Airway Management in Children With Mucopolysaccharidoses

Yeung, Andrea H.; Cowan, Morton J.; Horn, Biljana; Rosbe, Kristina W.

doi:10.1001/archoto.2008.515

Cited by 71 publications

(77 citation statements)

References 26 publications

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“…É bem conhecida a associação entre MPS e a ocorrência de hipertrofia adenotonsilar, língua volumosa e limitação da abertura bucal/anomalias mandibulares 5,[26][27][28][29][30] . Embora não tenha sido possível a avaliação da prevalência de hipertrofia adenotonsilar, os demais achados foram frequentes na amostra estudada.…”

Section: Alteração N (%)* N (%) N (%) Punclassified

“…Embora não tenha sido possível a avaliação da prevalência de hipertrofia adenotonsilar, os demais achados foram frequentes na amostra estudada. Eles trazem consigo um risco aumentado, para os pacientes, de ocorrência de respiração oral e de apneia obstrutiva e outras alterações do sono, além de uma maior necessidade de adenoidectomias e amigdalectomias [26][27][28][29][30] , também presentes nesta amostra. Contudo, é importante frisar que a maioria dos pacientes avaliados apresentou abertura normal de mandíbula.…”

Section: Alteração N (%)* N (%) N (%) Punclassified

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Evaluation of orofacial motricity in patients with mucopolysaccharidosis: a cross-sectional study

Turra

Schwartz

2009

J Pediatr (Rio J)

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Objective: To characterize the stomatognathic system and stomatognathic functions in patients with mucopolysaccharidosis. Methods:Cross-sectional and observational study of patients with mucopolysaccharidosis seen at the outpatient clinic at the Medical Genetics Service of Hospital de Clínicas de Porto Alegre. The inclusion criteria were the existence of a biochemical or molecular diagnosis of any type of mucopolysaccharidosis and the agreement to participate in the study by signing an informed consent form. Seventy-eight patients were evaluated through phonoaudiological anamnesis and physical exam.Results: Alterations in at least one item of each structure of the stomatognathic system or stomatognathic function were found in all patients who allowed evaluation of both items on physical examination (n = 76/78). The most frequently compromised structures and functions were respectively the dental arch and the tongue, swallowing and mastication. The only statistically significant difference found between types of mucopolysaccharidosis involved the habitual position of the tongue between the teeth (most frequent in mucopolysaccharidosis VI). Among patients with mucopolysaccharidosis I, II or VI who underwent enzyme replacement therapy or not, there was statistically significant difference in oral breathing mode (more frequent in the group without enzyme replacement therapy). Conclusions:Alterations in stomatognathic systems and functions are prevalent among individuals with mucopolysaccharidosis, even if enzyme replacement therapy is administered. Such finding suggests that speech therapy follow-up plays a major role in the treatment plan of this group of diseases; this hypothesis should be confirmed by additional studies. Resultados: Alterações em pelo menos um item de cada estrutura do sistema estomatognático ou função estomatognática foram encontradas em todos os pacientes que permitiram a avaliação de ambos estes itens do exame físico (n = 76/78). As estruturas e funções mais frequentemente comprometidas foram, respectivamente, a arcada dentária e a língua e a deglutição e a mastigação. A única diferença estatisticamente significativa encontrada entre os tipos de mucopolissacaridose envolveu a posição habitual da língua entre os dentes (mais frequente na mucopolissacaridose VI). Entre os pacientes com mucopolissacaridose I, II ou VI submetidos ou não à terapia de reposição enzimática, foi encontrada diferença estatisticamente significativa no modo oral de respiração (mais frequente no grupo sem terapia de reposição enzimática). J Pediatr (Rio J) Conclusões:Alterações dos sistemas e funções estomatognáticas são prevalentes em indivíduos com mucopolissacaridose, mesmo na vigência de terapia de reposição enzimática. Tal achado sugere que o acompanhamento fonoterápico tenha papel importante no plano de tratamento desse grupo de doenças, hipótese que deve ser confirmada por estudos adicionais. Fonte financiadora: Rede MPS Brasil, CAPES. J Pediatr (Rio J)Não foram declarados conflitos de interesse associados ...

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Evaluation of orofacial motricity in patients with mucopolysaccharidosis: a cross-sectional study

Turra

Schwartz

2009

J Pediatr (Rio J)

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“…In severe cases, such operations are impossible due to potential complications arising of anesthesia or surgical intervention [15]. Severe sleep respiratory disorders may be treated with continuous positive airway pressure (CPAP), when high-pressure air is pumped in the mask; this prevents airway collapse during respiration, improves sleep quality, reduces dyspnea and normalizes blood gas concentration [16,17]. …”

Section: Resultsmentioning

confidence: 99%

Obstructive Sleep Apnea Syndrome in Children With Type Ii Mucopolysaccharidosis (Hunter Syndrome)

et al. 2013

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“…Similarly to our study, Yeung et al (2009) used FFL and PSG to assess some patients with different types of MPS. They found that 19 of their 27 patients presented with upper airway obstruction.…”

Section: Discussionmentioning

confidence: 99%

Correlation Between Flexible Fiberoptic Laryngoscopic and Polysomnographic Findings in Patients with Mucopolysaccharidosis Type VI

et al. 2015

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This study aimed to compare flexible fiberoptic laryngoscopy (FFL) and polysomnography (PSG) findings in patients with mucopolysaccharidosis (MPS) type VI and to describe upper airway anatomical findings and abnormal PSG results in these patients. In this cross-sectional study, all MPS VI patients followed up at the genetic division of a hospital in southern Brazil were included. Overnight PSG was performed, and the results were classified as normal or mildly, moderately, or severely abnormal. FFL was performed between 7 days before and 7 days after PSG. FFL findings were classified as (1) no obstruction, (2) mild obstruction, (3) moderate obstruction, or (4) severe obstruction of the airways, using the highest score obtained in all the regions.Eleven patients with MPS VI were included. FFL detected severe airway obstruction in eight (72.7%) patients, moderate obstruction in two (18.2%), and mild obstruction in one (9.1%). PSG revealed obstructive sleep apnea syndrome (OSAS) in nine (81.8%) patients. Among these, mild OSAS was observed in five (45.5%) patients, moderate OSAS in three (27.2%), and severe OSAS in one (9.1%). Moderate to severe hypertrophy of the nasal turbinates was found in 81.8% of the patients, and 64% had severe infiltration in the supraglottic region. There was no association between FFL and PSG findings (p ¼ 0.454; k ¼ À0.09; 95%CI ¼ À0.34 to 0.17), indicating no agreement between the two methods. In the present study, all patients with MPS showed some degree of airway obstruction. We suggest performing PSG in MPS patients to determine disease severity.

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Airway Management in Children With Mucopolysaccharidoses

Cited by 71 publications

References 26 publications

Evaluation of orofacial motricity in patients with mucopolysaccharidosis: a cross-sectional study

Evaluation of orofacial motricity in patients with mucopolysaccharidosis: a cross-sectional study

Obstructive Sleep Apnea Syndrome in Children With Type Ii Mucopolysaccharidosis (Hunter Syndrome)

Correlation Between Flexible Fiberoptic Laryngoscopic and Polysomnographic Findings in Patients with Mucopolysaccharidosis Type VI

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