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. 2015 Sep-Oct;133(5):421-7.
doi: 10.1590/1516-3180.2015.00171105. Epub 2015 Aug 21.

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

Érika Bergamini Mastandréa  1 , Fátima Lucchesi  1 , Marcela Mayumi Gomes Kitayama  1 , Maria Stella Figueiredo  2 , Vanessa de Albuquerque Citero  1
Affiliations

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

Érika Bergamini Mastandréa et al. Sao Paulo Med J. 2015 Sep-Oct.

Abstract
in English, Portuguese

Context and 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.

Design and setting: 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).

Conclusion: 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.

CONTEXTO E OBJETIVO:: A qualidade de vida relacionada à saúde (QVRS) pode ser piorada em pacientes com doença falciforme na presença de transtornos psiquiátricos. O objetivo deste estudo é descrever a sintomatologia psiquiátrica presente no paciente brasileiro com doença falciforme e avaliar a relação desses sintomas com o genótipo da doença e a QVRS do sujeito.

TIPO DE ESTUDO E LOCAL:: Estudo com delineamento transversal, realizado no ambulatório de Hematologia do Hospital São Paulo.

MÉTODOS:: Adultos com doença falciforme responderam ao Questionário de Qualidade de Vida (SF-36) e ao Questionário sobre a Saúde do Paciente (PHQ). Dados clínicos foram obtidos no prontuário médico. Modelos de regressão linear foram desenvolvidos para estudar a dependência dos domínios de QVRS no genótipo com controle para sintomas psiquiátricos.

RESULTADOS:: No período do estudo, 110 pacientes foram avaliados. O sintoma psiquiátrico mais frequente foi o depressivo (30%), seguido do ansioso (12,7%) e de abuso de álcool (9,1%). Os pacientes com genótipo mais grave (SS e S βthal0) apresentaram menores médias nos domínios de QVRS de "saúde geral" e de "aspectos físicos", sem interferência dos sintomas psiquiátricos. No domínio "aspectos físicos", o genótipo mais grave funcionou como fator protetor da QVRS (β = 0,255; P = 0,007).

CONCLUSÃO:: Os genótipos mais graves pioraram a QVRS em dois domínios do componente físico ("aspectos físicos" e "saúde geral"), mas não influenciaram o componente mental, sugerindo que o médico deve estar atento aos aspectos da QVRS relacionados com a funcionalidade do portador da doença falciforme, conhecendo as limitações com as quais o paciente convive.

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Conflict of interest statement

Conflict of interest: None

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