RESUMO
La drepanocitosis es la anemia hemolítica determinada genéticamente más frecuente en el mundo. En Cuba, la frecuencia del estado de portador es del 3,08 por ciento en la población general. La fisiopatología de la oclusión vascular es muy compleja; involucra la polimerización de la Hb S, las alteraciones de la membrana del hematíe, las moléculas de adhesión, las citocinas inflamatorias, los factores de la coagulación y lesiones del endotelio vascular. Las manifestaciones clínicas más frecuentes son: las crisis vasooclusivas dolorosas, el síndrome torácico agudo, la crisis de secuestro esplénico, la crisis aplástica, la necrosis aséptica de la cabeza del fémur y la úlcera maleolar. El cuadro clínico es muy variable: desde niños que mueren temprano en la vida hasta pacientes que alcanzan la sexta década de la vida. En el Instituto de Hematología e Inmunología existe un Programa de Atención Integral que incluye: seguimiento sistemático desde temprano en la vida en una consulta especializada, la administración de ácido fólico de forma permanente y de penicilina oral profiláctica los primeros 5 años de la vida; así como la educación del niño y de sus padres. Desde 1986 se realiza esplenectomía parcial en la crisis de secuestro esplénico con excelentes resultados. Entre 2004-2008 fallecieron solamente 16 enfermos en todo el país y en 397 adultos la sobrevida fue de 53 años en la anemia drepanocítica y de 58 en la hemoglobinopatía SC. Como resultado de este programa, en los últimos años la sobrevida ha aumentado, la calidad de vida del paciente ha mejorado y han disminuido los costos invertidos en el tratamiento de las complicaciones
Drepanocytosis is the hemolytic anemia more frequent genetically determined in the world. In Cuba, the frequency of carrier status is of 3,08 percent in general population. The pathophysiology of vascular occlusion is very complex; includes the polymerization of the Hb S, the alterations of red-blood cells, the adhesion molecules, the inflammatory cytokines, the coagulation factors and the lesions of the vascular endothelium. The more frequent clinical manifestations are: painful vaso-occlusive crises, the acute thoracic syndrome, the splenic sequestration crisis, the aplastic crisis, the aseptic necrosis of femur head and malleolar ulcer. The clinical picture is very variable: from children dying early in life up to patients achieve the sixth decade of life. In the Institute of Hematology and Immunology there is an Integral Care Program including: systematic follow-up from early in life in a specialized consultation, permanent administration of folic acid and of prophylactic oral penicillin during the first 5 years of age; as well as the child education and of parents. From 1986 it is carried out the partial splenectomy in crises of splenic sequestration with excellent results. Between 2004-2008 in all the country deceased only 16 patients and in 397 adults the survival rate was of 53 years in the drepanocythemia and of 58 in the SC hemoglobinopathy. As result of this program, in past years the survival has increased, the quality of life of patient improved and the costs spent in treatment of complications has decrease
Assuntos
Humanos , Masculino , Feminino , Criança , Anemia Hemolítica/fisiopatologia , Anemia Hemolítica/genética , Assistência Integral à Saúde/métodos , Esplenectomia/métodos , Hemoglobinopatias/complicações , Educação em Saúde/métodosRESUMO
INTRODUCTION: The principal causes of morbidity and mortality during pregnancy in Mexico, are preeclampsia/eclampsia, obstetric hemorrhage and puerperium complications; this is, 62 of maternal deaths in last years. HELLP syndrome was observed between 5 to 25 of the mortality in pregnancies of 36 weeks or less. OBJECTIVE: To analyze patients with HELLP syndrome in ICU's (Intensive Care Unit) of a Gynecology and Obstetric Hospital, related to the abnormal hematological, hepatic and renal results with the obstetric case history and the clinical complications. MATERIALS AND METHODS: A transversal study in patients with HELLP syndrome during 1998 and 1999 were carry out. CASE DEFINITION: Peripheral blood with Microangiopathic hemolysis, elevated liver enzymes: AST, ALT over 40 UI/L, even when were LDH lower than 600 UI/L. It was evaluated the hepatic and renal function, platelets count, microangiopathic hemolysis, arterial pressure, seizures, icteric skin color, blindness, visual disturbances, nausea, vomiting and upper quadrant right abdominal pain. In newborn we analyzed gestational age, sex, weight and APGAR. We studied for an association between maternal and biochemical variables with Correlation Pearson Test, and dependence between variables with lineal regression model. RESULTS: 2878 patients with hypertensives disorders in pregnancy (11.64). The 1.15 (n = 33) had HELLP syndrome with specific maternal mortality of 0.4 per 10,000 live birth, perinatal mortality of 1.62 per 10,000 live birth; and renal damage in 84.5. Coefficient beta was higher between number of pregnancies to platelets count (-0.33) and creatinine clearance (-0.401). CONCLUSION: We found an important renal damage, low platelets, elevated liver enzymes in women with two or more pregnancies. Then we propose there are similarities between HELLP syndrome and Systemic Inflammatory Response Syndrome (SIRS) because they could have the same pathophysiology.