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A cross-sectional study was conducted with a national epidemiological survey to investigate the prevalence and demographic distribution of adult survivors of child abuse in Japan. A self-administered questionnaire was used to measure the history of child abuse and the demographic characteristics. The participants reported the following 4 types of child abuse: physical abuse (3%), sexual abuse (0.6%), neglect (0.8%), and psychological abuse (4%). Significant unequal distribution of child abuse was found to be associated with sex, living region, marital status, job status, and educational status. We determined the prevalence of adult survivors of child abuse in Japan and found that their demographic characteristics were unequally distributed. Policy makers and public health providers should take these demographic disparities into account in considering effective public health interventions for survivors of child abuse., (© 2013 APJPH.)

Purpose: To investigate the selection bias of an Internet panel survey organized by a commercial company., Methods: A descriptive study was conducted. The authors compared the characteristics of the Internet panel survey with a national paper-based survey and with national governmental statistics in Japan., Results: The participants in the Internet panel survey were composed of more women, were older, and resided in large cities. Regardless of age and sex, the prevalence of highly educated people in the Internet panel survey was higher than in the paper-based survey and the national statistics. In men, the prevalence of heavy drinkers among the 30- to 49-year-old population and of habitual smokers among the 20- to 49-year-old population in the Internet panel survey was lower than what was found in the national statistics., Conclusions: The estimated characteristics of commercial Internet panel surveys were quite different from the national statistical data. In a commercial Internet panel survey, selection bias should not be underestimated., (© 2012 APJPH.)

Background: The number of patients and incidence rate of Kawasaki disease (KD) are increasing in Japan. We have therefore characterized the latest epidemiological information on KD., Methods: The 22nd nationwide survey of KD, which targeted patients diagnosed with KD in 2011 and 2012, was conducted in 2013 and included a total of 1983 departments and hospitals. In order to report on all patients with KD during the 2 survey years, we targeted hospitals of 100 beds or more with pediatric departments, or specialized pediatric hospitals., Results: From a total of 1420 hospitals and departments (71.6% response rate), 26,691 KD patients were reported (12,774 in 2011 and 13,917 in 2012; 15,442 males and 11,249 females). The annual incidence rates were 243.1 per 100,000 population aged 0 to 4 years in 2011 and 264.8 in 2012. The number of cases of KD recorded in 2012 was the highest ever reported in Japan. The incidence rate of complete cases was also the highest ever reported in Japan and contributed to the increase in the rate of total cases in recent years. The number of patients diagnosed per month peaked in January, and additional peaks were noted during summer months, although these peaks were lower than those seen in winter. Age-specific incidence rate showed a monomodal distribution with a peak in the latter half of the year in which patients were born., Conclusions: The number of patients and the incidence rate of KD in Japan continue to increase. A similar trend has also been seen for patients with complete KD.

Objectives: Generalizable data on current satisfaction levels are required to establish a scientific basis for the political advancement of measures to improve satisfaction with hospital care among patients with diabetes. The present study made secondary use of existing official statistics in order to demonstrate the range of satisfaction levels with hospital care among diabetic outpatients and to closely examine related factors., Methods: Data sets that consolidated the Patient Survey, the Survey of Medical Care Institutions, and the Patient Behavior Survey (all from 2008) were created. Shared medical institution survey reference numbers were used to consolidate the data from the Patient Survey and the Survey of Medical Care Institutions, and in addition, sex and date of birth were used to consolidate the Patient Behavior Survey data. The range of satisfaction levels with hospital care among diabetic outpatients was investigated along with any relationship with the following potentially related factors: visitation status (first or repeat examination); waiting time until examination; examination duration; care-seeking status (any use of other medical facilities, etc.); diabetic complications; other complications; coverage under the Public Assistance Act; smoking cessation outpatient services; hospitals that specialized in treating diabetes (metabolic medicine); medical care on Saturday, Sunday, and public holidays; and provision of health checkups., Results: Overall, 62.3% of diabetic outpatients were either fairly or extremely satisfied with their hospital care, whereas 5.6% expressed dissatisfaction. Satisfaction levels with hospital care were found to be significantly related to visitation status, waiting time until examination, examination duration, care-seeking status, and Saturday medical care. Multivariate analysis with the factors demonstrated to be significantly related to satisfaction revealed significant relationships between high satisfaction levels and repeat examinations, short waiting times, no use of any other medical facilities, and long examinations., Conclusion: Consolidating official statistics from multiple sources indicated the range of satisfaction levels with hospital care among diabetic outpatients and facilitated the clarification of factors affecting satisfaction. Reducing waiting times and ensuring sufficient time spent on examinations are important for increasing satisfaction levels with hospital care among patients with diabetes. It is hoped that official statistics can be further applied to many future public health policy studies.

Purpose: To measure choroidal thickness in healthy pregnant women using enhanced depth imaging optical coherence tomography (EDI-OCT) and compare the measurements with those of healthy non-pregnant women., Methods: Choroidal thickness was measured at the fovea and at 3 mm superior, inferior, temporal, and nasal to the fovea using EDI-OCT in both healthy pregnant and non-pregnant women., Results: The mean choroidal thickness in pregnant women was 275 ± 84 μm at the subfoveal and 307 ± 89, 277 ± 89, 274 ± 74, and 159 ± 60 μm at 3 mm superior, inferior, temporal, and nasal to the fovea, respectively. In comparison, the mean choroidal thickness in non-pregnant women was 273 ± 92 μm at the subfoveal and 301 ± 73, 290 ± 76, 293 ± 77, and 149 ± 55 μm at 3 mm superior, inferior, temporal, and nasal to the fovea, respectively. No significant statistical differences were found for choroidal thickness between the pregnant and non-pregnant women at any of the measurement points., Conclusions: The results of this study demonstrate that the choroidal thickness was not significantly different between healthy pregnant and non-pregnant women.

Background: The long-term outcomes of Kawasaki disease (KD) are unknown., Methods: Fifty-two collaborating hospitals collected data on all patients who had received a new definite diagnosis of KD between July 1982 and December 1992. Patients were followed until December 31, 2009 or death. Standardized mortality ratios (SMRs) were calculated based on Japanese vital statistics data., Results: Of the 6576 patients enrolled, 46 (35 males and 11 females) died (SMR: 1.00; 95% CI: 0.73-1.34). Among persons without cardiac sequelae, SMRs were not high after the acute phase of KD (SMR: 0.65; 95% CI: 0.41-0.96). Among persons with cardiac sequelae, 13 males and 1 female died during the observation period (SMR: 1.86; 95% CI: 1.02-3.13)., Conclusions: In this cohort, the mortality rate among Japanese with cardiac sequelae due to KD was significantly higher than that of the general population. In contrast, the rates for males and females without sequelae were not elevated.

Objectives: The purpose of this study was to examine the epidemiological features of self-injury in Japan, and to investigate the factors associated with a history of self-injury, using nationwide random sample data on Japan in 2010., Methods: Questionnaires were distributed to 2,693 subjects, aged 16-49 years, randomly selected from the all over Japan using 2-stage stratified random sampling; the answers regarding self-injury were analyzed. Potential risk factors were compared between those who answered that they had a history of self-injury (self-injury group) and those who answered that they did not (non-self-injury group)., Results: Responses were obtained from 1,540 participants (response rate, 57.2%). Lifetime prevalence of having 1 or more self-injury events was 7.1% overall (3.9% for men; 9.5% for women) and approximately half of them reported a repetitive history of self-injury. Lifetime prevalence of self-injury was highest in those aged 16-29 years (9.9%, 16-29 years; 5.6%, 30-39 years; 5.7%, 40-49 years). Lifetime prevalence among women (16-29 years, 30-39 years, and 40-49 years) decreased with age (15.7%, 7.5%, and 5.8%, respectively), however, that among men increased with age (3.0%, 3.4%, and 5.5%, respectively). Compared with the non-self-injury group, those in the self-injury group were significantly more likely to have a history of cigarette smoking (self-injury group, 47.5%; non-self-injury group, 28.2%; adjusted odds ratio [95% confidence interval]: 2.18 [1.32-3.58]), childhood abuse (23.6% and 3.7%, respectively, 4.24 [2.18-8.25]), induced abortion (30.3% and 12.7%, respectively, 1.93[1.13-3.30]); moreover, they were significantly less likely to answer that they had a happy life when they were junior high school students (41.1% and 78.6%, respectively, 0.45 [0.25-0.79]). In addition, those in the self-injury group were more likely to report a history of parental divorce, that they did not have good communication with their parents, and that they did not have respect and appreciation for their parents; however, these factors were not significant after adjustment., Conclusion: These results are consistent with those of previous research reports in which the lifetime prevalence of self-injury was high among women aged 16-29 years, and in which self-injury was more likely to occur among individuals who had a history of cigarette smoking and childhood abuse. Such individuals should be provided care to prevent self-injury. In addition, from a social point of view, research examining family environments including these factors is required.

The second of two consecutively presented sounds may be perceived as being longer than if that sound had been presented in isolation. We performed five experiments using heterophonic patterns in which a sine tone was preceded by a frequency-band noise. We observed significant overestimations of sine-tone duration, the size of which depended on intensity and frequency differences between the band noise and the sine tone (Experiments 1 and 2). Band noises that were considerably shorter than the sine tone still caused significant overestimations (Experiment 3). A short silent gap between the band noise and the sine tone strongly reduced the amount of overestimation (Experiment 4). Both frozen and nonfrozen band noises yielded overestimations (Experiment 5). Our explanation for the overestimation is that the onset of the sine tone is blurred by the band noise and that such a blurred onset is restored at the level of perceptual organization following rules of a simple auditory grammar. This restoration takes mental processing time, which adds to the perceived duration of the sine tone. We call this illusion time stretching and discuss the notion that subsequent temporal assimilation and/or contrast effects can dilate or compress the amount of stretching.

Activity of an inwardly rectifying K(+) channel with inward conductance of about 40 pS in cultured human renal proximal tubule epithelial cells (RPTECs) is regulated at least in part by protein phosphorylation and dephosphorylation. In this study, we examined involvement of calcineurin (CaN), a Ca(2+)/calmodulin (CaM)-dependent phosphatase, in modulating K(+) channel activity. In cell-attached mode of the patch-clamp technique, application of a CaN inhibitor, cyclosporin A (CsA, 5 microM) or FK520 (5 microM), significantly suppressed channel activity. Intracellular Ca(2+) concentration ([Ca(2+)]( i )) estimated by fura-2 imaging was elevated by these inhibitors. Since inhibition of CaN attenuates some dephosphorylation with increase in [Ca(2+)]( i ), we speculated that inhibiting CaN enhances Ca(2+)-dependent phosphorylation, which might result in channel suppression. To verify this hypothesis, we examined effects of inhibitors of PKC and Ca(2+)/CaM-dependent protein kinase-II (CaMKII) on CsA-induced channel suppression. Although the PKC inhibitor GF109203X (500 nM) did not influence the CsA-induced channel suppression, the CaMKII inhibitor KN62 (20 microM) prevented channel suppression, suggesting that the channel suppression resulted from CaMKII-dependent processes. Indeed, Western blot analysis showed that CsA increased phospho-CaMKII (Thr286), an activated CaMKII in inside-out patches, application of CaM (0.6 microM) and CaMKII (0.15 U/ml) to the bath at 10(-6) M Ca(2+) significantly suppressed channel activity, which was reactivated by subsequent application of CaN (800 U/ml). These results suggest that CaN plays an important role in supporting K(+) channel activity in RPTECs by preventing CaMKII-dependent phosphorylation.

The activity of an inwardly rectifying K(+) channel in cultured human renal proximal tubule cells (RPTECs) is stimulated and inhibited by nitric oxide (NO) at low and high concentrations, respectively. In this study, we investigated the effects of IFN-gamma, one of the cytokines which affect the expression of inducible NO synthase (iNOS), on intracellular NO and channel activity of RPTECs, using RT-PCR, NO imaging, and the cell-attached mode of the patch-clamp technique. Prolonged incubation (24 h) of cells with IFN-gamma (20 ng/ml) enhanced iNOS mRNA expression and NO production. In these cells, a NOS inhibitor, N(omega)-nitro-l-arginine methyl ester (l-NAME; 100 microM), elevated channel activity, suggesting that NO production was so high as to suppress the channel. This indicated that IFN-gamma would chronically suppress channel activity by enhancing NO production. Acute effects of IFN-gamma was also examined in control cells. Simple addition of IFN-gamma (20 ng/ml) to the bath acutely stimulated channel activity, which was abolished by inhibitors of IFN-gamma receptor-associated Janus-activated kinase [P6 (1 microM) and AG490 (10 microM)]. However, l-NAME did not block the acute effect of IFN-gamma. Indeed, IFN-gamma did not acutely affect NO production. Moreover, the acute effect was not blocked by inhibition of PKA, PKG, and phosphatidylinositol 3-kinase (PI3K). We conclude that IFN-gamma exerted a delayed suppressive effect on K(+) channel activity by enhancing iNOS expression and an acute stimulatory effect, which was independent of either NO pathways or phosphorylation processes mediated by PKA, PKG, and PI3K in RPTECs.

Nitric oxide (NO) modulates the activity of an inwardly rectifying K(+) channel in cultured human proximal tubule cells. In this study, we investigated which NO synthase (NOS) isoform(s) was involved in the endogenous production of NO and hence the regulation of channel activity. The patch-clamp experiments using the cell-attached mode showed that a nonselective NOS inhibitor, N(omega)-nitro-L-arginine methyl ester (L-NAME; 100 microM), suppressed channel activity, whereas a NOS substrate, L-arginine (500 microM), stimulated it. A neuronal NOS (nNOS)/inducible NOS (iNOS)-selective inhibitor, 1-(alpha,alpha,alpha-trifluoro-o-tolyl)-imidazole (TRIM; 100 microM), suppressed channel activity to the same extent as L-NAME. TRIM also blocked the stimulatory effect of L-arginine. In contrast, an NO donor, sodium nitroprusside (10 microM) or 8-bromoguanosine 3',5'-cyclic monophosphate (100 microM) stimulated channel activity even in the presence of TRIM. RT-PCR revealed that iNOS mRNA alone was expressed in most of the cultures, i.e., 34 out of 40. In the other 6 cases, endothelial NOS (eNOS) and iNOS mRNA were simultaneously expressed. This finding was confirmed at the protein level by Western blotting. Indeed, in the patch-clamp experiments TRIM sometimes failed to suppress the channel activity, but the following addition of L-NAME suppressed it. However, since the suppressive effect of TRIM was usually similar to that of L-NAME, the involvement of eNOS in K(+) channel regulation would be relatively low. These results suggest that iNOS plays a pivotal role in the endogenous production of NO under the basal condition, which is involved in the activity of the inwardly rectifying K(+) channel in cultured human proximal tubule cells.

To examine the possibility of a vaccine for Candida albicans infection in the oral cavity, we induced salivary antibodies by immunization of killed-C. albicans ATCC 18804 on the palatine tonsils of rabbits. The enzyme-linked immunosorbent assay reaction of salivary antibodies was high against C. albicans serotype A. The saliva antibodies greatly inhibited C. albicans adherence to cloned epithelial cells from human gingiva. Tonsillar immunizations of C. albicans ATCC 18804 induce salivary antibodies that prevent C. albicans adherence to epithelial cells, and thus should prove useful in the prevention of oral candidiasis caused by C. albicans serotype A.

We performed [123I] MIBG myocardial scintigraphy in two of three patients with PARK2 from unrelated families and examined the heart tissues from the three patients immunohistochemically using an antibody against tyrosine hydroxylase (TH) to see whether cardiac sympathetic nerve is involved. Cardiac uptake of MIBG was normal except for a slight decrease in the late phase in one of the patients. Postmortem examination revealed that TH-immunoreactive nerve fibers in the epicardium were well preserved in all three patients. The present study confirmed that cardiac sympathetic nerve is well preserved in PARK2 with a homozygous exon deletion, which accounts for normal cardiac uptake of MIBG. Moreover, normal cardiac uptake of MIBG might be of potential diagnostic value to indicate the absence of Lewy body pathology, even in patients with levodopa-responsive Parkinsonism, as in PARK2., (Copyright (c) 2005 Movement Disorder Society.)

Schwannoma are benign, slowly growing nerve sheath tumors. They can arise from any peripheral nerve containing Schwann cells including distal portions of cranial nerves. Intracranial schwannomas arising from a subfrontal lesion are very rare. We report a case of subfrontal schwannoma in a 38-year-old woman who presented with seizures in February 2003. MRI features resembled an olfactory groove meningioma. The left carotid angiography revealed that the tumor was supplied from the anterior ethomoidal artery, but no remarkable tumor stain was detected. Bifrontal craniotomy with total excision of the tumor revealed a tumor in the left subfrontal lesion. The tumor was attached to the falx, the left frontal lobe, and the skull base, but those structures were not invaded. Unfortunately, we could not detect the left olfactory nerve. Subsequent histological examination proved that this tumor was schwannoma. A review of the literature found 27 cases including this case previously described. Arguments are raised that schwannomas in a subfrontal lesion are comparable to or even occur as intracranial schwannomas with frontal localization. Subfrontal schwannomas are very rare. For this reason they have not been diagnosed preoperatively. Further cases of subfrontal schwannoma are necessary to solve the enigma of the origin of this tumor.

Decreased cardiac uptake of meta-iodobenzylguanidine (MIBG) on [123I]MIBG myocardial scintigraphy has been reported in Parkinson's disease (PD) and dementia with Lewy bodies (DLB). We hypothesized that cardiac sympathetic denervation might account for the pathomechanism. To elucidate the extent, frequency and pattern of cardiac sympathetic nerve involvement in Lewy body disease and related neurodegenerative disorders, we immunohistochemically examined heart tissues from patients with PD (n=11), DLB (n=7), DLB with Alzheimer's disease (DLB/AD; n=4), multiple system atrophy (MSA; n=8), progressive supranuclear palsy (PSP; n=5), pure AD (n=10) and control subjects (n=5) together with sympathetic ganglia from patients with PD (n=5) and control subjects (n=4), using an antibody against tyrosine hydroxylase (TH). TH-immunoreactive nerve fibers in the hearts had almost entirely disappeared in nearly all the patients with PD, DLB and DLB/AD, whereas they were well preserved in all the patients with PSP and pure AD as well as in all except for one patient with MSA. In PD, neurons in the sympathetic ganglia were preserved in all except for one patient. Decreased cardiac uptake of MIBG in Lewy body disease reflects actual cardiac sympathetic denervation, which precedes the neuronal loss in the sympathetic ganglia.

Our patient developed Riedel's thyroiditis soon after having an exacerbation of multiple sclerosis (MS). MS has been associated with other autoimmune diseases, including thyroiditis, and both Hashimoto's thyroiditis and subacute thyroiditis have been described in connection with MS. Yet, we are not aware of any other patient reported to have concomitant MS and Riedel's thyroiditis. The association between MS and Riedel's thyroiditis remains obscure but may reflect an autoimmune disorder common to both diseases.

We performed [123I]-meta-iodobenzylguanidine (MIBG) myocardial scintigraphy in 180 patients with PD, 24 patients with DLB and 73 patients with the other related diseases. The heart-to-mediastinum (H/M) ratio in PD and DLB was significantly low compared to that in MSA, PSP, CBD and AD. The H/M ratio tended to decrease with the disease progression in PD. These findings suggest that MIBG myocardial scintigraphy could be a marker for differential diagnosis of PD and DLB. We immunohistochemically examined heart tissues from patients with pathologically confirmed PD, DLB and the other related diseases using monoclonal antibody against tyrosine hydroxylase (TH). The number of TH-immunoreactive nerve fibers in the epicardium was moderate or abundant in most patients with MSA, PSP, CBD and AD as well as in the control subjects. Contrarily, the number of TH-immunoreactive nerve fibers in the epicardium had almost completely disappeared in nearly all the patients with PD and DLB. These findings suggest that postganglionic cardiac sympathetic nerve denervation occurs in PD and DLB but not in MSA, PSP, CBD and AD, which accounts for the decreased cardiac uptake of MIBG in PD and DLB, but not in the other diseases.

We describe the usage of positron emission tomography (PET) as an aid in the initial diagnosis and follow-up of Riedel's thyroiditis. A 41-year-old patient was admitted for an enlarged and tender thyroid gland in association with severe systemic symptoms of inflammation. Imaging with fluorine-18 fluorodeoxyglucose (FDG) and PET demonstrated an intensive uptake of FDG in both lobes of the thyroid gland as an indication of severe inflammation. The diagnosis of Riedel's thyroiditis was confirmed by the histological findings of biopsy specimens taken during a palliative thyroid resection. The inflammatory symptoms and local pain dramatically disappeared after commencement of high-dose corticosteroid therapy. A follow-up PET scan after 2 weeks of corticosteroid treatment showed a 60% decrease in the uptake of FDG in the thyroid. This indicates that FDG metabolic activity can also be used to assess a patient's response to therapy in Riedel's thyroiditis.

The wearing behaviors of a hybrid composite resin for crown and bridge (ES) were examined using a two-body impacting-sliding wear test with a porcelain (PO), Au-Ag-Pd alloy (PD), direct restorative composite resin (CR) and tooth enamel (TO). Although PO was the hardest of all, it showed the largest wear together with ES in the combination of ES-PO, which was probably initiated from the superficial destruction by their impact. The wear in ES-PD was the second largest. It was noted in this combination that the surface of ES was partially contaminated by scraped thin layers of PD to a degree distinguished by the naked eye. The mutual wears of the components were relatively low in the combination of ES with CR, TO or ES itself. It is suggested from these findings that the hybrid composite resin may be useful as an alternative to porcelain for posterior crown and bridge unless it opposes porcelain or alloys.

The aim of this study was to investigate the usefulness of whole-body MRI(WB-MRI) in the evaluation of cellularity in bone marrow and the distribution of fatty marrow in aplastic anemia. WB-MRI was performed on five patients with aplastic anemia who ranged in age from 62 to 70 years of age, and on four controls with malignant lymphoma who ranged in age from 59 to 67 years. Coronal images were obtained using a body coil with an FOV of 48 cm x 48 cm, and with both fast short T1 inversion recovery(STIR) and spin-echo T1-weighted(T1-WI) in three regions: (1) head to thorax, (2) abdomen to pelvis, and (3) lower extremities. The findings on WB-MRI were compared with those of histological studies of bone marrow at the sternum and the posterior iliac crest. The results were as follows: (1) there was a correlation between the cellularity of histological studies of bone marrow and signal intensity on WB-MRI; (2) WB-MRI could detect the activity of bone marrow; and (3) in a comparison of signal intensity in aplastic anemia and control subjects, there were differences of signal intensity in the central marrow.

Although titanium alloys are known to be biocompatible with bone tissue after implantation in human beings, the effect of titanium on osteoclasts remains to be studied. We examined the effect of titanium salt on the formation and survival of osteoclasts in cell culture. The addition of 10 microM titanium tetrachloride caused a decrease in the cell number of osteoclast-like cells induced in bone marrow cell cultures taken from mice. The addition of 10 microM titanium tetrachloride caused degradation of the disaggregated osteoclasts taken from neonatal rats and a decrease in bone resorption. Along with the increase in the degradation of osteoclasts, the number of apoptotic cells increased. Titanium tetrachloride dose-dependently decreased the cell number and alkaline phosphatase activity of osteoblastic cell cultures taken from rat calvaria. However, these concentrations were 30-40 times higher than those in the case of osteoclast-like cell formation. These results showed that titanium ions caused a preferential degradation of osteoclasts rather than osteoblasts, most likely by apoptosis., (Copyright 2001 John Wiley & Sons, Inc. J Biomed Mater Res 55: 313-319, 2001)

Occlusal forces cause stress which morphologically affects the supporting tissues of implants. The aim of this study was to examine the effects of occlusal forces on the distribution of neurofilament protein (NFP)-positive nerve fibers in the tissue of peri-implant bone. The bilateral 2nd, 3rd and 4th mandibular premolars and the 1st molars were extracted from three mongrel dogs. After 4 months of healing, 4 screw-type implants were inserted in the oral cavity. Three months after insertion, the implants on the molar site were loaded by occlusal forces, while those on the premolar site were unloaded. After a further 3 months, the dogs were sacrificed, and specimens were prepared for immunohistochemical NFP-positive staining by the labeled-streptavidin-biotin method. Many NFP-positive nerve fibers were found in the tissues of the loaded site when compared with the unloaded site. These fibers were localized in both the bone marrow space and in the peri-implant fibrous tissue. They had two types of nerve endings: simple free nerve endings, and nerve endings with tree-like ramifications. The present results suggest that loading by occlusal force causes an increase in the number of NFP-positive nerve fibers, many of which have free nerve endings in the peri-implant tissue. The possible role of these NFP-positive nerve fibers is discussed.

The roughness of the bone matrix surface affects osteoblastic differentiation. However, the effect of the roughness of the matrix surface on osteoclastic bone resorption remains to be studied. We examined the latter effect using disaggregated osteoclasts from neonatal rats. The resorption pit number and the total pit area on the rough surface were not different from those on smooth surfaces after 1 day, but they were 2 or more times higher after 3 days. The number of osteoclasts was not different on bone slices with either smooth or rough surfaces at 3 days. The alkaline phosphatase (ALP)-positive osteoblasts were relatively rare in both types of slices at first, then the number and the diameter of the enzyme-positive cells and the clusters preferentially increased on the rough bone slices. When hydroxyurea was added to the culture in order to suppress the proliferation and the subsequent differentiation of osteoblastic cells on rough surfaces, the increase in resorption on the rough surfaces was effaced; however, this agent had little affect on resorption of the smooth surfaces. The addition of ALP-positive cells to disaggregated osteoclasts increased bone resorption on the smooth surface. The results suggest that osteoblast development and subsequently bone resorption by osteoclasts is enhanced by the roughness of matrix surfaces., (Copyright 1999 John Wiley & Sons, Inc.)

Myotonic dystrophy (DM) results from the amplification of an unstable CTG repeat in the 3' untranslated region of a transcript encoding an MtPK. Infants with congenital DM are shown to have on average a greater amplification of the CTG repeat than is seen in the noncongenital DM patients. The major clinical features of congenital DM are bilateral facial weakness, hypotonia, feeding difficulties, respiratory distress, delayed motor development and mental retardation. We present 6 patients, aged 11-35 years, from unrelated 5 families with clinical symptoms of congenital DM. The four of the patients were inherited paternally and only one showed a reduction in the CTG repeat size.

This study investigated the correlation between cardiac abnormalities and cytosine-thymine-guanine (CTG) trinucleotide repeat expansion in patients with myotonic dystrophy (MD). We studied 18 patients with the adult form of classical MD and 18 age-matched control subjects. In patients with MD, left ventricular systolic function at rest was not different from that in normal subjects. On the other hand, of Doppler parameters of diastolic function, mitral inflow peak early velocity and atrial velocity were significantly lower, and deceleration time and isovolumic relaxation time were significantly longer in patients with MD compared with normal controls. No significant correlation was observed between these diastolic parameters and CTG repeat expansion in the patients, although the parameters showed a positive correlation with age and neurologic symptom duration. Electrocardiographic conduction abnormalities were detected in 42% of patients. These patients showed a significantly longer symptom duration, although the size of CTG repeats was not different between the patients with and without conduction abnormalities. This study demonstrated that, in patients with MD, significant alterations in ventricular diastolic function (myocardial myotonia) occur in addition to conduction abnormalities. The size of CTG expansion is not a predictor of these cardiac involvements.

We have treated 18 patients with vaginal agenesis without functioning uterus (MRK syndrome) during the period between January 1984 and December 1995 by Frank's dilatation method followed by the surgical method without grafting. Clinical records were retrospectively examined in regard to the size and functions of the constructed vagina and problems of the treatment procedure. The treatment by non-invasive dilatation was first carried out starting at the age of 21 years 2 months +/- 2 years 9 months (Mean +/- SD) for the duration of 10.9 +/- 9.8 months. It was successful (constructed vaginal length by pressure: 60 mm or more) in 6 patients (35.3%). The non-grafting surgical method was then performed. The operation time was 2:11 +/- 0:37 hours and no complications were observed. The vaginal lengths 1 month after operation and at the latest follow-up were enough to have coitus and no severe complications including shrinkage were observed. Our method to construct a new vagina is simple and useful, since it left no operation scar in contrast to McIndoe's method and other methods with grafting.

Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant muscular disorder in which the disease locus has been mapped to chromosome 4q35-qter. In most patients, the DNA rearrangements associated with FSHD have been found in the EcoRI fragment detected by the p13E-11 probe, and deletions of the 3.2 kb repeat units within the fragment are thought to cause the disease. To examine FSHD-associated DNA rearrangements in the Japanese population, we performed Southern blot analysis of the genomic DNA, using the p13E-11 and pFR-1 probes, in 158 Japanese individuals, including 38 FSHD patients from 19 families. We found that all but one (a possible affected recombinant) of the Japanese FSHD patients (97.4%) had specific smaller (< 28 kb) EcoRI fragments which cosegregated with the disease; this included four patients who had severe inflammatory changes in the muscle and eight patients with de novo DNA rearrangements. We found no FSHD patient who had a fragment larger than 28 kb. By contrast, only two of 35 Japanese controls (5.7%) had EcoRI fragments smaller than 28 kb. Our patients showed anticipation, i.e. decreased size of the EcoRI fragment in parallel with earlier onset of the disease (r = 0.531, P = 0.003, with younger age at onset in children (17.8 +/- 7.0) than their affected parents (31.5 +/- 14.8) (P = 0.019). However, since each family had a specific small EcoRI fragment associated with the disease, the differing clinical severity within a family cannot be explained by the size of the fragment alone.(ABSTRACT TRUNCATED AT 250 WORDS)

Primary epidermoid tumors comprise about 1% of all central nervous system neoplasms, although the diploic epidermoid tumor is comparatively rare. Two cases of diploic epidermoid tumor are reported in this paper. Case 1: A 70-year-old man presented with a headache. A plain craniogram showed an osteolytic lesion of the occipital bone with a well defined sclerotic margin. A contrast enhanced CT confirmed a cystic lesion with rim enhancement. On MRI, the tumor appeared hypointense surrounded with irregular hyperintensity on the T1WI and hyperintensity on the T2WI. Gd enhancement on the MRI showed no enhancement effect. The tumor was totally removed and cranioplasty was performed. No tumor invasion of the dura mater was noticed. Case 2: A 90-year-old woman presented with a giant tumor of the left parietal region. She noticed a painless swelling at the age of 20, and the tumor slowly grew over a period of 70 years. Plain craniogram showed a bony defect with a sclerotic margin. CT scan confirmed an extracranial giant tumor with destruction of the outer table under the tumor, and also falx meningioma. Aspiration and irrigation inside the cystic tumor were performed under local anesthesia. Previous authors have also said that the plain craniogram is characteristic and diagnostic in the case of diploic epidermoid. Typical round or polylobular bony defect with well defined sclerotic margins was visualized.

Using helical strips of the bovine middle cerebral arteries, changes in vascular tension were measured during isometric contractions induced by endothelin. 1) Both Ca(++)-free media and Ca(++)-antagonists depressed the endothelin-induced contractions only by 40% of the control, suggesting the involvement of both Ca(++)-entry from outside the muscle cell and intracellular Ca(++)-release from the sarcoplasmic reticulum. 2) Endothelin-induced contractions were significantly depressed by 1 microgram/ml tetrodotoxin (TTX). Relative size of depression by TTX was practically the same as that observed in Na(+)-free media without TTX. These results indicated a partial involvement of Na(+)-entry through TTX-sensitive Na(+)-channels. 3) Endothelin-induced contractions were effectively depressed by NCDC, an inhibitor of phospholipase C, suggesting the involvement of PI-turnover in the contraction. 4) Protein kinase inhibitors such as H-7 and H-8 effectively depressed endothelin-induced contractions. This result suggested the phosphorylation of a certain protein by protein kinase C as a cause of long lasting contractions. 5) A phospholipase A2 (PL A2) inhibitor, quinacrine, significantly depressed the endothelin-induced contractions, suggesting a possible involvement of PL A2. However, neither the cyclooxygenase inhibitor nor the lipoxygenase inhibitor depressed endothelin-induced contractions.

A 23-year-old man with Von Recklinghausen's disease was admitted because of abdominal pain. The preoperative diagnosis was malignant mesenteric tumor. Laparotomy was done and the tumor was located in the mesentery of the ileum which showed numerous tortuous cords or bulbous projections from the surface. Partial resection of the involved ileum and the mesentery was performed. Histological examination revealed plexiform neurofibroma and malignant Schwannoma of the mesentery. It is very difficult to preoperatively diagnose early associated lesions of Von Recklinghausen's disease. Therefore, careful examination of the GI tract is important so that earlier therapy can be carried out.