Fat Embolism Syndrome in Duchenne Muscular Dystrophy Patients: Early Recognition and Aggressive Therapy

Murphy, Lee; Yabrodi, Mouhammad; Lutfi, Riad

doi:10.1155/2018/3686470

Cited by 5 publications

(2 citation statements)

References 13 publications

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“…Symptoms of central nervous system involvement may be subtle or misinterpreted as agitation or panic. [60][61][62] Fat embolism should always be considered as differential diagnosis in a patient with DMD who develops acute respiratory symptoms and rapid deterioration after trauma.…”

Section: Emergency Care Considerationsmentioning

confidence: 99%

Development of respiratory care guidelines for Duchenne muscular dystrophy in the UK: key recommendations for clinical practice

Childs,

Turner,

Astin

et al. 2023

Thorax

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Significant inconsistencies in respiratory care provision for Duchenne muscular dystrophy (DMD) are reported across different specialist neuromuscular centres in the UK. The absence of robust clinical evidence and expert consensus is a barrier to the implementation of care recommendations in public healthcare systems as is the need to increase awareness of key aspects of care for those living with DMD. Here, we provide evidenced-based and/or consensus-based best practice for the respiratory care of children and adults living with DMD in the UK, both as part of routine care and in an emergency.MethodologyInitiated by an expert working group of UK-based respiratory physicians (including British Thoracic Society (BTS) representatives), neuromuscular clinicians, physiotherapist and patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK respiratory teams and neuromuscular services, consensus was achieved on these best practice recommendations for respiratory care in DMD.ResultThe resulting recommendations are presented in the form of a flow chart for assessment and monitoring, with additional guidance and a separate chart setting out key considerations for emergency management. The recommendations have been endorsed by the BTS.ConclusionsThese guidelines provide practical, reasoned recommendations for all those managing day-to-day and acute respiratory care in children and adults with DMD. The hope is that this will support patients and healthcare professionals in accessing high standards of care across the UK.

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Section: Emergency Care Considerationsmentioning

confidence: 99%

Development of respiratory care guidelines for Duchenne muscular dystrophy in the UK: key recommendations for clinical practice

Childs,

Turner,

Astin

et al. 2023

Thorax

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“…Long bone fracture prevention is important because of the risk of fat embolism syndrome (FES), a rare life-threatening complication so far described in paediatric DMD patients. Sudden onset of breathlessness, cyanosis, rash, and confusion occurring soon after long bone fracture should raise suspicion of FES which requires urgent critical care [ 93 ].…”

Section: Nutrition and Gi Complicationsmentioning

confidence: 99%

Adult North Star Network (ANSN): Consensus Guideline For The Standard Of Care Of Adults With Duchenne Muscular Dystrophy

Quinlivan

Messer

Murphy

et al. 2021

JND

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There are growing numbers of adults with Duchenne Muscular Dystrophy living well into their fourth decade. These patients have complex medical needs that to date have not been addressed in the International standards of care. We sought to create a consensus based standard of care through a series of multi-disciplinary workshops with specialists from a wide range of clinical areas: Neurology, Cardiology, Respiratory Medicine, Gastroenterology, Endocrinology, Palliative Care Medicine, Rehabilitation, Renal, Anaesthetics and Clinical Psychology. Detailed reports of evidence reviewed and the consensus building process were produced following each workshop and condensed into this final document which was approved by all members of the Adult North Star Network including service users. The aim of this document is to provide a framework to improve clinical services and multi-disciplinary care for adults living with Duchenne Muscular Dystrophy.

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Neurologic Complications of Fat Embolism Syndrome

Morales-Vidal

2019

Curr Neurol Neurosci Rep

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Fat Embolism Syndrome in Duchenne Muscular Dystrophy Patients: Early Recognition and Aggressive Therapy

Cited by 5 publications

References 13 publications

Development of respiratory care guidelines for Duchenne muscular dystrophy in the UK: key recommendations for clinical practice

Development of respiratory care guidelines for Duchenne muscular dystrophy in the UK: key recommendations for clinical practice

Adult North Star Network (ANSN): Consensus Guideline For The Standard Of Care Of Adults With Duchenne Muscular Dystrophy

Neurologic Complications of Fat Embolism Syndrome

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