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We report on a Mexican mestizo with a multisystemic syndrome including neurological involvement and a type I serum transferrin isoelectric focusing (Tf IEF) pattern. Diagnosis of PMM2-CDG was obtained by clinical exome sequencing (CES) that revealed compound heterozygous variants in PMM2, the encoding gene for the phosphomannomutase 2 (PMM2). This enzyme catalyzes the conversion of mannose-6-P to mannose-1-P required for the synthesis of GDP-Man and Dol-P-Man, donor substrates for glycosylation reactions. The identified variants were c.422G>A (R141H) and c.178G>T, the former being the most frequent PMM2 pathogenic mutation and the latter a previously uncharacterized variant restricted to the Latino population with conflicting interpretations of pathogenicity and that we here report causes leaky non-functional alternative splicing (p.V60Cfs*3).
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INTRODUCTION: The treatment of acute lymphoblastic leukemia (ALL) includes the use of asparaginase (ASP), a drug associated with hypersensitivity reactions (HSR) that requires discontinuing its use. OBJECTIVE: To determine the incidence of HSR associated with ASP that require discontinuation of its use and des cribe them, and to verify if there is a relationship between HSR incidence and protocols or survival. PATIENTS AND METHOD: Retrospective study. Clinical records of all patients (1-15 years) diagnosed with ALL between January 2010 and December 2015 at the Hospital Luis Calvo Mackenna were reviewed. The incidence of HSR to ASP was determined and classified according to the CTCAE v5.0 severity score. We analyzed the relative risk of HSR using Fisher's test and the survival with the Kaplan-Meier estimator. RESULTS: 110 patients were collected. During the first treatment (ALL-IC- BFM), the incidence of HSR to L-ASP was 55%, therefore it was changed to PEG-ASP as second-line treatment, and 44% of them had HSR, and ASP should discontinued in 25% of patients. Of all the HSR to ASP, 77% were anaphylactic (CTCAE 3-5). Patients treated with augmented IB protocol were at higher risk of not completing ASP treatment due to HSR, RR 3.81 (95% CI, 1.98-7.31, p = 0.0001). Patients without HSR in ALL-IC-BFM were at lower risk of relapse, HR 0.29 (95% CI, 0.14-0.62, p = 0.0013). Considering all treatments (ALL-IC-BFM and relapse), patients who completed the ASP treatment had higher overall survival, HR 0.20 (95% CI, 0.07-0.57, p = 0.0026). CONCLUSIONS: HSR to ASP that require discontinuation of treatment are frequent in children with ALL, most of them were severe anaphylactic reactions. This study suggests a better prognosis in patients without HSR to ASP.
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Antineoplásicos/efeitos adversos , Asparaginase/efeitos adversos , Hipersensibilidade a Drogas/epidemiologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/tratamento farmacológico , Adolescente , Anafilaxia/induzido quimicamente , Antineoplásicos/uso terapêutico , Asparaginase/uso terapêutico , Criança , Pré-Escolar , Substituição de Medicamentos , Feminino , Humanos , Incidência , Lactente , Estimativa de Kaplan-Meier , Masculino , Polietilenoglicóis/uso terapêutico , Prognóstico , Estudos RetrospectivosRESUMO
PURPOSE: To determine the impact of an educational program provided by a nurse to parents of children with cancer to improve the level of knowledge of the disease and to decrease the levels of anxiety. PATIENTS AND METHODS: A prospective randomized study was conducted on parents of children recently diagnosed with cancer and treated in the Hospital Luis Calvo Mackenna. After informed consent, parents were randomized in two groups: one receiving the educational program and another without intervention. Both groups completed a questionnaire on social risk, and three tests to assess the levels of knowledge and anxiety. RESULTS: A total of 96 parents were enrolled (July 2010-November 2011). When comparing the number of correct responses on day 10, and day 90 after the intervention, a significant increase was observed in the level of parental knowledge in the group that received the educational program (P<.0001). No significant differences were observed in the levels of anxiety (P=.06) between both groups. CONCLUSIONS: An educational program provided by nurses to parents of children recently diagnosed with cancer, increased the knowledge of their children's disease. However there was no effect on the levels of anxiety. A feasible educational intervention is proposed that could be implemented at other cancer centers for children.
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Ansiedade/epidemiologia , Conhecimentos, Atitudes e Prática em Saúde , Neoplasias/psicologia , Pais/educação , Adulto , Criança , Pré-Escolar , Chile , Feminino , Humanos , Masculino , Cuidados de Enfermagem/métodos , Pais/psicologia , Estudos Prospectivos , Inquéritos e QuestionáriosRESUMO
Propósito: Determinar el impacto de un programa de educación entregado por la enfermera a los padres de niños con cáncer en el aumento del conocimiento de la enfermedad de sus hijos y disminución de la ansiedad. Pacientes y métodos: Estudio prospectivo y aleatorio. Incluyó a los padres de niños con diagnóstico reciente de cáncer, atendidos en el Hospital Dr. Luis Calvo Mackenna. Tras la firma de consentimiento informado se aleatorizó a los padres en 2 grupos, uno que recibió el programa educativo y otro sin intervención. Ambos grupos contestaron una encuesta de riesgo social y 3 evaluaciones de conocimiento y de ansiedad. Resultados: Un total de 96 padres fueron incluidos (julio 2010-noviembre 2011). Al comparar el número de respuestas correctas al día 10 y 90 posterior a la intervención entre ambos grupos, se encontró un aumento significativo en el nivel de conocimiento de los padres del grupo que recibió el programa educativo (p < 0,0001). Al comparar los resultados obtenidos en las evaluaciones de ansiedad entre ambos grupos, no se obtuvo una diferencia significativa (p = 0,06). Conclusiones: El estudio mostró que la realización de un programa educativo, entregado a los padres de niños con diagnóstico reciente de cáncer, aumentó el conocimiento de estos en mayor grado que aquellos que no la recibieron. Sin embargo, no hubo efectos en los grados de ansiedad de dichos padres en el período evaluado. Esta es una intervención factible que se podría implementar en los centros oncológicos infantiles del país.
Purpose: To determine the impact of an educational program provided by a nurse to parents of children with cancer to improve the level of knowledge of the disease and to decrease the levels of anxiety. Patients and methods: A prospective randomized study was conducted on parents of children recently diagnosed with cancer and treated in the Hospital Luis Calvo Mackenna. After informed consent, parents were randomized in two groups: one receiving the educational program and another without intervention. Both groups completed a questionnaire on social risk, and three tests to assess the levels of knowledge and anxiety. Results: A total of 96 parents were enrolled (July 2010-November 2011). When comparing the number of correct responses on day 10, and day 90 after the intervention, a significant increase was observed in the level of parental knowledge in the group that received the educational program (P < .0001). No significant differences were observed in the levels of anxiety (P = .06) between both groups. Conclusions: An educational program provided by nurses to parents of children recently diagnosed with cancer, increased the knowledge of their children's disease. However there was no effect on the levels of anxiety. A feasible educational intervention is proposed that could be implemented at other cancer centers for children.
Assuntos
Humanos , Masculino , Feminino , Pré-Escolar , Criança , Adulto , Ansiedade/epidemiologia , Pais/educação , Conhecimentos, Atitudes e Prática em Saúde , Neoplasias/psicologia , Pais/psicologia , Chile , Estudos Prospectivos , Inquéritos e Questionários , Cuidados de Enfermagem/métodosRESUMO
Los tumores infrecuentes están definidos como cánceres pediátricos con una incidencia anual <2 casos por millón. Aunque son percibidos como raros, constituyen el 15% de todos los cánceres en menores de 20 años y 30% de todos los tumores de pacientes entre 15 y 19 años. Se han desarrollado proyectos cooperativos nacionales e internacionales pediátricos con el fin de mejorar el manejo clínico y la investigación básica en estos tumores. Revisamos los procesos desarrollados y las dificultades que se han enfrentado, como bajas tasas de registro y participación en bancos de tumores y estudios clínicos. Esta experiencia inicial ha permitido desarrollar estrategias alternativas que permitirían implementar una iniciativa similar para América Latina. La experiencia demuestra la factibilidad de cooperación multidisciplinaria a nivel nacional y sugiere que se pueden realizar estudios internacionales, que aumenten nuestro entendimiento de la biología de estos tumores, mejorando los resultados de tratamiento de niños y adolescentes con cánceres infrecuentes.
Although perceived as rare, infrequent tumors, defined as childhood solid malignancies with an annual incidence < 2/million and not considered in other clinical trials, account for 15% of all cancers in patients younger than age 20 and for 30% of all tumors in patients ages 15 to 19. National and international cooperative projects on rare paediatric tumours have been developed to improve the clinical management and basic research on these tumors. We reviewed the process developed and the problems it had to face, as low rates of registration, low levels of participation in tumor banking, and clinical trials. This initial experience has allowed to develop alternative strategies that could help to launch a latinamerican initiative. Experience demonstrates the feasibility of a national multidisciplinary cooperation and suggests that international studies can be performed, increasing our knowledge to understand the biology and improving the treatment results of young patients with rare cancers.
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Humanos , Criança , Adolescente , Doenças Raras/epidemiologia , Neoplasias/epidemiologia , Registros , Desenvolvimento de Programas , Cooperação InternacionalRESUMO
BACKGROUND: The significance of polycystic ovarian morphology (PCOM) during adolescence is not clear. The aim of this study was to determine the relationship between PCOM and anti-Müllerian hormone (AMH), inhibin B, testosterone and insulin levels in healthy girls during the second decade of life. We also determined whether AMH could be used as a surrogate marker of PCOM during adolescence. METHODS: Seventy-four non-obese adolescents (age range: 13.5-19.75 years old) with regular menstrual cycles participated in this study. Transabdominal ultrasound and blood samples were obtained during the follicular phase. RESULTS: PCOM was present in 33.8% of the subjects. Girls with PCOM had higher AMH levels than girls without PCOM (72.5 ± 6.1 versus 33.4 ± 2.6 pmol/l; P < 0.0001) and lower FSH levels (5.4 ± 0.3 versus 6.2 ± 0.2 mUI/ml; P < 0.036). Similar levels of inhibin B, androgens and LH were observed in girls with and without PCOM. PCOM prevalence and AMH levels were not associated with age (P = 0.745 and 0.2, respectively) or BMI-SDS (P = 0.951 and 0.096, respectively). AMH levels positively correlated with the of 2-5 mm follicle number. AMH levels ≥ 60.15 pmol/l had a sensitivity and specificity of 64.0 and 89.8%, respectively, to diagnose PCOM (area under the curve = 0.873). CONCLUSIONS: These data confirm that PCOM in healthy non-hyperandrogenic girls with regular menstrual cycles is prevalent and is not associated with hyperandrogenism. The elevated AMH and lower FSH levels observed in healthy girls with regular menses and PCOM suggest that this ovarian pattern is secondary to a larger number of 2-5 mm follicles. An elevated AMH level is suggestive of the presence of PCOM during adolescence.
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Hormônio Antimülleriano/sangue , Regulação da Expressão Gênica , Ciclo Menstrual/fisiologia , Síndrome do Ovário Policístico/patologia , Adolescente , Adulto , Feminino , Humanos , Hiperandrogenismo/diagnóstico , Inibinas/sangue , Insulina/sangue , Ovário/diagnóstico por imagem , Curva ROC , Testosterona/sangue , UltrassonografiaRESUMO
Hypercalcemia is an infrecuent complication in pediatric oncology, with an incidence between 0,5 and 3 percent. It can occur at diagnosis, during the disease course or at relapse, and it is resolved by treating the underlying pathology, requiring in some cases the use of specific therapy such as calcitonin and biphosphonates. This article presents 3 cases of children with cancer and hypercalcemia during their illness, analyzing its clinical presentation, pathophysiology and treatment.
La hipercalcemia es una complicación infrecuente en niños con cáncer, con una incidencia que oscila entre 0,5 y 3 por ciento. Se puede presentar al diagnóstico, durante el tratamiento o en la recaída de una neoplasia, se resuelve al tratar la enfermedad de base, pudiendo además, requerir el uso de terapia específica como calcitonina y bifosfonatos. En el presente artículo se revisan 3 casos clínicos de niños con cáncer que presentaron hipercalcemia en algún momento de su enfermedad, se discute su forma de presentación, fisiopatología y manejo.
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Humanos , Masculino , Feminino , Criança , Hipercalcemia/etiologia , Neoplasias/complicações , Síndromes Paraneoplásicas , Diagnóstico Diferencial , Hipercalcemia/fisiopatologia , Hipercalcemia/terapia , Oncologia , PediatriaRESUMO
Methods: Retrospective analysis of clinical charts of 41 children (59 eyes) diagnosed with retinoblastoma and treated by a multidisciplinary team at Hospital Luis Calvo Mackenna in Santiago-Chile, between 1999 and 2007. The information included gender, laterality, diagnosis age, presenting signs, tumor spread, treatment modality and survival rate. Results: A total of 23 cases (56 percent) were unilateral and 18 cases (44 percent) were bilateral. The mean age at diagnosis was 21.6 months (range 2 - 84) and 27 children (65.9 percent) were male. The most common presenting signs were leucokoria (51.2 percent), strabismus (24.4 percent) and proptosis (4.9 percent). Enucleation was performed in 48 eyes (81.3 percent), being the only required treatment in 17 children (41.5 percent). The remaining 24 patients received systemic and/or local therapy with chemotherapy, focal therapy and external beam radiation. 5 children died during the follow - up study period, due to extraocular extension to the orbit, central nervous system and bone marrow. Conclusion: In spite of high enucleation rate as initial therapy for retinoblastoma, the survival rate with this current treatment protocol is similar to those from developed countries.
Se presenta un estudio retrospectivo de las fichas clínicas de 41 niños (59 ojos) con diagnóstico de retinoblastoma tratados por un equipo multidisciplinario en el Hospital Luis Calvo Mackenna, Santiago, Chile, entre los a±os 1999-2007. Se recolectó información respecto al género, edad al diagnóstico, signos de presentación, lateralidad, diseminación del tumor, tipos de tratamiento y sobrevida. Veintitrés casos (56 por ciento) fueron unilaterales y 18 (44 por ciento) bilaterales. La edad promedio al momento del diagnóstico fue de 21,6 meses (rango 2-84) y 27 niños (65,9 por ciento) fueron hombres. Los signos de presentación más frecuentes fueron leucocoria (51,2 por ciento), estrabismo (24,4 por ciento) y proptosis (4,9 por ciento). Se realizó enucleación en 48 ojos afectados (81,3 por ciento), siendo el único tratamiento necesario en 17 niños (41,5 por ciento). Los 24 pacientes restantes recibieron tratamientos complementarios locales y/o sistémicos en la modalidad de quimioterapia, terapia focal y radioterapia externa. Durante el período de seguimiento del estudio fallecieron 5 niños, todos ellos con extensión extraocular de la enfermedad hacia la órbita, sistema nervioso central o médula ósea. Conclusiones: No obstante el alto porcentaje de pacientes con retinoblastoma que requieren enucleación como terapia inicial, la tasa de sobrevida con el protocolo actual de tratamiento es comparable a la de países desarrollados.
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Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Neoplasias da Retina/epidemiologia , Neoplasias da Retina/terapia , Retinoblastoma/epidemiologia , Retinoblastoma/terapia , Terapia Combinada , Chile/epidemiologia , Enucleação Ocular , Seguimentos , Neoplasias da Retina/classificação , Neoplasias da Retina/mortalidade , Neoplasias da Retina/patologia , Estudos Retrospectivos , Retinoblastoma/classificação , Retinoblastoma/mortalidade , Retinoblastoma/patologia , Taxa de SobrevidaRESUMO
Background: In children with cancer and high risk febrile neutropenia (HRFN), the initial empirical treatment used in our hospital for 5 years includes the association of Cloxacillin + Ceftazidime + Amikacin. There is no chilean literature that reviews the effectiveness of this therapy. Objective: Evalúate the clinical and microbiological effectiveness of this associated therapy in children with HRFN. Method: A prospective-descriptive study evaluating children with HRFN admitted at Hospital Luis Calvo Mackenna between January 2005-August 2006. Results: 100 HRFN episodes were evaluated. In 48 percent of cases, the antimicrobial treatment was considered effective, whereas in 52 percent of episodes the therapy required modifications (15 percent cases within the first 72 hours). The most frequent diagnoses were fever without clinical focus (51 percent) and sepsis (13 percent). 36 percent had microbiological identification and the most frequently isolated bacteria were Escherichia coli (9 percent) and Staphylococcus aureus (9 percent). Conclusions: A favorable answer with the initial empirical therapy was obtained for 48 percent of cases; meanwhile in the remaining episodes, 28 percent required antibiotics modifications without justification. This fact remarks the importance of following the established guidelines for antimicrobial treatment modification in these patients.
Introducción: En los niños con cáncer y netropenia febril (NF) de alto riesgo, se utiliza en nuestro centro hace 5 años, como esquema empírico inicial, la asociación de ceftazidima-amikacina-cloxacilina. No hay literatura nacional que analice la eficacia de este esquema. Objetivo: Evaluar la eficacia clínica y microbiológica de la asociación de ceftazidima, amikacina y cloxacilina en niños con NF de alto riesgo. Método: Protocolo descriptivo, prospectivo. Evaluar niños con NF de alto riesgo hospitalizados en el HLCM entre enero 2005 y agosto 2006. Resultados: 100 episodios de NF de alto riesgo. El tratamiento antimicrobiano fue considerado eficaz en 48 por ciento de los casos, y requirió ajustes en 52 por ciento de los casos. En 13 por ciento se cambia tratamiento sin justificación y en 15 por ciento se realiza antes de 72 horas de iniciado el tratamiento antimicrobiano. Foco mßs frecuente fue: ausencia de foco clínico 51 por ciento, presentando sepsis 13 por ciento de los niños. Hubo identificación microbiológica en 36 por ciento, microorganismos mßs frecuentemente aislados Echerichia coli (9 por ciento) y Staphylococcus aureus (9 por ciento). Conclusiones: Se obtuvo una respuesta favorable de 48 por ciento con el esquema antimicrobiano empírico inicial, de el 52 por ciento restante, en un 28 por ciento se efectúan cambios de antibióticos no justificados, esto recalca la importancia de seguir las pautas ya establecidas para cambio de esquema antimicrobiano en estos pacientes.
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Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Antibacterianos/uso terapêutico , Febre/tratamento farmacológico , Infecções Bacterianas/tratamento farmacológico , Neoplasias/complicações , Neutropenia/tratamento farmacológico , Evolução Clínica , Quimioterapia Combinada , Escherichia coli/isolamento & purificação , Febre/microbiologia , Neutropenia/microbiologia , Estudos Prospectivos , Staphylococcus aureus/isolamento & purificação , Fatores de Tempo , Resultado do TratamentoRESUMO
Resultados de una encuesta con preguntas específicas contestadas por el 97,6 por ciento de los gineco-obstetras del Departamento de Obstetricia y Ginecología de Clínica Alemana de Santiago, para establecer un diagnóstico de situación sobre el uso de terapia de reemplazo hormonal (TRH). Se enfatizan preguntas sobre la aceptabilidad de las futuras usuarias tanto de TRH como de terapias alternativas; sobre la percepción de los eventuales beneficios desde un punto de vista general, o específicamente cardiovascular; así como las preferencias de hormonas a usar y predilecciones por las vías de administración; las asociaciones del uso y cáncer mamario; las variaciones sobre el uso de TRH pos publicación del Women Health Iniciative Study; los factores que inciden en la discontinuación del tratamiento; la importancia de edad para el uso, factores relacionados con los costos de las terapias y la preferencia o no de productos originales de investigación. Conclusión: Es necesaria una mayor capacitación sobre el uso de TRH en la actualidad tanto de los ginecólogos especialistas en climaterio y menopausia, como de los que no lo son. A falta de información concluyente sobre esta temática en el momento actual se sugiere que el manejo de este tipo de pacientes sea efectuado por los equipos médicos especializados.
In order to set a diagnosis about the use of hormonal replacement therapy (HRT) in peri and postmenopausal women by the obstetricians and gynecologists members of our staff, we did a survey that was answered by 97.6 percent of them. Questions about the future patients desire related to use of HRT or use of natural or alternative medicines were included. There were also questions related to the "risk-benefit" relationship on the use of HRT from an overall point of view as well as from a cardiovascular point of view. The preference of medical doctors (MD) on choice of hormone type in HRT use, in respect to the administration, were also asked. Among others, questions were asked in order to know MD opinions on the relationship with breast cancer, the changes on acceptability use after the WHI study, reasons for the treatment discontinuation, the importance of women's age and the cost of pharmaceuticals produces on HRT use. Conclusions: Are focused on a need of a solid educational program on use of HRT addressed to gynecologists, as well as to general practitioners, cardiologists, oncologists and others related. Educational programs are also quite beneficial to future users as well as women that are currently under treatment. In the absence of conclusive information available at the present moment, we advise that patients considering HRT should seek the counsel of medical teams whose specialties include menopause.
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Humanos , Ginecologia , Conhecimentos, Atitudes e Prática em Saúde , Obstetrícia , Prática Profissional , Terapia de Reposição Hormonal , Atitude do Pessoal de Saúde , Climatério , Coleta de DadosRESUMO
El cáncer es una enfermedad rara en pediatría. Para Chile, se estiman 500 casos nuevos por año. A pesar de su baja frecuencia, esta patología tiene gran impacto, ya que es la segunda causa de muerte en el grupo entre los cinco a 15 años, precedida sólo por accidentes. Con los protocolos actuales de tratamiento, aproximadamente el 65 por ciento de los niños en nuestro país se cura de cáncer según última revisión de octubre 2004. Para mejorar estas cifras y tener menos secuelas es fundamental el diagnóstico precoz. Esto es difícil por la baja frecuencia de esta patología, lo que incide en un bajo índice de sospecha por parte del médico, y porque el 85 por ciento de los cánceres infantiles se presentan con signos y síntomas inespecíficos. Ante la sospecha de cáncer en un niño se debe realizar anamnesis y examen físico completo, consignando hechos destacados. No se debe demorar la derivación realizando exámenes sofisticados.
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Humanos , Masculino , Adolescente , Feminino , Pré-Escolar , Criança , Diagnóstico Precoce , Neoplasias/diagnóstico , Neoplasias/prevenção & controle , Sintomas Cancerínicos , Chile/epidemiologia , Diagnóstico Diferencial , Anamnese , Encaminhamento e ConsultaRESUMO
Objetivo: Evaluar el efecto de la inyección de espuma de polidocanol como sustancia esclerosante vascular en la reducción del tamaño de hemangiomas y linfangiomas orbitarios. Material y Métodos: Estudios prospectivo intervencional de 2 casos con diagnóstico de hemangioma capilar, 7 casos de linfangioma de la región orbitaria y 1 malformación vascular superficial preseptal, que recibieron una o más inyecciones de polidocanol intralesional. Se evaluaron el tipo de tumor, disminución de su tamaño, variación de ptosis palpebral, proptosis y presencia de complicaciones con el procedimiento. Resultados: Se realizaron 4,5 inyecciones promedio con una dosificación final de 0,6 cc al 0,5 por ciento de polidocanol. El seguimiento promedio fue 16,2 meses. Todos los pacientes redujeron el tamaño de la lesión significativamente. La principal complicación fue dolor postinyección. Conclusiones: El uso de inyecciones de polidocanol en el tratamiento de hemangiomas capilares y linfangiomas orbitarios sería una alternativa novedosa y efectiva para reducir el tamaño de las lesiones sin mayores complicaciones.
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Humanos , Masculino , Adolescente , Adulto , Feminino , Lactente , Pré-Escolar , Criança , Escleroterapia/métodos , Polietilenoglicóis/uso terapêutico , Hemangioma Capilar , Linfangioma , Neoplasias Orbitárias/terapia , Órbita/lesões , Soluções Esclerosantes/uso terapêutico , Assistência Ambulatorial , Escleroterapia/efeitos adversos , Seguimentos , Polietilenoglicóis/administração & dosagem , Injeções Intralesionais , Estudos Prospectivos , Soluções Esclerosantes/administração & dosagem , Resultado do TratamentoRESUMO
El manejo de los pacientes pediátricos con cáncer y neutropenia febril (NF) requiere de su clasificación en alto o bajo riesgo de adquirir infecciones bacterianas invasoras (IBI), con el fin de implementar estrategias selectivas de tratamiento. Basados en nuestra experiencia y publicaciones internacionales al respecto, proponemos recomendaciones para el diagnóstico y manejo de niños con cáncer y NF, categorizadas según riesgo de IBI. Todos los pacientes pediátricos que presenten episodios de NF deben ser ingresados al hospital por al menos 24 horas. Durante este lapso se efectuará su evaluación clínica y de laboratorio con el objeto de clasificar el riesgo de este episodio y precisar el (los) posible(s) foco(s). Los pacientes de alto riesgo deben continuar internados hasta su recuperación. Los de bajo riesgo pueden ser manejados en forma ambulatoria. La elección de la terapia antimicrobiana inicial y los criterios para su ajuste deberán basarse en el hallazgo o no de focos infecciosos y en los patrones epidemiológicos e institucionales de susceptibilidad. La reevaluación de ambos grupos debe ser periódica (al menos en los días 3, 5 y 7 de evolución), y la respuesta terapéutica será clasificada como favorable o desfavorable según criterios clínicos y parámetros de laboratorio preestablecidos.
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Humanos , Criança , Antibacterianos/uso terapêutico , Antifúngicos/uso terapêutico , Febre/etiologia , Infecções Bacterianas/etiologia , Infecções Bacterianas/tratamento farmacológico , Neoplasias/complicações , Neutropenia/complicações , Neutropenia/etiologia , Neutropenia/terapia , Avaliação de Resultados em Cuidados de Saúde , Resultado do TratamentoRESUMO
Un paciente de 14 años, portador de leucemia linfoblástica aguda desarrolló una sinusitis subaguda con manifestaciones neurológicas. Mediante biopsia de mucosa nasal y resonancia magnética cerebral se diagnosticó una mucormicosis rinocerebral y basado en un teórico mal pronóstico se decidió efectuar tratamiento conservador: cirugía funcional de la sinusitis y terapia prolongada con anfotericina B deoxicolato completando tras 8,8 meses, 7.700 mg del fármaco. La quimioterapia de la leucemia fue reanudada durante el tratamiento antifúngico alcanzando la remisión oncológica. Tras 31 meses de seguimiento, el paciente está asintomático pero recayó de la leucemia.
Assuntos
Humanos , Adolescente , Anfotericina B , Mucormicose , Antifúngicos/uso terapêutico , Infecções Fúngicas do Sistema Nervoso Central/complicações , Infecções Fúngicas do Sistema Nervoso Central/tratamento farmacológico , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Doenças dos Seios Paranasais , SinusiteRESUMO
Background: Invasive fungal infections (IFI) cause prolonged hospitalizations and increase the possibility of death among patients with cancer and febrile neutropenia (FN). Up to 10 percent of febrile neutropenic episodes may be caused by IFI. Aim: To estimate the incidence of IFI among a large group of Chilean children with cancer and FN. Patients and Methods: Clinical and laboratory information was collected from a data base provided by the "Programa Infantil Nacional de Drogas Antineoplásicas" (PINDA) that included 445 FN episodes occurring in five hospitals in Santiago, Chile. This information was used to identify children that presented with signs and symptoms compatible with an IFI. According to predefined criteria based on a literature review, IFI episodes were categorized as "proven", "probable" or "possible". Results: A total of 41/445 episodes (9.2 percent) were compatible with an IFI of which 4 (0.9 percent) were proven, 23 (5.2 percent) probable, and 14 (3.1 percent) possible. Hospitalization was longer (27 vs 8 days, p <.01), new infectious foci appeared with higher frequency (71 vs 38 percent, p <.01), and mortality was higher (10 vs 1.6 percent, p <.001) in children with IFI compatible episodes, when compared to children who did not have an IFI. Conclusions: The estimated incidence of IFI in Chilean children with cancer and FN ranged between 6-9 percent depending on the stringency of criteria selection used for classification. This estimate is similar to that reported by other studies. The low detection yield of clinically compatible IFI underscores the need of improved diagnosis of fungal infections in this population
Assuntos
Humanos , Masculino , Pré-Escolar , Adolescente , Feminino , Micoses , Neoplasias , Fungemia , Febre , Micoses , Neutropenia , Antineoplásicos/efeitos adversosRESUMO
Background: Pediatric patients in treatment for cancer can have fatal bacterial infections. Thus, in the presence of fever or other signs infection, antimicrobials have to be prescribed empirically. Aim: To know the causative agents of bacteremia in children with cancer, their changes with time and between different hospitals and their patterns of susceptibility. Material and methods: We reviewed the blood cultores of children with cancer in five hospitals of Santiago, from 1994 at 1998. Results: During the study period, 707 agents were isolated. The most frequently isolated species or genus were coagulase negative Staphylococcus (43 percent), Staphylococcus aureus (16 percent), Escherichia coli (9 percent), Klebsiella spp. (8 percent), Pseudomonas spp. (5 percent) and Candida spp. (4 percent). Coagulase negative Staphylococcus was 55 percent resistant to meticilin and S. aureus was 44 percent resistant. Enterobacteriae had 15 percent resistance to gentamicin and amikacin, 2 percent to imipenem, 26 percent to ceftriaxone, 21 percent to cefotaxim and 20 percent to ceftazidim. Among non fermenting agents resistance was 6 percent for imipenem, 9 percent for amikacin 10 percent for ciprofloxacin, 19 percent for ceftazidim and 22 percent for cefoperazone. The resistance of Streptococcus spp. (non pneumoniae) to penicillin reached 50 percent and that of Enterococcus spp. was of 33 percent. Conclusions: Treatment for pediatric patients with cancer must be modified and new guidelines including more active medications for patients at risk for bacteremia, should be devised
Assuntos
Humanos , Masculino , Feminino , Bacteriemia , Neoplasias , Bactérias , Antibacterianos/uso terapêuticoRESUMO
Background: Cyclosporin A has an adequate immunosuppressive capacity and can be useful in the treatment of non infectious ocular inflammatory diseases. Aim: To describe the clinical effect of cyclosporin A treatment in low doses, along with corticosteroids, in the treatment of refractory ocular inflammatory diseases. Patients and methods: Twenty patients (13 female), aged 17 to 74 years old with severe and refractory ocular inflammatory diseases were studied. All except one, received variable doses of prednisone (10 to 60 mg/kg/day) and all received cyclosporin in doses that started in 2.5 mg/day and were increased to 5 mg/kg/day, according to clinical response. Patients were followed from 8 to 24 months, with monthly assessments of ocular inflammation (using a four point score), visual acuity and adverse effects of treatment. Results: A two points or more reduction in the ocular inflammation score was observed in 52 percent of patients. Visual acuity improved in 10 subjects, stabilized in 8 and worsened in 2. Prednisone doses were reduced in most patients. Observed adverse effects were hypertension in 2 patients, creatinine elevation in 2, gastrointestinal disturbances in 3 and hypertrichosis in 12. A reduction of cyclosporin dose was required in these cases, but it was discontinued only in one patient with a vascular purpura. Conclusions: Low cyclosporin doses, associated to prednisone, are useful to reduce inflammation and improve visual acuity in patients with non infectious ocular inflammatory diseases, refractory to other treatment methods
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Pessoa de Meia-Idade , Prednisona/farmacologia , Endoftalmite/tratamento farmacológico , Ciclosporina/farmacologia , Prednisona/administração & dosagem , Acuidade Visual/efeitos dos fármacos , Posologia Homeopática , Estudos Prospectivos , Resultado do Tratamento , Ciclosporina/administração & dosagem , Ciclosporina/efeitos adversos , Ciclosporina/toxicidade , Creatinina/sangue , HipertensãoRESUMO
Objetivos: Determinar el tipo de lesiones que ocupan la fosa de la glándula lagrimal en pacientes mayores de 15 años de edad. Material y Método: De un total de 144 pacientes con masas orbitarias se encontraron 42 con masas ubicadas en la región súpero-temporal de la órbita, determinando su naturaleza benigna o no, según el estudio clínico-patológico. Resultados: 22 (52,4 por ciento) lesiones malignas y 20 (47,6 por ciento) lesiones benignas. Entre las malignas, el linfoma no Hodgkin (8 casos) y el carcinoma adenoide quístico (5 casos), fueron los más frecuentes, y entre las benignas, el quiste dermoide (5 casos), el adenoma pleomórfico (4 casos) y la inflamación crónica inespecífica (en 4), fueron las principales. Conclusiones: Si se considera que entre las masas que ocupan espacio en la órbita de nuestros pacientes mayores de 15 años, el 61,8 por ciento son benignas y el 38,2 por ciento son malignas, cuando la masa se ubica en la fosa de la glándula lagrimal la posibilidad de malignidad sube a un 50 por ciento, estamos obligados a estar más alertas frente a este tipo de ubicación orbitaria