Airway abnormalities and pulmonary complications in long-term treated late-onset Pompe disease: Diagnostic and interventional by flexible bronchoscopy

This study evaluates the whole airway abnormalities of long-term treated late-onset Pompe disease (LOPD) patients, with interventions using the flexible bronchoscope (FB). As a retrospective study, we follow up with our five LOPD patients treated with Myozyme from 2012 to 2021 regularly, but with a focus on the whole airway abnormalities of these patients visualized through FB. The long-term clinical outcomes and relevant airway symptoms were assessed. Pulmonary function test and polysomnography were performed to evaluate the degree of respiratory compromise. All patients in the study had varying degrees of airway collapsibility, pulmonary complications, sleep apnea syndrome, and facial anomalies. Pulmonary function could preserve after Myozyme treatment, but potential deterioration thereafter. This is the first study that focuses on airway abnormalities and pulmonary complications in long-term treated LOPD patients using FB. Despite years of Myozyme treatment, we still observed airway abnormalities in these patients. In our series, the pulmonary complications seem more obvious than those observed in patients with infantile-onset Pompe disease, which might be related to the late diagnosis and treatment. We might recommend that FB could provide dynamic evaluation and interventions of airway abnormalities simultaneously. Early diagnosis of respiratory dysfunction is a critical prognostic factor of the long-term outcome of treated LOPD patients.     

The relationship between physician case volume and in-hospital mortality of critically ill children with a diagnosis of pneumonia: A cross-sectional observational analytical study

Purpose

The aim of this study is to examine the relationship between physician case volume and the outcomes of critically ill children with pneumonia.

Materials and methods

This is a population-based cohort study analyzed data provided from by the National Health Insurance Research Database of Taiwan, 2006-2009. Children (aged 3 months to 17 years) having records of intensive care unit (ICU) admission and a diagnosis of pneumonia were included. A total of 9754 critically ill children and 1042 attending physicians were enrolled. The children were assigned to 1 of 4 groups based on the physician's pneumonia case volume.

Results

The patients in the very high case volume group had a significantly lower length of hospital stay, in-hospital mortality rate, and hospitalization expenses, and a significantly higher ratio of ICU to hospital stays than the other 3 groups (P < .001). The probability of death tended to be lower when the physician's case volume was higher. The risk-adjusted odds ratio for in-hospital mortality of very-high case volume group was 0.48 (95% confidence interval, 0.35-0.65; P < .001) compared to low case volume group.

Conclusions

A higher physician's pneumonia case volume is associated with a lower length of hospital stay, lower in-hospital mortality rate, and lower hospitalization expenses among critically ill children with pneumonia.     

Using three-dimensional multigrid-based snake and multiresolution image registration for reconstruction of cranial defect

In cranioplasty, neurosurgeons use bone grafts to repair skull defects. To ensure the protection of intracranial tissues and recover the original head shape for aesthetic purposes, a custom-made pre-fabricated prosthesis must match the cranial incision as closely as possible. In our previous study (Liao et al. in Med Biol Eng Comput 49:203–211, 2011), we proposed an algorithm consisting of the 2D snake and image registration using the patient’s own diagnostic low-resolution and defective high-resolution computed tomography (CT) images to repair the impaired skull. In this study, we developed a 3D multigrid snake and employed multiresolution image registration to improve the computational efficiency. After extracting the defect portion images, we designed an image-trimming process to remove the bumped inner margin that can facilitate the placement of skull implants without manual trimming during surgery. To evaluate the performance of the proposed algorithm, a set of skull phantoms were manufactured to simulate six different conditions of cranial defects, namely, unilateral, bilateral, and cross-midline defects with 20 or 40 % skull defects. The overall image processing time in reconstructing the defect portion images can be reduced from 3 h to 20 min, as compared with our previous method. Furthermore, the reconstruction accuracies using the 3D multigrid snake were superior to those using the 2D snake.     

Influence of visual impairment and hearing impairment on functional dependence status among people in Taiwan—An evaluation using the WHODAS 2.0 score

Background

Visual impairment (VI) and hearing impairment (HI) are the two most common types of sensory disability encountered clinically. However, VI and HI result in different limitations in daily life. We assessed the level of functioning in patients with VI or HI based on the International Classification of Functioning, Disability, and Health.

Using World Health Organization Disability Assessment Schedule 2.0 in people with schizophrenia: a 4-year follow-up

European Archives of Psychiatry and Clinical Neuroscience - Little is known about the changes of people with schizophrenia disability in Taiwan who receive routine treatments under the current...     

Serum uric acid, hyperuricemia and body mass index in children and adolescents with intellectual disabilities

The aims of the preset study were to describe the profile of serum uric acid, the prevalence of hyperuricemia and its risk factors among children and adolescents with intellectual disabilities. We conducted a cross-sectional study of 941 children and adolescents with intellectual disabilities (aged 4–18 years) who participated in annual health examinations in three special schools in Taiwan. This study indicated 30.6% boys and 17.9% girls with intellectual disabilities were with hyperuricemia in Taiwan. The factors of gender, age and BMI were variables that can significantly predict the hyperuricemia occurrence in this vulnerable population. Those children and adolescents with intellectual disabilities were boys (OR = 2.93, 95% CI = 2.02–4.26) and older age (OR = 6.49, 95% CI = 2.19–19.21) were more likely to be hyperuricemia. With regard to BMI to hyperuricemia occurrence, those children and adolescents with intellectual disabilities were overweight (OR = 1.16–3.21, 95% CI = 1.16–3.21) and being obese (OR = 4.95–11.58, 95% CI = 4.95–11.58) was more likely to have a hyperuricemia than the normal weight group. This study provides the general profile of serum uric acid, hyperuricemia and its risk factors of children and adolescents with intellectual disabilities. Medical professionals should be highly alert to the possible consequences of hyperuricemia and provide useful information about the clinical manifestation of this condition for caregivers of children and adolescents with intellectual disabilities.     

Extrinsic high-effort and low-reward conditions at work among institutional staff caring for people with intellectual disabilities in Taiwan

The purposes of the present study were to determine whether extrinsic high-effort/low-reward conditions at work are associated with personal characteristics and the organizational environments. A cross-sectional survey was conducted (76.7% response rate, N=1243) by recruiting the staff caring for people with intellectual disabilities of Taiwan in 2006. Conditions at work were measured using Siegrist's Effort-Reward Imbalance (ERI) model, the questionnaire included 23 Likert scaled items and it divided into three scales: effort, reward and overcommitment. Multiple logistic regression modeling was conducted for extrinsic high-effort/low-reward status in relation to staff and working environmental factors. We found that 15.1% staff were in the low-effort/low-reward group, 35.9% was in the low-effort/high-reward group, 17.9% belonged to the high-effort/high-reward group and 31.1% was included in the high-effort/low-reward group. Controlling for many personal demographic and organizational characteristics, the factors of perceived job support (OR=0.91; 95% CI=0854-0.97), job control (OR=0.954, 95% CI=0.934-0.974), job demand (OR=1.155, 95% CI=1.109-1.203) and job stress (felt sometimes stressful compare to no stress at all, OR=2.305, 95% CI=1.161-4.575) of the staff were significantly correlated to the extrinsic high effort/low reward at work in the multiple logistic regression model. The present study highlights that the service providers need to be aware and understand the experiences that their staff encounters in the organizational, interpersonal and personal level regarding unfair working conditions such as high effort/low reward to improve the positive health of the staff.     

The AML microenvironment catalyzes a stepwise evolution to gilteritinib resistance

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