Search results for "Neon"
showing 10 items of 760 documents
Intravenous propofol allows fast intubation in neonates and young infants undergoing major surgery
2019
Aim of the study: In selected surgical neonates and infants, the rapidity of induction and intubation may represent an important factor for their safety. Propofol is an anesthetic characterized by a rapid onset and fast recovery time that may reduce time of anesthetic induction and improve post-anesthetic outcome. The aim of this study was to evaluate the safety and efficacy of anesthesia induction in full-term neonates and young infants after propofol bolus administration.Methods: A retrospective case-control study including infants below 6 months of age, undergoing general anesthesia between 2011 and 2013, was carried out. Patients that received intravenous propofol bolus to induce anesth…
The frequency of select adaptation disorders in preterm newborns
2021
Background: Preterm birth, defined as the birth of an infant before 37 complete weeks of gestation, is the single major cause of death and disability in children up to 5 years of age in the developed world. Aim of the study: The study aimed at analyzing select adaptation disorders in newborns delivered between 34–37 weeks of gestation and in particular, (1) determining the frequency of breathing, thermoregulatory, hypoglycemic and pathological hepatic disorders, and (2) examining underlying factors that determine their incidence. Material and methods: The study was carried out according to the documentoscopy on the basis of medical files collected between 2019–2020 at the Neonatal and Prete…
Translation of HTT mRNA with expanded CAG repeats is regulated by the MID1-PP2A protein complex.
2012
Expansion of CAG repeats is a common feature of various neurodegenerative disorders, including Huntington's disease. Here we show that expanded CAG repeats bind to a translation regulatory protein complex containing MID1, protein phosphatase 2A and 40S ribosomal S6 kinase. Binding of the MID1-protein phosphatase 2A protein complex increases with CAG repeat size and stimulates translation of the CAG repeat expansion containing messenger RNA in a MID1-, protein phosphatase 2A- and mammalian target of rapamycin-dependent manner. Our data indicate that pathological CAG repeat expansions upregulate protein translation leading to an overproduction of aberrant protein and suggest that the MID1-com…
Nascere diversi nell’antica Roma
2014
The analysis is mainly concerned with the condition of infants, in particular the problem of neonatal deformity in ancient Rome. The incidence of deformed births must have been considerable, if tradition records specific provisions on the exposure of deformed infants as far back as royal times. From the narratives of the historians, it can be deduced that deformity greatly impressed the ancient Romans, who were very superstitious in this regard, as can be seen from the very terminology used (monstrum, portentum, ostentum, prodigium etc.). The entity of the deformity thus became a discriminating factor for the very purpose of considering the child born as belonging to the human species, an e…
Segnalazione di un caso di morte improvvisa neonatale
2013
Il concetto di sindrome da morte improvvisa del lattante (SIDS) è definita come la morte improvvisa e inaspettata di un bambino di meno di un anno che posto in apparente buona salute nella culla viene poi rinvenuto cadavere senza che si sia manifestato alcun segno o sintomo di malattia e la cui causa rimane inspiegata dopo indagini approfondite. Esistono tuttavia altre ipotesi che rientrano nel capitolo della moprte improvvisa (SD), inattesa, che derivano da SD in corso di malattie paucisintomatiche, in genere di tipo infettivoovvero in corso di quadri patologici ben definiti o di malformazioni cardiache asintomatiche o in stato di pienop compenso o che, pur non essendo manifeste sul piano …
RNA-mediated therapies in myotonic dystrophy
2018
Myotonic dystrophy 1 (DM1) is a multisystemic neuromuscular disease caused by a dominantly inherited 'CTG' repeat expansion in the gene encoding DM Protein Kinase (DMPK). The repeats are transcribed into mRNA, which forms hairpins and binds with high affinity to the Muscleblind-like (MBNL) family of proteins, sequestering them from their normal function. The loss of function of MBNL proteins causes numerous downstream effects, primarily the appearance of nuclear foci, mis-splicing, and ultimately myotonia and other clinical symptoms. Antisense and other RNA-mediated technologies have been applied to target toxic-repeat mRNA transcripts to restore MBNL protein function in DM1 models, such as…
Myotonic dystrophy: candidate small molecule therapeutics
2017
Myotonic dystrophy type 1 (DM1) is a rare multisystemic neuromuscular disorder caused by expansion of CTG trinucleotide repeats in the noncoding region of the DMPK gene. Mutant DMPK transcripts are toxic and alter gene expression at several levels. Chiefly, the secondary structure formed by CUGs has a strong propensity to capture and retain proteins, like those of the muscleblind-like (MBNL) family. Sequestered MBNL proteins cannot then fulfill their normal functions. Many therapeutic approaches have been explored to reverse these pathological consequences. Here, we review the myriad of small molecules that have been proposed for DM1, including examples obtained from computational rational …
Derepressing muscleblind expression by miRNA sponges ameliorates myotonic dystrophy-like phenotypes in Drosophila
2016
AbstractMyotonic Dystrophy type 1 (DM1) originates from alleles of the DMPK gene with hundreds of extra CTG repeats in the 3′ untranslated region (3′ UTR). CUG repeat RNAs accumulate in foci that sequester Muscleblind-like (MBNL) proteins away from their functional target transcripts. Endogenous upregulation of MBNL proteins is, thus, a potential therapeutic approach to DM1. Here we identify two miRNAs, dme-miR-277 and dme-miR-304, that differentially regulate muscleblind RNA isoforms in miRNA sensor constructs. We also show that their sequestration by sponge constructs derepresses endogenous muscleblind not only in a wild type background but also in a DM1 Drosophila model expressing non-co…
Expanded CCUG repeat RNA expression in Drosophila heart and muscle trigger Myotonic Dystrophy type 1-like phenotypes and activate autophagocytosis ge…
2016
AbstractMyotonic dystrophies (DM1–2) are neuromuscular genetic disorders caused by the pathological expansion of untranslated microsatellites. DM1 and DM2, are caused by expanded CTG repeats in the 3′UTR of the DMPK gene and CCTG repeats in the first intron of the CNBP gene, respectively. Mutant RNAs containing expanded repeats are retained in the cell nucleus, where they sequester nuclear factors and cause alterations in RNA metabolism. However, for unknown reasons, DM1 is more severe than DM2. To study the differences and similarities in the pathogenesis of DM1 and DM2, we generated model flies by expressing pure expanded CUG ([250]×) or CCUG ([1100]×) repeats, respectively, and compared …
Mild Aerobic Exercise Training Hardly Affects the Diaphragm ofmdxMice
2017
In the mdx mice model of Duchenne Muscular Dystrophy (DMD), mild endurance exercise training positively affected limb skeletal muscles, whereas few and controversial data exist on the effects of training on the diaphragm. The diaphragm was examined in mdx (C57BL/10ScSn-Dmdmdx) and wild-type (WT, C57BL/10ScSc) mice under sedentary conditions (mdx-SD, WT-SD) and during mild exercise training (mdx-EX, WT-EX). At baseline, and after 30 and 45 days (training: 5 d/wk for 6 weeks), diaphragm muscle morphology and Cx39 protein were assessed. In addition, tissue levels of the chaperonins Hsp60 and Hsp70 and the p65 subunit of nuclear factor-kB (NF-kB) were measured in diaphragm, gastrocnemius, and q…