Case Report: Hemoglobin titusville: a specific case of cyanosis

Hemoglobin Titusville is a rare low-oxygen-affinity hemoglobinopathy characterized by normal arterial partial pressure of oxygen but low oxygen saturation. In this study, we describe a case of hemoglobinopathy presenting as cyanosis. A 12-year-old female patient was admitted to the hospital with cya...

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Main Authors: Meng Zhang, Kai Wang, Xinyi Xu, Wei Ji, Ying Guo, Wei Gao, Tingliang Liu
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-06-01
Series:Frontiers in Cardiovascular Medicine
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Online Access:https://www.frontiersin.org/articles/10.3389/fcvm.2025.1537704/full
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author Meng Zhang
Kai Wang
Xinyi Xu
Wei Ji
Ying Guo
Wei Gao
Tingliang Liu
author_facet Meng Zhang
Kai Wang
Xinyi Xu
Wei Ji
Ying Guo
Wei Gao
Tingliang Liu
author_sort Meng Zhang
collection DOAJ
description Hemoglobin Titusville is a rare low-oxygen-affinity hemoglobinopathy characterized by normal arterial partial pressure of oxygen but low oxygen saturation. In this study, we describe a case of hemoglobinopathy presenting as cyanosis. A 12-year-old female patient was admitted to the hospital with cyanosis and 87% blood oxygen saturation. Overall, the patient was healthy and preserved exercise tolerance. Upon admission, her arterial partial pressure of oxygen was 121 mmHg, and her blood oxygen saturation was 89.7%. First, the common causes were excluded, after which cardiovascular catheterization was performed. This detected no shunt between the systemic and pulmonary circulation. Finally, whole-exon sequencing confirmed a heterozygous mutation of the HBA1 gene (g.227115G>A). In this study, we review the entire course of this case, providing scientific insight into this disease and a differential diagnosis of cyanosis.
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publisher Frontiers Media S.A.
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series Frontiers in Cardiovascular Medicine
spelling doaj-art-cf125f3657744a23a50c108dc6ea9e9e2025-08-20T02:07:13ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2025-06-011210.3389/fcvm.2025.15377041537704Case Report: Hemoglobin titusville: a specific case of cyanosisMeng ZhangKai WangXinyi XuWei JiYing GuoWei GaoTingliang LiuHemoglobin Titusville is a rare low-oxygen-affinity hemoglobinopathy characterized by normal arterial partial pressure of oxygen but low oxygen saturation. In this study, we describe a case of hemoglobinopathy presenting as cyanosis. A 12-year-old female patient was admitted to the hospital with cyanosis and 87% blood oxygen saturation. Overall, the patient was healthy and preserved exercise tolerance. Upon admission, her arterial partial pressure of oxygen was 121 mmHg, and her blood oxygen saturation was 89.7%. First, the common causes were excluded, after which cardiovascular catheterization was performed. This detected no shunt between the systemic and pulmonary circulation. Finally, whole-exon sequencing confirmed a heterozygous mutation of the HBA1 gene (g.227115G>A). In this study, we review the entire course of this case, providing scientific insight into this disease and a differential diagnosis of cyanosis.https://www.frontiersin.org/articles/10.3389/fcvm.2025.1537704/fullhemoglobinopathyhemoglobin titusvillehypoxemiacyanosiscardiac catheterization
spellingShingle Meng Zhang
Kai Wang
Xinyi Xu
Wei Ji
Ying Guo
Wei Gao
Tingliang Liu
Case Report: Hemoglobin titusville: a specific case of cyanosis
Frontiers in Cardiovascular Medicine
hemoglobinopathy
hemoglobin titusville
hypoxemia
cyanosis
cardiac catheterization
title Case Report: Hemoglobin titusville: a specific case of cyanosis
title_full Case Report: Hemoglobin titusville: a specific case of cyanosis
title_fullStr Case Report: Hemoglobin titusville: a specific case of cyanosis
title_full_unstemmed Case Report: Hemoglobin titusville: a specific case of cyanosis
title_short Case Report: Hemoglobin titusville: a specific case of cyanosis
title_sort case report hemoglobin titusville a specific case of cyanosis
topic hemoglobinopathy
hemoglobin titusville
hypoxemia
cyanosis
cardiac catheterization
url https://www.frontiersin.org/articles/10.3389/fcvm.2025.1537704/full
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