Montreal Children's Hospital

About: Montreal Children's Hospital is a healthcare organization based out in Montreal, Quebec, Canada. It is known for research contribution in the topics: Population & Poison control. The organization has 3842 authors who have published 4816 publications receiving 200198 citations.

Management of hemolytic uremic syndrome.

Abstract: 2011 has been a special year for hemolytic uremic syndrome (HUS): on the one hand, the dramatic epidemic of Shiga toxin producing E. coli -associated HUS in Germany brought the disease to the attention of the general population, on the other hand it has been the year when eculizumab, the first complement blocker available for clinical practice, was demonstrated as the potential new standard of care for atypical HUS. Here we review the therapeutic options presently available for the various forms of hemolytic uremic syndrome and show how recent knowledge has changed the therapeutic approach and prognosis of atypical HUS.

Effects of intra-oral sucrose on crying, mouthing and hand-mouth contact in newborn and six-week-old infants.

Abstract: SUMMARY To determine whether a single intra-oral administration of sucrose would calm infants and elicit mouthing and hand-mouth contact, crying newborn and six-week-old infants were given sucrose solution before one feed and sterile water before another in a cross-over trial. Six-week-old infants were also given sucrose and water after feeding. For the newborn infants, the calming effect was rapid, substantial and lasted for at least four minutes. Mouthing and hand-mouth contact increased, but for shorter durations. For the six-week-old infants, sucrose calmed for one minute only before feeding, but had no effects on mouthing or hand-mouth contact. The results imply that intra-oral sucrose has acute age-related effects on crying and suckling-feeding behaviour mediated by a pre-absorptive mechanism. Sucrose may tap a functional system for reducing distress related to feeding and/or regulation of infant state. RESUME Effet du saccharose inlrabuccal sur les pleurs, grimaces el le contact matn/bouche chez le nouveau-ne el le nourrisson de six semaines Pour determiner si une administration unique de saccharose intrabuccal calmerait des nourrissons et provoquerait des mouvements de bouche, des contacts main/bouche, des nouveaux-nes et des nourrissons de six semaines pleurant, recurent une solution donnee de saccharose avant un repas et de ľeau sterile avant un autre repas dans un essai croise. Les nourrissons de six mois recurent egalement du saccharose et de ľeau apres le repas. Chez les nouveaux-nes ľeffet calmant fut rapide, substantiel, durant au moins quatre minutes. Les mouvements de bouche et les contacts main/bouche s'accruent mais pour des durees plus breves. Chez les nourrissons de six semaines, le saccharose ne calma une minute qu'avant le repas mais n'eut aucun effet sur les mouvements de bouche ou le contact main/bouche. Ces resultats impliquent que le saccharose intrabuccal a des effets sur les pleurs et le comportement de succion/deglutition provoque par un mecanisme de preabsorption qui est hautement relieaľâge. Le saccharose peut declancher un systeme fonctionnel de reduction de la detresse lieaľalimentation et/ou a la regulation de ľetat du nourrisson. ZUSAMMENFASSLNG Wirkung von intra-oruler /.uckergabe auf Schreien, Mouthing und Hand-Mund-Kontakt bet Neugeborenen und sechs-Wochen-alten Saugtingen Um fcstzustellcn, ob cine einmalige intra-orale Gabe von Zucker Sauglinge beruhigen und Mouthing und Hand-Mund-Kontakt auslosen konnte, wurde schreienden Neugeborenen und sechs-Wochen-alten Sauglingen vor einer Mahlzeit eine Zuckerlosung und vor einer anderen steriles Wasser gegeben. Die sechs-Wochen-alten Sauglinge bekamen aueh naeh der Mahlzeit Zuckerlosung bzw Wasser. Bei den Neugeborenen trat der Beruhigungseffekt schnell und eindeutig ein und dauerte mindestens vier Minuten. Mouthing und Hand-Mund-Kontakt nahmen zu, jedoch nur fur eine kurzere Dauer. Die sechs-Wochen-alten Sauglinge wurdcn durch die Zuckerlosung vor der Mahlzeit nur fur eine Minute beruhigt und zeigten kein Mouthing und Hand-Mund-Kontakt. Die Ergebnisse zeigen, das die intra-orale Gabe von Zucker altersabhangigc Einflusse auf das Schrei- und Saugverhalten hat, die durch einen pra-absorptiven Mechanismus ausgelost werden. Der Zucker wirkt moglicherweise auf ein funktionelles System, das die Ungeduld vor dem Futtern vermindert und/oder das Befinden des Kindes reguliert. RESUMEN Efectos de la sucrosa intraoral sobre el llanto, el ‘mouthing’ y el contacto mano-boca en recien nacidos y lactantes de sets meses de edad Para determinar si una tinica administracion oral de sucrosa podria calmar los lactantes y determinar el ‘mouthing’ y el contacto mano-boca, a recien nacidos y lactantes de seis semanas de edad que lloraban se les dio una solucion de glucosa antes de un alimento y agua destilada antes de otro en un estudio doble cruzado. Los lactantes de seis semanas recibieron tambien sucrosa y agua despues del alimento. En los recien nacidos, el efecto calmante fue rapido y claro y duro por lo menos cuatro minutos. El ‘mouthing’ y el contacto mano-boca aumento, pero durante menos tiempo. A los lactantes de seis meses de edad la sucrosa los calmo durante un minuto, solo antes del alimento, pero no tuva ningun efecto sobre el ‘mouthing’ y el contacto mano-boca. Los resultados implican que la sucrosa intraoral tiene efecto, en relation con la edad, sobre el llanto y el comportamiento de succion-alimentacion, por medio de un mecanismo preabsortivo. La sucrosa puede facilitar un sistema funcional para reducir el distres relacionado con la alimentation y/o la regulacion de la situation del nino.

Isolation of a cDNA encoding human holocarboxylase synthetase by functional complementation of a biotin auxotroph of Escherichia coli

Abstract: Holocarboxylase synthetase (HCS) catalyzes the biotinylation of the four biotin-dependent carboxylases in human cells. Patients with HCS deficiency lack activity of all four carboxylases, indicating that a single HCS is targeted to the mitochondria and cytoplasm. We isolated 21 human HCS cDNA clones, in four size classes of 2.0-4.0 kb, by complementation of an Escherichia coli birA mutant defective in biotin ligase. Expression of the cDNA clones promoted biotinylation of the bacterial biotinyl carboxyl carrier protein as well as a carboxyl-terminal fragment of the alpha subunit of human propionyl-CoA carboxylase expressed from a plasmid. The open reading frame encodes a predicted protein of 726 aa and M(r) 80,759. Northern blot analysis revealed the presence of a 5.8-kb major species and 4.0-, 4.5-, and 8.5-kb minor species of poly(A)+ RNA in human tissues. Human HCS shows specific regions of homology with the BirA protein of E. coli and the presumptive biotin ligase of Paracoccus denitrificans. Several forms of HCS mRNA are generated by alternative splicing, and as a result, two mRNA molecules bear different putative translation initiation sites. A sequence upstream of the first translation initiation site encodes a peptide structurally similar to mitochondrial presequences, but it lacks an in-frame ATG codon to direct its translation. We anticipate that alternative splicing most likely mediates the mitochondrial versus cytoplasmic expression, although the elements required for directing the enzyme to the mitochondria remain to be confirmed.

The intermediate filament cytoskeleton of myofibroblasts: an immunofluorescence and ultrastructural study.

Abstract: The intermediate filament cytoskeleton of stromal myofibroblasts from a series of twenty-eight infiltrating ductal breast carcinomas was examined by transmission electron microscopy (TEM) and indirect immunofluorescence (IF), the latter using antibodies to desmin, vimentin and prekeratin. Three cases of fibromatoses, selected as an additional source of myofibroblasts, were processed in the same manner. Stromal myofibroblasts from invasive ductal breast carcinomas rich in actin and readily identified by IF, were most numerous in the "young" edematous mesenchyme, areas corresponding to early stromal invasion or the peripheral invasive cellular front. Within the central sclerotic zone wherein clusters of neoplastic epithelial cells were surrounded by abundant collagen, most stromal cells corresponded by TEM to fibroblasts. In like fashion, myofibroblasts were most numerous in cellular, poorly collagenized portions of fibromatoses. By IF the only detectable intermediate filament protein of myofibroblasts in these two settings was vimentin. Since the appearance of stromal myofibroblasts appears to be associated with stromal invasion by malignant epithelium, their development by modulation of pre-existent periductal fibroblasts is postulated. With the exception of vascular smooth muscle cells and endothelial cells, the only periductal mesenchymal cells shown to contain vimentin were fibroblasts. The lack of desmin in myofibroblasts constitutes evidence against an origin from vascular smooth muscle cells. Because the molecular markers (intermediate filament proteins) of stromal cell differentiation presented quantitative but not qualitative modifications, the transformation of fibroblasts into myofibroblasts is quite likely, suggesting that myofibroblasts may be more closely related to fibroblasts than to smooth muscle cells.