Comparison of pulmonary function tests in patients with sickle cell anemia and sickle beta-thalassemia referring to the hematology clinic of Bandar Abbas Shahid Mohammadi Hospital with the control group in 2019-2020

Author(s):  
Arash Rahimi ◽  
Mohammad amin Rashidi ◽  
Abolhasan Rasti

Aim: Due to the high prevalence of sickle cell anemia and beta-shaped sickle cell thalassemia in this region, we decided to conduct this study with the aim of investigating the status of pulmonary function in these patients in Bandar Abbas. Method: The statistical population in this study included all patients with sickle cell anemia and sickle cell beta-thalassemia who had referred to the hematology clinic from 2019-2020, which was equal to 60 people. Also, the workers who had referred to the specialized lung clinic for periodic health tests entered the study by observing the criteria for leaving the review as a control group. Participants were subjected to spirometry and pulmonary volumes were measured. Result: The patients in the case group, 39 (65%) were from sickle cell anemia, and 21 (35%) were from the sickle cell beta-thalassemia. In this study, all the studied parameters had a significant difference between the study group and the control, except for the mean age (p=0.906). In the control group, the values of FEV1 (Forced expiratory volume in 1 second), FVC (Forced vital capacity), and FEF25-75% (Forced expiratory flow 25-75%) are higher than these values in the case group, and this difference is significant (P <0.001). Conclusion: The results of the present study showed that the levels of FEV1, FVC and FEF 25-75% in the control group are higher than these values in the patient group and this difference is significant.

2021 ◽  
Vol 9 (2) ◽  
Author(s):  
Uni Gamayani ◽  
Titin Junaidi ◽  
Nushrotul Lailiyya ◽  
Nur Suryawan ◽  
Nanan Sekarwana

Vitamin B9 (folic acid) and B12 (cobalamin) are essential vitamins that play roles in the process of hematopoiesis and maintaining the function of peripheral nerves. Therefore, these deficiencies may create a risk for peripheral neuropathy in beta-thalassemia major patients. The purpose of this study is to determine the relationship between vitamin B9 level, vitamin B12 level, and peripheral neuropathy in beta-thalassemia major children. It was an observational analytical study with a case-control design has been conducted at Dr. Hasan Sadikin General Hospital Bandung, Indonesia, in May–July 2019. There were 47 beta-thalassemia major children with peripheral neuropathy (case) and 41 healthy children (control). All subjects completed a general demographic questionnaire, underwent neurological examination, and were tested for vitamin B9 and B12 serum levels. Data were then analyzed using the unpaired t test to compare the vitamin levels between both groups and Spearman’s rank correlation test to investigate the correlation between vitamin levels and the number of affected nerves in the case group. Comparison of folic acid levels in the case group (21.52±6.22 ng/mL) and the control group (23.81±7.51 ng/mL) showed no significant difference (p=0.19). In contrast, cobalamin in the case group (288.57±168.61 ng/mL) and the control group (385.95±197.48 ng/mL) showed a significant difference (p=0.01). In addition, there was a moderate correlation (p=0.004, r=0.41) between folic acid level and the number of motoric nerves affected in the case group. In conclusion, cobalamin level correlates with peripheral neuropathy in beta-thalassemia major patients, and folic acid level correlates with the number of affected nerves, especially motoric nerves. HUBUNGAN ANTARA VITAMIN B9 (ASAM FOLAT), VITAMIN B12 (KOBALAMIN), DAN NEUROPATI PERIFER PADA ANAK DENGAN TALASEMIA BETA MAYORVitamin B9 (asam folat) dan B12 (kobalamin) merupakan vitamin esensial yang berperan dalam proses hematopoiesis dan menjaga fungsi saraf tepi. Defisiensi vitamin ini dapat menimbulkan risiko neuropati perifer pada pasien talasemia beta mayor. Tujuan penelitian ini mengetahui hubungan antara kadar vitamin B9, vitamin B12, dan neuropati perifer pada anak talasemia beta mayor. Metode penelitian ini adalah analitik observasional dengan rancangan studi kasus kontrol yang dilakukan di RSUP Dr. Hasan Sadikin Bandung, Indonesia pada Mei–Juli 2019. Terdapat 47 anak talasemia beta mayor dengan neuropati perifer (kelompok kasus) dan 41 anak sehat (kelompok kontrol). Seluruh subjek penelitian mengisi kuesioner demografi umum, menjalani pemeriksaan fisis neurologis, serta dilakukan tes kadar vitamin B9 dan B12 serum. Uji t test tidak berpasangan digunakan untuk membandingkan kadar vitamin pada kedua kelompok dan uji korelasi Spearman untuk membandingkan kadar kedua vitamin tersebut dengan jumlah saraf yang terkena pada kelompok kasus. Perbandingan kadar asam folat kelompok kasus (21,52±6,22 ng/mL) dan kelompok kontrol (23,81±7,51 ng/mL) menunjukkan perbedaan yang tidak bermakna (p=0,19), sedangkan perbandingan kadar kobalamin kelompok kasus (288,57±168,61 ng/mL) dan kelompok kontrol (385,95±197,48 ng/mL) menunjukkan perbedaan yang bermakna (p=0,01). Selain itu, terdapat korelasi sedang (p=0,004; r=0,41) antara kadar asam folat dam jumlah saraf motorik yang terkena pada kelompok kasus. Kesimpulan, kadar kobalamin berhubungan dengan neuropati perifer pada penderita talasemia beta mayor dan kadar asam folat berhubungan dengan jumlah saraf yang terkena, terutama saraf motorik.


2018 ◽  
Vol 23 (2) ◽  
pp. 122-125 ◽  
Author(s):  
Elif Tugba Sarac ◽  
Bilgehan Boke ◽  
Semsettin Okuyucu

Introduction: Sickle cell anemia is a disease characterized by a wide vaso-occlusive incident from micro-vascular incident to muscularactivity. The cochlear function can also get affected by this vaso-occlusion. Objective: It is aimed at determining what kind of effects sickle cell anemia has on hearing and balance system. Methods: This study has been conducted on 46 patients with sickle cell anemia and 45 healthy individuals. For all participants, their pure tone hearing thresholds and videonystagmography (VNG) findings have been determined in 17 frequencies between 125–16.000 Hz. Results: All hearing thresholds between 125 and 16,000 Hz, pure tone averages of patients with sickle cell anemia have been found statistically significant to be higher than the corresponding values in the control group(p < 0.05). The normal hearing rate of patients with sickle cell anemia has been determined to be 71.1% conductive hearing loss (CHL) to be 4.4%, sensorineural hearing loss (SNHL) to be 22.2%, and mixed type hearing loss to be 2.2% in right ear; the normal hearing rate has been determined to be 71.1%, CHL to be 2.2%, SNHL to be 22.2%, and mixed type hearing loss to be 4.4% in left ear. Statistically significant difference has not been found between head shake, spontaneous nystagmus, optokinetic, tracking test batteries, static and dynamic positional tests used in VNG, saccade accuracy and saccade peak velocity, which are saccadic test findings of 2 groups. However, saccadic latency, which is a saccadic test finding, has been determined to be longer in patients with sickle cell anemia in comparison to the control group. Conclusion: While sickle cell anemia causes hearing deficits, it does not have any effect on the central or peripheral vestibular system.


2010 ◽  
Vol 8 (4) ◽  
pp. 414-418 ◽  
Author(s):  
Darcielle Bruna Dias Elias ◽  
Rivelilson Mendes de Freitas ◽  
Romélia Pinheiro Gonçalves ◽  
Hemerson Yuri Ferreira Magalhães ◽  
Jacqueline Holanda de Sousa ◽  
...  

ABSTRACT Objective: To determine the serum levels of malondialdehyde and nitrite in patients with sickle cell anemia treated or not with hydroxyurea in outpatient's setting. Methods: Of the 65 patients with sickle cell anemia selected for the study, 51 were not treated with hydroxyurea (Group 1), 14 made chronic use of hydroxyurea (Group 2) and 20 individuals had no hemoglobinopathies (Control Group). Results: The Control Group had a lower and more homogeneous concentration of malondialdehyde levels as compared to the other groups. The results of Groups 1 and 2 showed increased values of malondialdehyde levels when compared to the Control Group. Considering the values of Groups 1 and 2, there were no significant changes in the malondialdehyde levels. There was no significant difference in the serum levels of nitrite between the groups. Group 2 presented a statistically significant correlation between serum malondialdehyde levels and the clinical variables investigated. In turn, Group 1 showed correlation only with occurrence of three or more vaso-occlusive crises. There was no correlation between nitrite levels and the clinical variables. Conclusion: The results revealed that during the pathogenesis of sickle cell anemia, an increase in lipid peroxidation was observed. On the other hand, no changes in oxidative parameters were detected during treatment with hydroxyurea, probably due to the short period of treatment of the patients studied.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 1435-1435 ◽  
Author(s):  
Elizabeth Record ◽  
Tamara New ◽  
Randall Brown ◽  
LeRoy Graham ◽  
R. Clark Brown

Abstract Background: Pulmonary function is particularly susceptible to acute and chronic injury occurring in patients with sickle cell anemia (SCA). Acute Chest Syndrome (ACS), a common and potentially fatal complication of SCA, can be the cause or the consequence of abnormal pulmonary function (Siddiqui & Ahmed, 2003). Use of hydroxyurea therapy (HU) is increasing for children with recurrent pulmonary complications, following the findings that HU reduces risk of ACS in adults (Charache et al, 1995). How HU may improve pulmonary function in children with SCA is still ill-defined. The purpose of this study was to assess the change observed on serial pulmonary function tests (PFT) for children prescribed HU for abnormal pulmonary function. Methods: Over 240 children with pulmonary complications, such as acute chest syndrome, reactive airway disease, and chronic hypoxia, have been evaluated in the Sickle Cell Pulmonary Clinic at Children’s Healthcare of Atlanta since beginning in July 2000. As part of the Clinic’s standard of care, PFT were routinely attempted on children over the age of 5. A retrospective review of PFT results was done for the children with SCA and prior abnormal PFT. Children were included in the HU cohort (+HU) if repeat PFTs were available following &gt; 3 months on HU therapy and if time on HU did not include frequent RBC transfusions. Children without history of HU therapy (−HU) were selected for comparison. PFTs were performed on a standard plethysmograph. Paired t-test was used to evaluate observed differences. Results: Thirty-one children with prior abnormal PFT had test before and following prescription of HU. Their mean age was 12.6 (range 6–20) years and mean duration of HU was 21 (range 4–47) months at the time of the repeat PFT. Hematologic changes expected on HU occurred for all 31 children. Twenty-four children followed for abnormal PFT had no history of HU (−HU) and matched the +HU group by gender, age (mean 12.3, range 7–19 years), duration followed at time of repeat PFT (mean 19, range 3–66 months; p=0.36), and initial hematologic parameters. Spirometry findings changed for +HU group, and remained stable for −HU group. Mean FVC and FEV1 values improved significantly on HU, when compared to initial (PreHU) PFT and to −HU controls. Conversion to a normal PFT, as interpreted by a pediatric pulmonologist masked to treatment status, occurred in 18 (58%) and 2 (8%) children in the +HU and −HU groups, respectively. Table 1: Serial PFT and hematologic parameters according to HU exposure −HU (n=24) +HU (n=31) Initial Repeat # PreHU Repeat 1 % predicted value for age, sex and height of subject. 2Mean (SD) #No significant changes compared to Initial, p &gt; 0.05. *P value &lt; 0.001 compared to PreHU; ++P value &lt; 0.001 compared to −HU repeat. PFT parameter 1 TLC 88 (13.6)2 85 (11.7) 85 (13.4) 90 (13.5) FVC 78 (9.8) 79 (9.9) 75 (12.6) 90 (13.2)*,++ FEV1 75 (9.6) 74 (12.2) 72 (11.4) 86 (10.2)*,++ FEF 25-75 75 (22) 72 (28.0) 77 (23.4) 79 (26.8) FEV1/FVC (%) 85(6.7) 82 (9.8) 87 (11.8) 86 (11.1) Pulse oximetry (%) 95 (3.1) 96 (2.4) 94 (4.4) 97 (2.8)* WBC (x103/ul) 14 (3.8) 13 (2.9) 13.02 (2.9) 9.0 (2.1) *,++ Hb (g/dl) 8.0 (0.1) 8.0 (0.9) 7.8 (1.1) 9.1 (1.3) *,++ MCV (fl) 84 (9.2) 85 (10.0) 87.3 (8.1) 101.2 (10.1) *,++ HbF (%) 4.3 (1.5) 5.2 (5.2) 6.0 (3.8) 14.0 (7.3) *,++ Conclusions: Serial PFTs showed improved pulmonary function, following initiation of HU therapy in children with prior abnormal PFT. Routine PFT assessments of children prescribed HU may serve as an objective measure of clinical response in children with SCA and pulmonary complications. These results will help in the design of future prospective studies examining the clinical benefits of HU therapy for children at risk for long-term pulmonary complications.


2019 ◽  
Vol 2019 ◽  
pp. 1-7
Author(s):  
Awal Prasetyo ◽  
Arindra Adi Rahardja ◽  
Dhiva Tsuroya Azzahro ◽  
Ika Pawitra Miranti ◽  
Indah Saraswati ◽  
...  

Background. Chronic occupational exposure in textile workers lowers the pulmonary function and levels of sinonasal IgA. A Nephrolepis exaltata herbal mask can protect the respiratory tract. This study aims to understand the effect of this herbal mask on the IgA levels and pulmonary function in textile workers. Thirty employees were selected for this study. Methods. The pre- and post-test randomized experimental control trials were conducted in a garment industry of Bawen, Semarang, Indonesia. The subjects that qualified to participate (n = 30) fulfilled the inclusion criteria i.e., 20–35 years old, healthy, and willing to be a research subject; and exclusion criteria i.e., having history of alcohol consumption, smoking, history of liver disease, autoimmune disease, cancer, pulmonary and heart disease and/or being pregnant. The subjects were then divided randomly into control group (n = 15), who used regular mask that was rewashed and changed every month for eight weeks, and treatment group (n = 15), who used Nephrolepis exaltata mask that was changed every two days for eight weeks. Pulmonary function tests were carried out using MIR Spirolab III before and after the experiment. IgA levels were measured by nasal wash method using ELISA. Results. IgA levels of the treatment group before and after usage of mask were significantly different (p<0.001) compared to the control group. There were significant difference in FVC of the control group, but no significant difference was observed for FEV1 (p=0.507) and PEF (p=0.001). In the treatment group, all three parameters showed significant differences [FVC (p=0.038), FEV1 (p=0.004), and PEF (p=0.001)]. The means of ΔFVC, ΔFEV1, and ΔPEF were significantly (p<0.05) higher in the treatment group with OR = 5.1 for higher IgA levels. Conclusions. The herbal mask is better in increasing IgA and improving the pulmonary function compared to the regular mask.


Author(s):  
Abdul Hamid Khan ◽  
Mehwish Majeed

Background: Corticosteroids are being widely used in conditions related to allergy and inflammation. There are great species differences in the responses to glucocorticoids that mean a “steroid resistant” species. Steroids have profound effect on inflammatory response by way of vasoconstriction, decreased chemotaxis and interference with macrophages. There still are enormous gaps in our knowledge of the action of glucocorticosteroids in patients of chronic obstructive lung disease (COPD).Methods: This study was done in the department of general medicine at SKIMS, Srinagar from December 2017 to December 2018 on patients of chronic obstructive pulmonary disease. A total number of 100 patients were enrolled for the study but 20 patients, 10 from each group lost their follow up. To see the effect of steroids on pulmonary function tests, patients were divided into case and control group. Patients in case group were given prednisolone 30 mg orally for two week (tapering dose). Patients in control group were given placebo for the same duration of two weeks. Steroid response was defined as 15% improvement in baseline forced expiratory volume (FEV).Results: Steroid response was defined as 15% increase in forced expiratory volume in one second/forced vital capacity (FEV1/FVC) after receiving tapering dose of prednisone 30 mg for 2 weeks, no patients in case group showed increase in FEV1/FVC of 15%. The change in pulmonary function tests was comparable in each group (p>0.5).Conclusions: The change in pulmonary function tests were comparable in each group (p>0.5). So, steroids in stable patients of COPD are best to be avoided.


2021 ◽  
Vol 33 (2) ◽  
pp. 127-134
Author(s):  
Josephat M Chinawa ◽  
Awore T Chinawa ◽  
Edmund N Ossai ◽  
Bartholomew F Chukwu ◽  
Ikenna K Ndu ◽  
...  

BackgroundEnumerating the relationship between cardiac structures, function and chamber sizes in children with sickle cell anemia would help in delineating some cardiovascular abnormalities which will aid the Pediatric cardiologist and the cardiac surgeons in a number of decision-making situations.ObjectivesThe objectives of this study are to assess the dimension of cardiac structures and left ventricular function in children with sickle cell anemia in steady state and controls using echocardiography.MethodsA cross-sectional prospective study that assessed cardiac structures and left ventricular function among fifty-one children with sickle cell anemia (HBSS) and compared with fifty children with HB AA type serving as controls.ResultsA significant high proportion of children with sickle cell anemia had abnormal Valvar dimension and left ventricular function above two standard deviations (2-SD) from the mean of the standard population compared to the control group, showing a statistically significant difference (χ2 = 10.42, p= 0.001). All the mean annular valves diameter, left ventricular internal dimension in systole and diastole, inter-sinus distance diameter and sinu-tubular junction diameter are higher in children with sickle cell anemia than controls and this is statistically significant. (p<0.005). ConclusionThis result shows that children with sickle cell anemia have increased valvar size diameter compared with those with normal hemoglobin type. A significantly higher proportion of respondents in type SS group had abnormal left ventricular systolic and diastolic dysfunction when compared with those in type AA group


2020 ◽  
Vol 29 (3) ◽  
pp. 272-280
Author(s):  
Heejeong Son ◽  
Yunah Jeon ◽  
Hyosik Kim

PURPOSE: The purpose of this study was to investigate the effects of training on improvement in pulmonary function and swimming performance by implementing static apnea training for 4 weeks in elite male swimmers.METHODS: Twenty elite swimmers were divided into two groups, the apnea training group (Apnea, n=11) and the control group (control, n=9). Each swimmer received identical swimming and ground training for 4 weeks. The apnea group performed 10 sets of breathholding a day additionally. In all participants, the pulmonary function tests [forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1)] were evaluated. The blood lactate level was also measured after each swimmer completed swimming 5 laps.RESULTS: After 4 weeks of static apnea training, the FVC of the apnea group significantly increased (p=.008), whereas the FEV1/FVC ratio did not change. Breath-holding time increased significantly after apnea training (p=.001). There were no significant differences between the groups in the 50-m time trial records. However, the apnea group show a significant difference at the 4th (p=.013) and 5th trial (p=.023). The blood lactate levels after the 50-m trial was not significantly different between the groups, but the levels in the apnea group showed a significant increase in the results of the 2nd trial compared to those before apnea training (p=.008).CONCLUSIONS: The results of our study reveal that static apnea training improves pulmonary function, in contrast to a few time trials that show varying differences in swimming records and blood lactate levels. These results warrant a review of the training protocol to evaluate the effect on performance. In conclusion, static apnea training is a potential exercise that can improve performance in competitive sports.


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