Prof L. Gesualdo Professor of Nephrology University of Foggia, Italy Chief of the Nephrology, Dialysis and Transplantation Unit Azienda Ospedaliera Policlinico Foggia, Italy

 

A 15 year old white boy (body weight: 65 kg; height: 176 cm) was referred to our out-patient clinic in June 2002 for recurrent macrohematuria. He had an history of upper respiratory infection (May 2002) prior the first episode of gross hematuria followed by 4 other episodes of macrohematuria “sine causa”. His medical history was otherwise unremarkable. His blood pressure was 120/70 mmHg, pulse rate 77/min and body temperature 36.5° C. His physical examination was normal. Giordano’s manoeuvre and flank pain was negative. Creatinine clearance was normal (120 ml/min) and urinary sediment irrelevant. The patient had no history of allergies and no significant past illnesses. The patient had not been on drugs. His routine laboratory tests showed an increase in serum LDH levels (901 IU/L).

QUESTION 1

Coombs’ test, both direct and indirect, was negative. No schistocytes were detected in the smear. Serum LDH returned to the normal levels after 4-6 days post gross hematuria. No drops in Hb levels were observed during the episodes of gross hematuria. Prothrombin time, partial thromboplastin time, and bleeding time were normal as well as all coagulation factors.
Urine cytology and cultures for tuberculosis bacilli were negative.

QUESTION 2

Renal ultrasonography showed normal renal size and outline with no anatomical defects. The length of the left kidney was 10.8 cm, and that of the right one was 10 cm. Bladder ultrasonography was also normal. The results of intravenous pyelography showed a “minus” in the right ureter. For this reason, a retrograde pielography was required. Both retrograde pielography and cystoscopy were normal. The presence of one episode of post-infective macrohematuria oriented us to perform a renal biopsy. Light microscopy and immunofluorescence examination were normal.

Figure 1

 

QUESTION 3

 

Figure 2	Figure 3

Renal ultrasonography and color Doppler sonography oriented our diagnosis to a Nutcracker syndrome. Indeed, we found the following ultrasound parameters: D/N ratio= 4.07; D/A ratio 0.91; RVD= 4.5 mm. The mean value was three (Okada’s criteria for the diagnosis of the Nutcracker syndrome= >2 indicates the presence of the Nutcracker syndrome). Abdominal computed tomography (CT) scan confirmed that the left renal vein was compressed between the aorta and the superior mesenteric artery.

Figure 4	Figure 5

 

QUESTION 4

The patient is still followed by our out-patient clinic. He is presenting continuous intermittent episodes of macroscopic haematuria (1 episode per week) without drop in his hemoglobin level. Thus, we are now evaluating a therapeutical approach.

References