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1.
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.

2.
Pediatr Allergy Immunol Pulmonol ; 32(2): 70-75, 2019 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-31508259

RESUMO

Background: Subcutaneous immunoglobulin G (SCIG) may be a better option than intravenous immunoglobulin G (IVIG) for patients with primary immunodeficiencies (PID) due to reduced systemic and serious adverse reactions and easier administration. The Infusione Bimensile di Immunoglobuline Sottocute (IBIS) study investigated the effects of Hizentra®, a 20%-concentrated SCIG, administered biweekly in patients with PID. This subanalysis aimed to evaluate clinical and laboratory outcomes in the IBIS pediatric subcohort. Methods: Thirteen children with PID were observed for 12 months retrospectively (with previous IVIG/SCIG) and prospectively with biweekly Hizentra. Results: Mean ± standard deviation serum IG levels during the retrospective (833.8 ± 175.7 mg/dL) and the prospective (842.0 ± 188.0 mg/dL) phases were comparable; there were also no differences in the number of infections. Conclusions: Biweekly Hizentra is a noninferior option with respect to previous IVIG/SCIG-based treatment.

3.
Eur J Pediatr ; 178(1): 51-60, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30269248

RESUMO

Selective IgA deficiency is defined as absolute or partial when serum IgA level is < 7 mg/dl or 2 SD below normal for age, respectively. Few data are available on partial selective IgA deficiency, as probably most children with low serum IgA are seldom referred to a specialist clinic in common pediatric practice. The aim of our study was to better define the profile of both symptomatic forms and their clinical outcome in a pediatric immunology setting. Thus, clinical and immunological data from 103 symptomatic patients with selective IgA deficiency (53 absolute and 50 partial), 4-18 years of age, were collected at diagnosis and 80 patients (44 absolute and 36 partial) were monitored for a mean period of 5 years. Also, the prevalence of TNFRSF13B mutations has been assessed in 56 patients. The most common clinical features were infections (86/103; 83%), allergy (39/103; 38%), and autoimmunity (13/103; 13%). No significative differences were observed between absolute and partial selective IgA deficiency patients. However, a significative difference in the rate of IgA normalization between partial and absolute selective IgA deficiency patients (33 vs 9%, p = 0.01) was detected. Furthermore, a lower incidence of infections was associated to a normalization reversal compared to a final absolute or partial defect status (12 vs 53 and 64% respectively, p < 0.01).Conclusions: Regardless of a diagnosis of absolute or partial defect, monitoring of symptomatic patients with selective IgA deficiency is recommended overtime for prompt identification and treatment of associated diseases. Further, diagnostic workup protocols should be revisited in children with IgA deficiency. What is Known: ● Selective IgA Deficiency is the most common primary immunodeficiency and is usually asymptomatic. ● Symptomatic pediatric patients with selective IgA deficiency mostly suffer with respiratory and gastrointestinal infections. What is New: ● Symptomatic children with partial IgA defect may have similar clinical, immunological, and genetic features than symptomatic children with absolute IgA deficiency. ● Symptomatic children with partial IgA deficiency deserve accurate monitoring for associated diseases as per children with absolute IgA deficiency.


Assuntos
Deficiência de IgA/diagnóstico , Imunoglobulina A/sangue , Proteína Transmembrana Ativadora e Interagente do CAML/genética , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Deficiência de IgA/complicações , Deficiência de IgA/genética , Masculino , Mutação , Prevalência
5.
J Clin Immunol ; 38(5): 602-609, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29951948

RESUMO

Immunoglobulin G (IgG) replacement therapy is a standard treatment for patients with primary immunodeficiency diseases (PIDs). Hizentra®, a 20% human subcutaneous IgG (SCIG), is approved for biweekly administration for PIDs. The aim of the multicenter IBIS study was to prospectively investigate the efficacy of biweekly Hizentra® compared with previous IVIG or SCIG treatment regimens in patients with PIDs. The study consisted of a 12-month retrospective period followed by 12-month prospective observational period. The main endpoints included pre-infusion IgG concentrations, proportion of patients with serious bacterial infections (SBIs), other infections, hospitalizations due to PID-related illnesses, and days with antibiotics during the study periods. Of the 36 patients enrolled in the study, 35 patients continued the study (mean age 26.1 ± 14.4 years; 68.6% male). The mean pre-infusion IgG levels for prior immunoglobulin regimens during the retrospective period (7.84 ± 2.09 g/L) and the prospective period (8.55 ± 1.76 g/L) did not show any significant variations (p = 0.4964). The mean annual rate of SBIs/patient was 0.063 ± 0.246 for both prospective and retrospective periods. No hospitalizations related to PIDs were reported during the prospective period versus one in the retrospective period. All patients were either very (76.5%) or quite (23.5%) satisfied with biweekly Hizentra® at the end of the study. In conclusion, the IBIS study provided real-world evidence on the efficacy of biweekly Hizentra® in patients with PIDs, thus verifying the data generated by the pharmacometric modeling and simulation study in a normal clinical setting.


Assuntos
Imunoglobulina G/administração & dosagem , Síndromes de Imunodeficiência/tratamento farmacológico , Adulto , Estudos de Coortes , Esquema de Medicação , Feminino , Humanos , Síndromes de Imunodeficiência/complicações , Síndromes de Imunodeficiência/diagnóstico , Masculino , Satisfação do Paciente , Resultado do Tratamento , Adulto Jovem
6.
Vaccine ; 36(24): 3541-3554, 2018 06 07.
Artigo em Inglês | MEDLINE | ID: mdl-29426658

RESUMO

Infectious complications are a major cause of morbidity and mortality in patients with primary or secondary immunodeficiency. Prevention of infectious diseases by vaccines is among the most effective healthcare measures mainly for these subjects. However immunocompromised people vary in their degree of immunosuppression and susceptibility to infection and, therefore, represent a heterogeneous population with regard to immunization. To date there is no well- established evidence for use of vaccines in immunodeficient patients, and indications are not clearly defined even in high-quality reviews and in most of the guidelines prepared to provide recommendations for the active vaccination of immunocompromised hosts. The aim of this document is to issue recommendations based on published literature and the collective experience of the Italian primary immunodeficiency centers, about how and when vaccines can be used in immunocompromised patients, in order to facilitate physician decisions and to ensure the best immune protection with the lowest risk to the health of the patient.


Assuntos
Infecções Bacterianas/prevenção & controle , Vacinas Bacterianas/administração & dosagem , Hospedeiro Imunocomprometido , Guias de Prática Clínica como Assunto , Vacinas Virais/administração & dosagem , Viroses/prevenção & controle , Adulto , Infecções Bacterianas/imunologia , Infecções Bacterianas/microbiologia , Infecções Bacterianas/virologia , Criança , Política de Saúde , Humanos , Esquemas de Imunização , Síndromes de Imunodeficiência/microbiologia , Síndromes de Imunodeficiência/virologia , Itália , Vacinação/métodos , Vacinas de Produtos Inativados , Viroses/imunologia , Viroses/virologia
8.
J Asthma ; 48(4): 419-26, 2011 May.
Artigo em Inglês | MEDLINE | ID: mdl-21410425

RESUMO

BACKGROUND: To investigate whether an active partnership between schools, parents, and pediatricians can improve the management of asthma and quality of life of children with asthma. METHODS: A comprehensive asthma program (Happy Air®), based on a strong family-physician-school relationship, was carried out over a period of 3 years in six primary schools (2765 children). This program provides educational intervention to families, school staff, and students, as well as the administration of written questionnaires to identify children with asthma, asthma diagnosis and management, and, last but not least, extracurricular activities to improve respiratory and psychological conditions. Quality of life of children and parents, at the beginning and end of the program, was assessed using PedsQL™ 4.0 (Pediatric Quality of Life Inventory) measurement model. RESULT: Asthma was diagnosed in 135 children, of which 37 (27%) were diagnosed de novo. In all children, both single item and total clinical asthma scores showed a significant increase (p < .001) at the end of the Happy Air® program. The average scores of both the total PedsQL™ 4.0 and the four Scales were significantly increased (p < .001). CONCLUSION: Happy Air® is a model for a strategy of education- and school-based intervention for children with asthma and their families. This multi-action program for diagnosis, clinical follow-up, education, self-management, and quality-of-life control aims to minimize the socioeconomic burden of asthma disease.


Assuntos
Asma/diagnóstico , Asma/prevenção & controle , Educação em Saúde , Serviços de Saúde Escolar/organização & administração , Instituições Acadêmicas , Estudantes , Asma/fisiopatologia , Asma/psicologia , Criança , Família , Nível de Saúde , Humanos , Qualidade de Vida , Índice de Gravidade de Doença , Espirometria
9.
Clin Immunol ; 128(3): 322-8, 2008 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-18708023

RESUMO

The identification of a Btk mutation in a male patient with <2% CD19(+) B cells warrants making the diagnosis of X-linked Agammaglobulinemia (XLA). Herein we report the case of a 31 year-old male with a gradual decline of peripheral B lymphocytes and low IgA and IgM but normal IgG levels. His clinical history revealed recurrent respiratory and skin infections, sclerosing cholangitis and chronic obstructive pancreatitis. Molecular studies revealed a novel aminoacidic substitution in Btk protein (T316A). His mother, maternal aunts and a maternal female cousin were heterozygotes for the same Btk mutation and were variably affected with pulmonary emphysema. This is a puzzling case where the patient's clinical history and laboratory findings divorce molecular genetics. Either this case confirms the variable expressivity of XLA disease or the T316A change in Btk SH2 domain is a novel non-pathogenic mutation and another unknown gene alteration is responsible for the disease.


Assuntos
Agamaglobulinemia/diagnóstico , Agamaglobulinemia/genética , Linfócitos B , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Subpopulações de Linfócitos/imunologia , Mutação de Sentido Incorreto , Proteínas Tirosina Quinases/genética , Adulto , Tirosina Quinase da Agamaglobulinemia , Agamaglobulinemia/imunologia , Substituição de Aminoácidos , Linfócitos B/imunologia , Feminino , Doenças Genéticas Ligadas ao Cromossomo X/imunologia , Doenças Genéticas Ligadas ao Cromossomo X/metabolismo , Humanos , Contagem de Linfócitos , Subpopulações de Linfócitos/metabolismo , Masculino , Linhagem , Proteínas Tirosina Quinases/química , Proteínas Tirosina Quinases/metabolismo
11.
Acta Paediatr ; 96(3): 469-71, 2007 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-17407484

RESUMO

UNLABELLED: Temporary henna tattoos have become increasingly popular as a safe alternative to permanent tattoos among American and European children and teenagers during the summer holidays. Currently, temporary henna tattoos contain not only henna, but also other additives such as para-phemylenediamine (PPD), which is considered to be the chemical agent that most frequently causes skin reactions associated with the use of commercial black henna. In this report, we describe an 11-year-old boy who applied a temporary black henna tattoo on his right arm during the summer holidays in Greece and developed a severe contact dermatitis at the tattoo site with residual hypopigmentation. He had no previous history of contact dermatitis, however he did suffer from seasonal allergic rhinitis and atopic dermatitis. Patch testing revealed a strong reaction to PPD, a substance commonly contained in temporary henna tattoo preparations. CONCLUSION: Henna tattoos are an increasing problem worldwide since they carry an increased risk of severe skin reactions; therefore we suggest that the use of temporary henna tattoos in children be discouraged.


Assuntos
Corantes/efeitos adversos , Dermatite Alérgica de Contato/etiologia , Naftoquinonas/efeitos adversos , Tatuagem/efeitos adversos , Criança , Dermatite Alérgica de Contato/epidemiologia , Dermatite Alérgica de Contato/imunologia , Dermatite Atópica/epidemiologia , Humanos , Masculino , Naftoquinonas/imunologia
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