Craniofacial morphology and growth in Muenke syndrome, Saethre-Chotzen syndrome, and TCF12-related craniosynostosis

Abstract Objectives To determine whether the midface of patients with Muenke syndrome, Saethre-Chotzen syndrome, or TCF12-related craniosynostosis is hypoplastic compared to skeletal facial proportions of a Dutch control group. Material and methods We included seventy-four patients (43 patients with Muenke syndrome, 22 patients with Saethre-Chotzen syndrome, and 9 patients with TCF12-related craniosynostosis) who were referred between 1990 and 2020 (age range 4.84 to 16.83 years) and were treated at the Department of Oral Maxillofacial Surgery, Special Dental Care and Orthodontics, Children’s Hospital Erasmus University Medical Center, Sophia, Rotterdam, the Netherlands. The control group consisted of 208 healthy children. Results Cephalometric values comprising the midface were decreased in Muenke syndrome (ANB: β = –1.87, p = 0.001; and PC1: p < 0,001), Saethre-Chotzen syndrome (ANB: β = –1.76, p = 0.001; and PC1: p < 0.001), and TCF12-related craniosynostosis (ANB: β = –1.70, p = 0.015; and PC1: p < 0.033). Conclusions In this study, we showed that the midface is hypoplastic in Muenke syndrome, Saethre-Chotzen syndrome, and TCF12-related craniosynostosis compared to the Dutch control group. Furthermore, the rotation of the maxilla and the typical craniofacial buildup is significantly different in these three craniosynostosis syndromes compared to the controls. Clinical relevance The maxillary growth in patients with Muenke syndrome, Saethre-Chotzen syndrome, or TCF12-related craniosynostosis is impaired, leading to a deviant dental development. Therefore, timely orthodontic follow-up is recommended. In order to increase expertise and support treatment planning by medical and dental specialists for these patients, and also because of the specific differences between the syndromes, we recommend the management of patients with Muenke syndrome, Saethre-Chotzen syndrome, or TCF12-related craniosynostosis in specialized multidisciplinary teams.

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OP0159 EFFICACY OF COMPREHENSIVE TECHNOLOGY-ASSISTED HOME-BASED EXERCISE IN ANKYLOSING SPONDYLITIS: A RANDOMIZED, CONTROLLED TRIAL

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.5100 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 100.3-100

Author(s):

Y. Wang ◽

X. Liu ◽

Y. Shi ◽

X. Ji ◽

W. Wang ◽

...

Keyword(s):

Exercise Intervention ◽

Medical Center ◽

Controlled Trial ◽

Intervention Group ◽

Exercise Program ◽

Group Differences ◽

Control Group ◽

Home Based ◽

The Mean

Background:Clinical practice guidelines recommend that exercise is an essential component in the self-management of Ankylosing Spondylitis (AS). Attending supervised interventions requiring periodic medical center visits can be burdensome and patients may decline participation, whereas, effective home-based exercise interventions that do not need regular medical center visits are likely to be more accessible and acceptable for patients with AS. Recently, increasing evidences have been accumulated that the wearable devices could facilitate patients with inflammatory arthritis by giving exercise instructions and improving self-efficacy. Therefore, patients with AS may benefit from an effective technology-assisted home-based exercise intervention.Objectives:To investigate the efficacy of a comprehensive technology-assisted home-based exercise intervention on disease activity in patients with AS.Methods:This study was a 16-week assessor-blinded, randomized, waiting-list controlled trial (ChiCTR1900024244). Patients with AS were randomly allocated to the home-based exercise intervention group and the waiting-list control group. A 16-week comprehensive exercise program consisting of a moderate intensity (64%-76% HRmax) aerobic training for 30min on 5 days/week and a functional training for 60min on 3 days/week was given to patients in the intervention group immediately after randomization, with 1.5h training sessions for two consecutive days by a study physical therapist at baseline and Week 8. The aerobic exercise intensity was controlled by a Mio FUSE Wristband with a smartphone application. The functional training consisted of the posture training, range of motion exercises, strength training, stability training and stretching exercises. Patients in control group received standard care during the 16-week follow-up and started to receive the exercise program at Week 16. The primary outcome was ASDAS at Week 16. The secondary outcomes were BASDAI, BASFI, BASMI, ASAS HI, peak oxygen uptake, body composition and muscle endurance tests. The mean difference between groups in change from baseline was analyzed with the analysis of covariance.Results:A total of 54 patients with AS were enrolled (26 in intervention group and 28 in control group) and 46 (85.2%) patients completed the 16-week follow-up. The mean difference of ASDAS between groups in change from baseline to 16-week follow-up was −0.2 (95% CI, −0.4 to 0.003, P = 0.032), and the mean change from baseline was -0.4 (95% CI, -0.5 to -0.2) in the intervention group vs -0.1 (95% CI, -0.3 to 0.01) in the control group, respectively. Significant between-group differences were found between groups for BASDAI (−0.5 [95% CI, −0.9 to −0.2], P = 0.004), BASMI (−0.7 [95% CI, −1.1 to −0.4], P <0.001), BASFI (−0.3 [95% CI, −0.6 to 0.01], P=0.035), peak oxygen uptake (2.7 [95% CI, 0.02 to 5.3] ml/kg/min, P=0.048) and extensor endurance test (17.8 [95% CI, 0.5 to 35.2]s, P=0.044) at Week 16. Between-group differences were detected in ASAS HI (−0.9 [95% CI, −1.7 to −0.1], P=0.030), body fat percentage (−1.0 [95% CI, −2.0 to −0.01] %, P=0.048) and visceral adipose tissue (−4.9 [95% CI, −8.5 to −1.4] cm2, P=0.008) at Week 8, but not at Week 16. No significant between-group differences were detected in the total lean mass, time up and go test and the flexor endurance test during the follow-up.Conclusion:Comprehensive technology-assisted home-based exercise has been shown to have beneficial effects on disease activity, physical function, spinal mobility, aerobic capacity, and body composition as well as in improving fatigue and morning stiffness of patients with AS.References:[1]van der Heijde D, Ramiro S, Landewé R, et al. Ann Rheum Dis 2017;76:978–991.Disclosure of Interests:None declared

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Evaluation of depression and self-esteem in children with monosymptomatic nocturnal enuresis: A controlled trial

Archivio Italiano di Urologia e Andrologia ◽

10.4081/aiua.2014.3.212 ◽

2014 ◽

Vol 86 (3) ◽

pp. 212 ◽

Cited By ~ 7

Author(s):

Orhan Koca ◽

Mehmet Akyüz ◽

Bilal Karaman ◽

Zeynep Yesim Özcan ◽

Metin Öztürk ◽

...

Keyword(s):

Children And Adolescents ◽

Nocturnal Enuresis ◽

Depression Scale ◽

Self Esteem ◽

Control Group ◽

Case Group ◽

Healthy Children ◽

Significant Difference ◽

Monosymptomatic Nocturnal Enuresis ◽

Age Range

Objectives: Nocturnal enuresis (NE) is very common and is one of the most common causes for patients to be admitted to urology, pediatrics, child psychiatry and child surgery departments. We aimed to investigate the effect on depression and self-esteem of this disorder that can cause problems on person's social development and human relations. Material and methods: 90 patients who were admitted to our clinic with complaints of nocturnal enuresis were enrolled. Investigations to rule out organic causes were performed in this group of patients. Out of them 38 children and adolescents (age range 8-18 years) with primary monosymptomatic nocturnal enuresis (PMNE) agreed to participate in the study In the same period 46 healthy children and adolescents with a similar age range without bed wetting complaint were included in the study as a control group. The age of the family, educational and socioeconomic level were questioned and Piers-Harris Children's Self-Concept Scale (PHCSCS) and Children's Depression Inventory (CDI) forms were filled out. Results: Mean age of the cases (18 females or 47.4% and 20 males or 52.6%) was 10.76 ± 3.82 years whereas mean age of controls (26 females or 56.5% and 20 males or 43.5%) was 10.89 ± 3.11 years. Depression scale was significantly higher (p = 0.001) in the case group than in the control group (10.42 ± 4.31 vs 7.09 ± 4.35). In both groups there was no statistically significant difference by age and sex in terms of depression scale (p > 0.05). Conclusion: NE is widely seen as in the community and is a source of stresses either for children and for their families. When patients were admitted to physicians for treatment, a multidisciplinary approach should be offered and the necessary psychological support should be provided jointly by child psychiatrists and psychologists.

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Do automatic push notifications improve patient flow in the emergency department? analysis of an ED in a large medical center in Israel

PLoS ONE ◽

10.1371/journal.pone.0258169 ◽

2021 ◽

Vol 16 (10) ◽

pp. e0258169

Author(s):

Daniel Trotzky ◽

Liron Posner ◽

Jonathan Mosery ◽

Aya Cohen ◽

Shiran Avisar ◽

...

Keyword(s):

Large Scale ◽

Medical Center ◽

Patient Flow ◽

Primary Objective ◽

Control Group ◽

Minimal Impact ◽

Notification System ◽

Patient Morbidity ◽

Improve Patient

Introduction Congestion in emergency departments [ED] is a significant challenge worldwide. Any delay in the timely and immediate medical care provided in the ED can affect patient morbidity and mortality. Our research analyzed the use of an innovative platform to improve patient navigation in the ED, as well as provide updated information about their care. Our hope is that this can improve ED efficiency and improve overall patient care. Objective The primary objective of our study was to determine whether the use of an automatic push notification system can shorten ‘length of stay’ (LOS) in the ED, improve patient flow, and decrease ED patient load. Methods This was a prospective cohort study utilizing data extrapolated from the electronic medical records of 2972 patients who visited the walk-in ED of a large-scale central hospital in Israel from January 17, 2021 to March 15, 2021. During this period, the automatic push text notification system was activated on a week-on week-off basis. We compared data from our experimental group with the control group. Results The results of this study indicate that the use of an automatic push notification system had a minimal impact on specific parameters of ED patient flow. Apart from a few significant reductions of specific timed-intervals during patients’ ED visit, the majority of results were not statistically significant. Conclusion This study concluded that the anticipated benefits of a push text notification system in the ED do not, at this stage, justify the system’s additional cost. We recommend a follow-up study to further investigate other possible benefits.

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QT dispersion in acute rheumatic fever

Cardiology in the Young ◽

10.1017/s1047951106000060 ◽

2006 ◽

Vol 16 (2) ◽

pp. 141-146 ◽

Cited By ~ 7

Author(s):

Tugcin Bora Polat ◽

Yalim Yalcin ◽

Celal Akdeniz ◽

Cenap Zeybek ◽

Abdullah Erdem ◽

...

Keyword(s):

Rheumatic Fever ◽

Acute Rheumatic Fever ◽

Qt Dispersion ◽

Control Group ◽

Healthy Children ◽

Rheumatic Carditis ◽

Normal Children ◽

A Value ◽

The Mean

Background:Disturbances of conduction are well known in the setting of acute rheumatic fever. The aim of this study is to investigate the QT dispersion as seen in the surface electrocardiogram of children with acute rheumatic fever.Methods:QT dispersion was quantitatively evaluated in 88 children with acute rheumatic fever. Patients were divided into two groups based on the absence or presence of carditis. As a control group, we studied 36 healthy children free of any disease, and matched for age with both groups. Repeat echocardiographic examinations were routinely scheduled in all patients at 3 months after the initial attack to study the evolution of valvar lesions.Results:The mean QT dispersion was significantly higher in children with rheumatic carditis. But there was no statistical difference between children without carditis and normal children. Among the children with carditis, the mean dispersion was higher in those with significant valvar regurgitation. Dispersion of greater than 55 milliseconds had a sensitivity of 85%, and specificity of 70%, in predicting rheumatic carditis, while a value of 65 milliseconds or greater had sensitivity of 81% specificity of 85% in predicting severe valvar lesions in acute rheumatic carditis. At follow-up examination, a clear reduction on the QT dispersion was the main finding, reflecting an electrophysiological improvement.Conclusions:These observations suggest that QT dispersion is increased in association with cardiac involvement in children with acute rheumatic fever.

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Ophthalmologic Manifestations and Retinal Findings in Children with Down Syndrome

Journal of Ophthalmology ◽

10.1155/2020/9726261 ◽

2020 ◽

Vol 2020 ◽

pp. 1-6

Author(s):

Adem Ugurlu ◽

Emre Altinkurt

Keyword(s):

Down Syndrome ◽

Control Group ◽

Healthy Children ◽

Fiber Layer ◽

Children With Down Syndrome ◽

Department Of Ophthalmology ◽

Significant Difference ◽

Snellen Chart ◽

Ophthalmologic Examination ◽

Age Range

Purpose. To evaluate ocular findings in children with Down syndrome and to compare with the healthy children group. Methods. The study patients were divided into two groups as the diagnosed Down syndrome group and the control group. The study was designed as a prospective and single-center study in Istanbul University Faculty of Medicine Department of Ophthalmology. The study included 93 patients in the age range from 7 to 18 years, who applied to the ophthalmology department of our clinic in the period from July 2017 to June 2018. The study included the patients allocated into the control group and the Down syndrome patients allocated into the patient group, containing 49 and 44 participants, respectively. All patients underwent complete ophthalmologic examination with biomicroscopy. Autorefractometer measurements were performed in all patients, and the best corrected visual acuity (BCVA) was determined with the use of the Snellen chart. All patients underwent spectral domain optical coherence tomography (SD-OCT) measurements for central foveal retinal (CRT), subfoveal choroidal (CCT), and peripapillary retinal nerve fiber layer (pRNFL) thicknesses. Results. The average CRT was 241.2 ± 25.7 microns in Down syndrome group and 219.4 ± 21.1 microns in the control group. There was a statistically significant difference between the groups in regards to CRT (p<0.001). The average pRNFL values were 123.1 ± 15.4 microns in the Down syndrome group and 102.2 ± 8.7 microns in the control group (p<0.001). Conclusions. In the subjects with Down syndrome, the incidence of lens opacities, strabismus, and amblyopia was higher than the control group. CRT and pRNFL were thicker in the Down syndrome group than in control group. This may represent retinal developmental changes in the patients with Down syndrome.

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THERAPEUTIC VALUE OF COMBINED THERAPY WITH DEFERASIROX AND SILYMARIN ON IRON OVERLOAD IN CHILDREN WITH BETA THALASSEMIA

Mediterranean Journal of Hematology and Infectious Diseases ◽

10.4084/mjhid.2013.065 ◽

2013 ◽

Vol 5 (1) ◽

pp. e2013065 ◽

Cited By ~ 14

Author(s):

Adel Abd elhaleim Hagag

Keyword(s):

Iron Overload ◽

Serum Ferritin ◽

Iron Status ◽

Iron Chelator ◽

University Hospital ◽

Beta Thalassemia ◽

Control Group ◽

Healthy Children ◽

Number Of Patients

abstractBackground: Beta thalassemia is an inherited hemoglobin disorder resulting in chronic hemolytic anemia requiring life-long blood transfusion that cause iron overload. Silymarin plays a role as oral iron chelator and hepatoprotective agents in thalassemic patients.The aim of this work was to determine silymarin value as an iron chelator in thalassemic patients with iron overload.Patients and Methods: This study was conducted on 40 children with beta thalassemia major under follow-up at Hematology Unit, Pediatric Department, Tanta University Hospital having serum ferritin level more than 1000 ng/ml and was divided in two groups. Group IA: Received oral Deferasirox (Exjade) and silymarin for 6 months. Group IB: Received oral Deferasirox (Exjade) and placebo for 6 months and 20 healthy children serving as a control group in the period between April 2011 and August 2012 and was performed after approval from research ethical committee center in Tanta University Hospital and obtaining an informed written parental consent from all participants in this research. Results: Serum ferritin levels were markedly decreased in group IA cases compared with group IB (P= 0.001). Conclusion: From this study we concluded that, silymarin in combination with Exjade can be safely used in treatment of iron-loaded thalassemic patients as it showed good iron chelation with no sign of toxicity. Recommendations: Extensive multicenter studies in large number of patients with longer duration of follow up and more advanced methods of assessment of iron status is recommended to clarify the exact role of silymarin in reduction of iron over load in children with beta thalassemia.

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Vitamin D levels in patients with childhood cancer.

Journal of Clinical Oncology ◽

10.1200/jco.2012.30.15_suppl.e20000 ◽

2012 ◽

Vol 30 (15_suppl) ◽

pp. e20000-e20000

Author(s):

Nurdan Tacyildiz ◽

Gulsah oktay Tanyildiz ◽

Deniz Tekin ◽

Can Ates ◽

Handan Dincaslan ◽

...

Keyword(s):

Vitamin D ◽

Inverse Correlation ◽

Control Group ◽

Healthy Children ◽

Vitamin D Levels ◽

Significant Difference ◽

Patient Groups ◽

Cell Growth And Differentiation ◽

Age Range ◽

Nonparametric Correlation

e20000 Background: There is increasing interest in the possible association between cancer incidence and vitamin D through its role as a regulator of cell growth and differentiation. Although there are several studies related cancer risk and prognosis of cancer, related vit D levels in adult patients there are only few recent studies in pediatric patients. Methods: Between 2010 and 2011, D Vit levels of 45 patients (25 boys, 20 girls; age range: 6 months-17 years, median: 11 years) have been compared to D Vit levels of 22 healthy children with similar age group. Patient groups were leukemias, lymphomas, bone tumors, retinoblastoma (RB), and other tumors. Kruskal-Wallis and Spearman nonparametric correlations test of SPSS has been used for statistics. Results: Although there was no statistically significant difference for vit D levels between control group (range: 7.2-22.8; median: 14.75 ng/ml) and patients (range: 5.5-40.2; median: 16.0 ng/ml),difference between patients groups were significant (Table). Patients with RB have statistically lower level of Vit D than leukemia (p:0.016) and lymphoma groups ( p: 0.047). “Other tumors group” has lower vit D levels than leukemia group (p: 0.024). Since RB and other tumors group have younger age than other patients, Spearsman’s nonparametric correlation has been performed to exclude age effect on the results and no statistically significant correlation between D vit levels and age (p: 0.779) was found, although there was an inverse correlation between age and vit D levels in patient group (p: 0.03). Conclusions: According to our preliminary results, most of the patients and healthy children have low level of Vit D. Especially patients with RB have statistically significant lower level of Vit D than other malignancies which can be subject to future studies for confirmation of our results. In addition, etiologic studies related RB may have a new area. [Table: see text]

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Growth and Specialized Growth Charts of Children with Congenital Hypothyroidism Detected by Neonatal Screening in Isfahan, Iran

ISRN Endocrinology ◽

10.1155/2013/463939 ◽

2013 ◽

Vol 2013 ◽

pp. 1-9 ◽

Cited By ~ 3

Author(s):

Awat Feizi ◽

Mahin Hashemipour ◽

Silva Hovsepian ◽

Zeynab Amirkhani ◽

Roya Kelishadi ◽

...

Keyword(s):

Head Circumference ◽

Relative Frequency ◽

Screening Program ◽

Control Group ◽

Growth Charts ◽

Healthy Children ◽

Growth Status ◽

Catch Up ◽

Growth Development

Objectives. The aim of the current study was to investigate the growth status of CH, generate specialized growth charts of CH infants, and compare them with their counterparts of regional normal infants. Methods. In this prospective cohort study, 760 (345 girls and 415 boys) neonates born in 2002–2009 diagnosed by neonatal CH screening program in Isfahan were followed up from the time of diagnosis. 552 healthy children were recruited as a control group. The empirical 3rd, 15th, 50th, 85th, and 97th percentiles for height, weight, and head circumference of both sexes were determined and compared with their counterpart values of the control group. The relative frequency of patients with impaired growth for each studied variable was determined. Also, specialized growth charts of CH patients were generated. Results. The percentiles of weight, height, and head circumference of studied patients are significantly different from regional healthy children (P<0.001). The relative frequency of impaired head circumference was decreased to less than 3% at the 3rd year of age and for height it reached gradually 3% and 9% at the 5th year of age for boys and girls, respectively (P<0.05); however for weight still it was statistically more than 3% in both sexes. Conclusion. CH patients had impaired growth development which was improved during follow up, but the catch-up time was earlier for head circumference and later for weight.

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Subclinical rheumatic valvitis: a long-term follow-up

Cardiology in the Young ◽

10.1017/s104795110300091x ◽

2003 ◽

Vol 13 (5) ◽

pp. 431-438 ◽

Cited By ~ 27

Author(s):

Cristina Costa Duarte Lanna ◽

Edward Tonelli ◽

Marcio Vinicius Lins Barros ◽

Eugenio Marcos Andrade Goulart ◽

Cleonice Carvalho Coelho Mota

Keyword(s):

Mitral Regurgitation ◽

Rheumatic Fever ◽

Aortic Regurgitation ◽

Confidence Intervals ◽

Doppler Echocardiography ◽

The Other ◽

Control Group ◽

Healthy Children ◽

Long Term Follow Up

In order prospectively to investigate the frequency and evolution of subclinical valvitis, we selected 40 consecutive patients suffering their initial attack of rheumatic fever, seen in our clinic from 1992 to 1994, and followed-up until 2001, with a mean period of follow-up of 8.1 years, and a standard deviation of 0.6 year. We also assembled a matched control group of 37 healthy children and adolescents. We discovered a murmur of mitral regurgitation in 28 (70.0%) of the patients. In 3 (7.5%) of these patients, there was also a murmur of aortic regurgitation. In the group of 28 symptomatic patients, Doppler echocardiography showed mitral regurgitation in all, and aortic regurgitation in 17. In the group of 12 patients without clinical evidence of cardiac involvement, Doppler echocardiography identified mitral regurgitation in 2, isolated in one and associated with aortic regurgitation in the other. Thus, the frequency of subclinical valvitis was 16.7%. In patients with subclinical valvitis only the aortic regurgitation regressed during the period of follow-up. In the group of 28 symptomatic patients, mitral regurgitation disappeared in 6 (21.4%), aortic regurgitation in 7 of the 17 having this feature (41.2%), while 2 patients (7.1%) developed mitral stenosis. The sensitivity and specificity of cardiac auscultation were, respectively, 93.3%, with 95% confidence intervals between 72.3% and 97.4%, and 100%, with 95% confidence intervals between 65.5% and 100%, for the diagnosis of mitral regurgitation, and 16.7%, with 95% confidence intervals between 4.4% and 42.3%, and 100%, with 95% confidence intervals between 81.5% and 100%, for that of aortic regurgitation. We conclude that the Doppler echocardiogram is an important means of diagnosing and assessing the evolution of subclinical rheumatic valvar lesions, which are not always transient. We suggest that Doppler echocardiography should be performed in all patients with acute rheumatic fever. Subclinical valvitis should be considered as mild carditis, provided that strict criterions are observed in the differential diagnosis from physiological regurgitation, and Doppler echocardiographic findings are analyzed in the context of the other manifestations of the disease.

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The Eveluation of Pulmonary Functions in Children with Sickle Cell Anemia.

Blood ◽

10.1182/blood.v114.22.4628.4628 ◽

2009 ◽

Vol 114 (22) ◽

pp. 4628-4628

Author(s):

Cem Kurt ◽

Ilgen Sasmaz ◽

Bulent Antmen ◽

Yurdanur Kilinc ◽

Sadi Kurdak ◽

...

Keyword(s):

Patient Group ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Exercise Test ◽

Acute Chest Syndrome ◽

Control Group ◽

Healthy Children ◽

Diffusion Test ◽

Pulmonary Functions

Abstract Abstract 4628 Aim In this study we evaluated to pulmonary functions and determined relations of these findings with clinical parameters in children with sickle cell anemia (SCA) who were at follow up in our pediatric heamatology clinic. Materials and Methods 24 children with sickle cell anemia and 9 healthy children as control group include to the study. Complete blood count, hemoglobin electrophoresis and biochemical values were eveluated for both groups. At pulmonology department, the carbonmonoxide diffusion test performing for both groups. At the same day spirometric respiratory function evaluation and exercise test performed both groups at department of sports physiology. The data recieved are compared statistics. Results HbS, HbF, SGPT, ferritin, total bilirubine, direkt bilirubine and Fe++ values were high at patient group (p<0.05). Hemoglobin and hematocrit values were low at patient group according to control group as expected (p<0.05). The number of SCA patient who had one-three venoocclusive crises (VOC) were 14 (58.3%), patient who had three or more VOC were 7 (29.2%) and patient who had no VOC were 3 (12.5%). The number of patient who had acute chest syndrome (ACS) were 5 (20.9%) and 19 patients had no ACS (79.1%). Ýmpaired isole carbonmonoxide diffusion test was established at the 62.5% of the patient's. At patient group, spirometric FEV1 and MEF25 measurement were found lower than the control group (p<0.05). At exercise test VO2/HR rate were lower for patient group (p<0.05). VE/VO2 rate (p=0.023) and R (p=0.016) measurement were found higher. Conclusion Pulmonary gas transfer was found difficult in patients with SCA. Respiratory airways established obstructed in spirometric evaluation. Obstructive defficiensies have to be follow up. Oxygen pulse and respiratory exchange rates were determined low and more oxygen usage was observed for aerobic metabolic activity. With these results, ýt can be say that chronic inflamation process at lung due to oxygen radicals and hipoksemia in sickle cell patients, the aerobic respiratory load was increased. Disclosures: No relevant conflicts of interest to declare.

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