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Biomedical subjects

G Cordone

Publications and source records attributed to G Cordone.

At least 19 recordsLinked to original sources

Comparison of growth retarding effects induced by two different glucocorticoids in prepubertal sick children: an interim long-term analysis.

The low interference with growth expected in child for a cortisol analogue, deflazacort (DFZ), prompted us to verify if DFZ could affect growth less than prednisone (PDN). An interim analysis relative to 27 girls and 38 boys (out of 100 expected) aged 3-12 yrs, after a median period of 14 mo.s is reported. Children with connective tissues (CTD) and glomerular disorders (KD) were randomly allocated to DFZ or PDN. Anthropometric measurements and maturity ratings were performed. Mean daily doses of PDN (or DFZ equivalent), from 0.57 to 0.64 mg/kg (DFZ 0.92 to 0.94 mg/kg) to induce control and from 0.19 to 0.39 mg/kg (DFZ 0.34 to 0.36 mg/kg) to maintain disease under control were given in CTD and KD, respectively. The increase in bone age delay over time was significantly greater than for PDN (-4.0 mo/yr) than DFZ (-1.8 mo/yr) in the overall group. The increases in statural age delay and loss over time were significantly greater than for PDN (-5.9 and -5.9 mo/yr) than DFZ (-2.4 and -2.4 mo/yr), only in children with "taller" midparents. Although doses of DFZ 1.1-1.8 times those of PDN were given, growth retardation in PDN-treated children was nevertheless 2.3-2.5 times that in DFZ-ones.

Age Determination by Skeleton

Abnormal tau-reactive filaments in olfactory mucosa in biopsy specimens of patients with probable Alzheimer's disease.

We immunocytochemically analyzed pieces of olfactory mucosa removed by biopsy in 8 patients with probable Alzheimer's disease (AD) and 6 age-matched controls, with tau and ubiquitin antisera. There were tau-reactive and, partially, ubiquitin-reactive dystrophic neurites in the lamina propria of olfactory mucosa in all AD cases. The tau-reactive neurites contained abnormal straight filaments, 15 to 18 nm in diameter, morphologically identical to those found in AD cerebral brain tissue obtained at autopsy. Tau and ubiquitin immunoreactivity were absent in controls. If these neuritic alterations are confirmed in a larger number of cases, analysis of olfactory mucosa may increase the current reliability of clinical diagnosis of AD.

Alzheimer Disease

[Infantile dermatomyositis. Clinical aspects and prospective treatments].

Juvenile dermatomyositis (DMI) is a rare illness of multisystem inflammatory disease of unknown etiology. It is usually considered as a part of the spectrum of polymyositis/dermatomyositis (PM/DM), and is distinguished from the adult form in having a number of different clinical features as well as distinctive pathologic findings. Although the clinical findings and course of the disease, laboratory evaluation and therapy have been reviewed, many of these studies are old and may not accurately reflect the current status of the disease as it is now defined and treated. Generally it is first diagnosed by the appearance of typical signs in both the skin and muscles. The clinical picture shows early signs of heterogeneity and is confirmed by these latter developments. Its classification falls into three groups: acute single cycle, chronic polycycle and continued chronic; this sickness invariably evolves into one or another of these three. Our current work involves 15 cases of DMI; 8 have acute monocycle, 4 chronic polycycle and 3 continued chronic. This illustrates the clinical and biological heterogenousness of this illness which in itself presents extreme difficulties when considering possible treatment. From our experience, high dose of corticosteroida intraveins have had the best result. Where cases have been acute at the beginning of the sickness and where there has been complications, particularly those of a bronco-pulmonary nature (we treated 3 such cases) it has proven to be beneficial to use corticosteroid together with the immunosuppresant: ciclofosfamide. The use of ciclosporina in one case who had resisted other forms of therapy has appeared to be effective, though this has yet to be verified.

Acute Disease

Cytosolic and nuclear androgen receptor activity in the cancer of the larynx.

It is well known that the larynx is a target organ for androgens and the cancer of larynx is more frequent in male subjects. We have evaluated the androgen receptors (AR) in the cytosol (ACR) and in salt extractable (ANR) and salt resistant nuclear fraction (AMR) in a group of 24 male patients with cancer of the larynx surgically removed. In addition specimens obtained from the normal mucosa of the same subjects were analyzed. In 5 patients estrogen (ER) and progesterone (PgR) receptors were also assayed. In all subjects blood samples were taken before surgery for the assay of the following hormones: LH, FSH, estradiol, testosterone, dihydrotestosterone, delta 4-androstenedione, dehydroepiandrosterone sulfate, cortisol. The results observed showed that 18 out of 24 normal larynx mucosa specimens and 17 out of 24 larynx cancer specimens were positive for ACR or ANR or AMR. The 5 samples of normal and cancer tissues analyzed for ER and PgR were negative. In conclusion there is no significant correlation between AR positivity from one size, histology, degree of differentiation and invasivity of the cancer, age of patients and hormonal blood levels from the other. The high ANR and AMR positivity (normal hormonal translocation and binding on DNA acceptors) confirm that the normal and cancer larynx are target tissue for androgens and establish the hormone dependence of this cancer. Hormonal therapy could be envisaged as an alternative or a complementary therapy for this type of cancer at least in the cases in which the analysis of hormone receptors will prove to be positive.

Adult

Xeroderma pigmentosum (complementation group D) mutation is present in patients affected by trichothiodystrophy with photosensitivity.

We studied the response to UV irradiation in cells from four patients, from three apparently unrelated families, affected by trichothiodystrophy (TTD). They showed all the symptoms of this rare autosomal recessive disorder (brittle hair with reduced sulfur content, mental and physical retardation, ichthyosis, peculiar face) together with photosensitivity. We found a decreased rate of duplicative DNA synthesis in stimulated lymphocytes, reduced survival in fibroblasts, and very low levels of unscheduled DNA synthesis (UDS) in Go lymphocytes and fibroblasts after UV irradiation. Complementation studies showed that normal values of UDS are restored in heterokaryons obtained by fusion of TTD cells with normal and xeroderma pigmentosum (XP)-complementation group A-cells. In contrast the defect is not complemented by fusion with XP-complementation group D-fibroblasts.

Cells, Cultured

Evaluation of the cochleovestibular function in patients treated with carboplatin for ovarian carcinoma.

Carboplatin (JM8), a new cisdiamminodichloroplatin (CDDP) analogue, has exhibited the same antitumor as CDDP. In our pilot study, aiming at optimizing the dosage of this drug in a combination regimen with other chemotherapics, we planned to evaluate the existence of drug related damage on cochleo-vestibular structures. The study was performed on 9 patients suffering from advanced ovarian carcinoma. The results obtained in our study showed that patients undergoing carboplatin chemotherapy do not require an audiometric check-up. In order to assess definitely the existence of vestibular toxicity of this drug, larger studies planned to test JM8 in a high-dosage regimen are desirable.

Aged

T-lymphocytes phenotype and functions in patients with head and neck cancer.

We studied the phenotype of T-lymphocytes isolated from 18 patients with head and neck cancer, their capacity to express Ia antigens upon activation by lectins in vitro, their capacity to function either as responder or stimulator cells in autologous mixed lymphocyte reaction, and their capacity to cooperate with the normal adherent suppressor cells (NASC). The T-lymphocytes isolated from these patients have several functional defects including an impaired capacity to activate allogeneic lymphocytes in mixed lymphocyte reactions (MLRs), a lack of a proliferative capacity in autologous MLRs, an impaired sensitivity to inhibition by NASC, and an impaired capacity to express Ia antigens upon activation by mitogens in vitro. These data indicate that, in patients with head and neck cancer, immune function is characterized by a defect in T-lymphocytes functions which concerns the process of cell to cell cooperation.

Aged

Decreased sensitivity of T lymphocytes to normal adherent suppressor cells in patients with head and neck cancer.

The sensitivity of T Lymphocytes to the inhibitory action of normal adherent cells in the mixed lymphocyte reaction (MLR) was studied in 20 subjects with head and neck cancer. T lymphocytes from cancer patients proliferated in the MLR both in the absence and in the presence of increasing numbers of autologous as well as allogeneic adherent cells, while the blastogenesis of T lymphocytes from controls was inhibited up to 70% by the addition of adherent cells to the culture. Such a lack of sensitivity to adherent cells in cancer patients occurred both in allogeneic and in autologous MLR. These observations indicate that the immunocompetence of patients with head and neck cancer may be related to a defect of macrophage-T lymphocyte interaction similar to the one described in patients with common varied immunodeficiency.

Adult