ENT and mucopolysaccharidoses

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mucopolysaccharidosis 10 endocrinologydiseases

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mucopolysaccharidosis 1795 visual disorders.BackgroundWhy is the paediatric otolaryngologist involved and what is their role in mucopolysaccharidosis ?Ear, nose, and throat (ENT) manifestations in mucopolysaccharidosis (MPS) are due to the accumulation
mucopolysaccharidosis 1863 involved and what is their role in mucopolysaccharidosis?Ear, nose, and throat (ENT) manifestations in mucopolysaccharidosis (MPS) are due to the accumulation of glycosaminoglycans (GAGs) in the head and neck region. The ENT
mucopolysaccharidosis 10184 II (3)MPS IV (6)MPS VI (3)MPS VII (4)UAO (50–90%)T&A (67%)Supraglottic narrowing (92%)OSA (89%)MPS mucopolysaccharidosis , OSA obstructive sleep apnoea, RI respiratory infection, T&A adenoid and/or tonsillar hypertrophy, UAO
mucopolysaccharidosis 10592 diseaseI+++++++++II++++++++IIIMinimalMinimalMinimalIV++++++VI++++++++VII++++++++Data from Rapoport et al. [[40]]MPS mucopolysaccharidosis Table 3Obstructive sleep apnoea (OSA) distribution and severity in each type of MPSNo. of patientsMPS
mucopolysaccharidosis 11624 [[43]]120MPS II48%NALeighton, 2001 [[13]]26MPS I (10)MPS II (6)MPS III (4)MPS IV (4)MPS VI (2)92%NAMPS mucopolysaccharidosis , NA not availableWhat is the possible management for respiratory disorders in MPS?Currently, treatment
mucopolysaccharidosis 14301 2009 [[29]]27MPS IH (8)MPS IH/S (3)MPS II (5)MPS IIIB (1)MPS VI (1)T&A (70.37%)A adenoidectomy, MPS mucopolysaccharidosis , T tonsillectomy, T&A adenotonsillectomyWhen local airway procedures are no longer adequate, or when
mucopolysaccharidosis 17448 (85.7%)Bredenkamp, 1992 [[38]]45MPS IH (13)MPS II (7)MPS III (12)MPS IV (6)MPS VI (5)MPS VII (2)16%MPS mucopolysaccharidosis Fig. 4Stomal narrowingIs hearing loss an important symptom for the diagnosis of MPS?Hearing loss is almost
mucopolysaccharidosis 19013 MPSSign/symptomMPS IMPS IIMPS IIIMPS IVMPS VIMPS VIIHearing loss++++++++++Recurrent ear infections++++++++++MPS mucopolysaccharidosis ++ exhibited by majority of patients with diagnosis, + exhibited by some patients with diagnosisTable
mucopolysaccharidosis 19744 severe87MPS IVType BType BMixedMixedModerateModerate92MPS IIIType BType BMixedMixedSevereProfoundMPS mucopolysaccharidosis Are these signs typical of early-onset MPS?Early recognition of MPS requires careful attention to the
mucopolysaccharidosis 20792 watery diarrhoeaJoint stiffnessDevelopmental delay and/or speech delay (in severe phenotype only)MPS mucopolysaccharidosis What is the therapeutic treatment for these audiological-otologic findings?Sensorineural or mixed conductive

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