Subcutaneous IgG Replacement Therapy by Push in 32 Patients with Primary Immunodeficiency Diseases in Argentine

Introduction: Regular replacement with immunoglobulin infusions is the mainstay of treatment in the majority of primary immunodeficiencies. Several studies showed that Subcutaneous Immunoglobulin (SCIG) has similar efficacy to Intravenous Immunoglobulin (IVIG) in preventing infections in PID patients. Here we report effectiveness, safety and tolerance of SCIG replacement therapy by push in 32 pediatric and adult patients with humoral PID in Argentina. Results: We describe 32 patients that received SCIG treatment between July 2011 and May 2012. 17 male and 15 female from 2 Immunology Centers; aged from 8 months to 40 years (median: 11 years). 30 patients previously received IVIG treatment. Among them, fifteen received 9 months of SCIG treatment administered by pump. The other 2 patients started the immunoglobulin replacement treatment directly with SCIG by push. The mean dose of SCIG was 133 mg/kg/week (range 100-192). The annual rate of any infection was 1, 2 infection/year/patient for subcutaneous treatment. The frequency of adverse effects was 0.02% with the SCIG. At the end of the study, all patients chose SCIG home-therapy regimen and referred much more comfort with SCIG by push. Conclusion: Self-administered subcutaneous immunoglobulin therapy by push is an effective and safe alternative therapy for patients with PID.Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Gómez Raccio, Andrea. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; ArgentinaFil: Regairaz, Lorena. Provincia de Buenos Aires. Ministerio de Salud. Hospital de Niños “Sor María Ludovica”; ArgentinaFil: Díaz Ballve, Damacia. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; ArgentinaFil: Seminario, Gisela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; ArgentinaFil: Moreira, Ileana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; ArgentinaFil: Giovanni, Daniela Di. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; Argentin

Hypogammaglobulinaemia secondary to cow-milk allergy in children under 2 years of age

Symptomatic hypogammaglobulinaemia in children younger than 2 years of age was studied to rule out a primary immunodeficiency. Thirty-four patients were referred to the Immunology Service to study the hypogammaglobulinaemia- associated clinical picture. Food allergy was documented in 10 patients by personal and familial history, presence of specific immunoglobulin E (IgE) and elevated total serum IgE levels. Coeliac disease and human immunodeficiency virus infection were also ruled out. Protein loss through stools was assessed by clearance of α1-antitrypsin (AAT). Serum immunoglobulin levels were determined by nephelometry and functional antibodies were studied by enzyme-linked immunosorbent assay. The cellular immune response was assessed by in vitro lymphocyte proliferation in response to mitogens and cell subsets were analysed by flow cytometry. In five patients of the 10 patients we suspected a protein loss through the mucosa. Four of these five patients showed an increased AAT and the other showed an extensive cutaneous lesion. Immunological studies revealed normal antibody function, in vitro lymphoproliferative responses and cell numbers in four of the 5 patients. One patient showed abnormally low numbers of CD4+ T cells as well as a defective proliferative response to mitogens. After diagnosis of cow milk allergy, milk was replaced with infant milk formula containing hydrolysed proteins. Recovery of immunoglobulin values and clinical resolution were achieved. Hypogammaglobulinaemia during early childhood in some children may be secondary to cow milk allergy, and immunoglobulins and cells may leak through the inflamed mucosa. Resolution of symptoms as well as normalization of immunoglobulin values may be easily achieved by avoidance of the offending allergen.Laboratorio de Investigaciones del Sistema Inmun

Hypogammaglobulinaemia secondary to cow-milk allergy in children under 2 years of age

Symptomatic hypogammaglobulinaemia in children younger than 2 years of age was studied to rule out a primary immunodeficiency. Thirty-four patients were referred to the Immunology Service to study the hypogammaglobulinaemia- associated clinical picture. Food allergy was documented in 10 patients by personal and familial history, presence of specific immunoglobulin E (IgE) and elevated total serum IgE levels. Coeliac disease and human immunodeficiency virus infection were also ruled out. Protein loss through stools was assessed by clearance of α1-antitrypsin (AAT). Serum immunoglobulin levels were determined by nephelometry and functional antibodies were studied by enzyme-linked immunosorbent assay. The cellular immune response was assessed by in vitro lymphocyte proliferation in response to mitogens and cell subsets were analysed by flow cytometry. In five patients of the 10 patients we suspected a protein loss through the mucosa. Four of these five patients showed an increased AAT and the other showed an extensive cutaneous lesion. Immunological studies revealed normal antibody function, in vitro lymphoproliferative responses and cell numbers in four of the 5 patients. One patient showed abnormally low numbers of CD4+ T cells as well as a defective proliferative response to mitogens. After diagnosis of cow milk allergy, milk was replaced with infant milk formula containing hydrolysed proteins. Recovery of immunoglobulin values and clinical resolution were achieved. Hypogammaglobulinaemia during early childhood in some children may be secondary to cow milk allergy, and immunoglobulins and cells may leak through the inflamed mucosa. Resolution of symptoms as well as normalization of immunoglobulin values may be easily achieved by avoidance of the offending allergen.Laboratorio de Investigaciones del Sistema Inmun

Controlo químico de infestantes

Uma planta é considerada infestante quando nasce espontaneamente num local e momento indesejados, podendo interferir negativamente com a cultura instalada. As infestantes competem com as culturas para o espaço, a luz, água e nutrientes, podendo atrasar e prejudicar as operações de colheita, depreciar o produto final e assegurarem a reinfestação nas culturas seguintes. Dado o modo de propagação diferenciado das diversas espécies de infestantes, com as anuais a propagarem-se por semente e as perenes ou vivazes a assegurarem a sua propagação através de órgãos vegetativos (rizomas, bolbos, tubérculos, etc.), assim, também o seu controlo quer químico, quer mecânico terá que ser diferenciado, ou seja, para controlar infestantes anuais será suficiente destruir a sua parte aérea, enquanto para controlar infestantes perenes teremos que destruir os seus órgãos reprodutivos. O controlo de infestantes poderá ser químico, através da utilização de herbicidas, ou mecânico pela utilização de alfaias agrícolas, tais como a charrua de aivecas, a charrua de discos, a grade de discos, o escarificador e a fresa. Quando a técnica utilizada na instalação das culturas é a sementeira directa, o controlo das infestantes terá que ser obrigatoriamente químico, enquanto se o recurso à mobilização do solo for a técnica mais utilizada (sistema de mobilização tradicional ou sistema de mobilização reduzida), o controlo das infestantes tanto poderá ser químico como mecânico. Neste trabalho iremos abordar apenas, o controlo químico de infestantes

Pediatric inborn errors of immunity causing hemophagocytic lymphohistiocytosis: Case report and review of the literature

Inborn errors of immunity are a group of genetic disorders caused by mutations that affect the development and/or function of several compartments of the immune system, predisposing patients to infections, autoimmunity, allergy and malignancies. In this regard, mutations that affect proteins involved in trafficking, priming, docking, or membrane fusion will impair the exocytosis of lytic granules of effector NK and cytotoxic T lymphocytes. This may predispose patients to hemophagocytic lymphohistiocytosis, a life-threatening immune disorder characterized by systemic lymphocyte and macrophage activation, and increased levels of cytokines, which lead to an uncontrolled hyperinflammation state and progressive multiorgan damage. In this review, we will describe a clinical case and recent advances in inborn errors of immunity predisposing to hemophagocytic lymphohistiocytosis. Summary sentence: Review of recent advances in inborn errors of immunity predisposing to hemophagocytic lymphohistiocytosis.Fil: Caldirola, Maria Soledad. Gobierno de la Ciudad de Buenos Aires. Instituto Multidisciplinario de Investigaciones en Patologías Pediátricas. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto Multidisciplinario de Investigaciones en Patologías Pediátricas; ArgentinaFil: Gómez Raccio, Andrea Cecilia. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Area de Inmunología. Grupo de Inmunología; ArgentinaFil: Di Giovanni, Daniela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Area de Inmunología. Grupo de Inmunología; ArgentinaFil: Gaillard, María Isabel. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutiérrez". Area de Inmunología. Grupo de Inmunología; ArgentinaFil: Preciado, María Victoria. Gobierno de la Ciudad de Buenos Aires. Instituto Multidisciplinario de Investigaciones en Patologías Pediátricas. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Houssay. Instituto Multidisciplinario de Investigaciones en Patologías Pediátricas; Argentin

Comparative Study of Subcutaneous Versus Intravenous IgG Replacement Therapy in Pediatric Patients with Primary Immunodeficiency Diseases: A Multicenter Study in Argentina

Purpose Several studies have shown that subcutaneous immunoglobulin (SCIG) infusions demonstrate similar efficacy to intravenous Ig (IVIG) in preventing infections in patients with primary immunodeficiency diseases (PID), and are safe and well tolerated in this population. This open, prospective/retrospective, multicenter study was designed to compare the effectiveness, safety and tolerability of a 16 % liquid human IgG preparation (Beriglobina P), administered SC, with previous IVIG treatment in PID pediatric patients in Argentina. Methods Fifteen subjects were enrolled in the study, and a total of 13 subjects (aged 6–18 years) completed the 36-week SCIG treatment period. All children had previously received IVIG treatment. The dose of SCIG equaled the previous IVIG dose and subjects received an average weekly dose of 139 mg/kg (range 105–181) during the SCIG period. Results Significantly higher serum IgG trough levels were recorded on SCIG treatment at 16, 24, and 36 weeks, when compared with previous IgG trough levels on steady-state IVIG treatment. The annualized infection rate was 1.4 infections/subject/year during the IVIG administration period compared with 0.4 infections/subject/year during the SCIG period. All subjects who completed the study chose to continue administering SCIG at home after the study had ended. Conclusions These data confirm that self-administered SCIG therapy is a well-tolerated and effective alternative to IVIG therapy for children with PID.Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Gómez Raccio, Andrea. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Belardinelli, Gabriela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Regairaz, Lorena. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentina. Provincia de Buenos Aires. Ministerio de Salud. Hospital de Niños "Sor Maria Ludovica" de la Plata; ArgentinaFil: Díaz Ballve, Damacia. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Seminario, Gisela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Moreira, Ileana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; ArgentinaFil: Riganti, Carlos. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Pedro Elizalde" (ex Casa Cuna); ArgentinaFil: Cantisano, Claudio. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Pedro Elizalde" (ex Casa Cuna); ArgentinaFil: Díaz, Héctor. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Pedro Elizalde" (ex Casa Cuna); ArgentinaFil: Di Giovanni, Daniela. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños "Ricardo Gutierrez"; Argentin

Pulmonary infection by Arthrographis kalrae in patient with chronic granulomatous disease

Fil: Campoverde Espinoza, Christian J. Hospital de Niños Ricardo Gutiérrez, Programa de Infectología Pediátrica, Universidad de Buenos Aires, Facultad de Medicina, Ciudad de Buenos Aires; Argentina.Fil: Carballo, Carolina M. Hospital de Niños Ricardo Gutiérrez, Programa de Infectología Pediátrica, Universidad de Buenos Aires, Facultad de Medicina, Ciudad de Buenos Aires; Argentina.Fil: Orlando, Nancy M. Hospital de Niños Ricardo Gutiérrez, Servicio de Microbiología; Argentina.Fil: Hevia, Alejandra I. ANLIS Dr.C.G.Malbrán. Instituto Nacional de Enfermedades Infecciosas. Departamento de Micología.Fil: Gómez Raccio, Andrea C. Hospital de Niños Ricardo Gutiérrez, Servicio de Inmunología; Argentina.Fil: Di Giovanni, Daniela. Hospital de Niños Ricardo Gutiérrez, Servicio de Inmunología; Argentina.Fil: Bezrodnik, Liliana. Hospital de Niños Ricardo Gutiérrez, Servicio de Inmunología; Argentina.Fil: Vázquez Orlando, Miryam S. Hospital de Niños Ricardo Gutiérrez, Servicio de Microbiología; Argentina.Fil: Cazes, Claudia I. Hospital de Niños Ricardo Gutiérrez, Programa de Infectología Pediátrica, Universidad de Buenos Aires, Facultad de Medicina, Ciudad de Buenos Aires; Argentina.Fil: López, Eduardo L. Hospital de Niños Ricardo Gutiérrez, Programa de Infectología Pediátrica, Universidad de Buenos Aires, Facultad de Medicina, Ciudad de Buenos Aires; Argentina.Arthrographis kalrae is a hyaline fungus that grows forming arthroconidia. It is an opportunistic pathogen that causes infections in immunocompromised as in immunocompetent people and has been rarely isolated from human clinical samples

BCG vaccination in patients with severe combined immunodeficiency: Complications, risks, and vaccination policies

BACKGROUND: Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected. OBJECTIVES: We sought to describe the complications and risks associated with BCG vaccination in patients with SCID. METHODS: An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed. RESULTS: Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (≤1 month) showed an increased prevalence of complications (P = .006) and death caused by BCG-associated complications (P < .0001). The odds of experiencing complications among patients with T-cell numbers of 250/μL or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P = .001) than among those with T-cell numbers of greater than 250/μL. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160 patients treated with antimycobacterial therapy for a symptomatic BCG infection (P < .0001). CONCLUSIONS: BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.Fil: Gómez Raccio, Andrea C.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; ArgentinaFil: Orellana, Julio Cesar. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; ArgentinaFil: Liberatore, Diana. Hospital Italiano; ArgentinaFil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Marciano, Beatriz E.. National Institutes of Health; Estados UnidosFil: Huang, Chiung Yu. National Institutes of Health; Estados UnidosFil: Joshi, Gyan. National Institutes of Health; Estados UnidosFil: Rezaei, Nima. Teheran University of Medical Sciences. Children's Medical Center Hospital. Pediatric Center of Excellence; IránFil: Costa Carvalho, Beatriz. Federal University of São Paulo; BrasilFil: Cunha, Luciana. Federal University of Minas Gerais; BrasilFil: Pinto, Jorge A.. Federal University of Minas Gerais; BrasilFil: Espinosa Padilla, Sara E.. Secretaría de Salud. Instituto Nacional de Pediatría; MéxicoFil: Hernandez Nieto, Leticia. Secretaría de Salud. Instituto Nacional de Pediatría; MéxicoFil: Elfeky, Reem A.. Ain Shams University; EgiptoFil: Ariga, Tadashi. Hokkaido University Graduate School of Medicine; JapónFil: Toshio, Heike. Kyoto University Hospital; JapónFil: Dogu, Figen. Ankara University Medical School; TurquíaFil: Cipe, Funda. Ankara University Medical School; TurquíaFil: Formankova, Renata. Charles University; República Checa. University Hospital Motol; República ChecaFil: Nuñez Nuñez, M. Enriqueta. Western National Medical Center; MéxicoFil: Gonçalo Marques, Jose. Santa María Hospital. Lisbon Academic Center; PortugalFil: Pereira, María I.. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; ArgentinaFil: Listello, Viviana. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; ArgentinaFil: Slatter, Mary A.. Great North Children's Hospital; Reino UnidoFil: Nademi, Zohreh. Great North Children's Hospital; Reino UnidoFil: Kowalczyk, Danuta. Children's University Hospital. Department of Clinical Immunology and Transplantology; PoloniaFil: Fleisher, Thomas A.. National Institutes of Health; Estados UnidosFil: Davies, Graham. Great Ormond Street Hospital for Children; Reino UnidoFil: Neven, Bénédicte. Necker Hospital. Immunology-Hematology and Rheumatology Service; FranciaFil: Rosenzweig, Sergio D.. National Institute of Health. National Institute of Allergy and Infectious. Laboratory of Host Defenses. Primary Immunodeficiency Clinic and Infectious Diseases Susceptibility Unit; Estados Unido

First Report of the Hyper-IgM Syndrome Registry of the Latin American Society for Immunodeficiencies: Novel Mutations, Unique Infections, and Outcomes

Hyper-IgM (HIGM) syndrome is a heterogeneous group of disorders characterized by normal or elevated serum IgM levels associated with absent or decreased IgG, IgA and IgE. Here we summarize data from the HIGM syndrome Registry of the Latin American Society for Immunodeficiencies (LASID). of the 58 patients from 51 families reported to the registry with the clinical phenotype of HIGM syndrome, molecular defects were identified in 37 patients thus far. We retrospectively analyzed the clinical, immunological and molecular data from these 37 patients. CD40 ligand (CD40L) deficiency was found in 35 patients from 25 families and activation-induced cytidine deaminase (AID) deficiency in 2 unrelated patients. Five previously unreported mutations were identified in the CD40L gene (CD40LG). Respiratory tract infections, mainly pneumonia, were the most frequent clinical manifestation. Previously undescribed fungal and opportunistic infections were observed in CD40L-deficient patients but not in the two patients with AID deficiency. These include the first cases of pneumonia caused by Mycoplasma pneumoniae, Serratia marcescens or Aspergillus sp. and diarrhea caused by Microsporidium sp. or Isospora belli. Except for four CD40L-deficient patients who died from complications of presumptive central nervous system infections or sepsis, all patients reported in this study are alive. Four CD40L-deficient patients underwent successful bone marrow transplantation. This report characterizes the clinical and genetic spectrum of HIGM syndrome in Latin America and expands the understanding of the genotype and phenotype of this syndrome in tropical areas.Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Jeffrey Modell FoundationLatin American Advisory Board on Primary Immunodeficiencies initiativeUniv São Paulo, Inst Biomed Sci, Dept Immunol, BR-05508000 São Paulo, BrazilCtr Invest & Estudios, Dept Biomed Mol, Mexico City, DF, MexicoDr Ricardo Gutierrez Childrens Hosp, Buenos Aires, DF, ArgentinaHosp Nacl Ninos Dr Carlos Saenz Herrera, San Jose, Costa RicaPediat Allergy & Immunol Clin, Caxias Do Sul, RS, BrazilAlbert Sabin Hosp, Fortaleza, Ceara, BrazilHosp Base Dist Fed, Brasilia, DF, BrazilIntegrated Ctr Pediat Specialties, Curitiba, PR, BrazilHosp Ninos VJ Vilela, Rosario, ArgentinaHosp Ninos Luis Calvo Mackenna, Santiago, ChileUniv Fed Parana, Dept Pediat, BR-80060000 Curitiba, Parana, BrazilUniv Estadual Campinas, Sch Med, Dept Pediat, Campinas, SP, BrazilConceicao Childrens Hosp, Dept Pediat, Div Allergy & Immunol, Porto Alegre, RS, BrazilChildrens Hosp Lucidio Portela, Teresina, PI, BrazilPontificia Univ Catolica Chile, Div Pediat, Santiago, ChileUniv Estadual Campinas, Sch Med, Dept Med, Campinas, SP, BrazilUniv São Paulo, Ribeirao Preto Med Sch, BR-14049 Ribeirao Preto, SP, BrazilHosp Nacl Edgardo Rebagliati Martins Alergia & In, Lima, PeruUniv Fed Rio de Janeiro, Sch Med, Dept Pediat, Rio de Janeiro, BrazilInst Nacl Pediat, Unidad Invest Inmunodeficiencias, Mexico City, DF, MexicoIMSS, Unidad Med Alta Especialidad 25, Monterrey, Nuevo Leon, MexicoClin Montefiori, Unidad Inmunol, Lima, PeruUNAL, Univ Hosp, Monterrey, Nuevo Leon, MexicoFac Med ABC, Santo Andre, SP, BrazilChildrens Hosp, Dept Pediat, New Orleans, LA USAHop Necker Enfants Malad, INSERM, Unite U768, Paris, FranceUniv Washington, Sch Med, Dept Pediat, Seattle, WA 98195 USASeattle Childrens Res Inst, Seattle, WA USAUniversidade Federal de São Paulo, Dept Pediat, Div Allergy Immunol & Rheumatol, São Paulo, BrazilUniversidade Federal de São Paulo, Dept Pediat, Div Allergy Immunol & Rheumatol, São Paulo, BrazilFAPESP: 2012/50515-4FAPESP: 2006/57643-7FAPESP: 2012/51745-3Web of Scienc