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

Risk factors for acute chest syndrome in patients from low socioeconomic background: a cohort study from Sergipe, Brazil.

A prospective cohort study to assess the risk factors for acute chest syndrome (ACS) in individuals with sickle cell disease was carried out in a referral center from Sergipe, Brazil. A total of 168 SS homozygotic individuals (ages between 12 wk and 26 y) were followed for 12 months. There were 134 admissions of 81 patients. There were 50 events of ACS, which was the second most frequent cause of hospital admission (after pain crisis). One patient died of ischemic stroke during follow up. In bivariate analysis, the following variables showed statistically significant associations with the occurrence of ACS: age less than 5 years, living in rural area, history of previous hospital admission; white blood cell count greater than 10,000/dL; hemoglobin concentration less than 7 g/dL and oxygen saturation ≤95% on admission. After controlling for confounding in multivariate logistic regression, only a history of previous admission remained as an independent predictor of ACS (relative risk=4.20; 95% confidence interval: 1.79-9.87; P=0.001). Patients with a positive history of hospital admission are under increased risk and should be monitored closely for prevention and early detection of ACS.

Risk factors for acute chest syndrome in patients from low socioeconomic background: a cohort study from Sergipe, Brazil.

A prospective cohort study to assess the risk factors for acute chest syndrome (ACS) in individuals with sickle cell disease was carried out in a referral center from Sergipe, Brazil. A total of 168 SS homozygotic individuals (ages between 12 wk and 26 y) were followed for 12 months. There were 134 admissions of 81 patients. There were 50 events of ACS, which was the second most frequent cause of hospital admission (after pain crisis). One patient died of ischemic stroke during follow up. In bivariate analysis, the following variables showed statistically significant associations with the occurrence of ACS: age less than 5 years, living in rural area, history of previous hospital admission; white blood cell count greater than 10,000/dL; hemoglobin concentration less than 7 g/dL and oxygen saturation ≤ 95% on admission. After controlling for confounding in multivariate logistic regression, only a history of previous admission remained as an independent predictor of ACS (relative risk=4.20; 95% confidence interval: 1.79-9.87; P=0.001). Patients with a positive history of hospital admission are under increased risk and should be monitored closely for prevention and early detection of ACS.

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...