scholarly journals Airway Abnormalities in Adult Mucopolysaccharidosis and Development of Salford Mucopolysaccharidosis Airway Score

2021 ◽  
Vol 10 (15) ◽  
pp. 3275
Author(s):  
Chaitanya Gadepalli ◽  
Karolina M. Stepien ◽  
Reena Sharma ◽  
Ana Jovanovic ◽  
Govind Tol ◽  
...  

(1) Background: Mucopolysaccharidoses (MPS) are a heterogeneous group of lysosomal storage disorders caused by the absence of enzymes required for degradation of glycosaminoglycans (GAGs). GAGs deposition in tissues leads to progressive airway narrowing and/or tortuosity. Increased longevity of patients has posed newer problems, especially the airway. This study aims to characterise various airway abnormalities in adult MPS from a regional centre and proposes a method to quantify the severity of the airway disease. (2) Methods: Retrospective analysis by case notes review, clinical examination, endoscopy, cross-sectional imaging, 3-dimensional reconstruction, and physiological investigations were used to assess the airway abnormalities. Quantitative assessment of the airway severity was performed a validated questionnaire of 15 parameters to derive Salford Mucopolysaccharidosis Airway Score (SMAS). (3) Results: Thirty-one adult MPS patients (21M/ 9F; median 26.7 years; range 19–42 years) were reviewed. There were 9 MPS I, 12 MPS II, 2 MPS III, 5 MPS IV, 2 MPS VI, and 1 MPS VII. Airway abnormalities in each MPS type are described. Patients scoring more than 35 on SMAS had some form of airway intervention. The area under curve of 0.9 was noted at a score of 25, so SMAS more than 25 may predict a difficult airway and potential to have complications. Pearson’s correlation between SMAS and height, weight, BMI were poor (p < 0.05). (4) Conclusions: Airway abnormalities in adult MPS are varied and complex. Assessment of the airway should be holistic and include multiple parameters. An objective multidimensional score such as SMAS may help to predict and manage difficult airways warranting further investigation and validation.

OTO Open ◽  
2018 ◽  
Vol 2 (1) ◽  
pp. 2473974X1775358 ◽  
Author(s):  
Lee S. McDaniel ◽  
William J. Poynot ◽  
Keith A. Gonthier ◽  
Michael E. Dunham ◽  
and Tyler W. Crosby

Objectives Describe a technique for the description and classification of laryngotracheal stenosis in children using 3-dimensional reconstructions of the airway from computed tomography (CT) scans. Study Design Cross-sectional. Setting Academic tertiary care children’s hospital. Subjects and Methods Three-dimensional models of the subglottic airway lumen were created using CT scans from 54 children undergoing imaging for indications other than airway disease. The base lumen models were deformed in software to simulate subglottic airway segments with 0%, 25%, 50%, and 75% stenoses for each subject. Statistical analysis of the airway geometry was performed using metrics extracted from the lumen centerlines. The centerline analysis was used to develop a system for subglottic stenosis assessment and classification from patient-specific airway imaging. Results The scaled hydraulic diameter gradient metric derived from intersectional changes in the lumen can be used to accurately classify and quantitate subglottic stenosis in the airway based on CT scan imaging. Classification is most accurate in the clinically relevant 25% to 75% range of stenosis. Conclusions Laryngotracheal stenosis is a complex diagnosis requiring an understanding of the airway lumen configuration, anatomical distortions of the airway framework, and alterations of respiratory aerodynamics. Using image-based airway models, we have developed a metric that accurately captures subglottis patency. While not intended to replace endoscopic evaluation and existing staging systems for laryngotracheal stenosis, further development of these techniques will facilitate future studies of upper airway computational fluid dynamics and the clinical evaluation of airway disease.


Urology ◽  
2014 ◽  
Vol 84 (2) ◽  
pp. 268-273 ◽  
Author(s):  
Jonathan L. Silberstein ◽  
Michael M. Maddox ◽  
Phillip Dorsey ◽  
Allison Feibus ◽  
Raju Thomas ◽  
...  

Author(s):  
R.M. Kirberger ◽  
S.L. Fourie

A rostrocaudal (RCd) nasal view was developed in large breed mesaticephalic dogs using a complete, subsequently sectioned, skull and cadaver specimens to optimise the radiographic technique and evaluate normal anatomic features. Gelatin was placed in one nasal passage of the cadaver specimens to mimic the effects of nasal pathology. The latter specimens and 18 clinical cases with suspected nasal disease were evaluated to determine the usefulness of the RCd view compared to standard nasal views. An optimal RCd view was obtained with the dog in dorsal recumbency and the head symmetrically positioned with the hard palate perpendicular to the table using a table top technique with 8 : 1 grid, collimating to the nasal region and centring the primary beam on the philtrum. The dorsolateral aspects of the maxillary bone, the nasal bones, septal sulcus of the vomer, mucosa lined nasal septum and conchae could be seen. A centrodorsal more radiolucent area representing the ethmoid bone region was also visible. Gelatin soft tissue opacification of the nasal passage could be seen more clearly in RCd nasal view than in occlusal dorsoventral view. In clinical cases the RCd view was useful to build up a 3-dimensional image of nasal passage pathology as well as to detect nasal septum and osseous nasal border pathology not visible in other views. This view is particularly useful in cases where cross-sectional imaging modalities are not available or where the nasal investigation is limited by cost considerations.


2002 ◽  
Vol 111 (6) ◽  
pp. 530-536 ◽  
Author(s):  
Ken Ishijima ◽  
Isamu Sando ◽  
Makoto Miura ◽  
Carey D. Balaban ◽  
Kenji Takasaki

The anatomic relationships among the levator veli palatini muscle (LVPM), the tensor veli palatini muscle (TVPM), and the eustachian tube (ET) cartilage were investigated by computer-aided 3-dimensional reconstruction and measurement methods. The study used 13 normal temporal bone–ET specimens obtained from 13 individuals (range of age at death, 3 months to 88 years). This study revealed several anatomic features of the anterior cartilaginous portion of the ET First, the LVPM is always located inferolateral to the inferior margin of the medial lamina (ML) of the ET cartilage. Second, the LVPM has a large cross-sectional area throughout the extent of the anterior cartilaginous portion of the ET. Third, although the LVPM lies close to the ML of the ET cartilage (0.44 ± 0.16 mm in children and 1.02 ± 0.58 mm in adults), there is no region of attachment. Finally, the TVPM is not attached to the lateral lamina (LL) of the ET cartilage of the anterior quarter of the cartilaginous portion. Accordingly, it could be assumed that the most anterior cartilaginous portion of the ET is opened primarily by the contraction of the LVPM, which causes a superior-medial rotation of the ML. Furthermore, since the contraction time of the LVPM is reported to be longer than that of the TVPM, the anterior cartilaginous portions of the ET may remain open, even after the middle to posterior cartilaginous portions are closed after relaxation of the TVPM. This process would produce a pumping action of the ET in the direction from the middle ear to the pharyngeal side. The pumping function may be beneficial to clearance of the middle ear.


Author(s):  
Robert Glaeser ◽  
Thomas Bauer ◽  
David Grano

In transmission electron microscopy, the 3-dimensional structure of an object is usually obtained in one of two ways. For objects which can be included in one specimen, as for example with elements included in freeze- dried whole mounts and examined with a high voltage microscope, stereo pairs can be obtained which exhibit the 3-D structure of the element. For objects which can not be included in one specimen, the 3-D shape is obtained by reconstruction from serial sections. However, without stereo imagery, only detail which remains constant within the thickness of the section can be used in the reconstruction; consequently, the choice is between a low resolution reconstruction using a few thick sections and a better resolution reconstruction using many thin sections, generally a tedious chore. This paper describes an approach to 3-D reconstruction which uses stereo images of serial thick sections to reconstruct an object including detail which changes within the depth of an individual thick section.


Author(s):  
C.W. Akey ◽  
M. Szalay ◽  
S.J. Edelstein

Three methods of obtaining 20 Å resolution in sectioned protein crystals have recently been described. They include tannic acid fixation, low temperature embedding and grid sectioning. To be useful for 3-dimensional reconstruction thin sections must possess suitable resolution, structural fidelity and a known contrast. Tannic acid fixation appears to satisfy the above criteria based on studies of crystals of Pseudomonas cytochrome oxidase, orthorhombic beef liver catalase and beef heart F1-ATPase. In order to develop methods with general applicability, we have concentrated our efforts on a trigonal modification of catalase which routinely demonstrated a resolution of 40 Å. The catalase system is particularly useful since a comparison with the structure recently solved with x-rays will permit evaluation of the accuracy of 3-D reconstructions of sectioned crystals.Initially, we re-evaluated the packing of trigonal catalase crystals studied by Longley. Images of the (001) plane are of particular interest since they give a projection down the 31-screw axis in space group P3121. Images obtained by the method of Longley or by tannic acid fixation are negatively contrasted since control experiments with orthorhombic catalase plates yield negatively stained specimens with conditions used for the larger trigonal crystals.


Author(s):  
Wah Chiu ◽  
Michael Sherman ◽  
Jaap Brink

In protein electron crystallography, both low dose electron diffraction patterns and images are needed to provide accurate amplitudes and phases respectively for a 3-dimensional reconstruction. We have demonstrated that the Gatan 1024x1024 model 679 slow-scan CCD camera is useful to record electron diffraction intensities of glucose-embedded crotoxin complex crystal to 3 Å resolution. The quality of the electron diffraction intensities is high on the basis of the measured intensity equivalence ofthe Friedel-related reflections. Moreover, the number of patterns recorded from a single crystal can be as high as 120 under the constraints of radiation damage and electron statistics for the reflections in each pattern.A limitation of the slow-scan CCD camera for recording electron images of protein crystal arises from the relatively large pixel size, i.e. 24 μm (provided by Gatan). The modulation transfer function of our camera with a P43 scintillator has been determined for 400 keV electrons and shows an amplitude fall-off to 0.25 at 1/60 μm−1.


Author(s):  
David L. Spector ◽  
Robert J. Derby

Studies in our laboratory are involved in evaluating the structural and functional organization of the mammalian cell nucleus. Since several major classes (U1, U2, U4/U6, U5) of small nuclear ribonucleoprotein particles (snRNPs) play a crucial role in the processing of pre-mRNA molecules, we have been interested in the localization of these particles within the cell nucleus. Using pre-embedding immunoperoxidase labeling combined with 3-dimensional reconstruction, we have recently shown that nuclear regions enriched in snRNPs form a reticular network within the nucleoplasm which extends between the nucleolar surface and the nuclear envelope. In the present study we were inte rested in extending these nuclear localizations using cell preparation techniques which avoid slow penetration of fixatives, chemical crosslinking of potential antigens and solvent extraction. CHOC 400 cells were cryofixed using a CF 100 ultra rapid cooling device (LifeCell Corp.). After cryofixation cells were molecular distillation dried, vapor osmicated, in filtra ted in 100% Spurr resin in vacuo and polymerized in molds a t 60°C. Using this procedure we were able to evaluate the distribution of snRNPs in resin embedded cells which had not been chemically fixed, incubated in cryoprotectants or extracted with solvents.


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