RESUMO
Atypical hemolytic uremic syndrome (HUS) is a part of a large category of diseases known as thrombotic microangiopathies that present with hemolytic anemia, thrombocytopenia, and target organ damage mostly characterized by acute kidney injury. It is a rare and challenging diagnosis due to the complex pathophysiology underlying the disease and its overlap with other conditions. We report the case of atypical hemolytic uremic syndrome in a 61-year-old female with a history of metastatic peritoneal serous carcinoma of the ovary presenting with anemia and acute renal failure.
RESUMO
Glomerular diseases continue to be the leading cause of end-stage renal disease globally. Renal biopsy plays a fundamental role in the evaluation of glomerular diseases not only to establish an accurate diagnosis but also help deciding on appropriate treatment and assessing prognosis. The prevalence of glomerular disease and the clinical indications for kidney biopsies are poorly delineated in Puerto Rico. We undertook a retrospective analysis of the indications, clinical presentation and pathologic reports in renal biopsies performed at the University District Hospital in San Juan, Puerto Rico from the year 1995 to 2008. A total of 208 kidney biopsies showed a predominance of membranous nephropathy representing 20% of the studied population. Women were more frequently biopsied than men (57.2% vs. 42.7%). Lupus nephritis, a condition affecting mostly women was identified in 16.9% of the patients. Minimal change disease was reported in 13.6% of the patients, a condition that affects mostly children and adolescents. In contrast to other geographical areas IgAN was reported only in 6.3% and FSG in 0.9% of patients. In our biopsied patient population, membranous nephropathy is the most common primary glomerular disease and lupus erythematosus the most frequent secondary glomerular disease.
Assuntos
Nefropatias/patologia , Glomérulos Renais , Rim/patologia , Sistema de Registros , Biópsia , Hospitais Universitários , Humanos , Porto Rico , Estudos RetrospectivosRESUMO
Resumen La hemofilia es una diátesis hemorrágica producida por la deficiencia hereditaria de un factor (proteína) de la coagulación sanguínea que afecta principalmente a los varones. Su grado de severidad puede variar desde casos con poco sangrado, hasta condiciones muy graves que en muchas ocasiones llevan a la muerte a los enfermos. Existen dos tipos de hemofilia: la A por carencia del factor VIII y la B por falta del factor IX. En este editorial se resume de manera global la situación actual de los avances de la hemofilia desde el punto de vista clínico y del laboratorio.
Abstract Hemophilia is a hemorrhagic diathesis that is caused by the hereditary deficiency of a factor (protein) of blood clotting and that affects mainly men. Its degree of severity can vary from cases with little bleeding, to very serious conditions that often lead to death. There are two types of hemophilia, A for lack of factor VIII, and B for lack of factor IX. This editorial summarizes the current state of progress of hemophilia from the clinical and laboratory point of view.
Assuntos
Humanos , Hemofilia B/tratamento farmacológico , Hemofilia A/tratamento farmacológico , Transtornos HemorrágicosRESUMO
La enfermedad de Chagas es una enfermedad parasitaria (Trypanosoma cruzi), endémica en 21 países de América y que las migraciones la han dispersado en distintos continentes. Una de las manifestaciones más precoces de esta enfermedad son las alteraciones disautonómicas o disfunción autonómica. La severidad de este inadecuado funcionamiento del sistema nervioso autónomo resulta mensurable, de modo que la evolución y/o progresión de la enfermedad puede constatarse mediante la alteración de estudios clínicos y detección de anticuerpos antimuscarínicos. Estos anticuerpos están presentes en un 30% de los infectados y aparecen muy precozmente una vez instalada la parasitosis; además otros estudios, como la dispersión del QT (>65 mseg) y la variabilidad de la frecuencia cardíaca (<100 mseg) presentan valores anormales. La utilización de nuevos paradigmas de atención, diagnóstico y tratamientos adecuados son imprescindibles para prevenir el desarrollo de esta cardiopatía.
Chagas disease is a parasitic disease (Trypanosoma cruzi), endemic in 21 countries of America and that migrations have dispersed it in different continents. One of the earliest manifestations of this disease is dysautonomic alterations or autonomic dysfunction. The severity of this inadequate functioning of the autonomic nervous system is measurable, so that the evolution and/or progression of the disease can be verified by altering clinical studies and detecting antimuscarinic antibodies. These antibodies are present in 30% of those infected and appear very early once the parasitosis is installed; In addition, other studies, such as QT dispersion (> 65 ms) and heart rate variability (<100 ms) show abnormal values. The use of new paradigms of care, diagnosis and appropriate treatments are essential to prevent the development of this heart disease.
A doença de Chagas é uma doença parasitária (Trypanosoma cruzi), endêmica em 21 países da América e que as migrações a dispersaram em diferentes continentes. Uma das primeiras manifestações desta doença são as alterações disautonômicas ou disfunção autonômica. A gravidade desse funcionamento inadequado do sistema nervoso autônomo é mensurável, de modo que a evolução e/ou progressão da doença pode ser verificada alterando os estudos clínicos e detectando anticorpos antimuscarínicos. Esses anticorpos estão presentes em 30% dos infectados e aparecem muito cedo, uma vez instalada a parasitose; Além disso, outros estudos, como a dispersão do QT (> 65 mseg) e a variabilidade da freqüência cardíaca (<100 mseg), mostram valores anormais. A utilização de novos paradigmas de atendimento, diagnóstico e tratamentos adequados são essenciais para prevenir o desenvolvimento desta doença cardíaca.
RESUMO
A review of leukemia worldwide is discussed, focusing on etiology, diagnosis and treatment. The history of research of this type of cancer in Costa Rica is presented through the first hospital diagnosis, the arrival of clinical and laboratory hematologists, the establishment of specialized laboratories, the local hematology teaching programs and the voluntary associations that help patients with leukemia. A brief review of Costa Rican publications in this area and the future of this problem in our country is also shown.
Assuntos
Bibliometria , Pesquisa Biomédica/estatística & dados numéricos , Leucemia , Costa Rica , Humanos , Leucemia/diagnóstico , Leucemia/etiologia , Leucemia/terapiaRESUMO
A review of leukemia worldwide is discussed, focusing on etiology, diagnosis and treatment. The history of research of this type of cancer in Costa Rica is presented through the first hospital diagnosis, the arrival of clinical and laboratory hematologists, the establishment of specialized laboratories, the local hematology teaching programs and the voluntary associations that help patients with leukemia. A brief review of Costa Rican publications in this area and the future of this problem in our country is also shown.
Assuntos
Humanos , Bibliometria , Leucemia , Pesquisa Biomédica/estatística & dados numéricos , Costa Rica , Leucemia/diagnóstico , Leucemia/etiologia , Leucemia/terapiaRESUMO
Se analizan las principales características clínicas y de laboratorio de las deficiencias aisladas y combinadas de los factores II, VII y X. Se señala la importancia del uso de diferentes reactivos y metodologías para poderlos clasificar, sobre todo las variantes, debido a que en estos casos se presentan niveles inmunológicos normales con niveles disminuidos de actividad funcional o coagulante. Se discute brevemente el diagnóstico diferencial de estos trastornos, asi como se esbozan algunas pautas para el manejo del paciente con estas complicaciones hemorragicas hereditarias