Síndrome de Hedinger secundario a Tumor neuroendocrino bronquial. Presentación de un caso

El síndrome carcinoide es un síndrome paraneoplásico que se presenta en tumores neuroendocrinos. Aunque es una entidad infrecuente suele ser la primera manifestación de la enfermedad. La baja incidencia junto a la presentación inespecífica genera retrasos diagnósticos importantes. Se presenta el caso de una paciente con síntomas digestivos y tuforadas que posteriormente agrega insuficiencia cardíaca, logrando mediante un ecocardiograma típico y marcadores analíticos el diagnóstico de síndrome carcinoide. Posteriormente se evidencia que su origen en un tumor neuroendocrino bronquial. Conocer las características de este síndrome es fundamental para mantener una alta sospecha clínica en pacientes con síntomas sugestivos logrando un diagnóstico precoz y adecuado.

Carcinoid syndrome is a paraneoplastic syndrome that occurs in neuroendocrine tumors. Although It is an uncommon entity, it is usually the first manifestation of the disease. The low incidence besides the non-specific presentation generates important diagnostic delays. We present the case of a patient presenting digestive symptoms and flushing that subsequently adds heart failure, achieving though a typical echocardiogram and analytical markers the diagnosis of carcinoid syndrome. Later it is discovered its origin in a bronchial neuroendocrine tumor. Knowing the characteristics of this syndrome is essential to maintain a high clinical suspicion in patients with suggestive symptoms, in order to achieve an early and adequate diagnosis.

El síndrome carcinoide é um síndrome paraneoplásico que ocorre em tumores neuroendócrinos. Embora seja uma entidade rara, geralmente é a primeira manifestação da doença. A baixa incidência, juntamente com a apresentação inespecífica, resulta em atrasos importantes no diagnóstico. Apresentamos o caso de uma paciente com sintomas digestivos e ruborização cutânea, que posteriormente desenvolve insuficiência cardíaca. O diagnóstico de síndrome carcinoide foi estabelecido por meio de um ecocardiograma característico e marcadores analíticos. Posteriormente, foi evidenciada a origem em um tumor neuroendócrino brônquico. Conhecer as características deste síndrome é fundamental para manter uma alta suspeita clínica em pacientes com sintomas sugestivos, permitindo um diagnóstico precoce e adequado.

Falta de adherencia al control médico en pacientes pediátricos con antecedente de glomerulonefritis infecciosa

Introducción: La glomerulonefritis pos infecciosa (GNPI) en la infancia es un factor de riesgo para el desarrollo de enfermedad renal crónica a largo plazo. La adherencia al control médico permite realizar la nefroprevención secundaria. Objetivo: evaluar la relación entre los factores de riesgo relacionados a la falta de adherencia al control médico de pacientes con GNPI en una cohorte pediátrica. Metodología: estudio descriptivo de asociación cruzada de cohorte retrospectiva de pacientes internados entre enero de 2000 a diciembre de 2018 en un hospital de referencia. Se analizó la relación entre: hacinamiento, colecho, escolaridad materna y paterna, número de hermanos, de convivientes y procedencia, con la falta de adherencia al control médico. Se utilizaron la prueba de chi cuadrado y regresión logística a un nivel de significancia de 0,05. Resultados: Se incluyeron 148 pacientes (103 niños y 45 niñas) entre 2 a 16 años (edad promedio: 8,5± 3,4 años). La falta de adherencia fue encontrada en 73 pacientes (49,3%) que se asoció a procedencia rural (p= 0,012, RR: 1,50, IC95%: 1,10-2,06), baja escolaridad materna (p= 0,046, IC95%: 1,54:1,14-2,08), baja escolaridad paterna (p= 0,02; RR: 1,483, IC95%: 1,09-2,01), >3 convivientes (p=0,007, RR: 1,630, IC95%: 1,21-2,19), colecho (p=0,026; RR: 1,52, IC95%: 1,02-2,27) y hacinamiento (p<0,0001; RR: 1,92, IC95%: 1,39-2,65). Por regresión logística, el hacinamiento (p=0,005; OR= 4,8) y procedencia rural (p=0,022; OR: 2,4) se mantuvieron asociados a la falta de adherencia. Discusión: El hacinamiento y la procedencia rural se asociaron en forma independiente con la pérdida de seguimiento. Se recomienda mayor intervención de la atención primaria de salud.

Introduction: Post-infectious glomerulonephritis (PIGN) in childhood is a risk factor for the development of long-term chronic kidney disease. Adherence to medical control allows secondary nephroprevention to be carried out. Objective: to evaluate the relationship between risk factors related to non-adherence to medical control of patients with IPGN in a pediatric cohort. Methods: descriptive study, with an analytical component of a retrospective cohort of patients hospitalized between January 2000 and December 2018 in a reference hospital. The relationship between: overcrowding, co-sleeping, maternal and paternal education, number of siblings, cohabitants and origin, with lack of adherence to medical control was analyzed. The chi-square test and logistic regression were used at a significance level of 0.05. Results: a total of 148 patients (103 boys y 45 girls) between 2 and 16 years old (mean age: 8.5± 3.4 years) were included. The lack of adherence was found in 73 patients (49.3%) that was associated with rural origin (p= 0.012, RR: 1.50, 95% CI: 1.10-2.06), low maternal education (p= 0.046, 95%CI: 1.54:1.14-2.08), low paternal education (p= 0.02; RR: 1.483, 95%CI: 1.09-2.01), >3 cohabitants (p=0.007, RR: 1.630, 95% CI: 1.21-2.19), co-sleeping (p=0.026; RR: 1.52, 95% CI: 1.02-2.27) and overcrowding (p<0.0001; RR: 1.92, 95% CI: 1.39-2.65). By logistic regression, overcrowding (p=0.005; OR= 4.8) and rural origin (p=0.022; OR: 2.4) remained associated with lack of adherence. Discussion: Overcrowding and rural origin were independently associated with loss to follow-up. Greater intervention by primary health care is recommended.

Effect of octreotide on oxidative stress in the erythrocyte and kidney tissue in adriamycin-induced experimental nephrotic syndrome model / Efeito do octreotide no estresse oxidativo em eritrócitos e no tecido renal no modelo de síndrome nefrótica experimental induzida por adriamicina

Abstract Introduction: Nephrotic syndrome (NS) is one of the reasons of end-stage kidney disease, and elucidating the pathogenesis and offer new treatment options is important. Oxidative stress might trigger pathogenesis systemically or isolated in the kidneys. Octreotide (OCT) has beneficial antioxidant effects. We aimed to investigate the source of oxidative stress and the effect of OCT on experimental NS model. Methods: Twenty-four non-uremic Wistar albino rats were divided into 3 groups. Control group, 2 mL saline intramuscular (im); NS group, adriamycin 5 mg/kg intravenous (iv); NS treatment group, adriamycin 5 mg/kg (iv) and OCT 200 mcg/kg (im) were administered at baseline (Day 0). At the end of 21 days, creatinine and protein levels were measured in 24-hour urine samples. Erythrocyte and renal catalase (CAT) and thiobarbituric acid reactive substance (TBARS) were measured. Renal histology was also evaluated. Results: There was no significant difference among the 3 groups in terms of CAT and TBARS in erythrocytes. Renal CAT level was lowest in NS group, and significantly lower than the control group. In treatment group, CAT level significantly increased compared with NS group. In terms of renal histology, tubular and interstitial evaluations were similar in all groups. Glomerular score was significantly higher in NS group compared with control group and it was significantly decreased in treatment group compared to NS group. Conclusions: Oxidative stress in NS might be due to the decrease in antioxidant protection mechanism in kidney. Octreotide improves antioxidant levels and histology in renal tissue and might be a treatment option.

Resumo Introdução: Síndrome nefrótica (SN) é uma das causas de doença renal em estágio terminal. É importante elucidar a patogênese e oferecer novas opções de tratamento. Estresse oxidativo pode desencadear a patogênese sistemicamente ou isoladamente nos rins. O octreotide (OCT) tem efeitos antioxidantes benéficos. Nosso objetivo foi investigar a fonte de estresse oxidativo e efeito do OCT no modelo experimental de SN. Métodos: Dividimos 24 ratos albinos Wistar não urêmicos em 3 grupos. Grupo controle, 2 mL de solução salina intramuscular (im); grupo SN, adriamicina 5 mg/kg intravenosa (iv); grupo tratamento SN, adriamicina 5 mg/kg (iv) e OCT 200 mcg/kg (im) foram administrados no início do estudo (Dia 0). Aos 21 dias, mediram-se os níveis de creatinina e proteína em amostras de urina de 24 horas. Mediu-se a catalase (CAT) eritrocitária e renal e a substância reativa ao ácido tiobarbitúrico (TBARS). Avaliou-se também histologia renal. Resultados: Não houve diferença significativa entre os três grupos em termos de CAT e TBARS em eritrócitos. O nível de CAT renal foi menor no grupo SN e significativamente menor que no grupo controle. No grupo tratamento, o nível de CAT aumentou significativamente em comparação com o grupo SN. Quanto à histologia renal, as avaliações tubular e intersticial foram semelhantes em todos os grupos. O escore glomerular foi significativamente maior no grupo SN em comparação com o grupo controle e diminuiu significativamente no grupo de tratamento em comparação com o grupo SN. Conclusões: Estresse oxidativo na SN pode ser devido à diminuição do mecanismo de proteção antioxidante nos rins. O octreotide melhora níveis de antioxidantes e histologia do tecido renal e pode ser uma opção de tratamento.

Síndrome de Brown-Séquard como forma de presentación de mielitis transversa idiopática / Brown-Séquard syndrome as a presentation of idiopathic transverse myelitis

El síndrome de Brown-Séquard es el conjunto de signos y síntomas causado por hemisección medular de diversos orígenes. Puede generarse por múltiples causas; las traumáticas son las más frecuentes. Las causas menos frecuentes son patología inflamatoria, isquémica, tumoral o infecciosa. Se presenta un niño de 12 años, con instauración aguda y progresiva de un síndrome de hemisección medular derecho, con parálisis hipo/arrefléctica homolateral y afectación de sensibilidad termoalgésica contralateral. En la resonancia magnética de médula espinal, se observó compromiso inflamatorio en hemimédula derecha a nivel de segunda y tercera vértebras torácicas. Con diagnóstico de mielitis transversa idiopática, inició tratamiento con corticoide intravenoso a altas dosis con evolución clínica favorable y restitución de las funciones neurológicas.

Brown-Séquard syndrome refers to a set of signs and symptoms caused by hemisection of the spinal cord from various sources. It may have multiple causes; traumatic injuries are the most frequent ones. The less common causes include inflammation, ischemia, tumors, or infections. This report is about a 12-year-old boy with an acute and progressive course of right hemisection of the spinal cord, with ipsilateral hypo/areflexic paralysis and contralateral loss of thermalgesic sensation. The MRI of the spinal cord showed inflammation in the right side of the spinal cord at the level of the second and third thoracic vertebrae. The patient was diagnosed with idiopathic transverse myelitis and was started on intravenous high-dose corticosteroids; he showed a favorable clinical course and recovered neurological functions.

Trasplante de médula ósea haploidéntico en un paciente pediátrico con síndrome de Wiskott-Aldrich. A propósito de un caso / Haploidentical bone marrow transplantation in a pediatric patient with Wiskott-Aldrich syndrome. A case report

El síndrome de Wiskott-Aldrich es un error innato de la inmunidad de herencia ligada al cromosoma X, producido por variantes en el gen que codifica la proteína del síndrome de Wiskott-Aldrich (WASp). Reportamos el caso clínico de un paciente de 18 meses con diagnóstico de Wiskott-Aldrich que no presentaba donante antígeno leucocitario humano (HLA) idéntico y recibió un trasplante de células progenitoras hematopoyéticas (TCPH) con donante familiar haploidéntico. La profilaxis para enfermedad de injerto contra huésped incluyó ciclofosfamida (PT-Cy). El quimerismo del día +30 fue 100 % del donante y la evaluación postrasplante de la expresión de la proteína WAS fue normal. Actualmente, a 32 meses del trasplante, presenta reconstitución hematológica e inmunológica y quimerismo completo sin evidencia de enfermedad injerto contra huésped. El TCPH haploidéntico con PT-Cy se mostró factible y seguro en este caso de síndrome de WiskottAldrich en el que no se disponía de un donante HLA idéntico.

Wiskott-Aldrich syndrome (WAS) is an X-linked genetic disorder caused by mutations in the gene that encodes the Wiskott-Aldrich syndrome protein (WASp). Here, we report the clinical case of an 18-month-old boy diagnosed with Wiskott-Aldrich syndrome, who did not have an HLA-matched related or unrelated donor and was treated successfully with a hematopoietic stem cell transplant (HSCT) from a haploidentical family donor. Graft-versus-host disease (GvHD) prophylaxis included post-transplant cyclophosphamide (PT-Cy). At day +30, the peripheral blood-nucleated cell chimerism was 100% and the WAS protein had a normal expression. Currently, at month 32 post-transplant, the patient has hematological and immune reconstitution and complete donor chimerism without evidence of GvHD. HSCT with PT-Cy was a feasible and safe option for this patient with WAS, in which an HLA matched donor was not available.

Arboviruses epidemic associates with Guillain-Barré syndrome: temporal series in northeast Brazil / Epidemia de arboviroses associada à síndrome de Guillain-Barré: série temporal no nordeste brasileiro / Epidemia de arbovirosis asociada al síndrome de Guillain-Barré: serie temporal en el noreste de Brasil

Arboviruses cause public health problems in several countries, and records show that they can generate central and peripheral neurological complications with permanent sequelae. However, it is not certain which arbovirus is responsible for outbreaks of the Guillain-Barré Syndrome (GBS), especially in Brazil. Thus, the objective of this study is to verify if there is a coincidence between the GBS outbreak and the most common arboviruses in Northeastern Brazil, as well as their relationship. An ecological time series study was designed with the federative units of Northeastern Brazil, using hospitalizations for Guillain-Barré syndrome and notifications of arbovirus infections between 2014 and 2019 as a data source. Distribution incidence curves were constructed for the conditions studied, and generalized estimating equations (GEE) models were applied to estimate the relationship between arboviruses and Guillain-Barré. The results showed a similar distribution for the incidences of Chikungunya virus (z=7.82; p=0.001), Zika virus (z=3.69; p=0.03), and Guillain-Barré syndrome (z=2.98; p=0.05) from 2014 to 2019. The GEE model revealed that the distribution of Chikungunya incidence is associated with the distribution of GBS incidence in each year (x2Wald=3,969; p=0.046). This pattern was repeated in seven of the nine states, while the Zika virus had a significant relationship with GBS in only two states. The outbreak of GBS in Northeastern Brazil appears to be probabilistically related to outbreaks of the Chikungunya virus.

As arboviroses são problemas de saúde pública em vários países e há registros de que podem produzir complicações neurológicas centrais e periféricas com sequelas permanentes. Entretanto, não se sabe ao certo qual delas é realmente responsável pelos surtos da Síndrome de Guillain-Barré (SGB), principalmente no Brasil. Assim, o objetivo é verificar se há coincidência entre o surto de SGB e as arboviroses mais comuns no Nordeste do Brasil e suas relações. Foi desenhado um estudo ecológico de série temporal com as unidades federativas do Nordeste do Brasil, adotando como fonte de dados as internações Guillain-Barré e as notificações de infecções por arbovírus entre 2014 e 2019. Curvas de distribuição de incidência foram construídas para as condições estudadas, e foram aplicados modelos de equações generalizadas estimadas (GEE) para estimar a relação entre arbovírus e Guillain-Barré. Evidencia-se que há distribuição semelhante para as incidências do vírus Chikungunya (z=7,82; p=0,001), vírus Zika (z=3,69; p=0,03) e síndrome de Guillain-Barré (z=2,98; p=0,05) entre 2014 e 2019. O modelo GEE revelou que a distribuição da incidência de Chikungunya está associada à distribuição da incidência de SGB em cada ano (x2Wald=3,969; p=0,046). Esse padrão se repetiu em sete dos nove estados, enquanto o zika vírus teve uma relação significativa com o GBS em apenas dois estados. Conclui-se, então, que o surto de SGB no Nordeste do Brasil parece estar probabilisticamente relacionado aos surtos do vírus Chikungunya.

Los arbovirus causan problemas de salud pública en varios países y, según indican los reportes, pueden producir complicaciones neurológicas centrales y periféricas con secuelas permanentes. Sin embargo, no se sabe cuál de ellos es realmente el responsable de los brotes del síndrome de Guillain-Barré (SGB), especialmente en Brasil. Así, el objetivo de este estudio es verificar si existen coincidencias entre el brote del SGB y los arbovirus más comunes en el Noreste de Brasil y sus asociaciones. Se diseñó un estudio de series temporales ecológico en las unidades federativas del Noreste de Brasil, adoptando como fuente de datos las hospitalizaciones y las notificaciones de arbovirosis de Guillain-Barré entre 2014 y 2019. Se construyeron curvas de distribución de incidencia para las condiciones científicas, y se aplicó una ecuación estimada generalizada (GEE) para estimar la relación entre arbovirus y Guillain-Barré. Se encontró que existe una distribución similar en las incidencias de virus del chikunguña (z=7,82; p=0,001), virus del Zika (z=3,69; p=0,03) y síndrome de Guillain-Barré (z =2,98; p=0,05) entre 2014 y 2019. El modelo GEE reveló que la distribución de la incidencia de chikunguña está asociada con la distribución de la incidencia de SGB en cada año (x2Wald=3,969; p=0,046). Este patrón se repitió en siete de los nueve estados, mientras que el virus del Zika presentó una relación significativa con el SGB en solo dos estados. El brote del SGB en el Noreste de Brasil parece estar relacionado probabilísticamente con los brotes del virus del chikunguña.

Dress por litio: reporte de un caso pediátrico / Lithium dress: a pediatric case report / Dress por Lítio: relato de caso pediátrico

La reacción a medicamentos con eosinofilia y síntomas sistémicos denominada DRESS(por sus siglas en inglés, Drug Reaction with Eosinophilia and Systemic Symptoms) hace parte de un amplio espectro denominado toxicodermias. La incidencia exacta no es conocida en niños; sin embargo, en la literatura se ha estimado una tasa de mortalidad que puede llegar a ser tan alta como el 10 %. Presentamos el caso de una paciente adolescente con antecedente personal de trastorno afectivo bipolar (TAB), quien recibía de forma ambulatoria sertralina, quetiapina y trazodona. Por presencia de alucinaciones se adicionó litio al manejo. Diez días después acude al servicio de urgencias por aparición de erupción cutánea y síntomas sistémicos, por lo que se sospechó un cuadro clínico secundario a hipersensibilidad a los medicamentos.

The reaction to drugs with eosinophilia and systemic symptoms called DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) is part of a broad spectrum called toxicodermias. The exact incidence is not known in children; However, a mortality rate that can be as high as 10% has been estimated in the literature. We present the case of a teenage patient with a personal history of bipolar affective disorder (BD), who received sertraline, quetiapine and trazodone on an outpatient basis. Due to the presence of hallucinations, lithium was added to the management. Ten days later she went to the emergency department due to the appearance of a skin rash and systemic symptoms, for which a clinical condition secondary to hypersensitivity to medications was suspected.

A reação a medicamentos com eosinofilia e sintomas sistêmicos denominada DRESS (Por suas siglas em inglês Drug Reaction with Eosinophilia and Systemic Symptoms) faz parte de um amplo espectro denominado toxicodermias. A incidência exata não é conhecida em crianças; No entanto, uma taxa de mortalidade que pode chegar a 10% foi estimada na literatura. Apresentamos o caso de um paciente adolescente com história pessoal de transtorno afetivo bipolar (TAB), que recebeu sertralina, quetiapina e trazodona em regime ambulatorial. Devido à presença de alucinações, foi adicionado lítio ao manejo. Dez dias depois, recorreu ao pronto-socorro devido ao aparecimento de erupção cutânea e sintomas sistêmicos, suspeitando-se de quadro clínico secundário a hipersensibilidade a medicamentos.

Síndrome de abstinência em Unidade de Tratamento Intensivo Pediátrico: protocolo de revisão sistemática / Abstinence syndrome in Pediatric Intensive Care Unit: a systematic review protocol

OBJETIVO: Verificar qual o tratamento mais indicado para a prevenção e redução dos sinais e sintomas de abstinência em crianças criticamente doentes por meio de uma revisão sistemática da literatura mundial. MÉTODO: A revisão sistemática será conduzida conforme a metodologia PRISMA e Cochrane, com registro no PROSPERO, sob o número de ID CRD42021274670, nas respectivas bases de dados PubMed, LILACS, Embase, Web of Science, Cochrane, CINAHL, Cochrane Database Systematic Review e CENTRAL. As buscas serão realizadas por dois avaliadores independentes, um terceiro realizará o intermédio se necessário. Os dados serão inseridos no programa de software Zotero que irá excluir os artigos duplicados, após o material selecionado será transferido para planilha Excel em instrumento próprio. Os estudos serão classificados quanto ao seu nível de evidência, viés e fator de risco. Os resultados serão analisados e tabulados e discutidos a fim de melhor compreensão dos resultados. Se possível, serão realizadas meta-análises para os resultados agregados.

OBEJECTIVE: To verify the most appropriate treatment for the prevention and reduction of the signs and symptoms of abstinence in critically ill children through a systematic review of the world literature. METHOD: The systematic review will be conducted according to the PRISMA and Cochrane methodology, with registration at PROSPERO, under the ID number CRD42021274670, in the respective databases, PUBMed, LILACS, Embase, Web of Science, Cochrane, CINAHL, Cochrane Database Systematic Review, and CENTRAL, searches will be carried out by two independent evaluators, and a third party will perform the intermediate if necessary. The data will be entered into the Zotero software program that will delete duplicate articles after the selected material is transferred to an Excel spreadsheet on its instrument. The studies will be classified according to their level of evidence, bias, and risk factors. The results will be analyzed, tabulated, and discussed to understand the results better. If possible, meta-analyzes will be carried out for the aggregated results.

Incontinencia pigmenti. progresión clínica y diagnóstico de un caso / Incontinentia pigmenti. clinical progression and diagnosis of a case

Resumen La incontinencia pigmenti(IP) es una genodermatosis infrecuente ysistémica del neuroectodermo que involucra la piel, el sistema nervioso central, los ojos y los dientes, entre otros. Los signos clínicos dermatológicos constituyen el principal criterio diagnóstico, debido a que suelen ser los primeros en manifestarse. Se describen cuatro estadios característicos de la enfermedad según las lesiones cutáneas predominantes. No obstante, su pronóstico depende de los signos y síntomas extracutáneos. El diagnóstico se centra en criterios clínicos, histopatológicos y/o genéticos. Visto que no existe una terapéutica específica, la atención médica de esta enfermedad es multidisciplinaria y sintomática, y debe acompañarse del asesoramiento genético a los afectados y sus familiares. Presentamos el caso de una niña, nacida a término completo, quien presentó un cuadro clínico compatible con incontinencia pigmenti, del cual detallamos su progresión clínica, diagnóstico y seguimiento.

Abstract Incontinentiapigmenti is a rare and multisystemic,neuroectodermal genodermatosis that involves the skin, central nervous system, eyes and teeth, among others. Dermatological clinical signs are the main diagnostic criteria because they are usually the first to manifest. Four characteristic stages of the disease are described according to the predominant skin lesions. Nonetheless, prognosis depends on extracutaneous clinical signs and symptoms. Diagnosis is based on clinical, histopathological and/or genetic criteria. Considering there is no specific treatment available, the management of this disease is multidisciplinary and symptomatic, and must be accompanied by genetic counseling for those affected and their families. We present in a full-tern newborn femalethat presented with a clinical picture compatible with incontinentiapigmenti, and we will detail the clinical progression, diagnosis, and follow-up.

Síndrome de Kartagener y artritis reumatoide. Reporte de caso / Kartagener Syndrome and Rheumatoid Arthritis. Case Report

Presentación del caso. Se trata de una mujer de 26 años de edad, en seguimiento por la especialidad de reumatología desde los 17 años, cuando consultó con historia de un año de evolución de síndrome poliarticular de grandes y pequeñas articulaciones, aditivo, simétrico acompañado de fatiga, rigidez matutina mayor de una hora. Se reportó además factor reumatoide positivo. La radiografía de ambas manos presentó erosiones, que confirmó el diagnóstico de artritis reumatoide. Adicionalmente, la paciente tenía el antecedente de procesos sinobronquiales a repetición desde su infancia. En la evaluación médica se identificó dolor en los senos paranasales, dextrocardia y bronquiectasias, confirmados por los estudios de imágenes, que permitió concluir en el diagnóstico de síndrome de Kartagener. Intervención terapéutica. La paciente presentaba actividad clínica severa de la artritis reumatoide, se inició el tratamiento con metotrexato 10 mg vía oral un día a la semana, prednisona 5 mg al día y ácido fólico 5 mg a la semana y citas periódicas, controlando los datos de actividad y efectos adversos de los medicamentos, con pruebas hepáticas, hemograma y transaminasas. La especialidad de neumología recomendó la inclusión de la paciente en un programa de rehabilitación respiratoria, así como el uso de azitromicina 500 mg cada día por tres días en los períodos de agudización. Evolución clínica. El tratamiento logró mantener una actividad leve de la artritis reumatoide y sin exacerbación de los síntomas respiratorios

Case presentation. A 26-year-old woman, under follow-up by the rheumatology specialty since she was 17 years old, when she consulted with a history of one year of evolution of polyarticular disease of large and small joints, additive, symmetrical, accompanied by fatigue and morning stiffness for more than one hour. Positive rheumatoid factor was also reported. Additionally, the patient had a history of repeated sinobronchial processes since childhood. Medical examination revealed sinus pain in the paranasal sinuses, dextrocardia, and bronchiectasis, confirmed by imaging studies, which led to the diagnosis of Kartagener's syndrome. Treatment. The patient presented the severe clinical activity of rheumatoid arthritis. The treatment was started with methotrexate 10 mg orally one day a week, prednisone 5 mg a day, and folic acid 5 mg a week and periodic appointments, controlling the activity data and adverse effects of the drugs, with liver tests, hemogram, and transaminases. The pneumology department recommended the inclusion of the patient in a respiratory rehabilitation program as well as the use of azithromycin 500 mg every day for three days during periods of exacerbation. Outcome. The treatment was successful in maintaining a mild activity of the rheumatoid arthritis and without exacerbation of respiratory symptoms

Evolución clínica en pacientes con síndrome del túnel carpiano tratados con ultrasonido o infiltración con corticoesteroides / Clinical Evolution in Patients with Carpal Tunnel Syndrome Treated with Ultrasound or Corticosteroid Infiltration

Introducción. El Síndrome del túnel carpiano es la neuropatía periférica compresiva más común de la extremidad superior, que se produce por la compresión del nervio mediano. Los casos leves y moderados pueden tratarse con métodos conservadores como ultrasonido terapéutico o infiltración con corticoesteroides. Objetivo. Describir la evolución clínica de pacientes con síndrome de túnel carpiano tratados con terapia por ultrasonido e infiltración de corticoesteroides. Metodología. Ensayo clínico abierto, en pacientes con síndrome del túnel carpiano leve y moderado, que consultaron del 1 de octubre 2021 al 30 de mayo 2022. Se formaron dos grupos; el que recibió tratamiento con ultrasonido con 12 casos y el grupo tratado con infiltración con corticoesteroides con seis casos. Ambos grupos fueron intervenidos en la consulta inicial, y luego, en las cuatro y ocho semanas posteriores al inicio del tratamiento. Resultados. Se muestran los resultados descriptivos relacionados con la intensidad de dolor, valorada con la Escala Visual Numérica, la infiltración obtuvo dos casos sin dolor y cuatro con dolor moderado, contrario a ultrasonido que se mantuvo con cuatro casos leves, tres moderados y cinco intensos. En los síntomas, la infiltración redujo el número de casos en cuatro de los síntomas estudiados, en cambio el ultrasonido únicamente en dos. En severidad, valorada con el cuestionario de Boston para túnel carpal, con infiltración se obtuvieron dos casos asintomáticos y ninguno con ultrasonido. Respecto a los signos clínicos, el signo de Tinel desapareció en cuatro casos en ambos grupos, mientras que signo de Phalen desapareció en cuatro casos en ultrasonido y dos en infiltración. Conclusión. En intensidad de dolor y grado de severidad, la infiltración generó casos asintomáticos y redujo mayor cantidad de síntomas que el ultrasonido. Ambos tratamientos disminuyeron la presencia de signos clínicos

Introduction. Carpal tunnel syndrome is the most common compressive peripheral neuropathy of the upper extremity, which is caused by compression of the median nerve. Mild and moderate cases can be treated with conservative methods such as therapeutic ultrasound or corticosteroid infiltration. Objective. To describe the clinical evolution of patients with carpal tunnel syndrome treated with ultrasound therapy and corticosteroid infiltration. Methodology. A prospective open clinical trial was conducted in patients with mild and moderate carpal tunnel syndrome who consulted from October 1, 2021 to May 30, 2022. Two groups were formed: the group that received ultrasound treatment with 12 cases and the group treated with corticosteroid infiltration with six cases. Both groups were treated at the initial consultation and then at four and eight weeks after the start of treatment. Results. The descriptive results related to the intensity of pain, evaluated with the Visual Numeric Scale, are shown. Infiltration obtained two cases without pain and four with moderate pain, contrary to ultrasound which was maintained with four mild, three moderate and five intense cases. In symptoms, infiltration reduced the number of cases in four of the symptoms studied, while ultrasound reduced the number of cases in only two. In severity, assessed with the Boston carpal tunnel questionnaire, with infiltration, there were two asymptomatic cases and none with ultrasound. Regarding clinical signs, Tinel's sign disappeared in four cases in both groups, while Phalen's sign disappeared in four cases in ultrasound and two in infiltration. Conclusion. Infiltration produced asymptomatic patients and reduced more symptoms than ultrasonography in terms of pain intensity and severity. Clinical symptoms were less common with both treatments.

Síndrome de desgaste profesional en el personal de salud durante la pandemia de COVID-19 / Burnout Syndrome in Health Care Personnel During the COVID-19Pandemic

La Organización Mundial de la Salud (OMS) define el estrés laboral como una reacción que puede manifestarse ante exigencias y presiones laborales que ponen a prueba la capacidad que tiene cada persona para afrontar ciertas situaciones y que se agravan en el personal de salud que atiende pacientes con la COVID-19. Es decir, lo que resulta del desequilibrio entre las presiones y exigencias a las que se enfrenta el individuo, por una parte, y los conocimientos adquiridos por otra parte. El Síndrome de desgaste profesional, conocido también como síndrome de agotamiento emocional o psicológico, o por el anglicismo burnout, es un tipo de estrés laboral que engloba un estado de agotamiento físico, emocional y mental que conlleva a consecuencias individuales y sociales. El objetivo de esta revisión narrativa es identificar los factores de riesgo para el desarrollo del Síndrome de desgaste profesional en el personal de salud relacionado con la atención de pacientes con la COVID-19. Se realizó una búsqueda en la base de datos PubMed, se incluyeron artículos originales, estudios aleatorizados, revisiones sistemáticas y otros textos en español e inglés, publicados durante el periodo 2020-2023. Los principales factores de riesgo identificados en la literatura para el desarrollo de Síndrome de desgaste profesional fueron la juventud, sexo femenino, la soltería, la carga de trabajo y el nivel de satisfacción laboral de los profesionales.

The WHO defines occupational stress as a reaction that may occur when a person is faced with work-related demands and pressures that test the individual's ability to cope with certain situations, and it exacerbates in healthcare personnel who provide care to patients with COVID-19. That is, what results from the imbalance between the pressures and demands that the individual faces, on the one hand, and the knowledge acquired on the other hand. Burnout syndrome is a type of work-related stress that encompasses a state of physical, emotional and mental exhaustion that leads to individual and social consequences. The objective of this systematic review is to identify the risk factors for the development of Burnout Syndrome in health personnel related to the care of patients with COVID-19. A search was carried out in the PubMed database, including original articles, randomized studies, systematic reviews, and textbooks in Spanish and English, published during the period 2020-2023. The main risk factors for the development of Burnout Syndrome identified in the literature were youth, female sex, singleness, workload and the level of job satisfaction of the professionals

Clinical characterization, physical frailty, and depression in elderly patients with psoriasis from a reference center in Brazil: a cross-sectional study

Abstract Background: There are few studies dedicated to the characterization of the geriatric population with psoriasis, which has particularities in terms of clinical manifestations and therapeutic limitations. As psoriasis is a chronic disease, presenting a higher prevalence with age, the increase in life expectancy in Brazil demands knowledge about the behavior of the disease among the elderly. Objectives: To characterize elderly people with psoriasis from a tertiary service, from the clinical-epidemiological point of view, presence of comorbidities, physical frailty, and affective impact, and to compare these aspects with adults with psoriasis and elderly people without the disease. Methods: Cross-sectional study of 64 elderly patients with psoriasis, 64 adults with psoriasis, and 64 elderly patients without the disease. Clinical-demographic aspects, the Beck depression scale, and Skindex-16 were evaluated. Indicators of physical frailty were evaluated in elderly patients: handgrip, sit-to-stand test, fatigue, and weight loss >5%. Results: In the elderly, the mean age (SD) of psoriasis onset was 44 (10) years, men represented 47% of the sample, the prevalence of arthritis was 22%, and ungual involvement occurred in 72%. Topical corticosteroids were used more often among elderly people with psoriasis (100%) than among adults with the disease (86%), with no difference among other systemic treatments. Diabetes mellitus occurred in 30% of the elderly. Hypertension (59%), dyslipidemia (52%), depression (34%), and fatigue (59%) were more prevalent among the elderly with psoriasis than among the healthy controls.

Post-COVID-19 syndrome: An investigation of speech-language-hearing symptoms / Síndrome Pós-COVID-19: investigação de sintomas fonoaudiológicos

ABSTRACT Purpose: to investigate speech-language-hearing symptoms in adults after the acute phase of COVID-19 and the impact of these persistent symptoms on their physical and emotional aspects, functional capacity, and social relationships. Methods: 204 adults who tested positive for COVID-19 between January 2021 and July 2022 and who completed an online questionnaire, addressing different variables. Data were analyzed with descriptive statistics. Results: the most prevalent symptoms up to 30 days after infection were tiredness (46%), memory loss (40.2%), and ageusia (26.5%). The most found long-term symptoms were memory loss (34.3%), tiredness (21.1%), and difficulties in starting a sentence or conversation (10.1%). Anosmia and ageusia were also cited. These persistent symptoms had an impact on their emotional aspect (33.3%), followed by the physical (26%) and occupational (25%) ones. Conclusion: this study found persistent symptoms after the acute phase of COVID-19, which can lead to speech-language-hearing disorders, such as impaired oral language and eating. These persistent symptoms impacted the participants' emotional, physical, and occupational aspects.

RESUMO Objetivo: investigar a presença de sintomas fonoaudiológicos em adultos após o período da fase aguda da COVID-19, além do impacto da manutenção desses sintomas nos aspectos físicos, emocionais, capacidade funcional e relações sociais. Métodos: participaram 204 adultos que testaram positivo para COVID-19 no período de janeiro de 2021 a julho de 2022 e que preencheram um questionário on- line, abordando diferentes variáveis. Os dados foram analisados por meio de estatística descritiva. Resultados: os sintomas mais prevalentes até 30 dias após a infecção foram cansaço (46%), perda de memória (40,2%) e ageusia (26,5%). Os sintomas de longa duração mais observados foram perda de memória (34,3%), cansaço (21,1%) e dificuldades para iniciar um diálogo ou frase (10,1%). Anosmia e ageusia também foram citadas. Houve impacto da manutenção desses sintomas no aspecto emocional (33,3%), seguido dos aspectos físicos (26%) e ocupacionais (25%). Conclusão: neste estudo foram encontrados sintomas persistentes após o período da fase aguda da COVID-19 que podem levar a alterações fonoaudiológicas, como prejuízo na linguagem oral e nas questões alimentares. A manutenção desses sintomas impactou nos aspectos emocionais, físicos e ocupacionais dos participantes.

Patau and Edwards Syndromes in a University Hospital: beyond palliative care / Síndrome de Edwards e Patau em um hospital universitário: além do paliativo

ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

RESUMO Objetivo: Descrever a população de recém-nascidos com síndrome de Patau (T13) e Edwards (T18) portadores de cardiopatias congênitas, que permaneceram em Unidades de Terapia Intensiva (UTI) de um complexo hospitalar quaternário, com relação a conduta cirúrgica ou não, cuidados paliativos e seus desfechos. Métodos: Série de casos de pacientes internados entre janeiro de 2014 a dezembro de 2018, com análise dos prontuários de portadores de T13 ou T18 que permaneceram internados em UTI que recebem neonatos nesse hospital quaternário. Utilizou-se análise estatística descritiva. Resultados: Foram identificados 33 prontuários para análise — 27 T18 (81,8%) e seis T13 (18,2%); 64% do sexo feminino e 36% do sexo masculino. Oito foram prematuros, nascidos com 30 a 36 semanas (24,2%), e apenas quatro nasceram com mais de 2500 g (12,1%). Quatro pacientes foram submetidos a cirurgia cardíaca e um deles foi a óbito. A mortalidade intra-hospitalar foi de 83% para T13 e 59% para T18. A maioria apresentava outras malformações e foi submetida a outras cirurgias. Cuidados paliativos foram oferecidos a 54% dos pacientes. A mediana do tempo de hospitalização para T18 e T13 foi respectivamente de 29 dias (variação: 2-304) e 25 dias (13-58). Conclusões: Pacientes com T13 e T18 cursam com alta morbimortalidade e longa permanência hospitalar em UTI. São necessários estudos multicêntricos para melhor análise de aspectos importantes para a criação de protocolos que, buscando proporcionalidade terapêutica, tragam melhor qualidade de vida para os pacientes e suas famílias.

When the fever will not stop, stop the pills! A case report

ABSTRACT Neuroleptic malignant syndrome (NMS) is a neurologic emergency potentially fatal. This rare side effect is most commonly associated with first-generation antipsychotics and less frequently with atypical or second-generation antipsychotics. The diagnosis relies on both clinical and laboratory criteria, with other organic and psychiatric conditions being ruled out. CASE REPORT: A 39-year-old female patient, who is institutionalized and completely dependent, has a medical history of recurrent urinary infections and colonization by carbapenem-resistant Klebsiella pneumoniae. Her regular medication regimen included sertraline, valproic acid, quetiapine, risperidone, lorazepam, diazepam, haloperidol, baclofen, and fentanyl. The patient began experiencing dyspnea. Upon physical examination, she exhibited hypotension and a diminished vesicular murmur at the right base during pulmonary auscultation. Initially, after hospitalization, she developed high febrile peaks associated with hemodynamic instability, prompting the initiation of antibiotic treatment. Despite this, her fever persisted without an increase in blood inflammatory parameters, and she developed purulent sputum, necessitating antibiotherapy escalation. The seventh day of hospitalization showed no improvement in symptoms, suggesting NNMS as a differential diagnosis. All antipsychotic and sedative drugs, as well as antibiotherapy, were discontinued, after which the patient showed significant clinical improvement. CONCLUSION: Antipsychotic agents are commonly employed to manage behavioral changes linked to various disorders. However, their severe side effects necessitate a high degree of vigilance, the cessation of all medications, and the implementation of supportive care measures. A prompt and accurate diagnosis of NMS is crucial to alleviating the severe, prolonged morbidity and potential mortality associated with this syndrome.

Hemoglobin/Red Cell Distribution Width Ratio is Associated With Poor Prognosis in Patients With Acute Coronary Syndrome in Long-Term Follow-Up

Abstract Background Acute coronary syndrome (ACS) is the most common subtype of coronary artery disease (CAD). It is one of the main reasons affecting the expected life expectancy and quality of life. Objetives In this study, we aimed to investigate the relationship between major adverse cardiac events (MACE) and Hemoglobin (Hb)/Red cell distribution width (RDW) in long-term follow-up after ACS. Methods A total of 1,146 ACS patients were included in the study, being classified according to the type of myocordial infarction (MI). MACE were recorded in long-term follow-up. The relationship between Hb/RDW and MACE was investigated. The statistical analyses of Mann-Whitney U test for comparison of two independent groups and chi-square test for categorical variables were used. In order to determine the diagnostic feature of the HB/RDW ratio, the diagnostic ratios were calculated by applying Receiver Operating Characteristic Curve (ROC) analysis. A p < 0.05 value was considered statistically significant in all analyses. Results: When the patients were analyzed according to MI types — ST segment elevation myocardial infarction (STEMI)/Non-ST segment elevation myocardial infarction (NSTEMI) —, it was observed that Hb/RDW (p = 0.038) was significantly higher in the STEMI group. The Hb/RDW ratio was statistically significant in predicting mortality. As a result of ROC analysis, Area Under the Curve (AUC) = 0.654 (p < 0.001) was found. The cut-off value for the Hb/RDW ratio was calculated as 0.947. The sensitivity and specificity of 76.9% and 48.4% for the diagnostic rates obtained were moderately acceptable. Conclusion The Hb/RDW-long-term mortality relationship was found to be significant in ROC analysis. It can be used in clinical practice as it is cheap, easy to apply, and reduces possible bias in post-ACS follow-up.

Doble tormenta tiroidea y arrítmica en una gestante con múltiples taquicardias refractarias mediadas por un haz de kent intermitente ablacionado exitosamente

La tormenta tiroidea es un estado crítico y poco frecuente que condiciona la disfunción de múltiples órganos por el efecto del exceso de las hormonas tiroideas, esta disfunción endócrina tiene una elevada mortalidad y genera manifestaciones típicas como la taquicardia, fiebre, alteraciones gastrointestinales, cardiovasculares y del sistema nervioso central. El embarazo se ha asociado con un incremento en la incidencia de arritmias. Necesitan un tratamiento inmediato con drogas antiarrítmicas, cardioversión eléctrica o cesárea de urgencia. El WPW es una anormalidad cardiaca congénita que consiste en la presencia de un haz anómalo (Haz de Kent) que evita el sistema normal de conducción uniendo directamente aurículas y ventrículos. Veremos el caso de una gestante de 32 semanas que presenta un cuadro de tormenta tiroidea y múltiples episodios de taquicardia paroxística supraventricular (TPS), de tórpida y sombría evolución clínica mediada por un haz anómalo de Kent intermitente. Es evidente que la tormenta tiroidea en el contexto de la gestación produjo cambios en las propiedades electrofisiológicas del haz anómalo de Kent intermitente lo cual propició el desarrollo de múltiples taquicardias paroxísticas supraventriculares refractarias a la cardioversión eléctrica y farmacológica. Tampoco mejoró con la tiroidectomía total, solamente cedió por completo con la ablación por catéter de radiofrecuencia del haz anómalo de Kent.

Thyroid storm is a critical and infrequent state that conditions the dysfunction of multiple organs due to the effect of excess thyroid hormones. This endocrine dysfunction has a high mortality and generates typical manifestations such as tachycardia, fever, gastrointestinal, cardiovascular and heart disorders, and the central nervous system. Pregnancy has been associated with an increased incidence of arrhythmias. They need immediate treatment with antiarrhythmic drugs, electrical cardioversion, or emergency caesarean section. WPW is a congenital cardiac abnormality that consists of the presence of an abnormal bundle (Kent bundle) that prevents the normal conduction system, directly joining the atria and ventricles. We will see the case of a 32-week pregnant woman who presented symptoms of thyroid storm and multiple episodes of paroxysmal supraventricular tachycardia (PST), with a torpid clinical course mediated by an abnormal intermittent Kent bundle. It is evident that the thyroid storm in the context of pregnancy produced changes in the electrophysiological properties of the intermittent Kent bundle, which led to the development of multiple PST refractory to electrical and pharmacological cardioversion. Moreover, it also did not improve with total thyroidectomy, only resolved completely with radiofrequency catheter ablation of the Kent bundle.