[Secondary amyloidosis of the bladder and massive hematuria]. / Amiloidosis vesical secundaria y hematuria masiva.

OBJECTIVE: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. MATERIALS AND METHODS: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. RESULTS: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massive, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor. CONCLUSIONS: Despite its rarity, as shown by the few cases reported, secondary amyloidosis of the bladder should be considered in patients already diagnosed with systemic amyloidosis and/or the conditions reported who require simple urethral catheterization.

Amiloidosis vesical secundaria y hematuria masiva / Secondary amyloidosis of the bladder and massive hematuria

Objetivo: Dar a conocer cuatro nuevos casos de amiloidosis vesical secundaria, cuya manifestación clínica es extraordinariamente rara, si atendemos a los escasos casos publicados que no llegan a la treintena. Material y métodos: Describimos los cuatro casos clínicos, manifestados todos ellos por hematuria, siendo en tres masiva y fulminante, con evolución fatal. Resultados: La amiloidosis vesical secundaria corresponde al tipo AA, más frecuente en mujeres y secundaria sobre todo a artritis reumatoide, pero también a espondilitis anquilopoyética y procesos inflamatorios crónicos de larga evolución. La hematuria es el síntoma fundamental, prácticamente único. El estudio patológico e inmunohistoquímico confirma el diagnóstico. Se da la circunstancia de que los tres casos de hematuria masiva y fatal presentaron una patología intercurrente que precisó practicar un sondaje uretral, siendo este el desencadenante. Conclusiones: A pesar de su rareza, confirmada por los pocos casos publicados, habrá que pensar en ella ante pacientes ya diagnosticados de amiloidosis sistémica y/o con las patologías descritas, que precisen un simple sondaje uretral(AU)

Objective: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. Materials and methods: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. Results: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massiva, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor. Objective: To report four additional cases of secondary amyloidosis of the bladder, an extremely rare condition, as shown by the cases reported in the literature. Materials and methods: Four clinical cases are reported, all of them occurring as hematuria, which was massive and fulminant and resulted in death in three patients. Results: Secondary amyloidosis of the bladder is of the AA type, which is more common in females and mainly secondary to rheumatoid arthritis, but also to ankylosing spondylitis and long-standing chronic inflammatory conditions. Hematuria is the main and virtually only symptom. A pathological and immunohistochemical study confirmed diagnosis. All three patients who experienced massiva, fatal hematuria had an intercurrent condition requiring urethral catheterization, which was the triggering factor(AU)

Manejo del síndrome aspirativo recurrente en el paciente pediátrico / Treatment of recurrent aspiration syndrome in children

El síndrome aspirativo recurrente es una causa importante de patología broncopulmonar. En su etiopatogenia intervienen alteraciones de la deglución, la presencia de reflujo gastroesofágico y la aspiración de saliva. Debido a su presentación clínica variable y a la inexistencia de criterios diagnósticos específicos, frecuentemente pasa desapercibido. El tratamiento debe plantearse en función del mecanismo implicado, evaluando los posibles factores predisponentes en cada paciente. Presentamos el caso de una niña que fue remitida a la consulta de neumología tras sufrir episodios respiratorios de repetición, con una inadecuada respuesta al tratamiento. Se hace una revisión exhaustiva de esta patología en el contexto pediátrico con el fin de definir una norma de actuación diagnóstica adecuada, evitar la realización de pruebas complementarias excesivas e inapropiadas y llevar a cabo una terapia dirigida y fundamentada (AU)

Recurrent aspiration syndrome is an important cause of bronchopulmonary pathology. Its etiopathogenesis involves wallowing alterations, the presence of gastroesophageal reflux and the aspiration of saliva. Because of its variable clinical presentation and the absence of specific diagnostic criteria, it often goes unnoticed. The treatment should be determined according to the mechanism involved, thereby evaluating the potential predisposing factors in each patient. We present the case of a girl who is referred to the Pneumology Department because of recurrent respiratory episodes and a poor response to the treatment. An in-depth review is conducted of this pathology in the field of paediatrics, with the aim of defining rules for adequate diagnostic action, there by trying to avoid excessive and inappropriate additional tests, and providing a targeted and justified therapy (AU)

Patología respiratoria en pacientes con enfermedad de células falciformes. Protocolo de actuación (I) / Respiratory disorders in patients with sickle cell disease: management protocol (I)

La drepanocitosis es una hemoglobinopatía que produce rigidez del eritrocito en situaciones de hipoxia, infección o deshidratación, con la consecuente oclusión microvascular y hemólisis. Es una enfermedad que afecta a múltiples órganos y sistemas, como el bazo, el sistema nervioso central, el aparato osteoarticular, los ojos, el sistema genitourinario y el aparato respiratorio. La patología respiratoria, tanto aguda como crónica, es una causa importante de morbimortalidad, aunque las crisis dolorosas afectan más a la calidad de vida de estos pacientes. Vamos a desarrollar a lo largo de este artículo las diferentes complicaciones respiratorias, con el fin de realizar una prevención adecuada y efectuar un tratamiento precoz y agresivo de ellas. También describiremos brevemente el resto de situaciones que pueden aparecer durante la evolución de la enfermedad y expondremos esquemáticamente el programa de control y seguimiento del paciente con enfermedad de células falciformes. Se resalta también el interés de la puesta en marcha de grupos de trabajo especializados en esta patología aún poco conocida en nuestro medio, aunque se está incrementando su detección debido a la inmigración y a la realización del cribado neonatal universal en algunas comunidades autónomas, lo que obliga a la elaboración de un plan de actuación mediante la creación de unidades multidisciplinarias que van a mejorar sobremanera la supervivencia y la calidad de vida de estos pacientes (AU)

Sickle-cell disease is a hemoglobinopathy that produces rigidity in red cells under conditions of hypoxia, infection or dehydration, with the subsequent microvascular occlusion and hemolysis. It is a disease that affects multiple organs and systems, such as the spleen, the central nervous system, the osteoarticular system, the eyes, the genitourinary tract and the respiratory tract. Respiratory diseases, both acute and chronic, are a major cause of morbidity and mortality, although painful crises have a greater impact on the quality of life of these patients. In this article, we will elaborate upon the various respiratory complications for the purpose of introducing proper preventive measures and initiating an early and aggressive treatment of these complications. We will also briefly describe other situations that may arise throughout the course of the disease and briefly describe a program for the control and monitoring ofpatients with sickle cell disease. This article will also highlight the interest in establishing specialized workgroups in this disease. Although it is still not very widespread among our general population, it is being detected with increasing frequency due to immigration, and due to the universal newborn screening in some autonomous regions, which calls for the development of a plan of action through the creation of multidisciplinary units that will greatly improve the survival and quality of life of these patients (AU)