Late-Life Presentation of Unsuspected G6PD Deficiency

Deficiency of glucose-6-phosphate dehydrogenase (G6PD) is the commonest enzyme deficiency in humans with a wide range of possible clinical manifestations depending on the specific genetic variant in each case. Here we present the case of an 86-year-old male of African descent who acutely developed s...

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Main Authors: Marcos Benchimol, Laura Bernardo Madeira, Ricardo de Oliveira-Souza
Format: Article
Language:English
Published: Wiley 2018-01-01
Series:Case Reports in Critical Care
Online Access:http://dx.doi.org/10.1155/2018/8198565
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author Marcos Benchimol
Laura Bernardo Madeira
Ricardo de Oliveira-Souza
author_facet Marcos Benchimol
Laura Bernardo Madeira
Ricardo de Oliveira-Souza
author_sort Marcos Benchimol
collection DOAJ
description Deficiency of glucose-6-phosphate dehydrogenase (G6PD) is the commonest enzyme deficiency in humans with a wide range of possible clinical manifestations depending on the specific genetic variant in each case. Here we present the case of an 86-year-old male of African descent who acutely developed symptoms of G6PD deficiency immediately after he received methylene blue for treating methemoglobinemia. The contrast between a low SO2 on pulse oximetry and a normal arterial gas sampling raised the possibility of methemoglobinemia. The patient was treated with packed red blood cells and folic acid, and a rapid clinical improvement followed by normalization of the red blood cell count ensued. In view of the patient’s advanced age, the lack of a history of similar episodes in the past, and the normal laboratory results during the hemolytic crisis, this case remained a diagnostic challenge for over three months, when a follow-up measure of G6DP activity eventually confirmed the diagnosis. A latent deficiency of G6PD may become clinically manifest under the appropriate triggering conditions even in elderly patients and in the absence of past or current clinical and laboratory evidence of G6PD deficiency.
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spelling doaj-art-9d27bd9c02854157ada64461cc18b74c2025-08-20T03:38:15ZengWileyCase Reports in Critical Care2090-64202090-64392018-01-01201810.1155/2018/81985658198565Late-Life Presentation of Unsuspected G6PD DeficiencyMarcos Benchimol0Laura Bernardo Madeira1Ricardo de Oliveira-Souza2Hospital Universitário Clementino Fraga Filho (UFRJ), Rio de Janeiro, BrazilHospital Universitário Clementino Fraga Filho (UFRJ), Rio de Janeiro, BrazilThe D’Or Institute for Research & Education (IDOR), Rio de Janeiro, BrazilDeficiency of glucose-6-phosphate dehydrogenase (G6PD) is the commonest enzyme deficiency in humans with a wide range of possible clinical manifestations depending on the specific genetic variant in each case. Here we present the case of an 86-year-old male of African descent who acutely developed symptoms of G6PD deficiency immediately after he received methylene blue for treating methemoglobinemia. The contrast between a low SO2 on pulse oximetry and a normal arterial gas sampling raised the possibility of methemoglobinemia. The patient was treated with packed red blood cells and folic acid, and a rapid clinical improvement followed by normalization of the red blood cell count ensued. In view of the patient’s advanced age, the lack of a history of similar episodes in the past, and the normal laboratory results during the hemolytic crisis, this case remained a diagnostic challenge for over three months, when a follow-up measure of G6DP activity eventually confirmed the diagnosis. A latent deficiency of G6PD may become clinically manifest under the appropriate triggering conditions even in elderly patients and in the absence of past or current clinical and laboratory evidence of G6PD deficiency.http://dx.doi.org/10.1155/2018/8198565
spellingShingle Marcos Benchimol
Laura Bernardo Madeira
Ricardo de Oliveira-Souza
Late-Life Presentation of Unsuspected G6PD Deficiency
Case Reports in Critical Care
title Late-Life Presentation of Unsuspected G6PD Deficiency
title_full Late-Life Presentation of Unsuspected G6PD Deficiency
title_fullStr Late-Life Presentation of Unsuspected G6PD Deficiency
title_full_unstemmed Late-Life Presentation of Unsuspected G6PD Deficiency
title_short Late-Life Presentation of Unsuspected G6PD Deficiency
title_sort late life presentation of unsuspected g6pd deficiency
url http://dx.doi.org/10.1155/2018/8198565
work_keys_str_mv AT marcosbenchimol latelifepresentationofunsuspectedg6pddeficiency
AT laurabernardomadeira latelifepresentationofunsuspectedg6pddeficiency
AT ricardodeoliveirasouza latelifepresentationofunsuspectedg6pddeficiency