50 research outputs found

    Erythropoietin reduces the expression of myostatin in mdx dystrophic mice

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    Erythropoietin (EPO) has been well characterized as a renal glycoprotein hormone regulating red blood cell production by inhibiting apoptosis of erythrocyte progenitors in hematopoietic tissues. EPO exerts regulatory effects in cardiac and skeletal muscles. Duchenne muscular dystrophy is a lethal degenerative disorder of skeletal and cardiac muscle. in this study, we tested the possible therapeutic beneficial effect of recombinant EPO (rhEPO) in dystrophic muscles in mdx mice. Total strength was measured using a force transducer coupled to a computer. Gene expression for myostatin, transforming growth factor-beta 1 (TGF-beta 1), and tumor necrosis factor-alpha (TNF-alpha) was determined by quantitative real time polymerase chain reaction. Myostatin expression was significantly decreased in quadriceps from mdx mice treated with rhEPO (rhEPO = 0.60 +/- 0.11, control= 1.07 +/- 0.11). On the other hand, rhEPO had no significant effect on the expression of TGF-beta 1 (rhEPO = 0.95 +/- 0.14, control= 1.05 +/- 0.16) and TNF-alpha (rhEPO = 0.73 +/- 0.20, control= 1.01 +/- 0.09). These results may help to clarify some of the direct actions of EPO on skeletal muscle.Fac Med ABC, BR-09060650 Santo Andre, SP, BrazilUniv São Paulo, Inst Ciencias Biomed, BR-05508 São Paulo, BrazilUniversidade Federal de São Paulo, Inst Ciencias Quim Ambientais & Farmaceut, Diadema, SP, BrazilUniversidade Federal de São Paulo, Inst Ciencias Quim Ambientais & Farmaceut, Diadema, SP, BrazilWeb of Scienc

    Clinical utility gene card: for incontinentia pigmenti

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    IKBKG/NEMO (Inhibitor of Kappa light polypeptide gene enhancer in B-cells, Kinase Gamma/NF-κB Essential MOdulator, NM_001099856.4) located in Xq28 chromosomal region. The corresponding protein is named IKKgamma/ NEMO

    Biochemical and molecular characterization of von Willebrand disease type 2N in a pregnant patient who gave birth under analgesia with remifentanil.

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    von Willebrand 's disease (vWD) is the commonest inherited bleeding disorder. Although in literature there are some cases reported of epidural analgesia for labor pain in pregnancies with Von Willebrand's disease, the technique is not free from risk of neurolocal complications. Authors reported a case of spontaneous labor in a pregnant woman with type II vWD, delivered under local analgesia administered through a continuous intravenous infusion of remifentanil integrated by boli. A 34-year-old woman at the 39th week of her second pregnancy was admitted for an active labor of a single fetus in cephalic presentation. The patient had been diagnosed with type II vWD by a hematologist during her first pregnancy. The patient coagulation panel was as follows: a reduction of VIIIth factor concentration (21 percent); a normal value of vWD functional assay; an increase of vWf:Ag (antigen) and a reduction of XIth factor. During labor she was put on remifentanil in PCA (patient controlled analgesia), administered with slow boli followed by continuous infusions at increasing doses. The woman delivered a female fetus weighing 3,550 g, in vertex presentation, in left anterior occipital position, with an A.P.G.A.R. of 8 at the first minute and 9 at the fifth minute. The total duration of labor was 3 hours and 10 minutes. The patient was satisfied with analgesia in labor. The bleeding during and after delivery was regular. In the authors ' opinion, it is important to know that an alternative to epidural analgesia can be used in order to avoid the risk of neurological complications in labor pain for patients with type II Von Willebrand's disease
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