Acta Scientific Medical Sciences (ISSN: 2582-0931)

Case Report Volume 4 Issue 3

Evaluation of Pathophysiological Characteristics in the Setting of Budd Chiari Syndrome and Renal Artery Stenosis Induced by Polycythemia Vera

Juna Musa1*, Ali Guy2, Elton Cekaj3, Erisa Kola4, Edlira Horjeti5, Ina Kola6, Carlos Rivera7 and Belinda Bejtja1

1General Practitioner, Tirana, Albania
2Clinical Instructor of Physical Medicine and Rehabilitation, New York University, School of Medicine, NYU Medical Center, USA
3Radiologist, Regional Hospital of Durres, Albania
4Pathologist, Department of Pathology and Forensic Medicine, Albania
5Family Doctor, Primary Care Center Nr. 2, Tirana, Albania
6Plastic Surgery Resident, Tirana, Albania
7Research Fellow, General Surgery Department, Metabolic and Bariatric Institute, Cleveland Clinic, Florida, USA

*Corresponding Author: Juna Musa, General Practitioner, Tirana, Albania

Received: March 18, 2020; Published: April 01, 2020

×

Abstract

  Budd-Chiari syndrome is a rare life-threatening disorder caused most frequently as a complication of Polycythemia Vera [1]. BDS is caused by an obstruction of the hepatic vein outflow at the level of either the hepatic veins or the sub-diaphragmatic segment of the inferior vena cava [2]. Myeloproliferative neoplasms and chronic inflammatory diseases are the most common causes of BCS [2]. The precise location and size of the obstruction is clinically and prognostically significant as it dictates the patient’s symptoms and guides through the appropriate therapeutic management [3]. A 54-year-old female presented with Primary Erythrocytosis from Polycythemia Vera and refractory hypertension secondary to renal arterial stenosis (RAS), findings of Reno vascular occlusive disease may be primarily due to underlying PV. Radiological assessments (CT scan with IV contrast) and the clinical presentation were consistent with the diagnosis of BCS. Along with literature review; we discussed the pathological events that take place in such rare associations.

Keywords: Budd-Chiari syndrome (BCS); Inferior Vena Cava (IVC); Polycythemia Vera; Renal Artery Stenosis

×

References

  1. Martens Pieter and Nevens Frederik. “Budd-Chiari syndrome”. United European Gastroenterology Journal 6 (2015): 489-500.
  2. Aydinli Musa and Bayraktar Yusuf. “Budd-Chiari syndrome: Etiology, pathogenesis and diagnosis”. World Journal of Hepatology 19 (2007): 2693-2696.
  3. Naomi Shin and Young H Kim. “Redefining Budd-Chiari syndrome: A systematic review”. World Journal of Hepatology 16 (2016): 691-702.
  4. Buzas Claudia and Sparchez Zeno. “Budd-Chiari Syndrome secondary to Policithemia Vera A case report”. Journal of Gastrointestinal and Liver Diseases 3 (2009): 363-366.
  5. Menon KV and Shah V. “The Budd-Chiari syndrome”. The New England Journal of Medicine 6 (2004): 578-585.
  6. DeLeve LD and Valla DC. “Vascular disorders of the liver”. Hepatology 49 (2009): 1729-1764.
  7. Mancuso A. “Budd-Chiari syndrome management: lights and shadows”. World Journal of Hepatology 3 (2011): 262-264.
  8. Mancuso A. “An Update on Management of Budd-Chiari syndrome”. Annals of Hepatology 3 (2014): 323-326.
  9. Garcia-Pagán JC and Buscarini E. “EASL Clinical Practice Guidelines: Vascular diseases of the liver”. The Journal of Hepatology 1 (2016): 179-202.
  10. Valla DC. “Primary Budd-Chiari syndrome”. The Journal of Hepatology 50 (2009): 195-203.
  11. Jae Young Kim and In Sook Woo. “JAK2 V617F-positive polycythemia vera accompanied by renal artery stenosis”. Blood Research 4 (2014): 283-285.
  12. Dharmendra Bhadauria and Raj Kumar Sharma. “A rare clinical syndrome of refractory secondary hypertension, renal artery stenosis and erythrocytosis”. NDT plus 3 (2011): 175-177.
  13. Dubose Jr D Thomas and Santos M Renato. “Vascular Disorders of the Kidney”. In Goldman's Cecil Medicine (Twenty Fourth Edition) (2012).
  14. Dworkin D Lance and Christopher J Cooper. “Renal-Artery Stenosis”. The New England Journal of Medicine 20 (2016): 1972- 1978.
  15. Ravinder Garg and Simmi Aggarwal. “Association of atherosclerosis with dyslipidemia and co-morbid conditions: A descriptive study”. Journal of Natural Science, Biology and Medicine 1 (2015): 163-168.
  16. Weber R Benjamin and Dieter S Robert. “Renal artery stenosis: epidemiology and treatment”. The International Journal of Nephrology and Renovascular Disease 7 (2014): 169-181.
  17. Gavriilaki E and Sampanis N. “An exceptional case of renal artery restenosis in a patient with polycythaemiavera”. Blood Coagulation and Fibrinolysis 8 (2014): 904-906.
  18. Mishima Eikan and Suzuki Takehiro. “Renovascular hypertension associated with JAK2 V617F positive myeloproliferative neoplasms treated with angioplasty: 2 cases and literature review”. The Journal of Clinical Hypertension4 (2018): 798-804.
  19. Ancochea Àgueda., et al. “The role of serum erythropoietin level and jak2 v617f allele burden in the diagnosis of polycythaemia vera”. British Journal of Haematology (2014).
  20. How J and Zhou A. “Splanchnic vein thrombosis in myeloproliferative neoplasms: pathophysiology and molecular mechanisms of disease”. Therapeutic Advances in Hematology 3 (2017) 107-118.
  21. Lao David., et al. “Atherosclerotic Renal Artery Stenosis-Diagnosis and Treatment”. Mayo Clinic Proceedings 7 (2011): 649-657.
  22. Florah Tshepo Moshapa and Kirsten Riches-Suman., et al. “Therapeutic Targeting of the Proinflammatory IL-6- JAK/STAT Signalling Pathways Responsible for Vascular Restenosis in Type 2 Diabetes Mellitus”. Cardiology Research and Practice (2019): 15.
×

Citation

Citation: Juna Musa., et al. “Evaluation of Pathophysiological Characteristics in the Setting of Budd Chiari Syndrome and Renal Artery Stenosis Induced by Polycythemia Vera". Acta Scientific Medical Sciences 4.3 (2020): 01-04.




Metrics

Acceptance rate30%
Acceptance to publication20-30 days
Impact Factor1.403

Indexed In





Contact US