PMID- 30779248
OWN - NLM
STAT- MEDLINE
DCOM- 20190424
LR  - 20190424
IS  - 1365-3148 (Electronic)
IS  - 0958-7578 (Linking)
VI  - 29
IP  - 1
DP  - 2019 Feb
TI  - Pulmonary functions in Egyptian children with transfusion-dependent 
      beta-thalassemia.
PG  - 55-60
LID - 10.1111/tme.12539 [doi]
AB  - BACKGROUND: In beta-thalassemia, there are varying degrees of ineffective 
      haematopoiesis, intermittent haemolysis and iron overload. Excess iron is 
      deposited in organs such as the heart, the liver, the endocrine glands and the 
      lungs. OBJECTIVES: To evaluate the pulmonary functions in asymptomatic beta 
      thalassemic children on regular transfusion therapy and their relation to iron 
      overload. METHODS: The study included 50 transfusion-dependent beta-thalassemic 
      children and 50 apparently healthy children as control. All children had 
      undergone pulmonary function tests (spirometry, lung volumes and diffusion 
      capacities). In addition, test to determine the mean serum ferritin of the last 
      2 years and pre-transfusion haemoglobin and chest radiograph and echocardiography 
      were performed for the thalassemic children only. RESULTS: A total of 70% of the 
      thalassemic children had diffusion impairment, whereas 34% of them had associated 
      restrictive abnormality. Thalassemic children with serum ferritin >2500 ng mL(-1) 
      had significantly lower values of forced vital capacity (FVC), forced expiratory 
      volume at one second (FEV1), peak expiratory flow (PEFR), total lung capacity 
      (TLC) and diffusing capacity of carbon monoxide (DLCO) (P < 0.05). Only diffusion 
      impairment had a significant positive correlation with serum ferritin level. 
      Restrictive impairment had significant positive correlations with age, duration 
      of blood transfusion and serum ferritin level and a significant negative 
      correlation with duration of chelation (P < 0.05). Having a serum ferritin 
      >2500 ng mL(-1) was the only predicting factor for diffusion impairment and the 
      strongest predicting factor for restrictive dysfunction. CONCLUSION: Despite 
      being asymptomatic, the majority of thalassemic children in this study suffered 
      from diffusion impairment either alone or in combination with restrictive 
      dysfunction. These pulmonary dysfunctions correlated significantly with body iron 
      stores measured by serum ferritin.
CI  - (c) 2019 British Blood Transfusion Society.
FAU - Abd El Hakeem, A A
AU  - Abd El Hakeem AA
AD  - Pediatric Department, Faculty of Medicine, Minia University, El-Minya, Egypt.
FAU - Mousa, S M O
AU  - Mousa SMO
AUID- ORCID: 0000-0002-0024-7914
AD  - Pediatric Department, Faculty of Medicine, Minia University, El-Minya, Egypt.
FAU - AbdelFattah, M T
AU  - AbdelFattah MT
AD  - Chest Diseases Department, Faculty of Medicine, Minia University, El-Minya, 
      Egypt.
FAU - AbdelAziz, A O
AU  - AbdelAziz AO
AD  - Chest Diseases Department, Faculty of Medicine, Minia University, El-Minya, 
      Egypt.
FAU - Abd El Azeim, S S
AU  - Abd El Azeim SS
AD  - Pediatric Department, Faculty of Medicine, Minia University, El-Minya, Egypt.
LA  - eng
PT  - Clinical Trial
PT  - Journal Article
DEP - 20190218
PL  - England
TA  - Transfus Med
JT  - Transfusion medicine (Oxford, England)
JID - 9301182
RN  - 9007-73-2 (Ferritins)
SB  - IM
MH  - Adolescent
MH  - *Blood Transfusion
MH  - Child
MH  - *Echocardiography
MH  - Egypt
MH  - Female
MH  - Ferritins/*blood
MH  - Humans
MH  - *Lung/diagnostic imaging/physiopathology
MH  - *Lung Diseases/blood/diagnostic imaging/therapy
MH  - Male
MH  - Respiratory Function Tests
MH  - *beta-Thalassemia/blood/diagnostic imaging/physiopathology/therapy
OTO - NOTNLM
OT  - diffusion impairment
OT  - pulmonary functions
OT  - restrictive defect
OT  - serum ferritin
OT  - transfusion-dependent thalassemia
EDAT- 2019/02/20 06:00
MHDA- 2019/04/25 06:00
CRDT- 2019/02/20 06:00
PHST- 2016/12/03 00:00 [received]
PHST- 2017/07/12 00:00 [revised]
PHST- 2018/03/28 00:00 [accepted]
PHST- 2019/02/20 06:00 [pubmed]
PHST- 2019/04/25 06:00 [medline]
PHST- 2019/02/20 06:00 [entrez]
AID - 10.1111/tme.12539 [doi]
PST - ppublish
SO  - Transfus Med. 2019 Feb;29(1):55-60. doi: 10.1111/tme.12539. Epub 2019 Feb 18.