The Quality of Life of Children and Adolescents with X-Linked Agammaglobulinemia

Soresina, Annarosa; Nacinovich, Renata; Bomba, Monica; Cassani, Morena; Molinaro, Anna; Sciotto, A.; Martino, Silvana; Cardinale, Fabio; Mattia, Domenico De; Putti, Caterina; Dellepiane, Rosa Maria; Felici, L.; Parrinello, Giovanni; Neri, Francesca; Plebani, Alessandro

doi:10.1007/s10875-008-9270-8

Cited by 36 publications

(35 citation statements)

References 9 publications

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“…Considering that HRQOL data provide direct information about the impact of a disorder and its treatment, HRQOL assessments could be a part of multimodal assessment and treatment approach in children with PID as well. However, the evidence of to what extent PID affects HRQOL in children is limited, because only three studies that evaluated HRQOL in this population are available 14–16…”

Section: Introductionmentioning

confidence: 99%

Health-related quality of life, anxiety, and depressive symptoms in children with primary immunodeficiencies

Kuburovic¹,

Pašić²,

Sušić³

et al. 2014

PPA

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IntroductionThe aims of this study were to evaluate levels of health-related quality of life (HRQOL) and the presence of anxiety and depressive symptoms in children with primary immunodeficiency disease (PID) in Serbia.Materials and methodsSelf- and parent-rated data from 25 children with PID were available. As controls, data from 50 children with juvenile idiopathic arthritis (JIA) and 89 healthy children were included. The Pediatric Quality of Life Inventory was used for HRQOL assessments. Anxiety symptoms were identified using the Screen for Child Anxiety-Related Emotional Disorders questionnaire, while depressive symptoms were identified using the Mood and Feeling Questionnaire.ResultsChildren with PID had significantly lower Pediatric Quality of Life Inventory total scores compared to children with JIA and healthy children as child-rated (P=0.02) and parent-rated (P<0.001). Specifically, they had significantly lowered emotional functioning compared to children with JIA, and social functioning compared to both children with JIA and healthy children. School functioning was significantly lower among children with PID (parent-rated only). By parent-rated responses, six (24%) out of 25 children with PID had significant anxiety symptoms, while five (20%) children had significant depressive symptoms, which was statistically higher than among children with JIA and healthy controls (P=0.05).ConclusionHRQOL could be significantly compromised in children with PID, particularly across such psychosocial domains as emotional, social, and school. These children were also found to be at an increased risk for suffering significant anxiety and depressive symptoms.

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Section: Introductionmentioning

confidence: 99%

Health-related quality of life, anxiety, and depressive symptoms in children with primary immunodeficiencies

Kuburovic¹,

Pašić²,

Sušić³

et al. 2014

PPA

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“…El PedsQL es el cuestionario que ha sido más utilizado en niños con diagnóstico de inmunodeficiencias primarias. [20][21][22][23][24][25][26][27][28] Existen algunos reportes, la gran mayoría de ellos en ensayos clínicos, en los que se ha utilizado para comparar dos o más tratamientos, pero hay muy poca información a escala mundial de la calidad de vida en las inmunodeficiencias primarias y es nula en el caso de nuestro país.…”

Section: Discussionunclassified

“…Modelos lineales para estimar el efecto del retraso de diagnóstico de inmunodeficiencia primaria en niños sin y con complicación pulmonar en Italia y se comparó con niños sanos y con pacientes con enfermedades reumatológicas, se reportó una calidad de vida menor en los pacientes con enfermedades reumatológicas (72.1 puntos), seguida por la de pacientes con agammaglobulinemia (75.8 puntos), en comparación con los niños sanos (86.8 puntos); hubo una asociación significativa entre la percepción de los pacientes y la de los padres. 25 A diferencia de otras enfermedades crónicas como el asma o la diabetes en las inmunodeficiencias primarias, a pesar de que son afecciones crónicas y que frecuentemente ya tienen complicaciones al momento del diagnóstico, no es común encontrar informes de calidad de vida; salvo en algunos ensayos clínicos que comparan opciones de tratamiento sustitutivo. Debido a que los pacientes incluidos en el estudio ya estaban diagnosticados y en tratamiento no contamos con una medición de la calidad de vida basal; es decir, antes del inicio del tratamiento o en el momento del diagnóstico (aunado a que sólo realizamos una medición de calidad de vida para cada paciente).…”

Section: Discussionunclassified

Calidad de vida de los pacientes con inmunodeficiencias primarias de anticuerpos

et al. 2016

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INTRODUCCIÓNLas inmunodeficiencias primarias son enfermedades hereditarias generalmente causadas por alteraciones monogénicas. Pueden afectar el sistema inmunitario en su desarrollo, su función, o en ambos.1 Se clasifican de acuerdo con la afección particular del sistema inmuno- ABSTRACTPrimary immunodeficiencies (PID) are genetic diseases affecting immunological normal response. In the great majority of cases affected individual sare predisposed to recurrent and / or severe infections with impaired quality of life (QoL). The most frequent PID are defects in the production of antibodies. When diagnosis and treatment are not done properly, permanent damage may occur in affected organs and further affecting QoL.Objective: To evaluate QoL in patients with antibody PID compared with QoL of healthy controls. Methods:Cross-sectional study with the application of the instrument "Pediatric Quality of Life Inventory" (PedsQL) in its Mexican Spanish validated version. The PedsQL score difference between children with PID and healthy children was determined by the Student t test. The effect of a delayed in diagnosis was evaluated by analysis in a covariance model. 28 patients were included. The median age was 5 years and 5 months.

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“…[18] In 2003 Porpiglia et al [19] reported successful long lasting donor B-cell engraftment using HCT in two murine models of B-cell deficiency without administration of irradiation or myelotoxic drugs. These findings suggest that it may not be necessary to make “space” in the bone marrow to achieve sufficient engraftment.…”

Section: Discussionmentioning

confidence: 99%

Successful hematopoietic cell transplantation in a patient with X‐linked agammaglobulinemia and acute myeloid leukemia

Abu‐Arja

Chernin

Abusin

et al. 2015

Pediatric Blood & Cancer

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X-linked agammaglobulinemia (XLA) is a primary immunodeficiency characterized by marked reduction in all classes of serum immunoglobulins and the near absence of mature CD19+ B-cells. Although malignancy has been observed in patients with XLA, we present the first reported case of acute myeloid leukemia (AML) in a patient with XLA. We also demonstrate the complete correction of the XLA phenotype following allogeneic hematopoietic cell transplantation for treatment of the patient’s leukemia.

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The Quality of Life of Children and Adolescents with X-Linked Agammaglobulinemia

Cited by 36 publications

References 9 publications

Health-related quality of life, anxiety, and depressive symptoms in children with primary immunodeficiencies

Health-related quality of life, anxiety, and depressive symptoms in children with primary immunodeficiencies

Calidad de vida de los pacientes con inmunodeficiencias primarias de anticuerpos

Successful hematopoietic cell transplantation in a patient with X‐linked agammaglobulinemia and acute myeloid leukemia

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