Therapeutic plasma exchange in rheumatic diseases: a university hospital experience / Troca plasmática terapêutica em doenças reumáticas: a experiência de um hospital universitário

Abstract Introduction: Each day, evidence accumulates related to the use of therapeutic plasma exchange (TPE) in patients with rheumatic diseases. San Ignacio University Hospital has recorded all of the TPE sessions performed by the institution's apheresis group. Objective: To describe the TPE experience of patients with rheumatologic diseases in a hospital setting. Methods: Descriptive, observational, retrospective analysis. This study included analyses of the TPE sessions that were performed in patients with rheumatic diseases from November 2009 to November 2013. Results: The apheresis group performed 136 sessions in 27 patients. The mean patient age was 43 years (SD 18.5), and 59.3% of the patients were female. Regarding the diagnosis, the most frequents ones where: ANCA-associated vasculitis followed by systemic lupus erythematosus and catastrophic antiphospholipid syndrome. The average number of sessions per patient was 5 (SD 1.8), and the average plasma exchange per patient was 1.3 plasma volume replacement units. The most used replacement solution was frozen fresh plasma (FFP; 63.2% of the sessions). Of all the sessions, 4.4% presented complications, and the majority of the complications were related to vascular access. Fifteen patients required renal replacement therapy (RRT) secondary to the same cause that created the need for TPE, 3 patients required RRT due to causes other than the TPE diagnostic intervention and 1 patient had undergone chronic dialysis. Conclusions: TPE is a therapeutic alternative that is needed for the management of patients with rheumatic diseases with renal involvement and those who are refractory to conventional management. Our clinical results were in agreement with the global literature.

Resumo Introdução: Diariamente acumulam-se evidências relacionadas com o uso da troca plasmática terapêutica (TPT) em pacientes com doenças reumáticas. O Hospital Universitário San Ignacio registrou todas as sessões de TPT feitas pelo grupo de aférese dessa instituição. Objetivo: Descrever a experiência do Hospital Universitário San Ignacio na TPT em pacientes com doenças reumatológicas. Métodos: Análise observacional, retrospectiva, descritiva. Incluiu análises das sessões de TPT feitas em pacientes com doenças reumáticas de novembro de 2009 a novembro de 2013. Resultados: O grupo de aférese fez 136 sessões em 27 pacientes. A idade média foi de 43 anos (DP 18,5) e 59,3% eram do sexo feminino. Quanto ao diagnóstico, os mais frequentes foram: vasculite associada ao anticorpo anticitoplasma de neutrófilos (ANCA) seguida de lúpus eritematoso sistêmico e síndrome antifosfolipídica catastrófica. A quantidade média de sessões por paciente foi de cinco (DP 1,8) e a média de troca plasmática por paciente foi de 1,3 unidade de substituição do volume de plasma. A solução de substituição mais usada foi o plasma fresco congelado (PFC, 63,2% das sessões). De todas as sessões, 4,4% apresentaram complicações, a maioria delas relacionadas com o acesso vascular. Quinze pacientes necessitaram de terapia de substituição renal (TSR) secundária à mesma causa que levou à necessidade de TPT; três pacientes necessitaram de TSR em decorrência de outras causas além da intervenção diagnóstica de TPT e um paciente tinha sido submetido à diálise crônica. Conclusões: A TPT é uma opção terapêutica necessária para o manejo de pacientes com doenças reumáticas com envolvimento renal e daqueles que são refratários ao tratamento convencional. Os resultados clínicos do presente estudo estão de acordo com o que é encontrado na literatura global.

Hemolytic uremic syndrome atypical after kidney transplantation: a case report and literature review / Síndrome hemolítico urémico atípico posterior a trasplante renal: presentación de un caso y revisión de la literatura

Abstract Haemolytic uremic syndrome (HUS) is a clinical entity characterized by the appearance of non-immune hemolytic anemia, thrombocytopenia and acute renal failure. It is a disease belonging to the group of thrombotic microangiopathy (MAT) which are part of thrombotic thrombocytopenic purpura also (PTT) and some other MAT associated with other medical conditions formerly known as secondary MAT. Moreover, the variety known as atypical HUS (aHUS) is an ultra-orphan disease that frequently progresses to chronic renal failure (CRF) and is associated with high morbidity and mortality if not properly treated. If a patient presents its first clinical manifestation of aHUS later receive a cadaveric renal transplant which not only makes it an even more exotic case but involves more complexity in their management is presented.

Resumen El síndrome hemolítico urémico (SHU) es una entidad clínica caracterizada por la aparición de anemia hemolítica no inmune, trombocitopenia e insuficiencia renal aguda. Se trata de una enfermedad perteneciente al grupo de las microangiopatías trombóticas (MAT) de la que hacen parte también la purpura trombocitopénica trombótica (PTT) y algunas otras MAT asociadas a otras condiciones médicas antes conocidas como MAT secundarias. Por otra parte, la variedad conocida como SHU atípico (SHUa) es una patología ultra-huérfana que frecuentemente evoluciona a insuficiencia renal crónica (IRC) y se asocia con elevada morbi-mortalidad si no recibe el tratamiento adecuado. Se examina el caso de un paciente que presenta su primera manifestación clínica de síndrome hemolítico urémico atípico después de trasplante renal cadavérico lo cual no solo lo hace un caso aún más exótico, sino que implica mayor complejidad en su manejo.

Síndrome pulmón-rinón: serie de casosdel Hospital Universitario San Ignacio / Pulmonary - renal syndrome: case series Hospital Universitario San Ignacio

El síndrome pulmón-rinón ˜ (SPR) se define como la presencia de hemorragiaalveolar y glomerulonefritis rápidamente progresiva. Fue descrito, inicialmente, como síndromeantimembrana basal glomerular, aunque existen otros mecanismos más frecuentesimplicados en la lesión, tales como vasculitis ANCA (anticitoplasma de neutrófilo), lupuseritematoso sistémico, síndrome antifosfolípido, crioglobulinemia y microangiopatía trombótica.Objetivo: Describir la experiencia de nuestro centro en el manejo de pacientes con SPR,analizar las variables demográficas, clínicas y sus desenlaces.Métodos: Se revisaron las bases de datos de la Unidad de Nefrología y la historia clínicaelectrónica SAHI del Hospital Universitario San Ignacio.Resultados: Se identificaron un total de 14 pacientes que presentaron SPR, entre enero de2009 y agosto de 2011, todos, inicialmente, manejados en la unidad de cuidado intensivodel hospital, con un promedio de edad de 44 anos ˜ y 57% de sexo femenino. El diagnósticomás frecuente fue vasculitis ANCA positivo en 8 pacientes, lupus en 4, un caso decrioglobulinemia y uno de microangiopatía trombótica. La mayoría recibió tratamiento concorticosteroides, ciclofosfamida y plasmaféresis. La mortalidad fue de 66%.Conclusiones: Presentamos los resultados de 14 pacientes con SPR en nuestro centro. La causamás frecuente fue vasculitis ANCA. Su presentación suele ser agresiva y el tratamientoincluye uso de esteroides, ciclofosfamida y plasmaféresis...

Pulmonary- renal syndrome (PRS) is defined as the presence of alveolar hemorrhageand rapidly progressive glomerulonephritis. It was initially described as glomerularbasement membrane syndrome, although other more common causes and mechanisms areinvolved such as, anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA), systemiclupus erythematosus, antiphospholipid syndrome, cryoglobulinemia, and thromboticmicroangiopathy.Objective: To describe the experience in the treatment of patients with PRS in our centerand analyze the demographic, clinical variables, and outcomes.Methods: The databases of the Nephrology Unit and the electronic medical records of thehospital were reviewed, and a total of 14 patients with PRS between January 2009 and August2011 were identified.Results: A total of 14 cases managed in the intensive care unit of the hospital where analyze,of which 57% were woman and the mean age was 44 years. The most frequent diagnosiswas positive ANCA vasculitis in 8 patients, lupus in 4, one case of cryoglobulinemia, andother with thrombotic microangiopathy. Most of them were treated with corticosteroids andcyclophosphamide, with 10 patients also receiving plasmapheresis. The overall mortalitywas 66%.Conclusions: The results of 14 patients with PRS in our center are presented. The mostcommon cause of this was positive ANCA vasculitis. It is an aggressive disease and itstreatment included the use of steroids, cyclophosphamide and plasmapheresis...

Intercambio plasmático terapéutico en enfermedades neurológicas mediadas inmunológicamente: experiencia de cuatro años del Hospital Universitario San Ignacio, Bogotá, Colombia / Therapeutic plasma exchange in immune-mediated neurological diseases: four-year experience at the University Hospital San Ignacio, Bogotá, Colombia

Introducción. El intercambio plasmático (IP) se ha convertido en una alternativa terapéutica para múltiples enfermedades. Cada día hay más evidencia científica que apoya el uso de esta terapia en varias enfermedades neurológicas. Es importante registrar la experiencia local en IP para conocer la respuesta de nuestra población a la terapia. Objetivo. Describir la experiencia del Hospital Universitario San Ignacio en IP realizado a pacientes con enfermedades neurológicas durante un período de cuatro años. Materiales y métodos. Estudio observacional retrospectivo en el que se describe a los pacientes, la técnica de la terapia, las complicaciones y la respuesta clínica de los pacientes con enfermedades neurológicas tratados con IP. Resultados. Se realizaron 106 sesiones de IP en 16 pacientes. El promedio de edad de los pacientes fue de 43 años, el 81% de los pacientes eran de sexo femenino. El 83% de las sesiones fueron indicadas por miastenia gravis, el resto por síndrome de Guillain-Barré, neuromielítis óptica, neuropatía motora multifocal y encefalitis autoinmune. Todas las sesiones fueron realizadas por técnica de filtración transmembrana. El promedio de recambios plasmáticos realizado fue 1.3. Se registraron 26 complicaciones en 20 sesiones de IP. La respuesta neurológica de los pacientes a la terapia fue la esperada y similar a la reportada en la literatura médica. Conclusión. Según la experiencia de la institución, el IP es una terapia segura y eficaz en pacientes con enfermedades neurológicas.

Introduction. Plasma exchange (PE) has become a therapeutic treatment for many neurological diseases. Each day more scientific papers are being published with evidence that approves PE for patients with immunological mediated neurological disorders. It is important to register our local experience with PE in order to understand the clinical answer to the therapy in our population. Objective. To describe the experience of the Hospital Universitario San Ignacio in treating patients with neurological disorders with PE, during a 4 year period. Materials and methods. Observational retrospective study, which describes the patients, PE technique, complications of therapy and clinical answer of patients with neurological conditions treated with PE. Results. 106 sessions of PE in 16 patients, corresponding to 23 procedures. The average age was 43 years, 81% of patients were female. 83% of sessions were done for Myasthenia gravis, the rest were done for treatment of Guillain-Barré, Optical Neuromyelitis, multifocal motor neuropathy and autoimmune encephalitis. All sessions were done by membrane filtration technique. The average plasma volume exchange was 1.3, and 20 sessions presented at least one complication. The neurological response of the patients was the expected and was similar to those reported in the literature. Conclusions. Based on the experience of our medical center, PE is a safe and effective therapy for patients with some immunological mediated neurological disorders.

Plasmaféresis terapéutica / Therapeutic plasmapheresis

Resumen Introducción: cada día hay más evidencia acerca de la utilidad de la plasmaféresis en diferentes entidades clínicas. Existen múltiples registros internacionales de plasmaféresis; sin embargo, la información en Colombia e incluso en Latinoamérica es limitada. Objetivo: realizar análisis descriptivo de la experiencia en plasmaféresis de una institución académica en Bogotá, Colombia. Analizar las características de la técnica, indicaciones, complicaciones y características demográficas de los pacientes. Material y métodos: se realiza un análisis descriptivo de las sesiones de plasmaféresis realizadas en el Hospital Universitario San Ignacio, en pacientes mayores de 18 años durante el periodo comprendido entre agosto de 2008 hasta agosto de 2011. Todas las sesiones se realizaron mediante la técnica de filtración transmembrana y no se usó anticoagulante en ninguna de ellas. Resultados: se analizaron 278 sesiones de plasmaféresis en 33 pacientes adultos durante un período de tres años. 69.7% de los pacientes eran mujeres, el promedio de edad era 42 años. 57% de las sesiones se realizaron en pacientes con diagnóstico de miastenia gravis y hemorragia alveolar. El volumen promedio de intercambios plasmáticos fue 1.28. El 9.3% de las sesiones presentaron al menosuna complicación. Se documentó frecuentemente hipotensión arterial en las sesiones realizadas con albúmina al 3.5%. 12% de los registros de electrolitos tenían hipocalcemia y 47% hipermagnesemia. No hubo muertes relacionadas con la terapia y pese al no uso de anticoagulante, no se presentó coagulación del circuito en ninguna de las sesiones. Conclusiones: dada nuestra experiencia consideramos que la plasmaféresis es una terapia segura. Es necesario evaluar la necesidad de la administración de anticoagulante al circuito de plasmaféresis, dado que no se documentó coagulación de éste en ninguna sesión y su uso podría estar asociado a eventos adversos. El monitoreo continuo de los electrolitos es fundamental y la reposición de los mismos debe ser individualizada. (Acta Med Colomb 2014; 39: 29-34).

Abstract Introduction: there is growing evidence for the usefulness of plasmapheresis in different clinical entities. There are many international records of plasmapheresis, but the information in Colombia and even in Latin America, is limited. Objective: to perform a descriptive analysis of the experience in plasmapheresis of an academic institution in Bogotá, Colombia. To analyze the characteristics of the technique, indications, complications and demographic characteristics of patients. Material and methods: descriptive analysis of plasmapheresis sessions performed in the Hospital Universitario San Ignacio in patients older than 18 years during the period from August 2008 to August 2011. All sessions were conducted by the technique of transmembrane filtration. No anticoagulation was used in any of them. Results: 278 sessions of plasmapheresis were analyzed in 33 adult patients over a period of three years. 69.7% of patients were female, with mean age of 42 years. 57% of the sessions were conducted in patients diagnosed with myasthenia gravis and alveolar hemorrhage. The average volume of plasma exchange was 1.28. 9.3% of the sessions presented at least one complication. Hypotension was frequently documented in the sessions performed with 3.5% albumin. 12% of electrolytes records had hypocalcemia and 47% hypermagnesemia. There were no therapy-related deaths and despite the non-use of anticoagulant, no clotting of the circuit in any of the sessions was presented. Conclusions: Given our experience, we believe that plasmapheresis is a safe therapy. It is necessary to assess the need for administration of anticoagulant to the plasmapheresis circuit since no clotting in it in any session was documented and its use could be associated with adverse events. Continuous monitoring of electrolytes is essential and its replacement should be individualized. (Acta Med Colomb 2014; 39: 29-34).

Actualidad de la enfermedad renalpoliquística / Polycystic Renal Disease: An Update

La enfermedad renal poliquística (PKD) es una enfermedad genética común queconsiste en la aparición progresiva de lesiones quísticas en los riñones, que remplazanel parénquima renal, lo que conduce a enfermedad renal crónica terminal. La PKDtiene dos patrones de herencia: autosómico dominante y autosómico recesivo. Laforma autosómica dominante es más común y menos grave que la autosómica recesiva.Se conoce que la PKD es causada por mutación en varios loci humanos. La formaautosómica dominante puede ser causada por mutaciones en dos genes diferentes(PKD1 y PKD2); en tanto que la forma autosómica recesiva solo tiene un gen causal(PKHD1). Existen numerosas publicaciones que buscan explicar la fisiopatología dela enfermedad. Esto refleja un esfuerzo internacional por comprender la naturalezade la enfermedad, para desarrollar terapias que eviten la aparición de los quistes o laprogresión de los que ya están instaurados. El objetivo de esta revisión es difundirel conocimiento que se tiene hasta el momento, acerca de la enfermedad renalpoliquística. Por lo tanto, realizamos un breve recuento de las características clínicasde la enfermedad y el tratamiento actual disponible...

Polycystic Kidney Disease (PKD) is a commongenetic condition, which is characterizedby gradual appearance of multiple cysts in thekidneys; this causes the destruction of renalparenchyma leading to chronic renal disease.PKD has two patterns of inheritance: autosomaldominant and autosomal recessive. Theautosomal dominant form is more commonand less severe than the autosomal recessive. Itis known that PKD is caused by mutation in severalhuman loci. The autosomal dominant formcan be caused by mutations in 2 different genes(PKD1 and PKD2). The autosomal recessiveform has only one causal gene (PKHD1). Thereare numerous publications worldwide that seekto explain the pathophysiology of the disease;this reflects an international effort to understandthe nature of the disease, to develop therapiesto prevent the appearance of cysts or the progressionof those already existent lesions. The objectiveof this review is to update the knowledge wehave so far, about polycystic kidney disease thereforewe decided to conduct a brief review ofthe clinical features of disease and the treatmentavailable today...

Prevalence of cholelithiasis in Buenos Aires, Argentina / Prevalence of cholelithiasis in Buenos Aires, Argentina.

BACKGROUND: Cholelithiasis is an important cause of morbidity in the world and it varies significantly depending on the region of study. OBJECTIVE: To assess the prevalence of cholelithiasis in Buenos Aires. MATERIAL AND METHODS: This is a cross-sectional, descriptive, observational study performed in a public hospital with tertiary care in Gastrointestinal Surgery. From July 2010 to December 2011, 1,875 healthy volunteers who underwent liver, gallbladder and pancreas ultrasound imaging were included and a questionnaire containing the individual’s medical history was administered. The presence of cholelithiasis and its eventual risk factors were studied. RESULTS: Of 1,875 individuals, 866 were males (46.2

) and 1,009 females (53.8

), older than 20 years old with an average age of 46.1 + 16.7 years. Cholelithiasis was found in 410 inidviduals (21.9

) diagnosed at the time of the study and 125 (67

) who had already undergone a cholecystectomy due to gallstones. CONCLUSIONS: The prevalence of cholelithiasis in Buenos Aires, capital city of Argentina, is 21.9

. A significant association was found between cholelithiasis and female gender, age, body mass index, history of colic pain, family history of cholelithiasis, smoking, fatty liver and number of pregnancies.

Sarcoidosis y compromiso renal: reporte de un caso y revisión de la literatura científica / Sarcoidosis and kidney disease: a case report and review of the literature

Se presenta el caso de una mujer de 30 años, que presentaba síntomas respiratorios y constitucionales de dos meses de evolución. Se documentó exudado linfocitario y proteinuria, y se descartó tuberculosis y neoplasia maligna. Se le practicó biopsia pleuraly de ganglio linfático, que reportaron sarcoidosis. Se tomó biopsia renal por presencia de proteinuria con pruebas serológicas negativas, y se encontró glomerulonefritis membranosa secundaria. Se inició tratamiento con prednisolona...

We describe the case of a 30 years old female with a two month history of respiratory symptoms and malaise. A lymphocytic pleural effusion and proteinuria were documented,tuberculosis and malignancy were ruled out. Biopsy of lymph nodes and pleura confirmed sarcoidosis. Due to non-nephrotic proteinuria with negative serology tests, kidney biopsy was performed, showing secondary membranous glomerulonephritis. Treatment with prednisolone was started...