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Case 216

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Female patient, two years nine months old, with a previous diagnosis of neurofibromatosis type 1 is sent to the emergency department of the HC-UFMG due to blood pressure measurement of 190x130mmHg MRS (p99 120x97). Her mother reported restless sleep and night sweats. Laboratory tests were performed: renin: 60ng /ml (VR 0.06 to 4.69), aldosterone: 1284pg/ml (VR 25-315), urinary normetanephrine: 276μg/24h (VR up to 800), urinary metanephrine: 62μg/24 (VR up to 400), TSH: 3,960μUI/ml (VR 0.3 to 5.0), free T4: 1,49ng/dL (VR 0.75 to 1.8). Prior ultrasound showed left kidney smaller than the right one and unchanged parenchyma. Then, it was asked for a angiography of renal arteries.

Based on clinical data and laboratory tests, which is the correct statement regarding the diagnosis and treatment?

a) Renal artery aneurysm and ACE inhibitor can be used

25%

b) Renal artery aneurysm and ACE inhibitor can not be used

25%

c) Renal artery stenosis and ACE inhibitor can be used

25%

d) Renal artery stenosis and ACE inhibitor can not be used

25%
   

Image analysis

Image 1: Angiography of the renal arteries, revealing path of iodinated contrast (in blue), with an area of obstruction to its passage at the beginning of the left renal artery (yellow arrow) compatible with vessel stenosis.

 

Image 2: Angiography of the renal arteries, revealing path of iodinated contrast (in red) with narrowing region of the proximal portion of the left renal artery (yellow arrow). 

Highlights

- Patients with neurofibromatosis type 1 are more likely to develop secondary hypertension to renovascular causes or pheochromocytoma;
- The gold standard for the diagnosis of renal artery stenosis is angiography, and its realization grounded by less invasive tests such as angioresonance, helical computed tomography and Doppler ultrasound;
- Stenosis of fibromuscular dysplasia preferably reaches the distal portion of the renal arteries, while stenosis associated with neurofibromatosis preferably reaches the proximal portion of these vessels;
- ACE inhibitors and ARBs are indicated for the treatment of unilateral renal artery stenosis, but should be used with caution in bilateral stenosis or solitary kidney;
- Percutaneous transluminal angioplasty is the first intervention option in cases of fibromuscular dysplasia and dysplasia associated with neurofibromatosis.

 References

- Olin JW, Gornik HL, Bacharach JM, Biller J, Fine LJ, Gray BH, et al. Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association. Circulation. 2014;129:1048–1078

- Salgado, CM; Carvalhaes, JTA. Hipertensão arterial na infância. J Pediatr, Rio de Janeiro (RJ), 2003;79 (Supl.1): S117-S121

- Sociedade Brasileira de Cardiologia; Sociedade Brasileira de Hipertensão; Sociedade Brasileira de Nefrologia. VI Diretrizes Brasileiras de Hipertensão. Arq Bras Cardiol 2010; 95 (Supl.1): 1-51

- Cooper, CJ; Murphy, TP. The case for renal artery stenting for treatment of renal artery stenosis. Circulation, 2007;115: 263-270

- Feldman A, Freitas LZF, Collet CA, Mota AR, Pimenta E, Sousa M, et al. A relação entre estenose de artéria renal, hipertensão arterial e insuficiência renal crônica. Rev Bras Hipertens, 2008; 15(3): 181-184

- Praxedes JN. Hipertensão renovascular: epidemiologia e diagnóstico. Rev Bras Hipertens, 2002;9(2): 148-153

Authors

Camila Martins Lopes, 4th year medical student at UFMG.

E-mail: martinslopesc[arroba]gmail.com

 

Carolina Kuchenbecker Soares, 4th year medical student at UFMG.

E-mail: carolinakuchenbecker[arroba]gmail.com

 

Luísa Bernardino Valério, 4th year medical student at UFMG.

E-mail: luisabernardino[arroba]gmail.com

Supervisor

Ana Carmen Quaresma Mendonça, doctor of the Pediatric Nephrology Unit of the HC-UFMG.

E-mail: anacarmenqm[arroba]gmail.com

Reviewers

Fellype Borges, Carla Faraco, Pétala Silva, Cairo Mendes, Bárbara Queiroz and Professor Viviane Parisotto.

Translated by

Luísa Bernardino Valério, 4th year medical student at UFMG.

E-mail: luisabernardino[arroba]gmail.com

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