Enfermedad de whipple e hipertensión pulmonar reversible / Whipple's disease and reversible pulmonary hypertension

Resumen La enfermedad de Whipple es una enfermedad multisistémica crónica, causada por la bacteria Tropherima whipplei. Se han descripto aproximadamente 1200 casos en la literatura. La incidencia mundial se estima en 9.8 casos por millón de personas. Los datos provenientes de Sudamérica y Europa muestran que afecta a varones de mediana edad. Se cree que los factores inmunológicos del huésped son los que influyen en el curso de la infección y no el genotipo del agente. Dado que las características clínicas no suelen ser específicas y el espectro de manifestaciones en órganos individuales puede ser subestimado, el diagnóstico sigue siendo muy difícil. Presentamos un caso confirmado por histopatología con compromiso multisistémico. Consideramos importante su difusión dados los escasos casos documentados en Sudamérica y la relevancia de tener presente la sospecha diagnóstica para el abordaje terapéutico precoz que mejora el pronóstico de esta rara enfermedad.

Abstract Whipple's disease is a chronic mutisystem disease caused by the bacteria Tropherima whipplei. Approximately 1200 cases have been described in the literature. The worldwide incidence is estimated at 9.8 cases per million people. Data from South America and Europe show that it affects middle-aged males. It is believed that host immunological factors rather than agent genotypic traits influence the course of the infection. Since the clinical characteristics are usually nonspecific and the wide spectrum of manifestations in individual organs may be underestimated, the diagnosis remains challenging. We present a case with multisystem compromise confirmed by histopathology. We consider its publication important given the few cases documented in South America and the relevance of bearing in mind the importance of an early diagnosis for a prompt treatment that improves the prognosis of this rare disease.

Whipple's disease: a fatal mimic

Whipple's Disease, a rare diagnosis caused by the slow-growing bacterium Tropheryma whipplei, most often presents with the classically described signs of malabsorption due to gastrointestinal colonization. However, it can also have signs and symptoms that clinically overlap with rheumatic diseases, potentially resulting in misdiagnosis. Furthermore, treatment with modern potent biologic immunosuppressive agents and classic disease modifying anti-rheumatic drugs (DMARDs) can lead to serious exacerbation of undiagnosed infections. We present the case of a middle-aged woman with long term complaints of arthalgias, who was diagnosed with seronegative rheumatoid arthritis and subsequently treated for almost 7 years with such immunosuppressive therapies. The patient's disease course included chronic diarrhea that abruptly intensified and culminated in fatal hypovolemic shock/sepsis. A diagnosis of WD was made by autopsy examination, wherein several organ systems were found to be heavily involved by Tropheryma whipplei organisms, and their identification was confirmed with histochemical and molecular evaluation. Notably, most bacterial organisms were located deeply in the submucosa/muscularis of affected organs, a practical reminder to practicing pathologists that challenges the classic histopathologic description of Whipple disease as an infiltration of predominantly lamina propria, and the potential for sampling bias in typically superficial endoscopic biopsies during routine procedures.

Seguridad del procedimiento de Whipple: experiencia de 10 años en un centro de tercer nivel / Whipple procedure safety: 10 year experience in a third level center / Whipple procedure safety: 10 year experience in a third level center

Resumen Antecedentes: el procedimiento de Whipple, apesar de los avances en cirugía, continúa siendo el procedimiento más complejo en cirugía general. Su principal indicación es la patología neoplásica de páncreas, duodeno y vías biliares que persiste como un desafío terapéutico debido a su alta morbilidad y mortalidad que en nuestro país alcanza 40% a 60% y de 15% a 30%, respectivamente. Objetivo: determinar la seguridad del procedimiento de Whipple en una institución privada de tercer nivel. Materiales y métodos: se realizó un estudio observacional retrospectivo, desde 2007 hasta 2017 en el Hospital Metropolitano. Datos recolectados: indicación quirúrgica, complicaciones, estancia hospitalaria, entre otros, que se analizaron con JASP 0.9.2.0. Resultados: universo 30 pacientes, edad promedio: 60,1 años IC (95%: 39 a 79 años); mujeres: 56%, hombres: 44%. Estancia hospitalaria promedio: 15 días con un DS:15 días. Principales indicaciones: adenocarcinoma de páncreas 33,3%, adenocarcinoma de ámpula de Vatter 16,7% colangiocarcinoma 16,7%. Según la escala Clavien Dindo, el 76,7% presentó complicaciones; sin embargo, de éstas el 59,9% fueron leves. Mortalidad intrahospitalaria: 6,7%. Conclusiones: atribuimos los resultados, entre otros motivos, a la disponibilidad de facilidades técnicas y a la experiencia del equipo quirúrgico que enfatiza en la prevención de las complicaciones o, si se presentare, al diagnóstico oportuno y tratamiento adecuado con todo el arsenal terapéutico requerido.

Abstract: Background: The Whipple procedure, despite advances in surgery, remains the most complex procedure in general surgery, its main indication is the neoplastic pathology of pancreas, duodenum and bile ducts. This persists as a therapeutic challenge due to its high morbidity and mortality that in our country reaches from 40% to 60% and from 15% to 30%, respectively. Objective: Determine the safety of performing the Whipple procedure in a third level private institution. Materials and methods: A retrospective observational study was conducted from 2007 to 2017 at the Hospital Metropolitano, the data collected included: surgical indication, complications, hospital stay, among others; these were analyzed with JASP 0.9.2.0. Results: We included 30 patients, their mean age was 60.1 years, 95% CI: 39 -79 years; 56% were women and 44% men. The average hospital stay was 15 days with a SD:15 days. The main indications were: adenocarcinoma of the pancreas 33.3%, adenocarcinoma of the Vatter ampulla 16.7% and cholangiocarcinoma 16.7%. According to the Clavien Dindo scale, 76.7% presented complications, however, 59.9% of these were mild. In-hospital mortality reached 6.7%. Conclusions: We attribute the results shown, among others, to the availability of technical facilities, as well as to the experience of the surgical team, emphasizing the prevention of complications or in the case of occurrence, provide a timely diagnosis and adequate treatment with allthe required therapeutic methods.

Enfermedad de Whipple en paciente con fiebre de origen desconocido / Whipple's disease in a patient with fever of unknown origin

La enfermedad de Whipple es una entidad multisistémica de origen infeccioso causada por una bacteria Gram positiva perteneciente a la familia de los actinomicetos denominada Tropheryma whippleii. Constituye una causa infecciosa infrecuente de fiebre de origen desconocido. Se presenta el caso de un paciente masculino de 65 años con cuadro febril de 3 años de evolución, poliartralgias, edema en miembros inferiores, adenopatías periféricas y diarreas ocasionales. Se describen la evolución clínica, los estudios de laboratorio, imagenológicos e histológicos que permitieron hacer el diagnóstico de enfermedad de Whipple. Se considera que la publicación de este caso es importante pues contribuye a mantener presente esta enfermedad entre las posibilidades diagnósticas de los pacientes con fiebre de origen desconocido, aún por lo atípico de esta forma de presentación(AU)

Whipple's disease is a multisystem entity of infectious origin caused by a Gram-positive bacterium called Tropheryma whippleii of the actinomycetes family. This is an infrequent infectious disease causing fever of unknown origin. We present the case of a 65-year-old male patient with a 3-year history of fever, polyarthralgia, lower limb edema, peripheral lymphadenopathy, and occasional diarrhea. The clinical evolution, the laboratory results, imaging and histological studies that allowed the diagnosis of Whipple's disease are described. The publication of this case is considered important because it contributes to keep this disease into account among the diagnostic possibilities of patients with fever of unknown origin, since the atypical nature of this form of presentation(AU)

Whipple disease without joint involvement in a patient with chronic diarrhea and HIV seropositive partner: case report / Doença de Whipple sem envolvimento articular em paciente com diarréia crônica e parceira HIV-soropositiva: Relato de caso

Introduction: Whipple's disease is a rare, infectious disease caused by the bacterium Tropheryma whipplei that affects multiple organs and systems. It is difficult to identifyand frequently presents as a diagnosis of exclusion due existence of conditions causing most prevalent chronic diarrhea, as AIDS and Inflammatory Bowel Disease. Case report: Herein we report the clinical findings of a 38-year-old male patient, married with a HIV seropositive woman, with a four-year course of chronic diarrhea until he received a definitive diagnosis. Treatment was started with a 15-day penicillin G regimen, followed by continued trimethoprim­sulfamethoxazole 80/400mg after hospital discharge up to current days. Conclusion: Currently, the patient remains asymptomatic and has completed clinical remission after two years of treatment. (AU)

Introdução: A doença de Whipple é uma doença infecciosa rara causada pela bactéria Tropheryma whipplei que afeta múltiplos órgãos e sistemas. É difícil de identificar e freqüentemente se apresenta como um diagnóstico de exclusão devido à existência de condições que causam diarréia crônica mais prevalentes, como AIDS e Doença Infçamatória Intestinal. Relato do Caso: Aqui, relatamos os achados clínicos de um paciente do sexo masculino de 38 anos, casado com uma mulher HIV-soropositiva, com um curso de quatro anos de diarréia crônica até receber o diagnóstico definitivo. O tratamento foi iniciado com penicilina G por 15 dias, seguido de sulfametoxazol- trimetoprim 400/80 mg após a alta hospitalar até os dias atuais. Conclusão: Atualmente, o paciente permanece assintomático e apresentado remissão clínica completa após dois anos de tratamento. (AU)

Doença de Whipple: relato de caso / Whipple's Disease: a case report

Descrevemos um caso de doença de Whipple diagnosticada em paciente com síndrome diarreica crônica, emagrecimento intenso, pesquisa de DNA da Tropheryma whipplei positiva em fluido biológico e resposta excelente ao tratamento antibiótico.

A case of Whipple's disease diagnosed in a patient with chronic diarrhea syndrome, intense weight loss, positive DNA searching for Tropheryma whipplei, and excellent clinical response to antibiotic therapy is described.

Tumoural form of Whipple's disease simulating carcinomatosis

Whipple's disease is a rare disease caused by the actinomycete bacteria Tropheryma whipplei, which cause intestinal infection. The most common symptoms are chronic diarrhoea, weight loss, abdominal pain, arthritis and neurological abnormalities, which can be fatal. This paper reports a case of a 57-year-old Brazilian woman with diarrhoea, vomiting, abdominal pain, appetite loss, intermittent fever, malaise, weight loss and malnutrition. Migratory polyarthralgia and recurrent visual scotomas preceded the symptoms. The retroperitoneal pseudotumour formation finding was associated with prolonged wasting syndrome, which did not respond to usual therapies, thus leading to the investigation of carcinomatosis disease. After laparotomy, biopsy and histochemical study of the lesions with negative results for malignancy, we proceeded to the investigation of Whipple's disease, which was then confirmed. The patient improved clinically and started gaining weight after using ceftriaxone (IV).

Whipple's disease in an Afro-Caribbean national / Enfermedad de whipple en un nacional afrocaribeño

Whipple's disease is a rare multi-organ infectious disease caused by Tropheryma whipplei. It is fatal without treatment. We report on a 40-year old Afro-Jamaican man who presented with a six-month history of weight loss and diarrhoea. Investigations revealed iron deficiency anaemia and hypoalbuminaemia. Upper gastrointestinal endoscopy revealed white patchy lesions in the duodenum. The duodenal biopsy showed broadening and thickening of the villi by a dense infiltrate of foamy histiocytes within the lamina propria and focally extending into the attached submucosa. Periodic Acid-Schiff stains were positive. Electron microscopy was confirmatory and polymerase chain reaction testing conclusively identified the organisms as T whipplei. Antibiotic treatment resulted in resolution of symptoms. Although the diagnosis of Whipple's disease is difficult, increased awareness should lead to an increase in reported cases with the improvements in diagnostic capabilities.

La enfermedad deWhipple es una rara enfermedad infecciosa multiorgánica causada por el Tropheryma whipplei. Es fatal sin tratamiento. Reportamos un hombre afro-jamaicano de 40 años que se presentó con una historia de seis meses de pérdida de peso y diarrea. Las investigaciones revelaron hipoalbuminemia y anemia ferropénica. La endoscopia gastrointestinal superior reveló lesiones blancas irregulares en el duodeno. La biopsia duodenal mostró la ampliación y engrosamiento de las vellosidades por un denso infiltrado de histiocitos espumosos dentro de la lámina propia, que se extienden hasta la submucosa adjunta. Las tinciones con ácido peryódico de Schiff fueron positivas. La microscopia electrónica fue confirmatoria y la prueba de la reacción en cadena de la polimerasa, identificó los organismos como T whipplei de forma concluyente El tratamiento antibiótico trajo como resultado la resolución de los síntomas. Si bien el diagnóstico de la enfermedad de Whipple es difícil, un aumento de la conciencia debe conducir a un aumento en los reportes de casos divulgados que reflejan un mejoramiento en la capacidad para hacer el diagnóstico.

Whipple's disease: rare disorder and late diagnosis / Doença de Whipple: patologia rara e de diagnóstico tardio

Whipple's disease is a rare systemic infectious disorder caused by the bacterium Tropheryma whipplei. We report the case of a 61-year-old male patient who presented to emergency room complaining of asthenia, arthralgia, anorexia, articular complaints intermittent diarrhea, and a 10-kg weight loss in one year. Laboratory tests showed the following results: Hb = 7.5 g/dL, albumin = 2.5 mg/dL, weight = 50.3 kg (BMI 17.4 kg/m²). Upper gastrointestinal endoscopy revealed areas of focal enanthema in the duodenum. An endoscopic biopsy was suggestive of Whipple's disease. Diagnosis was confirmed based on a positive serum polymerase chain reaction. Treatment was initiated with intravenous ceftriaxone followed by oral trimethoprim-sulfamethoxazole. After one year of treatment, the patient was asymptomatic, with Hb = 13.5 g/dL, serum albumin = 5.3 mg/dL, and weight = 70 kg (BMI 24.2 kg/m²). Whipple's disease should be considered a differential diagnosis in patients with prolonged constitutional and/or gastrointestinal symptoms. Appropriate antibiotic treatment improves the quality of life of patients.

Doença de Whipple é uma rara infecção sistêmica causada pelo Tropheryma whipplei. Caracteriza-se por fase prolongada de sintomas inespecíficos, levando longo período até o seu diagnóstico. Sem tratamento, pode ser grave e fatal, mas com antibioticoterapia tem ótima resposta clínica e laboratorial. Relatamos o caso de paciente masculino, 61 anos, internado por astenia, anorexia, diarréia intermitente e perda de 10 kg em um ano. Apresentava-se com hemoglobina (Hb) 7,5 g/dL, albumina de 2,5 mg/dL, peso 50,3 kg (IMC 17,4). Endoscopia digestiva alta com áreas de enantema focal da mucosa duodenal e biópsia compatível com doença de Whipple. O diagnóstico foi confirmado com PCR sérica positiva, sendo instituído tratamento com ceftriaxone seguido de sulfametoxazol-trimetropim. Após um ano de tratamento, encontrava-se assintomático, com Hb 13,5 g/dL, albumina sérica de 5,3 mg/dL e peso de 70 kg. Doença de Whipple deve fazer parte da lista de diagnósticos diferenciais em pacientes com sintomas constitucionais e/ou com queixas gastrointestinais com evolução prolongada. O tratamento antibiótico pode curar a infecção, recuperando a qualidade de vida do paciente.

Enfermedad de whipple en paciente HIV positivo / Whipple disease in an HIV patient

La enfermedad de Whipple es una enfermedad crónica sistématica de infrecuente presentación, causada por una bacteria, Tropheryma Whipplei, del orden Actinomycetales. Existen cerca de 1.000 casos reportados en la actualidad. Presentamos un caso de enfermedad de Whipple en un paciente HIV (+).

Evaluación de los factores condicionantes de morbi-mortalidad en la duodenopancreatectomía por neoplasias periampulares / Evaluation of conditioning factors or morbimortality in duodenopancreatectomy in periampullary neoplasms

El propósito de este estudio, es definir cuales son los factores que condicionan las complicaciones y la mortalidad en un grupo de 119 pacientes con neoplasias peri ampulares operados en un hospital general. MATERIAL Y MÉTODO: Desde Octubre del 2002 hasta Diciembre del 2006 se analizaron 119 pacientes operados de duodenopancreatectomía en el hospital Rebagliati por el diagnostico de neoplasia peri ampular.RESULTADOS: La edad y el sexo no fueron factores condicionantes de complicaciones. Se observo una diferencia significativa en el tipo de anastomosis pancreática utilizadadonde el telescopaje fue un factor condicionante de complicación (p<0.009) Cuando se comparó con a la anastomosis mucosa-mucosa. Al analizar el sangrado con cada una de las complicaciones, se pudo ver que existía una relación significativa entre el mayor volumen de sangrado en sala de operaciones con el desarrollo de fístula pancreática (p<0.03), reoperación (p<0.01), absceso (p<0.006), y fístula intestinal (p<0.001). Se evaluó cuales fueron las complicaciones relacionados a la mortalidad en los pacientes sometidos a Pancreatoduodenectomía (PD), se pudo observar que la fístula pancreática (p<0.003), la fístula intestinal (p<0.003), la fístula biliar (p<0.03), la hemorragia intra abdominal, el sangrado en Sala de Operaciones (SOP), las transfusiones y las reoperaciones fueron factores que aumentaron la mortalidad, en forma significativa. Cuando se evaluó al cirujano como factor de morbi-mortalidad, se pudo comprobar queexistía una relación entre el volumen quirúrgico y la incidencia de complicaciones y mortalidad. Teniendo una menor estancia hospitalaria una menor incidencia en el sangradointra operatorio y una morbi-mortalidad menor en el grupo de alto volumen quirúrgico. CONCLUSIONES: Las complicaciones más temidas ya que se relacionan directamente con la mortalidad son: la fístula intestinal, hemorragia intra abdominal, absceso intraabdominal, fístula biliar y la necesidad de...

OBJECTIVE: The purpose of this study is to define the factors that condition complications and mortality in a group of 119 patients with periampullary neoplasms operated on at a general hospital. MATERIALS & METHOD: Between October 2002 and December 2006, 119 patients who were diagnosed with periampullary neoplasms and underwent a pancreatoduodenectomyat Rebagliati Hospital were evaluated. RESULTS: Age and sex were not conditioning factors of complications. A significant difference in the type of pancreatic anastomosis used was noticed, whereby telescopingwas a conditioning factor of complication (p<0.009) compared with mucous-mucous anastomosis. After analyzing the bleeding associated with each complication, a significant connection was established between the greatest bleeding volume obtained in the operating room with the development of pancreatic fistulas (p<0.03), re-operation (p<0.01), abscesses (p<0.006) and intestinal fistulas (p<0.001). The complications related to mortality in patients who underwent a pancreatoduodenectomy (PD) were evaluated. It was observed that pancreatic fistulas (p<0.003), intestinal fistulas (p<0.003) and gallbladder fistulas (p<0.03), intra-abdominal hemorrhaging and bleeding during a standard operating procedure (SOP), transfusions and re-operations were factors that increased mortality significantly. When the surgeon was evaluated as a factor of morbimortality, it was proven that there was a connection between the surgical volume and the incidence of complications andmortality. There was a shorter hospital stay, a lower incidence of intra-operative bleeding and lower morbimortality in the high surgical volume group.CONCLUSIONS: The most-feared complications, since they are directly related to mortality, are intestinal fistulas, intra-abdominal hemorrhaging, intra-abdominal abscesses,gallbladder fistulas and the need to undergo repeated operations. Both complications and mortality are directly related to the...

Pancreaticoyeyunostomía ducto-seromuscular término-lateral en la operación whipple: experiencia en 27 casos / Seromuscular end pancreaticojejunostomy-duct-side in the whipple operation: experience with 27 cases

Presentar la experiencia con la anastomosis ductoserosa termino-lateral en la pancreaticoyeyunostomía del Whipple, en el Servicio de Cirugía General del Instituto Autónomo Hospital Universitario de Los Andes, Mérida-Venezuela. Estudio prospectivo, descriptivo en pacientes sometidos a duodenopancreatectomía por patología maligna a nivel pancreático y región periampular entre los años 2003 a 2007. En todos los casos se realizó preservación pilórica con anastomosis ducto-serosa termino-lateral del remanente pancreático, con colocación de stent intraductal, excepto en un caso donde se realizó la técnica invaginante término-terminal. Se evaluaron las complicaciones postoperatorias inherentes a la técnica quirúrgica, haciendo especial énfasis en la fístula pancreática. Un total de 27 pacientes durante el período 2003 a 2007 fueron sometidos a duodenopancreatectomía cefálica en forma electiva por etiología maligna tales como: ampuloma II casos, colangiocarcinoma distal 2 casos, cáncer de páncreas 12 casos, tumor neuroendocrino 1 caso, adenoma velloso malignizado de colédoco 1 caso. Las edades estuvieron comprendidas entre 42-81 años con promedio de 61 años, predominantemente del sexo femenino en una proporción 4:1. Entre las complicaciones posquirúrgicas relacionadas con la técnica encontramos 4 casos (14.8 por ciento) dado por: 2 fístulas pancreáticas (una de ellas por la ligadura accidental del stent intraductal), 1 dehiscencia de la duodenopancreatectomía, siendo la causa de muerte, y finalmente un caso de atonía gástrica manejado médicamente en forma satisfactoria. La anastomosis ducto-serosa en el manejo del remanente pancreático del Whipple constituye una técnica de fácil ejecución y con buenos resultados, ya que ofrece menos morbimortalidad.

Manifestaciones anatomoclínicas de las enfermedades cutáneas metabólicas y sistémicas misceláneas: Parte II / Anatomoclinical manifestations of metabolic and systemic diseases: Part II

Numerosas enfermedades sistémicas y metabólicas tienen manifestaciones cutáneas, muchas de estas manifestaciones pueden favorecer su diagnóstico. Dado el gran número de estas patologías, esta revisión no pretende ser un análisis exhaustivo de todas ellas, sino que presenta un análisis clínico-patológico de algunas enfermedades metabólicas y sistémicas seleccionadas.

Numerous systemic and metabolic diseases have coetaneous manifestations, many of these manifestations can favor diagnosis Due to the great number of these conditions, this review does not try to be a comprehensive analysis of all of them, but present a clinicopathological analysis of some selected metabolic and systemic diseases.

Doença de Whipple / Whipple's disease

Os autores apresentam uma revisão sobre a doença de Whipple, uma rara enfermidade causada pelo bacilo Gram-positivo Tropheryma whipplei, que cursa, principalmente, com manifestações gastrintestinais - entre as quais síndrome de má absorção -, musculoesqueléticas, com artralgia, envolvimento do sistema nervoso central e febre baixa, podendo ser fatal se não tratada. O diagnóstico é confirmado pela biópsia do duodeno ou de outros órgãos envolvidos e(ou) pela detecção do agente pela utilização da reação de polimerase em cadeia (PCR). A terapia consiste em antibioticoterapia, devendo ser buscada a confirmação da cura com nova biópsia.

Doença de Whipple: relato de caso / Whipple's disease: a case report

A doença de Whipple é uma doença infecciosa causada pelo bacilo Tropheryma whippelii, caracterizada pela sua apresentação inespecífica, o que dificulta o diagnóstico que, realizado através de biópsia da mucosa do jejuno e ¡leo. Os autores relatam um caso de paciente com doença de Whippie que foi submetido a inúmeros procedimentos até que se chegasse a um diagnóstico definitivo.

Hypersomnia in Whipple disease: case report / Hipersónia na doença de Whipple: relato de caso

Whipple disease (WD) is a rare systemic infection caused by Tropheryma whippelii. Neurological involvement has been recognised in 40 percent of patients, either as initial manifestations or during the course of the disease. We report on a 45 years-old man with WD with initial, persistent and irresistible episodes of daytime somnolence. The patient was HLA-DQB1*0602 positive (genetic marker for narcolepsy). WD diagnosis was suspected on clinical and MRI basis and confirmed by histological and immunohistochemical study of duodenal biopsy. Forty months later all clinical features improved, narcoleptic-like episodes disappeared and cerebral MRI and CSF normalised. Longitudinal neurophysiological studies revealed persistent sleep pattern abnormalities with sleep fragmentation, paucity of slow wave and of REM sleep. The disruption of the hypocretin circuitry in the hypothalamic - diencephalic region triggered by the infection was the probable cause of the hypersomnia and narcopleptic symptoms. WD should be added to the list of causes of secondary hypersomnia.

A doença de Whipple (DW) é infecção sistémica rara causada pelo Tropheryma whippelii. Cerca de 40 por cento dos doentes apresentam envolvimento neurológico, seja como manifestação inicial da doença, seja durante o seu curso. Apresentamos o caso de um homem de 45 anos com doença de DW com episódios iniciais, persistentes e irresistíveis de sonolência durante a actividade diurna. O doente era positivo para o HLA-DQB1*0602 (marcador genético de narcolepsia). A suspeita do diagnóstico de DW foi levantada com base na clínica e RM e confirmada por estudo imunocitoquímico do material de biópsia jejunal. Quarenta meses mais tarde, todas as manifestações clínicas melhoraram, os episódios narcolépticos desapareceram, e a RM e o LCR normalizaram. Os estudos neurofisiológicos seriados do sono revelaram alterações persistentes caracterizadas por fragmentação do sono, escassez de ondas lentas e sono REM. A perturbação do circuito da hipocretina na região hipotálamo-diencefálica, causada pela infecção, foi a causa provável da hipersónia num doente geneticamente susceptível. A DW deve ser incluída nas causas de hipersónia secundária.

Tratamento cirúrgico do insulinoma / Surgical treatment of insulinoma

O insulinoma é o tumor pancreático mais frequente (60%), mais comum no sexo feminino, geralmente são pequenos e solitários. Cerca de 90% desses tumores são benignos e 4% podem estar associados à síndrome endócrina múltipla tipo I. O diagnóstico clínico destes tumores se faz principalmente através da tríade de Whipple, caracterizada por hipoglicemia de jejeum, níveis de glicemia inferiores a 50 mg/dl e alívio dos sintomas após após administração de glicose. Vários métodos tem sido propostos para a localização desses tumores, como a ultrassonografia, tomografia computadorizada helicoidal, ressonância nuclear magnética, angiografia mesentérica e a ultrassonografia endoscópica, considerada o melhor método diagnóstico pré-operatório. Porém, com sensibilidade em torno de 83 a 100% está a palpação digital associada à ultrassonogradia intra-operatória. O tratamento desses tumores é eminentemente cirúrgico e a escolha da técnica cirúrgica deve ser definida com base na localização das lesões.

Doença de Whipple: relato de caso ­ recentes avanços no diagnóstico e tratamento / Whipple's disease: case report - recent advances in diagnosis and treatment

Os autores relatam um caso de Doença de Whipple em paciente do sexo masculino, de 55 anos, que apresentava predominância de sintomas gastrintestinais e importante comprometimento do estado geral, caquexia e anemia. O diagnóstico foi confirmado por exame histopatológico. Após o tratamento com antibióticos, observou-se excelente evolução clínica. Salientam-se a importância e a atualidade do diagnóstico, visto que, apesar de doença pouco frequente, é potencialmente fatal e responde drasticamente ao tratamento com antibióticos.

Whipple's disease with neurological manifestions: case report

A doença de Whipple (DW) é distúrbio multissistêmico raro causado pelo bacilo Tropheryma whipplei. O envolvimento do sistema nervoso central é um aspecto clássico da doença, sendo observado em 20 a 40% dos pacientes. Relatamos o caso de homem de 62 anos com DW que desenvolveu manifestações neurológicas durante sua evolução, com o objetivo de discutir os sinais e sintomas mais comuns e destacar os critérios diagnósticos e propostas terapêuticas mais recentes.