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1.
J Clin Immunol ; 2020 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-31993866

RESUMO

Patients with Down syndrome (DS) are characterized by increased susceptibility to autoimmunity and respiratory tract infections that are suggestive of humoral immunity impairment. Here, we sought to determine the follicular helper (Tfh) and follicular regulatory (Tfr) T cell profile in the blood of children with DS. Blood was collected from 24 children with DS, nine of which had autoimmune diseases. Children with DS showed skewed Tfh differentiation towards the CXCR3+ phenotype: Tfh1 and Tfh1/17 subsets were increased, while Tfh2 and Tfh17 subsets were reduced. While no differences in the percentage of Tfr cells were seen, the ratio of Tfh1 and CXCR3+PD-1+ subsets to Tfr cells was significantly increased in the affected children. The excessive polarization towards a CXCR3+ phenotype in children with DS suggests that re-calibration of Tfh subset skewing could potentially offer new therapeutic opportunities for these patients.

2.
Ann Hematol ; 2020 Jan 21.
Artigo em Inglês | MEDLINE | ID: mdl-31965272

RESUMO

Oral ferrous salts are standard treatment for children with iron deficiency anemia (IDA). The objective of our study was to monitor oral iron therapy in children, aged 3 months-12 years, with IDA. We prospectively collected clinical and hematological data of children with IDA, from 15 AIEOP (Associazione Italiana di Ematologia ed. Oncologia Pediatrica) centers. Response was measured by the increase of Hb from baseline. Of the 107 analyzed patients, 18 received ferrous gluconate/sulfate 2 mg/kg (ferrous 2), 7 ferrous gluconate/sulfate 4 mg/kg (ferrous 4), 7 ferric iron salts 2 mg/kg (ferric), 62 bis-glycinate iron 0.45 mg/kg (glycinate), and 13 liposomal iron 0.7-1.4 mg/kg (liposomal). Increase in reticulocytes was evident at 3 days, while Hb increase appeared at 2 weeks. Gain of Hb at 2 and 8 weeks revealed a higher median increase in both ferrous 2 and ferrous 4 groups. Gastro-intestinal side effects were reported in 16% (ferrous 2), 14% (ferrous 4), 6% (glycinate), and 0 (ferric and liposomal) patients. The reticulocyte counts significantly increased after 3 days from the start of oral iron supplementation. Bis-glycinate iron formulation had a good efficacy/safety profile and offers an acceptable alternative to ferrous iron preparations.

3.
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.

4.
Nutrients ; 11(10)2019 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-31597323

RESUMO

The diagnosis of cow's milk allergy (CMA) is particularly challenging in infants, especially with non-Immunoglobulin E (IgE)-mediated manifestations, and inaccurate diagnosis may lead to unnecessary dietary restrictions. The aim of this study was to assess the accuracy of the cow's milk-related symptom score (CoMiSSTM) in response to a cow's milk-free diet (CMFD). We prospectively recruited 47 infants (median age three months) who had been placed on a CMFD due to persisting unexplained gastrointestinal symptoms. We compared data with 94 healthy controls (median age three months). The CoMiSSTM score was completed at recruitment and while on the exclusion diet. In 19/47 (40%) cases a response to the diet occurred. At recruitment CoMiSSTM was significantly higher in cases compared to controls (median score 8 vs. 3; p-value: <0.05), 9 cases had a score ≥12 and 8/9 normalized on CMFD. An oral milk challenge was performed in all 19 responders and six of these had a positive reaction to cow's milk (CM). In eight infants IgE allergy tests were positive. The receiver operation characteristic (ROC) curve identified a CoMISSTM score of 9 to be the best cut-off value (84% sensitivity, 85% specificity, 80% positive (PPV) and 88% negative predictive value (NPV)) for the response to CMFD. We found CoMiSSTM to be a useful tool to help identify infants with persisting gastrointestinal symptoms and suspected CMA that would benefit from CMFD.

5.
J Pediatr Neurosci ; 14(1): 38-41, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31316642

RESUMO

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing sensorimotor disorder presumably due to antibody-mediated reactions. It is a rare condition in children, with estimated prevalence as 0.48 per 100,000 among patients younger than 20 years of age. Recommended treatments include immune modulators, intravenous immunoglobulins (IVIgs), steroids, and plasmapheresis. Management of pediatric CIDP is challenging because of the lack of evidence-based efficacy of the current therapies in children. Because of the rarity of this condition, there are no double-blind randomized studies to support the therapeutic choice as well as to identify the optimal first-line therapeutic regimen. IVIgs are widely used but the intravenous administration is usually uncomfortable, especially for children. Subcutaneous immunoglobulins (SCIgs) have proven to be effective in adults with CIDP and in children affected by antibody deficiencies and other different immune and inflammatory disorders. Herein, we described the case of a 7-year-old boy, affected by CIDP who clinically responded to IVIg but was dependent on this therapy. In order to improve his quality of life, we switched to SCIg with excellent result.

6.
Pediatr Hematol Oncol ; 34(5): 343-347, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29200317

RESUMO

Gastrointestinal stromal tumors (GIST) are tumors of the gastrointestinal (GI) tract originating from the myenteric ganglion cells (interstitial cells of Cajal), that are very rare in children and adolescents. The most common clinical manifestation is acute or chronic, overt or occult GI bleeding although these tumors are asymptomatic in 10-30% of patients. We report a case of gastric GIST in a 11-year-old girl presenting with an iron deficiency refractory anemia without gastrointestinal symptoms and stool evidence of GI bleeding that caused a slight diagnostic delay.


Assuntos
Anemia Ferropriva/diagnóstico , Hemorragia Gastrointestinal/diagnóstico , Neoplasias Gastrointestinais/diagnóstico , Criança , Feminino , Humanos
7.
Ital J Pediatr ; 39: 51, 2013 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-24004953

RESUMO

BACKGROUND: In the care of feverish children, symptomatic management is pivotal. Thus, the Italian Pediatric Society has recently published guidelines on fever management in children. Our aim was to investigate whether pediatric hospitalists, community pediatricians and pediatric residents differ in their every-day clinical practice with respect to symptomatic management of feverish children. METHODS: 79 out of 118 physicians involved in pediatric care in an area of Northern Lombardy (Italy) filled in a modified version of the questionnaire derived from the Swiss national survey on symptomatic fever management. RESULTS: Pediatric hospitalists (N = 29), community pediatricians (N = 30) and pediatric residents (N = 20) did not differ with respect to temperature threshold for symptomatic fever treatment, role of general appearance in modulating the threshold for fever management, first choice antipyretic drug, frequency of ibuprofen prescription, prescription of physical antipyresis, influence of exaggerated fear of fever on its management and potential to reassure families about this fear.On the other side, some significant differences were found. Pediatric residents more frequently lower the treatment threshold in children with a past history of febrile seizures (P < 0.001) and prescribe an aggressive treatment for fever not responding to the first antipyretic drug (P < 0.01) than their more experienced colleagues. Community pediatricians represent the unique investigated group using homeopathic remedies, both in the acute setting (P < 0.001) as well as a prophylaxis (P < 0.0001). Finally, paediatric residents less often (P < 0.05) stated to encounter exaggerated fear of fever among parents than their more experienced colleagues. CONCLUSIONS: The present explorative inquiry globally shows limited discordance among pediatric residents, community pediatricians and pediatric hospitalists with respect to symptomatic fever management.


Assuntos
Antipiréticos/administração & dosagem , Febre/tratamento farmacológico , Padrões de Prática Médica , Inquéritos e Questionários , Antipiréticos/farmacologia , Atitude do Pessoal de Saúde , Criança , Pré-Escolar , Serviços de Saúde Comunitária , Estudos Transversais , Feminino , Febre/diagnóstico , Médicos Hospitalares/estatística & dados numéricos , Hospitais Pediátricos , Humanos , Lactente , Internato e Residência/estatística & dados numéricos , Itália , Masculino , Pediatria/métodos , Resultado do Tratamento
8.
Case Reports Immunol ; 2013: 296827, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-25374739

RESUMO

Ataxia-telangiectasia (AT) is the most frequent progressive cerebellar ataxia in infancy and childhood. Immunodeficiency which includes both cellular and humoral arms has variable severity. Since the clinical presentation is extremely variable, a high clinical suspicion will allow an early diagnosis. Serum alpha-fetoprotein is elevated in 80-85% of patients and therefore could be used as a screening tool. Here, we present a case of a 5-year-old female infant who was admitted to our department at the age of 16 months because of gait disorders and febrile episodes that had begun at 5 months after the cessation of breastfeeding. Serum alfa-fetoprotein level was elevated. Other investigations showed leukocytopenia with lymphopenia, reduced IgG2 and IgA levels, and low titers of specific postimmunization antibodies against tetanus toxoid and Haemophilus B polysaccharide. Peripheral lymphocytes subsets showed reduction of T cells with a marked predominance of T cells with a memory phenotype and a corresponding reduction of naïve T cells; NK cells were very increased (41%) with normal activity. The characterization of the ATM gene mutations revealed 2 specific mutations (c.5692C > T/c.7630-2A > C) compatible with AT diagnosis. It was concluded that AT syndrome should be considered in children with precocious signs of cerebellar ataxia and recurrent fever episodes.

9.
Immunology ; 121(2): 183-8, 2007 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-17313486

RESUMO

The objective of this study was to describe a novel form of primary immune disorder characterized by circulating B cells with the exclusive transitional phenotype which fail to respond to CpG stimulation. The 12-year-old male patient suffered from recurrent bacterial infections since infancy. The immunological studies were based on extensive B cell immunophenotyping, humoral in vivo response to different vaccine antigens, and in vitro proliferation and immunoglobulin production after CpG stimulation. Sequence analysis for potentially candidate genes such as IRF8, MyD88, TLR9, T-bet were performed. The patient's serum immunoglobulin levels and the specific antibody response to tetanus toxoid were normal, whereas that to polysaccharide antigens was severely impaired. Flow cytometric analysis showed that almost all patient's peripheral B cells had the transitional phenotype (CD24(bright) CD38(bright) CD27(neg)). Furthermore, the patient's B cells did not proliferate and failed to secrete immunoglobulins after in vitro CpG stimulation. Sequence analysis for TLR9, MyD88, IRF8 and T-bet showed no mutations. To our knowledge, this is the first case of a novel primary immunodeficiency mimicking the clinical phenotype of common variable immunodeficiency, with a peculiar immunological phenotype characterized by normal immunoglobulin serum levels, circulating B cells with the exclusive transitional phenotype unable to respond to CpG stimulation. This defines a novel form of primary immunodeficiency mimicking common variable immunodeficiency in the presence of normal immunoglobulin serum levels.


Assuntos
Subpopulações de Linfócitos B/imunologia , Ilhas de CpG/imunologia , Síndromes de Imunodeficiência/imunologia , Criança , Imunodeficiência de Variável Comum/diagnóstico , Diagnóstico Diferencial , Humanos , Síndromes de Imunodeficiência/diagnóstico , Imunofenotipagem , Ativação Linfocitária/imunologia , Masculino , Infecções Oportunistas/imunologia , Pneumonia Bacteriana/imunologia , Recidiva
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