Morbimortalidade da anemia falciforme: uma revisão de literatura

Abstract

Sickle cell anemia is an autosomal recessive disease, being the hereditary disease monogenic disease more common in Brazil, occurring predominantly among afro, affecting approximately 0.1% to 0.3% of the black population. The clinical presentation is variable, and can lead to numerous complications and frequent hospitalizations. The aim of the present study is to analyze the scientific production related to morbidity and mortality of sickle cell anemia with a focus on clinical aspects.This is a systematic review without meta-analysis in the databases Scientific Electronic Library Online (SciELO ) and Virtual Health Library ( VHL ) with the keywords " sickle cell anemia " , " morbidity " , " death " , and " epidemiology " . They included original articles, complete and in digital version , published in Portuguese, with temporal cut from 2005 to 2015 were selected for review a total of eleven articles covering all the methodological steps proposed.From the data analysis it was observed that carriers of sickle cell anemia are affected by a number of complications and clinical manifestations resulting from minor changes to the underlying disease . Painful crises , splenic sequestration crises , anemia and infections are the most common causes of hospitalization. Furthermore , the carrier of the sickle cell anemia can present osteoarticular complications , osteomyelitis , septic arthritis , femoral head necrosis , cholelithiasis , cholecystitis , anemia , adenotonsillary hypertrophy, obstructive sleep apnea syndrome, delayed growth curve , stroke and priapism.The use of hydroxyurea, penicillin, folic acid and deferiprone has been identified. Mortality rates have been falling in the first ten years of life and life expectancy is increasing. The hospital death rates found ranged from 1.7% to 10.7%. The length of stay ranged from one to 51 days, with an average of 6.2 days. The estimated survival probability reaches up to 97.5% in the first year of life. Early diagnosis by newborn screening associated with monitoring on an outpatient basis, greater access to health services helped these changes. Efficiency in recognition of the acute event and the search for an emergency room is essential to counteract the rapid evolution of the signs and symptoms of serious illness. Patients should be monitored by a multidisciplinary team, assessing not only the physical, but also taking into account the emotional aspects, meeting all your needs, removing fears and giving support to confront the disease.