Growth Hormone Deficiency: Diagnosis and Therapy in Children

Short stature has been defined as a height below the 2 standard deviation for age, sex and ethnicity. Growth hormone deficiency (GHD) represents a condition characterized by reduced GH secretion, isolated or associated with other pituitary hormone deficiencies. In a child with short stature and growth deceleration, after the exclusion of other causes of growth failure, the diagnosis of GHD has to be confirmed by measurement of GH secretion after at least two stimulation tests. Patients with GHD should be treated with rhGH as soon as possible, to obtain normalization of growth and normal final height. The catch-up growth in response to rhGH therapy is maximal during the first years and could be affected by many variables, such as birth-weight, age and height at start of treatment and of puberty, and duration of treatment. Overall, rhGH is believed to be safe and significant side-effects in children are very rare, including benign intracranial hypertension, hyperglycaemia, arthralgia and myalgia. Patients with childhood onset GHD are usually retested in late adolescence to confirm the GHD persistence and to continue of GH therapy. In conclusion, the present chapter provides useful and updated information about the diagnosis, treatment and follow-up of children with GHD

Synergistic catalysis: cis-cyclopropanation of benzoxazoles

In the present paper we report our latest efforts in pushing the boundaries of Synergistic Catalysis. We propose the use of 3 different catalytic cycles working in concert for the formation of cis cyclopropane derivatives of benzoxazoles with excellent stereoselectivities. This is the proof of concept that synergistic catalysis could be successfully used in cascade reaction

Recent (1975-2003) changes in the Miage debris covere glacier tongue (Mont Blanc, Italy) from analysis of aerial photos and maps

The present study aims at identifying any changes in volume and thickness of the Miage Glacier tongue (Mont Blanc Massif, Italy) during the period 1975-2003. The Miage glacier developed the largest part of its debris cover over the last century, now found mostly between the glacier terminus (about 1850 m a.s.l.) and the upper ablation tongue (c. 2400 m a.s.l.) on a surface area of c. 4 km 2 The period examined (1975-2003) addresses climate conditions which were glacier-favourable (around the . 1980s), as well as glacier-unfavourable (since the early to mid-1990s), thus contributing to an understanding of the behaviour of debris covered glaciers under a changing climate. The analysis was based on the comparison between digital elevation models (DEMs), derived from historical records, specifically maps (1975; scale 1:10,000) and photogrammetric surveys (1991 and 2003, scale 1:15,000). The results show a general glacier volume loss (\u201316.640 x 10 6 m 3 ( from 1975 to 2003; nevertheless if we focus on the two time sub-windows (i.e.: 1975-1991 and 1991-2003) opposite trends are found: in the period 1975-1991 the volume variation of the Miage Glacier was about +19.25 710 6 m 3 , in the period 1991-2003, on the other hand, a volume decrease of about \u201336.2 710 6 m 3 occurred. Analysis shows that volume changes were strongly influenced by the supraglacial debris coverage which on Miage glacier tongue modulates the magnitude and rates of buried ice ablation

Role of Common-Gamma Chain Cytokines in NK Cell Development and Function: Perspectives for Immunotherapy

NK cells are components of the innate immunity system and play an important role as a first-line defense mechanism against viral infections and in tumor immune surveillance. Their development and their functional activities are controlled by several factors among which cytokines sharing the usage of the common cytokine-receptor gamma chain play a pivotal role. In particular, IL-2, IL-7, IL-15, and IL-21 are the members of this family predominantly involved in NK cell biology. In this paper, we will address their role in NK cell ontogeny, regulation of functional activities, development of specialized cell subsets, and acquisition of memory-like functions. Finally, the potential application of these cytokines as recombinant molecules to NK cell-based immunotherapy approaches will be discussed

Análise do comprometimento organizacional e da motivação em professores universitários.

Neste estudo, o comprometimento e a motivação são compreendidos a partir de três dimensões cada um: comprometimento afetivo, instrumental e normativo; e motivação intrínseca, internalizada e extrínseca. Objetivou-se identificar quais as possibilidades de aproximação teórica e de articulação empírica entre as dimensões da motivação e do comprometimento dos professores de uma instituição de ensino superior. Trata-se de um estudo de caso quantitativo, desenvolvido através da aplicação de um questionário, contendo 19 afirmativas para o comprometimento e 16 para a motivação, que deviam ser respondidas usando uma escala de concordância de 7 pontos. Os dados levantados junto a 193 docentes foram avaliados quanto sua confiabilidade através do coeficiente de Cronbach e, posteriormente, efetuadas diversas análises fatoriais considerando a soluções puras e rotacionadas. As matrizes de cargas fatoriais das análises referidas ao comprometimento e à motivação permitiram relacionar os fatores retidos às respectivas dimensões entanto que as matrizes de escores fatoriais daquelas soluções possibilitaram calcular as correlações entre os fatores do comprometimento com os da motivação. A partir dos resultados, conclui-se que ocorre articulação entre a motivação e o comprometimento organizacional; que existe correlação entre a motivação extrínseca e o comprometimento instrumental e entre a motivação intrínseca e o comprometimento afetivo o que deve ser considerado pela administração universitária

Same Phenotype in Children with Growth Hormone Deficiency and Resistance

By definition, about 2.5% of children show a short stature due to several causes. Two clinical conditions are characterized by serum IGF-I low levels, idiopathic GH deficiency (IGHD), and GH insensitivity (GHI), and the phenotypic appearance of these patients may be very similar. We studied two children with short stature and similar phenotypes. *e first case showed frontal bossing, dollface, acromicria, and truncal obesity, with a GH peak <0.05 ng/ml after stimuli and undetectable serum IGF-I levels. After PCR amplification of the whole GH1 gene, type IA idiopathic GHD was diagnosed. *e second case had cranium hypoplasia, a large head, protruding forehead, saddle nose, underdeveloped mandible, and a micropenis. Basal GH levels were high (28.4 ng/ml) while serum IGF-I levels were low and unchangeable during the IGF-I generation test. Laron syndrome was confirmed after the molecular analysis of the GH receptor (GHR) gene. IGHD type IA and Laron syndrome is characterized by opposite circulatinglevels of GH, while both have reduced levels of IGF-I, with an overlapping clinical phenotype, lacking the effects of IGF-I on cartilage. *ese classical cases show the importance of differential diagnosis in children with severe short stature.Fil: Ioimo, Irene. University of Pavia; ItaliaFil: Guarracino, Carmen. University of Pavia; ItaliaFil: Meazza, Cristina. University of Pavia; ItaliaFil: Domene, Horacio Mario. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Centro de Investigaciones Endocrinológicas "Dr. César Bergada". Gobierno de la Ciudad de Buenos Aires. Centro de Investigaciones Endocrinológicas "Dr. César Bergada". Fundación de Endocrinología Infantil. Centro de Investigaciones Endocrinológicas "Dr. César Bergada"; ArgentinaFil: Bozzola, Mauro. University of Pavia; Itali

Konsumsi dan Koefisien Cerna Protein Pakan Domba Lokal Yang Diberi Pakan Jerami Padi Amoniasi Mengandung Dfmams dan Konsentrat Disuplementasi Tepung Daun Waru

konsumsi dan koefisien cerna protein pakan dombalokal diberi pakan mengandung jerami padi amoniasi mengandung dfmams dankonsentrat disuplementasi tepung daun waru. penelitian bertujuan untuk mengkajipenambahan level tepung daun waru pada konsentrat terhadap konsumsi dan koefisiencerna protein pakan domba lokal yang diberi pakan jerami padi amoniasi. materi yang digunakan adalah 12 ekor domba lokal berumur ± 2 tahun dengan bobotrata rata 28,09 kg ± 2,56 kg. domba tersebut ditempatkan pada kandang individu denganukuran 1,5 m x 0,75 m. kandang tersebut dilengkapi dengan kawat jaring dan plastik yangdigantung pada bagian bawah untuk menampung dan memisahkan urin dan feses. pakanyang diberikan yaitu jerami padi amoniasi disuplementasi dfmams dan tepung daun waruyang dicampur pada konsentrat dengan berbagai level sebanyak 3% dari bobot badandalam bentuk segar (bk konsentrat 88,65%). pakan konsentrat diberikan setiap hari pukul07.00 dan 15.00 sedangkan jerami padi amoniasi dan air minum diberikan secara adlibitum. penelitian dirancang menurut penelitian ini dirancang menurut rancangan acaklengkap (ral) dengan perlakuan adalah taraf pemberian tepung daun waru dari bkkonsentrat masing masing 0%, 0,24% dan 0,48% untuk w0, w1 dan w2. masing masingperlakuan diulang 4 kali. peubah yang diukur adalah konsumsi dan koefisien cerna proteindengan metode koleksi total (khan dkk., 2003). analisis kadar air dan protein kasarterhadap cuplikan ganda pemberian, sisa pakan dan feses dilakukan menurut petunjukaoac (1994). analisis variansi menunjukkan bahwa suplementasi tepung daun waru berpengaruhnyata (p0,05) terhadap kecernaan protein. uji orthogonal polinomial menunjukkan peningkatan leveltepung daun waru menyebabkan penurunan konsumsi protein secara linear (p<0,05)dengan persamaan regresi y=80,01139-101,1227x (r =93%). pemberian tepung daun warutidak disarankan untuk memperbaiki konsumsi dan kecernaan protein domba lokal yangdiberi jerami padi amoniasi dan konsentrat.

Organophotocatalytic synthesis of phosphoramidates

We describe the use of visible light in conjunction with an organic dye for the synthesis of phosphoramidates. Cross dehydrogenative coupling reactions between phosphites and amines are reported using organic dyes and light as catalysts for the first time. It is not only a novel application of organic dyes but also fulfils the requirement of sustainability and green chemistry avoiding the use of chromatographic purification techniques. The product was simply isolated from the reaction mixture via an acid-base work-up procedure, rendering the pure product in good yields

Late diagnosis of celiac disease in an asymptomatic infant with growth failure

The clinical spectrum for celiac disease (CD) is broad and includes cases with either typical (intestinal) or atypical (extraintestinal) features, often making the diagnosis of CD very difficult. We describe the case of a girl presenting with stunted growth and malnourishment. She was evaluated at 14 months for decreased growth rate without any signs of gastrointestinal, renal or endocrine disorders. She was evaluated for CD, but resulted negative for anti-tTG antibodies. At the age of 4.1 years, she exhibited basal dental enamel hypoplasia, iron deficiency anaemia despite repeated iron supplementation, with persistent reduced height (-2.79 SDS), BMI (-0.76 SDS), growth velocity (-1.79 SDS) and delayed bone age (1.5 year). The CD screening was repeated and very high anti-tTG-IgA (128 IU/ml, normal values 40 IELs/100 epithelial cells) confirming the diagnosis of CD. A gluten-free diet was started and after only four months, her growth velocity increased from 4.83 cm/year (-1.79 SDS) to 6.53 cm/year (-0.15 SDS). In conclusion, we report the development of a positive serology for CD in an asymptomatic child with growth retardation, who previously was investigated for CD and resulted negative. Therefore, when faced with retarded growth in young patients, after excluding other malabsorption conditions and even when CD serological markers are negative, the paediatric endocrinologist should request HLA genotyping, before the intestinal biopsy, in order to check for the presence of risk alleles

patients with hypertensive nephropathy and chronic kidney disease might not benefit from strict blood pressure control

Background/Aims: In patients with chronic kidney disease (CKD) strict blood pressure (BP) control is reno-protective. However, renal benefits from BP control might depend also on the etiology of CKD. We investigated if maintenance of BP at target is equally effective in subjects with hypertensive nephropathy (HN+) and in those with other nephropathies (HN-). Methods: We evaluated 148 patients with CKD (stages 3-5) in two visits at least 12 months apart. BP was measured both as office BP and 24h ambulatory blood pressure (ABP). Glomerular filtration rate (eGFR) was estimated with CKD-EPI formula. The slope of eGFR variation (ΔeGFR) was calculated as: (eGFR1-eGFR0)/months of follow up. Results: Cohort characteristics were: HN-(n=82) and HN+ (n=66), age (71±9 vs 74±9 years; p=0.09); prevalence of diabetes (57 vs 43%; p=0.19); average follow up (19±7 vs 21±9 months; p=0.3). HN- and HN+ did not differ regarding both baseline eGFR (34±18 vs 35±14 ml/min; p=0.97) and ΔeGFR (0.00±0.53 vs -0.06±0.35 ml/min/month, p=0.52). The proportion of patients with BP at target at both visits was similar in HN- and HN+ (office BP: HN- 18% and HN+ 27%; p=0.21; ABP: HN- 42% and HN+ 43; p=0.96). In patients with office BP at target at both visits HN- showed a significant improvement of ΔeGFR respect to HN+ (HN-: 0.240 ± 0.395 and HN+: -0.140±0.313 ml/min/ month; p=0.026). In patients with office BP not at target HN- and HN+ did not show any difference in ΔeGFR (HN- 0.00±0.47; HN+ -0.030±0.420 ml/min/month; p=0.66). ABP was not associated with differences in ΔeGFR either if it was at target (HN- 0.104±0.383 and HN+ 0.00±0.476 ml/min/month; p=0.42) or not (HN- -0.057±0.503 and HN+ -0.092±0.325 ml/ min/month; p=0.87). Conclusion: In patients with CKD and HN+ maintenance of BP targets recommended by current guidelines is less reno-protective than it is in HN-