Early bisphosphonate treatment in infants with severe osteogenesis imperfecta.

Cyclical neridronate treatment, started just after diagnosis at birth, had positive effects on growth and fracture rat

Graves' ophthalmopathy evolution studied by MRI during childhood and adolescence.

The positive outcome of Graves' ophthalmopathy correlated with low TRAb (autoantibodies to thyroid-stimulating hormone receptor) titers at diagnosis and during follow-up and with prepubertal condition at diagnosis

Prevalence of polycystic ovary syndrome in young women who had idiopathic central precocious puberty.

To assess the prevalence of polycystic ovary syndrome (PCOS) in a cohort of young women with previous idiopathic central precocious puberty (ICPP) at least 3 years after menarche, and to look for any predictive factors of PCOS at the time ICPP was diagnosed. DESIGN: Longitudinal study. PATIENT(S): Forty-six young women (18.1 +/- 3.0 years) who had been treated with GnRH analogues during childhood, observed at gynecologic age of 6.23 +/- 3.3 years. INTERVENTION(S): Semistructured interview concerning cycles, physical exam, blood sampling, and transabdominal pelvic ultrasound. MAIN OUTCOME MEASURE(S): Oligomenorrhea, LH, FSH, E(2), T, DHEAS, free T, delta4-androstenedione, 17-OHP, P, polycystic ovary morphology (PCOM). RESULT(S): Fifteen percent of the young women had oligomenorrhea, 28% clinical hyperandrogenism, 48% biochemical hyperandrogenism, and 37% PCOM. A total of 32% of the patients had PCOS according to the Rotterdam definition and 30% had PCOS according to the Androgen Exess Society. The prevalent phenotype of PCOS was characterized by clinical and/or biochemical hyperandrogenism and PCOM. We did not find any predictive factors for PCOS at the time ICPP was diagnosed. CONCLUSION(S): Patients with ICCP are prone to developing PCOS. The prominent phenotype in this cohort was PCOM associated with clinical and/or biochemical hyperandrogenism. Further follow-ups of these young adult patients will clarify whether this phenotype persists and if it will have important long-term implications regarding increased risk of infertility or metabolic complications

Menstrual pattern and menstrual disorders among adolescents: an update of the Italian data.

Abstract Background The most striking event in the whole process of female puberty is the onset of menstruation. To our knowledge, no large population-based studies have been performed on the topic of menstrual health among Italian adolescents in recent years. The aims of this study were to produce up-to-date information on the menstrual pattern of Italian girls attending secondary school, and to estimate the prevalence of menstrual cycle abnormalities in this population. Methods This was a cross-sectional study on a population-based sample of Italian adolescents aged 13–21 years attending secondary school. Only girls who had already started menstruating were requested to participate. Information was collected by means of a questionnaire that included items on the girls’ demographic details, anthropometrics, smoking and drinking habits, use of contraceptive pills, and socioeconomic status. The questions on the girls’ menstrual pattern concerned their age at menarche, duration of the most recent menstruation intervals (35 days, variable), average days of bleeding (6 days), and any menstrual problems and their frequency. Results A total of 6,924 questionnaires were administered and 4,992 (71%) were returned. One hundred girls failed to report their date of birth, so 4,892 subjects were analyzed. The girls’ mean age was 17.1 years (SD ±1.4); their mean age at menarche was 12.4 (±1.3) years, median 12.4 years (95%CI 12.3–12.5). In our sample population, 3.0% (95%CI 2.5%-3.4%) of the girls had menstruation intervals of less than 21 days, while it was more than 35 days in 3.4% (95%CI 2.9%-3.9%). About 9% of the girls (95%CI 7.7%-9.4%) said the length of their menstruation interval was currently irregular. Short bleeding periods (6 days) in 19% (95%CI 17.9%-20.1%). Menstruation-related abdominal pain was reported by about 56% of our sample. About 6.2% of the girls (95%CI 5.4%-7.0%) were suffering from dysmenorrhea. Conclusions In conclusion, to the best of our knowledge, this is one of the largest studies on menstrual patterns and menstrual disorders among Italian adolescent girls. Adolescent girls referring persistent oligomenorrhoea, in first two years from menarche, had a higher risk for developing a persistent menstrual irregularity. They had longer bleeding periods (>6 days) and this has practical implications because it makes these adolescents potentially more susceptible to iron deficiency anemia. Clinicians need to identify menstrual abnormalities as early as possible in order to minimize their possible consequences and sequelae, and to promote proper health information. We recommend that adolescents should be encouraged to chart their menstrual frequency and regularity prospectively from the menarche onwards.</p

Body composition measurements: from the past to the future.

During growth, the human body increases in size and changes its proportions of various components due to hormone mediators. Growth is a complex, biological process regulated by multiple factors. These factors include genetics, nutritional intake, physical activity, age, gender and endocrine balance, all of which influence a child's body composition during the growth years. Quantifying the main components is integral to the study of growth, as the assessment of human physical characteristics is important both in the anthropological and medical fields. It is important to have the possibility to control the growth process and to predict adult status in order to reduce the risk factors of various diseases. CONCLUSION: This paper examines issues in the measurements of paediatric body composition, describing traditional and new tools in this field

Insulin-like growth factor binding protein-3 proteolysis and growth of athyreotic infants in the first weeks of life.

To gain a better understanding of the growth of athyreotic newborns in the first weeks of life, we evaluated auxological parameters and determined the serum levels of growth hormone (GH), insulin-like growth factor I (IGF-I) and free IGF-I, and IGF-binding protein-3 (IGFBP-3) in 15 hypothyroid infants (10 females) at a mean age of 25 d of life, immediately before the beginning of L-thyroxine therapy, and at 3 and 6 mo of life. Fourteen normal infants (9 females) of the same age were studied as controls. IGFBP-3 proteolytic activity was evaluated in 8 patients and in 8 controls at 25 d and 6 mo of life. There was no significant difference concerning weight and length between the patients and controls at birth, 25 d, 3 and 6 mo of life. The blood GH, IGF-I and IGFBP-3 levels were significantly lower in patients at diagnosis than in controls of the same age (p < 0.01 for all parameters), as well as IGFBP-3 studied by Western blotting. At diagnosis, the patients' free IGF-I level was within the control range, but the free IGF-I percentage of total IGF-I was higher than in the controls (p < 0.01). IGFBP-3 proteolytic activity was found to be greater in the patients (p < 0.01). At 6 mo of life, after therapy, none of these parameters was different from those of the controls. CONCLUSION: Increased IGFBP-3 proteolytic activity in our patients at diagnosis, favouring IGF-I bioavailability, could account for normal free IGF-I levels and in turn for their normal growth pattern during the first weeks of life and before the start of treatment

Growth parameters in newborns with hyperphenylalaninaemia

To understand the effects of hyperphenylalaninaemia on fetal growth, we studied growth parameters (weight, length and head circumference) of 23 phenylketonuric (PKU) and 60 hyperphenylalaninaemic (HPA) newborns from healthy mothers and of 1853 healthy neonates from north-east Italy. A comparison of the growth parameters for both PKU and HPA newborns, as well as for controls, showed a statistically significant higher percentage of PKU and HPA patients with reduced body length and cranial circumference (P < 0.05 for both parameters in affected neonates). The z-scores for all growth parameters regarding both PKU and HPA newborns and controls, and between PKU and HPA newborns according to the Mann\u2013Whitney non-parametric test, were statistically significantly lower in PKU newborns than in controls; in contrast, only body length was significantly lower in HPA newborns than in controls (P < 0.01). A comparison of growth parameter z-scores using the Kruskal\u2013Wallis test for PKU, HPA and control newborns showed that both body length (P < 0.01) and cranial circumference (P < 0.05) were significantly lower in both groups of affected neonates. Our results showed intrauterine growth retardation for both PKU and HPA newborns. Body length and cranial circumference appeared to be more important than birthweight in evaluating growth of PKU and HPA newborns

Polymalformative syndromes and puberty

Polymalformative syndromes may involve an alteration of the two pubertal components: sexual maturation and pubertal growth spurt. An absence of pubertal development, generally without changing of final height, or an advance of puberty, with reduction of adult height are possible in some syndrome