Life-threatening gross hematuria due to genitourinary manifestation of Klippel-Trenaunay syndrome.

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.

Successful (?) therapy of hemolytic-uremic syndrome with factor H abnormality.

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.