Reyna-Raygoza JR , Núñez-Gómez FE , López-Iñiguez A , Rubio-Reynoso R , Chávez-Iñiguez JS

Language: Spanish
References: 15
Page: 166-169
PDF size: 726.99 Kb.

ABSTRACT

Kidney polycystosis is a systemic disease that affects 1 in every 400-1000 births and in more than half of them the disease will remain silent all their life. It is characterized by the progressive cystic dilatation of the renal tube conducting to a chronic renal disease and in developed countries corresponds to 5 to 10 % of the patients under kidney replacement therapy. This entity is not limited to the kidney; there is an ample variety of concomitant disorders and complications. Within the extra-renal manifestations we can find cystic formations in other organs and non-cystic complications as diverticulum, inguinal and abdominal hernias. Amongst the latter group, it stands out, also the cardiovascular diseases as hypertension, valvular abnormalities, aneurisms and aortic dissection, which are the main cause of death of kidney polycystosis patients an occasionally its first manifestation is where frequently the kidney polycystosis is not identified and therefore poorly reported. We present the case of a 54 years old male diagnosed with kidney polycystosis and aortic dissection, besides, there is a second association barely described between aortic dissection and acute kidney damage.

REFERENCES

1. Davies F, Coles GA, Harper PS, Williams AJ, Evans C, Cochlin D. Polycystic kidney disease reevaluated: a population-based study. Q J Med. 1991; 79(290):477.

2. P. Molina Aguilar et al. Muerte súbita por disección aórtica en portador de enfermedad poliquística renal autosómica dominante. EJAutopsy 2007, 10-14.

3. Gabow PA. Autosomal dominant polycystic kidney disease. N Engl J Med. 1993; 329:332-342.

4. Perrone RD. Extrarenal manifestations of ADPKD. Kidney Int. 1997; 51:2022-2036.

5. Helal I, Reed B, Mettler P, Mc Fann K, Tkachenko O, Yan XD, Schrier RW. Prevalence of cardiovascular events in patients with autosomal dominant polycystic kidney disease. Am J Nephrol. 2012; 36:362-370.

6. E. Utku Unal et al. Aortic dissection as a first sign of polycystic kidney disease in a 26-year old young adult. European Journal of Cardio-Thoracic Surgery 2013; 43:1074.

7. Naoto Fukunaga, MD, Mitsuru Yuzaki, MD, Michihiro Nasu, MD. Dissecting aneurysm in a patient with autosomal dominant polycystic kidney disease. Ann Thorac Cardiovasc Surg 2012; 18: 375- 378.

8. Coto E, Sanz de Castro S, Aguado S, lvarez, Arias M, Menendez MJ, Lopez-Larrea C. DNA microsatellite analysis of families with autosomal dominant polycystic kidney disease types 1 and 2: evaluation of clinical heterogeneity between both forms of the disease. J Med Genet 1995; 32(6):442-5.

9. Gabow PA. Autosomal dominant polycystic kidney disease--more than a renal disease. Am J Kidney Dis 1990; 16:403.

10. E. Ros-Díe, F. Fernández-Quesada, R. Ros-Vidal. Historia natural de la disección aórtica. Angiología 2006; 58: S59-S67.

11. 11.Tolulola Adeola, MD, Olufemi Adeleye, MD, James L. Potts, MD, Thoracic aortic dissection in a patient with autosomal dominant polycystic kidney disease. J Natl Med Assoc. 2001; 93:282-287.

12. Kim J, Kim SM, Lee SY, Lee HC, Bae JW, Hwang KK, Kim DW, Cho MC, Byeon SJ, Kim KB. A case of severe aortic valve regurgitation caused by an ascending aortic aneurysm in a young patient with autosomal dominant polycystic kidney disease and normal renal function. Korean Circ J. 2012;42:136- 139.

13. Torra R, Nicolau C, Badenas C, BrúC, Pérez L, Estivill X, Darnell A. Abdominal aortic aneurysms and autosomal dominant polycystic kidney disease. J Am Soc Nephrol. 1996; 7(11):2483.

14. Joly D, Berissi S, Bertrand A, Strehl L, Patey N, Knebelmann B. Laminin 5 regulates polycystic kidney cell proliferation and cyst formation. J Biol Chem. 2006; 281(39):29181-9.

15. Hilhorst-Hofstee Y, Rijlaarsdam ME, Scholte AJ, et al. The clinical spectrum of missense mutations of the first aspartic acid of cbEGF-like domains in fibrillin-1 including a recessive family. Hum Mutat 2010; 31:E1915.