Considerable variations in growth hormone policy and prescription in paediatric end-stage renal disease across European countries-a report from the ESPN/ERA-EDTA registry.

BACKGROUND: Growth retardation in paediatric end-stage renal disease (ESRD) has a serious impact on adult life. It is potentially treatable with recombinant growth hormone (rGH). In this study, we aimed to quantify the variation in rGH policies and actual provided care in these patients across Europe. METHODS: Renal registry representatives of 38 European countries received a structured questionnaire on rGH policy. Cross-sectional data on height and actual use of rGH on children with ESRD aged <18 years were retrieved from the ESPN/ERA-EDTA Registry. RESULTS: In 21 (75%) of 28 responding countries, rGH is reimbursed for children with ESRD. The specific conditions for reimbursement (minimum age, maximum age and chronic kidney disease stage) vary considerably. Mean height standard deviation scores (SDS) at renal replacement therapy (RRT) [95% confidence interval (CI)] were significantly higher in countries where rGH was reimbursed -1.80 (-2.06; -1.53) compared with countries in which it was not reimbursed [-2.34 (-2.49;-2.18), P < 0.001]. Comparison of the mean height SDS at onset of RRT and final height SDS yielded similar results. Among the 13 countries for which both data on actual rGH use between 2007 and 2011 and data from the questionnaire were available, 30.1% of dialysis and 42.3% of transplanted patients had a short stature, while only 24.1 and 7.6% of those short children used rGH, respectively. CONCLUSION: Reimbursement of rGH associates with a less compromised final stature of ESRD children. In many countries with full rGH reimbursement, the actual rGH prescription in growth-retarded ESRD children is low and obviously more determined by the doctor's and patients' attitude towards rGH therapy than by financial hurdles.

Management of high-quality dehydrated grape in vinification to produce dry red wines.

Grape controlled dehydration of "Cesanese" and "Sangiovese" wine grapes, followed by an innovative vinification protocol, was studied. Fresh grapes of both varieties were processed into basic wines (IW = initial wine). 'Cesanese' must from pressed dehydrated grapes (solid and liquid) was directly added (15 and 30% v/v) into the IW activating a refermentation. 'Sangiovese' must (solid and liquid) from pressed dehydrated grapes was fermented and, when the wine reached 5% alcohol concentration, every day, the IW was added until a final concentration of 40 or 60% (v/v). The produced "blended wines" (BW) had significantly higher alcohol, glycerol, extract, and polyphenol concentration. Malolactic fermentation was completely ended in all BW with no malic acid and formation of lactic acid (0.5-1 g/L). All wines showed a significant higher concentration in 4-vinylguaiacol, acetovanillone, and 3-oxo-α-ionol, providing spicy and fruity notes at the sensory analyses, and being appreciated for their body balance, less acidity, and flavor intensity.

Hearing loss in Turner syndrome: results of a multicentric study.

UNLABELLED: The purpose of this article was to evaluate otological diseases in 173 patients (pts) with Turner syndrome (TS). STUDY DESIGN: One hundred and seventy-three pts, mean chronological age (CA) 12+/-6.2 yr. Patients were submitted to different therapies: GH, estrogen therapy (EE), no therapy (no tx). Seventy-nine pts (CA 11 yr) had no otological diseases. Conductive hearing loss (CHL) occurred in 38.7% (CA 11 yr) and otoscopy was: persistent secretory otitis media in 55.2%, chronic otitis media in 10.4%, pars flaccida retraction pocket in 19.4%, mostly bilateral. Cholesteatoma was present in 15%. Sensorineurinal hearing loss (SNHL) occurred in 15.6% (CA 16 yr), 11 of whom were affected by high tone loss, and 15 by loss in midfrequencies (dip between 0.5-3 kHz), bilateral in 93%. Degree of hearing loss (HL) was mild [20-40 decibel hearing level (dBHL)] in 15%, moderate (45-60 dBHL) in 31%, severe (65-80 dBHL) in 8%, profound (dBHL>85) in 2%. We found a significant association between CHL and karyotype 45, X (p<0.025), congenital cranio-facial abnormalities, prevalently with low-set ears (p<0.04), narrow and/or high arched palate (p<0.018), and micrognathia (p<0.004). Our study confirms that the high prevalence of middle ear infections and CHL in TS are probably due to growth disturbances of the structures from the first and second branchial arches. We did not find any association between EE, GH, and HL. We recommend a regular audiological follow-up, especially during childhood, to prevent important middle ear anatomic sequele and to identify HL at an early stage, as the impact on social functioning may be significant.

Management of postharvest grape withering to optimise the aroma of the final wine: A case study on Amarone.

Amarone wine is different from regular dry wine due to the postharvest withering of Corvina, Corvinone and Rondinella grapes. Grapes were withered in a commercial facility with variability in terms of temperature and relative humidity (R.H.). Sugar content reached 230-240gL(-1) and 280gL(-1) at 20% and 30% mass loss, respectively. Most of VOCs (volatile organic compounds) decreased during withering but few VOCs increased during withering and we considered as markers; in Corvinone they were methylhexanoate, dimethylsuccinate, nerol, nonanoic acid, and benzyl alcohol; in Corvina, benzyl alcohol, isoamyl alcohol, 1-hexanol, p-cymen-8-ol, 2,3 pinanediol, 3-oxo-ionol and 3-methyl-1-pentanol, coumaran and damascenone; in Rondinella, hexanol, nonanoic acid, methyl vanillate, damascenone, 3-oxo-ionol, eugenol, p-cymen-8-ol, 2,3 pinanediol, coumaran and raspberry keton. Olfactive descriptors of the wines and the potential aroma of the combination of Corvina wine with the wines of the other two varieties at different percentages of mass loss are reported.

Vis-NIR measurement of soluble solids in cherry and apricot by PLS regression and wavelength selection.

Experimental results are presented on the use of partial least squares (PLS) regression and wavelength selection for the definition of models for visible-near-infrared (Vis-NIR) evaluation of soluble solids content in fruits. First, the relatively easy to deal with-but still not studied in the literature-case of cherry fruit is presented in detail. By using a very simple selection scheme, involving the subsampling of the spectral interval from 600 to 1100 nm with a fixed step, accurate models were found, consistently showing very favorable combinations of SEC and SEP values, in the 0. 50 degrees Brix range for a total variation of about 15 degrees Brix. Apricot fruit represented a more difficult species, and wavelengths to be included in the calibration had to be individually selected for the best results. Nevertheless, parsimonious models could be found, including a total of 38 spectral lines and leading to SEP values at the 0.75 degrees Brix level.

Effects of exogenous propylene on softening, glycosidase, and pectinmethylesterase activity during postharvest ripening of apricots.

Apricots (Prunus armeniaca L. cv. Boccuccia spinosa) picked at the commercial ripening stage [soluble solids content (SSC) 12.6%] were left to reach full ripening in continuously humidified air at 20 degrees C. Changes in the rate of ethylene production, firmness, soluble solids concentration, and titratable acidity were measured. The alpha-D- and beta-D-glucosidases, alpha-L-arabinofuranosidase, alpha-D- and beta-D-galactosidases, beta-D-xylosidase, and alpha-D-mannosidase activities were assayed. To evaluate the influence of ethylene on glycosidase activity, propylene (500 microL x L(-1)) was applied to apricots for 24 and 48 h. In apricots ripened in air, ethylene production increased on the first day and exhibited a typical climacteric pattern. Good edible quality was reached in 5 days when SSC was at least 14% and acidity was between 1.1 and 1.2% (% malic acid). During postharvest ripening, alpha-L-arabinofuranosidase activity increased from 1.9 to 11.6 nkat until day 7. alpha-D-Galactosidase, alpha-D-mannosidase, and beta-D-galactosidase activity increased continuously but at a lower rate. beta-D-Xylosidase activity also increased, but the level of activity was lower than the other glycosidases assayed. Pectinmethylesterase (PME) decreased during the postharvest ripening, and propylene enhanced this pattern, by stimulating ethylene production. Even the activities of alpha-L-arabinofuranosidase, beta-D-xylosidase, alpha-D-mannosidase, and beta-D-galactosidase were greatly stimulated by the propylene treatment, which consequently induced rapid softening of the fruits.

Avascular necrosis of the trapezoid bone.

A case of idiopathic avascular necrosis of the right trapezoid is presented. The aetiology was not clear. Treatment consisted of bone curettage, autologous bone graft and revascularization with a dorsal metacarpal artery.