Cystic fibrosis (CF) patients present with a variety of symptoms, including mood and cognition deficits, in addition to classical respiratory, and autonomic issues. This suggests that brain injury, ...which can be examined with non-invasive magnetic resonance imaging (MRI), is a manifestation of this condition. However, brain tissue integrity in sites that regulate cognitive, autonomic, respiratory, and mood functions in CF patients is unclear. Our aim was to assess regional brain changes using high-resolution T1-weighted images based gray matter (GM) density and T2-relaxometry procedures in CF over control subjects.
We acquired high-resolution T1-weighted images and proton-density (PD) and T2-weighted images from 5 CF and 15 control subjects using a 3.0-Tesla MRI. High-resolution T1-weighted images were partitioned to GM-tissue type, normalized to a common space, and smoothed. Using PD- and T2-weighted images, whole-brain T2-relaxation maps were calculated, normalized, and smoothed. The smoothed GM-density and T2-relaxation maps were compared voxel-by-voxel between groups using analysis of covariance (covariates, age and sex; SPM12, p < 0.001).
Significantly increased GM-density, indicating tissues injury, emerged in multiple brain regions, including the cerebellum, hippocampus, amygdala, basal forebrain, insula, and frontal and prefrontal cortices. Various brain areas showed significantly reduced T2-relaxation values in CF subjects, indicating predominant acute tissue changes, in the cerebellum, cerebellar tonsil, prefrontal and frontal cortices, insula, and corpus callosum.
Cystic fibrosis subjects show predominant acute tissue changes in areas that control mood, cognition, respiratory, and autonomic functions and suggests that tissue changes may contribute to symptoms resulting from ongoing hypoxia accompanying the condition.
The population of children requiring home mechanical ventilation has evolved over the years and has grown to include a variety of diagnoses and needs that have led to changes in the care of this ...unique population. The purpose of this study was to provide a descriptive analysis of pediatric patients requiring home mechanical ventilation after hospitalization and how the evolution of this technology has impacted their care.
A retrospective, observational, longitudinal analysis of 164 children enrolled in a university-affiliated home mechanical ventilation program over 26 years was performed. Data included each child's primary diagnosis, date of tracheostomy placement, duration of mechanical ventilation during hospitalization that consisted of home mechanical ventilator initiation, total length of pediatric ICU stay, ventilator settings at time of discharge from pediatric ICU, and disposition (home, facility, or died). Univariate, bivariate, and regression analysis was used as appropriate.
The most common diagnosis requiring the use of home mechanical ventilation was neuromuscular disease (53%), followed by chronic pulmonary disease (29%). The median length of stay in the pediatric ICU decreased significantly after the implementation of a ventilator ward (70 d 30-142 vs 36 d 18-67,
= .02). The distribution of subjects upon discharge was home (71%), skilled nursing facility (24%), and died (4%), with an increase in the proportion of subjects discharged on PEEP and those going to nursing facilities over time (
= 0.02).
The evolution of home mechanical ventilation has allowed earlier transition out of the pediatric ICU and with increasing disposition to skilled nursing facilities over time. There has also been a change in ventilator management, including increased use of PEEP upon discharge, possibly driven by changes in ventilators and in-patient practice patterns.
Patients with congenital central hypoventilation syndrome (CCHS) show brain injury in areas that control chemosensory, autonomic, motor, cognitive, and emotion functions, which are deficient in the ...condition. Many of these abnormal characteristics are present from the neonatal period; however, it is unclear whether tissue injury underlying the characteristics progressively worsens with time. We hypothesized that several brain areas in subjects with CCHS would show increased gray matter volume loss over time.
We collected high-resolution T1-weighted images twice (4 years apart) from seven subjects with CCHS (age at first study, 16.1 ± 2.7 years; four males) and three control subjects (15.9 ± 2.1 years; three males) using a 3.0-Tesla magnetic resonance imaging (MRI) scanner, and evaluated regional gray matter volume changes with voxel-based morphometry (VBM) procedures.
Multiple brain sites in CCHS, including frontal, prefrontal, insular, and cingulate cortices; caudate nuclei and putamen; ventral temporal and parietal cortices; and cerebellar cortices showed significantly reduced gray matter volume over time. Only limited brain areas, including sensory, temporal, and medullary regions, emerged with increased gray matter at the later age.
Patients with CCHS show reduced gray matter volume with age progression in autonomic, respiratory, and cognitive regulatory areas, an outcome that may contribute to deterioration of functions found in the syndrome with increasing age.
Living lobar lung transplantation was developed as a procedure for patients considered too ill to await cadaveric transplantation.
One hundred twenty-eight living lobar lung transplantations were ...performed in 123 patients between 1993 and 2003. Eighty-four patients were adults (age, 27 ± 7.7 years), and 39 were pediatric patients (age, 13.9 ± 2.9 years).
The primary indication for transplantation was cystic fibrosis (84%). At the time of transplantation, 67.5% of patients were hospitalized, and 17.9% were intubated. One-, 3-, and 5-year actuarial survival among living lobar recipients was 70%, 54%, and 45%, respectively. There was no difference in actuarial survival between adult and pediatric living lobar recipients (
P = .65). There were 63 deaths among living lobar recipients, with infection being the predominant cause (53.4%), followed by obliterative bronchiolitis (12.7%) and primary graft dysfunction (7.9%). The overall incidence of acute rejection was 0.8 episodes per patient. Seventy-eight percent of rejection episodes were unilateral. Age, sex, indication, donor relationship, preoperative hospitalization status, use of preoperative steroids, and HLA-A, HLA-B, and HLA-DR typing did not influence survival. However, patients on ventilators preoperatively had significantly worse outcomes (odds ratio, 3.06,
P = .03; Kaplan-Meier
P = .002), and those undergoing retransplantation had an increased risk of death (odds ratio, 2.50).
These results support the continued use of living lobar lung transplantation in patients deemed unable to await a cadaveric transplantation. We consider patients undergoing retransplantations and intubated patients to be at significantly high risk because of the poor outcomes in these populations.
The aim of the present article was to determine the prevalence of liver involvement in Hispanic patients with cystic fibrosis (CF) and identify associations with age and severity of liver ...involvement.
We used 1994-2005 Epidemiologic Study of CF data to compare abnormal liver findings between Hispanic and non-Hispanic white patients with CF.
Of 30,727 patients with CF, 5015 had liver involvement. Of 1957 Hispanic patients, 20.8% had liver involvement compared with 16.0% of 28,770 non-Hispanic white patients (odds ratio OR 1.38, 95% confidence interval CI 1.23-1.54). This higher prevalence of liver involvement persisted after adjusting for demographics and meconium ileus and was especially high in the first year of life (adjusted OR 3.14, 95% CI 2.27-4.35). Ten percent of infants with only elevated liver enzymes progressed to more severe liver disease.
The Hispanic population with CF has more liver involvement (both elevated liver enzymes and clinical liver disease) than the non-Hispanic white population with CF, especially during the first year of life.
Overview of Lung Transplantation Woo, Marlyn S.
Clinical reviews in allergy & immunology,
12/2008, Letnik:
35, Številka:
3
Journal Article
Recenzirano
Odprti dostop
Although significant gains have been made in improving lung function and survival in cystic fibrosis (CF), ultimately respiratory failure is the leading cause of mortality in these patients. For CF ...patients with end stage lung disease, lung transplantation is an option for treatment. The field of lung transplantation has progressed markedly in the last 20 years. Nonetheless it remains a technically complex and challenging procedure, and patients are at risk for numerous short term and long term complications. Potential transplant recipients must be physically and psychologically prepared for the arduous process involved in lung transplantation. This article will review the history of lung transplantation, indications for transplantation, surgical techniques, and complications of transplantation.
ABSTRACT
Background and objective: Mongolia is experiencing rapid urbanization, and this presents a unique opportunity to assess the effects of this process on the lung health of children.
Methods: ...Two cross‐sectional cohorts of school‐age children (5–15 years of age) from the capital (Ulaanbaatar) (n = 116) and a rural district (Tuv Aimag) (n = 108) were studied. Demographical information, exposure to tobacco smoke, and ambient and exhaled CO, as well as FEV1 and FEF25–75% were recorded for each child.
Results: Ambient CO levels were threefold higher in the capital city than in the rural Aimag (0.63 vs 0.21 parts per million (ppm), P < 0.00005), while exhaled CO was twofold higher (0.94 vs 0.47 ppm, P < 0.00001). Rural Mongolian children were 6 cm shorter on average than urban children. However, when adjusted for age and height, FEV1 was 140% of predicted in rural children compared with 106% of predicted in urban children (P < 0.00001). There was no significant difference in small airway expiratory flow (FEF25–75%; 104 in urban children, 100 in rural children, P = 0.63).
Conclusions: ‘Normal’ FEV1 was actually 40% higher in rural Mongolian children than in urban children, suggesting that the FEV1 of apparently healthy children living in urbanized societies may in fact not be normal, but may instead reflect the deleterious effects of air pollution in cities, as indicated by increased levels of both environmental and exhaled CO.
FEV1 was 140% of predicted in rural Mongolian children compared with 100% of predicted in urban children, suggesting that FEV1 in apparently healthy urban children may not in fact be normal, but may instead reflect the deleterious effects of ambient air pollution, as indicated by threefold greater ambient CO levels.
Abstract Background EUR-1008 (Zenpep™ pancrelipase) is a new, enteric-coated, porcine-derived pancreatic enzyme product (PEP) developed for the treatment of cystic fibrosis (CF) patients with ...malabsorption associated with exocrine pancreatic insufficiency (EPI). Unlike currently marketed PEPs, EUR-1008 contains the label-claimed lipase content. Safety and efficacy were assessed in younger (< 7 years) and older (≥ 7 years) CF patients with EPI. Methods Two multicenter studies were conducted: a randomized, double-blind, placebo-controlled, crossover trial in patients ≥ 7 years of age ( N = 34) and a supplemental, open-label study in children < 7 years of age ( N = 19). Use of any medications altering gastric pH/motility was prohibited during the studies. Outcome measures in the randomized trial included changes in the coefficient of fat absorption (CFA), coefficient of nitrogen absorption (CNA), and signs/symptoms of malabsorption for EUR-1008 vs. placebo. Outcome measures in the supplemental study included safety and response (defined as no steatorrhea and no overt signs/symptoms of malabsorption) to EUR-1008 vs. previous enzyme treatment. Results In the randomized trial, EUR-1008 treatment compared to placebo resulted in a significantly higher mean CFA (88.3% vs. 62.8%, respectively) and CNA (87.2% vs. 65.7%, respectively) (both p < 0.001) and reduced the incidence of malabsorption signs and symptoms in 32 evaluable patients. In the supplemental study, 11 of 19 patients met the criteria for responder with EUR-1008 at the end of the study vs. 10 of 19 patients at screening (previous PEP), and improvements in clinical symptoms were reported with EUR-1008 treatment. EUR-1008 was safe and well tolerated, and no serious drug-related AEs were reported in either study. Conclusions EUR-1008 was safe, well tolerated, and effective in CF patients of all ages with EPI-associated malabsorption in two clinical trials. Treatment led to clinically and statistically significant improvements in CFA and CNA in the randomized study, and control of malabsorption and clinical symptoms in both studies.
Living lobar lung transplantation places two donors at risk for each recipient. We examined the perioperative outcomes associated with the 253 donor lobectomies performed at our institution during ...our first decade of living lobar lung transplantation. There have been no perioperative or long‐term deaths. 80.2% of donors (n = 203) had no perioperative complications, while fifty (19.8%) had one or more complication. The incidence of intraoperative complications was 3.6%. Complications requiring reoperation occurred in 3.2% of donors. 15.0% of donors had other perioperative complications; the most serious were two donors who developed pulmonary artery thrombosis, while the most common was the need for an additional thoracostomy tube or a thoracostomy tube for ≥14 d for persistent air leaks and/or drainage. Right‐sided donors were more likely to have a perioperative complication than left‐sided donors (odd ratio 2.02, p = 0.04), probably secondary to right lower and middle lobe anatomy. This experience has shown donor lobectomy to be associated with a relatively low morbidity and no mortality, and is important if this procedure is to be considered an option at more pulmonary transplant centers, given continued organ shortages and differences in philosophical and ethical acceptance of live
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