2,502 research outputs found
L'allantoina: legame tra metabolismo ossidativo e purinico in soggetti affetti da Sindrome di Down
No full textEarly diagnosis of mucopolysaccharidoses in developing countries: A low cost and easy execution approach.
No full textClin Chim Acta. 2017 Feb 28;468:150-151. doi: 10.1016/j.cca.2017.02.020. [Epub ahead of print]
Early diagnosis of mucopolysaccharidoses in developing countries: A low cost and easy execution approach.
Gabrielli O1, Zampini L1, Monachesi C1, Marchesiello RL1, Padella L1, Santoro L1, Volpi N2, Concolino D3, Fiumara A4, Rigon L5, Mazzoli M6, Carnielli VP6, Giovagnoni A7, Catassi C1, Galeazzi T1, Coppa GV1.
KEYWORDS:
DMB and electrophoresis; Glycosaminoglycans; Mucopolysaccharidoses; Newborn
Falsi positivi nella caratterizzazione dei glicosaminoglicani urinari in neonati a termine: crisi genitale del neonato?
No full textFalsi positivi nella caratterizzazione dei glicosaminoglicani urinari in neonati a termine: crisi genitale del neonato?
Chiara Monachesi1, Lucia Padella1, Rita Lucia Marchesiello1, Carlo Catassi1, Enrico Gasparrini2, Nicola Volpi3, Francesca Maccari3, Agata Fiumara4, Daniela Concolino5, Maria Teresa Moricca5, Lucia Zampini1
1Dip Sc Cl Spec Odont UNIVPM, 2UO Ped e Neonat Osp Macerata, 3 Dip Sc Vita, UNIMORE, 4Dip Ped Univ Catania, 5Div Ped Univ Magna Graecia
Introduzione
Nell’ambito di un progetto PRIN2012 era stata eseguita la valutazione qualitativa dei glicosaminoglicani (GAG) urinari in una popolazione di 900 neonati sani a termine, in 2° o 3° giornata di vita, utilizzando volumi ridotti di campione. Lo scopo del presente studio è la caratterizzazione delle bande elettroforetiche anomale evidenziate in 7 campioni di soggetti di sesso femminile.
Metodi
I 7 soggetti risultati positivi sono stati richiamati per la raccolta di un secondo campione di urine (circa 2 ml) a distanza di almeno un mese.
Sono stati inoltre raccolti 6 campioni di muco vaginale da neonate con diagnosi di crisi genitale. L’identificazione del pattern dei GAG escreti è stata eseguita mediante elettroforesi su acetato di cellulosa in bario acetato mentre l’analisi strutturale tramite elettroforesi su gel di agarosio, dopo digestione con condroitinasi.
Risultati
A differenza del 99% dei campioni di urine precedentemente analizzati, 7 hanno mostrato un pattern anomalo, analogo a quello evidenziato sui campioni di muco vaginale. La digestione selettiva con condroitinasi e eparinasi ha mostrato la presenza in elettroforesi di specie molecolari non appartenenti ai normali GAG urinari.
Inoltre, i risultati sul secondo campione di urina hanno mostrato normalizzazione del pattern.
Discussione
L’analisi seguita alla digestione con enzimi specifici ha permesso di escludere la presenza di GAG patologici nei campioni di urine. Il riscontro dello stesso tipo di bande anomale nei campioni di muco di neonate con diagnosi di crisi genitale accertata e la normalizzazione del pattern dopo pochi mesi sul secondo campione di urine, ci hanno portato ad ipotizzare che le bande anomale potrebbero essere riconducibili a glicoproteine o mucine, come risposta fisiologica agli elevati livelli di estrogeni e progesterone materni trasmessi attraverso la placenta durante la gravidanza
ANTIOXIDANT ACTIVITY OF DIFFERENT WHITE TEAS: COMPARISON OF HOT AND COLD TEA INFUSIONS
No full textThe study investigates the antioxidant characteristics of hot and cold infusions of various white teas in relation to their grades and steeping conditions. Antioxidant activity, chelating activity, total phenol (TPC), flavonoids (TFC), theaflavins and individual catechins content, were examined. The results confirm that extraction of tea leaves carried out with water at room temperature leads to the formation of infusions particularly rich in compounds with antioxidant activity. In fact, for all the white teas studied, cold infusions had a higher content of phenols (4.77-7.63 mmol/L Gallic Acid Equivalents GAE), flavonoids (1.47-2.53 mmol/L Catechin Equivalents CE) and catechins (441.5-1328.2 μg/mL) compared to the hot ones (1.43-4.02 mmol/L GAE, 0.70-1.13 mmol/L CE, 83.4-534.8 μg/mL, respectively). The same trend was also observed for antioxidant activities examined using the ABTS assay (cold: 17.09-34.23; hot: 5.26-17.07 mmol/L Trolox Equivalents) and by monitoring the effects of the infusions on LDL oxidation (lag time, cold: 172.4-271.2; hot: 88.4-145.9 min). A general trend in antioxidant activity and in polyphenolic compounds content can be delineated between Chinese teas, i.e. Bai Mu Dan ≥ Xue Ya ≥ White Lung Ching > Anji Needle Mao Feng > Yhin Zhen Bai Hao and between African teas, i.e. White Salima Peony > Thyolo Bsp > Bvumbwe Bsp. Concerning metal chelating activity, all the white teas displayed similar levels (0.3-0.6 mmol/L EDTA Equivalents) with no significant differences between the hot and cold infusions (except Bvumbwe Bsp and Thyolo Bsp). In conclusion, this paper contains key information on the antioxidant properties, TPC, TFC, and individual catechins content of several white teas commercially available and the outcomes suggest that preparing tea infused in room temperature water for a couple of hours, may constitute an alternative kind of beverage potentially richer in health benefits, compared to commonly used hot tea infusions
Early biochemical effects of velmanase alfa in a 7‐month‐old infant with alpha‐mannosidosis
No full textAlpha mannosidosis is an ultrarare pathology with variable phenotypic manifestations, characterized by the deficiency of lysosomal alpha mannosidase which causes accumulation of neutral oligosaccharides. Until recently, the hematopoietic stem cell transplantation was the only clinical feasible therapeutic option. Only in 2018, the European Medicines Agency's committee approved the recombinant enzyme velmanase alfa for long-term treatment of non-neurological manifestations in mild and moderate forms of alpha-mannosidosis. In this study, the very early biochemical effects of enzyme replacement therapy in in a 7-month-old patient with alpha-mannosidosis were described. Velmanase alpha was administered as supporting therapy awaiting for hematopoietic stem cell transplantation, the treatment chosen for the patient because of the early onset form. The results showed that the enzyme replacement therapy was able to reduce the content of three different mannosyl-oligosaccharides monitored by tandem mass spectrometry after 2 months of treatment. In particular, the mean relative changes from baseline values were -67% in urine and -53% in serum at the latest observation. The study also showed that the enzymatic activity detected in serum 1 week after the first infusion was four times higher than the normal values and constant in the following points of observation. These findings led us to assume that velmanase alfa might be biologically active in this young patient
On-line high-performance liquid chromatography-fluorescence detection-electrospray ionization-mass spectrometry profiling of human milk oligosaccharides dervatized with 2-aminoacridone
No full textA high resolution normal phase HLPC-fuorescence detection-ESI-MS separation and structural characterization of the main oligosaccharides along with lactose from human milk samples is described. Twenty-two commercialy available oligosaccharides were fluorotagged with 2-aminoacridone and separated on an amide column and identified on the basisi of their retention times and mass spectra. Derivatized species having mass lower than about 800-900 exhibited mainly [M-H](-1) aninons, oligomers with mass up to 1000-1100 were represented by both [M-H](-1) and [M-H](-2) anions, and oligomer greater than 1200-1300 were characterized by a charge state of -3. Furthermore, the retention times were directly related to the glycans molecular mass. Human milk samples from the four groups of donors (Se+/-, Le+/-) were analyzed for their composition and amount of free oligosaccharides after rapid and simple pre-purifucation and derivatization steps also in the presence of lactose in high content. This analytical approach enebled us to perform the determination of species not detected by traditional tecniques, such as sialic acid, as well as of species present in low content easily mistaken with other peaks. Finally, labelled human milk oligosaccharides were analyzed without any interference from excess fluorophore, or interference from proteins, peptides, salts and other impurities normally present in this complex biological flui
Education and Training in St.Lucia: A Partially Annotated Bibliography
No full textThis bibliography on “Education and Training in St. Lucia” has been specifically prepared for the UWI School of Continuing Studies’ St. Lucia Country Conference. An attempt has been made to be as comprehensive as possible, but because of the weak bibliographical coverage of the literature of the region, important items may have been omitted. This is especially true for policy documents emanating from official sources. It covers all aspects of education and training in St.Lucia including distance education, educational finance,health and family life education and educational reform
Antioxidant activity of white, green and black tea obtained from the same tea cultivar
No full textThe present study explored what effect manufacturing has on the antioxidant properties of teas coming from
a single cultivar but processed differently to give a white, two black (Orthodox and CTC) and two green
(low-caffeine and non-decaffeinated) teas. Total phenol (TPC), flavonoids (TFC), theaflavins, individual catechins
content, and chelating activity were also investigated. Using the ABTS, ORAC and LDL assays the following
‘antioxidant profile’ was obtained: green≥low-caffeine green>white≥black Orthodox>black CTC, with
statistically significant correlation between ORAC and LDL assays (r2=0.444, p=0.0067), whereas TPC and
TFC significantly correlate with the ABTS one (r2=0.871, p=0.000 and r2=0.438, p=0.007, respectively).
Metal chelating activity, which was lowest in the green teas, does not correlate with antioxidant activity
but appears to be influenced by theaflavins content. The results contribute to better understand how the
manufacturing process influences the antioxidant activity of tea when variables (geographical region, environmental
conditions, cultivar type, plucking techniques) are kept to a minimum. Secondly, we show that
novel African green, white and black Orthodox teas, made from tea varieties typically used in black CTC tea
production, may have potential health benefits comparable with commonly consumed teas
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