ABSTRACT
Opportunistic fungal pneumonia is a cause of concern in immunocompromised patients due to its high morbidity and mortality rates. One such opportunistic agent affecting immunocompromised patients is the microsporidia called Encephalitozoon cuniculi. This study aimed to evaluate pneumonia caused by E. cuniculi in mice treated with the immunosuppressive agent cyclophosphamide (Cy). This study also aimed to describe the immune cells associated with the microsporidial pneumonia. C57BL/6 mice were infected intravenously with E. cuniculi spores and treated with Cy (75 mg/kg/week, intraperitoneally). Thirty days post-infection, the fungal burden (qPCR), histopathological lesions, cytokine production, and the phenotype of the immune cells in the lung parenchyma were evaluated. Histologically, interstitial pneumonia with lymphocytic infiltrate was observed in the infected animals. The infiltrate mainly consisted of CD8+ and CD4+ T lymphocytes, with reduced populations of B lymphocytes and macrophages. The production of tumor necrosis factor-alpha (TNF-α) was significant in the animals of the infected groups. Also, the fungal burden was higher in the Cy-treated animals, which was confirmed by the immunohistochemical observation of spores. These results demonstrated that E. cuniculi infection of C57BL/6 mice caused lymphocytic interstitial pneumonia (characterized by a predominant lymphocytic infiltrate), which was aggravated by Cy-induced immunosuppression. Thus, these results can be used to understand the different pathological, immunological, and therapeutic aspects of lymphocytic interstitial pneumonia.
ABSTRACT
INTRODUCTION: Hereditary angioedema (HAE) with C1 inhibitor (C1-INH) deficiency is a rare autosomal dominant disease. Although the first symptoms can appear in childhood, the diagnosis's delay has a strong impact on the patient's quality of life. We analyzed clinical and laboratory characteristics and the drug therapy of pediatric patients with HAE in Brazil. METHODS: Medical records from 18 reference centers of HAE patients under 18 years of age were evaluated after confirmed diagnosis was performed by quantitative and/or functional C1-INH. RESULTS: A total of 95 participants (51 M:44 F; mean age: 7 years old) out of 17 centers were included; 15 asymptomatic cases were identified through family history and genetic screening. Angioedema attacks affected the extremities (73.5%), gastrointestinal tract (57%), face (50%), lips (42.5%), eyelids (23.7%), genitals (23.7%), upper airways (10%), and tongue (6.3%). Family history was present in 84% of patients, and the mean delay in the diagnosis was 3.9 years. Long-term prophylaxis (51/80) was performed with tranexamic acid (39/80) and androgens (13/80); and short-term prophylaxis (9/80) was performed with tranexamic acid (6/80) and danazol (3/80). On-demand therapy (35/80) was prescribed: icatibant in 7/35, fresh frozen plasma in 16/35, C1-INH plasma-derived in 11/35, and tranexamic acid in 12/35 patients. CONCLUSIONS: This is the first study on HAE pediatric patients in Latin America. Clinical manifestations were similar to adults. Drugs such as androgens and tranexamic acid were indicated off-label, probably due to restricted access to specific drugs. Educational programs should address pediatricians to reduce late diagnosis and tailored child therapy.
Subject(s)
Angioedemas, Hereditary/epidemiology , Adolescent , Anaphylaxis/etiology , Angioedemas, Hereditary/diagnosis , Angioedemas, Hereditary/therapy , Brazil/epidemiology , Child , Child, Preschool , Delayed Diagnosis , Disease Management , Female , Follow-Up Studies , Humans , Male , Public Health Surveillance , Quality of LifeABSTRACT
A síndrome de desregulação imune, poliendocrinopatia e enteropatia ligada ao X (IPEX) é uma síndrome de imunodeficiência primária rara, de herança recessiva, que afeta lactentes do sexo masculino. A doença cursa com enteropatia perdedora de proteínas, dermatite eczematosa e poliendocrinopatias, podendo ser fatal naqueles sem tratamento apropriado. O objetivo deste relato é descrever um caso de IPEX, enfatizando a importância da história familiar para o diagnóstico precoce. O caso descreve um lactente com tipo grave da síndrome, com apresentação clínica precoce e história familiar característica, com episódios de morte prematura em doze homens pertencentes à linhagem materna. O diagnóstico por mapeamento genético demostrando mutação no gene FOXP3 foi obtido após o óbito do paciente, decorrente de choque séptico. O transplante de células-tronco hematopoiéticas é o melhor tratamento disponível, e na sua ausência, a síndrome IPEX pode ser fatal nos primeiros dois anos de vida. Assim, assegurar um diagnóstico precoce é fundamental.
Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare recessive primary immunodeficiency syndrome that affects male infants. The disease course is characterized by protein-losing enteropathy, eczematous dermatitis, and polyendocrinopathies, and may be fatal if not appropriately treated. The aim of this report was to describe a case of IPEX, emphasizing the importance of family history for early diagnosis. The case describes an infant with a severe manifestation of the syndrome, with early clinical presentation and characteristic family history, with episodes of premature death affecting 12 men belonging to the mother's lineage. Diagnosis was established by genetic mapping after the patient's death due to septic shock; a mutation in the FOXP3 gene was found. Hematopoietic stem cell transplantation is the best treatment available; in its absence, the IPEX syndrome can be fatal in the first 2 years of life. Therefore, ensuring early diagnosis is critical.
Subject(s)
Humans , Male , Infant , Polyendocrinopathies, Autoimmune , Genetic Diseases, X-Linked , Early Diagnosis , Primary Immunodeficiency Diseases/mortality , Patients , Protein-Losing Enteropathies , Chromosome Mapping , Mortality, Premature , MutationABSTRACT
OBJETIVO: Avaliar se a presença de sintomas depressivos associa-se a uma evolução hospitalar desfavorável (aumento da permanência e mortalidade hospitalar), independente da capacidade funcional. MÉTODO: Coorte prospectivo em pacientes idosos internados nas enfermarias de Clínica Médica do Hospital Universitário Lauro Wanderley (HULW), avaliados em dois momentos (segundo dia de internação e na alta). Os instrumentos utilizados foram a Escala de Depressão Geriátrica, versão breve (EDG-15) e Índice de Barthel para Incapacidade em Atividades da Vida Diária (IBAIVD). RESULTADOS: A idade dos 100 pacientes variou entre 60 e 96 anos (69,47 ± 7,45). A mortalidade hospitalar relacionou-se com os escores da EDG-15 da internação (p = 0,001). Observou-se correlação entre os escores da EDG-15 e IBAIVD no início (p = 0,008) e final da internação (p = 0,01), verificando-se correlações lineares inversas (p = -0,30), porém de magnitudes fracas (p = -0,30 e p = -0,28, respectivamente). CONCLUSÃO: Sintomatologia depressiva associou-se à maior mortalidade, independente da capacidade funcional. Ressalta-se a importância da avaliação da sintomatologia depressiva nos idosos internados. Novos estudos e análises prospectivas poderão oferecer mais indicadores sobre esse problema de pesquisa.
OBJECTIVE: Evaluate whether the depressive symptoms are associated to an unfavorable hospital course (increased hospital stay and mortality), independent of the functional capacity. METHOD: Cohort-longitudinal and the inpatients in Medical Clinical on University Hospital Lauro Wanderley were submitted to interview in 2nd and in the day of hospital discharge. The GDS-15 (Geriatric Depression Scale) was utilizated to assess the depressive symptoms and the Barthel Indice to assess the disability in Activities of Daily Living. RESULTS: The age of hundred patients were between 60 to 96 years (69.47 ± 7.45). The hospital mortality was associated at the beginning of hospitalization (p = 0.001). Correlation was observed between the scores of the GDS-15 and IBAIVD early (p = 0.008) and end of hospitalization (p = 0.01), verifying inverse linear correlation (p = -0.30), but the magnitudes weak (p = -0.30 and p = -0.28 respectively). CONCLUSION: Depressive symptoms were associated with higher mortality, independent of functional capacity. It emphasizes the importance of assessing depressive symptoms in the elderly in hospitals. New studies and prospective analyzes may provide more indicators for the understanding of this research problem.
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OBJETIVO: O objetivo deste estudo foi avaliar validade e fidedignidade da Escala de Depressão Gerißtrica (EDG) nas versões de 30 e 15 itens na identificação de suspeitas de depressão em idosos nas enfermarias de clínica médica do Hospital Universitßrio Lauro Wanderley, em João Pessoa, Paraíba. MÉTODOS: Realizou-se um estudo observacional e transversal, com aplicação da EDG-30 e da EDG-15 por uma entrevistadora treinada, efetuando-se no mesmo dia exame mental dos pacientes por um psiquiatra de acordo com critérios da CID-10. A fidedignidade das duas versões da EDG foi avaliada por meio da técnica do teste-reteste e da equivalência interobservadores. RESULTADOS: A concordância entre a aplicação da EDG30 e o exame psiquißtrico foi significativa e moderada (kappa = 0,48; p = 0,04), enquanto a EDG-15 não apresentou concordância estatística (p = 0,62). A EDG-30 apresentou sensibilidade de 83 por cento e especificidade de 57 por cento, mas a EDG-15 apresentou sensibilidade de 50 por cento e especificidade de 62 por cento. Os escores da EDG-30 apresentaram correlação estatisticamente significativa entre si no teste-reteste e na equivalência entre observadores, porém a versão de 15 itens não foi fidedigna. CONCLUSÕES: Conclui-se que a EDG-30 é mais sensível e fidedigna que a EDG-15. Assim, a EDG-30 é útil para detectar como negativos os pacientes que realmente não apresentem depressão, porém são necessßrios estudos posteriores para adequação dos itens dessa escala à nossa clientela, a fim de aumentar sua especificidade e valor preditivo positivo.
OBJECTIVE: The aim of this study was to evaluate the validity and reliability of the Geriatric Depression Scale (GDS) in the versions of 30 and 15 items for the identification of suspicion of depression in the elderly in the infirmary of general practice of the Hospital Universitßrio Lauro Wanderley, in João Pessoa, Paraíba. METHODS: An observational and transversal study, with the application of GDS-30 and GDS-15 by a trained interviewer, was held at the same day the mental exam of the patients by a psychiatrist, according to the criteria of ICD-10. The concordance of the two versions of the GDS was evaluated by the test-retest method and inter-observers equivalence. RESULTS: The concordance between the application of the GDS-30 and the psychiatric exam was significant and moderated (kappa = 0.48, p = 0.04), while the GDS-15 didn't present statistics concordance (p = 0.62). The GDS-30 presented sensitivity of 83 percent and specificity of 57 percent, but the GDS-15 presented sensitivity of 50 percent and specificity of 62 percent. Scores of GDS-30 presented significant statistically correlation in the test-retest method and inter-observers equivalence, but the version of 15 items weren't reliable. CONCLUSION: The conclusion was that the GDS-30 is more sensitive and reliable than the GDS-15, however its sensitivity was inferior to the numbers related in other studies, while his specificity was not high. Therefore, the GDS-30 is useful to detect as negatives the patients who really do not have depression, however further studies are necessary to adjust the items of this scale to our clientele, in order to enhance its specificity and positive predictive value.
ABSTRACT
A presença de sintomas depressivos é fator agravante para idosos hospitalizados em enfermarias de clínica médica, embora seja freqüente a näo preocupaçäo de médicos generalistas com tais manifestaçöes nessa clientela. Avaliar a presença de sintomatologia depressiva através do auto-relato de pacientes geriátricos internados em enfermarias de clínica médica, verificando o grau de reconhecimento pelo clínico geral. Estudo observacional e transversal, a partir de entrevistas estruturadas com 100 idosos internados nas enfermarias de clínica médica do Hospital Universitário Lauro Wanderley/UFPB, usando a Escala de Depressäo Geriátrica (EDG), e confrontando as suspeitas de depressäo assim determinadas com os respectivos relatórios clínicos. Os escores variaram entre 0 e 24 pontos (11,2 ñ 1,4), com mediana de 10,5 e maiores pontuaçöes entre as mulheres (P=0,04); 49 por cento preencheram os critérios para suspeita de depressäo leve (EDG>10), enquanto 33 por cento relatavam dois ou mais dos sintomas depressivos maiores. Constatou-se, todavia, que nenhum idoso teve sua sintomatologia valorizada pelos clínicos. Apesar da alta freqüência de suspeitas de depressäo, näo houve atençäo à sintomatologia depressiva nessa amostra clínica. Tais evidências contribuem para confirmaçäo da teoria da morbidade oculta no doente geriátrico em serviços de clínica geral e apontam para deficiências atuais na semiologia médica do idoso.
