COVID-19 and Sickle Cell Disease: a new challenging dilemma in an old disease / COVID-19 e Doença Falciforme: um desafiador dilema em uma doença antiga

Abstract The authors bring reflections about people with sickle cell disease in the pandemic era. They comment on some common clinical situations in these two diseases which may delay or confuse the diagnosis of COVID-19 in patients with sickle cell disease. We consider that people with sickle cell disease are part of the risk group for the complications of COVID-19 and the topic should be addressed in the scientific literature.

Resumo Os autores trazem reflexões sobre as pessoas com doença falciforme na era da pandemia. Eles comentam algumas situações clínicas comuns nessas duas doenças que podem retardar ou confundir o diagnóstico de COVID-19 em pacientes com doença falciforme. Consideramos que as pessoas com doença falciforme fazem parte do grupo de risco para complicações da COVID-19 e o tema deve ser abordado na literatura científica.

Recambio por aféresis de glóbulos rojos. Primer registro de un hospital pediátrico en Quito-Ecuador / Red blood cell exchange. First report in a pediatric Hospital in Quito-Ecuador

Introducción: La anemia falciforme es la hemoglobinopatía estructural más frecuente en todo el mundo y es causada por la producción de hemoglobina S (HbS) a consecuencia de una mutación puntual en el gen de la beta globina.Objetivo: Mostrar los beneficios del recambio de glóbulos rojos por aféresis en la presentación del síndrome torácico agu-do, causado por la anemia drepanocítica.Presentación del caso: Se describe el manejo de una paciente de trece años de edad, con anemia drepanocítica que, al momento de su ingreso al hospital, presentó crisis vaso- oclusiva secundaria a su patología de base. Al segundo día presen-tó síndrome de tórax agudo, por lo que se solicitó al Servicio de Medicina Transfusional, el recambio eritrocitario. Analizado el caso, se realizó el cálculo de la volemia total de la paciente, se prepararon concentrados de glóbulos rojos (CGRs) com-patibles con la paciente: se filtraron; y se les cuantificó el hematocrito. El procedimiento se realizó con el equipo de aféresis COM.TEC. en el que se recambió 1.200 mililitros de eritrocitos totales. Discusión: El recambio eritrocitario por aféresis aportó una notable y visible mejoría clínica y laboratorial. Por lo que en nuestra experiencia consideramos que el procedimiento fue eficiente. Conclusiones: El recambio de eritrocitos por aféresis en el síndrome torácico agudo en crisis drepanocítica es un procedi-miento que se puede utilizar en pacientes que no responden a otras terapias por su mínima alteración de la viscosidad y volumen sanguíneo en el paciente, y disminuir la concentración de hemoglobina S.

Introduction: Sickle-cell anemia is the most common structural hemoglobinopathy worldwide and is caused by the pro-duction of hemoglobin S (HbS) as a result of a point mutation in the beta globin gene.Objective: Show the benefits of red blood cell replacement by apheresis in the presentation of acute chest syndrome, cau-sed by sickle-cell anemia.Case Presentation: We describe the management of a thirteen-year-old patient with sickle-cell anemia, who presented, at admission to the hospital, an occlusive vessel crisis, secondary to her underlying pathology. On the second day of admission, she presented acute chest syndrome. Erythrocyte replacement was requested to the hospital blood service. After analyzing the case, the total blood volume of the patient was calculated, red blood cell concentrates (RBCs) compatible with the pa-tient were prepared, all RBCs were filtered, and the hematocrit was quantified in all RBCs. The procedure was performed with the apheresis equipment COM.TEC. in which a total of 1,200 milliliters of erythrocytes was replaced. Discussion: The erythrocyte replacement by apheresis contributed a remarkable and visible clinical and laboratory impro-vement. In our view, we consider that the procedure was efficient.Conclusions: The replacement of erythrocytes by apheresis in the acute thoracic syndrome in sickle cell crisis is a procedure that may be used in patients who do not respond to other therapies, benefiting from minimal alteration of the viscosity and blood volume in the patient, as well as concomitant decrease of hemoglobin S concentration.

False ground-glass opacity and suspicion of COVID-19, beware of the technique for performing the CT

Ground-glass opacity is a CT sign that is currently experiencing renewed interest since it is very common in patients with COVID-19. However, this sign is not specific to any disease. Besides, the possibility of false positive ground-glass opacity related to insufficient inspiration during the acquisition of the chest CT should be known. We report the case of a 36-year-old patient suspected of COVID-19 and in whom a second acquisition of chest CT was necessary to remove false ground-glass opacities that erroneously supported the diagnosis of COVID-19

Pulmonary function in children and adolescents with sickle cell disease: have we paid proper attention to this problem? / Função pulmonar em crianças e adolescentes com doença falciforme: temos dado atenção adequada a esse problema?

ABSTRACT Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results: Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions: In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

RESUMO Objetivo: Avaliar a função pulmonar e a capacidade funcional em crianças e adolescentes com doença falciforme. Métodos: Estudo transversal com 70 crianças e adolescentes com doença falciforme (8-15 anos), submetidos a testes de função respiratória (espirometria) e de capacidade funcional (teste de caminhada de seis minutos). Os resultados da avaliação da função pulmonar foram comparados com variáveis relacionadas à gravidade da doença falciforme e à presença de história de asma e de síndrome torácica aguda. Resultados: Dos 64 pacientes submetidos à espirometria, 15 (23,4%) apresentaram resultados alterados: distúrbio ventilatório restritivo, em 8; (12,5%) e distúrbio respiratório obstrutivo, em 7 (10,9%). Dos 69 pacientes submetidos ao teste de caminhada de seis minutos, 18 (26,1%) apresentaram resultados alterados na distância em % do previsto para a idade, e houve uma queda ≥ 3% na SpO2 em 36 (52,2%) dos pacientes. Não houve associações significativas entre função pulmonar alterada e as outras variáveis analisadas, exceto para hipoxemia e distúrbio ventilatório restritivo. Conclusões: Observou-se uma significativa prevalência de alterações na função pulmonar nesta amostra de crianças e adolescentes com doença falciforme. A elevada prevalência de distúrbios ventilatórios sugere a necessidade de um olhar mais atento à função pulmonar desde a infância nessa população.

Spinal versus general anesthesia for Cesarean section in patients with sickle cell anemia / 대한마취과학회지

BACKGROUND: Sickle cell anemia (SCA) increases the rate of maternal and fetal complications. This pilot study was designed to compare the maternal and fetal outcomes of spinal versus general anesthesia (GA) for parturients with SCA undergoing cesarean delivery. METHODS: Forty parturients with known SCA scheduled for elective Cesarean delivery were randomized into spinal anesthesia (n = 20) and GA groups (n = 20). Perioperative hemodynamic parameters were recorded. Postpartum complications were followed up. Opioid consumption was calculated. Blood loss during surgery and the number of patients who received intraoperative or postpartum blood transfusion were recorded. Patient satisfaction with the type of anesthesia was assessed. The Apgar score at 1 and 5 min, neonatal admission to the intensive care unit, and mortality were also recorded. RESULTS: Blood loss was significantly higher in the GA than spinal group (P = 0.01). However, the number of patients who received an intraoperative or postpartum blood transfusion was statistically insignificant. Significantly more patients developed intraoperative hypotension and bradycardia in the spinal than GA group. Opioid use during the first 24 h was significantly higher in the GA than spinal group (P < 0.0001). More patients had vaso-occlusive crisis in the GA than spinal group without statistical significance (P = 0.4). There was one case of acute chest syndrome in the GA group. No significant differences were observed in postoperative nausea and/or vomiting, patient satisfaction, or hospital length of stay. Neonatal Apgar scores were significantly better in the spinal than GA group at 1 and 5 min (P = 0.006 and P = 0.009, respectively). Neonatal intensive care admission was not significantly different between the two groups, and there was no neonatal mortality. CONCLUSIONS: Spinal anesthesia may have advantages over GA in parturients with SCA undergoing Cesarean delivery.

Síndrome torácico agudo en niños con drepanocitosis en el Instituto de Hematología e Inmunología / Acute chest syndrome in children with sickle cell disease at the Institute of Hematology and Immunology

Introducción: el síndrome torácico agudo se define como la aparición de nuevas lesiones en la radiografía de tórax de pacientes con drepanocitosis, casi siempre acompañadas de fiebre y manifestaciones respiratorias y es la segunda causa de hospitalización en estos niños. Objetivo: conocer las características clínicas, de laboratorio y el tratamiento utilizado en los episodios de síndrome torácico agudo en niños con drepanocitosis. Métodos: se realizó un estudio ambispectivo, analítico que incluyó 112 episodios de síndrome torácico agudo en 62 pacientes entre 0 y 18 años atendidos en el Servicio de Pediatría del Instituto de Hematología e Inmunología en el período comprendido entre el enero 1 de 2005 y julio 30 de 2012. Resultados: predominaron los pacientes del sexo masculino (58,06 por ciento), y con anemia drepanocítica (67,85 por ciento). El grupo de edad que predominó fue el de 5 - 9 años. El 54,8 por ciento de los niños tuvo un solo episodio y el resto presentó dos o más. La fiebre, el dolor torácico y la tos fueron las principales manifestaciones clínicas al diagnóstico. El 52,0 por ciento de las lesiones radiológicas fueron en la base derecha. Se utilizó terapia transfusional en 83 episodios. Los antibióticos más usados fueron cefotaxima, azitromicina y ceftriaxona. Existió correlación entre el índice de gravedad del episodio y el recuento de leucocitos al ingreso (p = 0.009). No existió mortalidad asociada al síndrome torácico agudo. Conclusiones: el síndrome torácico agudo se presentó en los pacientes estudiados con similares características a lo reportado por otros autores. La correcta educación de pacientes y familiares así como un diagnóstico y tratamiento precoces por un equipo médico especializado, fueron decisivos para que ningún niño falleciera por esta causa

Introduction: acute chest syndrome describes new respiratory symptoms and new pulmonary infiltrate in chest radiograph in patients with sickle cell disease, and is the second most common cause of hospitalization in these children Objectives: to get acquainted with clinical and laboratory features and the treatment used in each episode of acute chest syndrome. Methods: an ambispective and analitic study was conducted involving 112 episodes of acute chest syndrome in 62 patients admitted to the Pediatric Service of the Institute of Hematology and Inmunology from January 1st 2005 through July 30th 2012. Results: the syndrome was more frequent in male children (58,06 percent) between 5 - 9 years old and in sickle cell anemia patients (67,85 percent). Only one episode occurred in 54,8 percent of the children and the rest presented two or more. Fever, chest pain and cough were the main features at diagnosis. X-ray findings showed that the right lung base was involved in 52 percent of the cases. All our patients received antibiotic, mainly cefotaxima, azitromicin and ceftriaxone. In 83 episodes blood therapy was applied. We found statistical correlation between white cell count at diagnosis and the severity of the episode (p = 0.009). No mortality associated to acute chest syndrome occurred. Conclusions: the acute chest syndrome was present in the patients studied with similar characteristics reported by other authors. A correct health education to patientes and family members, together with a precise and early diagnosis and treatment directed by a specialized medical team were significant for the survival of all our patients

Effect of air pollution, contamination and high altitude on bronchial asthma

Epidemiological studies have shown that the prevalence of asthma has risen dramatically worldwide and evidence suggests that environmental factors have an important role in the etiology of the disease. Most respiratory diseases are caused by airborne agents. Our lungs are uniquely vulnerable to contamination from the air we breathe. Air pollution exposure is associated with increased asthma and allergy morbidity and is a suspected contributor to the increasing prevalence of allergic conditions. Observational studies continue to strengthen the association between air pollution and allergic respiratory disease. The effects of air pollution should be viewed in two different groups: healthy people and people with chronic heart or lung disease. Although the fundamental causes of asthma are not completely understood, the strongest risk factors for developing asthma are inhaled asthma triggers. These include: indoor allergens [for example house dust mites in bedding, carpets and stuffed furniture, pollution and pet dander], outdoor allergens [such as pollens and moulds], tobacco smoke and chemical irritants in the workplace. Other triggers can include cold air, extreme emotional arousal such as anger or fear, and physical exercise. Even certain medications can trigger asthma such as aspirin and other non-steroid anti-inflammatory drugs, and beta-blockers. Urbanization has also been associated with an increase in asthma; however the exact nature of this relationship is unclear. Medication is not the only way to control asthma. It is also important to avoid asthma triggers - stimuli that irritate and inflame the airways. Prevalence of asthma is generally low within the Middle East, although high rates have been recorded in the Kingdom of Saudi Arabia, Kuwait, Lebanon, and Israel. The prevalence of asthma and asthma-related symptoms is high among 16- to 18-year-old adolescents in Saudi Arabia, and the symptoms are more common in boys than in girls, associated with a high rate of rhinitis symptoms and hay fever. In addition to bronchial asthma, prevalence of al ergic diseases in a sample of Taif citizens assessed by an original Arabic questionnaire [phase I] evidenced a high prevalence of allergic diseases as Urticaria, allergic rhinitis with or without other co-morbidities, and atopic dermatitis. Effect of high altitude on bronchial asthma is controversial; at high altitudes, the concentrations of the allergens are reduced due to the reduced amounts of vegetation, animal populations and human influences, high UV light exposure and low humidity could be contributing factors to the benefits of high altitude other than allergen avoidance. On the contrary, Lower altitudes have significant beneficial effects for bronchial asthma patients but lessen with increasing altitudes; the mountain climate can modify respiratory function and bronchial responsiveness of asthmatic subjects. Hypoxia, hyperventilation of cold and dry air and physical exertion may worsen asthma or enhance bronchial hyper-responsiveness while a reduction in pollen and pollution may play an important role in reducing bronchial inflammation. Increasing attention has to be paid to the potential of urban air toxics to exacerbate asthma. Continued emphasis on the identification of strategies for reducing levels of urban air pollutants is warranted to reduce respiratory diseases and other diseases related to pollution. Efforts for reducing the asthma burden must focus on primary prevention to reduce the level of exposure of individuals and populations to common risk factors, particularly tobacco smoke, frequent lower respiratory infections during childhood, and environmental air pollution [indoor, outdoor, and occupational]

Síndrome del outlet torácico: una patología siempre quirúrgica? / Thoracic outlet syndrome: an always surgical entity?

Objetivo: el objetivo de este trabajo es analizar los resultados obtenidos en una serie de cirugías realizadas en esta patología. Material y métodos: se analizaron todos los casos de cirugías de nervios efectuados en el período 2003-2012, separando los casos con diagnóstico de outlet torácico operados con un período de seguimiento postoperatorio mínimo de 6 meses. Se buscaron los siguientes datos: edad, sexo, presencia de síntomas sensitivos y/o motores, tipo de outlet (verdadero o disputado), resultado de los estudios neurofisiológicos y de imágenes, resultado de la cirugía, complicaciones postoperatorias y recidivas. Resultados: se incluyeron 31 cirugías realizadas en 30 pacientes, 9 con diagnóstico de OTV (8 mujeres) con un promedio de edad fue de 24.3 años, y 21 con OTD (18 mujeres) de 37.4 años en promedio. Un 90% de todos los casos de outlet presentaron alteraciones neurofisiológicas preoperatorios, y los estadios imagenológicos fueron anormales en 66.6%. Una vez realizada la exploración, el 100% de los OTV presentó una alteración anatómica claramente relacionada con la sintomatología, hecho observado sólo en el 36.7% de los OTD operados. El 87.5% de los OTV mejoraron sus síntomas sensitivos luego de la cirugía, mientras que 77.7% mejoraron desde el punto de vista motor. Por el contrario, 45.4% de los OTD mejoraron permanentemente, 36.3% no tuvieron cambios, 13.6% mejoraron transitoriamente y 405% (un caso) empeoró. Las complicaciones postoperatorias fueron más frecuentes aunque transitorias en el grupo de OTV (3 casos sobre 9 operados, 33.3%) que en los OTD (3 casos sobre 22, un 13.6%). Conclusión: el OTV es una patología infrecuente cuyo tratamiento quirúrgico suele evolucionar favorablemente. En cambio, el OTD constituye un diagnóstico de exclusión y su tratamiento quirúrgico da un resultado bueno aunque inferior al del OTV.

Thrombotic thrombocytopaenic purpura in a patient presenting with sickle cell -hemoglobin S disease vaso-occlusive crises and acute chest syndrome

The association of sickle cell disease [SCA] vaso-occlusive crises and Thrombotic thrombocytopaenic purpura [TTP] has been rarely described. Here we report a case of sickle cell - haemoglobin S disease [HbSS] in which a patient presented with painful vaso-occlusive crises and acute chest syndrome and TTP which was a diagnostic challenge. We also conducted a Medline search to review reported cases previously

Fisioterapia respiratória em crianças com doença falciforme e síndrome torácica aguda / Respiratory therapy in children with sickle cell disease and acute chest syndrome

OBJETIVO:Realizar uma revisão sistemática da literatura médica para identificar as técnicas de fisioterapia respiratória aplicadas em crianças com doença falciforme e síndrome torácica aguda, bem como descrever seu nível de evidência e recomendação. FONTES DE DADOS: Revisão bibliográfica nos bancos de dados Medline, Lilacs, SciELO e Cochrane no período de 1995 e 2009, com os descritores: "doença falciforme", "síndrome torácica aguda", "fisioterapia", "criança", "inspirometria de incentivo", em português e inglês, excluindo-se os estudos de revisão. Os artigos foram classificados por nível de evidência. SÍNTESE DOS DADOS: Foram encontrados cinco artigos; destes, três utilizaram a inspirometria de incentivo e observaram que ela evita as complicações pulmonares associadas à síndrome torácica aguda (nível de evidência II, II e IV), um deles (evidência II) comparou a inspirometria de incentivo com o dispositivo de pressão expiratória, sem diferenças entre ambos. Um artigo utilizou uma rotina de cuidados, incluindo a inspirometria de incentivo (evidência V), e observou redução do tempo de internação hospitalar e do uso de medicação oral para dor. Outro estudo com a ventilação não invasiva para crianças com desconforto respiratório e com incapacidade de realizar a inspirometria de incentivo relatou melhora da oxigenação e do desconforto respiratório (nível de evidência V). CONCLUSÕES: As técnicas de fisioterapia respiratória com dispositivos de inspirometria de incentivo, de pressão expiratória e a ventilação não invasiva podem ser aplicadas em crianças com doença falciforme e síndrome torácica aguda; o nível de recomendação é C.

OBJECTIVE:To systematically review the medical literature to identify chest physiotherapy techniques applied to children with sickle cell disease and acute chest syndrome, and to report their level of evidence and recommendation. DATA SOURCE: A bibliographic search of published articles found in Medline, Lilacs, SciELO and Cochrane databases, between 1995 and 2009, was carried out using the following keywords: "sickle cell disease", "acute chest syndrome", "physical therapy", "child", "incentive spirometry", in English and Portuguese; all review studies were excluded. The recovered studies were then classified according to their level of evidence and recommendation. DATA SYNTHESIS: Five papers were retrieved. Among them, three used incentive spirometry that played an important role in the prevention of pulmonary complications associated with acute chest syndrome (evidence levels II, III and IV); one of these studies (evidence II) compared incentive spirometry versus positive expiratory pressure and did not find differences between them. One paper reported a clinical bundle to improve the quality of care, including incentive spirometry (evidence level V). Incentive spirometry was associated with shorter length of stay and less requirement of oral pain medications. Another study evaluated the effect of non-invasive ventilation on respiratory distress in children that could not perform incentive spirometry and reported improvement in the oxygenation and in the respiratory distress (evidence level V). CONCLUSIONS: Physiotherapy techniques with incentive spirometry device, positive expiratory pressure and non-invasive ventilation can be performed in children with sickle cell disease and acute chest syndrome, with a C recommendation level.

Anemia falciforme e suas manifestações respiratórias / Pulmonary manifestations of sickle cell disease

A doença falciforme é a enfermidade monogênica mais comum no Brasil, sendo uma afecção sistêmica que potencialmente pode afetar vários órgãos e sistemas. O pulmão é um dos órgãos mais acometidos e as complicações na doença falciforme resultam em significante morbimortalidade na faixa pediátrica. Nesse contexto, o presente artigoapresenta as principais manifestações respiratórias da doença falciforme, em virtude da importância do diagnóstico precoce e da abordagem inicial por parte dos pediatras,especialmente da síndrome torácica aguda (STA), da hipertensão arterial pulmonar e da associação com a asma brônquica. O conhecimento por parte dos pediatras da abordagem adequada das manifestações respiratórias citadas no presente artigo de revisão é fundamental para o sucesso do tratamento, sendo também relevante o atendimento inicial adequado e o manejo do procedimento...

The Sickle Cell Disease is the most common inherited genetic disorder in Brazil being asystemic disease that can powerfully affect several organs and systems. The lungs are oneof the most affected and the consequences of the Sickle Cell Disease result in a significant morbid-mortality in pediatric patients and, in this context, the present article presents the main pulmonary manifestations of the stated disease. The article also considers the importance of an early diagnosis and the initial pediatric approach to these manifestations especiallyin: the Acute Chest Syndrome, Pulmonary Hypertension and the association of SickleCell Disease with Asthma. The acknowledgment by the Pediatricians of these respiratory manifestations is fundamental for the success of the treatment, and the initial assessment and adequate management of the procedure are also extremely relevant...

Educación medica continua: manejo del daño pilmonar agudo y del sindrome de dificultad respiratoria aguda

El Síndrome de Dificultad Respiratoria Aguda (SDRA) y la forma leve de la enfermedad, el Daño Agudo Pulmonar (DAP) son enfermedades inflamatoriaspulmonares devastadoras que causan una hipoxemia importante con la consiguiente elevada morbi-mortalidad tanto en adultos como en niños.

Effect of hydroxyurea in children with sickle cell disease in Saudi Arabia

The objective of this study is to demonstrate the effects of hydroxyurea in children with sickle cell disease at Madina Maternity and Children's Hospital and to evaluate its short term safety. This was a retrospective review over two years period from 2004 - 2006. The inclusion criteria were: Children with sickle cell disease who had three or more attacks of painful crisis per year Children with sickle cell disease who had two or more episodes of acute chest syndrome per year. The dose range of Hydroxyurea was 15 - 30 mg/kg/day. The clinical episodes and the laboratory investigations were monitored monthly. The total patients included initially were 14; 4 patients were excluded because of poor compliance to treatment. 10 patients were eligible for the study. 6 were male and 4 were female. 8 patients were Saudi and 2 patients were non Saudis. The age range was 5 - 15 years. The attacks of painful crisis and acute chest syndrome were significantly reduced after Hydroxyurea treatment, also laboratory investigation showed significant increase in MCV and Hemoglobin F values after Hydroxyurea. We conclude that Hydroxyurea is effective in children with sickle cell disease and had no major short term adverse effect. However long terms follow up is required to evaluate long term adverse effect