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V de Sanctis

Publications and source records attributed to V de Sanctis.

9 recordsLinked to original sources
PubMed2000

Osteochondrodystrophic lesions in chelated thalassemic patients: an histological analysis.

Some patients affected by thalassemia major and treated with an iron-chelating drug such as deferioxamine can suffer from severe osteochondrodystrophic lesions of the long bones. These lesions were radiologically and clinically evident in 2.8% of the patients we examined over the last 10 years. The aim of our research was to evaluate the morphology and morphometry of five tibial biopsy specimens taken from thalassemic patients with bone lesions and two bone biopsy specimens taken from thalassemic patients with no alteration of the long bones. All the bone samples showed a similar morphostructure. Abnormal chondrocytes, alteration of cartilage staining pattern, irregular columnar cartilage, and lacunae in the cartilaginous tissue were revealed histologically. Osteoid thickness was either normal or slightly increased. Some bone trabeculae had microfractures and some had cartilagineous oases. In five cases, iron deposition was detectable by Perls' Prussian Blue staining. It can be concluded that even in patients without radiographic signs of lesions, seriously damaged columnar cartilage, altered bone mineralization, and microfractures are common.

Adolescent↗
PubMed2000

Microstructural analysis of severe bone lesions in seven thalassemic patients treated with deferoxamine.

Osteochondrodystrophic lesions, mainly affecting long bone metaphyses, can be radiologically evident in homozygotic thalassemic patients treated with deferoxamine, and their incidence rate varies among authors. The clinical and radiological appearance of these lesions is described in the literature, but microstructural data are still lacking. The aim of our research was to evaluate the microstructure of five tibial biopsy specimens from thalassemic patients with bone lesions (5 cases out of 180 patients followed for the last 10 years, i.e., 2.8%) and two bone biopsy specimens from thalassemic patients with no radiological alteration of the long bones. As control, bone tissue taken from autoptic tibiae of two subjects with no skeletal pathology was used. Using microradiography and X-ray diffraction (XRD), we found a reduced and irregular mineralization of the bone (compared with controls) in thalassemic subjects. Bone tissue microhardness was also significantly reduced. Nevertheless, bone apatite lattice was unaltered and no 'foreign' crystallographic phase was recorded by XRD. In conclusion, all the patients shared a similar picture of abnormal bone, even with no radiological evidence of lesion.

Adolescent↗
PubMedDate not supplied

Coagulation study in patients who had undergone surgery for meningioma.

Abnormal fibrinolysis and thrombotic complications have been often observed in patients who had undergone surgery for meningioma. Fourteen patients, affected by meningioma, were studied before surgery, during surgery and 24 h after surgery in order to evaluate the modifications of the fibrinolysis system and the coagulation physiological inhibitors. Before surgery, no patient showed hyperfibrinolysis and/or modifications of coagulation physiological inhibitors. During surgery, an activation of fibrinolysis with pathological levels of tissue plasminogen activator activity (mean = 6.33 U/dl, SD = 7.9, p = 0.02) and increased levels of fibrin degradation products (mean = 0.21, SD = 0.18, p = 0.002) was noted. Modifications of the fibrinolysis parameters occurred only in 9/14 patients (64%). These patients presented a more vascularized tumour, revealed before surgery by computerized tomography scan and cerebral arteriography and directly confirmed during the resection. Twenty-four hours after surgery no patient presented fibrinolysis activation. There was no evidence of disseminated intravascular coagulation in our patients. None of them presented pathological decrease of the physiological coagulation inhibitors or thrombotic complications. In conclusion, during surgery, fibrinolysis parameters show important modifications in patients with vascularized meningioma suggesting an ongoing tumour-host interaction. These variations must be taken in account, in order to plan timely a correct therapeutic approach.

Adult↗
PubMedDate not supplied

McCune-Albright syndrome: a longitudinal clinical study of 32 patients.

We report the diagnostic clinical features and their long term evolution in 32 patients with McCune-Albright syndrome. Patient data are made up of two periods: the first, classified as personal history, is from birth until the time when the diagnosis of McCune-Albright syndrome was made; the second, classified as clinical observation, is from the first observation until the end of follow up. The total duration of these two periods was 9.6+/-2.9 yr; mean age at first observation was 5.7 yr (range 0.7-11 yr). The probability of manifesting main clinical signs according to age was calculated: almost all had skin dysplasia at birth, 50% probability of peripheral precocious puberty in females at 4 years and 50% of bone dysplasia at 8 years of age were found. Other clinical signs had diagnostic relevance when preceding the main signs leading to diagnosis of McCune-Albright syndrome even without specific genetic investigation. The most important clinical manifestations have different evolutions: skin lesions increase in dimensions according to body growth; precocious puberty in females evolves rapidly but periods of regression can be seen in some patients; bone dysplasia in most patients evolves with an increase both in the number of affected bones and in the severity of lesions.

Child↗