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Complicações da drepanocitose: análise retrospectiva de 5 anos numa Unidade de Cuidados Intensivos Pediátricos
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A drepanocitose (DRP) é uma das hemoglobinopatias estruturais mais comuns no mundo e tem predominância na raça negra.1 É causada por uma mutação no gene da globina β. A hemoglobina S (HbS) (α2ß2 6Glu→Val) sofre polimerização reversível quando desoxigenada, formando uma rede gelatinosa de polímeros fibrosos que enrijecem a membrana eritrocitária, aumentam a viscosidade e causam desidratação, dando a forma de foice a estas células. Estas perdem a flexibilidade necessária para atravessar os pequenos capilares provocando episódios imprevisíveis de vaso-oclusão microvascular e são alvo de destruição prematura (anemia hemolítica). Este componente veno-oclusivo habitualmente caracteriza a evolução clínica. As manifestações proeminentes consistem em episódios de dor isquémica (crises álgicas) e disfunção ou franco enfarte esplénico, hepático, pulmonar, cerebral, ósseo e renal. 2 Os recém-nascidos são assintomáticos, com as primeiras manifestações clínicas a aparecerem entre os 4 e 6 meses de vida. 1 São várias as complicações agudas na DRP: crises vaso-oclusivas (CVO), crises aplásticas, sequestro esplênico, infecções por microrganismos capsulados (principalmente do aparelho respiratório e septicemia), síndrome torácico agudo (STA), priapismo, acidente vascular cerebral (AVC), complicações renais, otorrinológicas e músculo-esqueléticas. O conhecimento das intercorrências é de extrema importância visto a detecção precoce das complicações possibilitar o tratamento adequado e diminuição da morbilidade e mortalidade associadas à doença. 3 A prevenção é a chave para o controlo da doença e suas complicações, na qual se encontra o rastreio neonatal, educação dos pais, vacinação e profilaxia antibiótica em doentes com idade inferior a 5 anos. 1 Neste trabalho realizámos uma análise retrospectiva de 5 anos de doentes internados por complicações de drepanocitose na Unidade de Cuidados Intensivos Pediátricos (UCIPed) do Centro Hospitalar Lisboa Norte (CHLN), com um total de 9 crianças entre os 9 meses e 17 anos de idade. A idade, raça, tipos de complicações e suas apresentações clínicas, exames complementares de diagnóstico (ECD), nomeadamente analíticos, medidas de suporte e terapêutica foram semelhantes às descritas em estudos anteriores. As principais complicações identificadas foram STA (44%) priapismo (22%), AVC isquémico (11%), sequestro hepatoesplénico (11%) e hemorragia digestiva alta consequente a hemobilia (11%).
The sickle-cell disease (SCD) is one of the most common worldwide structural hemoglobinopathies and has black breed predominance.1 It is caused by a mutation in the β globin gene. The HbS (α2ß2 6Glu → Val) undergoes polymerization reversible when deoxygenated forming a gelatinous polymer fibrous network that tighten the erythrocyte membrane, increase the viscosity and cause dehydration, giving these sickle cells. The sickle cells lose the flexibility needed to traverse the small capillaries causing unpredictable episodes of vase-microvascular occlusion and are subject to premature destruction (hemolytic anemia). The hemolysis occurs due to abnormal destruction of red blood cells by the spleen. This component veno-occlusive usually characterize the clinical evolution. Prominent manifestations consist of episodes of ischemic pain (pain crises) and ischemic dysfunction or splenic, liver, pulmonary, cerebral, bone and renal infarction.2 Newborns are asymptomatic with the first clinical manifestations appear between 4 and 6 months of life.1 The multiple acute complications on SCD are: vaso-occlusive crisis, aplastic crisis, splenic sequestration, infections by microorganisms encapsulated (mostly respiratory system and sepsis), acute chest syndrome, priapism, stroke, renal, otolaryngologyc and muscle-skeletal disorders. The knowledge of these complications is very important because the early detection allows appropriate treatment and reduction of the morbidity and mortality associated with the disease.3 Prevention is the key to the control of the disease and its complications, which is the neonatal screening, parents education, vaccination and antibiotic prophylaxis in patients with less than 5 years.1 In this paper it is done 5 years retrospective analysis of inpatients with complications from drepanocytosis in pediatric intensive care unit of Centro Hospitalar Lisboa Norte, with a total of 9 children between 9 months and 17 years of age. The age, race, types of complications and their clinical presentations, ECD, including analytical, support and therapeutic measures were similar to those described in previous studies. The main complications identified were acute chest syndrome (44%) priapism (22%), ischemic stroke (11%), hepato-splenic sequestration (11%) and upper gastrointestinal bleeding resulting from hemobilia (11%).
The sickle-cell disease (SCD) is one of the most common worldwide structural hemoglobinopathies and has black breed predominance.1 It is caused by a mutation in the β globin gene. The HbS (α2ß2 6Glu → Val) undergoes polymerization reversible when deoxygenated forming a gelatinous polymer fibrous network that tighten the erythrocyte membrane, increase the viscosity and cause dehydration, giving these sickle cells. The sickle cells lose the flexibility needed to traverse the small capillaries causing unpredictable episodes of vase-microvascular occlusion and are subject to premature destruction (hemolytic anemia). The hemolysis occurs due to abnormal destruction of red blood cells by the spleen. This component veno-occlusive usually characterize the clinical evolution. Prominent manifestations consist of episodes of ischemic pain (pain crises) and ischemic dysfunction or splenic, liver, pulmonary, cerebral, bone and renal infarction.2 Newborns are asymptomatic with the first clinical manifestations appear between 4 and 6 months of life.1 The multiple acute complications on SCD are: vaso-occlusive crisis, aplastic crisis, splenic sequestration, infections by microorganisms encapsulated (mostly respiratory system and sepsis), acute chest syndrome, priapism, stroke, renal, otolaryngologyc and muscle-skeletal disorders. The knowledge of these complications is very important because the early detection allows appropriate treatment and reduction of the morbidity and mortality associated with the disease.3 Prevention is the key to the control of the disease and its complications, which is the neonatal screening, parents education, vaccination and antibiotic prophylaxis in patients with less than 5 years.1 In this paper it is done 5 years retrospective analysis of inpatients with complications from drepanocytosis in pediatric intensive care unit of Centro Hospitalar Lisboa Norte, with a total of 9 children between 9 months and 17 years of age. The age, race, types of complications and their clinical presentations, ECD, including analytical, support and therapeutic measures were similar to those described in previous studies. The main complications identified were acute chest syndrome (44%) priapism (22%), ischemic stroke (11%), hepato-splenic sequestration (11%) and upper gastrointestinal bleeding resulting from hemobilia (11%).
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2016
Palavras-chave
Drepanocitose Células falciformes Crise vaso-oclusiva Anemia hemolítica Priapismo AVC isquémico