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Your search keyword '"ANDRIA, GENEROSO"' showing total 10 results
Start Over Author "ANDRIA, GENEROSO" Journal the journal of pediatrics
10 results on '"ANDRIA, GENEROSO"'

3. BRAIN DAMAGE IN GLYCOGEN STORAGE DISEASE TYPE I

Author

MELIS, DANIELA, PARENTI, GIANCARLO, CASA, ROBERTO DELLA, SIBILIO, MICHELINA, ROMANO, ALFONSO, DI SALLE, FRANCESCO, ELEFANTE, RAFFAELE, MANSI, GIUSEPPINA, SANTORO, LUCIO, PERRETTI, ANNA, PALUDETTO, ROBERTO, SEQUINO, LUIGI, and ANDRIA, GENEROSO

Published
2004

7. Brain damage in glycogen storage disease type I

Author

Generoso Andria, Roberto Della Casa, Francesco Di Salle, Daniela Melis, Raffaele Elefante, Anna Perretti, Giancarlo Parenti, Luigi Sequino, Lucio Santoro, Alfonso Romano, Roberto Paludetto, G. Mansi, Michelina Sibilio, Melis, Daniela, Parenti, Giancarlo, DELLA CASA, Roberto, Sibilio, Michelina, Romano, Alfonso, DI SALLE, Francesco, Elefante, Raffaele, Mansi, Giuseppina, Santoro, Lucio, Perretti, ANNA CARMELA AGNESE, Paludetto, Roberto, Sequino, Luigi, and Andria, Generoso

Subjects
Adult, Male, medicine.medical_specialty, complications, Adolescent, Brain damage, Glycogen Storage Disease Type I, Hypoglycemia, Electroencephalography, Somatosensory system, medicine, Humans, Preschool, Child, Evoked Potentials, Neurologic Examination, Brain Diseases, Glycogen storage disease type I, medicine.diagnostic_test, Brain Diseases, Metabolic, business.industry, Brain morphometry, Infant, Magnetic resonance imaging, medicine.disease, Magnetic Resonance Imaging, Surgery, Italy, Adolescent, Adult, Brain Diseases, Metabolic, epidemiology/etiology/pathology, Case-Control Studies, Child, Child, Preschool, Electroencephalography, Evoked Potentials, Female, Glycogen Storage Disease Type I, complications, Humans, Hypoglycemia, complications, Infant, Italy, epidemiology, Magnetic Resonance Imaging, Male, Neurologic Examination, Somatosensory evoked potential, epidemiology/etiology/pathology, Case-Control Studies, Child, Preschool, Anesthesia, Pediatrics, Perinatology and Child Health, epidemiology, Female, medicine.symptom, business
Abstract

OBJECTIVE: To investigate brain morphology and function in patients with glycogen storage disease type I (GSDI). STUDY DESIGN: Nineteen patients (13 females and 6 males, aged 0.9-22.6 years) and 38 sex- and age-matched controls entered the study. Neurological examinations, psychometric tests (IQ, tests of performance and verbal abilities), standard electroencephalogram (EEG), somatosensory (SEPs), visual (VEPs), and brain-stem auditory evoked potentials (BAEPs), and brain magnetic resonance imaging (MRI) were performed. RESULTS: The results of tests of performance ability were lower in patients than in controls (P

Published
2004

8. Recurrent fatal pulmonary alveolar proteinosis after heart-lung transplantation in a child with lysinuric protein intolerance

Author

G. Parenti, Paola Francalanci, Carlo Dionisi-Vici, Francesca Santamaria, Generoso Andria, C. Squitieri, Gianluca Brancaccio, Patrizia D'Argenio, Francesco Parisi, Gianfranco Sebastio, Santamaria, Francesca, Brancaccio, G, Parenti, Giancarlo, Francalanci, P, Squitieri, C, Sebastio, Gianfranco, DIONISI VICI, C, D'Argenio, P, Andria, Generoso, and Parisi, F.

Subjects
Male, Pathology, medicine.medical_specialty, Heart-Lung Transplantation, Pulmonary Alveolar Proteinosis, Pathogenesis, Fatal Outcome, Recurrence, medicine, Humans, medicine.diagnostic_test, business.industry, Lysine, Respiratory disease, Infant, respiratory system, medicine.disease, Lysinuric protein intolerance, Transplantation, Bronchoalveolar lavage, Pediatrics, Perinatology and Child Health, Amino Acid Transport Disorders, Inborn, Pulmonary alveolar proteinosis, business, Complication
Abstract

We present a case of recurrent pulmonary alveolar proteinosis after heart-lung transplantation in a child with lysinuric protein intolerance. The recurrence of the pulmonary disease provides further insight regarding the possible pathogenesis of pulmonary alveolar proteinosis and therapeutic options for this complication.

Published
2004

9. The Growth Hormone-Insulin-like Growth Factor Axis in Glycogen Storage Disease Type 1: Evidence of Different Growth Patterns and Insulin-like Growth Factor Levels in Patients with Glycogen Storage Disease Type 1a and 1b

Author

Giancarlo Parenti, Carolina Di Somma, Mariacarolina Salerno, F. Balivo, Daniela Melis, Pasquale Piccolo, Annamaria Colao, Roberto Della Casa, Rosario Pivonello, Generoso Andria, Melis, Daniela, Pivonello, R, Parenti, Giancarlo, DELLA CASA, Roberto, Salerno, Mariacarolina, Balivo, F, Piccolo, P, Di Somma, C, Colao, A, and Andria, Generoso

Subjects
Male, medicine.medical_specialty, Time Factors, Adolescent, medicine.medical_treatment, Enzyme-Linked Immunosorbent Assay, Glycogen Storage Disease Type I, Short stature, Body Mass Index, Young Adult, Basal (phylogenetics), Insulin-like growth factor, Risk Factors, Somatomedins, Internal medicine, Prevalence, Humans, Medicine, Glycogen storage disease, Prospective Studies, IGF, Child, Growth Disorders, business.industry, Growth factor, Insulin, Prognosis, Growth hormone–releasing hormone, medicine.disease, Body Height, Growth hormone secretion, Cross-Sectional Studies, Endocrinology, Italy, Case-Control Studies, Growth Hormone, Pediatrics, Perinatology and Child Health, Female, Immunoradiometric Assay, medicine.symptom, business, Biomarkers, Follow-Up Studies
Abstract

Objectives To investigate the growth hormone (GH)-insulin-like growth factor (IGF) system in patients with glycogen storage disease type 1 (GSD1). Study design This was a prospective, case-control study. Ten patients with GSD1a and 7 patients with GSD1b who were given dietary treatment and 34 sex-, age-, body mass index-, and pubertal stage-matched control subjects entered the study. Auxological parameters were correlated with circulating GH, either at basal or after growth hormone releasing hormone plus arginine test, insulin-like growth factors (IGF-I and IGF-II), and anti-pituitary antibodies (APA). Results Short stature was detected in 10.0% of patients with GSD1a, 42.9% of patients with GSD1b ( P = .02), and none of the control subjects. Serum IGF-I levels were lower in patients with GSD1b ( P = .0001). An impaired GH secretion was found in 40% of patients with GSD1a ( P = .008), 57.1% of patients with GSD1b ( P = .006), and none of the control subjects. Short stature was demonstrated in 3 of 4 patients with GSD1b and GH deficiency. The prevalence of APA was significantly higher in patients with GSD1b than in patients with GSD1a ( P = .02) and control subjects ( P = .03). The GH response to the provocative test inversely correlated with the presence of APA ( P = .003). Compared with levels in control subjects, serum IGF-II and insulin levels were higher in both groups of patients, in whom IGF-II levels directly correlated with height SD scores ( P = .003). Conclusion Patients with GSD1a have an impaired GH secretion associated with reference range serum IGF-I levels and normal stature, whereas in patients with GSD1b, the impaired GH secretion, probably because of the presence of APA, was associated with reduced IGF-I levels and increased prevalence of short stature. The ncreased IGF-II levels, probably caused by increased insulin levels, in patients with GSD1 are presumably responsible for the improved growth pattern observed in patients receiving strict dietary treatment.

Published
2010

10. Increased Prevalence of Thyroid Autoimmunity and Hypothyroidism in Patients with Glycogen Storage Disease Type I

Author

Giancarlo Parenti, Daniela Melis, Gaetano Lombardi, Annamaria Colao, Gianfranco Sebastio, Rosario Pivonello, Generoso Andria, Roberto Della Casa, Mariacarolina Salerno, Melis, Daniela, Pivonello, Rosario, Parenti, Giancarlo, DELLA CASA, Roberto, Salerno, Mariacarolina, Lombardi, Gaetano, Sebastio, Gianfranco, Colao, Annamaria, and Andria, Generoso

Subjects
Male, endocrine system diseases, medicine.medical_treatment, Thyrotropin, increased prevalence, Comorbidity, Thyroid Function Tests, Thyroiditis, Reference Values, Prevalence, Glycogen storage disease, Prospective Studies, Child, Glycogen storage disease type I, medicine.diagnostic_test, biology, Prognosis, glycogen storage disease type I, thyroid autoimmunity, Female, increased prevalence, thyroid autoimmunity, hypothyroidism, glycogen storage disease type I, hormones, hormone substitutes, and hormone antagonists, Adult, endocrine system, medicine.medical_specialty, Adolescent, Risk Assessment, Thyroid function tests, Statistics, Nonparametric, Age Distribution, Thyroid peroxidase, Internal medicine, medicine, Humans, Sex Distribution, Probability, hypothyroidism, Autoimmune disease, business.industry, Thyroiditis, Autoimmune, Case-control study, medicine.disease, Thyroxine, Endocrinology, Case-Control Studies, Pediatrics, Perinatology and Child Health, biology.protein, Thyroglobulin, business
Abstract

OBJECTIVE: To investigate the hypothalamus-pituitary-thyroid axis in patients with glycogen storage disease type 1(GSD1). STUDY DESIGN: Ten patients with GSD1a, 7 patients with GSD1b, and 34 sex- and age-matched healthy control subjects were enrolled in the study. RESULTS: The levels of serum-free thyroxine (FT4) were significantly lower in patients with GSD1a and GSD1b (P < .05), whereas thyrotropin was significantly higher compared with control subjects only in patients with GSD1b (P < .005). Thyroglobulin and thyroperoxidase auto-antibodies were significantly higher in patients with GSD1b than in patients with GSD1a and control subjects (P < .005). After thyrotropin-releasing hormone stimulation, an enhanced thyrotropin response was found in patients with GSD1a and patients with GSD1b (P < .005) compared with control subjects. The presence of a subclinical or overt hypothyroidism was found in 4 of 7 patients with GSD1b and in no patient with GSD1a (chi2 = 7.47, P < .005) or control subject (chi2 = 27.2, P < .0001). CONCLUSIONS: Patients with GSD1b have an increased prevalence of thyroid autoimmunity and hypothyroidism, although patients with GSD1a have little evidence of thyroid abnormalities. Concomitant damage at the level of the hypothalamus or pituitary gland might be hypothesized on the basis of the slightly elevated thyrotropin levels, even in patients with overt hypothyroidism.

Published
2007

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