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.     

Central Sensitization Theory of Migraine: Clinical Implications

The clinical science of migraine headache continues to evolve. Theories of the pathophysiology of migraine have progressed from the early vascular basis of migraine to more complex current theories that emphasize the centrality of neuronal dysfunction. The most recently articulated theory of migraine is the central sensitization hypothesis, which proposes that altered processing of sensory input in the brainstem, principally the trigeminal nucleus caudalis, could account for many of the temporal and symptomatic features of migraine, as well as its poor response to triptan therapy when such treatment is initiated hours after the onset of pain. Both preclinical and clinical data support the central sensitization theory. A critical clinical implication of this theory is that drugs that are capable of either aborting or arresting the process of central sensitization, most prominently dihydroergotamine, may have a unique role in the treatment of migraine. An additional, and highly practical, implication is based upon the finding that cutaneous allodynia—pain arising from innocuous stimulation of the skin, as in hair brushing or the application of cosmetics—is an easily identifiable marker of central sensitization. Thus, the presence or absence of cutaneous allodynia can be integrated into the routine clinical assessment of migraine and utilized as a determinant of treatment. Future basic and clinical research on central sensitization is likely to be of ongoing importance to the field.     

Intussusception in Taiwanese children: analysis of incidence, length of hospitalization and hospital costs in different age groups.

BACKGROUND AND PURPOSE: This study investigated the characteristics of intussusception in Taiwanese children of different age groups, including the incidence, length of hospitalization and hospital costs. METHODS: Children with a diagnosis of intussusception who were hospitalized from 1999 through 2001 were identified from a nationwide health insurance claims database. The incidence of intussusception was calculated by age, gender, and season. Length of hospitalization and hospital costs were also analyzed. RESULTS: A total of 6988 cases of intussusception were identified in Taiwan from 1999 to 2001. Among them, 4859 cases occurred in children below 15 years of age. The average incidence among children below age 15 years was 34.5 per 100,000, with a peak incidence of 118.8 per 100,000 observed among children younger than 24 months old. The highest incidence of intussusception in Taiwanese children occurred between 12 and 24 months of age. According to the data for patients below 15 years of age hospitalized for intussusception in year 2000, males were more likely to be affected than females (61.3% vs 38.7%). Intussusception-related hospitalizations were rare in infants in the first few months of life, increased in those 6 to 12 months old, and peaked among children 1 to 3 years old. Among the 952 patients with intussusception admitted to hospitals in 2000, 297 (31.2%) received surgery, incurring higher median medical costs (New Taiwan Dollars [NT dollars] 42,265 or US dollars 1234) and longer median hospital stay (6.2 days) than the 655 patients who did not require surgery (NT dollars 6290 or US dollars 185 for hospitalization of 2.4 days). CONCLUSIONS: The study found that the incidence of intussusception peaked in the second year of life in Taiwanese children. There was also a male predominance and lack of seasonal variation in incidence.     

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.