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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2017  |  Volume : 28  |  Issue : 1  |  Page : 174-177
A case of abdominal aortic thrombosis associated with the nephrotic syndrome

Department of Internal Medicine, Tunisian Military Hospital, Montfleury, Tunis, Tunisia

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Date of Web Publication12-Jan-2017


Thromboembolic disease is an important and frequent complication in patients with the nephrotic syndrome (NS), and the consequences are often severe. Usually, the venous system is affected. Arterial thrombosis has rarely been reported and occurs mainly in children. We report the case of a 27-year-old man with a history of NS due to focal and segmental glomerulosclerosis resistant to steroids and cyclosporine, admitted for bilateral pain in the calves. Aortogram revealed a suspended thrombus in the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery. Endarterectomy was performed followed by systemic heparinization with a good outcome. Arterial thrombosis is rare and must be prevented.

How to cite this article:
Labidi J, Selmi Y, Ariba YB, Elloumi Z, Othmani S. A case of abdominal aortic thrombosis associated with the nephrotic syndrome. Saudi J Kidney Dis Transpl 2017;28:174-7

How to cite this URL:
Labidi J, Selmi Y, Ariba YB, Elloumi Z, Othmani S. A case of abdominal aortic thrombosis associated with the nephrotic syndrome. Saudi J Kidney Dis Transpl [serial online] 2017 [cited 2022 Aug 12];28:174-7. Available from: https://www.sjkdt.org/text.asp?2017/28/1/174/198246

   Introduction Top

Thromboembolic disease is an important and severe complication in patients with the nephrotic syndrome (NS) and involves both arterial and venous systems. The incidence of arterial thromboembolism (ATE) in the NS is estimated to be 1.48%.[1] We report the occurrence of aortic thrombosis in a 27-year-old-man with a history of NS and discuss the predisposing factors.

   Case Report Top

A 27-year-old man with a five-year history of focal and segmental glomerulosclerosis (FSGS) resistant to corticosteroids and cyclosporine presented with pain in the calves accompanied with progressive development of pallor, coldness, and loss of power in the lower limbs over two weeks. His medication was prednisolone, ramipril, and oral warfarin. He was not taking the prescribed anticoagulants regularly.

On physical examination, the blood pressure was 120/70 mm Hg, and pulse rate was 67 bpm, the calves were tender and swollen, and both feet were cold and pale. The pedal and popliteal pulses were absent.

The laboratory data revealed 24 h protein excretion of 15.44 g/24 H, low serum total protein concentration of 42 g/L, and an albumin concentration of 11 g/L. The renal functions were normal. The hematocrit was 38%, hemoglobin level was 13 g/dL, and the platelet count was 352,000/μL. The coagulation investigations showed a fibrinogen level of 2.25 g/L, a cephalin kaolin time of 31/32, a protein C antigen activity of 120% (75-155), and protein S antigen activity of 100% (50-150). Screening for anticardiolipin antibodies, factor V and factor II mutation was negative, but a heterozygous C677T mutation of the methylene tetrahydrofolate reductase (MTHFR) gene was present.

Doppler ultrasonography of the lower limbs showed no thrombosis in the veins, but there was bilateral distal arteritis and thrombosis in the right anterior tibial artery and in the left anterior and posterior tibial arteries.

Computed tomography angiography ([Figure 1]) demonstrated thrombosis of the abdominal aorta just below the origin of the renal arteries with embolism into the left tibioperoneal trunk and the right anterior tibial artery. He was treated with anticoagulation and endarterectomy ([Figure 2]) with reperfusion of the lower limbs and resolution of the pain. The anatomopathologic examination of the thrombus extracted showed a fibrinocruoric thrombus without infiltration of the aorta wall. The patient remained anticoagulated on warfarin.
Figure 1. Computed tomographic angiography demonstrating abdominal aorta thrombosis.

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Figure 2. Clinical photograph of endarterectomy being performed.

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He developed complete remission of the NS shortly after receiving rituximab at 375 mg/m2 per week for one month.

   Discussion Top

Thromboembolism is a severe complication in patients with NS. Usually, the venous system is affected. Arterial thrombosis is rare and is mainly restricted to pediatric patients. The risk of vascular thrombosis has an annual incidence rate of 1.48% for arterial thromboembolism.[1]

In nephrotic patients, arterial thrombosis has been reported in the aorta, mesenteric, axillary, subclavian, brachial, femoral, ophthalmic, carotid, cerebral, renal, pulmonary, and coronary arteries.[2] To the best of our knowledge, only four cases of thrombosis in the aorta associated with NS have been reported in the literature[1] and it results in a high rate of limb loss or death. The overall mortality has been reported to be 52%.[1]

ATE can occur at any stage during NS and may arise from preferential loss of proteins involved in the inhibition of systemic hemostasis, increased synthesis of factors promoting thrombosis, or by local activation of the glomerular hemostasis system.[3] ,[4]

The abnormalities include increased levels of fibrinogen, Factor V, Von Willebrand factor, factor VII, and alpha-1 macroglobulin. This is thought to be due to increased hepatic synthesis of these factors stimulated by hypoalbuminemia[2] and decreased levels of antithrombin III, plasminogen, factor XI, factor XII, alpha one antitrypsin, protein S, and protein C due to urinary losses of these proteins.[2] The relationship between elevated levels of serum fibrinogen and decreased level of antithrombin III and thromboembolism in NS has been demonstrated.[2] ,[3] ,[5] ,[6]

In addition, hypoalbuminemia results in increased availability of normally albuminbound arachidonic acid, leading to increased formation of thromboxane A2 in platelets, a stimulus for platelet aggregation.[3]

Some genetic disorders may also lead to a hypercoagulable states such as factor V, factor II mutation, and C677T mutation of the MTHFR gene.[7] ,[8]

Increased blood viscosity due to hemoconcentration contributes to occurrence of thromboembolic complications, which is often aggravated by the use of diuretics.[6] Steroids shorten prothrombin as well as accelerated partial thromboplastin times that further aggravate the hypercoagulable state of the NS.[2]

The underlying nephropathy may play a role in the frequency of occurrence of thrombosis; it is often seen in association with membranous glomerulonephritis. However, the risk is equally important in other primary glomerular diseases, particularly membranoproliferative glomerulonephritis and minimal change disease or FSGS.[9]

Thrombosis in the aorta may be asymptomatic and revealed only by its complications. Clinically, it could be limb or other organ ischemia. That is why, we should treat it energetically when there are announcing signs. The management of arterial thrombosis is not well codified and depends essentially on the location, the extent of the thrombosis and the hypercoagulable state. The postoperative mortality is estimated to be 62%.[1] Ruud E and Share performed direct catheter-assisted thrombolysis with locally administrated streptokinase infusion for eight days. This method has the advantages of localizing inaccessible distal arteries by surgery and to avoid adverse effects of systemic thrombolysis.[10] ,[11] Computed tomography or magnetic resonance angiography is useful for the detection of this thromboembolic events.[12] Color Doppler ultrasonography is employed for the detection of thrombosis of the extremities.[12]

Prophylactic anticoagulation is recommended for patients with NS although no randomized controlled trials are available to guide the decision as to who should be given prophylactic anticoagulation and for how long.[12] Prescription of such treatment may be indicated in case of severe hypoalbuminemia, a deficit in antithrombin III and when combined with an aggravating factor such as trauma, immobilization, or use of diuretics.[9]

   Conclusion Top

Arterial thrombosis is a rare complication in patients with NS. It is presumably attributable to a hypercoagulable state. Their gravity is closely related to the location of the thrombosis.

Conflict of interest: None declared.

   References Top

Xiang L, Zhou H, Jiang Z, Pan J, Yang M. Abdominal aortic thrombosis associated with nephrotic syndrome. Am J Med Sci 2014;347: 91-2.  Back to cited text no. 1
Brahmbhatt BK, Mathew A, Rajesh R, Kurian G, Unni VN. Brachiocephalic artery thrombosis in adult nephrotic syndrome. Indian J Nephrol 2011;21:204-7.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
Singhal R, Brimble KS. Thromboembolic complications in the nephrotic syndrome: Pathophysiology and clinical management. Thromb Res 2006;118:397-407.  Back to cited text no. 3
Gargah T, Abidi K, Nourchene K, Zarrouk C, Lakhoua MR. Thromboembolic complications of childhood nephrotic syndrome. Tunis Med 2012;90:161-5.  Back to cited text no. 4
Nishimura M, Shimada J, Ito K, Kawachi H, Nishiyama K. Acute arterial thrombosis with antithrombin III deficiency in nephrotic syndrome: Report of a case. Surg Today 2000; 30:663-6.  Back to cited text no. 5
Kim HJ, Park CH, Kang CM, Park HC, Kim CY, Cho YS. Arterial thrombosis associated with nephrotic syndrome - A case report and review (adult cases in the English literature). J Korean Med Sci 1993;8:230-4.  Back to cited text no. 6
Queffeulou G, Michel C, Vrtovsnik F, Philit JB, Dupuis E, Mignon F. Hyperhomocysteinemia, low folate status, homozygous C677T mutation of the methylene tetrahydrofolate reductase and renal arterial thrombosis. Clin Nephrol 2002;57:158-62.  Back to cited text no. 7
D'Amato N, Correale M, D'Agostino C. Aortic thrombus and acute pulmonary embolism in an individual heterozygous for the MTHFR C677T mutation. Rev Esp Cardiol 2010;63: 1366.  Back to cited text no. 8
Haddad S, Ghédira-Besbes L, Lajmi K, et al. Lower-limb arterial thrombosis in a relapse of nephrotic syndrome. Arch Pediatr 2010;17: 1317-9.  Back to cited text no. 9
Ruud E, Holmstrøm H, Aagenaes I, et al. Successful thrombolysis by prolonged low-dose alteplase in catheter-directed infusion. Acta Paediatr 2003;92:973-6.  Back to cited text no. 10
Share A, Uzun O, Blackburn ME, Gibbs JL. Prolonged local infusion of streptokinase in pulmonary artery thrombosis with nephrotic syndrome. Pediatr Nephrol 1996;10:734-6.  Back to cited text no. 11
Yang GF, Schoepf UJ, Zhu H, Lu GM, Gray JC 3rd, Zhang LJ. Thromboembolic complications in nephrotic syndrome: Imaging spectrum. Acta Radiol 2012;53:1186-94.  Back to cited text no. 12

Correspondence Address:
Jannet Labidi
Department of Internal Medicine, Tunisian Military Hospital, Montfleury, 1008 Tunis
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.198246

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