The effect of a training webinar on decreasing inter-observer variability in making a radiologic diagnosis of bronchiectasis.

Non-cystic fibrosis bronchiectasis is a clinically important disease with an estimated 340,000-522,000 persons living with the disease and 70,000 being diagnosed annually. The radiographic diagnosis remains a pivotal part of recognizing the disease due to its protean clinical manifestations. As physicians are sensitized to this disease, a greater proportion of patients are being diagnosed with mild to moderate bronchiectasis. Despite the established use of CT chest as the main tool for making a radiologic diagnosis of bronchiectasis, the literature supporting the process of making that diagnosis is somewhat sparse. Concurrently, there has been an increased trend to have Web-based radiologic tutorials due to its convenience, the ability of the learner to set the pace of learning and the reduced cost compared to in-person learning. The COVID-19 pandemic has accelerated this trend. We wanted to look carefully at the effect of a Web-based training session on interrater reliability. Agreement was calculated as percentages and kappa and prevalence adjusted kappa calculated. We found that a single Web-based training session had little effect on the variability and accuracy of diagnosis of bronchiectasis. Larger studies are needed in this area with multiple training sessions.

Syringomatous adenoma of the nipple with microcalcifications on mammography: A case report.

Syringomatous adenoma of the nipple (SAN) is a rare benign lesion which often presents as a subareolar mass with associated nipple shape changes including pruritus, crusting, and discharge It is thought that syringomatous tumors arise from eccrine glands of the nipple and areola. Due to its locally infiltrative growth pattern the main differential is with low-grade adenosquamous carcinoma which requires more aggressive surgical treatment. Syringomatous adenoma of the nipple can recur, and therefore complete local excision is recommended. We present a case of a syringomatous adenoma of the nipple diagnosed after nearly 5 years of observation with microcalcifications noted on mammography.

Apendicitis aguda perforada con diagnóstico prequirúrgico y hernia inguinal bilateral en recién nacido de pretérmino / Acute perforated appendicitis with pre-surgical diagnosis and bilateral inguinal hernia in preterm infants

INTRODUCCIÓN: La apendicitis aguda neonatal es una patología de muy baja frecuencia, con escasos reportes en los últimos 30 años. La clínica e imágenes son inespecíficas, diagnosticándose la mayoría de las veces durante el procedimiento quirúrgico. OBJETIVO: Presentar un caso de apendicitis aguda perfora da en paciente prematura, con diagnóstico prequirúrgico y resolución laparoscópica, asociada a aparición posterior de hernia inguinal. CASO CLÍNICO: Recién nacido femenino de 34 semanas que a los 17 días de vida presentó fiebre, irritabilidad y aumento del residuo lácteo. Al examen físico destacaba distensión y sensibilidad del abdomen y tanto la radiografía como ecografía abdominales eran compatibles con apendicitis aguda perforada, por lo cual ingresó a pabellón para laparoscopía. Confirmado el diagnóstico, se realizó apendicectomía. Dos semanas después en consulta ambulatoria, se evidenció y confirmó con ecografía una hernia inguinal bilateral que se corrigió quirúrgicamente. CONCLUSIÓN: La apendicitis aguda debe considerarse dentro de los diagnósticos diferenciales en neonatos con clínica de abdomen agudo, principalmente en prematuros. En este caso, la ecografía permitió realizar el diagnóstico pre-operatorio. La posible asociación con hernia inguinal, debiera motivar a examinar siempre conductos inguinales durante la evaluación con imá genes y en el procedimiento quirúrgico.

INTRODUCTION: Acute neonatal appendicitis is a rare pathology, with few reports in the last 30 years. Since its clinical presentation and imaging studies are non-specific, most cases are diagnosed during a surgical pro cedure. OBJECTIVE: To describe a neonatal case of acute perforated appendicitis associated with later appearance of inguinal hernia, with pre-surgical diagnosis and treated through laparoscopy. CLINICAL CASE: A 17-day-old preterm female newborn presented with fever, irritability, and increased milk intolerance. Physical examination showed abdominal distention, tenderness and both, abdominal X- ray and ultrasound showed compatible images with acute perforated appendicitis. Once the diagnosis was confirmed, we performed an appendicectomy through laparoscopy. Two weeks later, during an outpatient visit, we observed a bilateral inguinal hernia which was confirmed by ultrasound, and then it was surgically corrected. CONCLUSION: Acute appendicitis should be considered within the differential diagnosis in neonates with acute abdominal symptoms, mainly in premature infants. In this case, ultrasound scan allowed us to make the pre-operative diagnosis. The possible association with inguinal hernias should motivate to examine inguinal ducts during imaging assessment and surgical procedure.

Benefits of prolonged-release pirfenidone plus standard of care treatment in patients with advanced liver fibrosis: PROMETEO study.

BACKGROUND AND AIMS: Pirfenidone (PFD), an oral antifibrotic drug, has been authorized by the EMA and FDA for treatment of idiopathic pulmonary fibrosis. Few studies have addressed its use in advanced liver fibrosis (ALF). We evaluated a prolonged-release formulation (PR-PFD) plus standard of care on disease progression in ALF. METHODS: 281 ALF patients from 12 centers receiving PR-PFD (600 mg bid) were screened; 122 completed 1 year of treatment. Additionally, 74 patients received only standard of care regimen. Average age was 64 ± 12 years, 58% female. 43.5% had fatty liver disease (NAFLD), 22.5% viral hepatitis C (VHC), 17% autoimmune hepatitis (AIH), and 17% alcoholic liver disease (ALD). Baseline fibrosis was F4 in 74% and F3 in 26%. Antifibrotic effects were assessed by transient elastography (Fibroscan®) and Fibro Test® (FT); Cytokines and PFD plasma levels were tracked and quality of life evaluated. RESULTS: We found a significant reduction in fibrosis in 35% of PR-PFD patients and only in 4.1% in non PR-PFD patients. Child-Pugh score improved in 29.7%. Biochemical values remained stable; 40.6% and 43.3% decreased ALT or AST, respectively. TGFß1 (pg/mL) levels were lower in PFD-treated patients. PFD serum concentration (µg/mL) was higher (8.2 ± 1.7) in fibrosis regression profile (FRP) patients compared to fibrosis progression profile (FPP) patients (4.7 ± 0.3 µg/mL, p < 0.01). 12% reported transient burning or nausea and 7% photosensitivity. Quality of life (Euro-Qol scale) improved from 62 ± 5 to 84 ± 3 (p < 0.001) and from 32 ± 3 to 42 ± 2 (p < 0.008) (FACIT scale). CONCLUSIONS: PR-PFD is efficacious and safe in ALF and associated with promising antifibrotic effects. TRIAL REGISTRATION: Clinical trial number: NCT04099407.

Recomendaciones para el uso de la hormona de crecimiento humana recombinante en pacientes pediátricos de talla baja en Colombia / Recommendations for treatment with recombinant human growth hormone in pediatric patients in Colombia

resumen está disponible en el texto completo

Abstract In Colombia there are no guidelines for diagnosis and management of patients with short stature and for the use of recombinant human growth hormone, mainly caused by the diversity of training centers in pediatric endocrinology. In response to this situation, the Asociación Colegio Colombiana de Endocrinología Pediátrica leds the first colombian short stature expert committee in order to standardize the use of human recombinant growth hormone. This work had the participation and endorsement of a consortium of clinical experts representing the Sociedad Colombiana de Pediatría, Secretaría Distrital de Salud de Bogotá- Subred Integrada de Servicios de Salud Suroccidente, Fundación Universitaria Sanitas, Universidad de los Andes and some public and private health institutions in the country, in addition to the participation of methodological experts from the Instituto Global de Excelencia Clínica Keralty. By reviewing the literature and with the best available evidence, we proposed to unify definitions, a diagnostic algorithm, biochemical and dynamic tests with their reference parameters, a description of the considerations about growth hormone use among the indications approved by regulatory agency for medications and food in Colombia and finally a proposal for an informed consent and a medication fact sheet available for parents and patients.

[Acute perforated appendicitis with pre-surgical diagnosis and bilateral inguinal hernia in preterm infants]. / Apendicitis aguda perforada con diagnóstico prequirúrgico y hernia inguinal bilateral en recién nacido de pretérmino.

INTRODUCTION: Acute neonatal appendicitis is a rare pathology, with few reports in the last 30 years. Since its clinical presentation and imaging studies are non-specific, most cases are diagnosed during a surgical pro cedure. OBJECTIVE: To describe a neonatal case of acute perforated appendicitis associated with later appearance of inguinal hernia, with pre-surgical diagnosis and treated through laparoscopy. CLINICAL CASE: A 17-day-old preterm female newborn presented with fever, irritability, and increased milk intolerance. Physical examination showed abdominal distention, tenderness and both, abdominal X- ray and ultrasound showed compatible images with acute perforated appendicitis. Once the diagnosis was confirmed, we performed an appendicectomy through laparoscopy. Two weeks later, during an outpatient visit, we observed a bilateral inguinal hernia which was confirmed by ultrasound, and then it was surgically corrected. CONCLUSION: Acute appendicitis should be considered within the differential diagnosis in neonates with acute abdominal symptoms, mainly in premature infants. In this case, ultrasound scan allowed us to make the pre-operative diagnosis. The possible association with inguinal hernias should motivate to examine inguinal ducts during imaging assessment and surgical procedure.

Deficiencia de 3-beta-hidroxiesteroide deshidrogenasa: una causa rara de hiperplasia adrenal congénita / 3-beta-hydroxysteroid dehydrogenase deficiency: a rare cause of congenital adrenal hyperplasia

Resumen: la hiperplasia adrenal congénita corresponde a un grupo de enfermedades heredadas con defectos enzimáticos que pueden comprometer la biosíntesis del cortisol. La deficiencia de la enzima 3β-hidroxiesteroide deshidrogenasa tipo 2 es una causa rara de este defecto en la que el desarrollo genital masculino se encuentra alterado y presenta una virilización leve en las mujeres afectadas. En humanos se han descrito dos isoenzimas, la tipo I y la tipo II, codificadas por los genes HSD3B1 y HSD3B2, respectivamente, con una distribución tisular específica.Los programas de tamización de la hiperplasia adrenal congénita reportan elevación paradójica de la 17-hidroxiprogesterona secundaria al efecto periférico de la 3β-hidroxiesteroide deshidrogenasa tipo 1, isoenzima de la 3β-hidroxiesteroide deshidrogenasa tipo 2, que tiene una constante de Michaelis menor con el sustrato.A pesar de la baja prevalencia el estudio de este defecto ha tenido importantes avances en cuanto a la información molecular y el diagnóstico hormonal, datos que han sido respaldados por la identificación de la alteración genética y han disminuido la posibilidad del sobrediagnóstico; evento que se estaba presentado frecuentemente con los puntos de cortes establecidos inicialmente para el diagnósticode la enfermedad, sobre todo en sus formas leves.

Abstract: the congenital adrenal hyperplasia corresponds to a group of inherited diseases with enzyme defects that alter the cortisol biosynthesis. The 3β-hydroxysteroid dehydrogenase type 2 deficiency is a rare cause of this defect, where the male genital development is altered but little virilization in affected women is present. In humans two isoenzymes have been described, type I and type II, coded by HSD3B2 and HSD3B1 genes, respectively, and with specific tissuedistribution. The screening programs to congenital adrenal hyperplasia report paradoxical elevation of 17-hydroxyprogesterone secondary to peripheral effect of 3β-hydroxysteroid dehydrogenase type 1, an isoenzyme of 3β-hydroxysteroid dehydrogenase type 2. Type 1 has a lower Michaelis constant with the substrate; additional condition that relates with the paradoxical effect of the 17-hydroxyprogesterone.Besides the low prevalence, the study of this defect has had important progress about molecular information and hormonal diagnosis, data that has been confirmed with the identification of genetic alteration in the described gene, reducing the possibility of overdiagnosis; an event that was showing frequently with the initially cut-point stablished especially for milder forms of the disease.

Control de calidad de la leucorredución de plaquetas obtenidas por aféresis por medio de cámara de Nageotte y CELL-DYN Ruby / Quality control of the leucorredución of platelets obtained by apheresis through Nageotte chamber and CELL-DIN Ruby

Comparar por medio de dos metodologías diferentes el conteo residual de glóbulos blancos con el fin de brindar componentes sanguíneos más seguros, Los componentes sanguíneos obtenidos con aféresis ofrecen mayor seguridad a los pacientes en el momento de transfusión sanguínea; la verificación de la leucorreducción es un proceso de calidad diferenciador, Materiales y métodos: Se realizó un estudio de corte transversal descriptivo en la Fundación Hematológica Colombia, Bogotá, Se realizó un muestreo aleatorio simple a partir del listado de unidades ingresadas en una colecta de sangre con el fin de realizar la verificación del recuento de glóbulos blancos residuales por cámara de Nageotte© y Cell Dyn Ruby©, Para el análisis de los datos se aplicaron medidas de tendencia central y de dispersión para las variables cuantitativas, (IC 95%) y correlación de Spearman para el análisis de procedimientos satisfactorios (Prueba T); el análisis de los datos se realizó en el programa SPSS©de IBM Versión 19. Resultados: La población de estudio estuvo conformada por 124 muestras de plaquetas obtenidas por aféresis, En relación al recuento de células las dos metodologías presentaron un promedio de 0,057 vs 0,003 células contadas; el valor máximo para las dos metodologías fue de 0,34 – 0,44, Conclusiones: Las muestras analizadas presentaron recuentos residuales de Glóbulos Blancos por debajo de 5,0x 106 valor estimado para definir la unidad como leucorreducida, con este hallazgo se pone en evidencia la seguridad de la transfusión de plaquetas obtenidas por aféresisen aspectos relacionados con reacciones a la transfusión por leucocitos residuales,adicionalmente se podría utilizar plaquetas con seguridad dejando en consideración el uso de filtros para leucorredución...

Caracterización de la población con carcinoma basocelular en el Centro Dermatológico Federico Lleras Acosta / Characteristics of patients with basal cell carcinoma in Centro Dermatológico Federico Lleras Acosta

Introducción: el cáncer de piel no melanoma es el tumor maligno más frecuente en el humano. De éstos, el 80 % corresponde a carcinomas basocelulares. Objetivo: caracterizar la población de los pacientes con carcinoma basocelular que consultaron al Centro Dermatológico Federico Lleras Acosta. Métodos: estudio descriptivo de todos los casos confirmados histológicamente de carcinoma basocelular en un periodo de tres años. Se obtuvo información acerca de las características socio-demográficas, subtipos clínicos e histológicos y los tiempos transcurridos desde la detección de la lesión por parte del paciente y la confirmación histológica del tumor, hasta la fecha de realización del tratamiento. Resultados: el estudio incluyó un total de 769 casos de carcinoma basocelular. El 75 % de los tumores se localizaron en la cara, el cuello y la piel cabelluda. El subtipo clínico e histológico más frecuente fue el nodular (67 % y 57 %, respectivamente), los subtipos histológicos mixtos fueron el 28%, y de estos el 75% correspondian a histologías de alto riesgo de recidiva tumoral. El tiempo transcurrido entre la detección de la lesión por parte del paciente y la consulta por primera vez a un servicio de salud fue de 28 meses. El tiempo transcurrido entre la toma de la biopsia y el tratamiento definitivo fue de un mes. Dentro de los tratamientos sugeridos, el más frecuente fue la cirugía convencional (89 %), seguida de la cirugía micrográfica de Mohs (7 %). Conclusiones: el subtipo clínico e histológico de carcinoma basocelular más frecuente fue el nodular. La mayoría de subtipos histológicos mixtos eran de alto riesgo para recidiva tumoral, lo cual refuerza la importancia de una buena aproximación clínica y la toma de biopsia antes de elegir el tratamiento del tumor. Los pacientes con carcinomas basocelular tienden a consultar tardíamente a su servicio de salud, lo que retarda el diagnóstico y el tratamiento.

ABSTRACT Introduction: non melanoma skin cancer is the most frequent malignant tumor in humans, 80% of these tumors are basal cell carcinomas (BCC). Objectives: To characterize the population of patients with BCC, who consulted at Centro Dermatologico Federico Lleras Acosta in a time lapse of 3 years. Methods: we performed a descriptive study of all the BCC cases confirmed by biopsy, within a 3 years period. Information was obtained related to the socio-demographic characteristics, clinical and histological subtypes, and the time elapsed since the patient's detection of their lesion and histological confirmation of the tumor, until the definitive treatment was performed. Results: A total of 769 BCC cases were included in the study, 75% of the lesions were located on the face, neck and scalp. The most frequent clinical and histological subtype was nodular BCC (67 and 57% respectively), mixed histological subtypes were seen in 28% of the cases, 75% of them showed high risk features of tumor relapse. The time elapsed between the detection of the lesion by the patient and the first time consultation to a health service was 28 months. The time elapsed between the biopsy and the definitive treatment was 1 month. The most frequent treatment was conventional surgery (89%) followed by Mohs micrographic surgery (7%). Conclusions: The most frequent clinical and histological subtype was nodular BCC, mixed histological subtypes showed high risk features of tumor relapse in more than 50%, this confirm the importance to suspect a BCC and take a skin biopsy before treat the tumor. BCC patients tend to consult late to health services, with the consequent delay in the diagnosis and treatment.

Flexibility of motor pattern generation across stimulation conditions by the neonatal rat spinal cord.

Previous studies have demonstrated that "locomotor-like" rhythmic patterns can be evoked in the isolated neonatal rat spinal cord by several means, including pharmacological neuromodulation and electrical stimulation of various pathways. Recent studies have used stimulation of afferent pathways to evoke rhythmic patterns, relying on synaptic activation of interneuronal systems rather than global imposition of neuromodulatory state by pharmacological agents. We use the in vitro neonatal rat spinal cord with attached hindlimb to examine the muscle activation patterns evoked by stimulation of these different pathways and evaluate whether stimulation of these pathways all evoke the same patterns. We find that the patterns evoked by bath application of serotonin (5-HT) and N-methyl-D-aspartic acid (NMDA) consisted of alternation between hip flexors and extensors and similar alternation was observed in the patterns evoked by electrical stimulation of the cauda equina (CE) or contralateral fifth lumbar (L(5)) dorsal nerve root. In contrast, the knee extensor/hip flexor rectus femoris (RF) and knee flexor/hip extensor semitendinosus (ST) were activated differentially across stimulation conditions. In 5-HT/NMDA patterns, RF was active in late flexion and ST in late extension. In CE patterns, these two muscles switched places with RF typically active in late extension and ST active in flexion. In L(5) patterns, ST was activated in extension and RF was silent or weakly active during flexion. There were also systematic differences in the consistency of rhythms evoked by each stimulation method: patterns evoked by electrical stimulation of CE or L(5) were less consistently modulated with the rhythm when compared with 5-HT/NMDA-evoked patterns. All differences were preserved following deafferentation, demonstrating that they reflect intrinsic properties of spinal systems. These results highlight the intrinsic flexibility of motor pattern generation by spinal motor circuitry which is present from birth and provides important information to many studies examining spinal pattern generating networks.