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1.
J Pediatr Hematol Oncol ; 44(7): 363-368, 2022 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-34966093

RESUMO

Severe hemolytic anemia is a rare complication of glucose-6-phosphate dehydrogenase (G6PD) deficiency. It occurs with the Mediterranean (Med) variant corresponding to a class 2 deficiency according to the World Health Organization (WHO) classification, and it correlates with a severe deficiency in G6PD activity. In Mayotte, the majority of patients have the African (A-) variant as a WHO class 3 deficiency. Yet we have observed numerous cases of severe hemolytic anemia defined by a hemoglobin level of <6 g/dL. In this study, we aimed to describe the epidemiological, clinical, and biological features as well as the treatment modalities of children presenting with a severe hemolytic crisis secondary to G6PD deficiency in Mayotte. The secondary objective was to study the disease genotype when this information was available. Between April 2013 and September 2020, 73 children presented with severe anemia because of G6PD deficiency in Mayotte. The median hemoglobin level during the hemolytic crises was 3.9 g/dL. All of the patients underwent a transfusion and hospitalization. Twenty patients had a disease genotype: 11 had the African mutation and 9 had the Med mutation. Although they are among the most common triggers of G6PD acute hemolytic anemia, drugs were found to not be present and fava bean ingestion was found in only 1 child. One of the specific triggers was traditional medicine, including Acalypha indica . Severe hemolytic crisis in children because of G6PD deficiency is a frequent occurrence in Mayotte. The patients had severe disease symptoms, but the severity did not correlate with the genotype: the African (A-) variant and the Med variant resulted in the same level of disease severity.


Assuntos
Anemia Hemolítica , Deficiência de Glucosefosfato Desidrogenase , Anemia Hemolítica/genética , Criança , Comores , Glucosefosfato Desidrogenase/genética , Deficiência de Glucosefosfato Desidrogenase/complicações , Deficiência de Glucosefosfato Desidrogenase/genética , Hemoglobinas , Hemólise , Humanos
2.
Wilderness Environ Med ; 29(2): 243-247, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29530471

RESUMO

Stingray injuries to the chest are rare but potentially life-threatening. They may occur in remote areas where advanced emergency healthcare services are unavailable. We describe the case of a 24-year-old man with tension pneumothorax due to a Himantura fai stingray injury to the left chest. The chest wound was unremarkable, with no external bleeding or evidence of a foreign body. Decompression was performed at the scene with an improvised knife procedure and a hollow writing pen, which served as a chest tube. At the local hospital, a standard-sized chest tube was inserted, the wound cleaned, and the patient given antibiotics active against marine organisms. Computed tomography visualized the stinger and revealed hemopneumothorax and pneumomediastinum. The local hospital did not have a thoracic surgeon, and the patient was transferred to a larger hospital with a thoracic surgery center. After surgical removal of the stinger, segmental lung resection was required to control bleeding. Management of life-threatening stingray injuries to the chest should begin at the scene. After stabilization, the patient should be transferred to a hospital equipped for cardiovascular and thoracic surgery. Surgery may be required to remove the retroserrated stinger and can be challenging.


Assuntos
Mordeduras e Picadas/cirurgia , Corpos Estranhos/cirurgia , Pneumotórax/cirurgia , Rajidae , Ferimentos Penetrantes/cirurgia , Animais , Mordeduras e Picadas/diagnóstico por imagem , Mordeduras e Picadas/etiologia , Comores , Corpos Estranhos/diagnóstico , Corpos Estranhos/etiologia , Humanos , Masculino , Pneumotórax/diagnóstico , Pneumotórax/etiologia , Resultado do Tratamento , Ferimentos Penetrantes/diagnóstico , Ferimentos Penetrantes/etiologia , Adulto Jovem
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