hepatitis with jaundice was reported by 23 patients, 17 of whom still showed abnormal enzyme tests. Chronic active hepatitis was very strongly suggested in 5 patients, who on repeated occasions showed, in addition to elevated SGOT and SGPT, abnormal values of serum albumins, immunoglobulins, alkaline phosphatase and Normotest. The majority of patients were free from clinical liver disease.

Comparison of the efficacy and safety of 12‐month low‐dose factor VIII tertiary prophylaxis vs on‐demand treatment in severe haemophilia A children

Haemophilia ◽  
2019 ◽  
Author(s):  
Novie A. Chozie ◽  
Fitri Primacakti ◽  
Djajadiman Gatot ◽  
Rahajuningsih D. Setiabudhy ◽  
Angela B. M. Tulaar ◽  
...  
Keyword(s):  
Factor Viii ◽  
Low Dose ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Efficacy And Safety ◽  
On Demand ◽  
Dose Factor

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.

HOME TREATMENT OF HAEMOPHILIA A FOLLOW‐UP STUDY

1981 ◽  
Vol 2 (1) ◽  
pp. 21-23 ◽  
Author(s):  
Henry Ekert ◽  
Michelle Moorehead ◽  
Graham Williamson
Keyword(s):  
Home Treatment ◽  
Haemophilia A ◽  
Follow Up Study

scholarly journals Cost-Utility of Prophylaxis VS. On-Demand Treatment In Severe Haemophilia A Patients

2016 ◽  
Vol 19 (7) ◽  
pp. A591
Author(s):  
Z Gharibnaseri ◽  
M Davari ◽  
A Cheraghali ◽  
P Eshghi ◽  
R Ravanbod ◽  
...  
Keyword(s):  
Cost Utility ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
On Demand

Secondary prophylaxis treatment versus on-demand treatment for patients with severe haemophilia A: comparisons of cost and outcomes in Taiwan

Haemophilia ◽  
2010 ◽  
Vol 17 (1) ◽  
pp. 45-54 ◽  
Author(s):  
W.-S. LIOU ◽  
T.-C. TU ◽  
S.-N. CHENG ◽  
T.-Y. CHOU ◽  
C.-F. LEE ◽  
...  
Keyword(s):  
Secondary Prophylaxis ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
On Demand

scholarly journals Association of frailty in hospitalized and institutionalized elderly in the community-dwelling

2016 ◽  
Vol 69 (4) ◽  
pp. 691-696 ◽  
Author(s):  
Suzele Cristina Coelho Fabrício-Wehbe ◽  
Rosalina Aparecida Partezani Rodrigues ◽  
Vanderlei Jose Haas ◽  
Jack Roberto Silva Fhon ◽  
Marina Aleixo Diniz
Keyword(s):  
Demographic Data ◽  
Functional Independence Measure ◽  
Functional Independence ◽  
Sampling Procedure ◽  
Community Dwelling ◽  
Specific Data ◽  
Follow Up Study ◽  
Dual Stage ◽  
The Subject

ABSTRACT Objective: to investigate the association between frailty with hospitalization and institutionalization in a follow-up study of elderly residents. Method: the follow-up study was performed in 2008 and 2013 with elderly of both genders, aged 65 years and older who were living in the community-dwelling. The sampling procedure performed was probabilistic, with dual-stage clustering. In 2008, 515 elderly people were interviewed and, in 2013, 262. We used the socioeconomic and demographic data, self-reported morbidity, specific data of hospitalization and institutionalization. Frailty was measured by the Edmonton Frail Scale (EFS), and functional capacity through the Functional Independence Measure. Results: we found the mean gross EFS score was higher among resident elderly who were hospitalized and institutionalized and was statistically significant in both investigated years. Conclusion: the confirmation of association between frailty and hospitalization and institutionalization reinforces the importance of the subject, and highlights frailty as an important tool for risk estimates for these adverse events.

Discontinuing early prophylaxis in severe haemophilia leads to deterioration of joint status despite low bleeding rates

2016 ◽  
Vol 115 (05) ◽  
pp. 931-938 ◽  
Author(s):  
Annelies Nijdam ◽  
Wouter Foppen ◽  
Piet de Kleijn ◽  
Evelien P. Mauser-Bunschoten ◽  
Goris Roosendaal ◽  
...  
Keyword(s):  
Functional Limitations ◽  
Objective Assessment ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
On Demand ◽  
Recommended Treatment ◽  
Objective Monitoring ◽  
Joint Health ◽  
Observational Cohort

SummaryProphylaxis is the recommended treatment for children with severe haemophilia A, but whether prophylaxis should be continued in adulthood is still under debate. Previous studies with limited follow-up have suggested that some patients may be able to stop prophylaxis in adulthood, while maintaining good joint health. This single-centre observational cohort study examined patients with severe haemophilia A born 1970–1988 without inhibitor development, and assessed the long-term consequences of discontinuing prophylaxis. Patient-initiated changes in prophylaxis, including all switches to on-demand treatment lasting a minimum of two consecutive weeks, were recorded from the time self-infusion began until the last evaluation. Sixty-six patients were evaluated at a median age of 32.4 years: 26 of patients had stopped prophylaxis for a median of 10 years, 15 had interrupted prophylaxis and 59 had continued prophylaxis. Annual joint bleeding rate (AJBR), Haemophilia Joint Health Score (HJHS-2.1; 0–124 points), radiological Pettersson score (0–78 points) and Haemophilia Activities List score (HAL; 100–0 points) were compared between patients who stopped and patients who continued prophylaxis. Although self-reported bleeding rates and functional limitations were similar in both groups (AJBR: 1.5 vs 1.2 and HAL: 84 vs 84 for those who stopped and continued prophylaxis, respectively), objective assessment of joint status showed increased arthropathy after 10 years of on-demand treatment in patients who stopped prophylaxis compared with those who continued (HJHS: 23 vs. 14 and Pettersson: 16 vs 5, respectively; P< 0.01). These results support continuation of long-term prophylaxis in adults and demonstrate the need for objective monitoring of joint status.Trial registration: Dutch Trial Registry number 3098; UTN U1111–1121–7069.

The Effect of Low-Dose Inhaled Budesonide on Pc20Amp Levels in Patients with Mild Asthma: A 3-Month Follow-Up Study

2009 ◽  
Vol 46 (3) ◽  
pp. 259-264
Author(s):  
Nurdan Kokturk ◽  
Sema Mullaoglu ◽  
Ezgi Ozyilmaz ◽  
Haluk Turktas
Keyword(s):  
Low Dose ◽  
Mild Asthma ◽  
Follow Up Study ◽  
Inhaled Budesonide
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