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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
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Management recommendations for complications of Krabbe disease[1]

Management recommendations for complications of Krabbe disease[1]
System/concern Manifestation Treatment
Gastrointestinal Vomiting and gastroesophageal reflux disease (GERD)
  • Maintainence of upright positioning during and after feeding
  • Consideration of proton pump inhibitors in those age >1 year
  • Nissen fundoplication with gastrostomy tube (G-tube) placement
Dysphagia
  • Modifying texture and thickness of foods using commercial thickening agents can help with swallowing difficulties
  • Swallowing ability may be improved by providing tiny tastes of food or juice several times per day in nonfeeders
  • Consideration of nasogastric tube or gastric tube placement
Constipation
  • Maintain appropriate fluid intake
  • Consideration of osmotic laxatives and/or use of stimulant medication in those with refractory constipation
Neurologic Spasticity
  • Baclofen and clonazepam may improve global spasticity
  • Botox injections at specific sites may be considered; spasticity typically decreases for 3 to 5 months after each injection
Neuropathic pain; seizures
  • Standard antipileptic medications; monotherapy is preferred, if possible
  • Gabapentin may decrease neuropathic pain and can be used for control of seizures in some
Musculoskeletal Contractures
  • Positioning devices (wedges, rolls, and cushions) to decrease spasticity and prevent contractures
  • Physical therapy
Respiratory Excessive airway secretions
  • Chest physiotherapy
  • Postural drainage
  • Suctioning device
Genitourinary Urinary tract infections
  • Bladder massage (Crede maneuver) to encourage complete bladder emptying
  • Intermittent catheterization
Eyes Delayed pupillary response, difficulty with upward gaze and palpebral weakness  
Corneal ulcers
  • Dark glasses to help reduce photophobia
  • Eye lubricants or protective ointments
Dental Delayed dentition  
Reference:
  1. Orsini JJ, Escolar ML, Wasserstein MP, Caggana M. Krabbe disease. In: GeneReviews [Internet], Adam MP, Ardinger HH, Pagon RA, et al. (Eds), University of Washington, Seattle 2018. www.ncbi.nlm.nih.gov/books/NBK1238/ (Accessed on July 09, 2020).
From: Escolar ML, West T, Dallavecchia A, et al. Clinical management of Krabbe disease. J Neurosci Res 2016; 94(11):1118-25. https://onlinelibrary.wiley.com/doi/abs/10.1002/jnr.23891. Copyright © 2016 Wiley Periodicals, Inc. Modified with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).
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