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1.
J Allergy Clin Immunol ; 144(3): 825-838, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30926529

RESUMO

BACKGROUND: Thrombocytopenia is a serious issue for all patients with classical Wiskott-Aldrich syndrome (WAS) and X-linked thrombocytopenia (XLT) because it causes severe and life-threatening bleeding. Lentiviral gene therapy (GT) for WAS has shown promising results in terms of immune reconstitution. However, despite the reduced severity and frequency of bleeding events, platelet counts remain low in GT-treated patients. OBJECTIVE: We carefully investigated platelet defects in terms of phenotype and function in untreated patients with WAS and assessed the effect of GT treatment on platelet dysfunction. METHODS: We analyzed a cohort of 20 patients with WAS/XLT, 15 of them receiving GT. Platelet phenotype and function were analyzed by using electron microscopy, flow cytometry, and an aggregation assay. Platelet protein composition was assessed before and after GT by means of proteomic profile analysis. RESULTS: We show that platelets from untreated patients with WAS have reduced size, abnormal ultrastructure, and a hyperactivated phenotype at steady state, whereas activation and aggregation responses to agonists are decreased. GT restores platelet size and function early after treatment and reduces the hyperactivated phenotype proportionally to WAS protein expression and length of follow-up. CONCLUSIONS: Our study highlights the coexistence of morphologic and multiple functional defects in platelets lacking WAS protein and demonstrates that GT normalizes the platelet proteomic profile with consequent restoration of platelet ultrastructure and phenotype, which might explain the observed reduction of bleeding episodes after GT. These results are instrumental also from the perspective of a future clinical trial in patients with XLT only presenting with microthrombocytopenia.

2.
Blood ; 132(22): 2362-2374, 2018 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-30254128

RESUMO

ARPC1B is a key factor for the assembly and maintenance of the ARP2/3 complex that is involved in actin branching from an existing filament. Germline biallelic mutations in ARPC1B have been recently described in 6 patients with clinical features of combined immunodeficiency (CID), whose neutrophils and platelets but not T lymphocytes were studied. We hypothesized that ARPC1B deficiency may also lead to cytoskeleton and functional defects in T cells. We have identified biallelic mutations in ARPC1B in 6 unrelated patients with early onset disease characterized by severe infections, autoimmune manifestations, and thrombocytopenia. Immunological features included T-cell lymphopenia, low numbers of naïve T cells, and hyper-immunoglobulin E. Alteration in ARPC1B protein structure led to absent/low expression by flow cytometry and confocal microscopy. This molecular defect was associated with the inability of patient-derived T cells to extend an actin-rich lamellipodia upon T-cell receptor (TCR) stimulation and to assemble an immunological synapse. ARPC1B-deficient T cells additionally displayed impaired TCR-mediated proliferation and SDF1-α-directed migration. Gene transfer of ARPC1B in patients' T cells using a lentiviral vector restored both ARPC1B expression and T-cell proliferation in vitro. In 2 of the patients, in vivo somatic reversion restored ARPC1B expression in a fraction of lymphocytes and was associated with a skewed TCR repertoire. In 1 revertant patient, memory CD8+ T cells expressing normal levels of ARPC1B displayed improved T-cell migration. Inherited ARPC1B deficiency therefore alters T-cell cytoskeletal dynamics and functions, contributing to the clinical features of CID.

3.
Immunol Res ; 66(4): 537-542, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-30084052

RESUMO

Newborn screening (NBS) programs for severe combined immunodeficiency (SCID), using the TREC-based assay, have enabled early diagnosis, prompt treatment, and eventually changed the natural history of affected infants. Nevertheless, it was believed that some affected infants with residual T cell, such as patients with MHC II deficiency, will be misdiagnosed by this assay. A full immune workup and genetic analysis using direct Sanger sequencing and whole exome sequencing have been performed to a patient that was identified by the Israeli NBS program for SCID. The patient was found to have severe CD4 lymphopenia with an inverted CD4/CD8 ratio, low TREC levels in peripheral blood, abnormal response to mitogen stimulation, and a skewed T cell receptor repertoire. HLA-DR expression on peripheral blood lymphocytes was undetectable suggesting a diagnosis of MHC II deficiency. Direct sequencing of the RFX5 gene revealed a stop codon change (p. R239X, c. C715T), which could cause the patient's immune phenotype. His parents were found to be heterozygote carriers for the mutation. Whole exome sequencing could not identify other potential mutations to explain his immunodeficiency. The patient underwent successful conditioned hematopoietic stem cell transplantation from healthy matched unrelated donor and is currently well and alive with full chimerism. Infants with MHC class II deficiency can potentially be identified by the TREC-based assay NBS for SCID. Therefore, MHC II molecules (e.g., HLA-DR) measurement should be part of the confirmatory immune-phenotyping for patients with positive screening results. This will make the diagnosis of such patients straightforward.

4.
J Pediatr ; 196: 154-160.e2, 2018 May.
Artigo em Inglês | MEDLINE | ID: mdl-29395171

RESUMO

OBJECTIVES: To describe the prevalence, natural course, outcome, and risk factors of post-transplant de novo allergy and autoimmunity. STUDY DESIGN: A cross-sectional, cohort study of all children (<18 years) who underwent a solid-organ transplantation, between 2000 and 2012, in a single transplant center, with a follow-up period of 6 months or more post-transplant and without history of allergy or immune-mediated disorder pretransplant. RESULTS: A total of 626 eligible patients were screened, and 273 patients (160 males; 59%) met the inclusion criteria; this included 111 liver, 103 heart, 52 kidney, and 7 multivisceral recipients. Patients were followed for a median period of 3.6 years. A total of 92 (34%) patients (42 males, 46%) developed allergy or autoimmune disease after transplantation, with a high prevalence among liver (41%), heart (40%), and multivisceral (57%) transplant recipients compared with kidney recipients (4%; P < .001). Post-transplant allergies included eczema (n = 44), food allergy (22), eosinophilic gastrointestinal disease (11), and asthma (28). Autoimmunity occurred in 18 (6.6%) patients, presenting mainly as autoimmune cytopenia (n = 10). In a multivariate analysis, female sex, young age at transplantation, family history of allergy, Epstein-Barr virus infection, and elevated eosinophil count >6 months post-transplantation were associated with an increased risk for allergy or autoimmunity. Two patients (0.7%) died from autoimmune hemolytic anemia and hemophagocytic lymphohistiocytosis, and 52 episodes of post-transplant allergy, autoimmunity, and immune-mediated disorders (37%) did not improve over time. CONCLUSIONS: Allergy and autoimmunity are common in pediatric liver, heart, and multivisceral transplant recipients and pose a significant health burden. Further studies are required to clarify the mechanisms behind this post-transplant immune dysregulation.

5.
J Allergy Clin Immunol Pract ; 6(4): 1238-1242, 2018 Jul - Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29198698

RESUMO

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening delayed drug-induced hypersensitivity reaction. The most frequently reported drugs causing DRESS are aromatic antiepileptic agents. Prompt withdrawal of the offending drug and administering systemic corticosteroids is the most widely accepted and used treatment. The treatment of severe DRESS not responsive to systemic corticosteroids is uncertain. OBJECTIVE: The objective of this study was to describe a case series of pediatric patients with DRESS who were treated successfully with intravenous immunoglobulins (IVIGs). METHODS: A retrospective review of all children hospitalized in a tertiary care children's hospital with severe DRESS syndrome who received IVIG in addition to offending drug withdrawal and systemic corticosteroids during 1999-2017 is performed. RESULTS: Seven severe DRESS patients (4 males, age: 9.5 ± 5.7 years) are described. The offending drugs were antiepileptics in all but one case. Clinical findings included fever, rash, lymphadenopathy, dyspnea, anasarca, and hepatic involvement. After IVIG treatment (total dosage: 1-2 g/kg), fever resolved within a median time of 1 (range, 0-5) day, rash disappeared after 6.3 ± 1.6 days, and liver enzymes substantially improved after 3.8 ± 1.6 days. Patients were discharged 6.1 ± 2.7 days after IVIG commencement. There was no mortality. CONCLUSION: The addition of IVIG in DRESS syndrome resistant to regular drug withdrawal and systemic corticosteroid therapy may hasten disease recovery.

6.
Front Immunol ; 8: 1448, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-29167666

RESUMO

Severe combined immunodeficiency (SCID), the most severe form of T cell immunodeficiency, is detectable through quantification of T cell receptor excision circles (TRECs) in dried blood spots obtained at birth. Herein, we describe the results of the first year of the Israeli SCID newborn screening (NBS) program. This important, life-saving screening test is available at no cost for every newborn in Israel. Eight SCID patients were diagnosed through the NBS program in its first year, revealing an incidence of 1:22,500 births in the Israeli population. Consanguine marriages and Muslim ethnic origin were found to be a risk factor in affected newborns, and a founder effect was detected for both IL7Rα and DCLRE1C deficiency SCID. Lymphocyte subset analysis and TREC quantification in the peripheral blood appear to be sufficient for confirmation of typical and leaky SCID and ruling out false positive (FP) results. Detection of secondary targets (infants with non-SCID lymphopenia) did not significantly affect the management or outcomes of these infants in our cohort. In the general, non-immunodeficient population, TREC rises along with gestational age and birth weight, and is significantly higher in females and the firstborn of twin pairs. Low TREC correlates with both gestational age and birth weight in extremely premature newborns. Additionally, the rate of TREC increase per week consistently accelerates with gestational age. Together, these findings mandate a lower cutoff or a more lenient screening algorithm for extremely premature infants, in order to reduce the high rate of FPs within this group. A significant surge in TREC values was observed between 28 and 30 weeks of gestation, where median TREC copy numbers rise by 50% over 2 weeks. These findings suggest a maturational step in T cell development around week 29 gestation, and imply moderate to late preterms should be screened with the same cutoff as term infants. The SCID NBS program is still in its infancy, but is already bearing fruit in the early detection and improved outcomes of children with SCID in Israel and other countries.

7.
J Immunol ; 199(12): 4036-4045, 2017 12 15.
Artigo em Inglês | MEDLINE | ID: mdl-29127144

RESUMO

Regulation of the actin cytoskeleton is crucial for normal development and function of the immune system, as evidenced by the severe immune abnormalities exhibited by patients bearing inactivating mutations in the Wiskott-Aldrich syndrome protein (WASP), a key regulator of actin dynamics. WASP exerts its effects on actin dynamics through a multisubunit complex termed Arp2/3. Despite the critical role played by Arp2/3 as an effector of WASP-mediated control over actin polymerization, mutations in protein components of the Arp2/3 complex had not previously been identified as a cause of immunodeficiency. Here, we describe two brothers with hematopoietic and immunologic symptoms reminiscent of Wiskott-Aldrich syndrome (WAS). However, these patients lacked mutations in any of the genes previously associated with WAS. Whole-exome sequencing revealed a homozygous 2 bp deletion, n.c.G623DEL-TC (p.V208VfsX20), in Arp2/3 complex component ARPC1B that causes a frame shift resulting in premature termination. Modeling of the disease in zebrafish revealed that ARPC1B plays a critical role in supporting T cell and thrombocyte development. Moreover, the defects in development caused by ARPC1B loss could be rescued by the intact human ARPC1B ortholog, but not by the p.V208VfsX20 variant identified in the patients. Moreover, we found that the expression of ARPC1B is restricted to hematopoietic cells, potentially explaining why a mutation in ARPC1B has now been observed as a cause of WAS, whereas mutations in other, more widely expressed, components of the Arp2/3 complex have not been observed.


Assuntos
Complexo 2-3 de Proteínas Relacionadas à Actina/genética , Plaquetas/patologia , Mutação da Fase de Leitura , Síndromes de Imunodeficiência/genética , Linfopoese/genética , Linfócitos T/patologia , Trombopoese/genética , Citoesqueleto de Actina/metabolismo , Complexo 2-3 de Proteínas Relacionadas à Actina/deficiência , Complexo 2-3 de Proteínas Relacionadas à Actina/metabolismo , Complexo 2-3 de Proteínas Relacionadas à Actina/fisiologia , Pré-Escolar , Códon sem Sentido , Consanguinidade , Evolução Fatal , Humanos , Lactente , Masculino , Complexos Multiproteicos , Linhagem , Polimerização , Recombinação V(D)J , Síndrome de Wiskott-Aldrich/genética , Proteínas de Peixe-Zebra/deficiência , Proteínas de Peixe-Zebra/genética
8.
Am J Hematol ; 92(1): 28-36, 2017 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-27701760

RESUMO

Chronic granulomatous disease (CGD) is an innate immunodeficiency with a genetic defect of the nicotinamide adenosine dinucleotide phosphate, reduced, oxidase components. This leads to decreased reactive oxygen species (ROS) production, which renders patients susceptible to life-threatening infections. Over the course of 30 years, we diagnosed CGD in 84 patients from 61 families using functional, molecular, and genetic studies. The incidence of CGD in Israel is 1.05 per 100,000 live-births in the Jewish population and 1.49 in the Israeli Arab population. We diagnosed 52 patients (62%) with autosomal recessive inheritance (AR-CGD) and 32 (38%) with X-linked recessive inheritance (XLR-CGD). Consanguinity was detected in 64% of AR-CGD families (14% in Jews and 50% in Israeli Arabs). We found 36 different mutations (23 in XLR-CGD and 13 in AR-CGD patients), 15 of which were new. The clinical spectrum of CGD varied from mild to severe disease in both XLR and AR forms, although the AR subtype is generally milder. Further, residual ROS production correlated with milder clinical expression, better prognosis and improved overall survival. Patients with recurrent pyogenic infections developed fibrosis and hyperinflammatory states with granuloma formation. The management of CGD has progressed substantially in recent years, evolving from a fatal disease of early childhood to one of long-term survival. Our present cohort displays an encouraging 81% overall long term survival. Early hematopoietic stem cell transplantation is advisable before tissue damage is irreversible. Successful transplantation was performed in 18/21 patients. Therapeutic gene modification could become an alternative cure for CGD. Am. J. Hematol. 92:28-36, 2017. © 2016 Wiley Periodicals, Inc.


Assuntos
Cromossomos Humanos X/genética , Genes Recessivos , Doença Granulomatosa Crônica/genética , Transplante de Células-Tronco Hematopoéticas , NADPH Oxidases/genética , Espécies Reativas de Oxigênio/metabolismo , Adolescente , Adulto , Idoso , Infecções Bacterianas/microbiologia , Criança , Pré-Escolar , Consanguinidade , Feminino , Doença Granulomatosa Crônica/metabolismo , Doença Granulomatosa Crônica/microbiologia , Doença Granulomatosa Crônica/terapia , Humanos , Lactente , Israel , Masculino , Pessoa de Meia-Idade , Mutação , Micoses/microbiologia , Adulto Jovem
9.
Pediatr Dermatol ; 32(5): 701-3, 2015 Sep-Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26223537

RESUMO

BACKGROUND: Nonbullous erythema multiforme (NBEM) is an acute, immune-mediated, self-limiting skin disease with distinctive target lesions. Its pathogenesis is unclear, but most cases are considered to be infection related or drug related. In adults, the main precipitating factor is infection. This study reviewed a 10-year experience with hospitalized children with NBEM in a tertiary pediatric medical center in Israel with a focus on precipitating factors. METHODS: The medical files of all children hospitalized from 2001 to 2011 with the diagnosis of NBEM were reviewed. RESULTS: Ninety-seven patients (55 boys and 42 girls) met the inclusion criteria. The mean age was 4.0 years. At least one precipitating factor was recognized in 72 cases; the remainders were classified as idiopathic. The most common factor was drugs (n = 45), particularly penicillin (n = 30), followed by infection with various pathogens (n = 27) including Epstein-Barr virus (7), group A Streptococcus (n = 6), Mycoplasma pneumoniae (n = 5) and herpes simplex virus (n = 4). Analysis according to age showed that medication was the most common precipitating factor in the first year of life, and infection was as common as drugs in the older age groups (1-18 years). CONCLUSIONS: Unlike adult NBEM, the majority of pediatric NBEM is associated with medications, especially penicillin. This may be due to the frequent use of antibiotics in children.


Assuntos
Eritema Multiforme/etiologia , Adolescente , Criança , Criança Hospitalizada , Pré-Escolar , Erupção por Droga/etiologia , Eritema Multiforme/diagnóstico , Feminino , Humanos , Lactente , Israel , Masculino , Estudos Retrospectivos , Fatores de Risco , Inquéritos e Questionários
10.
Pediatr Infect Dis J ; 34(7): 794-5, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25886786

RESUMO

Cervical discitis, though rare, should be included in the differential diagnosis of torticollis, neck pain and neurodevelopmental regression in motor skills in children and infants. Magnetic resonance imaging is the diagnostic method of choice. Treatment should be conservative with antibiotics only. The aim of this study was to describe the 10-year experience of a tertiary pediatric medical center with cervical discitis.


Assuntos
Antibacterianos/uso terapêutico , Vértebras Cervicais/patologia , Discite/diagnóstico , Discite/tratamento farmacológico , Vértebras Cervicais/diagnóstico por imagem , Criança , Pré-Escolar , Diagnóstico Diferencial , Discite/patologia , Feminino , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Radiografia , Centros de Atenção Terciária
12.
J Allergy Clin Immunol ; 128(5): 1050-7, 2011 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-21757226

RESUMO

BACKGROUND: CD3δ deficiency is a fatal form of severe combined immunodeficiency that can be cured by hematopoietic stem cell transplantation (HSCT). The presence of a thymus loaded with T-cell progenitors in patients with CD3δ deficiency may require special considerations in choosing the regimen of conditioning and the type of HSCT. OBJECTIVES: To study the outcome of CD3δ deficiency by using various modalities of stem cell transplantation. METHODS: We analyzed data on 13 patients with CD3δ deficiency who underwent HSCT in 7 centers. HSCT was performed by using different sources of donor stem cells as well as various conditioning regimens. RESULTS: One patient received stem cells from a matched related donor and survived after a second transplant, needing substantial conditioning in order to engraft. Only 2 of 7 other patients who received a mismatched related donor transplant survived; 2 of them had no conditioning, whereas the others received various combinations of conditioning regimens. Engraftment of T cells in the survivors appears incomplete. Three other patients who received stem cells from a matched unrelated donor survived and enjoyed full immune reconstitution. Two patients received unrelated cord blood without conditioning. One of them has had a partial but stable engraftment, whereas the other engrafted well but is only 12 months after HSCT. We also report here for the first time that patients with CD3δ deficiency can present with typical features of Omenn syndrome. CONCLUSIONS: HSCT is a successful treatment for patients with CD3δ deficiency. The small number of patients in this report prevents definitive statements on the importance of survival factors, but several are suggested: (1) HLA-matched donor transplants are associated with superior reconstitution and survival than are mismatched donor transplants; (2) substantial conditioning appears necessary; and (3) early diagnosis and absence of opportunistic infections may affect outcome.


Assuntos
Complexo CD3/imunologia , Transplante de Células-Tronco Hematopoéticas/métodos , Imunodeficiência Combinada Severa/imunologia , Imunodeficiência Combinada Severa/cirurgia , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Complicações Pós-Operatórias/epidemiologia , Condicionamento Pré-Transplante/métodos
13.
Eur J Pediatr ; 169(1): 35-7, 2010 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-19308447

RESUMO

A 4-month-old baby with a family history of hyper-IgE syndrome acquired Pneumocystis jirovecii pneumonia. The patient's hyper-IgE syndrome score was low, but a genetic study yielded a STAT3 mutation. P. jirovecii pneumonia can be added to the infections associated with hyper-IgE syndrome. In some cases, it may be the presenting manifestation of this immunodeficiency.


Assuntos
Imunoglobulina E/sangue , Síndrome de Job/complicações , Pneumocystis carinii/isolamento & purificação , Pneumonia por Pneumocystis/etiologia , Anticorpos Antibacterianos/análise , Líquido da Lavagem Broncoalveolar/microbiologia , DNA Bacteriano/análise , Diagnóstico Diferencial , Humanos , Lactente , Síndrome de Job/sangue , Síndrome de Job/imunologia , Masculino , Pneumocystis carinii/genética , Pneumocystis carinii/imunologia , Pneumonia por Pneumocystis/diagnóstico , Pneumonia por Pneumocystis/microbiologia , Radiografia Torácica , Linfócitos T Reguladores/imunologia
14.
Immunol Lett ; 130(1-2): 97-106, 2010 May 04.
Artigo em Inglês | MEDLINE | ID: mdl-20005258

RESUMO

Hyper IgE Syndrome (HIES) is a rare genetic disorder, characterized by elevated serum IgE levels and reduced inflammatory responses to bacterial infections. This leads to dermatitis, recurrent lung infections and "cold abscesses". Recently, progress was made in HIES research, when mutations in STAT3 were found in the autosomal dominant form of HIES, and impaired responses of T helper 17 cells were reported. However, the causes for reduced inflammatory responses in these patients were not fully elucidated. In view of studies that indicated that polymorphonuclear leukocytes (PMN) of HIES patients are defective in their chemotactic properties, we asked if the PMN of these patients have reduced expression of receptors for chemoattractants. To analyze this possibility, we focused on fMLP and ELR(+)-CXC chemokines - which are essential for mounting acute inflammatory responses - and determined the coding sequences and expression levels of their corresponding receptors: FPR (for fMLP) as well as CXCR1 and CXCR2 (the receptors for ELR(+)-CXC chemokines). The analyses of these receptors in HIES patients indicated that their coding sequences were intact and normal. However, the percentages of PMN that expressed FPR, CXCR1 and CXCR2 were significantly lower in HIES patients. In addition, lower expression levels per cell were denoted for CXCR1 in PMN of the patients. A cumulative score that was calculated for the three chemoattractant receptors together indicated that in some of the patients there were prominent reductions, of up to approximately 50% in the overall expression of the receptors (indicated by % positive cells and mean expression levels per cell). In addition, we asked whether deregulation of PMN activities in HIES may result from binding of IgE to corresponding receptors on HIES PMN. Our findings indicate that this is probably not the case, because similarly to normal PMN, the cells of HIES patients did not express notable levels of the IgE receptors FcvarepsilonRI and FcvarepsilonRII. Together, these results provide novel information on the expression of key determinants in PMN migration in HIES, suggesting that a defect in the expression of chemoattractant receptors may lead to impaired chemotaxis found in HIES patients, and to decreased inflammatory responses.


Assuntos
Regulação para Baixo , Síndrome de Job/imunologia , Neutrófilos/imunologia , Receptores de Formil Peptídeo/metabolismo , Adolescente , Adulto , Movimento Celular/genética , Movimento Celular/imunologia , Pré-Escolar , Feminino , Imunofluorescência , Humanos , Síndrome de Job/genética , Síndrome de Job/fisiopatologia , Masculino , Receptores de Formil Peptídeo/genética , Receptores de Interleucina-8A/genética , Receptores de Interleucina-8A/metabolismo , Receptores de Interleucina-8B/genética , Receptores de Interleucina-8B/metabolismo
15.
J Asthma ; 46(7): 652-5, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19728199

RESUMO

BACKGROUND: The seasonality of asthma morbidity is well recognized. A peak in asthma exacerbations in September has been noted for years at our center. OBJECTIVE: To examine the hypothesis that the increment in asthma exacerbations in September is influenced by the beginning of the kindergarten and school year. METHODS: The monthly admission rate for asthma in patients of different ages was retrospectively evaluated in seven hospitals from various areas in Israel from January 2003 to December 2005. RESULTS: Of the 408,242 hospital admissions during the study period, 8,011 were for asthma exacerbations: 4,091 in adults (1.3% of adult admissions) and 3,920 in children (3.8% of pediatric admissions). The asthma admission rates varied considerably throughout the year, with a peak of 4% of total admissions in the winter months and a nadir of 2% in the summer months. September was unique for its particularly high rate of admissions for asthma attacks in children (6% of total admissions), especially toddlers and the school-age group. In adults there was a progressive increase in asthma admissions from September through December without a unique peak in September. CONCLUSIONS: There is a characteristic increase in asthma exacerbations and admissions in September in the pediatric age group. This phenomenon might be explained by the increased exposure to respiratory viruses, to new allergen exposure in school or kindergarten, increased emotional stress due to start of the new school year, or poor compliance and withdrawal of treatment during the summer. Clinicians should consider administering prophylactic treatment for asthma in children before onset of the school year.


Assuntos
Asma/epidemiologia , Estações do Ano , Adolescente , Adulto , Asma/tratamento farmacológico , Asma/prevenção & controle , Criança , Pré-Escolar , Férias e Feriados/psicologia , Hospitalização/estatística & dados numéricos , Humanos , Lactente , Israel/epidemiologia , Adesão à Medicação/psicologia , Instituições Acadêmicas , Estresse Psicológico/complicações , Viroses/complicações
16.
Pediatr Emerg Care ; 25(5): 339-41, 2009 May.
Artigo em Inglês | MEDLINE | ID: mdl-19444032

RESUMO

OBJECTIVE: To present 3 children with hypersensitivity reaction to methylprednisolone sodium-succinate and review the literature regarding such reactions. METHODS: Data on the clinical features were obtained from the children's files. Skin prick tests were performed with a panel of corticosteroid preparations. RESULTS: Three patients (5, 7, and 8 years) with asthma who were treated with intravenous methylprednisolone succinate at the emergency department developed hypersensitivity reactions initially consider to be due to their primary disease. Two had a positive skin prick test to methylprednisolone sodium succinate but not to other corticosteroids or to the succinate ester. Skin prick tests to different corticosteroids, performed 4 years after the event in the third patient, were negative. CONCLUSIONS: Methylprednisolone sodium-succinate may cause anaphylactic/anaphylactoid reactions in children. Our patients' histories emphasize the importance of awareness to corticosteroid-induced reactions, especially in children with asthma in whom the symptoms may be considered as an exacerbation of their primary illness.


Assuntos
Anafilaxia/induzido quimicamente , Antiasmáticos/efeitos adversos , Hipersensibilidade a Drogas/etiologia , Hemissuccinato de Metilprednisolona/efeitos adversos , Antiasmáticos/administração & dosagem , Antiasmáticos/uso terapêutico , Asma/complicações , Asma/diagnóstico , Asma/tratamento farmacológico , Criança , Pré-Escolar , Erros de Diagnóstico , Erupção por Droga/etiologia , Hipersensibilidade a Drogas/diagnóstico , Emergências , Dermatoses Faciais/induzido quimicamente , Humanos , Infusões Intravenosas , Testes Intradérmicos , Hipersensibilidade a Leite/complicações , Hipersensibilidade a Leite/diagnóstico , Hipersensibilidade a Leite/tratamento farmacológico
18.
Eur J Pediatr ; 168(2): 233-5, 2009 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-18496713

RESUMO

Asplenia was diagnosed in four patients with autoimmune polyendocrine syndrome type-I (APS-I): two children, aged 2-4 years, from the same family and two adults, the father of the two children and his cousin. We have observed a worsening in splenic function in the children during a follow-up of a few years. Patients with APS-I should be evaluated for splenic function, since splenic dysfunction has important therapeutic implications, especially in children.


Assuntos
Poliendocrinopatias Autoimunes/genética , Baço/anormalidades , Adulto , Antibacterianos/administração & dosagem , Criança , Pré-Escolar , Consanguinidade , Feminino , Seguimentos , Humanos , Irã (Geográfico)/etnologia , Israel , Judeus/genética , Assistência de Longa Duração , Masculino , Fenótipo , Vacinas Pneumocócicas/administração & dosagem , Poliendocrinopatias Autoimunes/diagnóstico , Poliendocrinopatias Autoimunes/terapia
20.
Eur J Emerg Med ; 15(3): 158-61, 2008 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18460957

RESUMO

BACKGROUND: C-reactive protein (CRP) values are clinically useful in differentiating viral from bacterial illnesses in children, but the regular test is impractical in the emergency department (ED) setting. OBJECTIVE: To investigate the validity and feasibility of the 2-min bedside Quick Read (QR)-CRP test in the prediction of bacterial pneumonia in children in the ED. METHODS: Fifty randomly selected children aged 4 days to 17 years, who presented to a pediatric ED with symptoms and signs of pneumonia over a 6-month period, were prospectively studied. The diagnosis of bacterial/viral pneumonia was based on clinical and radiological findings. CRP was measured in leftover blood (0.2 ml) using the QR-CRP kit. Clinical and laboratory data were statistically analyzed against CRP values for ability to predict bacterial pneumonia. RESULTS: Thirty-six patients (72%) were diagnosed with bacterial pneumonia and 14 (28%) with viral pneumonia; mean CRP levels were 121.3+/-122 and 27.2+/-26 mg/l, respectively (P=0.007). Significantly higher CRP levels were associated with bacterial than with viral pneumonia in the patients who presented before 96 h of symptom onset (P=0.013-0.028), but not in those who presented later. On receiver operating characteristics analysis, CRP was a better indicator of a chest radiograph picture of bacterial pneumonia (area under the curve=0.79) than absolute neutrophil count (0.78) or white blood cell count (0.73). Combining all three parameters yielded an area of 0.865. CONCLUSION: The QR-CRP test seems to be an useful predictor of bacterial pneumonia in children, especially those with a shorter illness duration, and is feasible for use in the ED.


Assuntos
Proteína C-Reativa/análise , Serviço Hospitalar de Emergência , Pneumonia Bacteriana/diagnóstico , Kit de Reagentes para Diagnóstico/normas , Adolescente , Biomarcadores/sangue , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pneumonia Bacteriana/sangue , Pneumonia Viral/sangue , Pneumonia Viral/diagnóstico , Sistemas Automatizados de Assistência Junto ao Leito , Curva ROC
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