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Biomedical subjects

Salvatore Di Maio

Publications and source records attributed to Salvatore Di Maio.

6 recordsLinked to original sources
PubMed2006

The awareness among paediatricians of off-label prescribing in children: a survey of Italian hospitals.

OBJECTIVE: To investigate paediatricians' perception and awareness of off-label (OL) and unlicensed drug usage in clinical practice. METHODS: We conducted a questionnaire-based, observational study involving 28 departments/specialty units of four tertiary paediatrics Italian hospitals. The questionnaire was sent to a designated paediatrician in each department/specialty unit with the request that they summarise their awareness of OL prescriptions after a group discussion with all paediatricians working in the same department/unit. RESULTS: A total of 95 drugs were identified. The most common perceived reasons detected for OL use were age and formulation. Corticosteroids, ACE-inhibitors, beta-blockers and calcium-antagonists were most frequently indicated as OL for formulation, while ACE-inhibitors, new generation anti-epilepsy drugs, immunosuppressants, ketorolac and propofol as OL for age. Adrenaline, midazolam and ACE-inhibitors were indicated as unlicensed for route of administration. We found a high concordance between the OL drugs identified by physicians and those reported in the "Italian Summary Product Characteristics". CONCLUSIONS: Italian paediatricians perceived different drugs as unlicensed/OL from those identified by existing prescriptions studies. These perceived drugs may represent a priority in tackling the problem of OL usage in the hospital practice. The review of the Italian Summary Product Characteristics of some of the drugs identified, together with the monitoring of permanent clinical practice and with new clinical research, may be a step forward to reduce OL use in children.

Attitude of Health Personnel↗
PubMed2006

The prevalence of growth hormone deficiency and celiac disease in short children.

OBJECTIVES: To assess the occurrence of growth hormone deficiency (GHD) in patients with celiac disease (CD) referred for short stature. DESIGN: A retrospective, multi-center study. A total of 7066 children with short stature were referred to a number of centers for second-line evaluation over a 5-year period. All patients were screened for CD by antiendomysial antibodies (EMA) and antitissue transglutaminase IgA. Those with positive sera underwent intestinal biopsy. The EMA-negative patients and the EMA-positive ones who did not grow after 1 year of gluten-free diet underwent endocrinological investigation. RESULTS: Among the 7066 short children (age 2-14 years) evaluated, 650 (9.2%) had GHD and 44 (0.63%) had CD. An association of both CD and GHD was found in 16 short children (0.23%); these children did not grow after 1 year of gluten-free diet and therefore GH treatment was started. CONCLUSIONS: GH secretion should be evaluated in celiac patients showing no catch-up growth after an appropriate period on a gluten-free diet in spite of reversion to seronegativity for EMA.

Adolescent↗
PubMed2006

The role of transventricular neuroendoscopy in the management of craniopharyngiomas: three patient reports and review of the literature.

BACKGROUND: Management of craniopharyngiomas is problematic and often requires multimodal protocols. In the last decade neuroendoscopy has been increasingly used in the management of these lesions. PATIENT REPORTS: We report three cases of craniopharyngiomas in which various endsocopic procedures were performed. In the first case the endoscopic approach alone allowed the gross total removal of an intraventricular craniopharyngioma; in the second case the endoscopic fenestration of a cystic intraventricular craniopharyngioma allowed long-term control of the tumor with no further treatment; in the third case endoscopic fenestration and drainage of the cystic part allowed collapse of the cyst with resolution of mass effect on the visual pathway and control of hydrocephalus. The patient later underwent microsurgical removal of the solid portion of the tumor. CONCLUSIONS: Neuroendoscopy has shown great versatility in the management of intra/paraventricular craniopharyngiomas. It can be used alone to achieve gross total removal or marsupialization of cystic tumors, or, more often, in association with additional therapies, such as microsurgery, radiosurgery or intracavitary drug administration.

Biopsy↗
PubMed2005

Common carotid intima-media thickness in growth hormone (GH)-deficient adolescents: a prospective study after GH withdrawal and restarting GH replacement.

We prospectively investigated the risk of early atherosclerosis, by classical cardiovascular risk factors and intima-media thickness (IMT) at the common carotid arteries, in 23 adolescents diagnosed as GH deficient (GHD) during childhood and in 23 healthy sex-, age-, and BMI-matched controls. Measurements were performed in all subjects before stopping GH replacement. Because the diagnosis of GHD had been confirmed in 15 of the 23 adolescents, the protocol changed according to the diagnosis as follows: measurements were repeated after 6 months of GH withdrawal and 6 months of GH reinstitution in the 15 with GHD, and after 6 and 12 months of GH withdrawal, measurements were also taken in the eight non-GHD subjects. Serum IGF-I levels were in the normal range for age in all patients before GH withdrawal. When compared with controls, before GH withdrawal, GHD adolescents had reduced high-density lipoprotein cholesterol levels and increased total/high-density lipoprotein cholesterol ratio, fibrinogen, low-density lipoprotein cholesterol, and glucose levels; non-GHD adolescents had increased glucose, insulin, and homeostasis model assessment score. IMT at the common carotid arteries was similar in GHD and controls (0.52 +/- 0.03 vs. 0.55 +/- 0.06 mm; P = 0.23) and was higher in non-GHD than in controls (0.62 +/- 0.03 vs. 0.54 +/- 0.06 mm; P = 0.01). In GHD adolescents, 6 months of GH treatment withdrawal and 6 months of GH treatment reinstitution modified IGF-I levels, lipid profile, and insulin resistance but not IMT or systolic and diastolic peak velocities at the common carotid arteries. In non-GHD subjects, 12 months of GH treatment withdrawal significantly decreased IGF-I levels, IMT (to 0.54 +/- 0.06 mm; P < 0.001 vs. baseline), systolic and diastolic peak velocities, and improved insulin resistance. In conclusion, the discontinuation of GH in confirmed GHD adolescents is not followed by significant alterations of the common carotid arteries, despite the profound negative alterations of the lipid profile. In adolescents who were not confirmed to have GHD, IMT was increased while on GH therapy and normalized when they were taken off of GH.

Adolescent↗
PubMed2003

Molecular analysis of the GNAS1 gene for the correct diagnosis of Albright hereditary osteodystrophy and pseudohypoparathyroidism.

Pseudohypoparathyroidism (PHP) is a heterogeneous disease characterized by PTH resistance and classified as types Ia, Ib, Ic, and II, according to its different pathogenesis and phenotype. PHP-Ia patients show Gsalpha protein deficiency, PTH resistance, and typical Albright hereditary osteodystrophy (AHO). Heterozygous mutations in the GNAS1 gene encoding the Gsalpha protein have been identified both in PHP-Ia and in pseudopseudohypoparathyroidism (PPHP), a disorder with isolated AHO. A single GNAS1 mutation may be responsible for both PHP-Ia and PPHP in the same family when inherited from the maternal and the paternal allele, respectively, suggesting that GNAS1 is an imprinted gene. To evaluate whether molecular diagnosis is a useful tool to characterize AHO and PHP when testing for Gsalpha activity and PTH resistance is not available, we have performed GNAS1 mutational analysis in 43 patients with PTH resistance and/or AHO. Sequencing of the whole coding region of the GNAS1 gene identified 11 mutations in 18 PHP patients, eight of which have not been reported previously. Inheritance was ascertained in 13 cases, all of whom had PHP-Ia: the mutated alleles were inherited from the mothers, who had AHO (PPHP), consistent with the proposed imprinting mechanism. GNAS1 molecular analysis confirmed the diagnosis of PHP-Ia and PPHP in the mutated patients. Our results stress the usefulness of this approach to obtain a complete diagnosis, expand the GNAS1 mutation spectrum, and illustrate the wide mutation heterogeneity of PHP and PHP-Ia.

Adolescent↗