Restrictive Impairment Research Articles

The Immediate effect of Chest Mobilization Technique on Oxygen Saturation in Patients of COPD with Restrictive Impairment

The Immediate effect of Chest Mobilization Technique on Oxygen Saturation in Patients of COPD with Restrictive Impairment

Preoperative and Postoperative Pulmonary Function in Elderly Patients with Thoracolumbar Kyphoscoliosis.

Study DesignCase series.PurposeThe objective of this study was to investigate the change in pulmonary function in adult patients with a spinal deformity who underwent spinal corrective surgery.Overview of LiteratureDegenerative lumbar and/or thoraco-lumbar deformities are is often prominent in adult spinal deformity cases, whereas a thoracic deformity involving the chest wall is inconspicuous. A lumbar spine deformity could affect the pulmonary function; however, few reports have investigated pulmonary function in adult patients with a spinal deformity.MethodsThis study included 14 adult patients with a spinal deformity who underwent posterior corrective fusion (3 males, 11 females; mean age, 67.4 years). We measured percent vital capacity (%VC) and percent forced expiratory volume in 1 second (%FEV1) before surgery and six months after surgery. We investigated the change in pulmonary function after corrective surgery and the correlation between radiographic parameters and pulmonary function.ResultsMean preoperative %VC and %FEV1 values were 99.9% and 79.3%, respectively. Two cases were diagnosed with restrictive impairment, and two cases were diagnosed with obstructive impairment before surgery. %VC improved in the restrictive impairment cases six months after surgery. However, %FEV1 did not improve significantly after surgery in the obstructive impairment cases.ConclusionsRestrictive impairment was improved in adult patients with a spinal deformity by corrective spinal surgery. However, spinal surgery did not improve obstructive impairment.

Comparison of lower limit of normal values with a fixed ratio assessed by spirometry in obstructive and restrictive lung diseases in Indian population

Background and Aim: Spirometric parameters are approximated by the normal distribution and using lower limit of normal (LLN) minimize the misclassification with fixed-ratio protocol in interpretation of spirometry results. This study investigates prevalence and characterization of obstructive and restrictive lung function impairments, and dissimilarities between two guidelines interpreting spirometry. Methods: This study was conducted to assess the lung function in students and patients ( n = 74) of MGM Medical College, Kishanganj, Bihar, between September 2012 and May 2014, using computerized Spirometer. Results: Forced vital capacity (FVC), forced expiratory volume in the 1st s (FEV1)/FVC, forced expiratory flow25-75% (FEF25-75%), peak expiratory flow rate (PEFR) were normally distributed with inflection at their mean ± standard deviation. FEF25-75%, and PEFR were higher in normal individuals compared to individuals with respiratory disease. FEV1/FVC were lower, and FVC were higher in obstructive disease while the reverse results were obtained in restrictive disease category. Restrictive disease by fixed-ratio was found with 55% negative predictive value (NPV) and 5% positive predictive value (PPV), while the normal cases by fixed-ratio criteria was found with 91% NPV and 15% PPV versus LLN criteria. There were 58 individuals with normal spirometries under the LLN method but a discrepant of only 12 fixed-ratio normal results. Concordance analysis by kappa statistics yielded a κ = 0.017. Conclusion: Older adults are more pulmonary susceptible. The performance of LLN criteria in differentiating respiratory diseases is better than the fixed-ratio criteria. Poor agreement exist between the fixed-ratio and LLN protocols in interpreting spirometric results. There is the difference between the LLN and fixed-ratio criteria which need to be redressed with population-specific reference values.

Interstitial lung disease as an initial manifestation of dermatomyositis

To explore the clinical features and prognosis of dermatomyositis patients with interstitial lung disease (ILD) as an initial manifestation. Medical records of 184 dermatomyositis inpatients complicated with ILD, admitted into Peking Union Medical College Hospital from January 1999 to January 2013, were retrospectively analyzed. The clinical features, biochemical parameters, positive rates of autoantibodies, radiology, pulmonary function tests, pathology, treatments and prognosis were compared between two subgroups of ILD-initial and non-ILD-initial dermatomyositis. The incidence of ILD of dermatomyositis inpatients was 17%. The average age was 48 ± 12 years and the gender ratio of male-to-female was 63: 121. Eighty eight (47.8%) dermatomyositis patients had ILD as an initial manifestation, including (n = 42, 22.8%) of ILD concomitant dermatomyositis (within 1 month) and (n = 46, 25.0%) of ILD before dermatomyositis with an average ahead time of (11 ± 3) months. Patients of ILD-initial dermatomyositis had a higher incidence of dyspnea on exertion, cough and lung crackles, but there were lower incidences of heliotrope rash, chest V area rash, shawl sign and joint involvement than non-ILD-initial dermatomyositis (P < 0.05). The positive rate of anti-Jo-1 antibodies of ILD-initial dermatomyositis group was 13.6%. The main performances of ILD-initial dermatomyositis on pulmonary function tests were diffusing and restrictive ventilation impairment. And there was a lower diffusing rate of carbon monoxide than non-ILD-initial dermatomyositis group (P < 0.01). Organic and non-specific interstitial pneumonias were the major clinical pathology types of ILD-initial dermatomyositis. The mortality rate of ILD-initial dermatomyositis patients was 19.3% and there was no significant difference from non-ILD-initial dermatomyositis (P > 0.05). The main course of ILD-initial dermatomyositis was respiratory failure due to progressive ILD (n = 13, 76.5%). ILD as an initial manifestation is a common complication and a major mortality cause of dermatomyositis inpatients. And the frequent clinical pathology types are organic and non-specific interstitial pneumonias. Symptoms of skin and muscle, creatine kinase and anti-synthetase antibodies should be closely monitored.

Effect of active pulmonary rehabilitation on pulmonary function in patients with brain lesion.

The patients with brain lesion have a high risk of pulmonary complication due to restrictive pulmonary impairment. To investigate the effectiveness of active pulmonary rehabilitation on pulmonary functional improvement in these patients. Twnety-two patients of hemiplegia or quadriplegia with brain lesion were recruited who had restrictive pulmonary impairment. The patients were randomly assigned to an experimental group that receive active pulmonary rehabilitation training and a control group without it. The active pulmonary rehabilitation training is composed of air-stacking exercise by ambu-bagging and functional electrical stimulation (FES) on phrenic nerve and abdominal muscle. We examined respiration function test of all patients composed of peak cough flow (PCF), pulmonary function test (PFT), diaphragmatic motion evaluation and oxygen saturation (SaO2) at the beginning and 4 weeks after treatment. There was no significant difference in pulmonary function between experimental group and control group at the beginning. After 4 weeks treatment, FVC and FEV1 of PFT, PCF, diaphragmatic motion, SaO2 have significantly improved (p < 0.05) in experimental group, but no significant changes were detected in control group. It is suggested that active pulmonary rehabilitation with air-stacking exercise and FES would be useful to improve pulmonary function in patients with brain lesion.

Advanced pulmonary sarcoidosis.

To present an update on the most recent contributions in advanced pulmonary sarcoidosis (APS). Pathology is better described and the differences between fibrosing pulmonary sarcoidosis and usual interstitial pneumonia (UIP) are clarified. Serial spirometry is the most reliable tool for monitoring evolution. Survival may be predicted by an integrative algorithm based on pulmonary function and computed tomography (CT). APS is characterized by significant fibrocystic pulmonary lesions at CT and pathology. There are two main patterns of APS, one with predominant central bronchovascular distortion, often associated with airflow limitation, and the other with predominant honeycombing with a different location than in UIP with severe restrictive impairment and very low diffusion capacity of the lung for carbon monoxide. APS may be burnt out but is most often still active as evidenced by several findings, including on F-fluorodeoxyglucose-PET. There is an increased mortality and morbidity with chronic respiratory insufficiency, pulmonary hypertension stemming from multiple mechanisms, chronic pulmonary aspergillosis and extra infections. Acute worsening episodes are frequent. Serial spirometry, particularly forced vital capacity, is the most reliable tool for monitoring evolution. A new elegant algorithm based on pulmonary function and CT may predict survival. Despite important stakes, there is still a lack of therapeutic recommendations. However, the use of antisarcoidosis treatment is most often required at least as a temporary trial. Finally, the effect of pulmonary hypertension treatment has recently been the object of further evaluation.

Association between right ventricular dysfunction and restrictive lung disease in childhood cancer survivors as measured by quantitative echocardiography.

Restrictive lung disease is a complication in childhood cancer survivors who received lung-toxic chemotherapy and/or thoracic radiation. Left ventricular dysfunction is documented in these survivors, but less is known about right ventricular (RV) function. Quantitative echocardiography may help detect subclinical RV dysfunction. The aim of this study was to assess RV function quantitatively in childhood cancer survivors after lung-toxic therapy. We identified records of 33 childhood cancer survivors who (1) were treated with lung-toxic therapy and/or radiation, (2) were cancer-free for ≥ one year after therapy, and (3) had pulmonary function tests and echocardiograms from their most recent follow-up visit. Participants' mean age was 11.6 ± 4.5 years at cancer diagnosis and 23 ± 8.6 years at evaluation. The most common diagnosis was lymphoma/leukemia (n = 27). Twenty-nine subjects had anthracycline exposure. Eleven of the 33 subjects demonstrated restrictive pulmonary impairment (total lung capacity 3.69 ± 1.5 L [69.3 ± 22.4% predicted]). Among quantitative measures of RV function, isovolumetric acceleration (IVA), a measure of contractility, was significantly lower in the group with restrictive lung disease (2.42 ± 0.56 vs. 1.83 ± 0.78 m/sec(2); P < 0.05). There was a trend towards lower tissue Doppler derived S' and tricuspid annular plane systolic excursion in the group with restrictive lung disease. Subjects with restrictive lung disease were found to have ≥ 2 abnormal parameters (P < 0.01). IVA may detect early RV dysfunction in childhood cancer survivors with restrictive lung disease. Our findings require confirmation in a larger study population and validation by cardiac MRI.

Pulmonary function following allogeneic stem cell transplantation in childhood: a retrospective cohort study of 51 patients.

HSCT is associated with a high risk of late morbidity. The aim of this study was to evaluate the frequency, time frame, risk factors, and possible etiology of pulmonary dysfunction following allogeneic HSCT in childhood. We evaluated the pulmonary function of 51 HSCT patients (>6 yr), by including FVC and FEV1 values prior to (baseline) and annually up to five yr after HSCT. A Cox proportional hazards model was used to analyze the risk factors for a pulmonary event. Over half (59%) of the patients developed pulmonary dysfunction, mainly consisting of restrictive abnormalities. Acute GvHD (HR 4.31, 95% CI 1.47-12.63), chronic GvHD (HR 10.20, 95% CI 2.42-43.03), and an abnormal baseline pulmonary function (HR 4.82, 95% CI 1.02-22.84) were associated with post-transplant dysfunction. FEV1 (p < 0.001) and FVC (p < 0.001) declined significantly by 12 months after HSCT and both remained below the pre-HSCT level at up to four yr post-transplantation. HSCT in childhood is associated with early and persistent restrictive impairment of pulmonary function. Patients with extensive chronic GvHD are particularly vulnerable to severe pulmonary dysfunction. Scheduled pulmonary function testing is warranted as part of the follow-up of survivors of HSCT in childhood.

Stages and Conformations of the Tau Repeat Domain during Aggregation and Its Effect on Neuronal Toxicity

Several neurodegenerative diseases are characterized by the aggregation and posttranslational modifications of Tau protein. Its "repeat domain" (TauRD) is mainly responsible for the aggregation properties, and oligomeric forms are thought to dominate the toxic effects of Tau. Here we investigated the conformational transitions of this domain during oligomerization and aggregation in different states of β-propensity and pseudo-phosphorylation, using several complementary imaging and spectroscopic methods. Although the repeat domain generally aggregates more readily than full-length Tau, its aggregation was greatly slowed down by phosphorylation or pseudo-phosphorylation at the KXGS motifs, concomitant with an extended phase of oligomerization. Analogous effects were observed with pro-aggregant variants of TauRD. Oligomers became most evident in the case of the pro-aggregant mutant TauRDΔK280, as monitored by atomic force microscopy, and the fluorescence lifetime of Alexa-labeled Tau (time-correlated single photon counting (TCSPC)), consistent with its pronounced toxicity in mouse models. In cell models or primary neurons, neither oligomers nor fibrils of TauRD or TauRDΔK280 had a toxic effect, as seen by assays with lactate dehydrogenase and 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide, respectively. However, oligomers of pro-aggregant TauRDΔK280 specifically caused a loss of spine density in differentiated neurons, indicating a locally restricted impairment of function.

Assessment of Functional Lung Impairment in Hypothyroidism

Aims: To determine any functional lung impairment amongst patients diagnosed with hypothyroidism by spirometry. Methods: 21 hypothyroid subjects with TSH ≥10 mIU/L aged between 18-52 yrs were studied along with 21 healthy controls in the department of Physiology, Regional Institute of Medical Sciences (RIMS), Imphal. Hypothyroid subjects with other conditions/diseases which affect PFT were excluded. Spirometric parameters were conducted by means of computerised spirometer HELIOS 401/701(RMS, Chandigarh).The spirometric parameters studied are FVC, FEV1, FEV1/FVC, FEF25-75%, PEFR. Unpaired T-test and Pearson correlation was used and P value <0.05 was taken as significance. Result: Hypothyroid subjects showed a reduction of the % predicted value of FVC, FEV1, FEF25-75%, PEFR as compared to control subjects even though these values are within the normal values for their age, sex and gender. But the difference is statistically significant only for FVC. And the % predicted value for FEV1/FVC of hypothyroid subjects is found to be significantly increased than the controls. On analyzing and classifying only the hypothyroid subjects based on FVC values, 47% of the hypothyroid subjects were normal while 9% had mild restrictive lung impairment and the rest 43% had moderate to severe restrictive impairment. A negative correlation was found between TSH levels with the spirometric parameters FVC, FEV₁, FEV1/FVC and a positive correlation was found between T4 levels with FVC, FEV₁, FEV1/FVC. But none of the findings were not statistically significant. Conclusion: The pulmonary functions can be affected in hypothyroidism and they show a restrictive pattern of lung impairment.

Visual Assessment of CT Findings in Smokers With Nonobstructed Spirometric Abnormalities in the COPDGene® Study

Within the COPD Genetic Epidemiology (COPDGene®) study population of cigarette smokers, 9% were found to be unclassifiable by the Global Initiative for chronic Obstructive Lung Disease (GOLD) criteria. This study was to identify the differences in computed tomography (CT) findings between this nonobstructed (GOLDU) group and a control group of smokers with normal lung function. This research was approved by the institutional review board of each institution. CT images of 400 participants in the COPDGene® study (200 GOLDU, 200 smokers with normal lung function) were retrospectively evaluated in a blinded fashion. Visual CT assessment included lobar analysis of emphysema (type, extent), presence of paraseptal emphysema, airway wall thickening, expiratory air trapping, centrilobular nodules, atelectasis, non-fibrotic and fibrotic interstitial lung disease (ILD), pleural thickening, diaphragmatic eventration, vertebral body changes and internal thoracic diameters (in mm). Univariate comparisons of groups for each CT parameter and multiple logistic regression were performed to determine the imaging features associated with GOLDU. When compared with the control group, GOLDU participants had a significantly higher prevalence of unilateral diaphragm eventration (30% vs. 16%), airway wall thickening, centrilobular nodules, reticular abnormality, paraseptal emphysema (33% vs. 17%), linear atelectasis (60% vs. 35.6%), kyphosis (12% vs. 4%), and a smaller internal transverse thoracic diameter (255 ± 22.5 [standard deviation] vs. 264.8 ± 22.4, mm) (all p<0.05). With multiple logistic regression, all of these CT parameters, except non-fibrotic ILD and kyphosis, remained significantly associated with GOLDU status (p<0.05). In cigarette smokers, chest wall abnormalities and parenchymal lung disease, which contribute to restrictive physiologic impairment, are associated with GOLD-nonobstructed status.

Visual Assessment of CT Findings in Smokers With Nonobstructed Spirometric Abnormalities in The COPDGene® Study.

Within the COPD Genetic Epidemiology (COPDGene®) study population of cigarette smokers, 9% were found to be unclassifiable by the Global Initiative for chronic Obstructive Lung Disease (GOLD) criteria. This study was to identify the differences in computed tomography (CT) findings between this nonobstructed (GOLDU) group and a control group of smokers with normal lung function. This research was approved by the institutional review board of each institution. CT images of 400 participants in the COPDGene® study (200 GOLDU, 200 smokers with normal lung function) were retrospectively evaluated in a blinded fashion. Visual CT assessment included lobar analysis of emphysema (type, extent), presence of paraseptal emphysema, airway wall thickening, expiratory air trapping, centrilobular nodules, atelectasis, non-fibrotic and fibrotic interstitial lung disease (ILD), pleural thickening, diaphragmatic eventration, vertebral body changes and internal thoracic diameters (in mm). Univariate comparisons of groups for each CT parameter and multiple logistic regression were performed to determine the imaging features associated with GOLDU. When compared with the control group, GOLDU participants had a significantly higher prevalence of unilateral diaphragm eventration (30% vs. 16%), airway wall thickening, centrilobular nodules, reticular abnormality, paraseptal emphysema (33% vs. 17%), linear atelectasis (60% vs. 35.6%), kyphosis (12% vs. 4%), and a smaller internal transverse thoracic diameter (255 ± 22.5 [standard deviation] vs. 264.8 ± 22.4, mm) (all p<0.05). With multiple logistic regression, all of these CT parameters, except non-fibrotic ILD and kyphosis, remained significantly associated with GOLDU status (p<0.05). In cigarette smokers, chest wall abnormalities and parenchymal lung disease, which contribute to restrictive physiologic impairment, are associated with GOLD-nonobstructed status.

Pulmonary function and autoantibodies in a long-term follow-up of juvenile dermatomyositis patients

Pulmonary disease is a rare complication in JDM, described in only a few studies. This long-term follow-up study aimed to (i) describe pulmonary involvement in a national cohort of JDM patients estimated by conventional spirometry, (ii) compare pulmonary impairment with overall JDM outcome, and (iii) identify possible associations between pulmonary impairment and myositis-specific autoantibodies (MSAs). Fifty-one JDM patients performed conventional spirometry in a cross-sectional follow-up study. The scores of the Myositis Damage Index (MDI), Myositis Damage by visual analogue scale (MYODAM-VAS) and physician's global damage assessment were used to estimate JDM outcome. ANAs, MSAs and myositis-associated autoantibodies were analysed in all patients. Forty-two patients (82%) (mean follow-up time 14.3 years) had normal lung function. Four patients (8%) were diagnosed with JDM-related restrictive interstitial lung disease. No patients reported pulmonary symptoms. Patients with restrictive pulmonary function had increased long-term damage estimated by MDI (P = 0.008), MYODAM-VAS (P = 0.04), global assessment (P = 0.03) and number of organ systems involved (P = 0.009). We found significant correlation between the restrictive pulmonary function test and damage by the MDI (r = 0.43, P = 0.003), MYODAM-VAS (r = 0.44, P = 0.002), and global damage assessment (r = 0.43, P = 0.003). No association was found between the restrictive pulmonary function test and autoantibodies. In a long-term follow-up study of JDM patients, the majority of patients demonstrated normal lung function. However, restrictive pulmonary impairment was identified in 8% of patients, indicating a need for repetitive pulmonary follow-up in JDM patients. Restrictive pulmonary involvement was associated with increased long-term JDM damage.

Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension

The effect of splenectomy on pulmonary function test (PFT) and pulmonary hypertension (PH) in thalassemia remains unclear. We aimed to investigate PFT and their association with PH in patients with hemoglobin E/β-thalassemia stratified by their splenic status. Thirteen splenectomized patients (SP) and 12 non-splenectomized patients (NSP) were compared regarding to the PFT abnormalities and PH (mean pulmonary artery pressure from right-heart catheterization ≥25 mmHg or estimated systolic pulmonary artery pressure from echocardiography ≥40 mmHg). Eleven (84%) SP and 9 (75%) NSP had restrictive impairment (RI). Of these, more patients having severe RI in SP than in NSP (8 vs. 2, P = 0.035). FVC and PaO2 were lower in SP than in NSP (66 ± 15% vs. 77 ± 12%, P = 0.043, and 79.38 ± 1.6 mmHg vs. 98.83 ± 6.2 mmHg, P &lt; 0.001, respectively). Residual volume was higher in SP than in NSP (78 ± 17% vs. 64 ± 15%, P = 0.036). Seven (54%) SP who developed PH had a longer time interval between splenectomy and the onset of PH than those who did not (17 ± 4.9 years vs. 9.8 ± 6.1 years, P = 0.04). In conclusion, greater severity of extrapulmonary restrictive impairment and hypoxemia were more common in SP. These patients developed PH as a late complication unrelated to hypoxemia and PFT parameters.

Restrictive Impairment in Patients With Treated Pulmonary Tuberculosis

Restrictive Impairment in Patients With Treated Pulmonary Tuberculosis

Pulmonary function, chronic respiratory symptoms, and health-related quality of life among adults in the United States – National Health and Nutrition Examination Survey 2007–2010

BackgroundWe examined the association of impaired lung function and respiratory symptoms with measures of health status and health-related quality of life (HRQOL) among US adults.MethodsThe sample included 5139 participants aged 40–79 years in the National Health and Nutrition Examination Survey 2007–2010 who underwent spirometric testing and responded to questions about respiratory symptoms, health status, and number of physically unhealthy, mentally unhealthy, or activity limitation days in the prior 30 days.ResultsAmong these adults, 7.2% had restrictive impairment (FEV1/FVC ≥ 70%; FVC < 80% of predicted), 10.9% had mild obstruction (FEV1/FVC < 70%; FEV1 ≥ 80% predicted), and 9.0% had moderate–severe obstruction (FEV1/FVC < 70%; FEV1 < 80% predicted). Individuals with restrictive impairment or moderate–severe obstruction were more likely to report fair/poor health compared to those with normal lung function (prevalence ratio (PR) =1.5 [95% CI: 1.2-1.9] and 1.5 [1.3-1.8]), after controlling for sociodemographics, non-respiratory chronic diseases, body mass index, smoking, and respiratory symptoms. Frequent mental distress (FMD; ≥14 mentally unhealthy days), frequent physical distress (FPD; ≥14 physically unhealthy days), and frequent activity limitation (FAL; ≥14 activity limitation days) did not differ by lung function status. Adults who reported any respiratory symptoms (frequent cough, frequent phlegm, or past year wheeze) were more likely to report fair/poor health (PR = 1.5 [1.3-1.7]), FPD (PR = 1.6 [1.4-1.9]), FMD (PR = 1.8 [1.4-2.2]), and FAL (PR = 1.4 [1.1-1.9]) than those with no symptoms.ConclusionsThese results suggest the importance of chronic respiratory symptoms as potential risk factors for poor HRQOL and suggest improved symptom treatment and prevention efforts would likely improve HRQOL.

Unraveling restrictive chronic lung disease in sickle cell disease [Editorial

A LEADING CAUSE of decreased life expectancy in adults with sickle cell disease (SCD) is acute and chronic lung disease (CLD). CLD historically encompassed both pulmonary f brosis and pulmonary hypertension;1 however, we now understand that these are distinct entities that may not necessarily coexist in SCD patients. While the latter has been the subject of thorough investigations that have determined its high prevalence (approximately 10% by right heart catheterization), strong association with chronic hemolytic anemia, and its relevance as a risk factor for early mortality in SCD,2–5 the former has not been studied comprehensively in the modern era. Pulmonary fibrosis is associated with restrictive lung defects on spirometric evaluation. This is a common finding in adult SCD patients, probably as a result of longstanding chronic inflammation of the small airways from recurrent acute chest syndrome (ACS), infections, vascular infarction and extra-pulmonary restriction.1,6 Longitudinal changes in lung function have also recently been described in children with SCD, with a study reporting an average loss of approximately 3% of forced expiratory volume in one second per year.7,8 Few studies have addressed the pulmonary complications of SCD in sub-Saharan Africa. This is a major area of unmet need, as children with SCD in sub-Saharan Africa are exposed to high rates of pulmonary infectious co-morbidities— predominantly tuberculosis and HIV—expected to exacerbate CLD.9 The article by Cook et al. in this issue of the Journal has focused on determining lung function abnormalities and symptoms of asthma in a cohort of 25 Malawian children with SCD and comparing it to local and international reference ranges.10 The authors reported spirometric abnormalities suggestive of restrictive rather than obstructive defects and the prevalence of wheeze to be comparable to a cohort of children without SCD from urban areas. This is surprising given the high prevalence of obstructive lung disease in areas with high household pollution from burning biomass fuel, such as many urban and rural areas in Malawi.11 Cook et al. hypothesize that in the Malawian SCD population, progression to late stage restrictive lung disease may be occurring earlier and more rapidly with various environmental influences acting as disease modifiers. The authors did not report on history of ACS in the study cohort, but it is possible that an increased number of episodes of ACS might be an important predictor of development of restrictive lung disease at an earlier age. In addition, the small sample size greatly limits the analysis of associated co-morbidities. Despite these limitations, this study provides a useful snapshot of the prevalence of pulmonary function testing abnormalities in an international cohort of pediatric SCD patients. Moreover, it offers the potential to spur further research to develop preventive and therapeutic strategies to alter the pattern of CLD over a larger and varied SCD cohort followed longitudinally. As acknowledged by the authors, a more accurate determination of lung function by provocation testing might be helpful in detecting transient obstructive or even mixed obstructive/restrictive defects. Such studies are required, as asthma has been identified as a significant co-morbidity in SCD and there is growing evidence that a history of ACS in SCD children is a risk factor for reactive airway disease and airway hyper-responsiveness after provocation challenge testing.12–14 Although these are studies of associations and they do not establish causality, asthma exacerbations may precipitate vaso-occlusion and ACS by inducing ventilation-perfusion mismatches.15 In conclusion, this study highlights the importance of restrictive lung impairment in SCD patients, but leaves critical questions unanswered regarding the causes and co-morbidities of interstitial lung disease, as well as the prevalence and role of asthma in SCD outcomes in SCD patients.

Hernie de coupole diaphragmatique : devenir à long terme

The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.

Size matching in lung transplantation: An evidence-based review

The evidence base for size matching between donors and recipients in lung transplantation has not recently been reviewed in a comprehensive manner. Our aim in this study was to assimilate published studies that have addressed size matching of donors to recipients and to establish a pragmatic understanding of the range of lung sizes that may be used for lung transplantation. A comprehensive literature search was performed using Medline and PubMed up to and including September 2012, to identify scientific articles that relate to size matching between donors and lung transplant recipients. Seventy-two articles were identified, of which 21 had addressed the question of the impact of size mismatching on outcomes in lung transplantation. No study has specifically tested the consequences of intentionally mismatching above or below the hypothetical limits for double lung transplantation of a predicted total lung capacity for the donor of between 75% and 125% of the recipient predicted total lung capacity as set out in the ISHLT consensus report on lung donor acceptability criteria. Research is lacking that has robustly defined limits for size mismatch for single lung transplantation and for recipients with restrictive lung pathologies such as pulmonary fibrosis. Published research on the impact of size mismatching between lung transplant donors and recipients is limited by study design and size. It is centered on addressing the issue of mismatch in double lung transplantation in cohorts with a diagnostically heterogeneous make-up and in single lung transplant patients with chronic obstructive pulmonary disease.

Airway pressure release ventilation as a therapeutic approach in a patient with severe obesity hypoventilation syndrome and restrictive lung impairment

Airway pressure release ventilation as a therapeutic approach in a patient with severe obesity hypoventilation syndrome and restrictive lung impairment