RESUMEN
The Klippel-Trenaunay Syndrome (KTS) is defined as a triad of cutaneous capillary malformations, venous varicosities, bone and soft tissue hemi-hypertrophy. The urinary tract is involved in up to 10%. We report the clinical presentation and surgical management of a 9-year-old boy with extensive lympho-venous malformations of the bladder which led to massive recurrent gross hematuria.
Asunto(s)
Hematuria/etiología , Síndrome de Klippel-Trenaunay-Weber/complicaciones , Enfermedades de la Vejiga Urinaria/etiología , Niño , Cistectomía , Hematuria/cirugía , Humanos , Síndrome de Klippel-Trenaunay-Weber/cirugía , Masculino , Enfermedades de la Vejiga Urinaria/cirugíaRESUMEN
We report a patient with continuously recurring hemolytic-uremic syndrome due to factor H deficiency. First at the age of 3 months he showed signs of hemolytic anemia, thrombocytopenia and renal insufficiency, often recurring concomitantly with respiratory tract infections, despite weekly to twice weekly plasma substitution (20 ml/kg body weight). Now at the age of 3.5 years glomerular filtration rate is approximately 50 ml/min/1.73 m(2) and psychomotoric development is normal. Since factor H is mainly synthesized in the liver, hepatic transplantation has been proposed as curative treatment. Before justification of liver transplantation as the ultimate treatment for these patients, an international registry should be developed to optimize and standardize therapeutic alternatives.