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1.
Pediatr. aten. prim ; 24(96)oct.- dic. 2022. tab
Artigo em Espanhol | IBECS | ID: ibc-214394

RESUMO

El botulismo del lactante es una enfermedad causada por potentes neurotoxinas proteicas producidas por la bacteria Clostridium botulinum, las cuales interfieren en la liberación presináptica de acetilcolina a nivel de la unión neuromuscular. Es frecuente en lactantes y puede causar dolor abdominal, vómitos, parálisis aguda —incluyendo parálisis respiratoria—, diplopía y visión borrosa. El tratamiento con inmunoglobulina humana específica evita la evolución del cuadro clínico que puede ocasionar el fallecimiento. A continuación, presentamos un caso de botulismo diagnosticado en un centro de salud (AU)


Infant botulism is a disease caused by potent protein neurotoxins produced by Clostridium botulinum, which interfere with the presynaptic release of acetylcholine at the neuromuscular junction, being common in infants and causing abdominal pain, vomiting, acute paralysis including respiratory paralysis, diplopia and blurred vision. The treatment with a specific immunoglobulin prevents the evolution of the clinical picture that can cause death. We present a case of botulism in an infant in a medical center. (AU)


Assuntos
Humanos , Masculino , Lactente , Botulismo/diagnóstico , Botulismo/tratamento farmacológico , Imunização Passiva , Imunoglobulinas Intravenosas
6.
Allergol. immunopatol ; 48(6): 738-744, nov.-dic. 2020. tab
Artigo em Inglês | IBECS | ID: ibc-199265

RESUMO

OBJECTIVES: We aimed to determine adverse reactions and influencing factors, within the scope of the number of patients and total infusions, in patients with primary immunodeficiencies receiving intravenous immunoglobulin (IVIG) replacement. MATERIALS AND METHODS: Children with primary immunodeficiencies receiving IVIG replacement in Izmir Dr Behcet Uz Children's Hospital, between June 2014 and June 2016, were included in our study. RESULTS: The total number of the patients receiving IVIG replacement was 145 (37 female, 108 male). The number of total IVIG infusions was 1214. Adverse reactions were observed in 44.8% of the patients and 14.2% of the infusions. Common variable immunodeficiency was the most common diagnosis of the patients and adverse reactions most commonly developed in this group (24.2%). In all infusions the most frequent adverse reaction was headache (7.8%); fever was the most frequent immediate side effect (3.9%), whereas headache was the most common delayed adverse effect (5.1%). By logistic regression analyses, history of adverse reaction to IVIG in previous infusions, existence of concomitant infectious disease, past or family history of atopic disease, to receive IVIG infusion at the first time, or being under 10 years old were found associated with adverse reactions. There was no correlation between the concentration of IVIG preparations and the rate of side-effect development. CONCLUSIONS: In our study no severe adverse reaction to IVIG was observed, but many mild or moderate side effects occurred. Therefore, IVIG indications must be well identified. Patients, family of the patients and health care workers must be informed for adverse reactions


No disponible


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Imunoglobulinas Intravenosas/efeitos adversos , Síndromes de Imunodeficiência/tratamento farmacológico , Distribuição por Idade e Sexo , Estudos Retrospectivos , Estudos Transversais , Fatores de Risco , Modelos Logísticos , Fatores de Tempo
7.
Reumatol. clín. (Barc.) ; 16(5,pt.1): 359-361, sept.-oct. 2020. ilus, tab
Artigo em Inglês | IBECS | ID: ibc-195894

RESUMO

We report a case of acute left ventricular dysfunction due to myocarditis, in the setting of a scleroderma renal crisis. The case is particularly intriguing for the favorable outcome of both symptoms and heart function following immunosuppressive therapy. We also highlight the changes observed over time with image techniques as well as in electrocardiograms


Presentamos un caso de disfunción ventricular izquierda secundaria a miocarditis en el contexto de una crisis renal esclerodérmica. Su principal atracción reside en la gran mejoría experimentada por el paciente, no solo en lo que a los síntomas se refiere sino también en su función cardiaca, tras el inicio del tratamiento inmunosupresor. Es muy llamativa la evolución radiológica y electrocardiográfica documentada


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Miocardite/complicações , Nefropatias/complicações , Nefropatias/diagnóstico , Disfunção Ventricular Esquerda/etiologia , Disfunção Ventricular Esquerda/diagnóstico , Ciclofosfamida/uso terapêutico , Imunossupressores/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Nefropatias/diagnóstico por imagem , Eletrocardiografia , Disfunção Ventricular Esquerda/diagnóstico por imagem , Terapia de Imunossupressão
9.
J. investig. allergol. clin. immunol ; 30(1): 14-34, 2020. tab, graf
Artigo em Inglês | IBECS | ID: ibc-194103

RESUMO

Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. It is the most frequent symptomatic antibody deficiency, with a wide variety of infectious and noninfectious complications. Numerous studies have demonstrated that immunological and genetic defects are involved in the pathogenesis of CVID. However, in most cases, the genetic background of the disease remains unidentified. This review aims to discuss various aspects of CVID, including epidemiology, pathogenesis, symptoms, diagnosis, classification, and management


La inmunodeficiencia variable común (CVID) es un trastorno heterogéneo caracterizado por una hipogammaglobulinemia y por una mayor susceptibilidad a infecciones bacterianas recurrentes. Se trata de la inmunodeficiencia humoral sintomática más frecuente y cursa con una extensa variedad de complicaciones infecciosas y no infecciosas. En la patogenia de la CVID están involucrados diferentes defectos inmunológicos y genéticos. Sin embargo, en la mayoría de los casos, el fondo genético de la enfermedad permanece sin identificar. Esta revisión tiene como objetivo discutir diferentes aspectos de la CVID, incluyendo epidemiología, patogenia, síntomas, diagnóstico, clasificaciones y tratamiento de la enfermedad


Assuntos
Humanos , Animais , Imunodeficiência de Variável Comum/epidemiologia , Imunoglobulinas Intravenosas/uso terapêutico , Agamaglobulinemia , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/terapia , Interação Gene-Ambiente , Transplante de Células-Tronco Hematopoéticas , Fenótipo
10.
Angiol. (Barcelona) ; 71(4): 160-163, jul.-ago. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-190299

RESUMO

Presentamos el caso de un varón de 76 años, con antecedentes de hipertensión arterial (HTA), enfermedad pulmonar obstructiva crónica (EPOC), dislipemia, insuficiencia cardiaca, taquicardia paroxística supraventricular y septoplastia, con úlcera dolorosa en cara posterior de la pierna izquierda de dos semanas de evolución, sin clínica previa de claudicación ni pulsos distales en extremidades inferiores (EEII). Se descartaron úlceras mediante anamnesis, claudicometría, arteriografía y biopsia. Se diagnosticó de déficit mixto de inmunoglobulinas e infección sistémica por Aspergillus. Se estableció tratamiento con itraconazol e inmunoglobulinas y mejoró el estado general y de la úlcera


We present the case of a 76-year-old man, with a history of arterial hypertension (AHT), chronic obstructive pulmonary disease (COPD), dyslipidemia, heart failure, supraventricular paroxysmal tachycardia, septoplasty, with a painful ulcer on the left leg (two weeks of evolution), no previous clinical manifestation of claudication and no distal pulses in lower extremities. Ulcers were ruled out by anamnesis, claudicometry, arteriography and biopsy. Then, a mixed immunoglobulin deficit was diagnosed with a systemic infection by Aspergillus. Treatment with itraconazole andm immunoglobulins was established, improving the general condition and the ulcer


Assuntos
Humanos , Masculino , Idoso , Itraconazol/uso terapêutico , Antifúngicos/uso terapêutico , Imunoglobulinas Intravenosas , Aspergilose/diagnóstico , Aspergilose/tratamento farmacológico , Úlcera da Perna/tratamento farmacológico , Úlcera da Perna/microbiologia
12.
Actas dermo-sifiliogr. (Ed. impr.) ; 109(4): 323-330, mayo 2018. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-175507

RESUMO

El uso de las inmunoglobulinas intravenosas en la medicina se remonta a hace más de 50 años, tras el uso como terapia sustitutiva en enfermedades inmunodeficientes. Sin embargo, las indicaciones de este tratamiento han evolucionado de tal manera que actualmente está dirigido a enfermedades donde el sistema inmune desempeña un papel relevante. En el campo de la dermatología se ha hecho un hueco interesante en algunas enfermedades, como la dermatomiositis, las enfermedades autoinmunes ampollares o la necrólisis epidérmica tóxica, entre otras. En los últimos años se ha prestado especial atención al uso de las inmunoglobulinas intravenosas, de hecho se han publicado recientemente nuevas guías sobre su uso, y qué consideraciones debemos tener en cuenta durante su uso en dermatología. Nuestra intención con este artículo es reflejar de una manera práctica el uso de las inmunoglobulinas intravenosas en la dermatología


Intravenous immunoglobulin (IVIG) replacement therapy has been used in immune deficiency diseases for more than 50 years. The indications for this treatment have evolved, however, and IVIG therapy is now used in various diseases in which the immune system plays a prominent role. IVIG therapy has carved out a niche in dermatology for the treatment of such conditions as dermatomyositis, autoimmune bullous diseases, and toxic epidermal necrolysis. Special attention has been paid to this therapy in recent years. New guidelines have been published and should be taken into consideration in dermatology. This review provides a practical guide to IVIG use in our specialty


Assuntos
Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Dermatopatias/terapia , Dermatomiosite/terapia , Doenças Autoimunes/terapia , Imunoglobulinas Intravenosas/administração & dosagem , Imunoglobulinas Intravenosas/efeitos adversos , Infusões Subcutâneas , Seleção de Pacientes , Guias de Prática Clínica como Assunto , Pré-Medicação
13.
Pediatr. aten. prim ; 20(77): 15-24, ene.-mar. 2018. tab
Artigo em Espanhol | IBECS | ID: ibc-173577

RESUMO

Introducción: la enfermedad de Kawasaki es una vasculitis aguda, febril y autolimitada, que puede complicarse con alteraciones cardiovasculares. Su diagnóstico se basa en criterios clínicos. A pesar de un tratamiento eficaz, es la principal causa de cardiopatía adquirida en niños de países desarrollados. Objetivos: analizar la prevalencia de alteraciones coronarias en pacientes pediátricos diagnosticados en nuestro medio; evaluar las características demográficas, clínicas y analíticas de la población de estudio y describir los factores de riesgo de alteración coronaria en niños con enfermedad de Kawasaki. Métodos: se revisaron de forma retrospectiva las historias clínicas de los niños diagnosticados de enfermedad de Kawasaki desde enero de 1997 hasta diciembre de 2016 en el Hospital Clínico San Carlos (Madrid, España). Se consideró diagnóstico de enfermedad de Kawasaki la presencia de los criterios clínicos propuestos por la Academia Americana de Pediatría en 2017. Resultados: la enfermedad de Kawasaki se presentó fundamentalmente durante el invierno (n = 13; 56,5%) en mujeres (n = 12; 52,2%) de cinco años o menos (n = 18; 78,3%), que desarrollaron fiebre (n = 23; 100%) junto con cambios en la mucosa orofaríngea (n = 21; 91,3%) y presentaron proteína C reactiva y velocidad de sedimentación globular elevadas (n = 20; 86,95% y n = 13; 86,6%). Fueron tratados con inmunoglobulina intravenosa (n = 22; 95,7%) y ácido acetilsalicílico a dosis antiinflamatoria (n = 22; 95,7%) o antiagregante (n = 20; 87%). Tres pacientes (13%) desarrollaron alteraciones coronarias, que terminaron remitiendo. Conclusiones: en nuestra cohorte, la prevalencia de alteraciones coronarias fue similar a la reportada en nuestro medio y superior a la descrita en estudios internacionales. Los factores de riesgo de alteración coronaria son predominantemente niños con retraso en el diagnóstico y tratamiento, leucocitosis >12 000/ul, proteína C reactiva >3 mg/dl y trombocitosis


Introduction: Kawasaki disease is an acute, febrile and self-limiting vasculitis that may be complicated by cardiovascular changes. Its diagnosis is based on clinical criteria. Although effective treatment is available, it is the leading cause of acquired heart disease in children in developed countries. Objectives: to assess the prevalence of coronary artery abnormalities in paediatric patients diagnosed in our area, and the demographic, clinical and laboratory characteristics of the population under study, describing the risk factors for coronary involvement in children with Kawasaki disease. Methods: we performed a retrospective review of the medical records of children given a diagnosis of Kawasaki disease between January 1997 and December 2016 at the Hospital Clínico San Carlos (Madrid, Spain). We defined Kawasaki disease as the presence of the clinical criteria proposed for its diagnosis by the American Academy of Pediatrics in 2017. Results: cases of Kawasaki disease were most frequent in the winter (n = 13; 56.5%), in girls (n = 12; 52.2%) and in children aged 5 years or less (n = 18; 78.3%). Patients presented with fever (n = 23; 100%) and changes in the oropharyngeal mucosa (n = 21; 91.3%), with elevation of C-reactive protein and the erythrocyte sedimentation rate (n = 20; 86.95% and n = 13; 86.6%). Treatment consisted of intravenous immunoglobulin (n = 22; 95.7%) and acetylsalicylic acid at anti-inflammatory doses (n = 22; 95.7%) or antithrombotic doses (n = 20; 87%). Three patients (13%) developed coronary artery abnormalities that eventually resolved. Conclusions: in our cohort, the prevalence of coronary complications was similar to the prevalence reported for Spain and higher than the prevalence reported in international studies. The main risk factors for coronary artery abnormalities were delays in diagnosis or treatment, a white blood cell count greater than 12 000 cells/µl, a serum level of C-reactive protein greater than 3 mg/dl and thrombocytosis


Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Síndrome de Linfonodos Mucocutâneos/complicações , Fatores de Risco , Doença das Coronárias/complicações , Síndrome de Linfonodos Mucocutâneos/diagnóstico por imagem , Imunoglobulinas Intravenosas/uso terapêutico , Estudos Retrospectivos , Epidemiologia Analítica , Ecocardiografia/métodos , Estudos de Coortes , Aspirina/uso terapêutico
15.
Allergol. immunopatol ; 44(3): 226-231, mayo-jun. 2016. tab
Artigo em Inglês | IBECS | ID: ibc-152078

RESUMO

BACKGROUND: Common variable immune deficiency (CVID) is a heterogeneous syndrome with a wide variety of signs and symptoms. This study describes the phenotyping and survival of the CVID patients in the allergy and clinical immunology department of Rasol-E-Akram Hospital of Iran University of Medical Sciences in Tehran. METHOD: We retrospectively reviewed hospital files of CVID patients in our department until January 2014. All patients were diagnosed with standard diagnostic criteria of CVID, treated and visited monthly, during the follow-up period. We divided the patients into four phenotypes; infection only, cytopenia, polyclonal lymphocytic infiltration and unexplained enteropathy. The immunologic, demographic and clinical findings in different phenotypes were analysed. RESULTS: The study included 47 CVID patients with mean age at onset of symptoms and diagnosis of 11.2 and 20.2 years, respectively. Phenotyping of our patients was: only infection (62%), cytopenia (26%) and PLI (19%) and 94% of cases had only one phenotype. We did not find a significant relation between the clinical phenotypes and immunologic or demographic data. Rate of parental consanguinity in our cases was 47%. Parental consanguinity was related to lower age at onset, lower age at diagnosis and higher baseline IgG levels. Patients with malignancy and autoimmunity had significantly higher age at onset. Our patients were followed-up for 6.9 years and the mortality rate during this time was 6%. CONCLUSIONS: Parental consanguinity and age at onset of CVID symptoms may have important roles in CVID manifestations


No disponible


Assuntos
Humanos , Masculino , Feminino , Fenótipo , Imunodeficiência de Variável Comum/diagnóstico , Imunodeficiência de Variável Comum/imunologia , Imunodeficiência de Variável Comum/terapia , Autoimunidade/imunologia , Autoimunidade/fisiologia , Mortalidade , Morbidade , 50293 , Consanguinidade , Agamaglobulinemia/complicações , Agamaglobulinemia/imunologia , Agamaglobulinemia/patologia , Imunoglobulinas/análise , Imunoglobulinas/uso terapêutico , Imunoglobulinas Intravenosas/análise , Imunoglobulinas Intravenosas/farmacologia , Imunoglobulinas Intravenosas/uso terapêutico , Estudos Retrospectivos , Irã (Geográfico)
19.
Prog. obstet. ginecol. (Ed. impr.) ; 58(7): 327-329, ago.-sept. 2015.
Artigo em Espanhol | IBECS | ID: ibc-140047

RESUMO

Presentamos el caso de una mujer de 28 años, con 2 abortos tardíos previos causados por anticuerpos anti-M. En la actual gestación es tratada desde la semana 23 hasta la semana 34 con inmunoglobulinas intravenosas fetales, con resultado satisfactorio. Aunque no hay estudios randomizados y controlados que indiquen que las inmunoglobulinas fetales son efectivas en el manejo de la isoinmunización, pequeñas series de casos sugieren resultados prometedores (AU)


We present the case of a 28-year-old woman with two prior late miscarriages caused by anti-M antibodies, leading to alloimmunization of her previous pregnancies. During this pregnancy, she was successfully treated with intravenous immunoglobulins administered from the 23th to the 34th week of pregnancy. There are no randomized trials to indicate whether the antenatal use of intravenous immunoglobulin is effective in the management of fetal red blood cell alloimmunization. Several case series suggest a beneficial role in preventing severe fetal anemia (AU)


Assuntos
Adulto , Feminino , Humanos , Gravidez , Eritroblastose Fetal/induzido quimicamente , Eritroblastose Fetal/tratamento farmacológico , Imunoglobulinas Intravenosas/uso terapêutico , Complicações na Gravidez/diagnóstico , Complicações na Gravidez/terapia , Eritroblastose Fetal/diagnóstico , Eritroblastose Fetal/terapia , Fototerapia/métodos , Fototerapia , Isoimunização Rh/diagnóstico , Isoimunização Rh/terapia , Oxigenoterapia/instrumentação , Oxigenoterapia/métodos , Oxigenoterapia , Teste de Coombs/instrumentação
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