Causes of death in patients with tuberous sclerosis.

Abstract:

:Of the 355 patients with tuberous sclerosis complex (TSC) examined at the Mayo Clinic, 49 had died (9 of causes other than TSC). We attempted to determine what pattern of organ involvement occurred most often in the 40 patients who died of TSC. One baby died of cardiac failure due to cardiac rhabdomyomas, and one child died of rupture of an aneurysm of the thoracic aorta. Eleven patients died of renal disease, which was the commonest cause of death. Ten patients died as a result of brain tumors, and four patients (who were 40 years of age or older) died of lymphangiomyomatosis of the lung. Thirteen patients with severe mental handicaps died of either status epilepticus or bronchopneumonia; in all but one of these patients, the only source of information was the death certificate. Survival curves show a decreased survival for patients with TSC in comparison with that for the general population. Patients with TSC need lifelong follow-up for early detection of potentially life-threatening complications.

journal_name

Mayo Clin Proc

journal_title

Mayo Clinic proceedings

authors

Shepherd CW,Gomez MR,Lie JT,Crowson CS

doi

10.1016/s0025-6196(12)61196-3

subject

Has Abstract

pub_date

1991-08-01 00:00:00

pages

792-6

issue

8

eissn

0025-6196

issn

1942-5546

pii

S0025-6196(12)61196-3

journal_volume

66

pub_type

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