The autosomal dominant macrothrombocytopenia with leukocyte inclusions, May-Hegglin anomaly (MHA), Sebastian syndrome (SBS), and Fechtner syndrome (FTNS), are rare platelet disorders characterized by a triad of giant platelets, thrombocytopenia, and characteristic Döhle body-like leukocyte inclusions. The locus for these disorders was previously mapped on chromosome 22q12.3-q13.2 and the disease gene was recently identified as MYH9, the gene encoding the nonmuscle myosin heavy chain-A. To elucidate the spectrum of MYH9 mutations responsible for the disorders and to investigate genotypephenotype correlation, we examined MYH9 mutations in an additional 11 families and 3 sporadic patients with the disorders from Japan, Korea, and China. All 14 patients had heterozygous MYH9 mutations, including three known mutations and six novel mutations (three missense and three deletion mutations). Two cases had Alport manifestations including deafness, nephritis, and cataracts and had R1165C and E1841K mutations, respectively. However, taken together with three previous reports, including ours, the data do not show clear phenotype-genotype relationships. Thus, MHA, SBS, and FTNS appear to represent a class of allelic disorders with variable phenotypic diversity.

SynopsisDifferential thermal analysis was applied for the investigation of melting and crystallization behavior of high polymers. The heats of fusion of crystalline regions were obtained as follows: 45.6 cal./g. for nylon 6, 46.8 cal./g. for nylon 66, 47.9 cal./g. for nylon 610, 54.1 cal./g. for nylon 11, 58.7 cal./g. for polyoxymethylene, 64.8 cal./g. for high density polyethylene, 15.9 cal./g. for polypropylene, and 35.4 cal./g. for poly-(3,3-bischloromethyl oxacyclobutane). In the course of crystallization, the exothermic peak appears in the temperature range of 20 to 100°C. below the melting point. The effect of melting temperature and molecular weight on the thermograms of crystallization of nylon 6 and polyoxymethylene was discussed and compared with the results obtained by dilatometry. For polymers such as nylon 6 with higher molecular weight and polyoxymethylene, two exothermic peaks of crystallization were observed, and it was found that the peak shape depends on the molecular weight. Nylon 6 containing nucleating agent shows only one exothermic peak independent on the molecular weight. It is presumed that the discontinuity of the growth rate of crystals is responsible for the thermograms having two peaks. JntroductionMost of the studies on crystallization of high polymers have been restricted so far to the growth of spherulites, the morphology of spherulites, single crystals, and the' isothermal crystallization kinetics by the use of dilatometry.Recently, differential thermal analysis has become one of the useful techniques for the investigation of transition behavior of high polymers. However, this is used mainly for the study of thermal behavior such as melting temperature and heat of fusion. The glass transition of high crystalline polymers could hardly be detected by differential thermal analysis, since the endothermic mass in this transition is much smaller than that of melting transition.As to the thermal properties of polymers, the melting behaviors of polyamide, * single crystals of polyethylene12 p~lyethylene,~ and polyolefin have been r e p~r t e d .~ In this paper, the melting and crystallization phenomena are treated as a reversible transition ; the crystallization behaviors are studied mainly by differential thermal analysis. The overall rate of crystallization is 2697

The present study suggests that dentures are one of the factors associated with mortality rates especially in female subjects with less than 10 functional teeth.

No abstract

The burden of severe rotavirus disease among Japanese children is substantial and warrants consideration of vaccination as a prevention strategy.

Background:To study how dental status can become a predictor of overall mortality risk.Methods: Community residents (n = 5730) over 40 years old in the Miyako Islands, Okinawa Prefecture, Japan were followed up for 15 years, 1987-2002. Functional tooth numbers were examined by dentists and overall mortalities of subjects with functional tooth numbers of <10 and 310 were compared in the age groups 40-49, 50-59, 60-69, 70-79 and 80 years or more in both males and females.Results: Groups of 80 years or more showed a significantly higher rate of overall mortality in subjects with functional tooth numbers of less than 10 than 10 or more, and there was no significant difference in the other age groups. Conclusion:The present study suggests that systemic attention to dental status should be recommended in older males.

Significant associations between periodontal status and several health conditions were found in the adult population examined, including gender, smoking habit, diastolic blood pressure, white blood cell counts, C-reactive protein, and serum IgG antibodies to P. gingivalis fimbriae, IgG P. gingivalis whole cell, and IgG A. actinomycetemcomitans whole cell titers.

Respiratory papillomatosis is a recalcitrant, human papillomavirus-induced disorder of the upper airways. Using in situ hybridization and biotinylated deoxyribonucleic acid (DNA) probes, the type of human papillomavirus (HPV) in nine adults was determined both at presentation and during subsequent recurrences. Fifty-three biopsies obtained from seven men and two women over a 10-year period were examined for HPV 6, 11, 16 and 18. All patients had either type 6 or 11. One dual infection was found. Despite intensive therapy with lasers with or without alpha interferon, no elimination or change in viral type occurred. Despite identical histologies, the amount of HPV DNA present in a given patient's papillomata varied markedly. The results suggest that adult-onset respiratory papillomatosis is due to HPV 6 and/or HPV 11, and that its recurrent nature, despite therapy, is due to persistence of a viral reservoir.