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Thalassemia Reports
Volume 3
Issue 1
10.4081/thal.2013.e5
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Thalassemia Reports is published by MDPI from Volume 12 Issue 1 (2022). Previous articles were published by another publisher in Open Access under a CC-BY (or CC-BY-NC-ND) licence, and they are hosted by MDPI on mdpi.com as a courtesy and upon agreement with PAGEPress.
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Article

Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension

by
Prapaporn Pornsuriyasak
1,*,
Kulanee Vongvivat
1,
Khanchit Likittanasombat
2,
Thitiporn Suwatanapongched
3 and
Vichai Atichartakarn
4
1
Pulmonary and Critical Care Division, Department of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
2
Cardiology Division, Department of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
3
Department of Diagnostic and Therapeutic Radiology, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
4
Hematology Division, Department of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
*
Author to whom correspondence should be addressed.
Thalass. Rep. 2013, 3(1), e5; https://doi.org/10.4081/thal.2013.e5
Submission received: 6 June 2013 / Revised: 17 October 2013 / Accepted: 18 October 2013 / Published: 8 November 2013

Abstract

The effect of splenectomy on pulmonary function test (PFT) and pulmonary hypertension (PH) in thalassemia remains unclear. We aimed to investigate PFT and their association with PH in patients with hemoglobin E/β-thalassemia stratified by their splenic status. Thirteen splenectomized patients (SP) and 12 non-splenectomized patients (NSP) were compared regarding to the PFT abnormalities and PH (mean pulmonary artery pressure from right-heart catheterization ≥25 mmHg or estimated systolic pulmonary artery pressure from echocardiography ≥40 mmHg). Eleven (84%) SP and 9 (75%) NSP had restrictive impairment (RI). Of these, more patients having severe RI in SP than in NSP (8 vs. 2, P = 0.035). FVC and PaO2 were lower in SP than in NSP (66 ± 15% vs. 77 ± 12%, P = 0.043, and 79.38 ± 1.6 mmHg vs. 98.83 ± 6.2 mmHg, P < 0.001, respectively). Residual volume was higher in SP than in NSP (78 ± 17% vs. 64 ± 15%, P = 0.036). Seven (54%) SP who developed PH had a longer time interval between splenectomy and the onset of PH than those who did not (17 ± 4.9 years vs. 9.8 ± 6.1 years, P = 0.04). In conclusion, greater severity of extrapulmonary restrictive impairment and hypoxemia were more common in SP. These patients developed PH as a late complication unrelated to hypoxemia and PFT parameters.
Keywords: pulmonary function tests; thalassemia; splenectomy; pulmonary hypertension pulmonary function tests; thalassemia; splenectomy; pulmonary hypertension

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MDPI and ACS Style

Pornsuriyasak, P.; Vongvivat, K.; Likittanasombat, K.; Suwatanapongched, T.; Atichartakarn, V. Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension. Thalass. Rep. 2013, 3, e5. https://doi.org/10.4081/thal.2013.e5

AMA Style

Pornsuriyasak P, Vongvivat K, Likittanasombat K, Suwatanapongched T, Atichartakarn V. Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension. Thalassemia Reports. 2013; 3(1):e5. https://doi.org/10.4081/thal.2013.e5

Chicago/Turabian Style

Pornsuriyasak, Prapaporn, Kulanee Vongvivat, Khanchit Likittanasombat, Thitiporn Suwatanapongched, and Vichai Atichartakarn. 2013. "Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension" Thalassemia Reports 3, no. 1: e5. https://doi.org/10.4081/thal.2013.e5

APA Style

Pornsuriyasak, P., Vongvivat, K., Likittanasombat, K., Suwatanapongched, T., & Atichartakarn, V. (2013). Pulmonary Function Abnormalities in Non-Splenectomized and Splenectomized Adult Hemoglobin E/β-Thalassemia Patients and Their Correlation With Pulmonary Hypertension. Thalassemia Reports, 3(1), e5. https://doi.org/10.4081/thal.2013.e5

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