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Periodic remission in Cushing's disease with paradoxical dexamethasone response: an expression of periodic hormonogenesis.

A patient with Cushing's disease due to a chromophobe adenoma was studied for 243 days before pituitary surgery and evidence for periodicity in cortisol steroid production was found with cycles occurring every 85.8 days (peak-to-peak length), associated with laboratory remissions and paradoxical response to dexamethasone. The autonomy of ACTH secretion was suggested by the nonresponsiveness to repeated lysine-vasopressin stimulation tests and lack of increase in urinary 170HCS following metyrapone. A distinct response of the hyperplastic glands (as demonstrated by percutaneous adrenal venography) was obtained on several B1-24 corticotropin stimulation. The patient's hypercortisolism disappeared following removal of the chromophobe adenoma through transphenoidal hypophysectomy.

17-Hydroxycorticosteroids↗

Molecular characteristics of Haemophilus influenzae causing invasive disease during the period of vaccination in Switzerland: analysis of strains isolated between 1986 and 1993.

The broad use of conjugated vaccines against Haemophilus influenzae type b may select for strains to which the polysaccharide vaccine does not provide immunity. We analyzed 392 consecutive H. influenzae isolates from Swiss children 0 to 16 years of age with invasive disease during the years 1986 to 1993. Bacterial strains were characterized by serotyping, capsular genotyping, outer membrane protein (OMP) subtyping, and ribotyping. Of 392 strains, 372 were serotype b, 1 was serotype a, 3 were serotype f, and 16 were nontypeable H. influenzae. After the introduction of Haemophilus conjugate vaccines in 1990, there was a relative increase of nontypeable strains from 3 to 6.6% (P = 0.27). Of the type b strains, 281 (75.5%) had the same OMP subtype and ribotype pattern. This clone predominated in the pre- and postvaccine periods. After the year 1990, the proportions of OMP subtype 1c and OMP subtype 3 tended to increase. Isolates from previously vaccinated (n = 10) and nonvaccinated patients did not differ in their subtype distributions. We conclude that the administration of conjugated vaccines decreased invasive disease caused by the most prevalent H. influenzae type b clone. However, further surveillance of circulating H. influenzae strains during the period of vaccination is indicated.

Adolescent↗

Computer simulation analysis of Sartwell's incubation period model for diseases with uncertain etiology. The effect of competing risk.

Computer simulation was applied to Sartwell's model to examine the impact of competing risks of death on the underlying assumptions and the power to reject both uniform and normal incubation period distributions. Exponential and nonparametric survival functions were imposed onto lognormal, uniform, and normal distributions to create random samples reflecting competing risk. These random samples were evaluated with the Shapiro-Wilk's test to determine the proportion for which the lognormal distribution was rejected. The simulations indicated that competing causes of death do not significantly alter the lognormal distribution of incubation periods. In only approximately 5% of the samples drawn from a lognormal distribution was a lognormal hypothesis rejected with a goodness-of-fit test when sample size varied from 20 to 500. There was generally good power (> 80%) to reject a lognormal distribution if the random samples were generated from a uniform distribution of incubation times, but not when they were generated from a normal distribution, particularly with increasing ages at disease onset. Varying the standard deviation did not significantly change the simulation results if the random samples came from a lognormal or uniform distribution. These conclusions were further supported by application of Sartwell's model to published data on the ages of onset for several chronic diseases.

Adult↗

An autopsy case of Alzheimer disease with myoclonus and periodic spikes on EEG.

This is a case of Alzheimer disease with myoclonus and periodic spikes on EEG. A 56-year-old man developed progressive dementia and, 3 years later, generalized convulsions. Eight years later, he showed myoclonus and periodic spikes on EEG. Cranial CT showed cortical atrophy and ventricular dilatation. He became apallic and died of pneumonia at the age of 65.9 years after the onset of the disease. The brain weighed 1,050 g. Neuropathologically, diffuse neuronal loss, abundant neurofibrillary tangles and senile plaques, particularly diffuse plaques, were found extensively in the cerebral cortex. The white matter was preserved. In the Ammon's horn, abundant neurofibrillary tangles and senile plaques were observed. Grumose degeneration of the cerebellar dentate nucleus, Kuru plaques or prions were not found. Numerous diffuse plaques of the cerebral cortex have rarely been reported in autopsy cases of Alzheimer disease with myoclonus and periodic spikes on EEG.

Alzheimer Disease↗