Hemangioendotelioma epitelioide hepático diseminado: a propósito de un caso / A case of hepatic epithelioid hemangioendo thelioma

Introducción: el hemangioendotelioma epiteloide es un tumor mesenquinal de causa desconocida y en general diseminado al diagnóstico. Se describen alrededor de 400 casos en el mundo a la fecha. Material y método: se presenta el caso de una paciente joven que consulta por dolor en hipocondrio derecho constatándose hepatomegalia y esplenomegalia tumoral que seconfirman imagenológicamente y lesión lítica costal y vertebral. La biopsia hepática demuestra hemangioendotelioma epiteloide de alto grado. Se realiza revisión bibliográficadel tema, particularmente su presentación hepática. Resultados: se inicia radioterapia analgésica de columna, la paciente presenta un rápidodeterioro del estado general y fallece de sepsis de origen respiratorio. Conclusión: el hemangioendotelioma epiteloide debe ser considerado en el diagnóstico diferencial de los pacientes en estudio por tumoración hepática y es la cirugía el tratamiento de elección.

Introduction: epitheloid hemangiothelioma is a mesenchymaltumour of unknown etiology and typically disseminated for diagnosis. Until today, 400 cases have beendescribed around the world. Method: the case of a young woman who consultedfor pain in the right hypochondrium is presented. She presented tumoral hepatomegaly and splenomegaly that were imagenologically confirmed, and costal and vertebral lytic lesion. Hepatic biopsy shows high-grade epitheloidhemangiothelioma. A bibliographic review of the conditions is conducted, in particular regarding its hepatic presentation. Results: radiotherapy to the spine for pain was initiated and the patient presented a rapid deterioration of her general condition and died as a result of sepsis of respiratory origin.Conclusion: epitheloid emangiothelioma must be considered in the differential diagnosis for patients seen for hepatic tumors, and surgery is the best choice of treatment.

Introdução: o hemangioendotelioma epitelióide é um tumor mesenquimal de etiologia desconhecida geralmentedisseminado no momento de seu diagnóstico. Cerca de 400 casos foram descritos no mundo até o momento.Material e método: apresenta-se o caso de uma paciente jovem que consulta por dor no hipocôndrio direito constatando-se hepatomegalia e esplenomegalia tumoral confirmadas imagenológicamente e lesão lítica costal e vertebral. A biópsia hepática mostra hemangioendotelioma epitelióide de alto grau. Faz-se uma revisão da bibliografia sobre o tema, especialmente sobre a apresentação hepática. Resultados: a paciente foi submetida a radioterapiaanalgésica de coluna, mas apresentou um rápido deterioro do estado general e falece por sepse de origem respiratória.Conclusão: o hemangioendotelioma epitelióide deve ser considerado no diagnóstico diferencial de pacientesestudados por tumoraçao hepática sendo a cirurgia o tratamento de escolha.

Prevalence and characteristics of immunomediated neuropathies in a group of patients with autoimmune diseases.

OBJECTIVE: To determine the prevalence and the clinical-neurophysiological characteristics of immunomediated peripheral neuropathies (PN) in a group of patients with systemic autoimmune diseases. METHOD: Fifty-nine patients with proved systemic autoimmune diseases were included. Patients underwent clinical examination and nerve conduction studies to diagnose the PN. RESULTS: Immune PNs were detected in 18 patients (30.5%). Out of the total number of PNs (18), 39% were sensory-motor polyneuropathies, 33% mononeuritis multiplex, 11% pure sensory polyneuropathies, 11% cranial neuropathies, and 6% proximal motor neuropathies, such as the Guillain-Barré syndrome. Nine PNs (50%) appeared at the onset of the connective tissue disorders, and the rest of the cases appeared during the course of the disease. Of the total of PNs detected in this study, only 45% had a previous diagnosis. Vasculitis was the disease that presented more associated PNs. Systemic lupus erythematosus showed the widest range of PN clinical varieties. CONCLUSIONS: The first national prevalence rate of PNs in patients with systemic autoimmune diseases was provided: 30.5%. No comparative data were found in the international bibliography. Sensory-motor polyneuropathy was the most frequently observed form of PN, followed by mononeuritis multiplex. The NPs appeared with the same frequency both at the onset and during the course of the diseases under study; these predominated at the onset of vasculitis and primary Sjögren syndrome. The compromise of the peripheral nervous system is underdiagnosed.

Insuficiencia renal aguda post percusión de tamboril / Acute renal failure following drum percussion

Se analiza el caso clínico de un paciente joven, que instala insuficiencia renal aguada post percusión prolongada del tamboril. Presentando clínicamente orina herrumbrosa y oligoanuria, con evolución a la normofunción mediante medidas de sostén. Se plantea como etiología una necrosis tubular aguda por hemoglobinuria discutiendo los mecanismos lesionales involucrados y la posible participación de la deshidratación en el daño reanl.