[Assessment of the secular trend in puberty in boys and girls.]

An Pediatr (Barc). 2010 Sep 1;

Authors: García Cuartero B, Gónzalez Vergaz A, Frías García E, Arana Cañete C, Díaz Martínez E, Tolmo MD

OBJECTIVE: Changes in the onset of puberty have been reported in the last few years. The aim of this study is to determine pubertal trends in boys and girls. METHOD: Longitudinal study was conducted on 310 caucasian children. We analysed birth weight (BW), weight (kg), height (cm) and body mass index (BMI) (%), bone age, duration of puberty growth and pubertal height spurt. RESULTS: For boys the mean age at stage 2 was 12.4 (1.5) years with a bone age of 11.9 (1.3) years, and stage 5: 15.6 (1.5) with a bone age of 14.5. Mean age (years) (SD) for girls stage 2 was 10.1 (1.4), with a bone age of 10.3 (1.1). Age at menarche was 12.0 (1.3), with a bone age of 13.2 (0.9). Duration of puberty growth for boys was 3 years (1.2), and for girls 2.5 years (1.1). Pubertal height spurt in boys was 19.5cm (7.6) and for girls was 15.7cm (5.0). Girls with puberty onset<9 years of age show a greater pubertal height gain (19.7cm (4.3)) than girls >9 years of age [14.4 (4.5) (P<0.0001)] and a longer period of pubertal growth 3.1 years (0.8) versus 2.3 (0.9) (P<0.0001). Boys with puberty onset <11 years of age had a greater pubertal height gain [27.3cm (7.9)] than boys > 11 years of age [17.4 (5.9) (P<0.0001)] and a longer period of puberty growth of 3.9 years (1.2) versus 2.7 (1.1) (P<0.001). CONCLUSIONS: Boys presented secondary sex characteristics at the same age as other studies, but the girls reached puberty and menarche at a younger age than previous studies in the Mediterranean area. Bone age correlates with chronological age for both sexes at the beginning of puberty but not at the end. Early onset of puberty was associated with a greater pubertal height gain and a longer period of pubertal growth. There was no correlation between BW or BMI with onset of puberty.

PMID: 20817627 [PubMed - as supplied by publisher]

[Therapeutic approach in a giant cystic prolactinoma.]

An Pediatr (Barc). 2010 Sep 2;

Authors: Arguinzoniz L, Muñoz-Calvo MT, Pozo J, Martos-Moreno GA, Argente J

PMID: 20817580 [PubMed - as supplied by publisher]

17-AAG increases autophagic removal of mutant mutant receptor in spinal and bulbar muscular atrophy.

Neurobiol Dis. 2010 Aug 31;

Authors: Rusmini P, Simonini F, Crippa V, Bolzoni E, Onesto E, Cagnin M, Sau D, Ferri N, Poletti A

Several types of motorneuron diseases are linked to neurotoxic mutant proteins. These acquire aberrant conformations (misfolding) that trigger deleterious downstream events responsible for neuronal dysfunction and degeneration. The pharmacological removal of misfolded proteins might thus be useful in these diseases. We utilized a peculiar motorneuronal disease model, spinobulbar muscular atrophy (SBMA), in which the neurotoxicity of the protein involved, the mutant androgen receptor (ARpolyQ), can be modulated by its ligand testosterone (T). 17-(allylamino)-17-demethoxygeldanamycin (17-AAG) has already been proven to exert beneficial action in SBMA. Here we demonstrated that 17-AAG exerts its pro-degradative activity on mutant ARpolyQ without impacting on proteasome functions. 17-AAG removes ARpolyQ misfolded species and aggregates by activating the autophagic system. We next analyzed the 17-AAG effects on two proteins (SOD1 and TDP-43) involved in related motorneuronal diseases, such as amyotrophic lateral sclerosis (ALS). In these models 17-AAG was unable to counteract protein aggregation.

PMID: 20816782 [PubMed - as supplied by publisher]

Serum insulin-like growth factor-1 measurement in the diagnosis and follow-up of patients with acromegaly: preliminary data.

Front Horm Res. 2010;38:145-51

Authors: Guitelman M, Radczuk G, Basavilbaso NG, Oneto A, Basso A

Measurement of serum insulin-like growth factor-1 (IGF-I) is the current method for diagnosing and monitoring acromegaly. However, the use of commercially available kits needs to be validated. In our study, we have investigated the use of two different IGF-I immunoassays in patients already diagnosed with acromegaly. We compared a two-site immunoradiometric assay with ethanol-acid extraction (IRMA-DSL) and a solid-phase chemiluminescent immunometric assay (ICMA-IMMULITE), correlating the clinical finding with the biochemical results. A total of 102 samples (77 women and 25 men aged 18-79 years) were analyzed with the two different IGF-I assays. Sixty-four of samples had been taken from patients with acromegaly in different stages. Pearson regression showed a high correlation coefficient; otherwise, Bland and Altman analyses showed a mean difference of 177.6 ng/ml, with upper and lower limits of -183.5 and 538.7 ng/ml in the 102 samples studied. Normal serum IGF-I was found in 64 and 41.5% of patients with treated acromegaly when measured by ICMA and IRMA, respectively. In our study, IGF-I-ICMA had a better clinical correlation in patients with treated acromegaly. The reevaluation of current IGF-I immunoassays is necessary to correctly interpret treatment response in acromegalic patients and thus achieve a better correlation between clinical and biochemical results.

PMID: 20616505 [PubMed - indexed for MEDLINE]