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 Table of Contents  
ORIGINAL ARTICLE
Year : 2019  |  Volume : 17  |  Issue : 3  |  Page : 264-267

Evaluation of pulmonary functions in pediatric patients with beta-thalassemia major


1 Assistant Professor of Pediatrics, Al-Azhar Faculty of Medicine, Damietta, Egypt
2 Lecturer of Chest Diseases, Al-Azhar Faculty of Medicine, Damietta, Egypt
3 Resident at Pediatric Department, Ministry of Health, Kafr El Sheikh, Egypt

Date of Submission09-Mar-2019
Date of Decision12-Apr-2019
Date of Acceptance02-Jun-2019
Date of Web Publication26-Nov-2019

Correspondence Address:
Mokhles Abdel Fadil Zineldin
Al Radwan street, Atay Al Baroud, Beheira, 22951
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AZMJ.AZMJ_46_19

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  Abstract 


Background Thalassemia is a challenging health problem all over the world, especially in Mediterranean areas. Its effect on pulmonary function has not been well studied.
Aim of the work To describe pulmonary function changes in patients with β-thalassemia major.
Patients and methods This study included 60 patients with β-thalassemia major who received regular blood transfusion (40 males and 20 females) and 30 control children (20 males and 10 females). All participants were subjected to full history taking, clinical examination, and laboratory investigations (complete blood count, serum iron, and serum ferritin). In addition, all underwent pulmonary function tests.
Results Forced expiratory volume at first second, forced vital capacity, and forced expiratory flow 25–75% were significantly decreased in males and females in the patient group when compared with those in the control group. However, forced expiratory volume at first second/forced vital capacity was significantly increased in male and female patients when compared with those in the control group. Transfusion and chelation therapy seems to be the cause of reduced pulmonary function.
Conclusion Children with β-thalassemia had reduced pulmonary function, and this could be attributed to chelation therapy that affects serum iron and ferritin.

Keywords: forced expiratory volume, forced vital capacity, pulmonary function, thalassemia


How to cite this article:
Elsehaimy LA, Zineldin MF, Khalil WA. Evaluation of pulmonary functions in pediatric patients with beta-thalassemia major. Al-Azhar Assiut Med J 2019;17:264-7

How to cite this URL:
Elsehaimy LA, Zineldin MF, Khalil WA. Evaluation of pulmonary functions in pediatric patients with beta-thalassemia major. Al-Azhar Assiut Med J [serial online] 2019 [cited 2020 Jul 6];17:264-7. Available from: http://www.azmj.eg.net/text.asp?2019/17/3/264/271679




  Introduction Top


Thalassemia is a major health challenge worldwide, especially in Asia and Mediterranean regions [1],[2]. Each year, ∼23 000 cases of β-thalassemia major are reported in newborns, and ∼80 million are carriers for β-thalassemia worldwide [3]. More than half of infants with α-thalassemia or β-thalassemia require regular blood transfusion, and ∼5500 prenatal deaths are owing to hydrops fetalis caused by thalassemia major [4].

Pulmonary dysfunction as a complication of β-thalassemia major is one of the least understood, although it is not uncommon (∼80.0%) [5]. Pulmonary dysfunction in such patients includes restrictive lung disease and small airway disease. Reported abnormalities are varied and include restrictive and obstructive lung disease, small airway disease, and impaired diffusing capacity of lung for carbon monoxide (DLCO) [6],[7]. However, controversy exists about the nature and pathophysiology of pulmonary dysfunction among patients with thalassemia [8],[9].


  Aim of the work Top


The aim of this study is to describe and quantify the functional changes of the lung in patients with β-thalassemia major and to determine the correlation between pulmonary function test results with hemoglobin, ferritin, and age.


  Patients and methods Top


This study included 60 patients with β-thalassemia major who received regular blood transfusion [40 (67.7%) males and 20 (33.3%) females] and 30 control children [20 (67.7%) males and 10 (33.3%) females]. Patients were selected from Al-Azhar University Hospital in Damietta (30 cases=50%) and the unit of blood diseases at Medical National Institute, Damanhur (30 cases=50%).

Patients with other cardiopulmonary diseases caused by any diseases rather than β-thalassemia major, other blood diseases rather than β-thalassemia major, any musculoskeletal disease, any metabolic disease, and primary (congenital) cardiopulmonary diseases were excluded from the study.

A consent was obtained from each patient and parents before enrollment in the study. In addition, the study protocol was revised and approved by the local research and ethics committee of Al-Azhar University (Faculty of Medicine, Damietta).

All participants were subjected to the following: full history taking, clinical examination, and different investigations. Laboratory hematology included complete blood count, serum iron, serum ferritin, and hemoglobin electrophoresis. In addition, all underwent pulmonary function tests [forced expiratory volume at first second (FEV1), forced vital capacity (FVC), forced expiratory flow 25–75% (FEF 25–75%), and FEV1/FVC% ratio] [10],[11].

Statistical analysis

Data were collected, coded, and statistically analyzed by the Statistical Package for Social Science(SPSS), version 20 (IBM SPSS, Chicago, Illinois, USA). The categorical data were presented as number and percentages, whereas quantitative data with parametric distribution were expressed as mean and SDs, and if nonparametric, the median and interquartile range were calculated. The confidence interval was set to 95% and the margin of error accepted was set to 5%. So, the P value was considered significant if less than 0.05.


  Results Top


Both study and control groups were comparable regarding sex, age, and residence. However, the patient group had a significantly higher heart rate, respiratory rate, and temperature ([Table 1]).
Table 1 Comparison between studied groups regarding demographic and vital data

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Regarding respiratory function, FEV1, FVC, and FEF 25–75% were significantly decreased in the patient group when compared with the control group, in males and females. However, FEV1/FVC was significantly increased in patients when compared with control males or females ([Table 2]).
Table 2 Comparison between patients and controls regarding pulmonary function tests

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Data of males in the study group revealed a significant inverse correlation between transfusions/year and dose of iron chelating agents/day from one side and each of FEV1 percent of predicted and FVC percent of predicted. However, there was positive (proportional) correlation between FEV1/FVC percent of predicted and each of transfusions/year and dose of iron chelating agents/day. In female patients, however, there was an inverse correlation between each of FEV1 percent of predicted and FVC percent of predicted and each of transfusions/year and dose of iron chelating agent/day ([Table 3]).
Table 3 Correlation between pulmonary functions and clinical data in male and female patients

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In male patients of the study group, there was a significant positive correlation between the FEV1/FVC% of predicted and each of hemoglobin, iron, and ferritin. On the contrary, there was a negative (inverse) correlation between iron and ferritin from one side and each of FEV1 percent of predicted, FVC percent of predicted, and FEV 25–75% of predicted. In addition, female patients revealed significant negative correlation between FEV1% of predicted and FVC% of predicted and each of iron and ferritin and between FVC% predicted and total leukocytic count ([Table 4]).
Table 4 Correlation between pulmonary functions and hematological data in male and female patients

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In the present work, there was a statistically significant decrease in all anthropometric measures, weight for age percentile, height for age percentile, and BMI for age percentile in the patient group in comparison with that of the control group. In addition, FEV1 was reduced in 32.5% of males and 60.0% of females, whereas FVC was reduced in 52.5% of males and 75% of females, and finally, FEV 25–75% of predicted was reduced in 12% of males and 40.0% of females. The respiratory function was normal in 50% of males and restrictive lung disease was reported in 50.0%, whereas in females, respiratory function was normal in 20.0%, restrictive lung disease was reported in 75%, and obstructive lung disease was reported in 5.0%.


  Discussion Top


In the present work, all anthropometric measures in patients group were decreased mainly owing to anemia, repeated infection, unstable psychic state owing to repeated hospital admission, continuous administration of drugs, and society behavior toward them. These results are comparable to those reported by Arora et al. [12] who found that the mean height and weight of 30 children with thalassemia were below that of 20 matched controls.

Clinical examination revealed that 63.3% of patients group presented with jaundice, whereas all patients had mongoloid features and hepatomegaly. Ismaeil et al. [13] reported hepatomegaly in 50% of their patients, and this contradiction was attributed to the difference in age group in their study (10–45 years) and different regiment of chelation therapy.

In the present work, 60.0% of studied patients had reduced pulmonary function, where 58.3% had a restrictive pulmonary pattern and 1.6% had a pure obstructive airway disease. Arora et al. [12] reported restrictive abnormality in 86.6% of their cases. None of their patients had an obstructive or mixed pattern of pulmonary dysfunction. In addition, Ismaeil et al. [13] found that 46.7% showed restrictive pulmonary function. Furthermore, Abu-Ekteish et al. [14] reported restrictive pattern in 35% of studied cases. Obstructive airway disease was found in 15%. The differences among the published data may partly reflect the heterogeneity of the studies (different patient age, different iron chelation regimen), and they may also be partly caused by the multifactorial nature of the pathogenesis.

In the present study, reduced FVC was present in 60%, and reduced FEV1 was present in 41%. Ozyoruk and Misirlioglu [11] reported reduced FVC in 67% and reduced FEV1 in 30%. These results are comparable to those of the present work.

Results of correlation in the present work are in contradiction to those reported by Arora et al. [12] who reported that there was no correlation between severity of restrictive lung disease and number of transfusions/year and dose of iron chelating agent/day. This controversy may be owing to difference in number of transfusions/year, dose of iron chelating agent/day, age of patient groups, or sample size. However, their results in male patients agree with that of the present work, where they reported that there was no correlation between the number of transfusions/year, dose of iron chelating agent/day, and FEF 25–75%.

In the present work, no correlation between pulmonary functions and age at diagnosis and duration of illness and jaundice in male and female patients. These results agree with a study done by Arora et al. [12] and disagree with Ismaeil et al. [13] who found that FEV1 and FEF 25–75% have significant negative correlation with age.In the present work, hemoglobin level had no correlation with the predicted percentage of FEV1, FVC, and FEF 25–75%, whereas there was a positive correlation with FEV1/FVC% in male patient. A study done by Ismaeil et al. [13] reported that FEV1 and FEF 25–75% have significant positive correlation with hemoglobin. In addition, a study done by Arora et al. [12] reported that there was no correlation between pulmonary functions and hemoglobin at the time of test.

Serum ferritin had a negative correlation with pred % of FEV1, FVC, and FEF 25–75% and positive correlation with FEV1/FVC (pre%) in male patients, whereas in female patients, it had a negative correlation with pred % of FEV1 and FVC. Ozyoruk and Misirlioglu [11] and Ismaeil et al. [13] reported that FEF 25–75% only had significant negative correlation with ferritin, whereas there was no correlation between serum ferritin and other pulmonary functions. Another study by Abu-Ekteish et al. [14] had shown no correlation between the severity of restrictive or obstructive disease and the serum ferritin level. This difference between the published data and our study may partly reflect the heterogeneity of the studies (different patient age and different iron chelation regimen).

In short, the results of the present work revealed that children with β-thalassemia had reduced pulmonary function, and this could be attributed to chelation therapy that affects serum iron and ferritin.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hossain MS, Raheem E, Sultana TA, Ferdous S, Nahar N, Islam S et al. Thalassemias in South Asia: clinical lessons learnt from Bangladesh. Orphanet J Rare Dis 2017; 12:93.  Back to cited text no. 1
    
2.
Lai K, Huang G, Su L, He Y. The prevalence of thalassemia in mainland China: evidence from epidemiological surveys. Sci Rep 2017; 7:920.  Back to cited text no. 2
    
3.
De Sanctis V, Kattamis C, Canatan D, Soliman AT, Elsedfy H, Karimi M et al. β-Thalassemia distribution in the old world: an ancient disease seen from a historical standpoint. Mediterr J Hematol Infect Dis 2017; 9:e2017018.  Back to cited text no. 3
    
4.
Modell B, Darlison M. Global epidemiology of haemoglobin disorders and derived service indicators. Bull World Health Organ 2008; 86:480–487.  Back to cited text no. 4
    
5.
Carnelli V, D’Angelo E, Pecchiari M, Ligorio M, D’Angelo E. Pulmonary dysfunction in transfusion-dependent patients with thalassemia major. Am J Respir Crit Care Med 2003; 168:180–184.  Back to cited text no. 5
    
6.
Azarkeivan A, Mehrvar A, Pour HS, Mehrvar N, Vosough P. Pulmonary function test in transfusion-dependent beta-thalassemia patients. Pediatr Hematol Oncol 2008; 25:598–606.  Back to cited text no. 6
    
7.
Karimi M, Borzouee M, Mehrabani A, Cohan N. Echocardiographic finding in beta-thalassemia intermedia and major: absence of pulmonary hypertension following hydroxyurea treatment in beta-thalassemia intermedia. Eur J Haematol 2009; 82:213–218.  Back to cited text no. 7
    
8.
Piatti G, Allegra L, Fasano V, Gambardella C, Bisaccia M, Cappellini MD. Lung function in beta-thalassemia patients: a longitudinal study. Acta Haematol 2006; 116:25–29.  Back to cited text no. 8
    
9.
El-Beshlawy A, Youssry I, El-Saidi S, El Accaoui R, Mansi Y, Makhlouf A, Taher A. Pulmonary hypertension in beta-thalassemia major and the role of L-carnitine therapy. Pediatr Hematol Oncol 2008; 25:734–743.  Back to cited text no. 9
    
10.
Fabbri L, Pauwels RA, Hurd SS. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary updated 2003. COPD 2004; 1:105–141.  Back to cited text no. 10
    
11.
Ozyoruk D, Misirlioglu ED. Pulmonary functions in children with thalassemia major. J Pediatr Hematol Oncol 2015; 37:605–610.  Back to cited text no. 11
    
12.
Arora M, Chandra J, Suri JC, Narayan S, Dutta AK. Pulmonary function tests in beta-thalassemia. Indian J Pediatr 2001; 68:239–242.  Back to cited text no. 12
    
13.
Eidani I, Keikhaei B, Rahim F, Bagheri A. Evaluation of pulmonary function in ß-thalassemia major patients. Pak J Med Sci 2009; 25:749–754.  Back to cited text no. 13
    
14.
Abu-Ekteish FM, Al-Rimawi HS, Al-Ali MK, Shehabi IM. Pulmonary function tests in children with beta-thalassemia major. Chron Respir Dis 2007; 4:19–22.  Back to cited text no. 14
    



 
 
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  [Table 1], [Table 2], [Table 3], [Table 4]



 

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