Pulmonary Function Testing In Children Research Articles

Long-term Follow-up Study of Pulmonary Function Test in Children with History of Hydrocarbon Aspiration

Background Aspiration of hydrocarbons causes several acute and chronic pulmonary complications; it may even lead to death. The aim of this study was to investigate the types of long-term pulmonary complications in children with a history of hydrocarbon aspiration. Materials and Methods In this case-control study, 21 children with history of hydrocarbon aspiration in the past 1-10 years were considered as case group, and 63 children without history of toxicity were regarded as the control group. The two groups were matched in terms of age and gender. Both groups underwent physical examination (such as height, weight and body mass index), and pulmonary function tests by spirometry. Pulmonary function was categorized based on three spirometric patterns, normal, obstructive, and restrictive. In addition, the baseline characteristics of children and information obtained from clinical and paraclinical examinations during poisoning were recorded in a researcher-made checklist. The obtained data were analyzed using SPSS software (version 16.0). Results The percentage of normal, obstructive and restrictive spirometric patterns were 61.89%, 28.58%, and 9.52% in the case group, and 88.88%, 11.11% and 0% in the control group, respectively (P 0.05). The results of the pulmonary function test were not related to the time elapsed from poisoning (P> 0.05). Conclusion Based on the results, long-term hydrocarbon poisoning caused pulmonary dysfunction in terms of spirometric patterns in children.

The development and future of pulmonary function testing in children in China

The research and clinical application of pulmonary function testing in children in China has a history of more than 50 years, which started in the 1960s and developed rapidly during the past two decades.Chinese scholars have explored the normal reference values, standard of bronchodilation test and applications in the diagnosis and treatment of diseases of different pulmonary function tests in children, especially infants, and developed a series of guidelines to standardize the operations, reports and applications.In the past decade, the applications have been fully promoted, and quality control have been continuously improved through standardized training.At present, pulmonary function tests have become an important method in the diagnosis and treatment of respiratory diseases in children. Key words: Pulmonary function testing; Children; History; Development

Study of some pulmonary function tests in Egyptian children with end-stage renal disease under regular hemodialysis in correlation with dialysis duration

Pulmonary function tests can differentiate between obstructive and restrictive lung diseases and assess the severity of the disease in children. The aim of work was to study pulmonary function tests in children with end-stage renal disease (ESRD) undergoing hemodialysis (HD) and its correlation with dialysis duration. This study was conducted on 40 patients with ESRD on regular HD for at least six months selected from the Pediatric Nephrology unit of Pediatric Department of Tanta university hospital and 40 healthy children as a control group. All participants were subjected to full history taking, thorough clinical examination, laboratory investigation: arterial blood gases and pulmonary function tests, including resting spirometry included measurement of lung volumes. There were significantly lower forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), FEV1/FVC ratio, peak expiratory flow rate, and forced expiratory flow in patients compared with controls and significant positive correlations between dialysis duration and both of FVC and FEV1 in studied patients. There were restrictive spirometric pattern in 30 patients (75%) with ESRD under regular HD and mixed obstructive and restrictive pattern in 10 patients (25%) with highly significant differences between patients and controls regarding patterns of spirometry. There was impairment of lung function in patients with chronic renal failure undergoing HD predominantly of the restrictive pattern. Children with ESRD under regular HD should undergo pulmonary function tests as follow-up investigation to detect associated pulmonary complications included obstructive, restrictive, or mixed patterns of impaired pulmonary function.

Extensive Evaluation of Pulmonary Function Abnormalities by Different Techniques in Turkish Children and Young Adults with Sickle Cell Disease

Pulmonary involvement in sickle cell disease (SCD) is a major cause of morbidity and mortality. Recent data about pulmonary function tests in children with SCD are conflicting. The objective of thi...

Airway inflammation in sickle cell disease-A translational perspective.

Asthma and sickle cell disease (SCD) are common chronic conditions in children of African ancestry that are characterized by cough, wheeze, and obstructive patterns on pulmonary function. Pulmonary function testing in children with SCD has estimated a prevalence of obstructive lung disease ranging from 13% to 57%, and airway hyper-responsiveness of up to 77%, independent of a diagnosis of asthma. Asthma co-existing with SCD is associated with increased risk of acute chest syndrome (ACS), respiratory symptoms, pain episodes, and death. However, there are inherent differences in the pathophysiology of SCD and asthma. While classic allergic asthma in the general population is associated with a T-helper 2 cell (Th-2 cells) pattern of cell inflammation, increased IgE levels and often positive allergy testing, inflammation in SCD is associated with different inflammatory pathways, involving neutrophilic and monocytic pathways, which have been explored to a limited extent in mouse models and with a dearth of human studies. The current review summarizes the existent literature on sickle cell related airway inflammation and its cross roads with allergic asthma-related inflammation, and discusses the importance of further elucidating and understanding these common and divergent inflammatory pathways in human studies to facilitate development of targeted therapy for children with SCD and pulmonary morbidity.

Effect of adenoid hypertrophy and pulmonary function tests in children with mild asthma.

This study aims to assess the effect of adenoid hypertrophy on asthma in children with mild asthma. Between September 2010 and September 2012, 63 children (42 males, 21 females; median age 10.5 years; range 6 to 14 years) admitted to our clinic with asthma complaint were included in this study. These children were evaluated for adenoid hypertrophy, symptoms of asthma, medical treatment, pulmonary function tests at the beginning of the study and at the end of second month. There was no correlation between initial pulmonary function tests and adenoid hypertrophy. But we observed significantly lower forced vital capacity values in children with prominent adenoid hypertrophy (p=0.033). While there was no significant difference in pulmonary function tests in terms of inhaled steroid usage (p>0.05), final forced mid-expiratory flow rate (FEF25-75) values were statistically higher in patients who were using nasal steroids (p=0.015). Consequently, significantly higher values of FEF25-75 in the group that used nasal steroid suggest that adenoid hypertrophy affects the airway obstruction. Moreover, absence of airway obstruction symptoms in patients with mild adenoid hypertrophy suggests pulmonary function tests may help to decide performing adenoidectomy.

The Tools of the Trade - Physiological Measurements of the Lungs.

Pulmonary function assessment plays an integral part in the clinical management of school-aged children with respiratory disease. Pulmonary function tests (PFTs) are being increasingly applied in infants and preschool children too, albeit only in specialised centres. PFTs, when performed and interpreted accurately, provide objective outcome measures which can be used clinically to guide management, for prognostic purposes and in epidemiological research studies. They can be used to determine the nature and severity of lung disease, to ascertain response to treatment and to monitor disease progression. PFTs are rarely diagnostic in their own right with the exception of asthma, but are valuable adjuncts and before clinicians select a PFT they must know what the results are likely to be in the disease being considered. Spirometry and tests of airway calibre and function are the most widely used PFTs, as diseases in children commonly affect airway function. As such, spirometry should be a standard part of the assessment of school-age children who present to the pediatrician with chronic respiratory symptoms. This review will provide a bird's eye view of currently available PFTs in children to assist in the diagnosis and management of respiratory disorders.

Pulmonary Function testing in Children

Spirometry is a simple and valuable tool in the evaluation of Pulmonary Function testing in children with respiratory symptoms. The maximum utilities in paediatric age group is 111 diagnosis and follow up of children with asthma and other wheezing problems, although it is useful for screening of children with restrictive lung diseases. This requires accurate and reproducible spirometrie measurements, which should comply with the American Thoracic Society (ATS)/European Respiratory Society (ERS) guidelines. Training and refresher courses may produce and maintain good-quality testing, promote the use of spirometrie results in clinical practice and enhance the quality of interpretation. The use of correct ethnic reference equations is essential for comparative results.

The effect of obesity on asthma in children

Introduction: The prevalence of obesity and asthma is increasing in recent years. Obesity is a risk factor for asthma. The presence of obesity may affect the clinical course of the disease in patients with asthma. This study was conducted to determine the effect of obesity on clinical course of asthma and pulmonary function tests in children with asthma. Material and Method: One hundred and sixty children with asthma who were followed in Pediatric ImmunologyAllergy Clinic were included in this study. Patients were evaluated prospectively between January 2011-June 2011. The patients were interrogated with detailed history of allergic disease, age, kin marriage, familial atopy, exposure to tobacco smoke, number of asthma attacks within the previous year, and systemic steroid use. Asthma control test, pulmonary function tests, skin prick tests, eosinophilia and total immunoglobulin E measurements were performed. Patients with waist circumferences above the 90th percentile were considered obese. Results: Ninety-six (60%) patients were of normal weight and 64 (40%) of them were obese. There were no statistically significant difference between both groups in terms of age, gender, consanguinity, family history of atopy, smoking, and atopy. The number of attacks and need of systemic steroid within the previous one year were significantly higher in obese asthmatics compared to normalweight individuals (p<0.05). Asthma control in obese asthmatics was significantly worse (p<0.05). There were no statistically significant intergroup difference in terms of serum total IgE, eosinophilia and respiratory function parameters (p>0.05). Binary logistic regression analysis was used to investigate the variables that were effective on asthma control (age, family history of asthma, BMI, WC, number of attacks, systemic steroid use, FEV1, FVC, FEV1/FVC, PEF, and MEF 25-75). The effect of BMI was found to be greater (OR: 39 Cl: (0.36, 4435)). Discussion and Conclusion: Obesity worsens asthma control, increases systemic steroid use and the number of attacks in our patients with asthma. Weight loss together with appropriate treatment and avoidance from allergens may improve asthma control in these patients. 

Spirometry Findings Following Treatment with Oral and Inhalant Corticosteroids in Mild to Moderate Asthma Exacerbation in Children

Introduction: Asthma exacerbation is common in children. Treatment with oral corticosteroids (OCS) and inhaled corticosteroids are suggested for asthma exacerbation. It is shown that inhaled corticosteroids has similar outcome in reducing asthma symptoms compared to OCS. But few studies have evaluated the pulmonary function changes in these two treatments. In this study, we evaluated the changes in pulmonary function tests in children with mild-to-moderate asthma exacerbation receiving oral prednisolone and inhaled Budesonide. Methods and Materials: Forty-four children with mild-to-moderate asthma exacerbation were randomly assigned to receive oral prednisolone (2 mg/kg) or Budesonide spray (2 puffs every 12 hours, each puff contains 200 microgram Budesonide) using a spacer for one week. The first dose of the treatment was given in the emergency department. Children were followed for seven days and spirometry findings before and after treatment were evaluated. Results: There was no significant difference between pulmonary function tests before and after treatment between groups. Children receiving oral prednisolone had significantly more improvement in PEF (p=0.01). There was significant improvement in all respiratory parameters after treatment in both groups (p<0.05), but PEF had no significant change after treatment s in inhaled Budesonide group (p=0.63). Conclusion:Both inhaled Budesonide and oral prednisolone significantly improved respiratory function in children with mild-to-moderate asthma exacerbation. As there was no significant difference between groups in PFT findings and due to the low systemic effects of inhaled budesonide compared to oral prednisolone, this treatment seems to be more appropriate in mild to moderate exacerbations.

Ambient polycyclic aromatic hydrocarbons and pulmonary function in children.

Few studies have examined the relationship between ambient polycyclic aromatic hydrocarbons (PAHs) and pulmonary function in children. Major sources include vehicular emissions, home heating, wildland fires, agricultural burning, and power plants. PAHs are an important component of fine particulate matter that has been linked to respiratory health. This cross-sectional study examines the relationship between estimated individual exposures to the sum of PAHs with 4, 5, or 6 rings (PAH456) and pulmonary function tests (forced expiratory volume in one second (FEV1) and forced expiratory flow between 25% and 75% of vital capacity) in asthmatic and non-asthmatic children. We applied land-use regression to estimate individual exposures to ambient PAHs for averaging periods ranging from 1 week to 1 year. We used linear regression to estimate the relationship between exposure to PAH456 with pre- and postbronchodilator pulmonary function tests in children in Fresno, California (N=297). Among non-asthmatics, there was a statistically significant association between PAH456 during the previous 3 months, 6 months, and 1 year and postbronchodilator FEV1. The magnitude of the association increased with the length of the averaging period ranging from 60 to 110 ml decrease in FEV1 for each 1 ng/m(3) increase in PAH456. There were no associations with PAH456 observed among asthmatic children. We identified an association between annual PAHs and chronic pulmonary function in children without asthma. Additional studies are needed to further explore the association between exposure to PAHs and pulmonary function, especially with regard to differential effects between asthmatic and non-asthmatic children.

Study of some Pulmonary Function Tests in Children with Sickle Cell Anemia: Correlation with Iron Overload

Background: Sickle cell disease is hereditary hemoglobinopathy characterized by abnormal hemoglobin production, hemolytic anemia, and intermittent occlusion of small vessels, leading to acute and chronic tissue ischemia, and organ damage. Pulmonary function tests can differentiate between obstructive or restrictive lung diseases, assess the severity of the disease and evaluate the degree of efficacy of therapy in patients with sickle cell anemia. Objective: The aim of this study was to study pulmonary function tests in children with sickle cell anemia and its correlation with iron overload. Patients and methods: This study was carried out on 40 children with sickle cell anemia under follow up at Hematology unit, Pediatric Department, Tanta university hospital in the period between October 2012 and August 2014 including 24 males and 16 females with their age ranging from 9– 15 years and mean age value of 12.7+2.2 years and 40 healthy children as a control group including 22 males and 18 females with their age ranging from 7 – 15 years and mean age value of 10.3+2.36 years. All patients were subjected to the following investigations: Complete blood count, HB electrophoresis, serum ferritin, serum iron, total iron binding capacity and pulmonary function tests. Results: There were significantly higher serum ferritin, serum iron and lower TIBC in patients than controls, significantly lower Forced vital capacity (FVC), Forced expiratory volume in one second (FEV1), FEV1/FVC ratio, Peak expiratory flow rate, and Forced expiratory Flow in patients compared with controls, significant negative correlation between serum ferritin and both of FVC and FEV1 in studied patients. There were restrictive spirometric pattern in 30 patients with sickle cell anemia (75%) and mixed obstructive and restrictive pattern in 10 patients (25%) with highly significant differences between patients and controls regarding restrictive and mixed pattern of spirometry. Conclusion: Most SCD patients show abnormal pulmonary functions, predominantly of restrictive pattern. Recommendations: Patients with SCD should undergo pulmonary function tests to detect and confirm lung disease for early and proper therapeutic intervention.

Long-term respiratory impairment with the new BPD

In the presurfactant era, bronchopulmonary dysplasia (BPD) was common and was associated with significant long-term respiratory morbidity. Although survival after extreme preterm birth has been improving and the rate of severe BPD has declined over the past 20 years, a new pattern of lung injury with disruption of lung growth has emerged. This new pattern of BPD seems less severe but long-term data have been sparse. To investigate the long-term outcomes of former very low birth weight (VLBW) preterm infants born in the surfactant era, vom Hove et al from the University of Leipzig have completed a study of pulmonary function tests in children with a history of BPD and VLBW, compared with a matched preterm VLBW control group.

Pulmonary Function Tests in Idiopathic Inflammatory Myopathy: Association With Clinical Parameters in Children

To determine the association of decreased lung function in children with idiopathic inflammatory myopathies (IIMs) with specific clinical parameters. This study of 38 children ages 6-23 years diagnosed with definite/probable IIM evaluated the association of myositis-specific/-associated antibodies (MSAs/MAAs), duration of untreated disease at diagnosis, Disease Activity Score for muscle (DAS-M), muscle-derived enzymes (aldolase, lactate dehydrogenase [LDH], aspartate transaminase, and creatine phosphokinase [CPK]), neopterin and von Willebrand factor antigen, and the Childhood Myositis Assessment Scale (CMAS) scores with data from pulmonary function testing (PFT). Impaired PFTs were defined as total lung capacity (TLC) or diffusing capacity for carbon monoxide (DLCO) of <80% predicted. The PFTs documented that 37% of the children (14 of 38) had either decreased TLC or decreased DLCO; 5% (2 of 38) had both. Children with decreased TLC alone (7 [18%] of 38) were older both at the time of PFT and diagnosis, had anti-Jo-1 and anti-Scl-70 antibody, and had elevated levels of CPK and neopterin. Children with decreased DLCO alone (5 [13%] of 38) had a shorter duration of untreated disease at diagnosis, had higher DAS-M and total DAS, were positive for anti-Ro and anti-PL-12, had increased LDH, and had elevated levels of neopterin and aldolase, with low CMAS scores for items 1, 3, 10, 11, and 14. Assessment of PFTs in children with IIMs should be considered, since more than one-third of patients were found to be impaired. The presence of MSAs/MAAs, an elevated serum neopterin level (mean ± SD 12.4 ± 9.6 nmoles/liter, normal value <10.5), older age at diagnosis, and shorter duration of untreated disease at diagnosis suggest the presence of potential lung pathology.

Comparison of Deflazacort and Prednisone in Duchenne Muscular Dystrophy

How to Cite this Article: Karimzadeh P, Ghazavi A. Comparison of Deflazacort and Prednisone in Duchenne Muscular Dystrophy. IranianJournal of Child Neurology 2012;6(1):5-12. Objective Duchenne muscular dystrophy (DMD) is a degenerative disease that usually becomes clinically detectable in childhood as progressive proximal weakness. No cure is yet available for DMD, but the use of steroids improves muscle strength and function. This study has been carried out to select the best steroid for the management of DMD. Materials & Methods This study is a single-blind, randomized clinical trial with a sample volume of 34 DMD patients. Half of these patients were treated with deflazacort (0.9 mg/kg daily) and the other half with prednisone (0.75 mg/kg daily) for a period of 18 months. The motor function score and excess body weight were registered one year after the start and also at the end of the study and compared between the two groups. Results Deflazacort was more effective in the improvement of motor function after one year, but there was no significant difference between the two drugs at the end of the study (18 months after start). Weight gain after one year and at the end of the study was higher in prednisone group and steroid treatment with deflazacort appears to cause fewer side effects than prednisone regarding weight gain. Conclusion Deflazacort seems to be more effective than prednisone in the improvement of motor function causing fewer side effects, particularly weight gain. This medication may be important for the improvement of motor function and could be used as the best steroidal treatment for Duchenne muscular dystrophy. References Lankester BJA, Whitehouse MR, Gargan MF. Duchenne muscular dystrophy. Current Orthopedics 2007;21:298- 300. Wenger DR, Rang M. The art and practice of children’s orthopedics. Philadelphia, PA: Lippincott; Baltimore: Williams and Wilkins; 1993. Sussman M. Duchenne muscular dystrophy. J Am Acad Orthop Surg 2002 Mar-Apr;10(2):138-51. Escolar DM, Leshner RT. Muscular dystrophies. In: Swaiman KF, Ashwal S, Ferriero DM. Pediatric Neurology. 4th ed. Philadelphia: Mosby; 2006. p. 1967-86. Sarnat HB, Menkes JH. Disease of the motor unit. In: Menkes JH, Sarnat HB, editors. Child Neurology. 7th ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 984-9. Biggar WD, Politano L, Harris VA, Passamano L, Vajsar J, Alman B, et al. Deflazacort in duchenne muscular dystrophy: a comparison of two different protocols. Neuromuscul disord 2004 Sep;14(8-9):476-82. Radley HG, De Luca A, Lynch GS, Grounds MD. Duchenne muscular dystrophy: focus on pharmaceutical and nutritional interventions. Int J Biochem Cell Biol 2007;39(3):469-77. Biggar WD, Klamut HJ, Demacio PC, Stevens DJ. Duchenne muscular dystrophy: current knowledge, treatment and future prospects. Clin Orthop Relat Res 2002 Aug;401:88-106. Sohn RL, Gussoni E. Stem cell therapy for muscular dystrophy. Expert Opin Biol Ther 2004 Jan;4(1):1-9. Houde S, Filiatrault M, Fournier A, Dube J, D’Arcy S, Berube D et al. Deflazacort use in duchenne muscular dystrophy: an 8-year follow-up. Pediatr Neurol 2008 Mar;38(3):200-6. Bonifati MD, Ruzza G, Bonometto P, Berardinelli A, Gorni K, Orcesi K et al. A multicenter double-bind randomized trial of deflazacort versus prednisone in Duchenne muscular dystrophy: analysis after 2 years. Basic Appl Myol 2000;10:171-5. Reitter B. Deflazacort vs. prednisone in Duchenne muscular dystrophy: trends of an ongoing study. Brain Dev 1995;17 Suppl:39-43. Polgar G, Promadhat V. Standard values. In: pulmonary function testing in children: techniques and standards. Philadelphia: W.B. Saunders, 1971:102-3. Biggar WD, Gingras M, Febling DL, Harris VA, Steele CA. Deflazacort treatment of Duchenne muscular dystrophy. J Pediatr 2001 Jan;138(1):45-50.

Children Should Not Be Treated Like Little Adults in the PFT Lab

Over the years a great deal of effort has been made to standardize all pulmonary function tests on adults. Many of the "rules" concerning the interpretation of the spirogram have been based entirely on adult observations. In the age of increasing conformity, and attempts to relate "adult" literature to the pediatric population, the latter was given much less emphasis than the former. This review will attempt to show what areas in pulmonary function testing are similar in adults and children, but more specifically will show the areas that are different. The latest standards published by the American Thoracic Society/European Respiratory Society in 2005 have attempted to incorporate some differences for the pediatric population for spirometry, but more work needs to be done in this area. While it is recognized that spirometry is the primary pulmonary function test for children, there are a number of circumstances where the addition of plethysmography and lung diffusion measurements are necessary. The review will state some of the limitations of these tests when performed by children. Lung function testing, particularly spirometry, has much to offer in the diagnosis of lung disease in children and the monitoring of response to therapy. With better standardization of pulmonary function testing in children, and more trained technologists, the age limits for testing can be extended to below 6 years of age and sometimes below 5. Also with better standardization the results obtained are meaningful and when interpreted in context of age offer excellent diagnostic information to better treat the child with lung disease.

Pulmonary Function Testing in Young Children

Pulmonary function testing (PFT) is of great importance in the evaluation and treatment of respiratory diseases. Spirometry is simple, noninvasive, and has been the most commonly used technique in cooperative children, obtaining reliable data in only a few minutes. The development of commercially available equipment as well as the simplification of previous techniques that now require minimal patient cooperation applied during tidal breathing have significantly stimulated the use of PFT in younger children. Tidal breathing techniques such as impulse oscillometry, gas dilution, and plethysmography have permitted previously unobtainable PFT in children 2 to 5years of age. The purpose of this review is to help clinicians become familiar with available PFT techniques used in young children by discussing their general principles, clinical applications, and limitations.

Impact of Endoscopic Sinus Surgery on Pulmonary Function in Children with Cystic Fibrosis

Objective: Determine the impact of endoscopic sinus surgery on pulmonary function in children with cystic fibrosis. Method: Retrospective chart review of cystic fibrosis patients at a tertiary children’s hospital who underwent endoscopic sinus surgery between 1995 and 2010. Main outcome measures include presurgical and 1- and 6-month postsurgical pulmonary function tests (PFTs). Results: Sixty-two children underwent 110 endoscopic sinus surgeries. Pre- and post-PFT data (FEVI and FVC) were available for 66 surgeries. Postoperative, only 10 out of 66 children (15%) at 1 month and 11 out of 66 (16%) at 6 months showed improvement following surgery. A total of 41 out of 66 children (62%) at 1 month and 42 out of 66 (64%) at 6 months showed no difference, while 15 out of 66 (23%) at 1 month and 13 out of 66 (20%) at 6 months were worse following surgery. There was no difference in pulmonary function following primary (28/66 [42%]) or revision (38/66 [58%]) surgeries. Conclusion: Improvement of sinonasal symptoms achieved with endoscopic sinus surgery does not correlate with improvement in pulmonary function as measured by pre- and postoperative pulmonary function testing in children with cystic fibrosis.

Pulmonary Function Testing in Children with Restrictive Chest Wall Disorders.

Disorders of the chest wall, spine, and respiratory muscles lead to lung function abnormalities consistent with restrictive lung disease. Common disorders of the thorax include diseases with respiratory muscle weakness, growth abnormalities of the thorax and spine, such as scoliosis. Spirometry alone is not sufficient to diagnose these abnormalities but may be useful to serially monitor changes over time or in response to treatments. In contrast, obesity does not produce restrictive respiratory disease in most children and produces obstructive lung disease more often. Test of respiratory muscle function, breathing during sleep, and during exercise have led to additional insights about the pathophysiology of these conditions that may provide new avenues for future therapies.

Correlation of Spine Deformity, Lung Function, and Seat Pressure in Spina Bifida

Spinal deformity, a common problem in children with myelodysplasia, is associated with alterations in pulmonary function and sitting balance. Sitting imbalance causes areas of high pressure in patients already at high risk for developing pressure ulcers due to insensate skin. We asked: Does spinal deformity affect pulmonary function tests in children with myelodysplasia? Does the magnitude of spinal curvatures and pelvic obliquity affect seating pressures? Does spinal deformity and seated pressures correlate with a history of pressure ulcers? We retrospectively reviewed 32 patients with myelodysplasia and scoliosis (mean age, 14 years). The mean thoracic scoliosis was 64° with a mean pelvic obliquity of 15°. The mean forced vital capacity was 59% of predicted. The mean of the average and peak seated pressures were 24 and 137 mm Hg, respectively. We examined spinal radiographs, pulmonary function tests, and seated pressure maps and evaluated correlations of spinal deformity measures, pulmonary function, and seated pressures. The thoracic scoliosis inversely correlated with lung volume and weakly related with only the forced midexpiratory volume parameter (R(2) = 31%). The curve magnitude was associated with % seated area with pressures of 38 to 70 mm Hg while lesser degrees of pelvic obliquity were associated with % seating area with pressures of less than 38 mm Hg (R(2) = 25% and 24%, respectively). A history of pressure ulcers did not correlate with any spinal deformity or seated pressure measures. All patients displayed a reduced forced vital capacity, but this reduction was not related to increasing scoliosis. The smaller scoliosis curves and lesser degrees of pelvic obliquity were associated with larger areas of low seated pressures.