scholarly journals Pulmonary function tests in children with beta-thalassemia major

2020 ◽  
Vol 35 ◽  
pp. 52-56
Author(s):  
Jayaraj Harsoor ◽  
Vinod H. Ratageri ◽  
C. Shilpa ◽  
Shivanand Illalu ◽  
Prakash Wari
Keyword(s):  
Pulmonary Function ◽  
Serum Ferritin ◽  
Respiratory Symptoms ◽  
Pulmonary Function Tests ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Pulmonary Dysfunction ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
Function Tests

Objectives: The objective of the study was to study the pattern of lung functions in thalassemia major children and correlation of pulmonary function tests (PFTs) with serum ferritin. Materials and Methods: A hospital-based cross-sectional descriptive study done from January 2017 to December 2017. Inclusion criteria: Children with confirmed diagnosis of beta-thalassemia major in the age group of 5–15 years were included in the study. Exclusion criteria: Already diagnosed cases of pulmonary dysfunctions, CHD and RHD were excluded from the study. All enrolled children underwent a detailed clinical history, physical examination and blood sample were sent for Hb and serum ferritin before blood transfusion (BT). PFT was done within 24 h of BT using spirometer (Helios-401). Statistical analysis was done using SPSS (Version22). Results: Forty-five children enrolled in the study and majority of them were <10 years (37 children) with M:F ratio 1.6:1. The pulmonary dysfunction was present in 35 (77.8%), but none of them had respiratory symptoms. The pulmonary dysfunction observed was restrictive 31 (88.5%), obstructive 2 (5.7%), and combined 2 (5.7%). A reduced forced vital capacity (FVC) % in 33 (73.3%), a reduced forced expiratory volume in the 1st second (FEV1%) in 25 (55.5%), a normal FEV1/FVC in 41 (91.2%), and a reduced FEF 25–75% in 23 (51.1%) children were observed. Risk factors such as, age, height, and duration of chelation (>5 years) were significantly associated with pulmonary dysfunction (P < 0.05). There was no correlation between serum ferritin levels and PFT. However, PFT values were found to be decreased in patients with a high serum ferritin (>2500 ng/ml), but these differences were statistically not significant. Conclusion: Abnormal patterns of lung function were common (restrictive type, predominant), even though none of these children had any respiratory symptoms.

Pulmonary Dysfunction in Beta Thalassemia.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3851-3851
Author(s):  
Amit Kandhari ◽  
Anupam Sachdeva ◽  
Neeraj Jain ◽  
Subash C. Arya ◽  
Virender K. Khanna ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Serum Ferritin ◽  
Pulmonary Function Tests ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
P Value ◽  
Age Group ◽  
Pulmonary Dysfunction ◽  
Function Tests ◽  
Abnormal Results

Abstract Thalassemia is one of the commonest hereditary hemolytic anemia. Survival of patients with thalassemia major has greatly improved during recent years, with the introduction of moderate -transfusion programs and chelation therapy with subcutaneous Desferal (deferrioxamine) and/or Kelfer (Deferiprone). The repeated transfusions in thalassemics lead to deposition of iron in different tissues, leading to damage and dysfunction of various systems. Iron deposition is also reported to exist in the lungs at autopsy. Although lung impairment in thalassemic patients has been reported in the 1980’s, yet it is one of the most under evaluated and functionally not well-characterized complication. Since there have been contradictory results reported in literature ranging from restrictive spirometric pattern to an obstructive one, and there is total paucity of data from India, this study was designed to find out the pulmonary function tests of the beta thalassemic patients attending our hospital. A total of 30 cases of beta thalassemia major who were attending the thalassemia clinic at Sir Ganga Ram Hospital were taken up for the study after a written informed consent. These patients were on moderate -transfusion regimen, maintaining a hemoglobin concentration of more than 9 gm %. Patients with asthma and cardiac disease were excluded. Pulmonary function tests done included Forced vital capacity (FCV), Forced expiratory volume in one second (FEV1), FEV1/FVC percent (FEV1 %) and peak expiratory flow rate (PEFR). Results were interpreted as obstructive, restrictive and normal pulmonary Function test (PFT). The data was analyzed using software SPSS version 10. We included three age groups in our study, with total 30 patients in all. Age group 6–10 yr had 9 patients (mean Serum ferritin 2301 ng/ml), 11–15 yr had 11 children (mean Serum ferritin 3315) and &gt;15 yr had 11 children (mean Serum ferritin 5147 ng/ml) in the study. Mean age was 14.46 with standard deviation of 6.75 (mean ferritin was 3682 ng/ml). Overall out of 30 patients 13 had normal PFT and 17 had abnormal PFT (1 had obstructive PFT and 16 had restrictive PFT). In the age group of 6–10 years 7 had normal and 2 had abnormal PFT and in age group 11–15 yr 3 had normal and 6 had abnormal PFT and in age group &gt;15 yr 3 had normal and 8 had abnormal PFT. Of 17 patients on chelation with Desferal alone 11 had normal and 6 had abnormal PFT. 6 patients were on Kelfer alone of which 1 had normal and 5 had abnormal PFT. Of 7 patients who were on both Desferal and Kelfer, 1 had normal and 6 had abnormal PFT. All patients on Kelfer or Kelfer + Desferal aged &gt;10 years except one which may explain reason for increased number of abnormal results in this group. Critical age above which abnormal results were higher was 11.5 years. Mean Ferritin in patients with normal PFT was 2456 ng/ml and 4621 ng/ml for patients with abnormal PFT. (p value 0.037) We found a negative Pearson correlation between FVC (% of predicted) with increasing ferritin (p value 0.038). There was no difference detected between pre and post transfusion PFT results by using paired t test (p value 0.973). In conclusion the major pulmonary dysfunction in our study group is that of restrictive type. The abnormal PFT were found in older age group. FVC was negatively co-related with increasing ferritin level. The number of abnormal results was higher when the serum ferritin was &gt; 4000 ng/ml and blood transfusion had no effect on the PFT.

scholarly journals Study of pulmonary function tests in multitransfused children with beta thalassemia major: an observational study

2020 ◽  
Vol 7 (12) ◽  
pp. 2307
Author(s):  
Kavya Shivaswamy ◽  
Somashekhar Chikkanna ◽  
Jagdish Singh ◽  
Keerthana T. N. Gubbari
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Beta Thalassemia Major ◽  
Function Tests ◽  
Asymptomatic Patients ◽  
Multiple Blood ◽  
Age And Sex

Background: Thalassemia is a hemoglobinopathy requiring multiple blood transfusions leading to iron deposition in various organs. Abnormalities in pulmonary function in thalassemia major patients have been found in various studies. Our aim was to study pulmonary function tests (PFT) in multitransfused children with beta thalassemia major.Methods: PFT was done in forty beta thalassemia major (TM) patients and forty normal healthy age and sex matched individuals and the results were analyzed.Results: Out of 40 thalassemia major patients, 20 (50%) had normal PFT and the rest 20 (50%) had an abnormal PFT out of which 18 (90%) had restrictive PFT and 2 (10%) had obstructive PFT.  Out of 40 controls, all 40 (100%) had normal PFT.Conclusions: Alteration of pulmonary function can be seen even in well-chelated TM patients. The respiratory system should be evaluated annually by PFTs even in asymptomatic patients to prevent pulmonary sequelae.

Pulmonary function tests in children with beta-thalassemia major

2007 ◽  
Vol 4 (1) ◽  
pp. 19-22 ◽  
Author(s):  
F M Abu-Ekteish ◽  
H S Al-Rimawi ◽  
M K Al-Ali ◽  
I M Shehabi
Keyword(s):  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Beta Thalassemia Major ◽  
Function Tests

scholarly journals Pulmonary function tests in β thalassemia major and its correlation with serum ferritin levels

2019 ◽  
Vol 6 (2) ◽  
pp. 306
Author(s):  
Manaswita Gadiparthi ◽  
Nalini Bhaskaranand ◽  
Pushpa G. Kini ◽  
Shrikiran Hebbar ◽  
Suneel C. Mundkur
Keyword(s):  
Pulmonary Function ◽  
Serum Ferritin ◽  
Ferritin Level ◽  
Pulmonary Function Tests ◽  
Demographic Data ◽  
Thalassemia Major ◽  
Forced Expiratory Volume ◽  
Function Tests ◽  
Lung Dysfunction ◽  
Restrictive Pattern

Background: Extensive studies are conducted on heart, liver and endocrine abnormalities in thalassemia owing to their direct effect on survival, however, lung dysfunction has never been focused upon and is one of the least understood complications in β thalassemia. There’s a vacuum for data on pulmonary function tests in β thalassemia major in literature from India. Authors aimed to study pulmonary function and type of abnormality in cases with β thalassemia major above the age of 8yrs and to correlate the result with age and serum ferritin levels.Methods: Demographic data, hemoglobin value, serum ferritin levels, chelation details and transfusion requirement were analyzed. Spirometry was performed using COSMED pulmonary function test (PFT).Results: Among the 34 subjects studied, 21 were boys, and 13 were girls. Mean serum ferritin levels of the group was 3610.82±2679.51ng/mL and did not show a significant correlation with age, years of transfusion, and years of chelation. Forced vital capacity (FVC), forced expiratory volume in 1st second (FEV1) % values were lower in boys when compared to girls. PFT showed a restrictive pattern in the study group (FEV1/FVC=>0.7) with significant involvement in 73.5% of cases (FEV1<80%). A statistically significant negative correlation was observed between age and FEV1% (r=-0.577, p=<0.01) highlighting the importance of duration of iron overload. However, there was no significant linear correlation between restrictive lung disease and serum ferritin level (r=-0.06, p=0.75).Conclusions: Restrictive pattern was the most common abnormality, and it did not correlate with serum ferritin. Pulmonary function monitoring would help in identifying children with significant morbidity and help in initiating an early intervention to improve the quality of life.

scholarly journals Pulmonary dysfunction in children with beta thalassemia major in relation with iron overload - a cross sectional hospital based study

2015 ◽  
Vol 6 (5) ◽  
pp. 47-50 ◽  
Author(s):  
A Boddu ◽  
A Kumble ◽  
S Mahalingam ◽  
BS Baliga ◽  
B Achappa
Keyword(s):  
Iron Overload ◽  
Serum Ferritin ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
P Value ◽  
Pulmonary Dysfunction ◽  
Cross Sectional ◽  
Transfusion Therapy ◽  
Respiratory Dysfunction ◽  
Respiratory Impairment

Background: Previously many patients with thalassemia major died from severe anemia during first decade, but with modern transfusion therapy many now survive beyond childhood. Because of such therapy, however excessive iron deposition occurs in lungs and causing respiratory dysfunction. Respiratory impairment occurs because of defective chelation and repeated transfusions. Objectives: To a) study pattern of respiratory impairment using spirometry, b) estimate iron overload by measuring serum ferritin levels, c) correlate a&b. Methodology:Thalassemia children >7year, on regular blood transfusion were included in the study after getting institutional ethical clearance .Standardised pulmonary function test was done using spirometry. Iron overload was assessed using serum ferritin levels. Severity of pulmonary dysfunction was correlated with serum ferritin levels. Results:Total of 42 children were included 62% were males and 38% were females (with median age 12yrs). By spirometry 95% had restrictive pattern of respiratory dysfunction. Mean ferritin value was 4152. Out of them10 (23.8%) mild, 25(59%) moderate and 5(12%) severe dysfunction based on FEV1 and FVC. The mean ferritin values in severe respiratory dysfunction is 6275 which is significantly higher when compared to moderate (4249) and mild (3066) pattern of respiratory dysfunction. None of the children had evidence of CCF. Significant correlation (p value=0.003) was found between severity of pulmonary dysfunction with ferritin values and also with weight of the child (p value=0.007). No other significant correlation found between severity pattern and transfusion index, age or height. Conclusion:Restrictive pattern is most common pulmonary dysfunction seen in chronic iron overloaded thalassemia major children. Regular blood transfusions with adequate chelation decrease incidence of pulmonary dysfunction. Screening of all thalassemia children using spirometry is need of the hour. DOI: http://dx.doi.org/10.3126/ajms.v6i5.11782Asian Journal of Medical Sciences Vol.6(5) 2015 47-50

scholarly journals Iron chelation therapy needed for serum ferritin overloaded patients of beta thalassemia major

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Wasim Muhammad ◽  
Muhammad Ishaq ◽  
Muhammad J. Khan ◽  
Umair Ahmad ◽  
Muhammad Waseem
Keyword(s):  
Serum Ferritin ◽  
Ferritin Level ◽  
Iron Chelation ◽  
Care Facility ◽  
Health Care Facility ◽  
Thalassemia Major ◽  
Beta Thalassemia ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
Khyber Pakhtunkhwa Province

The main objective of the current study is to evaluate the level and overload of serum ferritin in multi-transfused beta Thalassemia major patients. There is an earnest need to defend the chelation treatment and to make mindfulness about the results of serum ferritin in the patients beta Thalassemia major. This is a Cross sectional analytical study performed in Fatimid foundation Hayatabad, Peshawar, Khyber Pakhtunkhwa province of Pakistan. Those patients who has beta thalassemia major are included in this study. In this study there are total 108 patients in which 54 males and 54 females. The highest mean of serum ferritin level in the category of male was in the age of 12 years were finds 8160.5 ng/mL. Among the female the highest mean of ferritin level was in the age of 17 years were finds 13,349.5 ng/mL. In this study majority of patient’s revealed much high levels of serum ferritin. These levels reveal insufficient chelation. Appropriate chelation of iron load can improve the quality of the life of these patients. The low level of education, Poverty problems, and insufficient health care facility of are the main obstacle in the effective management of ferritin overload in thalassemia patients.

scholarly journals Respiratory symptoms, asthma, exercise test spirometry, and atopy in schoolchildren from a Lima shanty town

Thorax ◽  
2001 ◽  
Vol 56 (8) ◽  
pp. 607-612
Author(s):  
M E Penny ◽  
S Murad ◽  
S S Madrid ◽  
T S Herrera ◽  
A Piñeiro ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Respiratory Symptoms ◽  
Pulmonary Function Tests ◽  
Forced Expiratory Volume ◽  
Cross Sectional Survey ◽  
Cross Sectional ◽  
Asthma Symptoms ◽  
Function Tests ◽  
Asthma Attack ◽  
Shanty Town

BACKGROUNDLittle is known about the associations between symptoms of asthma, pulmonary function tests, and atopy in developing countries. While asthma in children is often associated with atopy, some studies of wheezing illness have found little or no association, leading to suggestions that there are subgroups of wheezing illness. The ISAAC study recently reported that the prevalence of reported asthma symptoms in Lima, Peru was among the highest in the world, but did not report on the atopic status of the subjects.METHODSA cross sectional survey was conducted of children aged 8–10 years who had previously participated in a cohort study of respiratory and diarrhoeal illnesses in infancy. Questionnaires were administered asking about respiratory symptoms and asthma diagnoses, pulmonary function tests were performed before and after exercise on a treadmill, and atopy was determined from skin prick tests and specific serum IgE levels.RESULTSA total of 793 children participated in the survey. The prevalence of asthma related symptoms in the last 12 months was 23.2%, but only 3.8% of children reported a recent asthma attack. The mean differences in pretest percentage predicted forced expiratory volume in one second (FEV1) were 8.1% (95% CI 2.4 to 13.8) between children who did and did not report an asthma attack in the last 12 months, and 5.3% (95% CI 2.8 to 7.9) in children who did and did not report respiratory symptoms. The corresponding differences in mean percentage fall in FEV1 after exercise were 3.1% (95% CI –1 to 7.1) and 5.1% (95% CI 3.4 to 6.8). Recent asthma or respiratory symptoms were not associated with atopy in this population (odds ratios 1.29 (95% CI 0.56 to 2.97) and 0.91 (95% CI 0.61 to 1.37), respectively).CONCLUSIONSMost asthma in these children was unrecognised and mild. Asthma and asthma symptoms in this population do not seem to be related to atopy.

scholarly journals A cross-sectional study of metabolic and endocrine complications in beta-thalassemia major

2008 ◽  
Vol 28 (5) ◽  
pp. 361 ◽  
Author(s):  
Farzad Najafipour ◽  
Akbar Aliasgarzadeh ◽  
Naser Aghamohamadzadeh ◽  
Amir Bahrami ◽  
Majid Mobasri ◽  
...  
Keyword(s):  
Thalassemia Major ◽  
Cross Sectional Study ◽  
Beta Thalassemia ◽  
Sectional Study ◽  
Cross Sectional ◽  
Beta Thalassemia Major ◽  
Endocrine Complications
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