A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case report

Abstract Background There are some clinical conditions that can impact the accuracy of spO2 measurements, leading to an incorrect diagnosis of hypoxemia. Low oxygen affinity hemoglobinopathy can present with low spO2 and discordance with PaO2 and general clinical conditions. Case Presentation We rep...

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Main Authors: Maria Francesca Patria, Marta Piotto, Cristina Curcio, Silvana Gangi, Youssra Belhaj, Mara Lelii, Barbara Madini, Alessia Rocchi
Format: Article
Language:English
Published: BMC 2025-03-01
Series:Italian Journal of Pediatrics
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Online Access:https://doi.org/10.1186/s13052-025-01900-4
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author Maria Francesca Patria
Marta Piotto
Cristina Curcio
Silvana Gangi
Youssra Belhaj
Mara Lelii
Barbara Madini
Alessia Rocchi
author_facet Maria Francesca Patria
Marta Piotto
Cristina Curcio
Silvana Gangi
Youssra Belhaj
Mara Lelii
Barbara Madini
Alessia Rocchi
author_sort Maria Francesca Patria
collection DOAJ
description Abstract Background There are some clinical conditions that can impact the accuracy of spO2 measurements, leading to an incorrect diagnosis of hypoxemia. Low oxygen affinity hemoglobinopathy can present with low spO2 and discordance with PaO2 and general clinical conditions. Case Presentation We report the case of a 14-month-old male, born extremely premature, with severe bronchopulmonary dysplasia (BPD). He required continuous high-flow nasal cannula (HFNC) oxygen therapy and was hospitalized due to a viral respiratory infection causing dyspnea and desaturation. Once the child clinically recovered, all attempts to wean him off oxygen resulted in rapid drops in SpO2. This occurred despite the absence of increased work of breathing, cyanosis, or abnormal PaO2. Conclusions Severe BPD and recurrent respiratory issues could have per se justified the persistently low spO2. Incongruence between low spO2 values, normal PaO2 levels and good clinical condition, once the child was recovered, raised suspicion of low oxygen affinity hemoglobin (Hb) variants. Abnormal Hb peak detected through high-performance liquid chromatography allowed rare diagnosis of Hb Titusville, confirmed by molecular analysis. In conclusion, the case matched a rare low oxygen affinity hemoglobinopathy (Hb Titusville), highlighting its relevance in unexplained hypoxemia. Diagnosis challenges necessitate a systematic approach to prevent misinterpretations.
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publishDate 2025-03-01
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spelling doaj-art-d09da1c9d11c449db13663f5bc4d6a832025-08-20T03:40:48ZengBMCItalian Journal of Pediatrics1824-72882025-03-015111510.1186/s13052-025-01900-4A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case reportMaria Francesca Patria0Marta Piotto1Cristina Curcio2Silvana Gangi3Youssra Belhaj4Mara Lelii5Barbara Madini6Alessia Rocchi7Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Pediatria PneumoinfettivologiaUniversità degli Studi di MilanoFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Medical Genetics LaboratoryFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Neonatal Intensive Care Unit (NICU)Università degli Studi di MilanoFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Pediatria PneumoinfettivologiaFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Pediatria PneumoinfettivologiaFondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Pediatria PneumoinfettivologiaAbstract Background There are some clinical conditions that can impact the accuracy of spO2 measurements, leading to an incorrect diagnosis of hypoxemia. Low oxygen affinity hemoglobinopathy can present with low spO2 and discordance with PaO2 and general clinical conditions. Case Presentation We report the case of a 14-month-old male, born extremely premature, with severe bronchopulmonary dysplasia (BPD). He required continuous high-flow nasal cannula (HFNC) oxygen therapy and was hospitalized due to a viral respiratory infection causing dyspnea and desaturation. Once the child clinically recovered, all attempts to wean him off oxygen resulted in rapid drops in SpO2. This occurred despite the absence of increased work of breathing, cyanosis, or abnormal PaO2. Conclusions Severe BPD and recurrent respiratory issues could have per se justified the persistently low spO2. Incongruence between low spO2 values, normal PaO2 levels and good clinical condition, once the child was recovered, raised suspicion of low oxygen affinity hemoglobin (Hb) variants. Abnormal Hb peak detected through high-performance liquid chromatography allowed rare diagnosis of Hb Titusville, confirmed by molecular analysis. In conclusion, the case matched a rare low oxygen affinity hemoglobinopathy (Hb Titusville), highlighting its relevance in unexplained hypoxemia. Diagnosis challenges necessitate a systematic approach to prevent misinterpretations.https://doi.org/10.1186/s13052-025-01900-4HypoxemiaHb TitusvilleOxygen therapyHemoglobinopathyBronchopulmonary dysplasia
spellingShingle Maria Francesca Patria
Marta Piotto
Cristina Curcio
Silvana Gangi
Youssra Belhaj
Mara Lelii
Barbara Madini
Alessia Rocchi
A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case report
Italian Journal of Pediatrics
Hypoxemia
Hb Titusville
Oxygen therapy
Hemoglobinopathy
Bronchopulmonary dysplasia
title A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case report
title_full A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case report
title_fullStr A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case report
title_full_unstemmed A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case report
title_short A long-lasting low oxygen saturation hiding Titusville hemoglobin diagnosis in a premature 14-month-old child – case report
title_sort long lasting low oxygen saturation hiding titusville hemoglobin diagnosis in a premature 14 month old child case report
topic Hypoxemia
Hb Titusville
Oxygen therapy
Hemoglobinopathy
Bronchopulmonary dysplasia
url https://doi.org/10.1186/s13052-025-01900-4
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