Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data

Gocyk, Wojciech; Warmus, Janusz; Olechnowicz, H; Bik-Multanowski, Mirosław; Pawliński, Łukasz; Kieć‐Wilk, Beata

doi:10.1186/s12890-020-1143-9

Cited by 4 publications

(1 citation statement)

References 14 publications

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“…Antón-Pacheco et al used stenting treatment in one patient with MPS type IVA [ 35 ], but details of the stenting material and long-term outcomes of this case were not presented in the study report. Metallic balloon-expandable stents have been applied in MPS type II and type VI patients [ 36 , 37 , 38 , 39 ]. However, most patients had only short-term benefits from this type of stent, and their responses were complicated by considerable granulation formation due to mucosal inflammation and GAGs deposition [ 37 , 38 ].…”

Section: Discussionmentioning

confidence: 99%

Endoscopic and Image Analysis of the Airway in Patients with Mucopolysaccharidosis Type IVA

Lee

Lin

et al. 2023

JPM

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Mucopolysaccharidosis (MPS) is a hereditary disorder arising from lysosomal enzymes deficiency, with glycosaminoglycans (GAGs) storage in connective tissues and bones, which may compromise the airway. This retrospective study evaluated patients with MPS type IVA with airway obstruction detected via endoscopy and imaging modalities and the effects of surgical interventions based on symptoms. The data of 15 MPS type IVA patients (10 males, 5 females, mean age 17.8 years) were reviewed in detail. Fiberoptic bronchoscopy (FB) was used to distinguish adenotonsillar hypertrophy, prolapsed soft palate, secondary laryngomalacia, vocal cord granulation, cricoid thickness, tracheal stenosis, shape of tracheal lumen, nodular deposition, tracheal kinking, tracheomalacia with rigid tracheal wall, and bronchial collapse. Computed tomography (CT) helped to measure the deformed sternal angle, the cross-sectional area of the trachea, and its narrowest/widest ratio (NW ratio), while angiography with 3D reconstruction delineated tracheal torsion, kinking, or framework damage and external vascular compression of the trachea. The NW ratio correlated negatively with age (p < 0.01), showing that airway obstruction progressed gradually. Various types of airway surgery were performed to correct the respiratory dysfunction. MPS type IVA challenges the management of multifactorial airway obstruction. Preoperative airway evaluation with both FB and CT is strongly suggested to assess both intraluminal and extraluminal factors causing airway obstruction.

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Section: Discussionmentioning

confidence: 99%

Endoscopic and Image Analysis of the Airway in Patients with Mucopolysaccharidosis Type IVA

Lee

Lin

et al. 2023

JPM

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Airway collapse hinders recovery in bronchoscopy therapy for postintubation tracheal stenosis patients

Yang,

Li,

Zhou

et al. 2024

Eur Arch Otorhinolaryngol

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Background Expiratory central airway collapse (ECAC) following postintubation airway stenosis (PITS) is a rare phenomenon. The impact of airway malacia and collapse on the prognosis and the success rate of bronchoscopic interventional therapy in patients with PITS had been inadequately investigated. Objective The aim of this research was to assess the influence of airway malacia and collapse on the efficacy of bronchoscopic interventional therapy in patients with PITS. Design This retrospective analysis examined the medical documentation of individuals diagnosed with PITS who underwent bronchoscopic intervention at the tertiary interventional pulmonology center of Emergency General Hospital from 2014 to 2021. Main outcome measures Data pertaining to preoperative, perioperative, and postoperative stages were documented and subjected to analysis. Results The patients in malacia and collapse group (MC group) exhibited a higher frequency of perioperative complications, including intraoperative hypoxemia, need for reoperation within 24 h, and postoperative intensive care unit admission rate (P < 0.05, respectively). Meanwhile, patients in group MC demonstrated significantly worse postoperative scores (higher mMRC score and lower KPS score) compared to those in pure stenosis group (P < 0.05, respectively), along with higher degrees of stenosis after treatment and a lower success rate of bronchoscopic intervention therapy cured (P < 0.05, respectively). Pearson analysis results showed that these terms were all significantly correlated with the occurrence of airway malacia and collapse in the airway (P < 0.05, respectively). Conclusion The presence of malacia or collapse in patients with PITS was associated with increased perioperative complications following bronchoscopic interventional therapy, and significantly reduced the long-term cure rate compared to patients with pure tracheal stenosis. Trial registration Chinese Clinical Trial Registry on 06/12/2021. Registration number: ChiCTR2100053991.

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Causes of death in mucopolysaccharidoses

Rintz,

Banacki,

Ziemian

et al. 2024

Molecular Genetics and Metabolism

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Case report of endoprosthesis -Y implantation in severe respiratory failure in the MPSII patient; comparison with literature data

Cited by 4 publications

References 14 publications

Endoscopic and Image Analysis of the Airway in Patients with Mucopolysaccharidosis Type IVA

Endoscopic and Image Analysis of the Airway in Patients with Mucopolysaccharidosis Type IVA

Airway collapse hinders recovery in bronchoscopy therapy for postintubation tracheal stenosis patients

Causes of death in mucopolysaccharidoses

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