Tuberous sclerosis, first described by Bourneville in 1880, is a syndrome characterized essentially by mental deterioration, seizures and cutaneous sebaceous adenoma; an association with malformative lesions of the kidney and cardiovascular apparatus has been documented. Recently a case of a young woman with tuberous sclerosis has come to our observation; she was also affected by abdominal aortal aneurysm and stenosis due to compression of the truncus coeliacus; previously she operative elsewhere for right nephrectomy for breakage of nephric right arterial aneurysm: the histological examination of the removed kidney manifested the presence of a mesoblastic nephroma and afterward tuberous sclerosis was diagnosed. The angiomyolipomatosis evidence confirmed the suspicion of a notable inclination to polydistrict malformations in tuberous sclerosis.
ANEURISMA DELL'AORTA ADDOMINALE E STENOSI DA COMPRESSIONE DEL TRIPODE CELIACO IN GIOVANE DONNA CON SCLEROSI TUBEROSA GIA' TROVATA AFFETTA DA NEFROMA MESOBLASTICO
OCCHIONORELLI, Savino;GALEOTTI, Roberto;
1991
Abstract
Tuberous sclerosis, first described by Bourneville in 1880, is a syndrome characterized essentially by mental deterioration, seizures and cutaneous sebaceous adenoma; an association with malformative lesions of the kidney and cardiovascular apparatus has been documented. Recently a case of a young woman with tuberous sclerosis has come to our observation; she was also affected by abdominal aortal aneurysm and stenosis due to compression of the truncus coeliacus; previously she operative elsewhere for right nephrectomy for breakage of nephric right arterial aneurysm: the histological examination of the removed kidney manifested the presence of a mesoblastic nephroma and afterward tuberous sclerosis was diagnosed. The angiomyolipomatosis evidence confirmed the suspicion of a notable inclination to polydistrict malformations in tuberous sclerosis.I documenti in SFERA sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
