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Franca Gori

Publications and source records attributed to Franca Gori.

9 recordsLinked to original sources

Annuloaortic ectasia and giant cell arteritis.

BACKGROUND: Thoracic aortic aneurysm, aortic dissection and aortic valve regurgitation have been widely described in patients with Horton disease, also known as giant cell arteritis. We present our midterm experience with patients with these features. METHODS: A total of 386 cases of ascending aorta and aortic valve replacement performed for thoracic aortic aneurysm and aortic insufficiency between 1998 and 2004 were reviewed. Among them 10 cases of histopathologically confirmed GAA were identified. Patients were predominantly female (90%); the mean age was 74.5 +/- 4.6 years. RESULTS: Eight patients (80%) showed typical annuloaortic ectasia, leading to significant aortic valve regurgitation. These subjects underwent a Bentall operation. Two patients whose sinuses seemed undilated and macroscopically normal had separate valve graft replacement at first operation and underwent reoperation due to dilatation of the native sinuses. Eight patients had partial aortic arch replacement (hemiarch), and 1 underwent total arch replacement. Six-year survival was 0.9 +/- 0.09; freedom from reoperation at 6 years was 0.77 +/- 0.13. CONCLUSIONS: Annuloaortic ectasia is a common finding in giant cell arteritis. In patients with Horton disease, the aortic root should always be replaced regardless of macroscopic findings.

Aged↗

Isolated inflammatory aneurysm of superior mesenteric artery: unexpected pathologic diagnosis.

A 78-year-old woman was admitted to our department because of the incidental finding during routine abdominal ultrasound examination of a large aneurysm of the superior mesenteric artery. Presence of the aneurysm was confirmed at computed tomography and digital subtraction angiography, but its inflammatory origin was not recognized. No other aneurysm localization was found. Macroscopic intraoperative findings suggested the inflammatory nature of the lesion, but only histopathologic examination was diagnostic. Aneurismorraphy with prosthetic in-line reconstruction was performed. This report presents the previously undescribed possibility of an isolated inflammatory aneurysm of the superior mesenteric artery.

Aged↗

Giant cell arteritis presenting with annuloaortic ectasia.

Four cases of giant cell arteritis causing severe aortic regurgitation secondary to an aneurysm of the ascending aorta are described. In two cases, the nature of the aortic pathology could be suspected considering the past clinical evidence of temporal arteritis and/or polymyalgia rheumatica. In the two other cases, the cardiothoracic manifestations represented the onset of Horton disease.

Aged↗

Familial amyloid polyneuropathy with genetic anticipation associated to a gly47glu transthyretin variant in an Italian kindred.

The most frequent localization of amyloid in transthyretin (TTR) mutations is in the peripheral nerve, causing familial amyloidpolyneuropathy (FAP). It is generally accompanied by involvement of other organs such as the myocardium and kidney. To date, over 70 TTR point mutations have been reported in literature, with different phenotypes depending on the location of the mutation in the TTR gene. This paper deals with a point mutation in exon 2 position 47 of the TTR gene, encoding the substitution of glycine with glutamate. The mutation was found in an Italian family with 5 patients over 3 generations. The phenotype was characterised by peripheral neuropathy and autonomic dysfunction, associated in some patients with cardiomyopathy and renal involvement. The symptoms were very severe and the patients did not survive long, thus suggesting the aggressive nature of the pathological process. Moreover, in the succeeding generations of this family, there was genetic anticipation in the age of onset of the disease.

Adult↗

Malignant fibrous histiocytoma as a primary cardiac tumor.

We describe the case of a malignant fibrous histiocytoma (MFH) of the heart, presenting with an obstinate cough and clinically misdiagnosed as myxoma, in an 80-year-old woman. Fourteen months after surgical removal, an embolic episode revealed recurrence of the tumor. To plan the best surgical management, MFH, rare as it is, should be borne in mind when evaluating a cardiac intracavitary mass, especially if located in the left atrium.

Aged↗