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V De Sanctis

Publications and source records attributed to V De Sanctis.

At least 19 recordsLinked to original sources
PubMed2006

Menstrual disorders in adolescence.

Altered frequency of the menstrual cycle accompanied by pain are manifestations of functional anomalies of the female reproductive system. These symptoms require prompt and accurate diagnosis and therapy to prevent a chronic condition that can seriously disturb the adolescent's psychic well being. The most common anomalies of the menstrual cycle and the causes of altered cycle frequency are outlined, as are useful criteria for diagnosing premenstrual syndrome dysmenorrhea and for distinguishing the causes and alterations in frequency and amount of menstrual discharge from other disturbances, including amenorrhea and abnormal uterine bleeding. The treatment of dysmenorrhea and quantitative alterations of the menstrual cycle is the focus of this article.

Adolescent↗
PubMed2006

Geometry and bone density.

Bone development is a key process in the growing child. It is, therefore, of paramount importance to survey this process, which is characterized by increasing length and size of the bone together with its progressive mineralization. The bone status can be evaluated by different techniques, each of them having its pros and cons. Furthermore, it should be underlined that the results of bone assessment depend not only from the employed technique but also from the auxological characteristics of the subjects. It is, therefore, the aim of this review to examine the characteristics of the various methods of bone evaluation, such as dual energy X-ray absorptiometry (DEXA), peripheral quantitative computed tomography (pQCT), ultrasound and metacarpal index and to explain how changes in bone structure and geometry may influence the results.

Absorptiometry, Photon↗
PubMed2005

Final height in short polytransfused thalassemia major patients treated with recombinant growth hormone.

We measured the final height (FH) of 25 short polytransfused thalassemia major (Th) patients (18 males) with a reduced GH reserve treated for 3.3 +/- 1.2 yr with recombinant GH (rhGH), 0.2 mg/kg/week sc. At baseline, all patients were clinically prepubertal; their chronological (CA) and bone ages (BA) were 13.6 +/- 2.0 and 11.4 +/- 1.6 yr, respectively. In 9 out of 18 males and 5 out of 7 females, the onset of puberty occurred spontaneously during the treatment. At the end of the rhGH administration, the height of the enrolled children was not significantly increased when calculated for CA (HxCA), while it was significantly decreased (p=0.004) when calculated for BA (HxBA); the BA increase (3.29 +/- 1.65 yr) was significantly higher (p<0.001) than the height age increase (2.16 +/- 0.98 yr). The FHxCA showed a significant increase (p=0.001) compared to both baseline and the end of therapy, while the FHxBA was significantly decreased (p<0.001) compared with the corresponding value at baseline. At the end of therapy, both HxCA and HxBA resulted positively related to the BA at baseline (r=0.50 and 0.42, p=0.012 and 0.034, respectively). FH was positively correlated with CA (r=0.63, p=0.001), BA (r=0.68, p<0.001) and HxBA (r=0.59, p=0.002) evaluated at baseline, and with both HxCA and HxBA (r=0.82 and 0.74, respectively, p<0.001), evaluated at the end of treatment. A negative correlation was found between FH and the length of treatment (r=-0.56, p=0.004). Our data seem to exclude that prolonged rhGH therapy could improve FH in Th patients; on the contrary, a negative effect may be hypothesized.

Adolescent↗
PubMed2004

Antioxidant capacity in seminal plasma of transfusion-dependent beta-thalassemic patients.

BACKGROUND: Homozygous beta-thalassemia is a haemolytic disorder with a high potential for oxidative damage, due to the high circulating iron levels. Enzymatic and non enzymatic antioxidant capacities, as well as lipoperoxide content, were investigated in seminal plasma of these patients to evaluate a possible oxidative stress. METHODS: Semen samples from 10 transfusion-dependent beta-thalassemic patients and 18 control subjects were examined. The assessment of the seminal antioxidant capacity included spectrophotometrical assays for determination of superoxide dismutase and catalase activity and of the total antioxidant status value. Furthermore, malondialdehyde level was detected as marker of lipoperoxidation. RESULTS: All the beta-thalassemic patients showed high serum ferritin levels, progressive sperm motility below 50 %, and normal sperm count (median: 43 x 10 (6) sperm/ml). Increased superoxide dismutase ( p < 0.01) and catalase ( p < 0.001) activities, but unaltered total antioxidant status values, were detected in the patients' seminal plasma. Furthermore, augmented malondialdehyde levels ( p < 0.001) were measured in the patients. CONCLUSIONS: Seminal antioxidant pattern of iron overloaded beta-thalassemic patients indicated the hyperactivation of the enzymatic free-radical scavengers which could be explained as a compensatory mechanism to possible high levels of reactive oxygen species. Furthermore, the increase of seminal lipoperoxidation suggested an oxidative stress in semen of these patients and it could have contributed to the impairment of sperm motility.

Adult↗
PubMed2004

Hyperparathyroidism.

Hyperparathyroidism is a disease characterized by hypercalcemia with hypophosphoremia resulting from increased secretion of parathyroid hormone (PTH). The disease may be divided into 3 forms: a) primary, b) secondary, c) tertiary (secondary refractory form). Primary hyperparathyroidism is rare in children; hyperplasia is more frequent during the early years of life (neonates and infants) and is difficult to distinguish from adenoma in children. The disease may be asymptomatic; elevated calcemia levels (>12 <13.5 mg/dl) are accompanied by anorexia, asthenia and persistent stipsis; severely elevated concentrations (>13.5 mg/dl) are accompanied by nausea, vomiting, polyuria due to osmosis, with dehydration and progressive onset of lethargy, stupor and coma. Osteopenia or osteitis fibrosa cystica may be present due to augmented bone resorption. Height and weight increases are altered due to anorexia and dehydration. Differential diagnosis includes iatrogenic causes of hypercalcemia (excessive vitamin D intake, prolonged immobilization, etc.) and idiopathic familial hypercalcemia. Emergency treatment is required in cases of extremely elevated hypercalcemia (Ca >13.5-14 mg/dl), due to risk of injury to the heart, the central nervous system, the gastrointestinal tract and the kidneys. The 4 cardinal points of treatment are: hydration, calciuresis, inhibition of bone calcium resorption, treatment of the cause underlying hyperparathyroidism. Secondary hyperparathyroidism is found in cases where chronic hypocalcemia is present, particularly in chronic renal failure, untreated deficiency rickets, chronic intestinal malabsorption, hepatobiliary disease, types I and II vitamin D-dependent rickets, tubular acidosis or Fanconi's syndrome. The tertiary form is distinguished by the autonomous nature of the parathyroid glands which have become hypertrophic/hyperplastic due to uncontrollable, chronic severe renal failure. It can also be of iatrogenic origin due to excessive intake of inorganic phosphates in familial hypophosphatemic rickets or chronic vitamin D deficiency.

Calcium↗
PubMed2003

Defective function of Fas in T cells from paediatric patients with autoimmune thyroid diseases.

Triggering of the Fas receptor induces T cell apoptosis and is involved in shutting-off the immune response. Inherited defects impairing Fas function cause the autoimmune lymphoproliferative syndrome, and may play a role in other autoimmune diseases. The aim of this work was to analyse the Fas function in paediatric patients with thyroid autoimmunities. We found that T cells from 24/28 patients with Graves' disease (GD) and 12/35 patients with Hashimoto's thyroiditis (HT) displayed defective Fas function. In HT, the defect was more frequent in patients requiring replacement therapy (11/20) than in those not requiring (1/15); moreover, in untreated HT the highest defect was displayed by patients with the highest levels of autoantibodies. Fas was always expressed at normal levels and no Fas mutations were detected. Analysis of the healthy parents of seven Fas-resistant patients showed that several of them were Fas-resistant, which suggests a genetic component. Fusion of Fas-resistant T cells with the Fas-sensitive HUT78 T cell line generated Fas-resistant hybrid cells, which suggests the presence of molecules exerting a dominant negative effect on Fas function. Analysis of Fas-induced activation of caspase-8 and -9 showed decreased activity of both caspases in HT, whereas activity of caspase-9 was increased and that of caspase-8 was decreased in GD. These data suggest that heterogeneous inherited defects impairing Fas function favour the development of thyroid autoimmunities.

Adolescent↗
PubMed2003

Growth and puberty in thalassemia major.

Present transfusional regimen protocols increase the life expectancy of patients with beta-thalassemia major, but cause a progressive iron overload that can be prevented or limited only by appropriate iron chelation. Siderosis is responsible for the clinical complications of the disease. Short stature and hypogonadism are extremely frequent in patients with thalassemia. Many factors are responsible for short stature in patients with thalassemia, the most important of which are dysfunction of the GH-IGF-I axis and desferoxamine (DFX)-induced bone dysplasia. Hypogonadism is the most frequent endocrine complication, mostly due to gonadotrophins deficiency secondary to iron overload. Sex steroid treatment for induction of puberty and/or maintenance of sexual characteristics is necessary. Both short stature and hypogonadism are present in a significant percentage of bone marrow transplanted patients with thalassemia. Factors responsible for short stature are previous iron overload, liver impairment, DFX treatment, and toxicity of chemotherapeutic agents. In some patients absence of pubertal development is due to gonadotropin insufficiency, probably secondary to previous iron overload; other patients exhibit hypergonadotrophic hypogonadism due to the toxic effect of chemotherapeutic agents on the gonads. Both groups need hormonal replacement therapy. These data support the need for vigilant follow-up of patients with thalassemia before and after transplantation, in order to treat endocrine dysfunctions at the appropriate age.

Body Height↗
PubMed2002

Regression of autoimmunity and abnormal glucose homeostasis in an adolescent boy with silent coeliac disease.

UNLABELLED: We report on a 15-y-old adolescent boy affected by silent coeliac disease, abnormalities in glycoregulation and with autoantibodies specific to diabetes mellitus type 1 (ICA: islet cell antibodies) and GAD 65 (autoantibodies against glutamic acid decarboxylase), in whom normalization of glycoregulation and disappearance of the immunological markers of pre-diabetes were observed after 6 mo on a gluten-free diet. The patient was followed-up for 36 mo and showed a normal insulin response to an intravenous glucose tolerance test and no markers of autoimmunity. It is possible that undiagnosed coeliac disease over a long period could lead to a direct autoimmune mechanism against pancreatic beta cells. CONCLUSION: Our findings seem to confirm the theory that undiagnosed coeliac disease can induce an autoimmune process against the pancreatic beta cells and that, following a gluten-free diet, the immunological markers for diabetes mellitus type 1 will disappear.

Adolescent↗
PubMed2002

Growth and puberty and its management in thalassaemia.

The purpose of this review is to report the personal experience on growth and pubertal development in a large number of thalassaemic and ex-thalassaemic patients followed at the Pediatric and Adolescent Unit of Ferrara. Secondary amenorrhoea (SA), hypogonadism and short stature are the commonest endocrine and auxological complications. The anterior pituitary gland is particularly sensitive to free radical oxidative stresses and exposure to this. Magnetic resonance imaging (MRI) shows that even a modest amount of iron deposition within the anterior pituitary can interfere with its function. Other possible cause of hypogonadism in beta-thalassaemia major include liver disorders, chronic hypoxia, diabetes mellitus and zinc deficiency. The treatment of pubertal disorders consists of hormone replacement therapy with sex steroids. Successful induction of spermatogenesis and ovulation has been reported after hormonal stimulation with gonadotrophins. Height above the 10th centile was achieved in 50% of males and 64% of females. Eight prepubertal thalassaemic patients, 6 males and 2 females, ranging in age from 8.6 to 11.7 years, were treated with GH. After the first 12 months of GH treatment a significant increase of growth velocity was observed in 6 patients who doubled growth velocity before basal value (4 cm or more above the basal value), 2 patients had a partial response (2-4 cm above the basal value). In the following 3 years all thalassaemic patients had a partial response to the treatment with GH. These data indicate that despite somewhat reduced sensitivity to GH, compared to GH deficiency children, there is evidence indicating that thalassaemic patients may benefit from GH treatment. Sixty-eight thalassaemic patients (30 males and 38 females) who had successfully undergone bone marrow transplantation (BMT) during childhood were studied. Following BMT growth rate decelerated when compared to Tanner and Whitehouse standards. Twenty-nine ex-thalassaemics reached final height. The patterns of growth during puberty was variable in ex-thalassaemic males, while in all but 3 ex-thalassaemic females we observed an improvement in the percentile of standing height. A gonadal dysfunction was found in 68% of ex-thalassaemic patients. Since the quality of life of these patients is an important aim, it is vital to monitor carefully the growth and pubertal development in order to detect abnormalities and to initiate appropriate and early treatment.

Adolescent↗
PubMed2002

Thyroid nodules.

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Biopsy, Needle↗
PubMed2002

Hypothyroidism.

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Humans↗
PubMed2002

[Chest pain in adolescents].

In children and in adolescents, chest pain is relatively common and self-limiting. The close association between chest pain, cardiopathies and sudden death is the cause of intense anxiety in boys and their parents and even doctors. The most frequent causes of chest pain, the diagnosis and the eventual treatment are examined. Finally, the causes of chest pain due to drug abuse (in particular cocaine) and to CO poisoning are also examined. Good knowledge of the problem, an accurate anamnesis and a careful objective exam are useful to choose the most suitable treatment.

Adolescent↗
PubMed2002

[Asthenia in adolescents].

Fatigue is defined as a subjective sensation of tiredness or weariness that occurs at rest. The perception of fatigue among 12-15 years-old Italian adolescents in a school survey was about 70%. Generally the symptoms are reported after a viral illness or an infection. In adolescents with persistent or severe fatigue a selected screening evaluation to look for an underlying organic disorder is warranted. A practical diagnostic approach is given and a brief description of chronic fatigue syndrome is reported according to CDC revised diagnostic criteria published in 1997.

Adolescent↗