SummaryThis report describes the clinical history and laboratory findings of three sisters with severe inherited factor VII deficiency. We present current knowledge about factor VII structure and function, and discuss clinical presentation as well as management options for patients affected by factor VII deficiency.

Introduction: To evaluate therapeutic results till 5 years after therapy of obstructive pyelonephritis (OPN) emphasizing regular follow-up. Material and Methods: During 5 years, 57 patients with OPN were treated. The patients' charts were reviewed retrospectively for clinical data. These were completed by a questionnaire. Results: In the group of 57 patients (average age 56 years), about two third were women. Urolithiasis (65%) and tumors (21%) were the main causes of obstruction; fever (91%) and loin pain (86%) the main symptoms. Three fourth of the patients showed renal insufficiency and nearly 50% anemia. E. coli and Proteus spp. were the dominating organisms. Sonography detected obstruction in 93% cases. In one third of cases, CT scan was added; 81% percutaneous nephrostomy and 19% ureteral stenting were the initial methods of urinary drainage. During therapy, 23% nephrectomies (19% complete, 4% partial) were performed. Long-term follow-up showed 11% recurrent OPN and 33% recurrent UTI. Conclusions: After diagnosis of OPN, primary nephrostomy or ureteral stenting and antibiotic therapy are the first measures. If recurrent urinary tract infections or OPN occur, long-term follow-up and low-dose antibiotic prophylaxis may be discussed.

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