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AIMS: Cigarette smoke often induces pulmonary and systemic inflammation. In animal models, mesenchymal stem cells (MSC) tend to ameliorate these effects. We aimed to explore the local and systemic expression of cytokines in guinea pigs chronically exposed to cigarette smoke, and their modifications by MSC. MAIN METHODS: Concentrations of IL-1ß, IL-6, IL-8, IL-12, TNF-α, INF-É£, TSG-6, MMP-9, TIMP-1, and/or TIMP-2 in serum and bronchoalveolar lavage (BALF) from animals exposed to tobacco smoke (20 cigarettes/day, 5 days/week for 10 weeks) were determined, and mRNA expression of some of them was measured in lung tissue. Intratracheal instillation of allogeneic bone marrow MSC (5x106 cells in 1 ml) was done at week 2. KEY FINDINGS: After cigarette smoke, IL-6 and IFN-γ increased in serum and BALF, while IL-1ß and IL-12 decreased in serum, and TSG-6 and TIMP-2 increased in BALF. IL-1ß had a paradoxical increase in BALF. MSC had an almost null effect in unexposed animals. The intratracheal administration of MSC in guinea pigs exposed to cigarette smoke was associated with a statistically significant decrease of IL-12 and TSG-6 in serum, as well as a decrease of IL-1ß and IFN-γ and an increase in TIMP-1 in BALF. Concerning mRNA expression in lung tissue, cigarette smoke did not modify the relative amount of the studied transcripts, but even so, MSC decreased the IL-12 mRNA and increased the TIMP-1 mRNA. SIGNIFICANCE: A single intratracheal instillation of MSC reduces the pulmonary and systemic proinflammatory pattern induced by chronic exposure to cigarette smoke in guinea pigs. TRIAL REGISTRATION: Not applicable.
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Fumar Cigarrillos , Células Madre Mesenquimatosas , Cobayas , Animales , Citocinas/metabolismo , Inhibidor Tisular de Metaloproteinasa-1/metabolismo , Inhibidor Tisular de Metaloproteinasa-2 , Interleucina-6/farmacología , Fumar Cigarrillos/efectos adversos , Pulmón/metabolismo , Interleucina-12/farmacología , ARN Mensajero , Células Madre Mesenquimatosas/metabolismo , Líquido del Lavado BronquioalveolarRESUMEN
INTRODUCTION: Neonates with congenital heart disease can develop neurological problems, which is why it is important to know the time and extent at which these lesions occur in order to elucidate their causes and implications. OBJECTIVE: To describe brain morphological alterations in autopsies of neonates with congenital heart disease. METHODS: The cases of neonates with congenital heart disease and complete autopsy registered in the pathology department from 2009 to 2019 were included. Descriptive statistics were used with the calculation of frequencies and percentages. RESULTS: Of a total of 21 patients, 61.9% were full-term males; median weight and age at admission were 2500 g and five days, respectively; mean hospital stay was seven days. The predominant heart disease was aortic arch pathology. Fifteen patients (71.3%) underwent surgery; 50% died of cardiogenic shock, 100% had hypoxic-ischemic brain lesions, 71% had incipient lesions, and 33.3%, parenchymal hemorrhage. CONCLUSIONS: There are various risk factors for neurological damage in patients with complex congenital heart disease, which is impossible to be entirely controlled. This study allows us to know, for the first time in our milieu, the changes in the central nervous system that could exist in these patients.
INTRODUCCIÓN: Los neonatos con cardiopatía congénita pueden desarrollar problemas neurológicos, por lo que es importante conocer el momento en el que ocurren dichas lesiones y su extensión, para dilucidar sus causas e implicaciones. OBJETIVO: Describir las alteraciones morfológicas cerebrales en autopsias de neonatos con cardiopatía congénita. MÉTODOS: Se incluyeron los casos de neonatos con cardiopatía congénita y autopsia completa registrados en un servicio de patología, de 2009 a 2019. Se utilizó estadística descriptiva con el cálculo de frecuencias y porcentajes. RESULTADOS: De 21 pacientes, 61.9 % fue a término del sexo masculino; las medianas del peso y edad al ingreso fueron 2500 g y cinco días, respectivamente; la media de la estancia hospitalaria fue siete días. La cardiopatía predominante fue la patología de arco aórtico. Quince pacientes (71.3 %) fueron sometidos a cirugía; 50 % falleció por choque cardiogénico, 100 % presentó lesiones hipóxico-isquémicas cerebrales; 71 %, lesiones incipientes; 33.3 %, hemorragia parenquimatosa. CONCLUSIONES: Existen diversos factores de riesgo para daño neurológico en los pacientes con cardiopatía congénita compleja, los cuales es imposible controlar en su totalidad. Este estudio permite conocer, por primera vez en nuestro medio, los cambios en el sistema nervioso central que podrían existir en estos pacientes.
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Cardiopatías Congénitas , Enfermedades del Sistema Nervioso , Autopsia , Encéfalo , Cardiopatías Congénitas/cirugía , Humanos , Recién Nacido , Masculino , Factores de RiesgoRESUMEN
Resumen Introducción: Los neonatos con cardiopatía congénita pueden desarrollar problemas neurológicos, por lo que es importante conocer el momento en el que ocurren dichas lesiones y su extensión, para dilucidar sus causas e implicaciones. Objetivo: Describir las alteraciones morfológicas cerebrales en autopsias de neonatos con cardiopatía congénita. Métodos: Se incluyeron los casos de neonatos con cardiopatía congénita y autopsia completa registrados en un servicio de patología, de 2009 a 2019. Se utilizó estadística descriptiva con el cálculo de frecuencias y porcentajes. Resultados: De 21 pacientes, 61.9 % fue a término del sexo masculino; las medianas del peso y edad al ingreso fueron 2500 g y cinco días, respectivamente; la media de la estancia hospitalaria fue siete días. La cardiopatía predominante fue la patología de arco aórtico. Quince pacientes (71.3 %) fueron sometidos a cirugía; 50 % falleció por choque cardiogénico, 100 % presentó lesiones hipóxico-isquémicas cerebrales; 71 %, lesiones incipientes; 33.3 %, hemorragia parenquimatosa. Conclusiones: Existen diversos factores de riesgo para daño neurológico en los pacientes con cardiopatía congénita compleja, los cuales es imposible controlar en su totalidad. Este estudio permite conocer, por primera vez en nuestro medio, los cambios en el sistema nervioso central que podrían existir en estos pacientes.
Abstract Introduction: Neonates with congenital heart disease can develop neurological problems, which is why it is important to know the time and extent at which these lesions occur in order to elucidate their causes and implications. Objective: To describe brain morphological alterations in autopsies of neonates with congenital heart disease. Methods: The cases of neonates with congenital heart disease and complete autopsy registered in a pathology department from 2009 to 2019 were included. Descriptive statistics were used with the calculation of frequencies and percentages. Results: Of a total of 21 patients, 61.9% were full-term males; median weight and age at admission were 2500 g and five days, respectively; mean hospital stay was seven days. The predominant heart disease was aortic arch pathology. Fifteen patients (71.3%) underwent surgery; 50% died of cardiogenic shock, 100% had hypoxic-ischemic brain lesions, 71% had incipient lesions, and 33.3%, parenchymal hemorrhage. Conclusions: There are various risk factors for neurological damage in patients with complex congenital heart disease, which is impossible to be entirely controlled. This study allows us to know, for the first time in our milieu, the changes in the central nervous system that could exist in these patients.
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Introduction: In the 18th Century, Giovanni Battista Morgagni demonstrated that localized injuries to different organs caused a great variety of clinical symptoms and signs. Since then, medical practice has evolved considerably and maintains the correlation of pathological changes with the signs and symptoms of the disease as part of the academic activity known as "anatomic-clinical session". The evaluation of the aptitude for the anatomic-clinical correlation has not been empirically estimated in pediatric pathology. Objective: Construct and validate an evaluation instrument to quantitatively estimate the aptitude for the anatomical-clinical correlation in pediatric pathology (AA-CCPP). Method: Analytical cross-sectional study. An evaluation instrument was constructed with 90 true, false and I don't know type statements, grouped based on three indicators: I, diagnostic integration; II, identification of pathogenic mechanisms and III, identification of clinical and paraclinical data. The instrument was applied to three groups of residents. The Kruskal-Wallis test was used to compare the results between the groups. Results: The AA-CCPP grade was very low in most residents. When comparing the global scores between the different groups, the pediatric pathology group was the highest. The indicator that obtained the best results was that of Diagnostic Integration. Conclusions: The level of development of the AA-CCPP observed allows us to deduce that the learning strategy known as clinical-pathological conference should be strengthened.
Introducción: En el siglo xviii, Giovanni Battista Morgagni demostró que las lesiones localizadas en diferentes órganos condicionaban gran variedad de síntomas y signos clínicos. Desde entonces, la práctica médica evolucionó considerablemente y conserva la correlación de los cambios anatomopatológicos con los signos y síntomas de la enfermedad como parte de la actividad académica conocida como «sesión anatomoclínica¼. La evaluación de la aptitud para la correlación anatomo-clínica no ha sido estimada empíricamente en patología pediátrica. Objetivo: Construir y validar un instrumento de evaluación para estimar de manera cuatitativa la aptitud para la correlación anatomo-clínica en patología pediátrica (ACA-CPP). Método: Estudio tranversal analítico. Se construyó un instrumento de evaluación con 90 enunciados tipo verdadero, falso y no sé, agrupados en tres indicadores: I) integración diagnóstica; II) identificación de mecanismos patogénicos; y III) identificación de datos clínicos y paraclínicos. Se aplicó el instrumento a tres grupos de residentes. Para comparar los resultados entre los grupos se utilizó la prueba de Kruskal-Wallis. Resultados: El grado de ACA-CPP fue muy bajo en la mayoría de los residentes. Al comparar las puntuaciones globables entre los diferentes grupos, el de patología pediátrica fue el más alto. El indicador que obtuvo mejores resultados fue el de integración diagnóstica. Conclusiones: El nivel de desarrollo de la ACA-CPP observado permite deducir que la estrategia de aprendizaje conocida como sesión anatomo-clínica debe fortalecerse.
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Anatomía/educación , Aptitud , Patología Clínica/educación , Pediatría/educación , Estudios Transversales , Humanos , AprendizajeRESUMEN
Infantile systemic juvenile xanthogranuloma (ISJXG) is an uncommon form of juvenile xanthogranuloma, a non-Langerhans cell proliferation of infancy and early childhood. In a small percentage of patients, the visceral involvement-most commonly to the central nervous system, liver, spleen, or lungs-may be associated with severe morbidity, and eventually fatal outcome. Here we describe the clinical and pathological findings of a 28-day-old girl with ISJXG who died with respiratory distress syndrome. She had few cutaneous lesions but massive liver and spleen infiltration; other affected organs were multiple lymph nodes, thoracic parasympathetic nodule, pleura, pancreas, and kidneys. Additional findings were mild pulmonary hypoplasia and bacteremia. Immunohistochemistry on fixed tissues is the standard for diagnosis. Immunophenotype cells express CD14, CD68, CD163, Factor XIIIa, Stabilin-1, and fascin; S100 was positive in less than 20% of the cases; CD1a and langerin were negative. No consistent cytogenetic or molecular genetic defect has been identified. This case demonstrates that the autopsy is a handy tool, because hepatic infiltration, which was not considered clinically, determined a restrictive respiratory impairment. In our opinion, this was the direct cause of death.
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Infantile systemic juvenile xanthogranuloma (ISJXG) is an uncommon form of juvenile xanthogranuloma, a non-Langerhans cell proliferation of infancy and early childhood. In a small percentage of patients, the visceral involvementmost commonly to the central nervous system, liver, spleen, or lungsmay be associated with severe morbidity, and eventually fatal outcome. Here we describe the clinical and pathological findings of a 28-day-old girl with ISJXG who died with respiratory distress syndrome. She had few cutaneous lesions but massive liver and spleen infiltration; other affected organs were multiple lymph nodes, thoracic parasympathetic nodule, pleura, pancreas, and kidneys. Additional findings were mild pulmonary hypoplasia and bacteremia. Immunohistochemistry on fixed tissues is the standard for diagnosis. Immunophenotype cells express CD14, CD68, CD163, Factor XIIIa, Stabilin-1, and fascin; S100 was positive in less than 20% of the cases; CD1a and langerin were negative. No consistent cytogenetic or molecular genetic defect has been identified. This case demonstrates that the autopsy is a handy tool, because hepatic infiltration, which was not considered clinically, determined a restrictive respiratory impairment. In our opinion, this was the direct cause of death.
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Humanos , Femenino , Recién Nacido , Xantogranuloma Juvenil/complicaciones , Hepatopatías/diagnóstico , Síndrome de Dificultad Respiratoria del Recién Nacido , Autopsia , Xantogranuloma Juvenil/congénito , Xantogranuloma Juvenil/patología , Resultado FatalRESUMEN
Pulmonary malformations are rare disorders, with cystic and pseudocystic pulmonary malformations (CPPM) the most frequent, and constitute the first cause of lobectomy in children <1â¯year of age. Morphological overlap of congenital cystic pulmonary lesions might correspond to a spectrum of lesions in which bronchial atresia is a common etiopathogenetic mechanism. We aimed to report the frequency of CPPM resected in a tertiary-level hospital and to evaluate the degree of agreement between presurgical and anatomopathological diagnoses. We studied 44 surgical pieces with a diagnosis of CPPM received at the Pathology Service from 2009 to 2014, resected from 39 patients, 51.3 % males, with a median age of 16.8â¯months. Up to 69.2% of the patients had adenomatoid malformation of pulmonary airway (AMPA), with type 2 the most frequent (55.5%). Pulmonary sequestration was present in 15.4% of patients; in two cases the diagnosis was an incidental finding during surgery for the repair of a diaphragmatic hernia. Congenital lobar hyperinflation (CLH) occurred in 7.6% cases. Bronchogenic cyst (BC) was present in 7.6% cases. Presurgical and anatomopathological diagnoses in all patients coincided in 71.8% of cases. Kappa coefficient was 0.56 for global concordance in patients with AMPA, and 0.72, 0.64, 0.37 and 0.33 for CLH, BC, and types 1 and 2 AMPA, respectively. This relatively low interobserver agreement could reflect the low reproducibility of diagnoses used in the current nomenclature. Thus, the new nomenclature must be promoted in order to allow for better reproducibility and greater clinico-pathological concordance. The anatomopathological analysis must include the intentional search for bronchial atresia.
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Procedimientos Quirúrgicos Pulmonares/métodos , Anomalías del Sistema Respiratorio/diagnóstico , Anomalías del Sistema Respiratorio/cirugía , Adolescente , Quiste Broncogénico/diagnóstico , Quiste Broncogénico/patología , Quiste Broncogénico/cirugía , Secuestro Broncopulmonar/diagnóstico , Secuestro Broncopulmonar/patología , Secuestro Broncopulmonar/cirugía , Niño , Preescolar , Estudios Transversales , Malformación Adenomatoide Quística Congénita del Pulmón/diagnóstico , Malformación Adenomatoide Quística Congénita del Pulmón/patología , Malformación Adenomatoide Quística Congénita del Pulmón/cirugía , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Variaciones Dependientes del Observador , Enfisema Pulmonar/congénito , Enfisema Pulmonar/diagnóstico , Enfisema Pulmonar/patología , Enfisema Pulmonar/cirugía , Anomalías del Sistema Respiratorio/patología , Estudios Retrospectivos , Centros de Atención TerciariaRESUMEN
Anaplasia may be identified in a subset of tumors with a presumed pilocytic astrocytoma (PA) component or piloid features, which may be associated with aggressive behavior, but the biologic basis of this change remains unclear. Fifty-seven resections from 36 patients (23 M, 13 F, mean age 32 years, range 3-75) were included. A clinical diagnosis of NF1 was present in 8 (22%). Alternative lengthening of telomeres (ALT) was assessed by telomere-specific FISH and/or CISH. A combination of immunohistochemistry, DNA sequencing and FISH were used to study BRAF, ATRX, CDKN2A/p16, mutant IDH1 p.R132H and H3-K27M proteins. ALT was present in 25 (69%) cases and ATRX loss in 20 (57%), mostly in the expected association of ALT+/ATRX- (20/24, 83%) or ALT-/ATRX+ (11/11, 100%). BRAF duplication was present in 8 (of 26) (31%). H3-K27M was present in 5 of 32 (16%) cases, all with concurrent ATRX loss and ALT. ALT was also present in 9 (of 11) cases in the benign PA precursor, 7 of which also had ATRX loss in both the precursor and the anaplastic tumor. In a single pediatric case, ALT and ATRX loss developed in the anaplastic component only, and in another adult case, ALT was present in the PA-A component only, but ATRX was not tested. Features associated with worse prognosis included subtotal resection, adult vs. pediatric, presence of a PA precursor preceding a diagnosis of anaplasia, necrosis, presence of ALT and ATRX expression loss. ALT and ATRX loss, as well as alterations involving the MAPK pathway, are frequent in PA with anaplasia at the time of development of anaplasia or in their precursors. Additionally, a small subset of PA with anaplasia have H3-K27M mutations. These findings further support the concept that PA with anaplasia is a neoplasm with heterogeneous genetic features and alterations typical of both PA and diffuse gliomas.
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Astrocitoma/genética , Astrocitoma/patología , Neoplasias Encefálicas/patología , Adolescente , Adulto , Anciano , Anaplasia/patología , Biomarcadores de Tumor/genética , Encéfalo/patología , Niño , Preescolar , Femenino , Glioma/patología , Histonas/genética , Histonas/metabolismo , Humanos , Inmunohistoquímica , Hibridación Fluorescente in Situ , Masculino , Persona de Mediana Edad , Mutación , Proteínas Nucleares/genética , Telómero/genética , Telómero/fisiología , Homeostasis del Telómero/genética , Proteína Nuclear Ligada al Cromosoma X/genética , Proteína Nuclear Ligada al Cromosoma X/fisiologíaRESUMEN
BACKGROUND: Although pulmonary involvement is common in patients with cancer, its frequency and nature is seldom reported in the medical literature. OBJECTIVE: To determine the frequency and type of lung pathological conditions revealed by autopsy in children with cancer. METHODS: All reports from autopsies performed in children with cancer from 1989 to 2012 in a pediatric hospital were reviewed. RESULTS: In the analyzed period, 118 autopsies (10.2% of all autopsies) corresponded to children who died with cancer; 76 had complete information and were included in the analysis. Children were seen in the Hematology (41 cases) or the Oncology (35 cases) services. Their median age at decease was 7 years (range, 15 days to 16.1 years) and 46.1% were females. Main diagnoses were acute lymphoblastic (31 patients) or myeloblastic (10 patients) leukemias and tumors of the central nervous system (12 patients). A pathological respiratory condition was diagnosed antemortem in 31 (40.8%) patients, and at autopsy in 62 (81.6%) cases. Omitted diagnoses occurred in 58 (76.3%) children, being pneumonia (24 cases) and pulmonary hemorrhage (23 cases) the most frequent omissions. Nine patients had clinically unsuspected tumor infiltration or metastases. CONCLUSIONS: In these children with cancer, more than 80% of autopsies revealed some lung pathology, mainly of infectious or hemorrhagic nature. Thus, pulmonary involvement should be investigated in all children with cancer in a timely and intentional manner.
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Hemorragia/epidemiología , Enfermedades Pulmonares/epidemiología , Neoplasias/complicaciones , Adolescente , Autopsia , Niño , Preescolar , Femenino , Hemorragia/diagnóstico , Humanos , Lactante , Recién Nacido , Enfermedades Pulmonares/diagnóstico , Enfermedades Pulmonares/patología , Masculino , Neoplasias/patologíaRESUMEN
BACKGROUND: The pineal anlage tumor is a very infrequent malign neoplasm. Even though it has been documented in literature, it is not listed yet in the World Health Organization's last nervous system classification (2007). It is a primitive pineal tumor with neuroepithelial and ectomesenchyme differentiation. Due to its low frequency, the understanding of its biological behavior and a suitable treatment are incomplete. In a search performed in PubMed with the term pineal anlage tumor, only seven informed cases were identified between 1989 and 2011. CLINICAL CASE: An 8-month-old infant was brought to medical attention because he had a progressive enlargement of the cephalic perimeter, and convergent strabismus of two months of evolution. A pineal tumor was identified. The histology showed glial tissue, ganglia cells, pigmented neuroepithelium and striate muscle cells. A ventriculoperitoneal derivation was done to diminish hydrocephalic pressure and also to led the complete surgical resection. The patient was treated with two courses of chemotherapy with carboplatine, ifosfamide and mesna. One year after the treatment, the patient is asymptomatic. CONCLUSIONS: This is the first case reported in Spanish language. Given that it is a really infrequent tumor, it could be misdiagnosed as teratome, melanotic or mesoblastic medulloblastoma, or a melanotic neuroectodermal tumor of childhood (melanotic prognoma).
INTRODUCCIÓN: el tumor pineal anlage es una neoplasia maligna muy poco frecuente. Aunque ya ha sido informado en la literatura, aún no se encuentra listado en la última clasificación de los tumores del sistema nervioso central de la Organización Mundial de la Salud publicada en 2007. Es un tumor primario de la pineal con diferenciación neuroepitelial y ectomesenquimal. Debido a su poca frecuencia, el entendimiento de su comportamiento biológico y su tratamiento apropiado son incompletos. En una búsqueda realizada en PubMed como pineal anlage tumor solo se identificaron siete casos informados entre 1989 y 2011. CASO CLÍNICO: niño de ocho meses de edad que fue llevado a consulta por aumento progresivo del perímetro cefálico y estrabismo convergente de dos meses de evolución. Se identificó tumor pineal conformado por glia, células ganglionares, neuroepitelio pigmentado y células musculares estriadas. Se realizó derivación ventrículo-peritoneal para mitigar la hidrocefalia y resección quirúrgica completa. El paciente recibió dos cursos de quimioterapia con carboplatino, ifosfamida y mesna. Al año permanecía asintomático. CONCLUSIONES: el caso constituye el primero que se describe en lengua española. Dada su rareza, esta entidad puede ser mal diagnosticada como teratoma, meduloblastoma con diferenciación mioblástica y melanótica o tumor neuroectodérmico melanótico de la infancia (progonoma melanótico).
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Neoplasias Encefálicas/patología , Glándula Pineal , Pinealoma/patología , Humanos , Lactante , Lenguaje , MasculinoRESUMEN
As a medical discipline, pathological anatomy was born between the 16th and 17th centuries, when the bases for scientific and technological development, as we know them today, were established. Giovanni Battista Morgagni (1682-1771), one of the greatest clinicians of the 18th century, introduced the concept of correlation between clinical manifestations and pathological anatomic structures. Just like that the pathology has contributed to the characterization of many diseases. Correlation of anatomopathological changes with signs and symptoms of disease is still common practice to date, which constitutes the basis for one of the most relevant pedagogical activities in medicine: the clinical pathological conference. The American Society of Investigative Pathology describes pathology as "the medical specialty that provides the scientific foundation of medical practice". Advances in this discipline have been transmitted mainly in periodical publications as early as the 19th century, and many scientific journals dedicated to communication of relevant findings from all over the world have been created since. The uninterrupted publication of a scientific journal for 51 years, the journal Patología. Revista Latinoamericana, dedicated to one of the most important medical disciplines is, undoubtedly, an achievement worthy of celebration, for being the only one in Spanish in Latin America.
La anatomía patológica como disciplina médica tuvo su origen entre los siglos XVI y XVII. En esa época se sentaron las bases de la ciencia y del desarrollo tecnológico como los conocemos actualmente. Giovanni Battista Morgagni (1682-1771), uno de los clínicos más eminentes del siglo XVIII, demostró que las lesiones localizadas en diferentes órganos y tejidos explicaban los síntomas y signos clínicos; es así como la patología ha contribuido a la caracterización de muchas enfermedades. Hasta la fecha se conserva el ejercicio de correlacionar los cambios anatomopatológicos con los signos y síntomas de la enfermedad, lo que constituye el fundamento de una de las actividades educativas más relevantes dentro de la medicina: la sesión clínico-patológica. La American Society of Investigative Pathology describe a la patología como "la especialidad que proporciona el fundamento científico a la práctica médica". Desde el siglo XIX, los avances en esta disciplina se difunden mayoritariamente por escrito y, desde entonces, en el mundo ha nacido un sinnúmero de revistas científicas dedicadas a la comunicación de investigaciones en esta área. La publicación durante 51 años en México de una revista científica de la especialidad, Patología. Revista Latinoamericana, dedicada a una de las disciplinas médicas más importantes es, sin duda, un logro digno de conmemoración, por ser la única de habla hispana en América Latina.
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Patología Clínica , Publicaciones Periódicas como Asunto , Historia del Siglo XX , Historia del Siglo XXI , América Latina , Patología Clínica/historia , Publicaciones Periódicas como Asunto/historiaRESUMEN
Background: the confirmatory diagnosis of Hirschsprung's disease is made by histopathological study. However, this procedure is limited with only hematoxylin and eosin staining, especially in biopsies of premature babies or when non-expert pathologists make the evaluation. The immunohistochemistry from ganglia cell calretinin has been used to reduce the risk of misdiagnosis. Our objective was to show the benefits of this antibody in diagnosis of Hirschsprung's disease in biopsy specimens. Methods: we evaluated patients with histopathological diagnosis of Hirschsprung's disease made by hematoxylin and eosin staining. We determined if there was enough paraffin block for immunohistochemistry with two markers: calretinin and neurofilaments. Three controls of autopsy of children under 3 years of age with other diagnosis were included. Results: of a total of 48 cases with histopathological diagnosis of Hirschsprung's disease only 13 had adequate tissue for immunohistochemistry. The immunohistochemistry confirmed the diagnosis in nine cases. In the other four cases there were initial misdiagnosis due to evidence of calretinin (ganglion cells) and, thus, Hirschsprung's disease was discarded. Conclusions: the use of immunohistochemistry allows confirming the diagnosis of Hirschsprung's disease and reduce the risk of a false-positive result with only hematoxylin and eosin staining.
Introducción: el diagnóstico de certeza de la enfermedad de Hirschsprung es histopatológico, aunque puede ser erróneo si solo se utiliza hematoxilina y eosina, sobre todo en muestras de niños prematuros o evaluadas por patólogos inexpertos. Se ha recomendado la inmunohistoquímica para identificar las células ganglionares, lo que descarta la enfermedad. Nuestro objetivo es mostrar las ventajas de esta técnica. Métodos: se evaluaron los casos de enfermedad de Hirschsprung con diagnóstico histopatológico mediante tinción con hematoxilina y eosina. Se realizó inmunohistoquímica para dos marcadores: la calretinina y los neurofilamentos. Se incluyeron tres muestras de tejido de autopsias de niños menores de tres años con colon sin alteraciones aparentes. Resultados: de 48 casos con diagnóstico histopatológico de enfermedad de Hirschsprung, solo 13 tuvieron bloque de parafina con tejido suficiente. La inmunohistoquímica confirmó el diagnóstico en nueve. En los otros cuatro hubo error diagnóstico inicial, dada la evidencia de calretinina (células ganglionares) y con ello se descartó la enfermedad de Hirschsprung. Conclusiones: el uso de la inmunohistoquímica para identificar calretinina permitió descartar el diagnóstico de enfermedad de Hirschsprung y evitar la emisión de diagnósticos falsos positivos.
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INTRODUCTION: The examination carried out by the COMMAP for the certification process assessed pathologist formed in dissimilar institutions. In 2007 COMMAP's governing body in turn, decided to digitize it. The purpose of this study is to investigate whether the conversion to virtual slides in the microscopy section, compared with the traditional have had an impact on the scores of the candidates. METHOD: The slides were scanned with high resolution. The virtual microscope is a standard computer screen where there is a program (Aperio Scope Image Viewer) that can display the scanned slides. The results of the microscopy section of the past nine years were compared; two groups were formed: 1) those without digitized examination, and 2) with it. The results were compared by Student t-test and Mann-Whitney. RESULTS: Of a 461 results 240 belonged to the first group and 221 to the second one. On a scale of 1-10, the average scores were 6.6 and 6.8, respectively (p > 0.6 and > 0.5).The minimum and maximum scores were also similar in each group. CONCLUSIONS: According to the results, the digitized exam in the COMMAP's certification process shows no difference between the digitized and the conventional versions.
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Microscopía/métodos , Patología Clínica/métodos , Procesamiento de Señales Asistido por Computador , Estudios RetrospectivosRESUMEN
Pulmonary complications in children with leukemia often display nonspecific clinical and radiologic manifestations that lead to a delay in diagnosis. The role of fiberoptic bronchoscopy (FOB) and the proper time for its performance are controversial. The aim of our study was to evaluate the frequency and nature of specific diagnoses revealed by FOB. Children with leukemia submitted to FOB because of suspicion of pulmonary involvement (mainly pneumonia) were retrospectively analyzed. A total of 33 FOB procedures performed in 31 patients (20 males) with an average age of 9.4 years (range, 3.5 to 15 y) were evaluated. Microorganisms isolated from 21 (63.6%) bronchoalveolar lavage samples were mainly fungi including Candida in 13 cases (39.4%) and Aspergillus in 3 cases (9.1%). Isolation rate in 10 procedures performed within the first 3 days was 90%. Tracheobronchitis was present in > 50% of patients, pulmonary hemorrhage was seen in 7 (21.0%) patients, and leukemic infiltration was demonstrated in 2 patients (6.1%), among other conditions visualized by FOB. Complications of FOB were minimal and transient. Our study suggests that FOB is a useful and safe procedure in patients with leukemia and pulmonary infiltrates. The earlier the FOB was performed, the higher the isolation rate of causative agents. In addition, this procedure allowed the identification of noninfectious airway comorbidities. Further studies in regard to this issue are warranted.
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Bronquitis/diagnóstico , Broncoscopía , Leucemia/complicaciones , Infiltración Leucémica/diagnóstico , Neumonía/diagnóstico , Traqueítis/diagnóstico , Enfermedad Aguda , Adolescente , Algoritmos , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Bronquitis/etiología , Broncoscopía/efectos adversos , Candidiasis/diagnóstico , Candidiasis/etiología , Niño , Preescolar , Diagnóstico Precoz , Femenino , Tecnología de Fibra Óptica , Trastornos Hemorrágicos/inducido químicamente , Humanos , Huésped Inmunocomprometido , Laringismo/etiología , Leucemia/tratamiento farmacológico , Masculino , Neutropenia/inducido químicamente , Neutropenia/complicaciones , Neumonía/etiología , Aspergilosis Pulmonar/diagnóstico , Aspergilosis Pulmonar/etiología , Estudios Retrospectivos , Trombocitopenia/inducido químicamente , Trombocitopenia/complicaciones , Traqueítis/etiologíaRESUMEN
Alveolar soft part sarcoma (ASPS) is a rare, malignant tumor of uncertain histogenesis that has no benign counterpart. In some cases, a structural rearrangement of chromosome 17 involving band q25 has been reported. The neoplasm occurs most frequently in female adolescents and young adults, where it arises predominantly in the extremities. In contrast, the most common sites of occurrence in infants and children are the orbit (41%) and the tongue (25%). The primary therapeutic option is a complete surgical excision. Because of the indolent growth and lack of pain associated with the mass, 20% of patients have metastases at the time of initial diagnosis. Median survival time reported for all sites of the body is 79 months. When ASPS presents in the tongue region, however, the patients involved are usually children and have a better prognosis than patients affected in the extremities. The utility of adjuvant chemotherapy or radiation therapy in children is open to question. Because metastases may occur after several decades, children with ASPS should be followed throughout adolescence and well into adulthood. Only 10 cases of ASPS occurring in the tongues of children younger than 5 years of age were indexed by MEDLINE between 1952 and 2006. Here, we describe the 1st case consistent with typical ASPS of the tongue in 15 years at our hospital. The patient is a 2-year-old girl who has been disease-free for 32 months.
Asunto(s)
Sarcoma/patología , Neoplasias de los Tejidos Blandos/patología , Neoplasias de la Lengua/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biomarcadores de Tumor/análisis , Preescolar , Ciclofosfamida/uso terapéutico , Dactinomicina/uso terapéutico , Supervivencia sin Enfermedad , Femenino , Humanos , Sarcoma/química , Sarcoma/terapia , Neoplasias de los Tejidos Blandos/química , Neoplasias de los Tejidos Blandos/tratamiento farmacológico , Neoplasias de la Lengua/química , Neoplasias de la Lengua/tratamiento farmacológico , Vimentina/análisis , Vincristina/uso terapéuticoRESUMEN
Ki-1 anaplastic large cell non-Hodgkin lymphoma is a well recognized clinical entity. The main clinical feature includes lymphadenopathy with mediastinal sparing. In the extranodal disease, which occurs in approximately half of the cases, the skin is the most common site; bone marrow, lung and central nervous system are generally not involved. Anaplastic large cell lymphoma is more common among young people, in whom the prognosis is more favorable. Primary anaplastic large cell lymphoma of the lung is a rare clinical entity. Its clinical expression is similar to a high grade malignant lymphoma, and in most cases the diagnosis is made in advanced stages. We present a pediatric case with ALK-1 positive anaplastic large cell lymphoma of the lung.
Asunto(s)
Neoplasias Pulmonares/química , Linfoma de Células B Grandes Difuso/química , Proteínas de la Membrana/análisis , Receptores de Activinas Tipo II , Niño , Humanos , Neoplasias Pulmonares/diagnóstico , Linfoma de Células B Grandes Difuso/diagnóstico , MasculinoRESUMEN
OBJECTIVE: 1) To develop and validate an instrument to evaluate the clinical pathology correlation aptitude in pneumopathology (CPCAP). 2) Compare two groups of students: those who had already taken the respiratory pathology course and students that hadn't. METHODS: An instrument with real anatomopathology respiratory cases was developed. The measurement instrument was validated through expert rounds. It included 116 items of the "true", "false", or "don't know" type, with an agreement of 80 % or more in the answerers between the judges. The internal consistency was determined with Spearman Brown proficiency, reaching a value of 0.78. The sample studied was all the groups of each hospital of an organic pathology undergraduate universitary course. Two sampled were taken, students who had already been through respiratory pathology (Group A), and students who hadn't (Group B). RESULTS: The sample studied was of 485 applicants, from 64 different universitary groups. These were divided in two groups, with 245 (A) and 240 (B) students each. Its global medians were 51 and 36 for groups A and B respectively (p < 0.001). In the 55 % of the subgroups when compared individually taking into account each one of the hospital where they study the test results were significantly better in group A. DISCUSSION: In a general way, students in group A show a major development into CPCAP than students in B. However, development is not enough to reach the expected by the educative program. The results suggest that the teachers put greater effort in the correlation between anatomopathological changes and the clinical and paraclinical manifestations of the patients.