Methods A retrospective study was performed at two tertiary refe

Methods. A retrospective study was performed at two tertiary referral institutions. Preoperative patient demographics and intraoperative surgical variables were analyzed to explore the potential association with outcomes. The outcomes recorded were death, length of hospital and intensive care unit (ICU) stay, discharge to a rehabilitation facility, major medical complications, neurologic deficits, and the additional unplanned

surgeries that patients subsequently underwent.

Results. One hundred three consecutive patients underwent a fusion extending from the thoracic spine to the pelvis for degenerative conditions from 2003 to 2007. There was a 4% mortality rate. The mean OSI-744 order hospital stay and mean ICU stay was 12 +/- 7 and 2.7 +/- 4 days, respectively. Fifty-eight percent of patients were discharged to a rehabilitation facility. Twelve percent of patients experience at least one major medical complication. Seventeen percent of patients had a documented new persistent neurologic deficit that was still present at the final clinic visit. Thirty-five percent of patients

underwent at least one unplanned return to the operating selleck chemicals llc theater. There were no perioperative demographics or surgical variables that had a statistically significant association with mortality. Major medical complications were associated with the American Society of Anesthesiologists’ (ASA) score (P = 0.030) and the Charlson Comorbidity Index (P = 0.028) but not age (P = 0.273).

Conclusion. Complex spine reconstruction involving fusions from the thoracic spine to the pelvis continues to be a high-risk procedure

in spite of more advanced surgical and perioperative techniques.”
“Background. Selleck AZD9291 Acute renal failure (ARF) is a common critical disorder. To decrease the mortality, it is important to prevent ARF from invading other organs in clinical setting. It is not known whether there is a dysfunction in pancreas during the pathogenesis of ARF. This study aimed to investigate the changes of morphology and function on pancreas in ARF rabbits. Methods. Sixty rabbits were randomly divided into four groups. The ARF model of groups 1 and 2 rabbits was established by hypodermic injection of 1% HgCl(2) (1.3 mL/kg) and intramuscular injection of 50% glyceritum (10 mL/kg), respectively. The control groups 1 and 2 were injected with same volume of normal saline. After 24 hours, urea and creatinine contents and pancreatic amylase (AMY) activities in serum were measured using an automatic biochemical analyzer; the insulin levels were measured with radioimmunoassay method. Moreover, morphological alterations were examined by light microscopy; free radicals, nitric oxide (NO), and nitric oxide synthase (NOS) in pancreas homogenate were determined. Results. Morphological study showed that there were vacuolar degeneration and necrosis in pancreas of ARF for both groups 1 and 2.

There was no difference

There was no difference LOXO-101 research buy in frequency of lethality and renal failure in the two study groups. In patients who are PR3- and/or MPO-ANCA positive, SBE and HCV infection should be excluded. Although similar in renal and skin manifestations in comparison to AAV, only patients with infections developed multiple serological abnormalities. In patients with infections, concomitant presence of ANCA, cryoglobulins, and hypocomplementemia was associated with severe glomerulonephritis.

The serological profile should be repeated after specific antimicrobial or surgical therapy, since some cases might require IST.”
“The polyamide 6/acrylonitrile-butylene-styrene copolymer (PA 6/ABS) blends were prepared using the twin-screw extruder with ethylene-acrylate-glycidyl methacrylate copolymer (EAGMA) as the reactive compatibilizer. The structure, morphology, thermal, and mechanical properties of the blends were carefully

investigated. The results indicated that the ring-opening reaction of epoxy groups of EAGMA in the presence of amine and carboxyl groups of PA 6 led to the formation of EAGMA grafted PA 6 copolymer. The grafted copolymer serving as the compatibilizer eventually led to the decrease of the phase diameters as observed from the scanning electron microscope (SEM). The crystallization and melting behavior of PA 6 were restricted by the incorporation of both ABS and EAGMA, and the addition of EAGMA improved the thermal stability of the selleck kinase inhibitor PA 6/ABS blends. Heat deformation temperature (HDT) of the blends reached a maximum value at EAGMA content of 12 wt %, which is about 45 degrees C above the HDT of pristine PA 6. The tensile strength of the blends decreased with an increasing EAGMA content, and the impact GDC-0994 concentration strength of the blends showed the highest value at EAGMA content of 20 wt %, which is about 323% higher than that of pristine PA 6. (C) 2011 Wiley Periodicals, Inc. J Appl Polym Sci 122: 586-592, 2011″
“In this work, Pt nanocrystals (NCs) were embedded into different oxide films

by codeposition of Pt and oxide at 400 degrees C and 10(-8) Torr using a pulse laser deposition system. Films of Pt (25 mol. %) NCs embedded in (Al2O3, ZnO, or SnO2) matrix showed room temperature ferromagnetism (RTFM), while Pt (25 mol. %) NCs/(MgO or SiO2) films were not magnetic. The detailed study of Pt NCs/Al2O3 films with different concentrations of Pt revealed that RTFM depended on Pt NC size and conductivity of the film. Resistance-temperature study of this film showed that the hopping conduction may be associated with RTFM. (C) 2011 American Institute of Physics. [doi:10.1063/1.3562872]“
“Polystyrene/alpha-zirconium phosphate (PS/OZrP) nanocomposites were prepared based on the organically modified alpha-ZrP(OZrP) with hexadecyltrimethyl ammonium bromide (C16) by solvothermal technique and solution refluxing.

Setting: University hearing research laboratories

and aud

Setting: University hearing research laboratories

and audiology clinics.

Patients: Fifty-five adults with hearing loss in at least 1 ear ranging from mild to severe.

Intervention: Automated measurement of pure tone air conduction thresholds using the following: a software-controlled adaptive method, and a user-controlled method of adjustment, both implemented on a calibrated iPad and using standard audiometry earphones.

Main Outcome Measure: Test-retest reliability of both methods, comparison of thresholds measured with automated techniques to thresholds measured using manual audiometry.

Results: For both iPad methods, Screening Library cell line test-retest differences were smaller than those reported in other studies for manual audiometry. Average automated versus manual threshold

differences were within the range of expected variance of manual audiometry. Subjects preferred the adjustment method.

Conclusion: Both iPad self-test methods yield accurate and reliable pure tone air conduction thresholds.”
“Although relative survival for breast cancer has improved in recent years, patients who present with metastatic disease have a less than 30% 5-year survival. Thus, improvements in treatment for these patients have the potential to have a significant impact on outcomes. Historically, removal of the primary breast tumor has been offered to these patients only for palliation. However, there have been recent reports that removal of the primary tumor may improve survival. Here, click here we review the theories and data at the center of the debate, the landmark studies that historically guided treatment, the retrospective data that revisited the role of removal of the primary tumor, as well as the latest advances in basic science and the accruing clinical studies to provide for future directions in this field. Although the definitive role of removal of the primary tumor in metastatic breast cancer is not settled, it is critical to understand the complexities of this debate in order to make further gains in breast cancer survivorship.”
“Objective: To review and discuss the risks and impact of hypoglycemia and provide guidance for the prevention of hypoglycemia in type

2 diabetes (T2DM).

Methods: We review and discuss the risks and impact of hypoglycemia, providing specific guidance regarding the prevention of hypoglycemia and judicial selection of glucose-lowering agents CBL0137 clinical trial in individuals with T2DM.

Results: Hypoglycemia in T2DM is underrecognized and underreported. Emerging evidence from large clinical trials suggest that hypoglycemia may be an important risk factor for morbidity and mortality in T2DM. In addition, hypoglycemia is associated with reduced quality of life, greater healthcare utilization costs, and poor adherence to medical regiments.

Conclusion: These findings have led professional organizations to emphasize the prevention of hypoglycemia as an important consideration when initiating or intensifying treatment regimens.

The separation of acetone-tetrahydrofuran mixtures is often faced

The separation of acetone-tetrahydrofuran mixtures is often faced in the pharmaceutical and special chemical industries. As acetone and tetrahydrofuran can form a minimum azeotrope, they cannot be separated by conventional distillation. But acetone and tetrahydrofuran are important organic raw materials and solvents, so the mixture should be separated for reuse.

ResultsThe process of continuous extractive distillation was used to separate the mixture of acetone (62% mass fraction) and

tetrahydrofuran (38% mass fraction) using butyl ether as solvent. PF-562271 Angiogenesis inhibitor The characteristics of the continuous extractive distillation were simulated via ASPEN and experiments also showed the feasibility of separating the acetone-tetrahydrofuran mixture. Effects of the reflux mass ratio (R),

mixture feed stage (FS), the solvent feed stage (SFS) on the extractive distillation column and the volume ratio of solvent to mixture (S/F) on the distillate mass fraction of acetone and bottom product mass fraction of acetone see more were investigated. The results of the extractive distillation simulation were verified by experiment data. With the following operation conditions for the extractive distillation column: number of theoretical plates53; mixture feed at 24th plate; solvent feed at 7th plate, solvent to mixture ratio 3 and reflux mass ratio 3, the mass fraction of acetone in the distillate can reach 99%.

ConclusionsThe process of continuous extractive distillation using butyl ether as solvent can separate the acetone-tetrahydrofuran Dibutyryl-cAMP solubility dmso mixture. The solvent to mixture ratio and reflux mass ratio are important factors that affect

the mass fraction of the product. (c) 2013 Society of Chemical Industry”
“Nearly zero magnetostrictive microwires have been proven to be very versatile, due to their extremely soft magnetic properties, the changes in their magnetization mechanism with dimensions, and the appearance of sensitive application-related effects, such as the large Barkhausen and the giant magnetoimpedance effects. Their sensitive magnetoimpedance response originates in the specific domain structure, composed of an inner core and an outer shell (OS), since the OS with a circumferential easy axis is the main region involved in the magnetoimpedance effect. The circumferential OS is believed to display a bamboolike structure with consecutive rings circumferentially magnetized in opposite directions. However, earlier theoretical studies predicted a uniformly magnetized OS as being energetically favored. This paper aims to clarify this contradiction. The variation of the magnetostatic energy from the bamboo domain to the uniformly magnetized state has been calculated and it has been found to be null. Therefore, a uniformly magnetized OS has lower energy due to the absence of domain walls.

Coping strategies were assessed using the Brief COPE questionnair

Coping strategies were assessed using the Brief COPE questionnaire. Social support and coping strategies were assessed only at T1. PTG was assessed

using the Post-Traumatic Growth Inventory at T1 and T2. Correlation and regression analyses were used to examine the association between social support and coping strategies with short-term reports of post-traumatic growth.

Results: Regression analyses showed that autonomy-supportive caregivers and a problem-focused strategy of coping significantly predicted greater PTG at T2.

Conclusions: This study contributes to a deeper understanding of the type of social support that may specifically aid cancer patients in experiencing PTG. Furthermore, findings confirm the important role of problem-focused coping strategies in growing psychologically. Copyright (C) 2010 John Wiley & Sons, Ltd.”
“A multiphase lattice Boltzmann numerical model driven by selleck chemicals llc an isothermal interaction potential is applied for the splitting of magnetic droplets in electrowetting-on-dielectric devices.

A hydrophilic magnetic plug is considered inside the liquid GS-9973 in vitro droplet and successive uniform force fields are applied in order to split this droplet. The numerical results are compared with experiments on water droplets containing plugs of superparamagnetic beads and good agreement is obtained. (C) 2009 American Institute of Physics. [DOI: 10.1063/1.3068486]“
“There is a pressing need to develop new antiviral treatments; of the 60 drugs currently available, half are aimed at HIV-1 and the remainder target only a further six viruses. This demand has led to the emergence of possible peptide therapies, with 15 currently in clinical trials. Advancements in understanding the antiviral potential of naturally occurring host defence peptides highlights the potential of a whole new class of molecules to be considered as antiviral therapeutics. Cationic host defence peptides, such as defensins Dactolisib and cathelicidins, are important components of innate immunity with antimicrobial and immunomodulatory capabilities.

In recent years they have also been shown to be natural, broad-spectrum antivirals against both enveloped and non-enveloped viruses, including HIV-1, influenza virus, respiratory syncytial virus and herpes simplex virus. Here we review the antiviral properties of several families of these host peptides and their potential to inform the design of novel therapeutics.”
“Objective: Counsellor familiarity and engagement with technology-mediated communication represents an important factor in the ability to implement support programs to cancer patients. This study describes the experiences of a cohort of expert psycho-oncology counsellors who learned to facilitate online support groups (OSGs) and identifies the important elements of their learning experience that led to their engagement.

During the present study, we compared results from a conventional

During the present study, we compared results from a conventional large scale wet chemistry analyzer to a widely used

dry chemistry point of care system (POC) and established a best practice for pre-analytical SB273005 datasheet dilutions. Furthermore, stability of enzyme activities in seminal plasma during storage at -18 degrees C for 24 h was evaluated. The average activity of AP in the 2nd fraction of normal ejaculates measured by Reflotron (R) was 107 328 IU?/?l. After 24 h of frozen storage, activities did not differ significantly (96 844 IU?/?l, p > 0.05). Fresh and frozen samples were analysed in parallel by the POC and conventional chemistry analyser, and the results compared that did not reveal a significant difference (p > 0.05). A dilution Fludarabine solubility dmso of seminal plasma with physiologic saline 1 : 100 prior to analysis was sufficient for the qualitative information whether AP activity is below or above 5000 IU?/?l. Present data show that AP measurement by a POC dry chemistry system is sufficiently accurate in diluted seminal plasma for the diagnosis of azoospermia and that seminal plasma can be stored frozen for 24 h before analysis.”
“OBJECTIVE: Amniotic fluid is a complex biological material that provides a unique window into

the developing human. Residual amniotic fluid supernatant contains cell-free fetal RNA. The objective of this study was to develop an understanding of the amniotic fluid core transcriptome by analyzing the transcripts ubiquitously present in the amniotic fluid supernatant of euploid midtrimester fetuses.

METHODS: This was an in silico (computational) investigation using publicly available gene expression data previously produced by our group from 12 euploid midtrimester amniotic

fluid samples. Functional analyses were performed using a web-based software analysis tool. Vorinostat clinical trial Organ specificity was examined for each transcript using a gene expression atlas. For fetal organs not represented in the atlas, manual literature searching and the web-based software analysis tool were used to generate fetal organ-associated gene lists.

RESULTS: There were 476 well-annotated genes present in 12 of 12 amniotic fluid samples. Functional analysis identified six physiologic systems represented in the amniotic fluid core transcriptome, including musculoskeletal and nervous system development and function and embryonic and organismal development. Mammalian target of rapamycin signaling was identified as a key canonical pathway. Twenty-three highly organ-specific transcripts were identified; six of these are known to be highly expressed in the fetal brain.

CONCLUSION: Amniotic fluid cell-free fetal RNA can provide biological information on multiple fetal organ systems.

01) and MBL deficiency (<100 ng/mL) with male gender (p= 0 004

01) and MBL deficiency (<100 ng/mL) with male gender (p= 0.004) in patients with CD. We failed to confirm any correlation between MBL deficiency and serum autoantibodies or NOD2/CARD15 variants.

Conclusions: Our results suggest that low MBL associated with paediatric-onset IBD and ileal CD

may be considered an additional marker of the IBD pathogenesis. (C) 2012 European Crohn’s and Colitis Organisation. Published by Elsevier B.V. All rights reserved.”
“Introduction and objectives. Surfactant protein B (SP-B) is a marker of damage to the alveolar-capillary barrier that could Copanlisib be useful for monitoring functional impairment in patients with chronic heart failure (HF).

Methods. Dyspnea-limited cardiopulmonary exercise testing was carried out in 43 outpatients with Combretastatin A4 clinical trial chronic HF (age 51+/-10 years, 77% male, left ventricular ejection fraction [LVEF] 33 +/- 11%). Peripheral blood serum samples were obtained at rest and during the first minute of peak exercise. The presence and concentration of SP-B

in the serum samples were determined by Western blot analysis.

Results. At rest, SP-B was detected in 35 (82%) patients compared with only six (23%) healthy volunteers in a control group (n=26, age 51+/-10 years, 77% male). The median circulating SP-B level was higher in HF patients, at 174 [interquartile range, 70-283] vs. 77 [41-152] (P<.001) in the control group. In HF patients, the presence of circulating SP-B was associated with a lower LVEF (31.4 +/- 9.6% vs. 41.8 +/- 15%; P=.01). Multivariate analysis showed that the resting SP-B level correlated with a greater VE/VCO2 slope (beta=1.45; P=.02). The peak-exercise SP-B

ACY-1215 research buy level correlated almost perfectly with the resting level (r=0.980; P<.001), but there was no significant increase with exercise (P=.164). Nor was there a correlation with any other exercise parameter.

Conclusions. In patients with chronic HF, the level of pulmonary surfactant protein B in the peripheral circulation is increased and is correlated with ventilatory inefficiency during exercise, as indicated by the VE/VCO2 slope.”
“A small portion of Type 2 diabetes mellitus (T2DM) is familial, but the majority occurs as sporadic disease. Although causative genes are found in some rare forms, the genetic basis for sporadic T2DM is largely unknown. We searched for a copy number abnormality in 100 early-onset Japanese T2DM patients (onset age < 35 years) by whole-genome screening with a copy number variation BeadChip. Within the 1.3-Mb subtelomeric region on chromosome 4p16.3, we found copy number losses in early-onset T2DM (13 of 100 T2DM versus one of 100 controls). This region surrounds a genome gap, which is rich in multiple low copy repeats. Subsequent region-targeted high-density custom-made oligonucleotide microarray experiments verified the copy number losses and delineated structural changes in the 1.3-Mb region.

Additional

clinical data are required to fully explore it

Additional

clinical data are required to fully explore its DMH1 use.”
“Aim: The aim of this preliminary study was to detect cytological changes in the oral mucosa after using a mouth wash with alcohol.

Material and Methods: A prospective double-blind, controlled study was performed, for 6 months. Group 1 consisted of 30 subjects who used a mouth rinse with 26.9% of alcohol [Listerine (R)] and Group 2 consisted of 30 subjects who used a mouth rinse with the same ingredients but with no alcohol. We obtained three cytological samples from the oral mucosa. The presence of cytological atypia, binucleation and karyorrhesis, and type of cells were studied. We also used a fluorescent in situ hybridization technique (FISH) in 15 samples in each group, for the micronucleus.

Results: We found no clinical mucosal alteration after using the mouth wash at the end of the study in either group. We observed no cytological differences between the groups at the end of the study (p>0.05). Regarding the study of the micronucleus by FISH, we observed no significant Selleckchem Navitoclax difference between the groups (p>0.05).

Conclusions:

Our results showed no cytological alteration in patients using a mouth rinse with alcohol, but these findings should be considered preliminary results, to be confirmed in a greater sample of patients.”
“Psoriasis is a chronic systemic disease with important skin Evofosfamide clinical trial manifestations, affecting 2 % of the population. It is more frequent and severe in obese patients, and both have been associated

with a higher cardiovascular risk. Recent studies suggest that weight loss may improve psoriasis and metabolic comorbidities in obese patients. We reviewed our prospective electronic database for all patients with psoriasis who underwent bariatric surgery between 2008 and 2011. We assessed surgical complications, weight progression, and psoriasis-related outcomes. The Dermatology Life Quality Index was used retrospectively to assess quality of life (QoL) before and after the operation. Ten patients were included; body mass index was 38.8 +/- 5.2 kg/m(2). Pre-surgical treatment for psoriasis was topical (50 %) and systemic (40 %). Eight patients underwent laparoscopic Roux-en-Y gastric bypass and two underwent laparoscopic sleeve gastrectomy. At follow-up, the mean percent excess weight loss 1, 6, and 12 months after surgery was 45 %, 80 %, and 88 %, respectively. Comorbidity resolution was 75 % for diabetes mellitus, 100 % for insulin resistance, and 57 % for hypertension. Related to psoriasis, 70 % of the patients stayed in remission 6 months after surgery and three of the four patients who were previously medicated with systemic drugs discontinued medication. The impact of psoriasis on QoL improved from 14.9 +/- 6.8 before surgery to 5 +/- 6.3 after surgery ( = 0.005).

Cross-breeding of hypoactive HAB and hyperactive LAB mice resulte

Cross-breeding of hypoactive HAB and hyperactive LAB mice resulted in offspring showing a low level of locomotion comparable to HAB mice, indicating that the HAB alleles are dominant over LAB alleles in determining the level of locomotion. In a holeboard test, LAB mice spent less time in hole exploration, as shown in patients with schizophrenia and ADHD; however, LAB mice displayed no impairments

in social interaction and prepulse inhibition Taselisib purchase (PPI), implying a unlikelihood of LAB as an animal model of schizophrenia. Although LAB mice displayed hyperarousal, active coping styles, and cognitive deficits, symptoms shared by mania and ADHD, they failed to reveal the classic manic endophenotypes, such as increased hedonia and object interaction. The neuroleptic haloperidol reduced locomotor activity in all mouse lines. The mood stabilizer lithium and the psychostimulant amphetamine, in contrast, selectively reduced hyperactivity in LAB mice. Based on the behavioral and pharmacological profiles, LAB mice are suggested as a novel rodent model of ADHD like symptoms.”
“SETTING: Eleven referring hospitals in South Korea.

OBJECTIVE: To compare therapeutic responses in chronic obstructive pulmonary disease (COPD) subgroups, classified by diffusing Anlotinib capacity of the lung for carbon monoxide (DL(CO)) and lung volume.

DESIGN: A total of 130 stable male COPD patients were

classified into four subgroups according to baseline DL(CO) and residual volume/total lung capacity (RV/TLC) ratio. We compared therapeutic responses to short acting beta(2)-agonist (SABA) and 3-month combined inhalation of long-acting Elacridar concentration beta(2)-agonist (LABA) and corticosteroid among patients with these subgroups.

RESULTS: Among the 130 COPD patients, 41 (31.5%) had normal DL(CO) and RV/TLC, 28 (21.5%) low DL(CO) and normal RV/TLC, 31 (23.8%) normal DL(CO) and high RV/TLC, and 30 (23.1%)

low DL(CO) and high RV/TLC. The normal DL(CO)/high RV/TLC subgroup showed a significantly larger flow response (changes in forced expiratory volume in 1 s) to salbutamol than the normal DL(CO)/RV/TLC subgroups, and a larger volume response (changes in forced vital capacity) than the two normal RV/TLC subgroups. The normal DL(CO)/high RV/TLC subgroup also showed significantly larger flow and volume response to 3-month combined inhalation of LABA and corticosteroid than the two normal RV/TLC subgroups.

CONCLUSION: COPD subgroups classified by DLco and RV/TLC may have different pulmonary function responses to pharmacological treatment.”
“Background: The rationale for commissioning community pulmonary rehabilitation programmes is based on evidence from randomised clinical trials. However, there are a number of reasons why similar programmes might be less effective outside the environment of a clinical trial.

An epidural lavage was performed, followed by injection of marcai

An epidural lavage was performed, followed by injection of marcaine/depo-medrol. Subjects scored their pain before and 3 months after the procedure. The immunoreactivity of an array of cytokines was measured in lavage samples and compared with clinical response to the therapeutic injection. Ten 5-Fluoracil purchase subjects underwent repeat epidural lavage sampling 3 months after the steroid injection.

Results. Interferon gamma (IFN gamma) was the most consistently detected cytokine. IFN gamma-immunoreactivity also highly correlated with reported reduction of pain 3-months after the epidural steroid injection. In subjects reporting significant pain relief (>50%)

from the injection, mean [IFN gamma] was significantly Nirogacestat purchase greater compared with patients experiencing no significant relief. The IFN gamma-immunoreactivity in repeat lavage samples decreased to trace residual concentrations in patients who reported pain relief from the steroid injection.

Conclusion. The presence of epidural IFN gamma-immunoreactivity corresponding to >10 pg/mL predicted significant pain relief after epidural steroid injection with >95% accuracy. These results suggest that IFN gamma may be part of a biochemical cascade triggering pain in

sciatica; IFN gamma-immunoreactivity may aid as a biomarker for predicting the response to steroid therapy and/or surgical intervention, and may serve as a future therapeutic target.”
“Aim: To assess the effect of bone marrow transplantation (BMT), enzyme replacement therapy (ERT), and a fiberoptic endotracheal intubation technique in patients with mucopolysaccharidosis type I (MPS I, Hurler syndrome). Background: The mucopolysaccharidoses are inherited metabolic conditions with a well-documented association with difficult airway management. We present the largest series to date of patients with Hurler syndrome (MPS I) and look at the impact of new treatments, such as BMT and learn more ERT, on anesthesia and airway management. Methods/Materials: We carried out a retrospective chart review of patients with MPSI undergoing anesthesia over 9 years

at the Royal Manchester Childrens Hospital. Data were collected on incidence of difficult and failed intubation and airway difficulties under anesthesia. Results: There were 39 patients identified, of which 20 had the attenuated form of MPS I and received ERT, 18 were treated by BMT and one patient received neither treatment. These patients had a total of 114 general anesthetics for 141 procedures. The incidence of airway complications overall is lower than previously reported at 31%. Patients with the attenuated form of the disease on ERT still have a high incidence of airway problems at 57% and a failed intubation rate of 3%. BMT patients on the other hand have a much lower incidence of airway complications at 14%, and there were no failed intubations in this group.