Abstract
A 5-year-old girl with red urine was referred to our hospital with proteinuria, hematuria and chylous serum. Urine appeared whitish red in color and gave strong positive results for protein, glucose, occult blood and ketone bodies. Since urinary sediment showed many isomorphic red blood cells and fatty balls, hematochyluria was diagnosed. Laboratory data revealed increased levels of blood glucose (430 mg/dl), HbA1C (16.9%), triglycerides (19,910 mg/dl), VLDL (551.6 mg/dl) and apo C-II (28.6 mg/dl). Urinary levels of C-peptide were below detection limit. Lipoprotein lipase activity and apo E genotype were normal. Type1diabetes mellitus and secondary type V hyperlipidemia were thus diagnosed. Lymphoscintigraphy demonstrated leakage from lymphatic vessels to the bladder. Based on these findings, hematochyluria might have been caused by marked hypertriglyceridemia, resulting in stasis of the lymphatics and rupture of lymphatic vessels into the bladder.
