Patterns of cognitive change over time and relationship to age following successful treatment of Cushing's disease.

Chronically elevated levels of cortisol have been associated with changes in cognitive functioning and brain morphology. Using Cushing's disease as a model to assess the effects of high levels of cortisol on cognitive functioning, 27 patients with Cushing's disease were examined at baseline and three successive follow-up periods up to 18 months after successful surgical treatment. At all follow-up periods, patients were administered cognitive tests as well as measures of plasma and urinary free cortisol. Structural MRIs and a depression measure were taken at baseline and one-year follow-up. Results showed that there is a specific pattern of significant cognitive and morphological improvement following successful treatment. Verbal fluency and recall showed recovery, although brief attention did not. Age of participants was a significant factor as to when recovery of function occurred; younger patients regained and sustained their improvement in cognitive functioning more quickly than older participants. Improvement in verbal recall also was associated with a decrease in cortisol levels as well as an increase in hippocampal formation volume one year after treatment. Overall, these findings suggest that at least some of the deleterious effects of prolonged hypercortisolemia on cognitive functioning are potentially reversible, up to at least 18 months post treatment.     

Transforming growth factor alpha treatment alters intracellular calcium levels in hair cells and protects them from ototoxic damage in vitro

To determine if transforming growth factor alpha (TGFα) pretreatment protects hair cells from aminoglycoside induced injury by modifying their intracellular calcium concentration, we assayed hair cell calcium levels in organ of Corti explants both before and after aminoglycoside (i.e. neomycin, 10⁻³M) exposure either with or without growth factor pretreatment. After TGFα (500ng/ml) treatment, the intracellular calcium level of hair cells showed a five-fold increase as compared to the levels observed in the hair cells of control cultures. After ototoxin exposure, calcium levels in hair cells of control explants showed an increase relative to their baseline levels, while in the presence of growth factors pretreatment, hair cells showed a relative reduction in calcium levels. Pretreatment of organ of Corti explants afforded significant protection of hair cell stereocilia bundle morphology from ototoxic damage when compared to explants exposed to ototoxin alone. This study correlates a rise in hair cell calcium levels with the otoprotection of hair cells by TGFα in organ of Corti explants.     

Hirschsprung's disease: associated abnormalities and demography.

We here examine the demographic parameters of patients with Hirschsprung's disease. The study population includes all patients with histologically confirmed disease treated at the Children's Hospital Medical Center of Boston over the 25-year period extending from 1961 through the first quarter of 1986. There were 179 cases. Overall, children with Hirschsprung's disease were found less likely to be first born (P less than .01). This relationship was seen to persist irrespective of maternal age, maternal race, or type of disease. The implications of this finding are discussed. Overall, 22% of these children had one or more associated abnormalities involving the neurological, cardiovascular, urological, and gastrointestinal systems. Many of the disorders appeared to be related to neurocrestopathies. Frequent associations included Down's syndrome, defects in cardiac septation, tetralogy of Fallot, and Dandy-Walker syndrome. These conditions occurred more frequently than would have been predicted through chance alone. This study found no association between an increased maternal age and the occurrence of Hirschsprung's disease. This study also found that approximately 7% of the affected children had been born prematurely.     

Multiple myxomata of soft tissue associated with polyostotic fibrous dysplasia. A case report

Polyostotic fibrous dysplasia with associated myxomata of soft tissue has been reported rarely. In most cases, the patient appears first with fibrous dysplasia and many years later presents with soft-tissue tumors. This is a report of a 57-year-old woman--an unusual case of soft-tissue myxomas with fibrous dysplasia. This case suggests that a patient developing myxomatous tumors of the soft tissues might be evaluated for the possibility of associated fibrous dysplasia.     

Revascularization of a thrombosed aortopulmonary shunt with the use of the AngioJet thrombectomy system.

We report the use of the AngioJet F140 rheolytic catheter to recannalize an acutely thrombosed aortopulmonary shunt in a 21-year-old female with palliated, complex congenital heart disease. After extracting the thrombus that filled the entire length of the shunt, three stents were placed at sites where the lumen was compromised by distortion or thrombus. Unobstructed flow was restored to the left pulmonary artery that persisted at 1-year follow-up.     

WAIS-R verbal-performance IQ discrepancies in persons with lateralized lesions: utility of a seven subtest short form.

WAIS-R Verbal-Performance IQ difference scores for Ward's (1990) seven subtest short form and the complete WAIS-R were examined in patients with lateralized and diffuse lesions. For both versions, the expected Performance > Verbal pattern was observed in the right hemisphere lesion group, while no summary score differences were seen in the left hemisphere group. Verbal-Performance IQ discrepancies for the short form fell within +/- 5 points of the WAIS-R discrepancy scores in about 75%of the cases, regardless of lesion location. Statistically reliable IQ differences between the complete and abbreviated WAIS-R attained 66%, 91%, and 89% agreement for the left, right, and diffuse groups, respectively. The results support the clinical utility of the seven subtest short form.