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1.
Psychol Health Med ; : 1-11, 2020 Jan 26.
Artigo em Inglês | MEDLINE | ID: mdl-31984755

RESUMO

The investigation of mental health among persons with haemophilia is mostly focused on negative and disease-related indicators. Literature however shows that psychosocial resources and optimal daily functioning can co-exist with chronic disease. The Dual Continua Model operationalizes positive mental health as 'flourishing', a condition comprising emotional, psychological, and social well-being dimensions. In the present study physical and mental health were comparatively assessed through positive and negative indicators in adults with haemophilia and a control group. Participants included 84 Italian persons with severe haemophilia (Mage = 43.44; SDage = 13.04) and 164 adults without history of chronic illness (Mage = 40.98; SDage = 12.26), who completed the Short Form Health Survey, the Positive and Negative Affect Schedule, and the Mental Health Continuum Short Form. MANOVA and post-hoc t-tests provided evidence of worse general health, lower negative affect and higher psychological well-being among participants with haemophilia compared with the control group. Moreover, the percentage of flourishing individuals was higher among participants with haemophilia. Results support previous evidence suggesting that a chronic disease does not prevent mental well-being attainment. The identification of assets and strengths allowing people with haemophilia to flourish can be fruitfully used to design resource-centered interventions.

2.
J Allergy Clin Immunol Pract ; 8(1): 273-282, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31377437

RESUMO

BACKGROUND: Rituximab (RTX; anti-CD20 mAb) is a treatment option in children with refractory immune thrombocytopenia, autoimmune hemolytic anemia (AHA), and Evans syndrome (ES). Prevalence and clinical course of RTX-induced hypogammaglobulinemia in these patients are poorly known. OBJECTIVE: To evaluate the prevalence and risk factors for persistent hypogammaglobulinemia (PH) after RTX use. METHODS: Clinical and immunologic data from children treated with RTX for immune thrombocytopenia, AHA, and ES were collected from 16 Italian centers and 1 UK center at pre-RTX time point (0), +6 months, and yearly, up to 4 years post-RTX. Patients with previously diagnosed malignancy or primary immune deficiency (PID) were excluded. RESULTS: We analyzed 53 children treated with RTX for immune thrombocytopenia (n = 36), AHA (n = 13), and ES (n = 4). Median follow-up was 30 months (range, 12-48). Thirty-two percent of patients (17 of 53) experienced PH, defined as IgG levels less than 2 SD for age at last follow-up (>12 months after RTX). Significantly delayed B-cell recovery was observed in children experiencing PH (hazard ratio, 0.55; P < .05), and 6 of 17 (35%) patients had unresolved B-cell lymphopenia at last follow-up. PH was associated with IgA and IgM deficiency, younger age at RTX use (51 vs 116 months; P < .01), a diagnosis of AHA/ES, and better response to RTX. Nine patients with PH (9 of 17 [53%]) were eventually diagnosed with a PID. CONCLUSIONS: Post-RTX PH is a frequent condition in children with autoimmune cytopenia; a sizable proportion of patients with post-RTX PH were eventually diagnosed with a PID. In-depth investigation for PID is therefore recommended in these patients.

3.
Artigo em Inglês | MEDLINE | ID: mdl-31486759

RESUMO

BACKGROUND: Immune thrombocytopenia (ITP) is an acquired immune mediated disorder characterized by isolated thrombocytopenia. Pediatric ITP patients can develop autoantibodies such as anti-thyroglobulin (TG) and anti-thyroperoxidase (TPO), even in absence of clinical signs of autoimmune disease. Objective; The purpose of this article is to provide a review about: 1) the prevalence of positivity of anti-thyroid antibodies (TPO and TG) in pediatric patients with chronic ITP; 2) the role of autoimmune thyroiditis on the outcome of chronic ITP. METHOD: The authors individually completed a review of the literature for this article. Retrospective and prospective clinical studies with pediatric cohorts were considered. RESULTS: From the analysis of data, we found 4 papers which included studies only on pediatric population, and which corresponded to selected criteria. Pediatric ITP patients have been shown to have a statistically significant prevalence of anti-thyroid antibodies over healthy controls (11.6-36% versus 1.2-1.3%). No correlation has been found between the platelet count and the prevalence of positive anti-thyroid antibodies at any time of the follow up. CONCLUSION: The results of our bibliographic research demonstrated that: a) pediatric patients with chronic ITP tend to have a statistically significant prevalence of anti-thyroid antibodies positivity respect to general pediatric population; b) there are no clear data about the role of autoimmune thyroiditis as prognostic factor for chronic course of ITP in pediatric age.

6.
Artigo em Inglês | MEDLINE | ID: mdl-31203812

RESUMO

BACKGROUND: Immune thrombocytopenia (ITP) is an acquired immuno-mediated disorder characterized by thrombocytopenia with an increased risk of bleeding. In recent years 1,25[OH]2D3 has been rediscovered as an immune modulator. We decided to evaluate serum D vitamin levels in a cohort of children with immune thrombocytopenia in order to discover if D vitamin concentrations may predict ITP duration. MATERIALS AND METHODS: Thirty children were enrolled in this study (sixteen with chronic ITP and fourteen with newly diagnosed ITP) to assess serum D vitamin levels. RESULTS: The results showed that 80% of the enrolled children presented a D hypovitaminosis status. Children with newly diagnosis ITP showed no statistically significant higher median values of D vitamin compared to chronic ITP. CONCLUSIONS: Its, in our case, may suggest that D vitamin deficiency not represent a chronicity factor for ITP. However, further studies are needed to understand the role of D vitamin in ITP pathogenesis.

7.
Front Pediatr ; 7: 163, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31134165

RESUMO

Objective: Primary immune thrombocytopenia (ITP) is a hemorrhagic disorder. Spontaneous recovery within 12 months occurs in the majority of pediatric patients. Nevertheless, in 20-30% of children the disease is chronic. The impact extends to the patients' families, whose everyday life, in terms of interpersonal relationships and financial status, is adversely affected. This study investigated the ability of a narrative instrument to improve the quality of life of pediatric chronic ITP patients and their families and quantified the familial burden imposed by the illness. Method: A quantitative survey and a narrative plot delivered through an online platform were adopted for the analysis. Results: Pediatricians of ten Italian Hematologic Centers explained the projects to patients and their family in the outpatient clinic. 70 caregivers of children with ITP filled the ad-hoc questionnaire. Data from 53 caregivers revealed the emotional impact of pediatric chronic ITP. The narrative approach highlighted the specific resources used by patients and their families to cope with the disease and its chronicity. Discussion: Caregivers underlined the need for "humaneness" in their interactions with clinical personnel. The majority of respondents provided positive feedback regarding the narrative project, defining the experience as "liberating" and improving their quality of life.

8.
J Allergy Clin Immunol ; 144(2): 584-593.e7, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30910492

RESUMO

BACKGROUND: Lacking protective antibodies, patients with primary antibody deficiencies (PADs) experience frequent respiratory tract infections, leading to chronic pulmonary damage. Macrolide prophylaxis has proved effective in patients with chronic respiratory diseases. OBJECTIVE: We aimed to test the efficacy and safety of orally administered low-dose azithromycin prophylaxis in patients with PADs. METHODS: We designed a 3-year, double-blind, placebo-controlled, randomized clinical trial to test whether oral azithromycin (250 mg administered once daily 3 times a week for 2 years) would reduce respiratory exacerbations in patients with PADs and chronic infection-related pulmonary diseases. The primary end point was the number of annual respiratory exacerbations. Secondary end points included time to first exacerbation, additional antibiotic courses, number of hospitalizations, and safety. RESULTS: Eighty-nine patients received azithromycin (n = 44) or placebo (n = 45). The number of exacerbations was 3.6 (95% CI, 2.5-4.7) per patient-year in the azithromycin arm and 5.2 (95% CI, 4.1-6.4) per patient-year in the placebo arm (P = .02). In the azithromycin group the hazard risk for having an acute exacerbation was 0.5 (95% CI, 0.3-0.9; P = .03), and the hazard risk for hospitalization was 0.5 (95% CI, 0.2-1.1; P = .04). The rate of additional antibiotic treatment per patient-year was 2.3 (95% CI, 2.1-3.4) in the intervention group and 3.6 (95% CI, 2.9-4.3) in the placebo group (P = .004). Haemophilus influenzae and Streptococcus pneumoniae were the prevalent isolates, and they were not susceptible to macrolides in 25% of patients of both arms. Azithromycin's safety profile was comparable with that of placebo. CONCLUSION: The study reached the main outcome centered on the reduction of exacerbation episodes per patient-year, with a consequent reduction in additional courses of antibiotics and risk of hospitalization.

9.
Am J Hematol ; 94(2): 216-222, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30456824

RESUMO

Autoimmune neutropenia of infancy (AIN) is characterized by low risk of severe infection, tendency to spontaneously resolve and typically onset at ≤4-5 years of age; it is due to auto-antibodies whose detection is often difficult. In case of negativity of 4 antineutrophils autoantibody tests, after having excluded ethnic, postinfection, drug induced, or congenital neutropenia, according to the Italian guidelines the patients will be defined as affected by "idiopathic neutropenia" (IN). We describe the characteristics of 85 IN patients enrolled in the Italian neutropenia registry: they were compared with 336 children affected by AIN. The 2 groups were clinically very similar and the main differences were detection age (later in IN), length of disease (longer in IN) and, among recovered patients, age of spontaneous recovery: the median age at resolution was 2.13 years in AINs and 3.03 years in INs (P = .00002). At bivariate analysis among AIN patients earlier detection age (P = .00013), male sex (P = .000748), absence of leucopenia (P = .0045), and absence of monocytosis (P = .0419) were significantly associated with earlier recovery; in the IN group only detection age (P = .013) and absence of monocytosis (P = .0333) were significant. At multivariate analysis detection age and absence of monocytosis were independently significant (P = 6.7e-05 and 4.4e-03, respectively) in the AIN group, whereas in the IN group only detection age stayed significant (P = .013).


Assuntos
Neutropenia/congênito , Fatores Etários , Autoimunidade , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Itália , Leucopenia , Masculino , Neutropenia/diagnóstico , Neutropenia/epidemiologia , Sistema de Registros , Fatores de Risco , Fatores Sexuais
10.
Pediatr Blood Cancer ; 66(1): e27452, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30270575

RESUMO

BACKGROUND: Immune thrombocytopenia (ITP) is an acquired immune-mediated disorder characterized by isolated thrombocytopenia. Pediatric ITP patients are prone to develop autoantibodies such as antithyroglobulin (TG) and antithyroperoxidase (TPO), even in the absence of clinical signs of autoimmune disease. The aim of this multicenter retrospective study was to evaluate (1) the prevalence of positivity of antithyroid antibodies (TPO and TG) in a large cohort of pediatric patients with chronic ITP; (2) the role of autoimmune thyroiditis as a prognostic factor for chronicity of ITP. PROCEDURE: For this retrospective study, we collected data from patients diagnosed as affected by chronic ITP between 2011 and 2014 in six centers belonging to the Italian Association of Pediatric Haematology and Oncology (AIEOP). RESULTS: From the analysis of data, we found a significantly higher prevalence of antithyroid antibodies in children with chronic ITP (11.6%) than in the pediatric population (1.2%-1.3%). No correlation has been found between the platelet count and the prevalence of positive antithyroid antibodies at any detection time of the study. CONCLUSIONS: The results of our study demonstrated that (1) the prevalence of positivity for antithyroid antibodies (anti-TPO and anti-TG) in pediatric patients with chronic ITP results is significantly higher than in the pediatric population; (2) autoimmune thyroiditis does not seem to play a role as a prognostic factor for chronicity of ITP in pediatric patients.


Assuntos
Autoanticorpos/sangue , Biomarcadores/sangue , Iodeto Peroxidase/imunologia , Púrpura Trombocitopênica Idiopática/sangue , Tireoidite Autoimune/fisiopatologia , Adolescente , Autoanticorpos/imunologia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Itália/epidemiologia , Masculino , Prevalência , Prognóstico , Púrpura Trombocitopênica Idiopática/epidemiologia , Púrpura Trombocitopênica Idiopática/imunologia , Estudos Retrospectivos , Tireoidite Autoimune/imunologia
11.
Blood ; 133(12): 1346-1357, 2019 03 21.
Artigo em Inglês | MEDLINE | ID: mdl-30591527

RESUMO

Inherited thrombocytopenias (ITs) are a heterogeneous group of disorders characterized by low platelet count that may result in bleeding tendency. Despite progress being made in defining the genetic causes of ITs, nearly 50% of patients with familial thrombocytopenia are affected with forms of unknown origin. Here, through exome sequencing of 2 siblings with autosomal-recessive thrombocytopenia, we identified biallelic loss-of-function variants in PTPRJ . This gene encodes for a receptor-like PTP, PTPRJ (or CD148), which is expressed abundantly in platelets and megakaryocytes. Consistent with the predicted effects of the variants, both probands have an almost complete loss of PTPRJ at the messenger RNA and protein levels. To investigate the pathogenic role of PTPRJ deficiency in hematopoiesis in vivo, we carried out CRISPR/Cas9-mediated ablation of ptprja (the ortholog of human PTPRJ) in zebrafish, which induced a significantly decreased number of CD41+ thrombocytes in vivo. Moreover, megakaryocytes of our patients showed impaired maturation and profound defects in SDF1-driven migration and formation of proplatelets in vitro. Silencing of PTPRJ in a human megakaryocytic cell line reproduced the functional defects observed in patients' megakaryocytes. The disorder caused by PTPRJ mutations presented as a nonsyndromic thrombocytopenia characterized by spontaneous bleeding, small-sized platelets, and impaired platelet responses to the GPVI agonists collagen and convulxin. These platelet functional defects could be attributed to reduced activation of Src family kinases. Taken together, our data identify a new form of IT and highlight a hitherto unknown fundamental role for PTPRJ in platelet biogenesis.


Assuntos
Plaquetas/patologia , Predisposição Genética para Doença , Megacariócitos/patologia , Mutação , Trombocitopenia/patologia , Adolescente , Adulto , Animais , Plaquetas/metabolismo , Sistemas CRISPR-Cas , Criança , Feminino , Seguimentos , Hematopoese , Humanos , Masculino , Megacariócitos/metabolismo , Pessoa de Meia-Idade , Linhagem , Prognóstico , Proteínas Tirosina Fosfatases Classe 3 Semelhantes a Receptores/antagonistas & inibidores , Proteínas Tirosina Fosfatases Classe 3 Semelhantes a Receptores/genética , Trombocitopenia/etiologia , Trombocitopenia/genética , Peixe-Zebra
13.
Eur J Endocrinol ; 176(2): 111-121, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27913605

RESUMO

BACKGROUND: Childhood acute lymphoblastic leukaemia (ALL) survivors have an increased risk of metabolic and cardiovascular disease. We aimed to assess the presence of non-alcoholic fatty liver disease (NAFLD) in childhood ALL and if it is associated with early cardiovascular dysfunction. METHODS: In total, 53 childhood ALL survivors and 34 controls underwent auxological evaluation, biochemical assay, liver, heart and vascular ultrasound study. RESULTS: NAFLD was more frequent in ALL patients than in controls (39.6% vs 11.7%, P < 0.01). Patients with NAFLD were more obese and insulin resistant than patients without NAFLD. Flow-mediated dilatation and interventricular septum were lower in the ALL group than those in the control group (P < 0.001 for both). The patients with NAFLD showed lower left ventricular ejection fraction than those without NAFLD (P = 0.011). In ALL survivors, BMI-SDS and subcutaneous fat were the strongest predictors of NAFLD, whereas preperitoneal adipose tissue and C-reactive protein were the strongest predictors of left ventricular ejection fraction. CONCLUSIONS: Childhood ALL survivors had higher prevalence of NAFLD than healthy controls, which is associated with early left ventricular impairment. In the case of fatty liver, a comprehensive heart evaluation is mandatory. We strongly recommend to prevent visceral adiposity in ALL survivors, to search for metabolic syndrome or its components and to reinforce the need of intervention on diet and lifestyle during the follow-up of these patients.


Assuntos
Hepatopatia Gordurosa não Alcoólica/etiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Disfunção Ventricular Esquerda/complicações , Disfunção Ventricular Esquerda/etiologia , Adolescente , Criança , Feminino , Humanos , Masculino , Hepatopatia Gordurosa não Alcoólica/epidemiologia , Hepatopatia Gordurosa não Alcoólica/fisiopatologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/epidemiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/fisiopatologia , Fatores de Risco , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/fisiopatologia
15.
Pediatr Res ; 79(2): 262-70, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26492283

RESUMO

BACKGROUND: Immune thrombocytopenic purpura (ITP) is characterized by reduced platelet count secondary to immune-mediated destruction, this results in an increased bleeding risk. Autoantibodies binding to platelets tag them for premature destruction in the spleen. For this reason, splenectomy is often performed as treatment of chronic forms of disease that are resistant to pharmacological therapy. METHODS: We studied 30 patients with ITP and compared them with age-matched controls. RESULTS: We show that B cells of patients with chronic ITP are intrinsically hyperreactive, producing more than normal IgG in vivo and plasma cells in vitro. In normal individuals after splenectomy, a significant depletion of memory B cells is observed, associated with loss of reactivity to CpG oligodeoxynucleotide and consequent inability to form antibody-producing cells. In Enzyme-Linked ImmunoSpot Methods, we compared three splenectomized ITP patients relapsing after surgery, 30 healthy controls, and 37 individuals splenectomized for trauma, spherocytosis, thalassemia, nonhematological tumor, and other diseases. CONCLUSIONS: We confirmed that B cells of ITP patients remain hyperreactive in vitro and form high numbers of antibody-producing cells after splenectomy. Thus, chronic ITP may be associated with intrinsic B-cell hyperfunction, leading to the production of antibodies with multiple specificities including that against platelets.


Assuntos
Autoanticorpos/imunologia , Linfócitos B/imunologia , Plaquetas/imunologia , Imunoglobulina G/imunologia , Plasmócitos/imunologia , Púrpura Trombocitopênica Idiopática/cirurgia , Esplenectomia , Adolescente , Estudos de Casos e Controles , Proliferação de Células , Células Cultivadas , Criança , Pré-Escolar , Doença Crônica , Feminino , Humanos , Memória Imunológica , Ativação Linfocitária , Masculino , Contagem de Plaquetas , Púrpura Trombocitopênica Idiopática/sangue , Púrpura Trombocitopênica Idiopática/diagnóstico , Púrpura Trombocitopênica Idiopática/imunologia , Recidiva , Resultado do Tratamento
16.
Pediatr Blood Cancer ; 62(10): 1826-31, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-25976056

RESUMO

BACKGROUND: The clinical benefits of prophylaxis in patients with hemophilia are well-established and include the following: reduced bleeding episodes, prevention of joint damage, decreased inhibitor development, and improved health-related quality of life. However, the cost-effectiveness of prophylaxis is still not clear. PROCEDURES: We reviewed the published hemophilia prophylaxis economic models focusing on utility assumptions. RESULTS: We found six cost-utility studies that compared prophylaxis and on-demand regimens. These studies reported remarkably different results, using utility values based on different assumptions and data sources. CONCLUSIONS: We suggest that cooperation among key stakeholders (clinicians, patient organizations and health-care decision makers) as a means of collecting evidence-based and experiential data to represent both the utility and the quality of life changes for patients with Hemophilia A who are treated with prophylaxis or receive on-demand treatments may represent a winning strategy with which to resolve the outstanding issues related to health technology assessments in the care of patients with hemophilia.


Assuntos
Análise Custo-Benefício , Hemofilia A/tratamento farmacológico , Hemofilia A/economia , Modelos Econômicos , Garantia da Qualidade dos Cuidados de Saúde , Hemorragia/etiologia , Hemorragia/prevenção & controle , Humanos , Garantia da Qualidade dos Cuidados de Saúde/normas
18.
Pediatr Hematol Oncol ; 32(1): 1-10, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25325764

RESUMO

Low-molecular weight heparins are currently the most commonly used anticoagulants in children and newborns. However, since thrombotic complications rarely occur outside large children's hospitals, physicians often encounter some practical problems in managing these treatments when a pediatric thrombosis specialist is not available. The drug of choice is enoxaparin, due to its favorable FXa/FIIa ratio and the availability of pharmacokinetic and pharmacodynamic data. The treatment of acute thrombosis should be started with two daily injections but when compliance is an issue, a single daily administration schedule could be chosen for secondary prophylaxis ensuring careful measurement of the post 24-hour anti-FXa activity. Furthermore, a subcutaneous device may be a useful tool and a topical dermal anesthetic could be effective in controlling pain without affecting anti-FXa levels. In neonate and toddlers, where mini doses are frequently needed, the dead space of syringes and needles could represent an issue and therefore the use of insulin syringes without dead space is advisable, while a dilution of the drug is useful with other syringes. This article derives from a nonsystematic review of the available literature, with special attention to recent international guidelines and expert recommendations, combined to authors' clinical practice in large tertiary pediatric hospitals and will provide concise and practical information for the use of low-molecular weight heparin in childhood and infancy in a sort of "answering frequently asked questions."


Assuntos
Anticoagulantes/uso terapêutico , Heparina de Baixo Peso Molecular/uso terapêutico , Tromboembolia Venosa/tratamento farmacológico , Adolescente , Anticoagulantes/efeitos adversos , Criança , Pré-Escolar , Feminino , Heparina de Baixo Peso Molecular/efeitos adversos , Humanos , Lactente , Recém-Nascido , Masculino , Tromboembolia Venosa/sangue
19.
Pediatr Hematol Oncol ; 31(8): 687-702, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25006797

RESUMO

Hemophilias are the most known inherited bleeding disorders. The challenges in the management of hemophilic children are different from those in adults: prophylaxis regimen removed the hallmark of crippling disease with lifelong disabilities; individualized regimens are being implemented in order to overcome venous access problems. Presently, at least in high-income countries, advances in treatment of hemophilia resulted in continuous improvement of the patients' quality of life and life expectancy. Inhibitors remain the most severe complication of hemophilia therapy. The treatment' compliance is the key to achieve a successful management. The patient, his family, the medical and psychological team are the players of a comprehensive care system. The current management of hemophilic children is the example of huge resource investments enabling long-term benefits in particular quality of life as a primary objective of the healthcare process.


Assuntos
Hemofilia A/terapia , Qualidade de Vida , Criança , Pré-Escolar , Análise Custo-Benefício , Assistência à Saúde/economia , Gerenciamento Clínico , Fator IX/administração & dosagem , Fator IX/genética , Fator IX/metabolismo , Fator VIII/administração & dosagem , Fator VIII/genética , Fator VIII/metabolismo , Hemofilia A/sangue , Hemofilia A/complicações , Hemofilia A/economia , Hemofilia A/prevenção & controle , Humanos , Qualidade de Vida/psicologia , Fatores de Risco
20.
Pediatr Hematol Oncol ; 31(6): 534-47, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24852656

RESUMO

BACKGROUND: The ITP-QoL is a disease-specific questionnaire for the assessment of health-related quality of life (HRQoL) in children with immune thrombocytopenia (ITP) and their parents. The aim of this study was to test the psychometric characteristics of the ITP-QoL in the Italian pediatric population in terms of validity and reliability. PROCEDURE: Children aged 8-16 years with acute or chronic ITP and their parents were recruited in Italy. Participants completed the ITP-QoL together with other patient-reported outcomes (PROs). Reliability was calculated using Cronbach's alpha. Convergent validity was determined by means of the Pearson correlation coefficients. RESULTS: A total of 91 ITP patients, mean age of 12.11 ± 2.47 years, and their parents participated; 61.5% of the patients were female. Two patients had acute ITP and 30.2% had a moderate to severe status of ITP. Cutaneous symptoms were more frequent than mucosal symptoms. Due to item and scale analyses 20 items were deleted from the original ITP-QoL. Internal consistency of the ITP-QoL was found to be good with Cronbach's alpha exceeding α = 0.70 for all but one subscale. Concerning convergent validity "moderate" to "high" negative correlations were found between ITP-QoL and KINDL subscales. The ITP-QoL was able to discriminate between clinical subgroups such as number of days lost at school due to ITP and hospitalization. CONCLUSIONS: Our study was able to demonstrate that the Italian version of ITP-QoL (for children aged 8-16 years) is a valid and reliable instrument for the assessment of HRQoL in children with ITP.


Assuntos
Psicometria/métodos , Púrpura Trombocitopênica Idiopática/psicologia , Adolescente , Criança , Comparação Transcultural , Feminino , Humanos , Itália , Masculino , Qualidade de Vida , Inquéritos e Questionários , Traduções
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