Results 71 to 80 of about 120,158 (339)

Aminosäuren – Leitlinie Parenterale Ernährung, Kapitel 4 [PDF]

, 2009
Protein catabolism should be reduced and protein synthesis promoted with parenteral nutrion (PN). Amino acid (AA) solutions should always be infused with PN. Standard AA solutions are generally used, whereas specially adapted AA solutions may be required
Blumenstein, Irina Ursula, Böhles, Hansjosef, Goeters, Christiane, Schulz, Ralf-Joachim, Stein, Jürgen   +4 more
core  

LIVER TRANSPLANTATION FOR TYPE I GLYCOGEN STORAGE DISEASE [PDF]

, 1983
A 16½-year-old girl with type I glycogen storage disease was treated by orthotopic liver transplantation under cyclosporin/steroid immunosuppression. All metabolic stigmata of the disease were relieved and 1 year postoperatively she follows a normal diet
Alper, Alper Ca, Con, Folkman, Greene, Greene, Kashiwagi, Kashiwagi, Merrill, Nordlie, Raum, Starzl, Starzl, Starzl, Starzl, Starzl   +15 more
core   +1 more source

Triazine‐Trione Thermosets with High Processability for Scaffold Applications in Bone Tissue Engineering

Advanced Healthcare Materials, EarlyView.
Novel photo‐clickable triazine‐trione thermosets can be shaped and cured under mild conditions, including room and physiological temperatures. These materials are biocompatible and support osteogenic differentiation of bone marrow–derived mesenchymal stem cells on their surface.
Åshild Johansen, Shuntaro Yamada, Daniel J. Hutchinson, Mohammed A. Yassin, Samih Mohamed‐Ahmed, Cecilie Gjerde, Michael Malkoch, Kamal Mustafa   +7 more
wiley   +1 more source

Event-based real-life outcomes of patients with non-neuronopathic Gaucher disease receiving ert

Orphanet Journal of Rare Diseases
Background Gaucher Disease (GD) is a lysosomal storage disorder. Mutations in the GBA1 gene cause glucocerebrosidase enzyme deficiency that leads to the accumulation of its substrates.
Ayşe Akyüz, Aslı İnci, İlyas Okur, Leyla Tümer, Fatih Süheyl Ezgü   +4 more
doaj   +1 more source

The Integrity of Thinking [PDF]

, 2018
When we look at our political landscape today, I wonder where has our integrity gone? Teachers want to know how to explain (if that’s the right word) the language and behavior of the current American president to children in their ...
Tillmanns, Maria daVenza
core  

Kidney Organoids in Drug Development: Integrating Technological Advances and Standardization for Effective Implementation

Advanced Healthcare Materials, EarlyView.
This review examines how emerging enabling technologies enhance the physiological relevance, scalability, and reproducibility of kidney organoids, while advanced analytical approaches support model validation and deepen mechanistic insight into nephrotoxicity.
Helen Kearney, Silvia M. Mihăilă, Lorenzo Moroni, Carlos Mota   +3 more
wiley   +1 more source

Identification of the iduronate-2-sulfatase proteome in wild-type mouse brain

Heliyon, 2019
Iduronate-2-sulfatase (IDS) is a lysosomal enzyme involved in the metabolism of the glycosaminoglycans heparan (HS) and dermatan (DS) sulfate. Mutations on IDS gene produce mucopolysaccharidosis II (MPS II), characterized by the lysosomal accumulation of
Carolina Cardona, Eliana Benincore, Natalia Pimentel, Luis H. Reyes, Camilo Patarroyo, Alexander Rodríguez-López, M. Martin-Rufian, Luis Alejandro Barrera, Carlos J. Alméciga-Díaz   +8 more
doaj   +1 more source

Start, Stop, Rewind, Repeat—Cyclic Exposure of Adipose Stromal Cells‐derived Cartilage Organoids to Chondrogenic and Proliferative Cues to Achieve Scaled‐up and Customizable Bone Formation by Endochondral Ossification

Advanced Healthcare Materials, EarlyView.
This study exploits the plasticity of ASCs‐derived cartilage organoids which generate a perichondrial layer of MSCs when exposed to cyclic chondrogenic/proliferative cues. Using these organoids as building blocks, we develop (i) Phalange Shaped Tissue Engineered Cartilage (Pa‐TECs), recapitulating endochondral ossification suitable for the treatment of
Pablo Pfister, Emilien Lhospice, Andrés García‐García, Robert Paillaud, Sebastian Jung, Romain Schaller, Elisabeth A. Kappos, Claude Jaquiéry, Tarek Ismail, Dirk J. Schaefer, Michael de Wild, Ivan Martin, Alexandre Kaempfen, Arnaud Scherberich, Adrien Moya   +14 more
wiley   +1 more source

Immunization coverage and timeliness of vaccination in young patients with inborn errors of metabolism: a French multicentric study

Orphanet Journal of Rare Diseases
Background Inborn errors of metabolism (IEMs) are rare disorders that are heterogeneous in severity and clinical presentation. Patients with IEMs should receive the vaccination schedule recommended for the whole population, and specific vaccinations ...
Anne-Sophie Renous, Lena Damaj, Magali Gorce, Magalie Barth, Antoine Bedu, Elise Sacaze, Delphine Lamireau, Cécile Laroche-Raynaud, Laurent Pasquier, Zoha Maakaroun-Vermesse, Marine Tardieu, François Labarthe   +11 more
doaj   +1 more source

Tricarboxylic acid cycle enzyme activities in a mouse model of methylmalonic aciduria [PDF]

, 2019
Methylmalonic acidemia (MMA) is a propionate pathway disorder caused by dysfunction of the mitochondrial enzyme methylmalonyl-CoA mutase (MMUT). MMUT catalyzes the conversion of methylmalonyl-CoA to succinyl-CoA, an anaplerotic reaction which feeds into ...
Baumgartner, Matthias R, Chapman, Kimberly A, Cunningham, Gary, Forny, Patrick, Pumbo, Elena, Summar, Marshall L, Wongkittichote, Parith   +6 more
core   +1 more source