Hemophilia
Conditions
Keywords
Haemophilia, Arthropathy, Bilateral deficit phenomenon, Proprioception
Brief summary
Trial to assess the bilateral deficit phenomenon during dynamic plantar flexion task in patients with haemophilic arthropathy Describe the differences in terms of the physical variables (muscular strength, range of motion and proprioception) in patients with hemophilia who have conducted a home treatment with a digital tool. Bookmark the relationship between clinical history of joint bleeds and clinical manifestations in standing and walking.
Detailed description
* Assess the bilateral deficit phenomenon during dynamic plantar flexion task in patients with haemophilic arthropathy. * Indicate the independent variables that influence the development of strength and contractile activity, with or without previous joint bleeds in haemophilia patients with minors. * Observe the descriptive variables people influence the standing, the incorporation from sitting and jumping.
Interventions
Assessment of bilateral deficit phenomenon during dynamic plantar flexion task with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA)
Sponsors
Universidad Católica San Antonio de Murcia
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
MALE
Age
18 Years to 50 Years
Healthy volunteers
No
Inclusion criteria
* Patients diagnosed with hemophilia A and B. * With or without joint involvement clinically diagnosed, and with or without a previous history of hemarthrosis joint load legs. * In prophylactic regimen with FVIII / FIX concentrates . * Residents in the Autonomous Community of the Region of Murcia.
Exclusion criteria
* Patients without walking ability. * Patients diagnosed with other congenital coagulopathy (von Willebrand disease, etc..). * Patients with neurological or cognitive impairments that prevent understanding the questionnaires and physical tests. * Patients whose parents or guardians have not signed the informed consent document.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Assessment of Maximal Velocity of Movement | Screening visit | Assessment of maximal velocity of movement with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA) |
| Assessment of Bilateral Index of Rate of Development During the Preparation Phase | Screening visit | Assessment of Bilateral index of rate of development during the preparation phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA) |
| Assessment of Bilateral Index of Rate of Development During the Acceleration Phase | Screening visit | Assessment of bilateral index of rate of development during the acceleration phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA) |
| Assessment of Maximal Peak Force | Screening visit | Assessment of maximal peak force with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA) |
| Assessment of Rate of Development During the Preparation Phase | Screening visit | Assessment of rate of development during the preparation phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA) |
| Change of Joint Condition Based on Clinical Assessment | Screening visit | Spanish version of the Haemophilia Joint Health Score 2.1 (HJHS). Additive scale that assesses from 0 to 24 points joint status of patients with haemophilia (0: no joint damage; 24: maximum joint damage). The variables studied in this scale are: Swelling (range 0-3); Duration of swelling (range 0-1); Muscle atrophy (range 0-2); Crepitant in motion (range 0-2); Loss of Flexion (range 0-3); Loss of extension (range 0-3); Joint pain (range 0-2): Strength (range 0-4); Gait (range 0-4) |
| Assessment of Rate of Development During the Acceleration Phase | Screening visit | Assessment of rate of development during the acceleration phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA) |
| Assessment of Bilateral Index of Maximal Peak Force | Screening visit | Assessment of bilateral index of maximal peak force with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | Screening visit | Patients fill out a registration key clinical data (type, severity of hemophilia, hemarthrosis in the previous month and current drug therapy). |
| Joint Bleeding Before the Assessment | Screening visit | — |
| Height of the Patients With Haemophilia | Screening visit | The height of patients was calculated using a TANITA equipment (TBF-300WA model, Tanita Corporation of America, Inc., Illinois, USA). |
| Body Mass Index of Patients With Haemophilia | Screening visit | The body mass index of patients was calculated using a TANITA equipment (TBF-300WA model, Tanita Corporation of America, Inc., Illinois, USA). |
| Age of Patients With Haemophilia | Screening visit | — |
| Weight of the Patients With Haemophilia | Screening visit | The weight were collected using a TANITA equipment (TBF-300WA model, Tanita Corporation of America, Inc., Illinois, USA) |
Countries
Spain
Participant flow
Recruitment details
Patients with haemophilia
Participants by arm
| Arm | Count |
|---|---|
| Patients With Haemophilia Assessment of bilateral deficit phenomenon during dynamic plantar flexion task
Patients with haemophilia: Assessment of bilateral deficit phenomenon during dynamic plantar flexion task with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA) | 17 |
| Total | 17 |
Baseline characteristics
| Characteristic | Patients With Haemophilia |
|---|---|
| Age, Continuous | 32.47 years STANDARD_DEVIATION 7.82 |
| Region of Enrollment Spain | 17 participants |
| Sex: Female, Male Female | 0 Participants |
| Sex: Female, Male Male | 17 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | — / — |
| other Total, other adverse events | 0 / 0 |
| serious Total, serious adverse events | 0 / 0 |
Outcome results
Primary
Assessment of Bilateral Index of Maximal Peak Force
Assessment of bilateral index of maximal peak force with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA).
Time frame: Screening visit
Population: Bilateral rates was calculated during the preparation phase and during the acceleration phase to express relative difference in strength between bilateral and unilateral conditions. Based on information in the Howard and Enoka (1991), the calculation detail for the Bilateral Index is \[100x (bilateral) / (right unilateral + left unilateral)\] - 100
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Assessment of Bilateral Index of Maximal Peak Force | -40.86 percentage | Standard Deviation 4.24 |
Primary
Assessment of Bilateral Index of Rate of Development During the Acceleration Phase
Assessment of bilateral index of rate of development during the acceleration phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA)
Time frame: Screening visit
Population: Bilateral rates was calculated during the acceleration phase to express relative difference in strength between bilateral and unilateral conditions (calculated in percentages). Based on information in the Howard and Enoka (1991), the calculation detail for the Bilateral Index is \[100x (bilateral) / (right unilateral + left unilateral)\] - 100
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Assessment of Bilateral Index of Rate of Development During the Acceleration Phase | 20.05 percentage | Standard Deviation 38.2 |
Primary
Assessment of Bilateral Index of Rate of Development During the Preparation Phase
Assessment of Bilateral index of rate of development during the preparation phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA)
Time frame: Screening visit
Population: Bilateral rates was calculated during the preparation phase to express relative difference in strength between bilateral and unilateral conditions (calculated in percentages). Based on information in the Howard and Enoka (1991), the calculation detail for the Bilateral Index is \[100x (bilateral) / (right unilateral + left unilateral)\] - 100
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Assessment of Bilateral Index of Rate of Development During the Preparation Phase | 31.34 percentage | Standard Deviation 56.42 |
Primary
Assessment of Maximal Peak Force
Assessment of maximal peak force with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA)
Time frame: Screening visit
Population: The maximum peak force (MPF) is defined as the maximum achieved in the force-time curve during the dynamic test plantar flexion for both bilateral conditions as right and left unilateral, normalized to body weight of the participants (N / kg value)
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Assessment of Maximal Peak Force | 15.67 N/kg | Standard Deviation 1.36 |
Primary
Assessment of Maximal Velocity of Movement
Assessment of maximal velocity of movement with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA)
Time frame: Screening visit
Population: The maximum speed (Vmax) was calculated as the maximum value of the first integral of the force-time curve for bilateral contractions, left-sided and right-sided
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Assessment of Maximal Velocity of Movement | 0.48 m/s | Standard Deviation 0.11 |
Primary
Assessment of Rate of Development During the Acceleration Phase
Assessment of rate of development during the acceleration phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA)
Time frame: Screening visit
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Assessment of Rate of Development During the Acceleration Phase | 8945.25 N/s | Standard Deviation 2807.85 |
Primary
Assessment of Rate of Development During the Preparation Phase
Assessment of rate of development during the preparation phase with a force platform (Kistler 9286 BA model, Kistler Instruments, Amherst, NY, USA)
Time frame: Screening visit
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Assessment of Rate of Development During the Preparation Phase | 6190.86 N/s | Standard Deviation 2818.36 |
Primary
Change of Joint Condition Based on Clinical Assessment
Spanish version of the Haemophilia Joint Health Score 2.1 (HJHS). Additive scale that assesses from 0 to 24 points joint status of patients with haemophilia (0: no joint damage; 24: maximum joint damage). The variables studied in this scale are: Swelling (range 0-3); Duration of swelling (range 0-1); Muscle atrophy (range 0-2); Crepitant in motion (range 0-2); Loss of Flexion (range 0-3); Loss of extension (range 0-3); Joint pain (range 0-2): Strength (range 0-4); Gait (range 0-4)
Time frame: Screening visit
| Arm | Measure | Group | Value (MEAN) | Dispersion |
|---|---|---|---|---|
| Patients With Haemophilia | Change of Joint Condition Based on Clinical Assessment | Right knee | 4.88 Units on a scale (range 0-24) | Standard Deviation 4.076 |
| Patients With Haemophilia | Change of Joint Condition Based on Clinical Assessment | Left knee | 3.00 Units on a scale (range 0-24) | Standard Deviation 2.398 |
| Patients With Haemophilia | Change of Joint Condition Based on Clinical Assessment | Right ankle | 5.82 Units on a scale (range 0-24) | Standard Deviation 3.575 |
| Patients With Haemophilia | Change of Joint Condition Based on Clinical Assessment | Left ankle | 6.35 Units on a scale (range 0-24) | Standard Deviation 4.527 |
Secondary
Age of Patients With Haemophilia
Time frame: Screening visit
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Age of Patients With Haemophilia | 32.47 years | Standard Deviation 7.82 |
Secondary
Body Mass Index of Patients With Haemophilia
The body mass index of patients was calculated using a TANITA equipment (TBF-300WA model, Tanita Corporation of America, Inc., Illinois, USA).
Time frame: Screening visit
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Body Mass Index of Patients With Haemophilia | 23.36 kg/m2 | Standard Deviation 4.48 |
Secondary
Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand)
Patients fill out a registration key clinical data (type, severity of hemophilia, hemarthrosis in the previous month and current drug therapy).
Time frame: Screening visit
| Arm | Measure | Group | Value (NUMBER) |
|---|---|---|---|
| Patients With Haemophilia | Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | Haemophilia A | 15 participants |
| Patients With Haemophilia | Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | Haemophilia B | 2 participants |
| Patients With Haemophilia | Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | Severe haemophilia | 7 participants |
| Patients With Haemophilia | Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | Moderate haemophilia | 5 participants |
| Patients With Haemophilia | Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | Mild haemophilia | 5 participants |
| Patients With Haemophilia | Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | Prophylaxis treatment | 8 participants |
| Patients With Haemophilia | Diagnosis, Severity of Hemophilia, Treatment (Prophylactic or on Demand) | On demand treatment | 9 participants |
Secondary
Height of the Patients With Haemophilia
The height of patients was calculated using a TANITA equipment (TBF-300WA model, Tanita Corporation of America, Inc., Illinois, USA).
Time frame: Screening visit
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Height of the Patients With Haemophilia | 176.53 cm | Standard Deviation 6.82 |
Secondary
Joint Bleeding Before the Assessment
Time frame: Screening visit
| Arm | Measure | Value (NUMBER) |
|---|---|---|
| Patients With Haemophilia | Joint Bleeding Before the Assessment | 7 participants |
Secondary
Weight of the Patients With Haemophilia
The weight were collected using a TANITA equipment (TBF-300WA model, Tanita Corporation of America, Inc., Illinois, USA)
Time frame: Screening visit
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Patients With Haemophilia | Weight of the Patients With Haemophilia | 73.48 Kg | Standard Deviation 15.43 |