Abstract/Text: Chyluria denotes the urinary excretion of chyle, which is a lymphatic fluid rich in chylomicrons. Chyle flows from the intestinal lacteals to the left subclavian vein through the thoracic duct. When an abnormal connection between these structures and the urinary tract develops, chyluria appears. The syndrome is often associated with a nephrotic-range proteinuria, and this could be a wrong indication to perform renal biopsy. Chyluria is classified as parasitic or nonparasitic, the former being induced by lymphatic filariasis, whereas the latter is caused by medical, traumatic or inherited diseases. The patient usually reports excretion of milky urines, monolateral flank pain, malnutrition, weight loss and weakness. Urinalysis demonstrates lymphocyturia associated with chylomicrons and triglycerides in the supernatant. The diagnostic approach is aimed to define the site of lymphourinary fistula. A selective ureteral catheterization allows to collect urine samples from each kidney, demonstrating a monolateral source of proteins and lipids and making renal biopsy superfluous. Other diagnostic tools include nuclear magnetic resonance urography and lymphoangiography. Many therapeutic options have been proposed. Sclerosing solution instillation into the renal pelvis and laparoscopic renal pedicle disconnection are the invasive procedures most commonly employed. Among the medical alternatives, a low-fat diet supplemented with medium-chain triglycerides is often followed by complete clinical and biochemical remission.

Copyright © 2011 S. Karger AG, Basel.

Abstract/Text: Porphyrias are a group of eight rare inherited metabolic disorders of heme biosynthesis pathway. Porphyrias are still underdiagnosed, although examinations of urine and plasma are first-line tests for detecting excess of porphyrins or heme precursors in suspected patients. Diagnosis, particularly for the acute forms, is essential to avoid precipitating factors and the use of triggering drugs. Mutation screening of family members is recommended to identify presymptomatic carriers and to prevent acute attacks. The therapeutic approach should be appropriate regarding specific forms of porphyria and treatment should be started promptly.

Abstract/Text: An unusual urine color can occasionally be alarming to patient or physician. Abnormal urine color may indicate a range of normal or pathologic conditions. Variables that affect urine color include concentration, pH, ingested substances, and various metabolic abnormalities. Most causes can be determined by a careful history focusing on medications, foods, occupation, and family history. A few simple laboratory tests can confirm the diagnosis or narrow the list of possible causes. The evaluation should start with gross examination of the urine. Each abnormal color has a fairly limited differential diagnosis, which can be further narrowed by determining specific gravity and pH, and performing dipstick and microscopic examinations. The differential diagnosis can be narrowed further with ferric chloride or an ultraviolet (UV) light source. Rarely, more specific tests are useful. We present an algorithm that may be of benefit in the logical, inexpensive, and efficient evaluation of abnormal urine color.