Résumé
Purpose@#Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. @*Materials and Methods@#A total of nine pediatric patients—four with MPS I and five with MPS II—underwent treatment with ERT and hGH at Samsung Medical Center. @*Results@#The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from –4.09±0.83 to –3.68±0.43 after 1 year of hGH treatment, and to –3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. @*Conclusion@#This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.
Résumé
BACKGROUND: Total knee arthroplasty (TKR) is associated with a significant loss of blood. Fluid substitution with crystalloid or colloid solutions to correct perioperative hypovolemia is essential. Colloid solutions, and especially hydroxyethyl starches (HES), are used to treat hypovolemia, but they may affect blood coagulation. The purpose of this study was to test the efficacy and the safety of colloid solutions in patients undergoing TKR. METHODS: The patients undergoing TKR were divided into a group that underwent fluid management with Voluven(R) (n = 22) and a group that was managed with Hextend(R) (n = 24). The blood loss, the autotransfused blood volume, the hemoglobin level, the allogenic blood requirement, the urine output and the complications were assessed. RESULTS: There were no significant differences in the amount of blood loss, the autotransfused blood volume, the allogenic requirement, the urine output and the complications between the two groups. CONCLUSIONS: Voluven(R) and Hextend(R) are equally efficacious plasma volume substitutes when performing TKR with an autotransfusion of drained blood.
Sujets)
Humains , Arthroplastie , Coagulation sanguine , Transfusion sanguine autologue , Volume sanguin , Colloïdes , Hémoglobines , Hydroxyéthylamidons , Hypovolémie , Solution isotonique , Genou , Volume plasmatique , Hémorragie postopératoireRésumé
Intraoperative transesophageal echocardiography (TEE) has become an important monitoring device for patients undergoing cardiac or noncardiac surgery. Complications associated with TEE are unusual, but the potential for TEE probe compression of the posterior vascular structures has been reported in pediatric patients. We present here a case of occlusion of the right subclavian artery in an adult patient with a vascular ring after insertion of a TEE probe.
Sujets)
Adulte , Humains , Échocardiographie transoesophagienne , Artère subclavièreRésumé
To investigate the pattern of drug-resistance of human influenza virus (A/H1N1) isolated in Korea during 2001~2002, the sequence analysis of hemagglutinin (HA) and neuraminidase (NA) genes and cell-based assay against neuraminidase inhibitor (NI) were performed. Analyses on the nucleotide sequences of NA genes showed that Korean isolates had 98.2 to 98.5% homology with that of the vaccine strain in 2001~2002 season, A/New Caledonia/20/99-like strain. However, there were no significant amino acid substitutions related to the drug-resistance such as E119V, R152K, I222R/Q, H274Y, and R292K. In the sequences of HA gene, no differences were observed on the major antigenic sites as well as the motifs related to the drug resistance. 50% inhibitory concentration (IC50) value against oseltamivir, one of NA inhibitors widely used in the treatment for the influenza, was determined by WST-1 assay. The SI values of Korean isolates against oseltamivir were 7.2 to 383.3, showing that these isolates displayed relatively low SI value against the drug. This result provides the useful information for the surveillance of drug-resistant influenza virus and the control of influenza in Korea.