RESUMO
Abstract Primary intestinal lymphangiectasia (PIL) is a rare disease that was discovered in 1961 by Waldmann et al., who first described the concept of intestinal lymphangiectasia. PIL is manifested by large and dilated intestinal lymphatic vessels that lead to the loss of lymphatic fluid to the intestinal lumen, including severe hypoalbuminemia and, consequently, generalized edema. The etiology of PIL is unknown; however, several genes are involved in the development of the lymphatic system. The case of a two-month-old patient with a prenatal diagnosis of ascites on third-trimester ultrasound was presented. The patient was born with rapidly progressive ascites and showed an endoscopic study with evidence of snowflake images and histology compatible with intestinal lymphangiectasia. The patient remained on octreotide infusion at baseline and subsequently with refractory ascites the dose was increased. Several evacuatory paracentesis were performed and he remained on enteral nutrition based on an extensively hydrolyzed formula with a 54% contribution of medium chain triglycerides (MCTs) and parenteral nutrition (PN). Subsequently, the patient was switched to a subcutaneous infusion of octreotide, which allowed hospital discharge and adequate outpatient follow-up with a favorable evolution.
Resumen La linfangiectasia intestinal primaria (LIP) es una enfermedad rara que se descubrió en 1961 por Waldmann y colaboradores, quienes describieron por primera vez el concepto de linfangiectasia intestinal. La LIP se manifiesta por vasos linfáticos intestinales grandes y dilatados que conllevan la pérdida de líquido linfático hacia la luz intestinal, siendo responsable de una hipoalbuminemia grave y, en consecuencia; de un edema generalizado. La etiología de la LIP es desconocida, sin embargo, varios genes están implicados en el desarrollo del sistema linfático. Se presenta el caso de una paciente de dos meses de edad con diagnóstico prenatal de ascitis en ecografía de tercer trimestre. Nació con ascitis rápidamente progresiva y presentó un estudio endoscópico con evidencia de imágenes en copos de nieve e histología compatible con linfangiectasia intestinal. Se mantuvo en tratamiento con infusión de octreotida al inicio y posteriormente con ascitis refractaria se aumentó la dosis. Se realizaron varias paracentesis evacuatorias y permaneció con nutrición enteral a base de una fórmula extensamente hidrolizada con aporte del 54% de triglicéridos de cadena media (TCM) y nutrición parenteral (NPT). Más tarde, se cambió a infusión de octreotida por vía subcutánea, lo que permitió el alta hospitalaria y un seguimiento adecuado por consulta externa con una evolución favorable.
RESUMO
Intestinal lymphangiectasia is a group of rare diseases characterized by dilation of lymphatic channels. Its pathophysiology comprises obstruction of small bowel lymphatic drainage with secondary dilation of mucosal, submucosal, or subserous lymphatic vessels, distorting villous architecture and causing loss of lymph into the intestinal lumen, leading to malabsorption. The affected lymphatic vessels are primarily located in the small intestine, which is affected to a varying extent. Its etiology is still unknown. The following report presents a rare case of intestinal lymphangiectasia in an adult patient.
RESUMO
La mucopolisacaridosis tipo III B es una enfermedad de depósito lisosomal causada por la deficiencia de la enzima N-acetil-alfa-d-glucosaminidasa, implicada en el catabolismo del heparán sulfato, que produce su acúmulo en diversos tejidos. Se presenta a un paciente de 8 años, afectado de mucopolisacaridosis tipo III B, con historia de diarrea crónica y hallazgos endoscópicos e histológicos compatibles con linfangiectasia intestinal. Tras tratamiento dietético con restricción de ácidos grasos de cadena larga y rica en triglicéridos de cadena media, presentó mejoría clínica, mantenida hasta la actualidad.La patogenia de la diarrea crónica en pacientes con mucopolisacaridosis tipo III B es aún desconocida. Debe investigarse la presencia de linfangiectasia intestinal en estos pacientes e iniciar, en caso de confirmarse, un tratamiento dietético adecuado para mejorar así su calidad de vida.
Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with medium-chain triglyceride, our patient presents clinical improvement until today. The pathogenesis of chronic diarrhea in patients with mucopolysaccharidosis type IIIB is still unknown. The presence of intestinal lymphangiectasia in these patients should be investigated, and appropriate dietary treatment should be initiated, if confirmed, to improve their quality of life.
Assuntos
Humanos , Masculino , Criança , Linfangiectasia Intestinal/diagnóstico por imagem , Doenças por Armazenamento dos Lisossomos , Mucopolissacaridose III , Dieta com Restrição de Gorduras , Diarreia , Linfangiectasia Intestinal/terapiaRESUMO
Mucopolysaccharidosis type IIIB is a lysosomal storage disease caused by a deficiency of the N-acetyl-alpha-d-glucosaminidase enzyme involved in the catabolism of heparan sulfate, causing its accumulation in various tissues. We present an 8-year-old patient with mucopolysaccharidosis type IIIB, with a history of chronic diarrhea and endoscopic and histological findings compatible with intestinal lymphangiectasia. After a dietary treatment with a low-fat diet supplemented with mediumchain triglyceride, our patient presents clinical improvement until today. The pathogenesis of chronic diarrhea in patients with mucopolysaccharidosis type IIIB is still unknown. The Linfangiectasia intestinal en un paciente afectado de síndrome de Sanfilippo B Intestinal lymphangiectasia in a patient with Sanfilippo B syndrome presence of intestinal lymphangiectasia in these patients should be investigated, and appropriate dietary treatment should be initiated, if confirmed, to improve their quality of life.
La mucopolisacaridosis tipo III B es una enfermedad de depósito lisosomal causada por la deficiencia de la enzima N-acetil-alfad- glucosaminidasa, implicada en el catabolismo del heparán sulfato, que produce su acúmulo en diversos tejidos. Se presenta a un paciente de 8 años, afectado de mucopolisacaridosis tipo III B, con historia de diarrea crónica y hallazgos endoscópicos e histológicos compatibles con linfangiectasia intestinal. Tras tratamiento dietético con restricción de ácidos grasos de cadena larga y rica en triglicéridos de cadena media, presentó mejoría clínica, mantenida hasta la actualidad. La patogenia de la diarrea crónica en pacientes con mucopolisacaridosis tipo III B es aún desconocida. Debe investigarse la presencia de linfangiectasia intestinal en estos pacientes e iniciar, en caso de confirmarse, un tratamiento dietético adecuado para mejorar así su calidad de vida.
Assuntos
Mucopolissacaridose III , Acetilglucosaminidase , Criança , Diarreia/etiologia , Heparitina Sulfato , Humanos , Mucopolissacaridose III/complicações , Mucopolissacaridose III/diagnóstico , Qualidade de VidaRESUMO
Lymphangiectasia is a heterogenous inflammatory bowel disease characterized by lymphatic vessel dilation, chronic diarrhea and protein loss such as serum albumin and globulin. The most common cause of lymphangiectasia is considered to be the congenital malformation of the lymphatics. The study was conducted between 2012-2015 on 76 dogs suffering from intestinal disorders and manifesting digestive symptoms such as diarrhea or weight loss. In order to assess the origin of disorder, physical examination, biochemistry profile, ultrasound and endoscopic examinations were performed. Ultrasound examination tried to assess the changes of intestines' echogenicity, changes in wall thickness, wall layering and presence of striations or / and speckles (hyperechoic structures along intestinal mucosal layer). Endoscopic examination ï¬ndings included dilated lacteals (59.2%) and erythema (21.1%). Although increased friability was observed in 33 dogs, it was not considered in the study due to limitations represented by the evaluation of the endoscopic images only. The study proved that an extremely significant statistical correlation exists between the presence of speckles and dilated lacteals in dogs with lymphangiectasia (P<0.05). Up to now, there is no other study to make an association between the white spots observed in ultrasound examination and dilated lacteals revealed after endoscopy in dogs with intestinal lymphangiectasia.(AU)
A linfangiectasia é uma doença inflamatória intestinal heterogênea, caracterizada por dilatação dos vasos linfáticos, diarreia crônica e perda de proteínas, como albumina sérica e globulina. A causa mais comum de linfangiectasia é considerada a malformação congênita dos linfáticos. O presente estudo foi realizado entre 2012 e 2015, em 76 cães que sofrem de distúrbios intestinais e manifestam sintomas digestivos, como diarreia ou perda de peso. Para avaliar a origem do distúrbio, foram realizados exame físico, perfil bioquímico, ultrassonográfico e endoscópico. O exame ultrassonográfico tentou avaliar as alterações da ecogenicidade do intestino, as alterações na espessura da parede, a estratificação e a presença de estrias e / ou de manchas (estruturas hiperecoicas ao longo da camada mucosa intestinal). Os resultados do exame endoscópico incluíram lacteais dilatadas (59,2%) e eritema (21,1%). Embora tenha sido observada maior friabilidade em 33 cães, ela não foi considerada no estudo devido às limitações representadas pela avaliação apenas das imagens endoscópicas. O estudo demonstrou que existe uma correlação estatística extremamente significativa entre a presença de manchas e lacteais dilatadas em cães com linfangiectasia (P <0,05). Até o momento, não há outro estudo para associar as manchas brancas observadas no exame ultrassonográfico e lacteais dilatadas reveladas após endoscopia em cães com linfangiectasia intestinal.(AU)
Assuntos
Animais , Cães , Linfangiectasia Intestinal/veterinária , Linfangiectasia Intestinal/diagnóstico por imagem , Endoscopia do Sistema Digestório/veterinária , Ultrassonografia/veterináriaRESUMO
Resumo A linfangiectasia intestinal consiste em um grupo de doenças raras caracterizadas pela dilatação dos canais linfáticos. A fisiopatologia compreende a obstrução da drenagem linfática do intestino delgado com dilatação secundária dos vasos linfáticos mucosos, submucosos ou subserosos, que distorcem a arquitetura das vilosidades e conduzem à perda de linfa para a luz intestinal, levando à má absorção. Os vasos linfáticos afetados localizam-se primariamente no intestino delgado, que é atingido em extensão variável. A sua etiologia é ainda desconhecida. O relato a seguir apresenta um raro caso de linfangiectasia intestinal em paciente adulto.
Abstract Intestinal lymphangiectasia is a group of rare diseases characterized by dilation of lymphatic channels. Its pathophysiology comprises obstruction of small bowel lymphatic drainage with secondary dilation of mucosal, submucosal, or subserous lymphatic vessels, distorting villous architecture and causing loss of lymph into the intestinal lumen, leading to malabsorption. The affected lymphatic vessels are primarily located in the small intestine, which is affected to a varying extent. Its etiology is still unknown. The following report presents a rare case of intestinal lymphangiectasia in an adult patient.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Vasos Linfáticos/fisiopatologia , Intestino Delgado/fisiopatologia , Linfangiectasia Intestinal/fisiopatologia , Doenças Raras , Linfangiectasia Intestinal/diagnóstico , Linfangiectasia Intestinal/dietoterapiaRESUMO
Introducción: La linfangiectasia renal es una afección infrecuente en la que existe dilatación de los vasos linfáticos renales. Usualmente es bilateral y de buen pronóstico. Objetivos: Presentar un caso de linfangiectasia renal bilateral, sus características clínicas y aspectos del diagnóstico imaginológico. Caso clínico: Paciente femenina de 59 años de edad, hipertensa controlada, que asistió a la consulta externa de urología por dolor lumbar bilateral, de moderada intensidad y coloración rojiza de la orina, ambos de forma intermitente. El examen físico general y regional fue negativo. Se confirmó microhematuria en el examen general de la orina. Los estudios de la analítica sanguínea fueron normales. El ultrasonido renal reportó pielocaliectasia bilateral moderada y quistes parapiélicos, bilaterales, a predominio izquierdo. La tomografía axial computarizada renal contrastada, reveló la presencia de formaciones hipodensas renales bilaterales, de aspecto quístico parapiélicas y en el seno renal, compatibles con linfangiectasia bilateral. La paciente ha evolucionado favorablemente. Conclusiones: La linfangiectasia renal se debe tener en cuenta en el diagnóstico deferencial de la enfermedad quística renal. Para el diagnóstico imaginológico de certeza es esencial la tomografía axial computarizada renal contrastada(AU)
Introduction: The renal linfangiectasia is an uncommon, in which dilatation of the lymphatic renal vessels exists. It is usually bilateral and of good presage. Objectives: To present a case of renal bilateral lymphangiectasia, their clinical characteristics and aspects of the imaginologic diagnosis. Clinical case: Patient feminine of 59 years of age, with controlled hypertension that attended the external consultation of Urology for lumbar bilateral pain, of moderate intensity, and reddish coloration of the urine, both in an intermittent way. The general and regional physical exam was negative. Microhematuria was confirmed in the general exam of the urine. The sanguine studies of the analytic one were all normal ones. The renal ultrasound reported bilateral moderate pielocaliectasia and parapielic cysts, with left prevalence. The renal contrasted computed tomography revealed the presence of bilateral renal hipodenses formations, of aspect cystic parapielic and in the renal sinus, and compatible with bilateral linfangiectasia. The patient has evolved favorably. Conclusions: The renal linfangiectasia it should be kept in mind in the deferential diagnosis of the cystic renal illness. For the imaginologic diagnostic of certainty it is essential the renal contrasted computed tomography(AU)
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Anormalidades Congênitas , Tomografia Computadorizada por Raios X/métodos , Rim/anormalidades , Linfangiectasia/diagnóstico por imagem , Linfangioma/diagnósticoRESUMO
Presentamos el caso de lactante de 6 meses de edad referido por aumento progresivo de perímetro abdominal. A la valoración con datos de ascitis. La paracentesis diagnóstica muestra líquido ascítico quiloso. El diagnóstico de linfangiectasia se realizó a través de endoscopia digestiva alta, con biopsia intestinal
We present the case of 6-month-old infants referred for ascites. The paracentesis shows uid of chilosas characteristics. The diagnosis of lymphangiectasia was made through upper digestive endoscopy, with intestinal biopsy
RESUMO
La linfangiectasia intestinal primaria en una patología infrecuente causada por la malformación de los conductos linfáticos intestinales. Normalmente se diagnostica antes de los 3 años de edad, pero puede aparecer en población adulta. Los síntomas más frecuentemente encontrados son la presencia de anasarca y dolor abdominal acompañado de malnutrición. El tratamiento es sintomático y se basa en la sustitución dietética de los triglicéridos de cadena larga por triglicéridos de cadena mediana con aumento del aporte proteico. En este trabajo se reporta el caso de una paciente femenina de 22 años de edad la cual presenta manifestación clínicas, imaginológicas y anatomopatológicas que permiten realizar el diagnóstico de una linfangiectasia intestinal primaria, caso extremadamente infrecuente y sobre todo a esta edad(AU)
Primary intestinal lymphangiectasia in an uncommon pathology caused by malformation of the intestinal lymphatic ducts. It is usually diagnosed before 3 years of age, but may appear in the adult population. The most frequent symptoms are the presence of anasarca and abdominal pain accompanied by malnutrition. The treatment is symptomatic and is based on the dietary substitution of long chain triglycerides by medium chain triglycerides with increased protein intake. This paper reports the case of a female patient of 22 years of age who presents clinical, imaging and anatomopathological manifestations that allow the diagnosis of primary intestinal lymphangiectasia, an extremely rare case, especially at this age(AU)
Assuntos
Humanos , Feminino , Adulto , Espironolactona/uso terapêutico , Enteroscopia de Duplo Balão/métodos , Furosemida/uso terapêutico , Linfangiectasia Intestinal/diagnóstico por imagemRESUMO
El sangrado digestivo oscuro constituye entre 10 y 20% de todas las causas de sangrado digestivo. La etiología está claramente relacionada con el grupo etario en que se presente. Las linfangiectasias del yeyuno son una causa rara de sangrado digestivo, pero puede llegar a ser de alta mortalidad en caso de que no se detecte tempranamente. Reportamos un caso de un paciente joven sin historia previa de enfermedad, con sangrado digestivo manifestado por melenas, con anemización severa, con estudios endoscópicos iniciales normales, en quien hacemos abordaje con estudios para intestino delgado (video cápsula endoscópica y enteroscopia anterógrada de doble balón), con los que hacemos el diagnóstico y es llevado a cirugía, con adecuada evolución clínica y curación de la enfermedad. (Acta Med Colomb 2015; 40: 158-161).
Obscure gastrointestinal bleeding is between 10 to 20% of all causes of gastrointestinal bleeding. The etiology is clearly related to the age group in which it is presented. Lymphangiectasia in jejunum is a rare cause of gastrointestinal bleeding, but can have high mortality if not detected early. A case of a young patient with no history of previous disease presenting gastrointestinal bleeding manifested by melaena, with severe anemia and normal initial endoscopic studies, is presented. The diagnosis was made by endoscopic video capsule and antegrade double-balloon enteroscopy and surgery was performed with adequate clinical evolution and cure of the disease. (Acta Med Colomb 2015; 40: 158-161).
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Linfangiectasia , Cápsulas Endoscópicas , Enteroscopia de Duplo Balão , Hemorragia , Intestino DelgadoRESUMO
Firstly described by Rudolf Virchow in the 19th century, congenital generalized lymphangiectasia is a rare entity characterized by dilation of lymphatic vessels, and was recently classified in primary or secondary lymphangiectasia. Generalized forms may be diagnosed during pre-natal follow-up with ultrasound examination, and, depending on its severity, the newborn outcome is very poor. The authors report the case of a female newborn with a previous diagnosis of fetal hydropsy who was born after a full-term gestation with respiratory failure due to bilateral voluminous pleural effusion and ascites. Physical examination also disclosed syndromic facies. Despite all efforts of the intensive supportive care, the patient died after 24 days of life. The autopsy findings were consistent with the diagnosis of congenital pulmonary lymphangiectasia. The authors call attention to this rare diagnosis in patients with cavitary effusion and respiratory insufficiency at birth.
RESUMO
BACKGROUND: Primary intestinal lymphangiectasia is a rare congenital disease described by Waldmann in 1961 that is a consequence of obstruction of the lymphatic drainage of the small bowel with secondary lymph vessel dilation. This distorts the architecture of the villi and causes a leakage of lymph into the intestinal lumen, resulting in protein-losing enteropathy and malabsorption. AIM: To describe the clinical, biochemical, radiologic, endoscopic, and histologic characteristics in children with primary intestinal lymphangiectasia. METHOD: A retrospective observational, descriptive, cross-sectional study was conducted that reviewed the case records of children diagnosed with primary intestinal lymphangiectasia that were seen at the Department of Gastroenterology and Nutrition of the Instituto Nacional de Pediatría within the time frame of January 1, 1992 to September 30, 2012. RESULTS: Four patients were found that presented with primary intestinal lymphangiectasia. Three of them had been diagnosed before 3 years of age. All the patients presented with chronic diarrhea, edema, lymphopenia, hypocalcemia, and hypogammaglobulinemia, and 3 patients presented with hypocholesterolemia. Bowel transit time, endoscopy, and intestinal biopsies were characteristic of this pathology. CONCLUSIONS: Intestinal lymphangiectasia should be suspected when there is a clinical picture of chronic diarrhea and protein-losing enteropathy accompanied with edema at any level, as well as hypoalbuminemia, hypocalcemia, lymphopenia, hypogammaglobulinemia, and hypocholesterolemia, which are the main biochemical findings of this pathology. All children presenting with intestinal lymphangiectasia should undergo an upper gastrointestinal series with bowel transit time and endoscopy with biopsies taken at the level of the duodenum. Treatment includes diet and the periodic administration of albumin and gamma globulin.
Assuntos
Linfangiectasia Intestinal/terapia , Centros de Atenção Terciária , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Diarreia/diagnóstico , Diarreia/etiologia , Dieta , Endoscopia Gastrointestinal , Feminino , Humanos , Lactente , Linfangiectasia Intestinal/complicações , Linfangiectasia Intestinal/diagnóstico , Masculino , México , Estudos RetrospectivosRESUMO
A propósito del caso de un paciente de 7 meses de vida remitido desde Yopal a la ciudad de Bogotá, se revisa el tema de la linfagiectasia intestinal. Esta es una rara enfermedad que involucra los vasos linfáticos intestinales, y origina hipoproteinemia, edemas, ascitis y enteropatía perdedora de proteínas.
This is the case report of a 7 month old child from Yopal with intestinal lymphangiectasia who was sent to Bogota. We also review the issue of intestinal lymphangiectasia, a rare disease involving intestinal lymphatic vessels which caused hypoproteinemia, edema, ascites and protein-losing enteropathy.
Assuntos
Humanos , Masculino , Recém-Nascido , Ascite , Hipoproteinemia , Linfangiectasia Intestinal , Enteropatias Perdedoras de ProteínasRESUMO
La linfangiectasia intestinal primaria es una enfermedad caracterizada por dilatación de los vasos linfáticos del intestino, manifestándose como una enteropatía perdedora de proteínas. Presentamos un paciente con diarrea crónica, prolapso rectal recurrente y hemihipertrofia de miembro superior izquierdo, asociado a linfopenia e hipoalbuminemia. Por endoscopia digestiva superior y biopsia se diagnostica linfangiectasia intestinal primaria y se inicia tratamiento nutricional exitosamente.
Primary intestinal lymphangiectasia is a disease characterized by dilated intestinal lymph vessels which manifests as a protein losing enteropathy. We present a patient with chronic diarrhea, recurrent rectal prolapse and left upper limb hemihypertrophy associated with lymphopenia and hypoalbuminemia. Primary intestinal lymphangiectasia was diagnosed with upper endoscopy and biopsy. Nutritional treatment was successfully begun.
Assuntos
Humanos , Masculino , Pré-Escolar , Linfangiectasia Intestinal , Terapia Nutricional , Enteropatias Perdedoras de ProteínasRESUMO
Linfangiectasia renal é uma condição rara, caracterizada por coleções parapiélicas e perirrenais, que pode progredir de assintomática para insuficiência renal crônica. É apresentado um caso de achado incidental em tomografia computadorizada de linfangiectasia renal bilateral em paciente assintomático, com descrição dos principais achados à luz dos métodos de imagem e ampla revisão da literatura.
Renal lymphangiectasia is a rare condition characterized by parapyelic and perirenal fluid collections, which may progress from asymptomatic condition to chronic renal failure. The present report describes a case of incidental computed tomography finding of bilateral lymphangiectasia in an asymptomatic patient, as well as the main imaging findings with a comprehensive literature review.
Assuntos
Idoso , Cistos , Diagnóstico Diferencial , Rim , Sistema Linfático , Linfangiectasia/diagnóstico , Tórax , Dispneia , Tomografia Computadorizada por Raios X , UltrassonografiaRESUMO
O quilotórax corresponde ao acúmulo de fluido linfático em um ou em ambos os espaços pleurais, resultante do extravasamento de linfa do ducto torácico ou de uma de suas divisões principais. Este pode estar associado à ascite quilosa em pacientes com distúrbios linfáticos sistêmicos, como na linfangiectasia, em decorrência de defeitos no transporte linfático-venoso do quilo da cavidade torácica para a circulação sistêmica. A linfangiectasia intestinal é um distúrbio obstrutivo que envolve o sistema linfático do órgão e revela-se como a causa mais comum de enteropatias associadas à má absorção e perda de proteínas em cães. São apresentados no presente relato o controle clínico com o uso de dieta caseira com baixa gordura e a quantificação da função digestiva, por meio da determinação da digestibilidade do alimento, em um cão acometido por quilotórax secundário à linfangiectasia intestinal. A dieta caseira incluiu arroz, peito de frango, cenoura, carbonato de cálcio, levedura de cerveja, suplemento vitamínico e mineral e sal. O ensaio de digestibilidade foi conduzido pelo método de coleta total de fezes. A dieta caseira foi eficaz em interromper o acúmulo de efusão quilosa torácica, restabelecer as concentrações de proteína total e albumina e promover ganho de peso no paciente. Os resultados da digestibilidade demonstraram menor aproveitamento de todos os nutrientes no cão com linfangiectasia em relação ao controle sadio.(AU)
Chylothorax corresponds to accumulation of lymphatic fluid in one or both pleural spaces, resulting from the leak of thoracic duct or of one of their main divisions. This can be associated to chylous ascites in patients with systemic lymphatic disturbances, as in lymphangiectasia, due to defects in the lymphatic-veined transport of chylo from thoracic cavity to systemic circulation. Intestinal lymphangiectasia is an obstructive disturbance that involves lymphatic system of gastrintestinal tract and its the more common cause of enteropathies associated to reduced-absorption and protein loss in dogs. A clinical management of a dog assaulted by chylothorax secondary to intestinal lymphangiectasia is described. A homemade low fat diet was used, and the quantification of digestive function, through the determination of diets digestibility, was performed. The homemade diet included rice, chicken chest, carrot, calcium carbonate, yeast, vitamin and mineral supplement and salt. The digestibility assay was carried out by total feces collection method. Homemade diet was efficient in stop recurrence of chylous thoracic effusion accumulation, recovery of serum concentrations of total protein and albumin, and promotes weight gain. Compared to the healthy control dog, animal with lymphangiectasia showed reduced digestibility of all analyzed nutrients.(AU)
Assuntos
Animais , Cães , Cães , Quilotórax/veterinária , Linfangiectasia/veterinária , Dieta com Restrição de Gorduras/veterinária , Fenômenos Fisiológicos da Nutrição , Fenômenos Fisiológicos da Nutrição AnimalRESUMO
O quilotórax corresponde ao acúmulo de fluido linfático em um ou em ambos os espaços pleurais, resultante do extravasamento de linfa do ducto torácico ou de uma de suas divisões principais. Este pode estar associado à ascite quilosa em pacientes com distúrbios linfáticos sistêmicos, como na linfangiectasia, em decorrência de defeitos no transporte linfático-venoso do quilo da cavidade torácica para a circulação sistêmica. A linfangiectasia intestinal é um distúrbio obstrutivo que envolve o sistema linfático do órgão e revela-se como a causa mais comum de enteropatias associadas à má absorção e perda de proteínas em cães. São apresentados no presente relato o controle clínico com o uso de dieta caseira com baixa gordura e a quantificação da função digestiva, por meio da determinação da digestibilidade do alimento, em um cão acometido por quilotórax secundário à linfangiectasia intestinal. A dieta caseira incluiu arroz, peito de frango, cenoura, carbonato de cálcio, levedura de cerveja, suplemento vitamínico e mineral e sal. O ensaio de digestibilidade foi conduzido pelo método de coleta total de fezes. A dieta caseira foi eficaz em interromper o acúmulo de efusão quilosa torácica, restabelecer as concentrações de proteína total e albumina e promover ganho de peso no paciente. Os resultados da digestibilidade demonstraram menor aproveitamento de todos os nutrientes no cão com linfangiectasia em relação ao controle sadio.
Chylothorax corresponds to accumulation of lymphatic fluid in one or both pleural spaces, resulting from the leak of thoracic duct or of one of their main divisions. This can be associated to chylous ascites in patients with systemic lymphatic disturbances, as in lymphangiectasia, due to defects in the lymphatic-veined transport of chylo from thoracic cavity to systemic circulation. Intestinal lymphangiectasia is an obstructive disturbance that involves lymphatic system of gastrintestinal tract and its the more common cause of enteropathies associated to reduced-absorption and protein loss in dogs. A clinical management of a dog assaulted by chylothorax secondary to intestinal lymphangiectasia is described. A homemade low fat diet was used, and the quantification of digestive function, through the determination of diets digestibility, was performed. The homemade diet included rice, chicken chest, carrot, calcium carbonate, yeast, vitamin and mineral supplement and salt. The digestibility assay was carried out by total feces collection method. Homemade diet was efficient in stop recurrence of chylous thoracic effusion accumulation, recovery of serum concentrations of total protein and albumin, and promotes weight gain. Compared to the healthy control dog, animal with lymphangiectasia showed reduced digestibility of all analyzed nutrients.
RESUMO
The clinical and histopathological aspects of a case of intestinal lymphangiectasia are described in a 5 years old Dobermann dog. The symptoms associated with the process were: persistent diarrhea resistent to theraphy, and ascites. The integrity of the abdominal organs, lymphatic vessels and cistema chyli were determined through exploratory laparotomy. The main histopathological alterations observed in a fragment of the small intestine were: flattening* of the villus, ectasis of the lymphatic vessels and pools of histiocytic cells and lymphocytes in the submucous. The radiograph of the chest revealed both the presence of a denser image localized at the base of the heart near the lung hilus and pleural effusion. The analyses of the fluid revealed large amounts of protein and lymphocytes. The clinical observations, the laboratory data and the histopathological alterations of the small intestine lead to the conclusion the animal had intestinal lymphangiectasia, caused by a compressive process in the thoracic duct, probably a tumoral formation of lymphoid origin localized in the mediastinum.
Descrevem-se os aspectos clínicos e histopatológicos de um caso de linfangiectasia intestinal em um cão de raça Dobermann, fêmea de 5 anos de idade. Os sintomas associados ao processo foram diarreia persistente, refratária a qualquer terapia e ascite. Através da laparotomia exploratória pôde-se constatar a integridade dos órgãos abdominais, dos vasos linfáticos e da cisterna do quilo. O encurtamento das vilosidades, vasos linfáticos ectásicos e agrupamentos de células histióides e linfócitos na submucosa foram as principais alterações histopatológicas observadas no fragmento do intestino delgado. O exame radiológico do tórax revelou a presença de uma formação arredondada (de imagem mais densa), localizada na base do coração, ao nível do hilo pulmonar e a presença de líquido na cavidade pleural, líquido esse incolor, denso, de alto conteúdo proteico e rico em linfócitos. As observações clínicas, os dados laboratoriais e as alterações histopatológicas do intestino delgado permitem concluir ser este um caso de linfangiectasia intestinal, causada por processo compressivo de dueto torácico, provavelmente formação tu moral de origem linfoide, localizada na região mediastínica.
RESUMO
The clinical and histopathological aspects of a case of intestinal lymphangiectasia are described in a 5 years old Dobermann dog. The symptoms associated with the process were: persistent diarrhea resistent to theraphy, and ascites. The integrity of the abdominal organs, lymphatic vessels and cistema chyli were determined through exploratory laparotomy. The main histopathological alterations observed in a fragment of the small intestine were: flattening* of the villus, ectasis of the lymphatic vessels and pools of histiocytic cells and lymphocytes in the submucous. The radiograph of the chest revealed both the presence of a denser image localized at the base of the heart near the lung hilus and pleural effusion. The analyses of the fluid revealed large amounts of protein and lymphocytes. The clinical observations, the laboratory data and the histopathological alterations of the small intestine lead to the conclusion the animal had intestinal lymphangiectasia, caused by a compressive process in the thoracic duct, probably a tumoral formation of lymphoid origin localized in the mediastinum.
Descrevem-se os aspectos clínicos e histopatológicos de um caso de linfangiectasia intestinal em um cão de raça Dobermann, fêmea de 5 anos de idade. Os sintomas associados ao processo foram diarreia persistente, refratária a qualquer terapia e ascite. Através da laparotomia exploratória pôde-se constatar a integridade dos órgãos abdominais, dos vasos linfáticos e da cisterna do quilo. O encurtamento das vilosidades, vasos linfáticos ectásicos e agrupamentos de células histióides e linfócitos na submucosa foram as principais alterações histopatológicas observadas no fragmento do intestino delgado. O exame radiológico do tórax revelou a presença de uma formação arredondada (de imagem mais densa), localizada na base do coração, ao nível do hilo pulmonar e a presença de líquido na cavidade pleural, líquido esse incolor, denso, de alto conteúdo proteico e rico em linfócitos. As observações clínicas, os dados laboratoriais e as alterações histopatológicas do intestino delgado permitem concluir ser este um caso de linfangiectasia intestinal, causada por processo compressivo de dueto torácico, provavelmente formação tu moral de origem linfoide, localizada na região mediastínica.