MRI evaluation of cranial nerve abnormalities and extraocular muscle fibrosis in duane retraction syndrome and congenital extraocular muscle fibrosis.

PURPOSE: To investigate the alterations in extraocular muscles (EOMs) by magnetic resonance imaging (MRI) among patients diagnosed with Duane retraction yndrome (DRS) and congenital fibrosis of the extraocular muscles (CFEOM), who present with various cranial nerve anomalies in an attempt to enhance the clinical diagnostic process. METHODS: A case-control study was conducted to evaluate 27 patients with DRS and 14 patients with CFEOM. All patients underwent MRI scans of the brainstem and orbital examination. Neurodevelopmental assessments were conducted through MRI, and maximum cross-sectional area and volumes of EOMs were obtained. Three types of models were constructed using machine learning decision tree algorithms based on EOMs to predict disease diagnosis, cranial nerve abnormalities, and clinical subtypes. RESULTS: Patients with bilateral CN VI abnormalities had smaller volumes of LR, MR, and IR muscles compared to those with unilateral involvement (P < 0.05). Similarly, patients with CFEOM and unilateral third cranial nerve abnormalities had a smaller maximum cross-section of the affected eye's SR compared to the contralateral eye (P < 0.05). In patients with both CN III and CN VI abnormalities, the volume of SR was smaller than in patients with CN III abnormalities alone (P < 0.05). The prediction model using EOMs volume showed a diagnostic precision of 82.5% for clinical cases and 60.1% for predicting cranial nerve abnormalities. Nonetheless, the precision for identifying clinical subtypes was relatively modest, at only 41.7%. CONCLUSION: The distinctive volumetric alterations in EOMs among individuals exhibiting distinct cranial nerve anomalies associated with DRS or CFEOM provide valuable diagnostic insights into to Congenital Cranial Neurodevelopmental Disorders (CCDDs). MRI analysis of EOMs should thus be regarded as a crucial diagnostic modality.

Point-of-care diagnosis of tissue fibrosis: a review of advances in vibrational spectroscopy with machine learning.

Histopathology is the gold standard for diagnosing fibrosis, but its routine use is constrained by the need for additional stains, time, personnel and resources. Vibrational spectroscopy is a novel technique that offers an alternative atraumatic approach, with short scan times, while providing metabolic and morphological data. This review evaluates vibrational spectroscopy for the assessment of fibrosis, with a focus on point-of-care capabilities. OVID Medline, Embase and Cochrane databases were systematically searched using PRISMA guidelines for search terms including vibrational spectroscopy, human tissue and fibrosis. Studies were stratified based on imaging modality and tissue type. Outcomes recorded included tissue type, machine learning technique, metrics for accuracy and author conclusions. Systematic review yielded 420 articles, of which 14 were relevant. Ten of these articles considered mid-infrared spectroscopy, three dealt with Raman spectroscopy and one with near-infrared spectroscopy. The metrics for detecting fibrosis were Pearson correlation coefficients ranging from 0.65-0.98; sensitivity from 76-100%; specificity from 90-99%; area under receiver operator curves from 0.83-0.98; and accuracy of 86-99%. Vibrational spectroscopy identified fibrosis in myeloproliferative neoplasms in bone, cirrhotic and hepatocellular carcinoma in liver, end-stage heart failure in cardiac tissue and following laser ablation for acne in skin. It also identified interstitial fibrosis as a predictor of early renal transplant rejection in renal tissue. Vibrational spectroscopic techniques can therefore accurately identify fibrosis in a range of human tissues. Emerging data show that it can be used to quantify, classify and provide data about the nature of fibrosis with a high degree of accuracy with potential scope for point-of-care use.

External iliac artery endofibrosis in an elite female endurance cyclist / Endofibrose da artéria ilíaca externa em uma ciclista de resistência de elite

Abstract External iliac artery endofibrosis is a rare pathology that affects high-level endurance athletes, especially cyclists. Classical symptoms include pain, loss of power, and/or cramp in the affected limb while training at maximal effort. The patient's lack of atherosclerotic risk factors makes clinical suspicion of arteriopathy challenging. Moreover, the best management of such patients is still a subject of discussion. We report the case of a 36-year-old professional female endurance cyclist who presented with lower extremity pain during training. Right external iliac artery endofibrosis was diagnosed and the patient underwent surgical treatment. At two-months follow-up, she reported significant improvement in symptoms. This case highlights the importance of diagnosing peripheral vascular disease in young patients and athletes, who do not fit the ordinary profile of patients with atherosclerotic risk factors.

Resumo A endofibrose da artéria ilíaca externa é uma doença rara que afeta atletas de resistência (endurance) de nível competitivo, principalmente ciclistas. Os sintomas clássicos incluem dor, perda de força e/ou câimbras no membro afetado ao fazer esforço máximo durante o treino. A ausência de fatores de risco ateroscleróticos nesses pacientes torna a suspeita clínica de arteriopatia desafiadora. Além disso, o melhor manejo ainda é motivo de debate. Relatamos o caso de uma ciclista de resistência profissional, de 36 anos, que apresentou dor nos membros inferiores durante o treino. Foi diagnosticada endofibrose da artéria ilíaca externa, e a paciente foi submetida a tratamento cirúrgico. Após seguimento por dois meses, a paciente relatou melhora significativa dos sintomas. Este caso destaca a importância do diagnóstico de doença vascular periférica em pacientes jovens e atletas, os quais não se encaixam no perfil comum do paciente com fatores de risco ateroscleróticos.

Cannabinoid receptor-1 antagonism: a new perspective on treating a murine schistosomal liver fibrosis model

BACKGROUND Formation of schistosomal granulomata surrounding the ova can result in schistosomiasis-associated liver fibrosis (SSLF). The current standard of treatment is praziquantel (PZQ), which cannot effectively reverse SSLF. The role of the cannabinoid (CB) receptor family in liver fibrosis has recently been highlighted. OBJECTIVES This study aimed to assess the therapeutic effect of CB1 receptor antagonism in reversing SSLF in a murine model of Schistosoma mansoni infection. METHODS One hundred male Swiss albino mice were divided equally into five groups: healthy uninfected control (group I), infected control (group II), PZQ treated (group III), rimonabant (RIM) (SR141716, a CB1 receptor antagonist)-treated (group IV) and group V was treated with combined PZQ and RIM. Liver sections were obtained for histopathological examination, alpha-1 smooth muscle actin (α-SMA) immunostaining and assessment of CB1 receptor expression using real-time polymerase chain reaction (RT-PCR). FINDINGS The most effective reduction in fibrotic marker levels and granuloma load was achieved by combined treatment with PZQ+RIM (group V): CB1 receptor expression (H = 26.612, p < 0.001), number of α-SMA-positive cells (F = 57.086, p < 0.001), % hepatic portal fibrosis (F = 42.849, p < 0.001) and number of granulomata (F = 69.088, p < 0.001). MAIN CONCLUSIONS Combining PZQ with CB1 receptor antagonists yielded the best results in reversing SSLF. To our knowledge, this is the first study to test this regimen in S. mansoni infection.

Fibrosis: a distinguishing feature in the pathology of neural leprosy

BACKGROUND Fibrosis in the peripheral nerve is the end stage of leprous neuropathy and the cause of the resulting permanent neural function impairments. Preventive measures to avoid this irreversible pathological state are a relief strategy for leprosy sufferers. OBJECTIVES The present study describes the frequency of fibrosis along with its characterisation and pathogenic development. METHODS Six-hundred-and-thirteen nerve samples were sorted from 278 neural leprosy (NL) and 335 non-leprosy neuropathy patients (ON). The total number of samples was histologically examined by routine staining methods (haematoxylin-eosin, Wade staining and Gomori's trichrome) and fibrosis was evaluated via semi-quantitative estimation. FINDINGS Fibrosis was most frequent in the NL group (33% against 0.4% in ON) while fibrosis in association with endoneurial microfasciculation was found in 38 (41.3%) of the NL samples in the examination of semithin sections. Pericytic activation in the perivascular environment was confirmed to be the source of the fibroblasts and perineurial cells delimiting microfascicles. End-stage fibrosis in leprosy displays an arrangement of microfascicles devoid of neural components (i.e., Schwann cells and axons) lined by an intermediate phenotype of fibroblastic-perineurial cells filled with bundles of collagen fibres. MAIN CONCLUSIONS The present study underscores that fibrosis is frequently the severe end stage of neural leprosy NL pathogeny after analysing the notably distinct development of fibrosis within the neural environment.

Lactant de dos mesos amb nòduls plantars bilaterals / No disponible

No disponible

Reactivación de hepatitis B en paciente con hepatitis crónica C tratado con antivirales de acción directa / Hepatitis B reactivation in a patient with chronic hepatitis C treated with direct-acting antivirals

No disponible

Neumonitis por hipersensibilidad. Estudio diagnóstico menos invasivo / Hypersensitivity Pneumonitis. Towards a Less Invasive Diagnostic Procedure

No disponible

Fibrosis miocárdica y extrasistolia ventricular. Caso clínico / Myocardial fibrosis and premature ventricular contraction. A clinical case

La extrasistolia ventricular aislada es un hallazgo común en la prueba de esfuerzo de algunos deportistas. No hay evidencia científica extensa en el manejo de esta situación, particularmente cuando es un hallazgo en un individuo asintomático sin antecedentes. En este caso se presenta un deportista asintomático, sin antecedentes, de 26 años, jugador de rugby profesional, con 2 extrasístoles ventriculares en la fase de recuperación de la prueba de esfuerzo (una con imagen de bloqueo de rama derecha y la otra izquierda), pruebas complementarias cardiológicas normales (Holter, ecocardiograma doppler, ECG señal promediada, tomografía coronaria) excepto el hallazgo de fibrosis transmural del ventrículo izquierdo en la resonancia cardiaca, así como la toma de decisión en relación a la aptitud deportiva en el contexto de la escasa evidencia científica disponible en estos casos (AU)

An isolated premature ventricular contraction is a common finding in some athletes. There is no extensive scientific evidence on the management of this situation, particularly when it is a finding in an asymptomatic individual with no history. The case is presented on a 26 year-old asymptomatic athlete, a professional rugby player, with no history, who had two premature ventricular contractions in the recovery phase of the effort test (one with an image of a right bundle branch block and the other with a left block). Complementary cardiological tests (Holter, Doppler echocardiogram, ECG signal averaged ECG, and coronary tomography) were normal, except for finding a left ventricular transmural fibrosis in the cardiac resonance. There is scarce scientific evidence available for decision-making in relation to sports aptitude in the context of these cases

Placa alopécica frontoparietal / Frontal and Parietal Alopecia Plaque

No disponible

Avaliação da Função do Ventrículo Direito e Fibrose Miocárdica por Ressonância Magnética Cardíaca em Pacientes com Infarto do Miocárdio da Parede Inferior / Assessment of Right Ventricle Function and Myocardial Fibrosis by Cardiovascular Magnetic Resonance in Patients with Inferior Wall Myocardial Infarction

Fundamentos: Disfunção do ventrículo direito (VD) é observada em 30-50% dos pacientes com infarto do miocárdio na parede inferior (IM-I) e é fator preditor de mortalidade precoce. A fibrose miocárdica está associada com disfunção ventricular progressiva e prognóstico grave. Nesses pacientes, a ressonância magnética cardíaca (RMC) é um importante método de estratificação de risco. Objetivos: Avaliar a associação entre disfunção do VD e fibrose miocárdica em pacientes com IM-I por RMC. Métodos: Estudo coorte realizado em um centro de cardiologia de referência. Quarenta indivíduos com IM-I foram incluídos no estudo. RMC foi realizada durante a internação para estimar função do VD e quantificar fibrose miocárdica pela técnica de realce tardio com gadolínio (TRT). Os pacientes foram estratificados quanto à função ventricular, e características clínicas foram comparadas entre os grupos. Resultados: Quarenta pacientes foram incluídos no estudo, 75% eram homens e 43% idosos (idade ≥ 60 anos). Entre os fatores de risco cardiovasculares, hipertensão (45%) e tabagismo (33%) foram os mais prevalentes. A disfunção do VD estava presente em 33% dos pacientes. A massa de fibrose média foi 22 ± 12g nos pacientes com disfunção do VD e 15 ± 8 g nos pacientes com função ventricular preservada (p = 0,051). Conclusões: Os resultados deste estudo indicam uma possível associação entre disfunção do VD e fibrose miocárdica nos pacientes com IM-I. No entanto, outros estudos envolvendo um maior número de pacientes são necessários para confirmar nossos achados

Background: Right ventricular dysfunction (RVD) can be found in 30-50% of patients with inferior wall myocardial infarction (I-MI) and predicts early mortality. Myocardial fibrosis is associated with progressive ventricular dysfunction and severe prognosis. In these patients, cardiovascular magnetic resonance (CMR) is an important risk stratification method. Objectives: This study sought to evaluate the association between RVD and myocardial fibrosis in patients with I-MI, using CMR. Methods: Cohort study conducted in a prominent center of cardiology. Forty individuals with I-MI were included in the study. CMR was performed during hospitalization to estimate parameters of right ventricle function and to quantify myocardial fibrosis through late gadolinium enhancement (LGE) technique. Patients were stratified by ventricular function, and clinical characteristics were compared between study groups. Results: Forty patients were included in the study, 75% were male and 43% elderly (age ≥ 60 years). Hypertension (45%) and smoking (33%) were the most prevalent cardiovascular risk factors. RVD was found in 33% of patients. Mean fibrosis mass was 22 ± 12 g in patients with RVD compared with 15 ± 8 g in patients with preserved ventricular function (p = 0.051). Conclusions: The findings of our study indicate a possible association between RVD and myocardial fibrosis in patients with I-MI. However, further studies with larger series are needed to confirm our findings

Concordancia entre el resultado de la biopsia hepática y el índice APRI (Ast to Platelet Ratio Index) en el diagnóstico de cirrosis en pacientes con enfermedad hepática autoinmune / Concordance between hepatic biopsy and the APRI index (Ast to Platelet Ratio Index) for the diagnosis of cirrhosis in patients with autoimmune liver disease

Introducción y objetivos: Se ha propuesto que métodos no invasivos pueden remplazar la biopsia hepática en el diagnóstico del daño tisular en pacientes con hepatopatía autoinmune (EHA). Este estudio evalúa el rendimiento diagnóstico y grado de concordancia entre el índice Ast to Platelet Ratio Index (APRI) y la biopsia hepática en el diagnóstico de cirrosis en estos pacientes. Material y métodos: En una cohorte de pacientes con EHA se determinó el valor del índice APRI y los resultados de la biopsia hepática según la escala METAVIR. Se evaluó el área bajo la curva (AUC) y la concordancia entre un valor de APRI > 2 y un puntaje METAVIR F4 como marcadores de la presencia de cirrosis hepática mediante un estadístico de kappa. Resultados: Se incluyeron 70 pacientes (51 ± 13 años). Las hepatopatías autoinmunes más frecuentes fueron la cirrosis biliar primaria (CBP) (40%), Hepatitis autoinmune (HAI) (24,3%) y el síndrome de sobreposición HAI–CBP (32,9%). Se confirmó cirrosis por biopsia en 16 pacientes (22,9%); 15 pacientes (21,4%) presentaron índice APRI > 2 (cirrosis) y solo seis cumplieron ambos criterios. El AUC del APRI fue de 0,77 (IC 95% 0,65-0,88). La concordancia entre las pruebas fue baja para un punto de corte APRI > 2 (kappa 0,213; IC 95% 0,094 - 0,332), o para puntos de corte > 1,5, > 1 o > 0,5 (kappa 0,213, 0,255, 0,257, respectivamente). Conclusiones: Nuestros resultados sugieren que existe un pobre acuerdo entre el resultado del APRI y la biopsia hepática en el diagnóstico de cirrosis en pacientes con EHA, por lo tanto, no se debe utilizar como método diagnóstico único para determinar la presencia de cirrosis.(AU)

Introduction and objectives: It has been proposed that non-invasive methods may replace liver biopsy for the diagnosis of tissue damage in patients with autoimmune liver disease (ALD). The aim of this study was to determine diagnostic performance and degree of concordance between the APRI index and liver biopsy for diagnosing cirrhosis in these patients. Material and methods: In a cohort of patients with ALD, the value of the APRI index and liver biopsy results were determined according to the METAVIR score. The AUC and the degree of concordance between an APRI value >2 and a METAVIR score of F4 were evaluated as markers of liver cirrhosis, through a kappa statistic. Results: In total, 70 patients (age 51 ± 13 years) were included. The most common autoimmune liver diseases were primary biliary cirrhosis (PBC) (40%), autoimmune hepatitis (AIH) (24.3%) and AIH-PBC overlap syndrome (32.9%). Cirrhosis was confirmed by biopsy in 16 patients (22.9%). 15 patients (21.4%) had an APRI index >2 (Cirrhosis) and only six met both criteria. The AUC of the APRI was 0.77 (95% CI 0.65-0.88). The degree of concordance between the tests was low for an APRI cut-off point >2 (kappa 0.213; 95% CI 0.094-0.332), as well as for cut-off points >1.5, >1 and >0.5 (kappa 0.213, 0.255, 0.257, respectively). Conclusion: Our results suggest that there is little concordance between APRI and liver biopsy for the diagnosis of cirrhosis in patients with ALD. It should therefore not be used as a single diagnostic method to determine cirrhosis.(AU)

Necrosis esofágica aguda (esófago negro) / Acute oesophageal necrosis (black oesophagus)

No disponible

Técnicas actuales de reparación nerviosa / Current techniques in peripheral nerve repair

Las lesiones del sistema nervioso periférico son uno de los retos terapéuticos de nuestra especialidad, no sólo por la dificultad técnica e instrumental necesaria para resolverlas, sino por la importancia de sus secuelas y los pobres resultados obtenidos con una deficiente técnica quirúrgica. Para obtener un óptimo resultado, es preceptivo, no sólo realizar un correcto diagnóstico, sino además, conocer y dominar las distintas opciones terapéuticas. El objetivo de la presente revisión es abordar las distintas indicaciones y técnicas quirúrgicas usadas actualmente para el tratamiento de las lesiones nerviosas

The injuries of the peripheral nervous system are one of the therapeutic challenges in our speciality given their technical and instrumental difficulty, the importance of the disability and the few results obtained with a deficient surgical technique. For an optimum result, a correct diagnosis is needed as well as to know and to dominate the different therapeutics options. The objetive of the current review is to deal with the different indications and surgery techniques currently used for the treatment of the nervous injuries

Formação de membrana fibrótica cicatricial em paciente submetido à ceratoplastia penetrante / Fibrotic scar membrane formation in patients undergoing penetrating keratoplasty

RESUMO O surgimento de uma membrana fibrótica opacificada na córnea transplantada é pouco descrito nas literaturas nacional e mundial. O objetivo é relatar o caso de um paciente com leucoma total de olho esquerdo que foi submetido à ceratoplastia penetrante levando a formação de dupla câmara anterior devido ao surgimento de uma membrana fibrótica cicatricial. Paciente do sexo masculino, 54 anos, com leucoma total secundário a ceratite herpética, diabético há 20 anos, em uso de insulina, com retinopatia diabética não proliferativa. Realizou-se cirurgia de membranectomia com complicações pós-operatória.

ABSTRACT The emergence of opaque fibrotic membrane in transplanted cornea is little described in national and world literature. The goal is to report the case of a patient with leucoma total of left eye that was submitted to the penetrating keratoplasty leading to formation of double anterior chamber due to the emergence of a fibrotic scar membrane. Male patient, 54 years, with total herpetic keratitis secondary leucoma, diabetic for 20 years, using insulin, with non-proliferative diabetic retinopathy. Held membranectomia surgery with postoperative complications.

Biomarcadores, índices y algoritmos para el diagnóstico de fibrosis hepática en pacientes con hepatitis crónica por virus C / Biomarkers, indexes and algorithms for the detection of liver fibrosis in chronic hepatitis C patients

Determinar el estadio de fibrosis hepática es esencial en el manejo de la enfermedad hepática en pacientes con hepatitis crónica por virus C. La biopsia hepática ha sido considerada el gold standard para diagnosticar la enfermedad, la actividad necroinflamatoria y el estadio de fibrosis, pero tiene algunas limitaciones técnicas y riesgos. Teniendo en cuenta estas limitaciones, existe cierta exigencia en introducir biomarcadores séricos no invasivos para el diagnóstico de fibrosis que podrían ser capaces de reemplazar parcialmente a la biopsia hepática. El biomarcador sérico ideal debería ser específico para el hígado y fácil de determinar. Los marcadores séricos se dividen en directos e indirectos. Los directos reflejan el recambio de la matriz extracelular, mientras que los indirectos reflejan alteraciones en la función hepática. Aunque todavía está limitado el grado de implementación de los test no invasivos de fibrosis hepática, esta revisión es una visión de conjunto de los biomarcadores, índices y algoritmos diagnósticos de fibrosis hepática estudiados en hepatitis crónica C pero con un interés potencial en otras hepatopatías crónicas (AU)

Precise staging of liver fibrosis is essential in the management of liver disease activity in chronic hepatitis C patients. Liver biopsy has been considered the reference method for diagnosing disease, grading necroinflammatory activity, and staging fibrosis, but it has some technical limitations and risks. Taking into account these limitations, there is a need to introduce non-invasive serum markers for fibrosis that would be able to partially replace liver biopsy. Ideal serum markers should be specific for the liver and easy to perform. Serum markers of hepatic fibrosis are divided into direct and indirect. Direct markers reflect extracellular matrix turnover, whereas indirect markers reflect alterations in hepatic function. The degree of implementation of non-invasive diagnostic tests for liver fibrosis still remains limited. This review provides a current overview of biomarkers, indexes and algorithms for hepatic fibrosis diagnosis in chronic hepatitis C patients (AU)

La criobiopsia transbronquial en la enfermedad pulmonar intersticial: excelente relación coste/beneficio / No disponible

No disponible

Últimos avances en pancreatitis crónica / Latest advances in chronic pancreatitis

Los avances más recientes con impacto en la práctica clínica de los pacientes con pancreatitis crónica se centran en el diagnóstico precoz de la enfermedad, la predicción del grado de fibrosis del páncreas, la evaluación de los pacientes con elevación asintomática de los valores séricos de enzimas pancreáticas, el tratamiento quirúrgico y farmacológico del dolor y el conocimiento de la historia natural de la pancreatitis autoinmune. En pacientes con hallazgos ecoendoscópicos indeterminados para pancreatitis crónica, la repetición de la ecoendoscopia en el seguimiento permite confirmar o excluir el diagnóstico. El hábito tabáquico, las reagudizaciones, los resultados de las pruebas funcionales pancreáticas y los hallazgos ecoendoscópicos permiten predecir el grado de fibrosis en pancreatitis crónica. En el tratamiento del dolor, la administración oral de antioxidantes parece ser eficaz, aunque queda por demostrar su combinación y dosis óptima. El desarrollo de sobrecrecimiento bacteriano es muy frecuente en pacientes con pancreatitis crónica, aunque su impacto sobre los síntomas es desconocido. Por último, la pancreatitis autoinmune recidiva en alrededor de la mitad de los pacientes y lo hace habitualmente en los primeros 2 años de seguimiento, sin diferencias entre tipos 1 y 2

This article summarizes some of the recent and clinically relevant advances in chronic pancreatitis. These advances mainly concern the early diagnosis of the disease, the prediction of the fibrosis degree of the gland, the evaluation of patients with asymptomatic hyperenzimemia, the medical and surgical treatment of abdominal pain and the knowledge of the natural history of the autoimmune pancreatitis. In patients with indetermined EUS findings of chronic pancreatitis, a new endoscopic ultrasound examination in the follow-up is of help to confirm or to exclude the disease. Smoking, number of relapses, results of pancreatic function tests and EUS findings allow predicting the degree of pancreatic fibrosis in patients with chronic pancreatitis. Antioxidant therapy has shown to be effective in reducing pain secondary to chronic pancreatitis, although the type and optimal dose of antioxidants remains to be elucidated. Development of intestinal bacterial overgrowth is frequent in patients with chronic pancreatitis, but its impact on symptoms is unknown and deserves further investigations. Finally, autoimmune pancreatitis relapses in about half of the patients with either type 1 or type 2 disease; relapses frequently occur within the first two years of follow-up