EFFICACY OF HOMOGENOUS BONE GRAFTING IN MCCUNE-ALBRIGHT SYNDROME

A case of McCune-Albright syndrome is reported. The patient was a girl aged 8 years. The chief complaints were gait disturbance and a limp. Roentgenograms showed collapse and severe varus deformity of the femoral neck on the right side with 64° of neck-shaft angle. She had an episode of vaginal bleeding at the age 7 years. Valgus osteotomy of the right proximal femur for the correction of coxa vara and a bone transplantation of an allograft from her mother were performed. Seventy degrees of correction were obtained and the discrepancy of limb length was 1cm after the operation. Roentgenograms showed sufficient callus and solid union between the fragments and the grafted bone 1 year after the operation. Frozen allograft bone was very effective in stimulating bone induction even in fibrous lesions of a patient with McCune-Albright syndrome. Immunology of bone grafts, HLA antigens, osteogenesis in grafts, and the etiology of McCune-Albright syndrome were discused

Normal mitochondrial respiratory function is essential for spatial remote memory in mice

<p>Abstract</p> <p>Background</p> <p>Mitochondrial DNA (mtDNA) with pathogenic mutations has been found in patients with cognitive disorders. However, little is known about whether pathogenic mtDNA mutations and the resultant mitochondrial respiration deficiencies contribute to the expression of cognitive alterations, such as impairments of learning and memory. To address this point, we used two groups of <it>trans</it>-mitochondrial mice (mito-mice) with heteroplasmy for wild-type and pathogenically deleted (Δ) mtDNA; the "low" group carried 50% or less ΔmtDNA, and the "high" group carried more than 50% ΔmtDNA.</p> <p>Results</p> <p>Both groups had normal phenotypes for not only spatial learning, but also memory at short retention delays, indicating that ΔmtDNA load did not affect learning and temporal memory. The high group, however, showed severe impairment of memory at long retention delays. In the visual cortex and dentate gyrus of these mice, we observed mitochondrial respiration deficiencies, and reduced Ca²⁺/calmodulin-dependent kinase II-α (α-CaMKII), a protein important for the establishment of spatial remote memory.</p> <p>Conclusion</p> <p>Our results indicated that normal mitochondrial respiratory function is necessary for retention and consolidation of memory trace; deficiencies in this function due to high loads of pathogenically mutated mtDNA are responsible for the preferential impairment of spatial remote memory.</p

知的障害養護学校教員の個別の指導計画作成に対する捉えの構造

本研究は，知的障害養護学校教員の個別の指導計画作成に対する捉えの構造を因子分析によって探索することを目的とした。因子分析の結果，「優先性」と「既知性」の2つの因子が抽出され，知的障害養護学校員は個別の指導計画作成を優先的・中核的な職務として捉え，その概要を既に知っていると感じる意識構造にあることが示唆された。さらに，2つの因子の因子得点を用いて，教員の属性が個別の指導計画作成に対する捉えに及ばす影響についての検討を行った。その結果，主事・主任を担当している教員は，担当していない教員よりも，個別の指導計画作成を優先的・中核的な職務として，かつその概要を既に知っていると強く意識していた。また，中学部数員は，小学部教員よりも，個別の指導計画作成を優先的・中核的な職務して強く意識していた。This study was designed to explore the factors that compose the consciousness for individualized teaching programs. Four hundred and five teachers from special schools for children with intellectual disabilities were interviewed by questionnaire. The questionnaire focused on questions concerning the consciousness of individualized teaching programs and teacher attributes. The data was analyzed using "factor analysis." As a result, two factors were found: "Priority" and "Known." Through "analysis of variance" with the factor score, teachers with management roles were highly conscious of "Priority" and "Known." Junior high school teachers were highly conscious of "Priority.

Case report: Treatment of persistent atypical odontalgia with attention deficit hyperactivity disorder and autism spectrum disorder with risperidone and atomoxetine

Chronic pain has recently been associated with developmental disorders [autism spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD)]. Regarding chronic pain in adulthood, fibromyalgia, migraine, and chronic low back pain have been associated with ADHD. The ICD-11 disease classification categorizes these pain diseases as chronic primary pain, suggesting high comorbidity with developmental disorders in chronic primary pain. Atypical odontalgia (AO) is a persistent tooth pain that occurs in the absence of any of the usual dental causes, most of which are triggered by dental treatment. Conditions characterized by tooth pain with no apparent cause are also classified as chronic primary pain. Approximately half the patients with AO are diagnosed with psychiatric disorders; the most common are depression (15.4%) and anxiety disorders (10.1%). However, there are no reports on neurodevelopmental disorders comorbid with AO. In the present study, we report a case of a 46-year-old man with numerous complaints (e.g., occlusal instability, difficulty eating, difficulty speaking), who took work leave due to worsening of his symptoms after periodontal scaling (“gingival recession” and “aggressive periodontal treatment”) and frequently expressed dissatisfaction and anger at the hospital, making the dental treatment difficult. After a referral to a psychiatrist specializing in chronic pain, AO and previously undiagnosed comorbidity of ASD and ADHD were confirmed. Atypical antipsychotic risperidone for ASD irritability and an ADHD medication, atomoxetine dramatically reduced anger, pain, anxiety, depression, and pain catastrophizing thoughts, leading to reduced obsession with his symptoms and less frequent complaints. After risperidone (1 mg/day) + atomoxetine (120 mg/day) were ultimately prescribed after adjustment, he was able to return to work 226 days after initiation of psychiatric treatment. Recent studies show that comorbidity of developmental disorders in patients with chronic pain is likely to be undetected. Clinicians should include screening for ASD and ADHD not only in cases of fibromyalgia, migraine, and chronic low back pain, but also in orofacial pain such as AO and other treatments for chronic primary pain. For patients diagnosed with ASD or ADHD, an effective drug therapy for ASD and ADHD should be considered

Comprehensive Behavioral Analysis of Calcium/Calmodulin-Dependent Protein Kinase IV Knockout Mice

Calcium-calmodulin dependent protein kinase IV (CaMKIV) is a protein kinase that activates the transcription factor CREB, the cyclic AMP-response element binding protein. CREB is a key transcription factor in synaptic plasticity and memory consolidation. To elucidate the behavioral effects of CaMKIV deficiency, we subjected CaMKIV knockout (CaMKIV KO) mice to a battery of behavioral tests. CaMKIV KO had no significant effects on locomotor activity, motor coordination, social interaction, pain sensitivity, prepulse inhibition, attention, or depression-like behavior. Consistent with previous reports, CaMKIV KO mice exhibited impaired retention in a fear conditioning test 28 days after training. In contrast, however, CaMKIV KO mice did not show any testing performance deficits in passive avoidance, one of the most commonly used fear memory paradigms, 28 days after training, suggesting that remote fear memory is intact. CaMKIV KO mice exhibited intact spatial reference memory learning in the Barnes circular maze, and normal spatial working memory in an eight-arm radial maze. CaMKIV KO mice also showed mildly decreased anxiety-like behavior, suggesting that CaMKIV is involved in regulating emotional behavior. These findings indicate that CaMKIV might not be essential for fear memory or spatial memory, although it is possible that the activities of other neural mechanisms or signaling pathways compensate for the CaMKIV deficiency

An Incarcerated Colon Inguinal Hernia That Perforated into the Scrotum and Exhibited an Air-Fluid Level

There are few reports of a transverse colon inguinal hernia; furthermore, an inguinal hernia perforating the scrotum is rare. Here we report the case of a 79-year-old man who died after developing an incarcerated colon inguinal hernia that perforated the scrotum and exhibited an air-fluid level. The patient was referred to our hospital in November 2011 with a complaint of inability to move. Physical examination revealed an abnormally enlarged left scrotum and cold extremities. He reported a history of gastric cancer that was surgically treated more than 30 years ago. His white blood cell count and C-reactive protein level were elevated. Abdominal and inguinal computed tomography revealed that his transverse colon was incarcerated in the left inguinal canal. Free air and air-fluid level were observed around the transverse colon, suggestive of a perforation. The patient and his family refused any surgical intervention; therefore, he was treated with sultamicillin tosilate hydrate and cefotiam hydrochloride. However, he succumbed to panperitonitis 19 days after admission. The findings from this case indicate that the transverse colon can perforate into an inguinal hernia sac

The Cilium: Cellular Antenna and Central Processing Unit

Cilia mediate an astonishing diversity of processes. Recent advances provide unexpected insights into the regulatory mechanisms of cilium formation, and reveal diverse regulatory inputs that are related to the cell cycle, cytoskeleton, proteostasis, and cilia-mediated signaling itself. Ciliogenesis and cilia maintenance are regulated by reciprocal antagonistic or synergistic influences, often acting in parallel to each other. By receiving parallel inputs, cilia appear to integrate multiple signals into specific outputs and may have functions similar to logic gates of digital systems. Some combinations of input signals appear to impose higher hierarchical control related to the cell cycle. An integrated view of these regulatory inputs will be necessary to understand ciliogenesis and its wider relevance to human biology

Citizen science: a new approach to advance ecology, education, and conservation

Citizen science has a long history in the ecological sciences and has made substantial contributions to science, education, and society. Developments in information technology during the last few decades have created new opportunities for citizen science to engage ever larger audiences of volunteers to help address some of ecology’s most pressing issues, such as global environmental change. Using online tools, volunteers can find projects that match their interests and learn the skills and protocols required to develop questions, collect data, submit data, and help process and analyze data online. Citizen science has become increasingly important for its ability to engage large numbers of volunteers to generate observations at scales or resolutions unattainable by individual researchers. As a coupled natural and human approach, citizen science can also help researchers access local knowledge and implement conservation projects that might be impossible otherwise. In Japan, however, the value of citizen science to science and society is still underappreciated. Here we present case studies of citizen science in Japan, the United States, and the United Kingdom, and describe how citizen science is used to tackle key questions in ecology and conservation, including spatial and macro-ecology, management of threatened and invasive species, and monitoring of biodiversity. We also discuss the importance of data quality, volunteer recruitment, program evaluation, and the integration of science and human systems in citizen science projects. Finally, we outline some of the primary challenges facing citizen science and its future.Dr. Janis L. Dickinson was the keynote speaker at the international symposium at the 61th annual meeting of the Ecological Society of Japan. We appreciate the Ministry of Education, Culture, Sports, Science and Technology in Japan for providing grant to Hiromi Kobori (25282044). Tatsuya Amano is financially supported by the European Commission’s Marie Curie International Incoming Fellowship Programme (PIIF-GA-2011- 303221). The findings and conclusions in this report are those of the authors and do not necessarily represent the views of the funding agencies or the Department of the Interior or the US Government.This is the final version of the article. It was first available from Springer via http://dx.doi.org/10.1007/s11284-015-1314-

Surveys of full–coverage restorations made of titanium at Matsumoto Dental University Hospital

Considering the presence of metal allergies and the sharp rise in the prices of precious metals, full–coverage restorations made of titanium have been covered by Japanese insurance since June 1, 2020. The clinical application of these restorations is expected to increase in the future. However, due to its high melting point and high affinity to oxygen at high temperatures, titanium has not been widely used in prosthetic clinical practice. This study aimed to survey the actual use of full–coverage metal restorations at Matsumoto Dental University Hospital. Patients who had full–coverage metal restorations placed on their molars from June 1 to December 28, 2020, were consecutively enrolled, and data on the type of metal, placement site, and time were recorded. Besides, the dentists who used titanium restorations (Ti–Rs) were given a questionnaire. We asked them to rate their impressions of Ti–Rs in comparison with full–coverage restorations made of Ag–Pd–Cu–Au alloy (Pd–R) in six categories, including occlusal adjustment, polishing, and luting operations. In all, 338 molars received full–coverage metal restorations. Ti–R was used in 33 cases and Pd–R in 305 cases; 14 upper and 19 lower, 12 first molars, 18 second molars, and 3 third molars received Ti–Rs, while 137 upper and 168 lower, 174 first molars, 174 second molars, and 3 third molars received Pd–Rs. Seven dentists responded to the questionnaire survey. Many dentists stated that Ti–R was more challenging to use than Pd–R for occlusal adjustment, polishing, and adjustment time. On the other hand, most of them stated that the luting process was similar for both. Besides, Ti–R was favorably evaluated in terms of metal allergy. The number of patients who received Ti–Rs increased during the study period, suggesting that Ti–Rs will be a commonly used metal allergy–friendly prosthetic in the future. We plan to continue the survey and study the prognosis