Letter to the Editor
Nutcracker syndrome associated with thin basement membrane nephropathy
Síndrome del cascanueces asociado a la enfermedad de la membrana basal fina
Marta Cano-Megías
, Loreto Fernández-Rodríguez, Patricia Martínez-Miguel
Corresponding author
Departamento de Nefrología, Hospital Universitario Príncipe de Asturias, Alcalá de Henares, Madrid, Spain
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NCS is the compression of the left renal vein (LRV) between the angle formed by the superior mesenteric artery (SMA) and the aorta. The most popular theory for its cause is an abnormal branching of the SMA from the aorta with compression of the underlying LRV, causing retrograde venous hypertension, renal hilar and ureteral varices. Microscopic rupture of thin-walled collateral veins into the calyceal fornix results in hematuria and proteinuria into the renal collecting system.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The specific examinations include radiological tests such as renal venography, computed tomography scanning (CT), contrast-enhanced magnetic resonance angiogram and renal Doppler ultrasound. There is controversy about the specific radiological criteria, because some degree of LRV entrapment is a common finding in asymptomatic children and young adults.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a></p><p id="par0015" class="elsevierStylePara elsevierViewall">There are reports of concomitant glomerular diseases associated with NCS but its physiopathology is not clear. Hematuria and proteinuria are associated with orthostatism and intense exercise. In cases of maintained or persistent proteinuria, associated glomerulopathy should be suspected. The most common glomerulopathy coexisting with NCS is IgA nephropathy. The association with thin basement membrane nephropathy disease (TBMN) is exceptional.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> We present an unusual case of NCS associated with TBMN disease.</p><p id="par0020" class="elsevierStylePara elsevierViewall">A 31-year-old male was referred to clinic with episodes of macroscopic gross hematuria, anemia and 2 grams of proteinuria per day in the urine analysis. He did not have a family history of chronic kidney disease or episodic hematuria. He has not left flank pain or varicocele. Several three-day long episodes of recurrent hematuria were described at the age of 17, 23 and when he was 30 years old; none of them with a trigger. Glomerular filtration rate was normal. The red blood cells observed in the urine were isomorphic, compatible with non-glomerular origin. Between these episodes, the patient was asymptomatic and the urine analysis confirmed the absence of hematuria or proteinuria.</p><p id="par0025" class="elsevierStylePara elsevierViewall">CT and a Doppler sonographic were performed. CT showed a decreased angle in the superior mesenteric artery origin, with an entrapment of the LRV, change in its caliber and retrograde venous congestion without increased of pressure measurements in Doppler. Images of varicocele were not found. A kidney biopsy was performed because of persistent proteinuria. Renal biopsy only showed peritubular capillary dilation with hemosiderin pigment deposits in tubular epithelial cells. However, electron microscopy showed structural changes compatible with TBMN.</p><p id="par0030" class="elsevierStylePara elsevierViewall">NCS is a benign disease and treatment depends on the severity of symptoms. Renin-angiotensin system inhibitors can improve orthostatic proteinuria. Surgical treatment has been reported in severe cases.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">NCS pathophysiology and its association with renal glomerular disease is not clear. Increased capillary wall permeability caused by LRV congestion and hemodynamic changes in glomerular capillaries could produce local tissue anoxia, oxidative damage, atrophy and necrosis of epithelial glomerular and tubular cells. Also, thickness of the glomerular capillary wall, cell proliferation and immunological deposits have been described, which suggests subclinical local immune damage.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">The recommendation is to start performing Doppler ultrasound imaging studies.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Peak flow velocity of LRV is higher in symptomatic patients and the peak velocity ratio above and below the affected venous segment increases in children with NCS.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> In some difficult cases, it is recommended to perform velocity measurements after the patient has been standing for 15<span class="elsevierStyleHsp" style=""></span>min so that hemodynamic changes can be detected.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Another helpful sonographic criterion is observing the change in the LRV diameter in relation to the stenosis of the aortomesenteric portion and to the expansion of the renal hilum portion.<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> CT imaging may be useful. A decreased aortomesenteric angle and a shortened aortomesenteric distance are complementary diagnostic measures.</p><p id="par0045" class="elsevierStylePara elsevierViewall">This clinical case highlights that NCS may coexist with a glomerulopathy that can establish the renal prognosis. TBMN affects 1% of the population and is caused by a heterozygous mutation in the collagen genes COL4A3 or COL4A4. TBMN disease has an excellent prognosis. It is characterized by persistent glomerular hematuria without proteinuria. Renal biopsy should be done in cases of proteinuria, hypertension or renal function deterioration.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Cano-Megías M, Fernández-Rodríguez L, Martínez-Miguel P. Síndrome del cascanueces asociado a la enfermedad de la membrana basal fina. Med Clin (Barc). 2017;148:526–527.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Nutcracker and SMA syndromes: what is the normal SMA angle in children?" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "O.J. Arthurs" 1 => "U. Mehta" 2 => "P.A. 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