Naslov
An extreme case of neurofibromatosis type 1

Autori
Štimac, Davor ; Novak, Srđan ; Guina, Tina

Izvornik
Wiener klinische Wochenschrift (0043-5325) 119 (2007), 3-4; 90-90

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, prikaz, stručni

Ključne riječi
Giant peduncular neurinomas; Neurofibromatosis type 1; Von Recklinghausen disease

Sažetak
Neurofibromatosis type 1 or von Recklinghausen disease is one of the most common autosomal dominant genetic disease and is associated with plexiform neurofibromata. They affect peripheral nerves, leading occasionally to a massive overgrowth of nerves and connective tissue. A 59-year-old man with neurofibromatosis type 1 was admitted to our clinic because of tumour mass overgrowth (weight 170 kg ; circumference of the tumour mass 190 cm), scrotum oedema, dyspnoea and oliguria. He was unable to walk ; large subcutaneous neurofibromata with café-au-lait spots were palpabled over both gluteal regions and thighs, he had scrotal pain. Urine volume was less than 500 ml/day. He was treated with furosemid 250 mg daily and ACE inhibitors. He lost more than 35 L of fluids in two weeks. Gradually, scrotal oedema diminished, the tumour mass was moderately reduced, urea and creatinine values normalised and he was able to perform daily activities.

Izvorni jezik
Engleski

Znanstvena područja
Kliničke medicinske znanosti

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