ESPN 51th Annual Meeting

ESPN 2018


 
ANOTHER ETIOLOGY OF PROTEINURIA AND HEMATURIA IN A FOUR YEARS OLD BOY WITH HENOCH-SCHONLEIN NEPHRITIS (HSN): NUTCRACKER SYNDROME
Belde Kasap Demir 1 Eren Soyaltın 2 Caner Alpaslan 2 Gülcan Erbaş 3 Demet Alaygut 2 Önder Yavaşcan 2 Seçil Arslansoyu Çamlar 2 Fatma Mutlubaş 2

1- IZMIR KATIP ÇELEBI UNIVERSITY DEPARTMENT OF PEDIATRICS DIVISION OF NEPHROLOGY, IZMIR, TURKEY
2- IZMIR TEPECIK TRAINING AND RESEARCH HOSPITAL DEPARTMENT OF PEDIATRICS DIVISION OF NEPHROLOGY, IZMIR, TURKEY
3- IZMIR TEPECIK TRAINING AND RESEARCH HOSPITAL DEPARTMENT OF PEDIATRICS, IZMIR, TURKEY
 
Introduction:

 Henoch-schönlein purpura (HSP) is a primary vasculitis of childhood, usually associated with skin, gastrointestinal system and joint involvement. The most frequent urine finding of IgA vasculitis nephritis is microscopic hematuria, and proteinuria. We wanted to present a case of Nutcracker Syndrome diagnosed during the follow-up with IgA vasculitis nephritis in persistant hematuria and non-nephrotic proteinuria.

Material and methods:

A four-year-old boy admitted to our clinic with rashes on foots and legs, edema, abdominal pain, bloody diarrhea and macroscopic hematuria. There were purpuric rashes on the lower extremities and the gluteal regions and no other pathology was detected on physical examination. The blood cell count, coagulation parameters, renal and hepatic function tests were in normal ranges. The dipstick urine anaylsis revealed +3 proteinuria and +2 erythrocyte. The nephrotic range of proteinuria (54 mg/m2/h) were detected. There were diffuse dysmorphic erythrocytes in urine examination under microscope. Renal ultrasonography was normal. The patient underwent renal biopsy. Immunofluorescence revealed IgA +++ mesangial granular accumulation. The renal biopsy was consired as ISKDC class 1–2 IgA vasculitis nephritis. Angiotensin converting enzyme inhibitor and fish oil treatments were initiated. Intermittent macroscopic hematuria, microscopic hematuria with isomorphic erythrocytes and proteinuria at the non-nephrotic level continued during the follow-up period. 

Results:

The case was evaluated for isolated non-nephrotic proteinuria and hematuria. In renal doppler USG, the left renal vein diameter in distal / proximal ratio was 4 and the angle between abdominal aorta and SMA was 14°. In the abdominal CT angiography; Nutcracker syndrome was diagnosed with the left renal vein trapped between the aorta and superior mesenteric artery.

Conclusions:

Nutcraker syndrome is a rare anatomico-pathological condition caused by compression of the left renal vein between the aorta and the superior mesenteric artery. Diagnosis can be easily made with radiological findings if this syndrome is brought to mind when the other causes of hematuria and proteinuria are excluded.