Abstract


Background: The prevalence of hypertension is very high in kidney trasplantation patients and secondary causes should be excluded.

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Background: The prevalence of hypertension is very high in kidney trasplantation patients and secondary causes should be excluded.

Clinical Case: We present a 25-year-old man with a history of neurofibromatosis 1 (NF1) and end-stage renal disease due to renal dysplasia who underwent kidney transplantation in 2010. He maintained controlled blood pressure and normal kidney function until 2014. His blood pressure readings were around 200/110 mmHg despite maximun doses of five anhypertensive drugs including diuretics. He was admitted for resistant hypertension and renal failure. He reported episodes sweating, headaches and palpitations. Clinical examination revealed hypertension (210/110 mm Hg), tachycardia (110 BPM), café-au-lait spots, skin neurofibromas and genu varum. He had a family history of NF1 (mother, grandmother,aunt and sister). CT scans showed 4 cm bilateral adrenal masses. The 24- hour urinary metanephrines were highly elevated: total metanephrines 20055 mcg/24 hours (150-1200), metanephrine: 5380 mcg/24 hours (50 - 400) and normetanephrine 14675 mcg/24 hours (100 – 800). Blood pressure was controlled with doxazosin, nebivolol and amlodipine, and the patient underwent a successful simultaneous bilateral laparoscopic adrenalectomy. The pathology report confirmed PHEO diagnosis. Presently, he is under prednisone and fludrocortisone treatment. Four months after surgery he is asymptomatic and normotensive without antihypertensive therapy.
Renal function and urinary metanephrines were within the normal range.

Conclusions: The overall prevalence of PHEO in NF1 patients is 2.9%. Clinicians should be aware of PHEO diagnosis with the genetic predisposition diseases. There are only a few cases reported in the literature of PHEO after solid organ transplantation.

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