Persistent dysphonia and constitutional syndrome. Laryngeal amyloidosis

Objetivo: describir un caso de amiloidosis laríngea que se define como una enfermedad por depósito de una determinada proteína, cuyo tratamiento si el diagnóstico no es precoz, puede ser muy incapacitante. Método: presentamos el caso de un paciente de 64 años con disfonía de años de evolución, su diagnóstico fue casual, pues la paciente nunca interpretó el cambio de su voz como patológico. Resultado: comentó pérdida de peso no cuantificada en los últimos meses. Se deriva a ORL que tras laringoscopia se aprecia aspecto granuloso en vestíbulo laríngeo por encima de las bandas ventriculares, sin aspecto neoplásico, pero que precisa biopsia, y posterior laringuectomía por hemorragia. Conclusiones: en la amiloidosis es esencial el diagnóstico precoz. El tratamiento debe ser individualizado, por lo que en estos pacientes son fundamentales el tratamiento de soporte y el manejo multidisciplinar, en especial en pacientes con afectación renal o cardiaca.Purpose: describe a case of laryngeal amyloidosis that is defined as a disease due to the deposition of a certain protein, whose treatment if the diagnosis is not early, can be very disabling. Method: here we present the case of a 64 year old patient with dysphonia of years of evolution, his diagnosis was casual, because the patient never interpreted the change of his voice as pathological. Results: commented on unquantified weight loss in recent months. ENT specialist is derived that after laryngoscopy a granular appearance is seen in the laryngeal vestibule above the ventricular bands, without neoplastic appearance, but that requires a biopsy, and subsequent laryngectomy due to hemorrhage. Conclusions: in early amyloidosis, early diagnosis is essential. The treatment must be individualized, so in these patients, supportive treatment and multidisciplinary management are fundamental, especially in patients with renal or cardiac involvement

Propranolol, simultaneous therapeutic effect in a patient. Is there a polypill?

Ante la presencia de pacientes con diferentes problemas clínicos, el reto para el médico es minimizar al máximo el número de fármacos para conseguir un adecuado cumplimiento terapéutico. Diariamente surgen en el mercado nuevas alternativas farmacológicas que son capaces de conseguir un efecto terapéutico más potente y duradero, reduciendo a su vez los efectos adversos de sus predecesores. Propranolol, es un fármaco ampliamente conocido y empleado para el tratamiento de diversas patologías. La aparición de nuevas opciones en la prevención y tratamiento de eventos cardiovasculares ha disminuido su prescripción en los últimos años y ha favorecido su sustitución en pacientes estables. Como sabemos, es importante hacer una valoración individualizada e integral del paciente ante cualquier modificación de tratamiento. Presentamos el caso clínico de una paciente con múltiples patologías potencialmente graves en la que la suspensión del tratamiento con propranolol fue el desencadenante de la descompensación de su patología de base.In patients with different clinical problems, the challenge for the physician is to minimize number of drugs to achieve adequate therapeutic compliance. New pharmacological alternatives are emerging in the market that are capable of achieving a more potent and lasting therapeutic effect, while reducing the adverse effects of its predecessors. Propranolol, is a drug widely known and used for the treatment of various pathologies. The emergence of new options in the prevention and treatment of cardiovascular events has decreased its prescription in the last years and has favored its substitution in stable patients. As we know, it is important to make an individualized and comprehensive assessment of the patient before any treatment modification. We present the case of a patient with multiple potentially serious pathologies in which the suspension of the treatment with propranolol was the trigger of the decompensation of its pathology

Fast Track Algorithm: How To Differentiate A “Scleroderma Pattern” From A “Non-Scleroderma Pattern”

Objectives: This study was designed to propose a simple “Fast Track algorithm” for capillaroscopists of any level of experience to differentiate “scleroderma patterns” from “non-scleroderma patterns” on capillaroscopy and to assess its inter-rater reliability. Methods: Based on existing definitions to categorise capillaroscopic images as “scleroderma patterns” and taking into account the real life variability of capillaroscopic images described standardly according to the European League Against Rheumatism (EULAR) Study Group on Microcirculation in Rheumatic Diseases, a fast track decision tree, the “Fast Track algorithm” was created by the principal expert (VS) to facilitate swift categorisation of an image as “non-scleroderma pattern (category 1)” or “scleroderma pattern (category 2)”. Mean inter-rater reliability between all raters (experts/attendees) of the 8th EULAR course on capillaroscopy in Rheumatic Diseases (Genoa, 2018) and, as external validation, of the 8th European Scleroderma Trials and Research group (EUSTAR) course on systemic sclerosis (SSc) (Nijmegen, 2019) versus the principal expert, as well as reliability between the rater pairs themselves was assessed by mean Cohen's and Light's kappa coefficients. Results: Mean Cohen's kappa was 1/0.96 (95% CI 0.95-0.98) for the 6 experts/135 attendees of the 8th EULAR capillaroscopy course and 1/0.94 (95% CI 0.92-0.96) for the 3 experts/85 attendees of the 8th EUSTAR SSc course. Light's kappa was 1/0.92 at the 8th EULAR capillaroscopy course, and 1/0.87 at the 8th EUSTAR SSc course. C Conclusion: For the first time, a clinical expert based fast track decision algorithm has been developed to differentiate a “non-scleroderma” from a “scleroderma pattern” on capillaroscopic images, demonstrating excellent reliability when applied by capillaroscopists with varying levels of expertise versus the principal expert and corroborated with external validation.Wo