Goldenhar’s Syndrome associated with congenital cyanotic heart disease

A case of Goldenhar’s Syndrome in a 10 year old girl is reported. The unusual features are the absence of epibulbar dermoid which is one of the major hallmarks of the Syndrome and the presence of an associated Cyanotic Heart disease.     

Role of pylorus in mediating cholecystokinin-stimulated satiety in the Zucker rat

Obese Zucker rats are less responsive than their lean littermates to the effects of cholecystokinin-octapeptide on satiety and pancreatic growth and exocrine function. We hypothesized that the hyperphagia observed in obese Zucker rats may be caused by a decreased pyloric contractile response to cholecystokinin, resulting in an increased rate of gastric emptying, decreased postprandial gastric distention, and thus decreased satiety. Pyloric muscle strips from six obese Zucker rats and six lean littermates were mounted in separate tissue baths and isometric contraction was measured in response to acetylcholine and cholecystokinin-octapeptide. The dose-response curves for acetylcholine-and cholecystokinin-octapeptide-stimulated pyloric muscle contraction were similar for both the obese and the lean rats. (For cholecystokinin, D₅₀ obese=4.0±0.6 nM, D₅₀ lean=3.4±0.2 nM;P=0.16). We conclude that the decreased satiety response to cholecystokinin-octapeptide observed in obese Zucker rats is not secondary to a decreased pyloric responsiveness to cholecystokinin.This work was supported by NIH grant AM28303-03.     

Possible involvement of prostaglandin endoperoxides in cartilage-synovial interactions

The depletion of proteoglycans in cartilage matrix is the beginning of cartilage breakdown. Prostaglandins and related compounds might play an important role in inhibition of cartilage metabolism under arthritic conditions. Prostaglandin endoperoxides, intermediate metabolites of arachidonic acid, are more potent chemical mediators than prostaglandins, but their action can only be demonstrated in cartilage co-incubated with synovial tissue, because they are short-lived and active only within a small restricted space. Human rheumatoid synovialis highly inhibited sulfation of cartilage matrix co-incubated. The inhibition of cartilage metabolism was released by indomethacin added to the co-incubating system, showing its responsiveness to indomethacin. The magnitude of inhibition was time-dependent and substantially greater than that by prostaglandin in cell-free rheumatoid synovial culture media. The results suggest a possible involvement of prostaglandin endoperoxides in potent inhibition of cartilage metabolism under arthritic conditions.