A case of inherited metabolic disorder with airway difficulties in intensive care- challenges faced
DOI:
https://doi.org/10.22270/ijmspr.v10i2.94Keywords:
hurlers sybdrome, glycosoaminoglycans, pnuemonia, ventilation, airway, intensive care, cellular dysfunction, clinical abnormalitiesAbstract
Uncommon disease like MPS imposes great challenges to intensivists in case of emergency due to their disease nature which involves GAG accumulation leading to cellular dysfunction and various clinical abnormalities. Airway management in Pediatric patients is extremely demanding even with expert hands. So, this case report discusses mainly the disease progress, and anticipated anesthetic challenges with its skillful handling strategy that has to be contemplated in case of emergency situation.
Keywords: intensive care, cellular dysfunction, clinical abnormalities
References
Khan SA, Peracha H, Ballhausen D, Wiesbauer A, Rohrbach M, Gautschi M, e al.. Epidemiology of mucopolysaccharidoses. Mol Genet Metab. 2017;121:227-40. https://doi.org/10.1016/j.ymgme.2017.05.016 PMid:28595941 PMCid:PMC5653283
Muenzer J Wraith JE Clarke LA International International Consensus Panel on Management Treatment of Mucopolysaccharidosis I . Mucopolysaccharidosis I: management and treatment guidelines. Pediatrics. 2009;123:19-29. https://doi.org/10.1542/peds.2008-0416 PMid:19117856
Martins AM, Dualibi AP, Norato D, Takata ET, Santos ES, Valadares ER, et al.. Guidelines forthe management of mucopolysaccharidosis type I. J Pediatr. 2009;155 (4 Suppl.):S32-46. https://doi.org/10.1016/j.jpeds.2009.07.005 PMid:19765409
Muhlebach S.M., Wooten W., Muenzer J. Respiratory manifestation in mucopolysaccharidoses. Pediatric Respir. Rev. 2011;12:133-138. doi: https://doi.org/10.1016/j.prrv.2010.10.005 PMid:21458742
Wooten 3rd W.I., Muenzer J., Vaughn B.V., Muhlebach M. Relationship of sleep to pulmonary function in mucopolysaccharidosis II. J. Pediatrics
Hendriksz CJ, Al-Jawad M, Berger KI, Hawley SM, Lawrence R, Mc Ardle C, Summers CG, Wright E, Braunlin E. Clinical overview and treatment options for non-skeletal manifestations of mucopolysaccharidosis type IVA. J Inherit Metab Dis. 2013 Mar;36(2):309-22. https://doi.org/10.1007/s10545-012-9459-0 PMid:22358740 PMCid:PMC3590399
Moretto A, Bosatra MG, Marchesini L, Tesoro S. Anesthesiological risks in mucopolysaccharidoses. Ital J Pediatr. 2018 Nov 16;44(Suppl 2):116. https://doi.org/10.1186/s13052-018-0554-1 PMid:30442160 PMCid:PMC6238251
Published
How to Cite
Issue
Section
Copyright (c) 2024 T Krishna Prasad, Priyadharsini K Soundarya , K Shiek Abdulla , Sri Krishna Varun

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

.