Search results for "Mucopolysaccharidosis IV"

showing 2 items of 12 documents

Relationship Between Patient-Reported Outcomes and Clinical Outcomes in Patients With Morquio A Syndrome

2019

Abstract This cross-sectional analysis assessed the correlation between patient-reported outcomes (PROs) and clinical outcomes in 24 German patients with Morquio A. Clinical outcomes included 6-minute walk test (6MWT), 3-minute stair climb (3MSC) test, and joint range of motion as measures for endurance/mobility, forced vital capacity (FVC) and maximum voluntary ventilation (MVV) as measures for respiratory function, and height as an important manifestation. The PROs included the EuroQoL (EQ) 5D-5L (EQ5D-5L), to measure health-related QoL (HRQoL), and patients’ rating of their ability to walk, climb, or breathe. In adults, endurance and pulmonary function measures and height showed strong a…

Vital capacitymedicine.medical_specialtyEndocrinology Diabetes and Metabolismmaximal voluntary ventilationPulmonary function testingFEV1/FVC ratioMaximal Voluntary VentilationPhysical medicine and rehabilitationQuality of lifepatient’s perceptionMorquio A6MWTmedicineRespiratory functionGenetics (clinical)lcsh:R5-920EQ5D-5Lbusiness.industryrespiratory function testsFVCmobilityhumanitiesTest (assessment)quality of lifepatient-reported outcomesPediatrics Perinatology and Child HealthPhysical therapyRange of motionbusiness3MSC testlcsh:Medicine (General)physical endurancehuman activitiesmucopolysaccharidosis IVJournal of Inborn Errors of Metabolism and Screening
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The Burden Endured by Caregivers of Patients With Morquio A Syndrome: Results From an International Patient-Reported Outcomes Survey

2019

Abstract This international survey performed by direct personal interview or mail evaluated the global burden among primary caregivers of patients with Morquio A syndrome. Collected outcomes included self-reported time spent on caregiving, proportion of daily activities (from the Mucopolysaccharidosis Health Assessment Questionnaire) requiring caregiver assistance, and how the patient’s age and wheelchair use affect these. In addition, the impact of caregiving on the caregivers’ relationship with family and friends, physical and mental health, and employment status and income was evaluated. Caregiver burden increased with disease progression. Adult patients always using a wheelchair require…

medicine.medical_specialtycaregiversbusiness.industryEndocrinology Diabetes and MetabolismeducationInternational surveyMorquio A syndromewheelchairsdisease burdenQuality of life (healthcare)quality of lifePediatrics Perinatology and Child HealthMucopolysaccharidosis IVMedicinebusinessPsychiatryPersonal interviewMorquio A syndromeGenetics (clinical)Disease burdenmucopolysaccharidosis IV
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