ABSTRACT
Chyluria results from an abnormal connection between lymphatic bed and urinary tract, causing lymph leakage into the urine. The clinical picture often begins with the appearance of cloudy, milky urines accompanied by monolateral flank pain, malnutrition, weight loss and weakness. We report a case of chyluria that occurred in a young woman who was referred to our unit for nephrotic-range proteinuria. Before performing a renal biopsy, we found that urine analysis demonstrated a massive lipiduria. Therefore, we collected urine samples from each kidney with a selective ureteral catheterization, demonstrating a monolateral source of lipids and proteins. We suspended the renal biopsy and performed a lymphography that showed an inherited lymphangioma on the left lumbar lymphatic bed. Sclerosing solution instillation, renal pedicle lymphatic disconnection or laser therapy are invasive therapeutical options that may cause severe adverse effects. Instead of these procedures, a conservative therapy based on a low-fat diet supplemented with medium-chain triglycerides was chosen. This dietetic schedule was followed by complete resolution of proteinuria and lipiduria. The patient progressively gained body weight and improved quality of life. No relapses were observed after 3 years of follow-up. This case emphasizes the possible role of a noninvasive therapeutical option for patients with chyluria.
Subject(s)
Chyle , Lymphangioma/diagnosis , Nephrotic Syndrome/diagnosis , Urinalysis , Adult , Biopsy , Diet, Fat-Restricted , Female , Humans , Lymphangioma/complications , Lymphangioma/congenital , Lymphangioma/diet therapy , Lymphangioma/pathology , Lymphangioma/urine , Magnetic Resonance Imaging , Malnutrition/etiology , Nephrotic Syndrome/diet therapy , Nephrotic Syndrome/etiology , Nephrotic Syndrome/pathology , Nephrotic Syndrome/urine , Predictive Value of Tests , Proteinuria/etiology , Treatment Outcome , Triglycerides/administration & dosageABSTRACT
La linfangiomatosis musculoesquéletica ha sido descripta esporádicamente en la bibliografía radiológica y ortopédica. Este caso documenta el espectro imagenológico de una enfermedad poco frecuente y ayuda a identificar un potencial patrón diagnóstico. Se presenta una paciente de 35 años de edad que consulta por dolor dorsolumbar inespecífico, es estudiada mediante Rx simple, Tomografía Computada (TC) y Resonancia Magnética (RM). Los hallazgos radiológicos evidenciados permitieron confirmar la naturaleza benigna de la enfermedad. La linfangiomatosis musculoesquelética es una entidad benigna poco frecuente de etiología desconocida, que compromete a los vasos sanguíneos y linfáticos, produce lesiones quísticas difusas a nivel del esqueleto axial y apendicular y se acompaña de afectación visceral. El empleo de los distintos métodos de imágenes permite realizar el diagnóstico correcto (AU)
Subject(s)
Humans , Adult , Female , Lymphangioma/diagnosis , Fatty Acids/therapeutic use , Fractures, Spontaneous/etiology , Ascites/etiology , Pleural Effusion/etiology , Lymphangioma/diagnostic imaging , Lymphangioma/diet therapy , Lymphangioma/therapy , Lymphangioma/drug therapy , Chylous Ascites/etiology , Osteolysis/etiology , Chylothorax/etiology , Chylothorax/diet therapy , Muscle, Skeletal/pathology , Musculoskeletal Diseases , Magnetic Resonance Imaging , Tomography, X-Ray Computed , Dietary Proteins/therapeutic useABSTRACT
We encountered an unusually severe case of intra-abdominal lymphangiomatosis associated with protein-losing enteropathy and intestinal bleeding. A low-fat diet effectively raised the patient's serum levels of hemoglobin and the total serum protein, perhaps by inducing a reduction in intestinal lymph flow and pressure.
Subject(s)
Abdominal Neoplasms/complications , Gastrointestinal Hemorrhage/etiology , Intestine, Small/pathology , Lymphangioma/complications , Protein-Losing Enteropathies/etiology , Abdominal Neoplasms/diet therapy , Adult , Blood Proteins/analysis , Diet, Fat-Restricted , Female , Follow-Up Studies , Gastrointestinal Hemorrhage/diet therapy , Hemoglobins/analysis , Humans , Lymph/physiology , Lymphangioma/diet therapy , Pressure , Protein-Losing Enteropathies/diet therapyABSTRACT
Lymphangiomyomyoma is a tumor of lymphatic channels and lymph nodes, clinically manifested by recurrent chylous pleural effusions and ascites. The disease is usually progressive and unresponsive to surgery, chemotherapy, or irradiation. A case of lymphangiomyomatosis in a 24-year-old woman who presented with chylous ascites is described. The patient was treated with dietary fat restriction supplemented with medium chain triglycerides and has remained free of recurrent effusions for 2 years.
