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At least 199 records · Page 11Linked to original sources

Zürich experience with preoperative, high dose methotrexate-containing chemotherapy in patients with extremity osteosarcomas (OSA).

From 1979 to 1990, 37 patients with extremity osteosarcomas, 22 of them males and 15 females, median age 19 years, received pre- and postoperative chemotherapy. The period of observation, calculated from after the primary operation, ranged from 1-114 months, median 25 months. After preoperative chemotherapy, 9 (24%) underwent a primary amputation, in 28 (76%) a limb salvage procedure was possible, 4/29 (14%) later developed local recurrences, metastases were diagnosed in 8/9 amputees and 5/28 after limb-sparing surgery. The five-year disease-free and overall survivals after amputation are 11% and 33%, respectively, compared to 68% and 75%, respectively (p = 0.001 and 0.018, respectively, for long rank). Results of histologic assessment after preoperative chemotherapy are of significant prognostic impact. The earlier prognostic groups of 0%-49% necrosis versus 50%-100% necrosis were statistically no longer suitable for distinguishing useful prognostic groups. In this second analysis, patients with 0%-79% necrosis versus 80%-100% necrosis had 5-year disease-free survivals of 81% versus 44% (p = 0.032) and 5-year overall survivals of 88% In summary, 24% of our patients with extremity osteosarcomas, most of them with large primaries close to joints, had to undergo primary amputation and had only a 33% 5-yr-survival, whereas limb salvage procedures with pre-and postoperative chemotherapy were associated with a 5-yr survival of 75% and thus had no adverse impact. Careful selection of patients for successful management of osteosarcomas is important; necrosis of 80% and more after preoperative chemotherapy is a prerequisite for a favourable outcome.

Adolescent↗

Tiredness and somnolence despite initial treatment of obstructive sleep apnea syndrome (what to do when an OSAS patient stays hypersomnolent despite treatment).

From a database of 4,129 patients with sleep-disordered breathing (SDB), we found 207 subjects (43 women) that still complained of daytime tiredness, fatigue, and/or sleepiness despite treatment. In 25 subjects the sleepiness developed 1 to 36 months following treatment and was related to noncompliance (8 subjects), significant weight increase and/or inappropriate treatment (10 subjects), or development of new medical problems (7 subjects). In the remaining 182 subjects, sleepiness was noted within 1 month after what was judged appropriate treatment for SDB. In this group, the reason for persistent complaint was divided into four categories: 1) inappropriate treatment as a result of not using the measurement of esophageal pressure (Pes) in the initial diagnosis (41 subjects), 2) nonfunctional treatment (3 subjects), 3) associated narcolepsy-like syndrome (2 subjects), and 4) emergence of obesity and/or periodic leg movements as significant factors (135 subjects). The 135 subjects in this last category could be subdivided into three subgroups: 1) younger subjects, severely overweight with lower mean nocturnal saturated arterial oxygen (SaO2) values; 2) older subjects, of normal weight, with high numbers of periodic leg movements (PLM); and 3) moderately overweight subjects, with a combination of PLM and lower mean SaO2 values than expected. Treatments were aimed at eliminating the identified problems; they included standard medications for PLM and nasal bilevel positive airway pressure (BiPAP) for low SaO2 measurements. These treatments were not effective in specific cases, and stimulant medications had to be prescribed.

Adult↗

The osa gene of pSa encodes a 21.1-kilodalton protein that suppresses Agrobacterium tumefaciens oncogenicity.

The incompatibility group W plasmid pSa suppresses Agrobacterium tumefaciens oncogenicity (J. Loper and C. Kado, J. Bacteriol. 139:591-596, 1979). The oncogenic suppressive activity was localized to a 3.1-kb region of pSa by Tn5 mutagenesis and deletion analysis. Within this fragment, a 1.1-kb subclone bearing oncogenic suppressive activity was subjected to further characterization. Nucleotide sequencing of the 1.1-kb fragment revealed a 570-bp open reading frame (ORF1) that has a coding capacity for a protein of 21.1 kDa. Sequencing of flanking regions revealed a second ORF (ORF2) located 3 bp upstream of ORF1, with a coding capacity for a protein of 22.8 kDa. Gene fusions of these ORFs to a T7 phi 10 expression system in Escherichia coli resulted in the synthesis of polypeptides of the predicted sizes. An E. coli promoter consensus sequence was not found in the expected positions in the region preceding ORF1. However, several sequences with similarity to the consensus -10 sequence of the A. tumefaciens vir gene promoters were found upstream of ORF1. Potential translational start signals are upstream of ORF1 and ORF2. These sequences showed no significant similarity at the nucleotide or amino acid levels with those in available data bases. However, the C-terminal portion of the ORF1 protein is rich in hydrophobic residues. Perhaps oncogenicity suppression is effected by an association of this protein with the Agrobacterium membrane such that T-DNA transfer is blocked.

Amino Acid Sequence↗

Gregor Mendel, OSA (1822-1884), founder of scientific genetics.

Gregor Mendel, an Augustinian monk and part-time school teacher, undertook a series of brilliant hybridisation experiments with garden peas between 1857 and 1864 in the monastery gardens and, using statistical methods for the first time in biology, established the laws of heredity, thereby establishing the discipline of genetics.

Austria↗

Two sisters with a syndrome of ocular, skeletal, and abdominal abnormalities (OSA syndrome).

Two adult sisters are described who had a unique association of facial, ocular, and skeletal defects, and abdominal muscle hypoplasia, indicating autosomal recessive inheritance. Many of these features overlap those previously found in other malformation syndromes. However, the constellation of defects observed in these patients appears to represent a previously unreported syndrome and autosomal recessive inheritance is likely.

Abdomen↗