Deterioration of hearing function in mice with neural crest defect

Cochlear potentials were recorded in black-eyed white-mutant mice (strains W/Wv and S1/S1d) during the first months of postnatal life. In these animals melanocytes from the neural crest do not reach the stria vascularis. Hearing thresholds indicated by the compound action potential of the auditory nerve progressively increased. Hearing loss in comparison with the control CBA mice amounted 40 dB six weeks after birth, at eight months practically all mutants were deaf. Endocochlear potential was near zero already in the majority of 6 week-old animals. Abnormalities were observed also in intracellular potentials of the stria vascularis cells which were recorded in vitro and dye-marked. While in marginal cells of CBA mice positive potentials of about 10 mV were recorded, potentials of marginal cells in mutants were near zero. Basal cell potentials were negative in CBA mice (approximately -25 mV) and positive in young mutants (approximately +12 mV). However, in mutants older than 3 months the intracellular potentials of basal cells changed to negativity (approximately -10 mV). The results demonstrate gradual deterioration of hearing function observed in both W/Wv and S1/S1d mutants during first months of the postnatal life. In addition to the dysfunction of stria vascularis, where intermediate cells are missing, also the function of receptors progressively deteriorates.     

Biochemical profile and survival in nonagenarians.

OBJECTIVES: Old age is associated with an increase in frequency of disorders involving virtually all organ systems, resulting in a rise of mortality. The aim of the project was to study the relationship between biochemical markers and all-cause mortality in a defined age group. DESIGN AND METHODS: Thirty-eight nonagenarians, aged 92 +/- 2 (range 90-100) years entered the study. At the start of the study, a sample of peripheral blood and urine were obtained for analysis of 50 basic biochemical, hematologic and biologic parameters. The assessment was then repeated in 6 to 12 months intervals. The significance of difference between surviving subjects and those who died was examined by Mann-Whitney U test and the correlation between the variables was studied by Spearman rank correlation coefficient. RESULTS: During the observation period, 21 of the studied subjects died leaving 17 persons still alive at the end of the study. The mean time from the first measurement to the death was 12 +/- 10 (range 0-33) months. The mean follow-up time in surviving subjects was 31 +/- 12 (range 4-45) months. Serum vitamin E and calcium were significantly higher, and serum alanine aminotransferase (ALT) and urinary neopterin were significantly lower in survivors compared to the subjects who died. No other parameters were significantly different in survivors and in persons who died. Urinary neopterin exhibited a significant negative correlation with serum sodium concentration (RS = -0.50, p < 0.01), but the other parameters did not correlate significantly. CONCLUSION: In conclusion, among the parameters studied, differences between survivors and nonsurvivors were observed only for serum vitamin E, calcium, ALT and urinary neopterin. These findings may form a basis for prospective interventional trials in this group of patients.     

Preparation and quality control of [166Ho]-macroaggregates for radiosynoviorthesis

(166)Ho is a beta-emitter (E(_max) = 1.84 MeV, T1/2 = 26.7 hours) with a mean penetration in the soft tissue of 2.2 mm and gamma-photons 81keV and 1380keV. In this paper, we present our experiences with preparation of (166)Ho-macroaggregates ((166)Ho-MA) for radiosynoviorhesis. Ho-MA were prepared by reacting the aqueous solution of holmium nitrate pentahydrate with sodium borohydride solution in 0.2 M NaOH. After centrifugation, washing, and drying at 105 degrees C, final fineness of 1-15 microm was attained using a homogenizer working at 70,000 rpm. Neutron activation was carried out in screwed titanium ampules in a nuclear reactor at the neutron flux 10E12-10E14 neutrons.cm-24.s-1. The irradiated samples were allowed to cool 24 hours before further manipulation. (166)Ho-MA were clinically tested on 20 patients suffering from gonarthrosis (15), rheumatoid arthritis (5), psoriatic arthritis (1), and gout arthropathy (1). (166)Ho-MA are currently produced under GMP and production facility allows producing radioactivity for 6 patients within one production run.     

Large diaphragmatic defect: are skeletal deformities preventable?

Purpose  

The aim of study was to compare growth, nutritional status and incidence of chest wall deformities and scoliosis in survivors of large congenital diaphragmatic hernia (CDH) defect (Gore-Tex patch reconstruction) with survivors with smaller defects and primary reconstruction.