The “Nutcracker” Phenomenon in Combination with IgA Nephropathy

Abstract

The "nutcracker" phenomenon results from compression of the left renal vein between the superior mesenteric artery and the aorta. The main features of this phenomenon are non-glomerular haematuria on urinalysis and stenosis of the left renal vein with dilatation of the vein distal to the stenosis. The characteristics of two patients with the "nutcracker" phenomenon complicated by immunoglobulin A nephropathy were compared with those of 10 patients showing only the "nutcracker" phenomenon. Patients with the "nutcracker" phenomenon complicated by immunoglobulin A nephropathy showed aggravation of haematuria after upper respiratory infections, urinary red cell morphology indicating haematuria of glomerular origin, elevation of serum IgA, persistence of proteinuria, and granular casts in the urine. The coexistence of immunoglobulin A nephropathy was suspected when these characteristics were observed in patients with the "nutcracker" phenomenon. In such cases, a renal biopsy is needed for a final diagnosis.

Nutcracker syndrome combined with IgA nephropathy in a child with recurrent hematuria

“…Although coexistence of these two diseases in children has not been reported, this association is not rare and is sometimes experienced in the pediatric nephrology unit. In addition, there is one report in the literature of two adults with nutcracker syndrome and IgA nephropathy 4 …”

“…4 Ozono et al described two adult patients with nutcracker syndrome complicated by IgA nephropathy, who showed aggravation of hematuria after upper respiratory infections, urinary red cell morphology indicating hematuria of glomerular origin, elevation of serum IgA, persistence of proteinuria, and granular casts in the urine compared to those with nutcracker syndrome alone. 4 However, these are not consistent fi ndings since even patients with nutcracker syndrome alone can show recurrent gross hematuria after upper respi ratory infections and persistent recumbent proteinuria. 5 Although urinary RBC morphology may be normal with non-glomerular bleeding and dysmorphic from glomerular bleeding, cell morphology does not reliably correlate with the site of hematuria.…”

Síndrome del cascanueces: A propósito de un caso y revisión de la literatura

“…Existen casos de pacientes con fenómeno de cascanueces complicados con nefropatía IgA, en cuyo caso presentan empeoramiento de la hematuria tras infecciones del tracto respiratorio superior 20 , sin que pueda establecerse ninguna relación entre ambos procesos.…”

Nutcracker phenomenon in IgA nephropathy

“…To date, only six cases of concurrent left renal vein entrapment and idiopathic IgA nephropathy have been reported [ 1 – 5 ]: An 11-year-old Italian boy with postural proteinuria who developed gross hematuria [ 2 ], a 12-year-old German girl with microscopic hematuria [ 3 ], a 9-year-old Korean girl with isolated microscopic hematuria and recurrent gross hematuria [ 1 ], a 25-year-old Taiwanese woman with recurrent gross hematuria and left flank pain [ 4 ] and two Japanese, 20-year-old woman and 22-year-old man, who, in the context of a pharyngitis, developed gross hematuria and a tendency to microscopic hematuria with persisting proteinuria [ 5 ]. Four out of six cases were Asian and the remaining two cases were White.…”