Principales modificaciones asociadas a la esplenectomía / Main modifications associated with splenectomy

Introducción. El bazo es un órgano linfoide implicado en el reconocimiento antigénico, la depuración de patógenos y la remoción de eritrocitos envejecidos o con inclusiones citoplasmáticas. La esplenectomía es una técnica utilizada tanto para el diagnóstico (linfomas), el tratamiento (trombocitopenia inmune, anemia hemolítica adquirida) y la curación (microesferocitosis hereditaria) de diversas enfermedades. Métodos. Describir los principales cambios hematológicos y complicaciones asociadas al procedimiento de esplenectomía. Discusión. Los cambios posteriores a la esplenectomía pueden ser inmediatos, como la aparición de cuerpos de Howell-Jolly, la trombocitosis y la presencia de leucocitosis durante las primeras dos semanas. Otras complicaciones tempranas incluyen la presencia de trombosis, en especial en pacientes con factores de riesgo secundarios (edad, sedentarismo, manejo hospitalario, obesidad) o un estado hipercoagulable (diabetes, cáncer, trombofilia primaria), siendo tanto el flujo de la vena porta como el volumen esplénico los principales factores de riesgo para su aparición. Las complicaciones tardías incluyen la alteración en la respuesta inmune, aumentando el riesgo de infecciones por bacterias encapsuladas, en conjunto con una reducción en los niveles de IgM secundario a la ausencia de linfocitos B a nivel de bazo. Debido al riesgo de infecciones, principalmente por Streptococcus pneumoniae, la esplenectomía parcial se ha considerado una opción. Conclusión. Una adecuada valoración de la indicación de esplenectomía y la identificación precoz de complicaciones posoperatorias son fundamentales para reducir la mortalidad asociada a la esplenectomía

Introduction. The spleen is a lymphoid organ involved in antigen recognition, pathogen clearance, and removal of aged erythrocytes or those with cytoplasmic inclusions. Splenectomy is a technique used for diagnosis (lymphomas), treatment (immune thrombocytopenia, acquired hemolytic anemia), and cure (hereditary microspherocytosis) of various diseases. Methods. To describe the main hematological changes and complications associated with the splenectomy procedure. Discussion. Changes after splenectomy can be considered immediate: the appearance of Howell-Jolly bodies, thrombocytosis, and leukocytosis during the first two weeks. Other complications include the presence of thrombosis, especially in patients with risk factors (age, sedentary lifestyle, long hospital stay, obesity) or a hypercoagulable state (diabetes, cancer, primary thrombophilia), with both portal vein flow and splenic volume being the main risk factors for its appearance. Late complications include altered immune response, increased risk of infections by encapsulated bacteria, and a reduction in IgM levels secondary to the absence of B lymphocytes in the spleen; due to the risk of diseases mainly by Streptococcus pneumoniae, partial splenectomy has been considered an option. Conclusion. An adequate assessment of the indication for splenectomy and the early identification of complications are essential to reduce the mortality associated with splenectomy

Síndrome DRESS secundária ao uso de alopurinol: um relato de caso / DRESS syndrome secondary to the use of allopurinol: a case report

A Síndrome de DRESS (do inglês, Drug Rash with Eosinophilia and Systemic Symptoms) é uma patologia rara que consiste em uma severa reação medicamentosa mediada por células T. O presente relato de caso retrata uma paciente do sexo feminino, 59 anos, que apresentou icterícia, febre não termometrada, acolia, colúria, mialgia, placas hipercrômicas e lesões pruriginosas. Referiu uso recente de alopurinol, paracetamol e nimesulida, apresentando melhora importante e espontânea após a suspensão das medicações. A extensão do tempo de exposição ao medicamento agressor ocasiona um maior período de internação e risco de mortalidade. Além disso, os dados restritos sobre a Síndrome de DRESS impõe desafios ao seu diagnóstico. Sendo assim, este estudo busca destacar a importância do diagnóstico clínico precoce, a suspensão do medicamento agressor e a instituição da terapêutica adequada para um prognóstico favorável

The Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a rare pathology that consists of a severe drug reaction mediated by T cells. The present case report depicts a female patient, 59 years old, who presented jaundice, non thermometered fever, acholia, choluria, myalgia, hyperchromic plaques and pruritic lesions. She mentioned recent use of allopurinol, paracetamol and nimesulide, showing significant and spontaneous improvement after discontinuation of medications. The extension of time of exposure to the offending drug causes a longer period of hospitalization and risk of mortality. In addition, the restricted data on DRESS Syndrome poses challenges to its diagnosis. Therefore, this study seeks to highlight the importance of early clinical diagnosis, suspension of the offending drug and the institution of appropriate therapy for a favorable prognosis

Aspergilosis pulmonar asociada a COVID-19: reporte de caso / Pulmonary aspergillosis associated with COVID-19: case report

La aspergilosis pulmonar clásicamente se ha considerado como una enfermedad de paciente inmunocomprometido (neoplasias hematológicas, neutropenia severa, trasplante de órgano sólido, virus de la inmunodeficiencia humana/síndrome de inmunodeficiencia adquirida VIH/SIDA) o que afecta a pacientes inmunocompetentes, pero críticamente enfermos. Sin embargo, durante la pandemia por COVID-19 el comportamiento de las infecciones fúngicas ha cambiado, siendo observada con mayor frecuencia en la actualidad. Se presenta el caso de un paciente masculino de 20 años, procedente de la Isla de San Andrés, al norte de Colombia, sin patología previa conocida, ingresa al servicio de urgencias cursando con cuadro de tres días de fiebre y tos seca. A su ingreso el paciente está consciente, con fluctuación de la atención, taquicardia y febril. Examen pulmonar y abdominal sin alteraciones. Paraclínicos con leucocitosis, neutrofilia, prueba rápida para COVID-19 negativa y NS1 para dengue negativa. La radiografía de tórax en proyección lateral evidencia infiltrados intersticiales y en tomografía, lesiones nodulares de bordes mal definidos algunos espiculados, con infiltrado intersticial vecino (depósitos tumoral o infeccioso). Resultado de RT- PCR SARSCoV-2 positivo: por hallazgos radiológicos se realiza biopsia pulmonar por toracoscopia, cuyo estudio histológico revela nódulos múltiples y estructuras fúngicas en su interior compatible con aspergillus. Se inicia terapia de primera línea con voriconazol y egreso asintomático al completar tratamiento. Se concluye que las infecciones fúngicas representan un reto diagnóstico, su retraso, por falta de sospecha en el paciente inmunocompetente impacta en la morbimortalidad. El aumento de las infecciones fúngicas durante la pandemia, obliga a considerar la aspergilosis como diagnóstico diferencial en paciente con COVID-19, con fiebre persistente y hallazgos radiológicos atípicos.

Pulmonary Aspergillosis has classically been considered a disease of immunocompromised patients (hematological neoplasms, severe neutropenia, solid organ transplantation, human immunodeficiency virus / acquired immunodeficiency syndrome HIV / AIDS) or immunocompetent but critically ill patients. However, during the COVID-19 pandemic the behavior of fungal infections has changed, being observed more frequently today. A case of 20-year-old male patient, from San Andrés islands, north of Colombia; With no known prior pathology, he was admitted to the emergency department with a 3-day fever and dry cough. On admission, a conscious patient with fluctuating attention, tachycardia and fever. Pulmonary and abdominal examination without alterations. Paraclinical patients with leukocytosis, neutrophilia, rapid test for covid19 negative and NS1 for dengue negative. The chest X-ray in lateral projection shows interstitial infiltrates and in tomography as nodular lesions with poorly defined edges, some spiculated, with neighboring interstitial infiltrate (tumor or infectious deposits). A positive SARS COV 2 RT-PCR result, due to radiological findings, a lung biopsy was performed by thoracoscopy, the histological study of which revealed multiple nodules fungal structures inside compatible with aspergillus. First-line therapy with voriconazole was started, asymptomatic discharge upon completion of treatment. As conclusion fungal infections represent a diagnostic challenge, their delay, due to lack of suspicion in the immunocompetent patient, impacts morbidity and mortality. The increase in fungal infections during the COVID-19 pandemic makes it necessary to consider aspergillosis as a differential diagnosis in a patient with COVID-19, with persistent fever and atypical radiological findings.

Predictive factors of prolonged ventilation following cardiac surgery with cardiopulmonary bypass

Abstract Introduction: In this trial, we initially aimed to investigate the major predictive factors for prolonged mechanical ventilation (PMV) following cardiac surgery with cardiopulmonary bypass (CPB) in our center and, secondarily, we tried to find out the effects of the independent factors on mortality. Methods: Between July 2017 and August 2018, 207 patients who underwent cardiac surgery with CPB were retrospectively investigated. The patients were randomly divided into two subgroups according to the duration of ventilator dependence (group 1 <24 hours, n=164, 79%; group 2 >24 hours, n=43, 21%). Results: 207 patients (mean age 59.47±10.56) who underwent cardiac surgery with CPB were enrolled in this study (n=145, 70% of male patients; n=62, 30% of female patients). Amid these patients, 43 (n=43, 20.77%) had prolonged intubation time. After multivariate logistic regression analysis among preoperative factors, female gender (OR=2.321, P=0.028), leukocytosis (OR=1.233, P=0.006), perioperative lactate level (OR=1.224, P=0.027), CPB time (OR=1.012, P=0.012) and postoperative revision for bleeding (OR=23.125, P=0.040) were significantly detected. The effect of predictive factors on mortality after cardiac surgery was determined and found that PMV did not affect hospital mortality (OR=1.979, P=0.420). Conclusion: In our report, we revealed, differently from previous studies, that intraoperative lactate levels which manifest organ perfusion and oxygenation were included and were significantly different in the early extubation group compared to the PMV group. Female gender, preoperative leukocytosis, intraoperative CPB time, lactate levels and postoperative revision for bleeding were the independent predictive factors for PMV. Moreover, PMV did not affect the early-term mortality during hospital stay.

Clinical findings of equine leukoencephalomalacia / Achados clínicos de leucoencefalomalácia equina

Equine leukoencephalomalacia (LEM) is a disease caused by the ingestion of food, especially corn, contaminated by fumonisin, a Fusarium verticillioides (synonymous with F. moniliforme) metabolite. The clinical signs of brain injuries have an acute onset and rapid evolution. This study aimed to describe the clinical findings in 11 animals diagnosed with LEM, including cerebrospinal fluid (CSF) analysis. Of these animals, 91% (10/11) were horses, and only 9% (1/11) were asinine. The clinical localization of the lesions was 64% (7/10) cerebral, manifested mainly by altered mental state and behavioral disturbance, and 36% (4/11) were brainstem lesions, manifested by incoordination, head tilt, nystagmus, facial hypoalgesia, difficulty in apprehension, chewing, and swallowing food. Postmortem findings revealed that 82% (9/11) of the lesions were in the cerebrum and 18% (2/11) in the brainstem. CSF findings, such as xanthochromia (43%, 3/7), hyperproteinorrachia (50%, 3/6), and pleocytosis (43%, 3/7) were observed. The affected animals showed neurological signs that were compatible with cerebral and/or brainstem injuries. The CSF from animals with LEM may present with xanthochromia, hyperproteinorrachia, and pleocytosis, reinforcing the fact that this disease should be included in the differential diagnosis of encephalomyelopathies.(AU)

A leucoencefalomalácia (LEM) é uma enfermidade que acomete equídeos causada pela ingestão de milho e seus derivados e feno contaminados pela micotoxina fumonisina, um metabólito do fungo Fusarium verticillioides (sinônimo para F. moniliforme). Os sinais clínicos apresentam início agudo e evolução rápida e são decorrentes de lesões encefálicas. O objetivo deste estudo é descrever os achados clínicos de 11 equídeos diagnosticados com LEM, incluindo a análise do líquido cefalorraquidiano (LCR). 91% dos animais afetados eram equinos e somente 9% (1/11) era asinino. A localização clínica das lesões era 64% (7/10) cerebrais, manifestadas por alterações no estado mental e comportamento e 36% (4/10) no tronco encefálico, manifestadas por incoordenação, desvio lateral de cabeça, nistagmo, hipoalgesia da face e dificuldade de apreensão, mastigação e deglutição de alimentos. Comparativamente, os achados post mortem revelaram que 82% (9/11) das lesões eram no cérebro e 18% (2/11) no tronco encefálico. Alterações no LCR, tais como xantocromia (43%, 3/7), hiperproteinorraquia (50%, 3/6) e pleocitose (43%, 3/7), foram observadas. Os animais afetados apresentaram sinais clínicos compatíveis com lesões encefálicas e/ou de tronco cerebral. O LCR de animais com LEM pode apresentar xantocromia, hiperproteinorraquia, e pleocitose, reforçando que esta doença deve ser incluída como diagnóstico diferencial de encefalomielites.(AU)

Ultrasound diagnosis of reticular diaphragmatic hernia in bovines / Diagnóstico ultrassonográfico da hérnia reticular diafragmática em bovinos

A reticular diaphragmatic hernia is a congenital or acquired alteration resulting from protrusion of the reticulum into the thoracic cavity. In ruminants, lesions to the diaphragmatic muscle, due to penetration of sharp metallic objects, is the most common cause of this disease. Therefore, given the low number of reports on this disease in the bovine species, the current study aims to describe the clinical, laboratory, and anatomopathological findings, with special emphasis on the ultrasound diagnosis of five cattle with reticular diaphragmatic hernia. The laboratory data were analyzed using mean and standard deviation, and clinical, ultrasound, and pathological findings were evaluated using descriptive statistics. Clinically the animals exhibited varying degrees of dehydration, abdominal distension, tympany, and alterations in ruminal motility, in addition to cardiorespiratory alterations such as murmur, dyspnea, and muffling of lung sounds. The laboratory examination showed neutrophilic leukocytosis and hyperfibrinogenemia. The ultrasonographic images demonstrated reticulum inside the thoracic cavity adjacent to the lung and heart, although no reticular motility was observed. The pathological lesions confirmed the findings of the ultrasound exams. Thus, the current study demonstrated that ultrasonography was efficient in diagnosing reticular diaphragmatic hernia in the bovine species.(AU)

A hérnia reticular diafragmática é uma alteração congênita ou adquirida resultante da protrusão do retículo para o interior da cavidade torácica. Em ruminantes, lesões no músculo diafragmático devido a penetração de objetos metálico pontiagudos constitui a causa mais comum dessa enfermidade. Portanto, diante dos poucos relatos a cerca dessa enfermidade nos animais da espécie bovina, este estudo tem como objetivo descrever os achados clínicos, laboratoriais, anatomopatológicos e dar ênfase especial no diagnóstico ultrassonográfico de cinco bovinos acometidos com hérnia reticular diafragmática. Os dados laboratoriais foram analisados utilizando-se media e desvio padrão e os achados dos exames clínico, ultrassonográfico e anatomopatológicos foram avaliados através de estatística descritiva. Clinicamente os animais exibiam desidratação em variados graus, distensão abdominal, timpania e alterações na motilidade ruminal. Além de alterações cardiorrespiratórias como sopro, dispneia e abafamento dos sons pulmonares. O exame laboratorial revelou leucocitose por neutrofilia e hiperfibrinogenemia. As imagens ultrassonográficas revelaram retículo no interior da cavidade torácica adjacente ao pulmão e coração, porém nenhuma motilidade reticular foi observada. As lesões anatomopatológicas confirmaram os achados dos exames ultrassonográficos. Dessa maneira, este trabalho demonstrou que a ultrassonografia foi eficiente no diagnóstico da hérnia reticular diafragmática nos animais da espécie bovina.(AU)

Descripción de líneas celulares sanguíneas de pacientes con blastos provenientes de cinco ciudades de Colombia. Serie de casos / Description of cellular lines in peripheral blood with blasts in different regions of Colombia, 2017. Case series

RESUMEN Introducción: las leucemias constituyen un grupo complejo de hemopatías que, por su etiología, patogenia y mortalidad, son consideradas un problema de salud pública. Su detección temprana permite la remisión oportuna para la confirmación del diagnóstico y el inicio del tratamiento. Objetivo: describir la celularidad sanguínea de pacientes con presencia de blastos en sangre periférica, provenientes de cinco ciudades de Colombia durante el año 2017. Métodos: estudio tipo descriptivo de serie de casos. A partir de hemogramas y sus respectivos extendidos de sangre periférica, se analizó cualitativa y cuantitativamente las células sanguíneas de los pacientes con blastos que cumplieron con los criterios de inclusión. Resultados: fueron incluidos 39 pacientes con presencia de blastos de novo en sangre periférica procedentes de Cúcuta 48,7 %, Valledupar 33,3 %, Tunja 10,3 %, Socorro 5,1 % y Villavicencio 2,6 %. El 100 % eran afiliados al sistema general de seguridad social en salud en donde predominó el sexo femenino con el 54 %, la edad promedio para las mujeres fue de 32,5 años y para los hombres fue de 44,2 años. El 76 % de los pacientes presentó leucocitosis de moderada a grave y 69,2 % neutropenia. El 95 % presentó anemia, 51 % hipocromía y 38 % microcitos. El 74 % presentó trombocitopenia y 15 % macroplaquetas. Conclusiones: los hallazgos concuerdan con lo reportado por la literatura en relación con el predominio de la enfermedad de acuerdo con la edad y el sexo. La anemia, trombocitopenia y neutropenia, se mantienen como característica constante en los pacientes con leucemia.

SUMMARY Introduction: Leukemia constitutes a complex group of haemopathies, which due to their etiology, pathogenesis and mortality, can be considered a public health problem. Its early detection allows for timely remission for confirmation of the diagnosis and early start of treatment. Objective: To describe the sanguineous cellularity in a population with presence of blasts in peripheral blood coming from different regions of Colombia du-ring the year 2017. Methods: A descriptive study, type case series. Hemograms and peripheral blood smears from patients with blast presence or a presumptive diagnosis of leukemia were analyzed in five cities of Colombia, all of whom met the inclusion criteria. Results: 39 patients with presence of de novo blasts from Cúcuta 48.7%, Valledupar 33.3%, Tunja 10.3%, Socorro 5.1% and Villavicencio 2.6% were found. All the individuals were affiliated to the Colombian health-care system. Fifty four percent of the subjects were female, with an average age of 44.2 years for men and 32.5 for women. Seventy six percent of the patients presented moderate to severe leukocytosis and 69.2% neutropenia. Ninety-five percent had anemia, 51% hypochromia and 38% microcytes, 74% presented thrombocytopenia and 15% macroplates. Conclusions: These findings are a match to those found in the literature, as for the predominance of the disease by age and sex. Anemia, thrombocytopenia and neutropenia are a set of characteristics that remain constant in patients with leukemia.

Aspectos clínicos, laboratoriais e tratamento da leucemia mieloide crônica em cão: relato de caso / Clinical aspects, hematological changes and treatments of chronic myeloid leukemia in dog: case report

O objetivo deste relato é apresentar o caso clínico de uma cadela, sem raça definida, com cinco anos de idade, diagnosticada com leucemia mieloide crônica (LMC). As leucemias são neoplasias malignas que se originam de células precursoras da medula óssea e as consequências podem ser trombocitopenia, anemia, leucocitose persistente e presença de células neoplásicas no sangue. O tratamento de escolha envolve o uso de inibidores de tirosina quinase, porém este não pode ser usado neste caso. Dessa forma a cadela recebeu diferentes protocolos quimioterápicos que incluíram inicialmente hidroxiureia, citarabina, doxorrubicina e prednisona. Devido a remissão parcial dos sinais clínicos e a resposta terapêutica pouco duradoura a essas medicações o protocolo foi alterado para quimioterapia metronômica com clorambucil. O uso desses quimioterápicos não foram eficazes em reduzir a leucocitose e controlar a anemia e trombocitopenia da paciente, devido a ocorrência do surgimento de células imaturas no sangue e resistência aos quimioterápicos. Na ausência da crise e da possibilidade do uso dos inibidores de tirosina quinase, a hidroxiureia permanece sendo o quimioterápico de eleição. O animal apresentou sobrevida de 210 dias, devido a leucocitose e anemia severas pouco responsivas ao protocolo terapêutico utilizado e o surgimento no hemograma de precursores neutrofilicos que ocorreu 46 dias após ao início do tratamento com hidroxiureia.

The aim of this report is to present the clinical case of a five-year-old mixed breed female dog diagnosed with chronic myeloid leukemia (CML). Leukemias are malignant neoplasms that originate from bone marrow precursor cells and the consequences can be thrombocytopenia, anemia, persistent leukocytosis and the presence of neoplastic cells in the blood. The treatment of choice involves the use of tyrosine kinase inhibitors, but it cannot be used in this case. Thus, the dog received different chemotherapy protocols that initially included hydroxyurea, cytarabine, doxorubicin and prednisone. Due to the partial remission of clinical signs and the short-term therapeutic response to these medications, the protocol was changed to metronomic chemotherapy with chlorambucil. The use of these chemotherapeutic agents was not effective in reducing leukocytosis and controlling the patient's anemia and thrombocytopenia, due to the occurrence of immature cells in the blood and resistance to chemotherapeutic agents. In the absence of the crisis and the possibility of using tyrosine kinase inhibitors, hydroxyurea remains the chemotherapy of choice. The animal had a 210-day survival, due to severe leukocytosis and anemia, which were not responsive to the therapeutic protocol used and the appearance in the blood count of neutrophilic precursors that occurred 46 days after the beginning of hydroxyurea treatment.

Doença de Still do adulto: um desafio diagnóstico / Adult-onset Still's disease: a diagnostic challenge

A doença de Still do adulto é uma rara condição inflamatória, cujo diagnóstico é um desafio, por se tratar de diagnóstico de exclusão, após vasta investigação. Manifesta-se com febre alta diária, amigdalite não supurativa, artrite, rash evanescente, leucocitose e hiperferritinemia. O presente caso demonstra a doença de Still do adulto e sua vasta investigação, motivando a realização de revisão bibliográfica sobre inovações na fisiopatologia, no diagnóstico e no tratamento.

Adult onset Still's disease is a rare inflammatory condition, the diagnosis of which is a challenge, because it is a diagnosis of exclusion, and demands extensive investigation. It manifests with high daily fever, nonsuppurative tonsillitis, arthritis, evanescent rash, leukocytosis, and hyperferritinemia. The present case de­monstrates adult-onset Still's disease and its extensive inves­tigation, motivating literature review on innovations of its pathophysiology, diagnosis, and treatment.

Inflammatory signs and epileptic crisis in patients admitted in an emergency unit / Signos inflamatorios y crisis epiléptica en pacientes admitidos en una unidad de emergencia / Sinais inflamatórios e crise epiléptica em pacientes admitidos em unidade de emergência

ABSTRACT Objective: To evaluate inflammatory signs presented in medical records of patients with a main diagnosis of epileptic seizures, admitted in an emergency unit. Method: Cross-sectional and retrospective study. The sample was composed of 191 medical records, from children, adolescents, adults, and elders, with a clinical diagnosis of epileptic seizures, admitted between June 2016 and June 2017 at the emergency unit of a hospital in Porto Alegre/RS. Results: The prevalent inflammatory signs were tachypnea (33.5%) and/or fever (27.2%) associated with leukocytosis (P=0.030). Children/adolescents had seizures less frequently (P=0.010) and these were due to fever (P=0.000). Adults presented seizures more frequently (P=0.006), which were related to medication/intoxication (P=0.000). In elders, seizures occurred due to metabolic or circulatory abnormalities (P=0.000), less often due to fever (P=0.005). Conclusion: Seizures are related to fever and tachypnea, being caused by different etiologies according to age, being more frequent in adults. Fever is related to leukocytosis, regardless of age.

RESUMEN Objetivo: Evaluar signos inflamatorios registrados en prontuarios de pacientes con diagnóstico principal de crisis epilépticas, admitidos en unidad de emergencia. Método: Estudio transversal, retrospectivo. Muestra compuesta por 191 prontuarios de pacientes pediátricos, adolescentes, adultos y ancianos, diagnosticados con crisis epilépticas, admitidos entre junio de 2016 a junio de 2017 en unidad de emergencia de un hospital de Porto Alegre/RS. Resultados: Prevalencia del taquipnea (33,5%) y/o fiebre (27,2%) como signos inflamatorios, fiebre relacionada a leucocitosis (P=0,030). Niños/adolescentes tienen crisis menos frecuentes (P=0,010) de origen febril (P=0,000). Los adultos presentaron mayor número de eventos (P=0,006), provocados por medicamentos/intoxicaciones (P=0,000). En ancianos, crisis ocurrieron debido a disturbios metabólicos/circulatorios (P=0,000),menor ocurrencia de fiebre (P=0,005). Conclusión: Crisis epilépticas están relacionadas a fiebre y taquipnea, presentando diferentes etiologías según grupo de edad, con mayor ocurrencia entre adultos. Fiebre relacionada con el leucocitosis, independientemente de la edad.

RESUMO Objetivo: Avaliar os sinais inflamatórios registrados em prontuários de pacientes com diagnóstico principal de crise epiléptica, admitidos em unidade de emergência. Método: Estudo transversal, retrospectivo. Amostra composta por 191 prontuários de pacientes pediátricos, adolescentes, adultos e idosos, com diagnóstico clínico de crise epiléptica, admitidos entre junho de 2016 a junho de 2017, na unidade de emergência de um hospital de Porto Alegre/RS. Resultados: Prevalência do relato de taquipneia (33,5%) e/ou febre (27,2%) como sinais inflamatórios, estando febre relacionada à leucocitose (P=0,030). Crianças/adolescentes tiverem crises menos frequentes (P=0,010) ede origem febril (P=0,000). Adultos apresentaram maior número de eventos (P=0,006), provocados por medicações/intoxicações (P=0,000). Nos idosos, crises ocorreram por distúrbios metabólicos/circulatórios (P=0,000), com menor ocorrência de febre (P=0,005). Conclusão: Crises epilépticas estão relacionadas à presença de febre e taquipneia, apresentando diferentes etiologias conforme faixa etária, com maior frequência de ocorrência entre adultos. Febre está relacionada à leucocitose, independentemente da idade.

Correlação de alterações hematológicas em doenças parasitárias / Correlation of hematological changes in parasitic diseases

Os enteroparasitos causam aos seus portadores, entre outros agravos, desnutrição seguida por perda de peso, diarreias, vômitos, desidratação, anemia e enterites. Todos esses sintomas podem desencadear distúrbios dos índices hematológicos como contagem de hemácias, hematócrito, hemoglobina, leucócitos totais e eosinófilos. Este estudo teve como objetivo avaliar e analisar as alterações hematológicas em indivíduos com diagnóstico parasitológico positivo para enteroparasitos. Foi realizado um estudo com base nos dados laboratoriais, em que se analisou o hemograma de 412 indivíduos positivos para enteroparasitoses. Os dados demonstraram que existem relações entre as alterações hematológicas, principalmente a anemia, leucocitose e a eosinofilia, com as infecções parasitárias intestinais. Verificou-se também a frequência de poliparasitismo e suas alterações mais frequentes. O estudo apresentou uma possível contribuição para futuros trabalhos que relatem a importância da prevenção e tratamento das doenças parasitárias em humanos.

The enteroparasites cause to their carriers, among other damages, malnutrition followed by weight loss, diarrhea, vomiting, dehydration, anemia and enteritis. All these symptoms can trigger hematological indices disorders such as red blood cell count, hematocrit, hemoglobin, total leukocytes and eosinophils. This study aimed to evaluate and analyze the hematological alterations in individuals with positive parasitological diagnosis for enteroparasites. A study was carried out based on the laboratory data, in which the hemogram of 412 positive individuals for enteroparasitoses was analyzed. The data demonstrated that there are relationships between hematological changes, mainly anemia, leukocytosis and eosinophilia, with intestinal parasitic infections. The frequency of polyparasitism and its most frequent alterations were also verified. The study presented a possible contribution to future studies that report the importance of the prevention and treatment of parasitic diseases in humans

Precordial pain, leukocytosis and bicytopenia in a teenager with systemic juvenile idiopathic arthritis under immunosuppressive therapy / Dor precordial, leucocitose e bicitopenia em adolescente com artrite idiopática juvenil sistêmica em terapia imunossupressora

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.

Revisiting the complete blood count and clinical findings at diagnosis of childhood acute lymphoblastic leukemia: 10-year experience at a single center

Abstract Background Heterogeneity regarding clinical and laboratory findings at diagnosis of acute lymphoblastic leukemia exists. The frequency of complete blood count abnormalities and its combinations, symptoms and physical findings were investigated in Hispanic children from an open population at the diagnosis of acute lymphoblastic leukemia. Methods The patient charts and electronic records of under 16-year-old children diagnosed with acute lymphoblastic leukemia over 10 years at a regional hematology center of a university hospital were analyzed to retrieve data concerning the complete blood count at first evaluation. Type and distribution of abnormal data, frequency of symptoms and physical findings at presentation were documented. Results The records of 203 children aged 0-15 years diagnosed with acute lymphoblastic leukemia from 2006 to 2016 were revisited. The results of the blood workup showed a median white blood cell count of 7120 × 109/L (range: 450-600,000 × 109/L), and a median hemoglobin concentration of 7.5 g/dL (range: 2.4-15.3 g/dL), whereas the median platelet count was 47,400 × 109/L (range: 4000-544,000 × 109/L). Leukocytosis and leukopenia were present in 36.6% and 36.1% of cases, respectively; anemia was diagnosed in 82.9% children. The order of frequency for major clinical symptoms was fatigue 62%, fever 60%, bone and joint pain 39%, hyporexia 33% and weight loss 21%, while main physical findings were hepatomegaly 78%, splenomegaly 63%, lymphadenopathy 57%, pallor 48%, and purpura 30%. Conclusion Data differing from those classically expected at diagnosis of acute lymphoblastic leukemia in children were documented in a cohort of Hispanic children over one decade with a wide spectrum of complete blood count abnormalities, forms of presentation and frequency of physical findings.

Doença celíaca: revisão bibliográfica e relato de caso / Celiac disease: literature review and case report

RESUMO A doença celíaca (DC) é uma forma crônica de enteropatia de mecanismo imunológico que afeta o intestino delgado de crianças e adultos geneticamente predispostos, precipitada pela ingestão de alimentos contendo glúten. Também é conhecida como espru celíaco, enteropatia sensível ao glúten ou espru não tropical. A doença pode se manifestar na forma Clássica, Não Classica e assintomática. Para o diagnostico é imprescindível a realização de endoscopia digestiva alta com biópsia de intestino delgado, considerado o padrão-ouro. Os marcadores sorológicos são úteis para identificar os indivíduos que deverão ser submetidos à biópsia de intestino delgado e também são úteis para acompanhamento do paciente celíaco. Os principais testes sorológicos para a detecção da intolerância ao glúten são o anticorpo antigliadina, o anticorpo antiendomísio e o anticorpo antitransglutaminase (TTG). O tratamento da DC consiste na introdução de dieta isenta de glúten de forma permanente, devendo-se, portanto, excluir da dieta os seguintes cereais e seus derivados: trigo, centeio, cevada, malte, aveia. Neste trabalho relatamos o caso de um paciente com diagnóstico tardio de DC, que iniciou o quadro com enteropatia perdedora de proteína, com quadro infeccioso vigente, PCR aumentado, leucocitose, plaquetose e hipoalbuminemia. Realizada EDA com biópsia que juntamente com anti endomisio IgA positivo resultaram no diagnóstico de DC. Paciente manteve-se com leucocitose e plaquetose. O objetivo deste relato é apresentar uma paciente com diagnóstico de DC e revisar aspectos clínicos e terapêuticos atuais da doença. Palavras-chave: Doença Celíaca; Enteropatia Perdedora de Proteína ; Plaquetose; Leucocitose.

Primary central nervous system lymphoma with intramedullary spinal cord involvement mimicking inflammatory demyelinating disease

BACKGROUND: Spinal cord involvement of primary central nervous system lymphoma (PCNSL) is rare in a young immunocompetent patient and can be misdiagnosed as an inflammatory demyelinating disease (IDD) of the central nervous system.

Hepcidin-orchestrated Hemogram and Iron Homeostatic Patterns in Two Models of Subchronic Hepatic injury / 生物医学与环境科学（英文）

OBJECTIVE@#This study was designed to evaluate hematological disorders and the orchestrating roles of hepcidin and IL-6 in rat models of thioacetamide (TAA) and carbon tetrachloride (CCl4) hepatotoxicity.@*METHODS@#Rats were intraperitoneally injected with TAA (10 mg/100 g rat weight dissolved in isosaline) or CCl4 (100 μL/100 g rat weight diluted as 1:4 in corn oil) twice weekly for eight consecutive weeks to induce subchronic liver fibrosis. Blood and tissue samples were collected and analyzed.@*RESULTS@#CCl4 but not TAA significantly decreased the RBCs, Hb, PCV, and MCV values with minimal alterations in other erythrocytic indices. Both hepatotoxins showed leukocytosis, granulocytosis, and thrombocytopenia. By the end of the experiment, the erythropoietin level increased in the CCl4 model. The serum iron, UIBC, TIBC, transferrin saturation%, and serum transferrin concentration values significantly decreased, whereas that of ferritin increased in the CCl4 model. TAA increased the iron parameters toward iron overload. RT-PCR analysis revealed increased expression of hepatic hepcidin and IL-6 mRNAs in the CCl4 model and suppressed hepcidin expression without significant effect on IL-6 in the TAA model.@*CONCLUSION@#These data suggest differences driven by hepcidin and IL-6 expression between CCl4 and TAA liver fibrosis models and are of clinical importance for diagnosis and therapeutics of liver diseases.

Neutrophil-Lymphocyte Ratio Predicts Organ Salvage in Testicular Torsion with Marginal Diagnostic Delay

PURPOSE: Though prompt diagnosis to minimize symptom duration (SD) is highly associated with organ salvage in cases of testicular torsion (TT), SD is subjective and hard to determine. We thus investigated the clinical implications of systemic inflammatory responses (SIRs) as potential surrogates of SD to improve testis survival. MATERIALS AND METHODS: Sixty men with TT that underwent immediate operation among orchiectomy and orchiopexy following a visit to a single emergency department were retrospectively enrolled. Mandatory laboratory tests conducted included neutrophil, lymphocyte, and platelet counts. RESULTS: Mean age and SD was 15.7±3.7 years and 8.27±4.98 hours, respectively. Thirty-eight (63.3%) underwent orchiectomy and the remaining 22 underwent orchiopexy. Leukocytosis (p=0.001) and neutrophil-lymphocyte ratio (NLR, p < 0.001) were significantly lower in the orchiopexy group as was SD (3.27±1.88 vs. 11.16±3.80, p < 0.001). Although multivariate model showed that the only single variable associated with receipt of orchiopexy was SD (odds ratio [OR]=0.259, p < 0.001), it also revealed NLR as a sole SIR associated with SD (B=0.894, p < 0.001). While 93.3% with a SD of within 3 hours underwent orchiopexy, only 26.6% of affected testes were preserved between 3 to 12 hours (n=30). When multivariable analysis was applied to those with window period, NLR alone predicted orchiopexy rather than orchiectomy (p=0.034, OR=0.635, p=0.013). The area under curve between SD (0.882) and NLR (0.756) was similar (p=0.14). CONCLUSIONS: This study showed NLR independently predicted testis survival by proper surgical correction particularly for patients with marginally delayed diagnosis, which suggest the clinical usefulness for identifying candidates for orchiopexy in emergency setting.

Leukostasis by hyperleukocytic childhood acute leukemia: pathophysiology, clinical presentation, and emergency care

Hyperleukocytosis (HL), defined by a peripheral white blood cell (WBC) count exceeding 100,000/mm³, is occasionally observed in childhood acute leukemia. The increased viscosity in the micro-circulation by HL and the interaction between the leukemic blasts and endometrium of blood vessels sometimes result in leukostasis. Leukostasis can incur life-threatening manifestations, such as respiratory distress, brain infarction and hemorrhage, and renal failure, needing an emergency care. Although early stage of leukostasis is difficult to detect due to nonspecific manifestations, an emergency care is mandatory because leukostasis can proceed to a fatal course. Initial management includes an aggressive fluid therapy that can reduce WBC count, and prevent other metabolic complications implicated by HL. Packed red blood cells should be judiciously transfused because it increases blood viscosity. Conversely, transfusion of platelet concentrates or fresh frozen plasma, which does not affect blood viscosity, is recommended for prevention of hemorrhage. To reduce tumor burden, leukapheresis or exchange transfusion is commonly performed. However, the efficacy is still controversial, and technical problems are present. Leukapheresis or exchange transfusion is recommended if WBC count is 200,000–300,000/mm³ or more, especially in acute myelocytic leukemia, or manifestations of leukostasis are present. In addition, early chemotherapy is the definite treatment of leukostasis.

A Child of Severe Mycoplasma pneumoniae pneumonia with Multiple Organ Failure Treated with ECMO and CRRT

Mycoplasma pneumoniae (MP) is the most common causative agent of community-acquired pneumonia in school-aged children. An 8-year-old boy who had been diagnosed with autism looked severely ill when he presented to our hospital due to dyspnea and lethargy. He had fever and cough 7 days prior to hospitalization. He had signs and symptoms of severe respiratory distress. The percutaneous oxygen saturation was 88% at high oxygen supply. Chest radiography showed diffusely increased opacity with moderate pleural effusion. He was intubated immediately and admitted to the intensive care unit. Under the clinical impression of mycoplasmal pneumonia, intravenous clarithromycin was started. Laboratory findings showed leukocytosis, hepatitis, decreased renal function, and presence of serum MP immunoglobulin (Ig) M (+) IgG (+) and sputum MP polymerase chain reaction (+). On hospital day 2, the patient developed multiple organ failure with acute respiratory distress syndrome (ARDS). Veno-venous extracorporeal membrane oxygenation (ECMO) was performed with continuous renal replacement therapy (CRRT) and was weaned successfully. This is the first reported case of an ARDS due to MP infection complicated by multiple organ failure that was successfully treated with ECMO and CRRT in South Korea.