title: The relationship between genotype, psychiatric symptoms and quality of life in adult patients with sickle cell disease in São Paulo, Brazil: a cross-sectional study.
abstract: OBJECTIVE
  Health-related quality of life (HRQoL) may be worsened in sickle cell patients due to the presence of psychiatric disorders. The aims of this study were to describe the psychiatric symptoms in Brazilian sickle cell patients and to evaluate the relationship of these symptoms to the genotype of the disease and the subject's HRQoL.
  
  
  METHODS
  Cross-sectional study conducted at the hematology outpatient clinic, Hospital São Paulo.
  
  
  METHODS
  Adult patients with sickle cell disease completed the Medical Outcome Study - Short Form 36 and the Patients' Health Questionnaire. Clinical data were gathered from their medical files. Linear regression models were developed to study the dependency of HRQoL domains on the genotype controlling for psychiatric symptoms.
  
  
  RESULTS
  In the study period, 110 patients were evaluated. The most frequent psychiatric symptom was depression (30%), followed by anxiety (12.7%) and alcohol abuse (9.1%). Patients with the more severe genotype (SS and Sβthal0) showed lower scores for the "general health" and "role-physical" HRQoL domains, without interference from psychiatric symptoms. In the "role-physical" domain, the more severe genotype operated as a protective factor for HRQoL (β = 0.255; P = 0.007).
  
  
  CONCLUSIONS
  The more severe genotypes worsened HRQoL in two domains of physical health (general health and role-physical), but they did not have any influence on mental health, thus suggesting that physicians should be more attentive to aspects of HRQoL relating to the functionality of sickle cell disease patients, so as to be aware of the limitations that these patient live with.
authors.affiliation: Department of Psychiatry, Escola Paulista de Medicina, São Paulo, SP, Brazil.
authors.forename: Érika Bergamini
authors.initials: ÉB
authors.lastname: Mastandréa
authors.affiliation: Department of Psychiatry, Escola Paulista de Medicina, São Paulo, SP, Brazil.
authors.forename: Fátima
authors.initials: F
authors.lastname: Lucchesi
authors.affiliation: Department of Psychiatry, Escola Paulista de Medicina, São Paulo, SP, Brazil.
authors.forename: Marcela Mayumi Gomes
authors.initials: MM
authors.lastname: Kitayama
authors.affiliation: Department of Clinical and Experimental Oncology, Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo, SP, Brazil.
authors.forename: Maria Stella
authors.initials: MS
authors.lastname: Figueiredo
authors.affiliation: Department of Psychiatry, Escola Paulista de Medicina, São Paulo, SP, Brazil.
authors.forename: Vanessa de Albuquerque
authors.initials: Vde A
authors.lastname: Citero
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000437:Alcoholism / Q000523:psychology; D000755:Anemia, Sickle Cell / Q000235:genetics* / Q000523:psychology*; D001007:Anxiety / Q000523:psychology; D001938:Brazil; D003430:Cross-Sectional Studies; D003863:Depression / Q000523:psychology; D005260:Female; D005838:Genotype; D006801:Humans; D008297:Male; D001523:Mental Disorders / Q000523:psychology*; D008603:Mental Health; D008875:Middle Aged; D011788:Quality of Life / Q000523:psychology*; D012720:Severity of Illness Index; D012737:Sex Factors; D011795:Surveys and Questionnaires; D055815:Young Adult