[Bloom syndrome].
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Biomedical subjects
Publications and source records attributed to Manabu Maeda.
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A new quantitative calibration algorithm, called "Moment Combined Partial Least Squares (MC-PLS)", which combines the moment of spectrum and conventional PLS was proposed. Its calibration performance was evaluated for the analyses of three import petroleum and petrochemical products: gasoline, naphtha and polyol samples. The selected properties for these products included the research octane number (RON) and Reid vapor pressure (RVP) for gasoline, the distillation temperature at 10% (D 10%) for naphtha and the hydroxyl (OH) number for polyol. The major concept presented here used the moment to find the closest spectrum of a sample in a given dataset, and generate the difference spectrum and the corresponding difference in the property. These difference spectra and property differences were then used for PLS calibration. The moment has been employed in spectroscopic fields as a simple and effective "spectral feature characteristic" using just a few scalar values (moments). MC-PLS showed improved prediction performance over PLS for each case. In MC-PLS, the difference spectra generated using the moments were used as explained; therefore, additional detail in spectral variations can be utilized for calibrations. Additionally, the difference in the property was employed as reference data, so that its variation range was smaller when compared with that of the original property. Consequently, the MC-PLS performance could be better since the feature-enhanced spectra were used to model a narrower range of property variations. In the case of the D 10% prediction for naphtha, a non-linear prediction pattern that occurred in conventional PLS was effectively corrected using the MC-PLS method.
The purpose of this study was to determine whether there are any differences in the appearance of tongue-surface structure in different kinds of collagen diseases, because red tongue is well known to be a very important feature of suspected Sjögren's syndrome (Sjs). To clarify some features of the filiform papillae on the dorsal surfaces of the tongues of patients with speculated Sjs, we observed a total of 565 individuals with primary Sjs (n = 24, M/F = 0/24), secondary Sjs (n = 16, M/F = 1/15), possible Sjs (n = 96, M/F = 21/75), collagen diseases (CD) (n = 55, M/F = 15/40; 15 SSc, 10 SLE 10, two MCTD, six dermatomyositis, and 22 others), various cutaneous disorders (n = 324, M/F = 118/206), and healthy controls (HC) (n = 50, M/F = 32/18) by using a dermoscope. The average ages of the patients with Sjs, CD (non-Sjs), cutaneous disorders (non-Sjs/CD), and HC were 56.4 14.8, 55.1 116.4, 51.1 121.2, and 37.1 110.6 years of age, respectively. The filiform papillae were classified into four patterns by their structural characteristics: normal papillae (no abnormality with clear cornified tips) (pattern I, n = 162), slightly rounded papillae with unclear cornified tips (pattern II, n = 239), rounded papillae without cornified tips (pattern III, n = 86), and completely flattened papillae (pattern IV, n = 28). Their patterns were reversely related to the volume of salivary fluid (gum test) (P = 0.046) and the degree of furor coating of the tongue (P = 0.051). Pattern IV is predominant in definitive Sjs (primary and secondary Sjs) (n = 15; 53.6%) with positive anti-SS-A or -B antibody (n = 8) with a specificity of 53.6% and a sensitivity of 36.6%. The present dermoscopic finding that the completely flattened pattern (IV) is predominant in definite Sjs patients may indicate a useful marker for suspicion of Sjs.
We examined autonomic nerve function in patients with systemic scleroderma (SSc) using power spectral analysis of heart rate variability. In the SSc group, both in nighttime (0:00a.m.-5:00a.m.) and daytime (10:00a.m.-5:00p.m.), HF elements, a parasympathetic nerve index, were lower and LF/HF ratios, a sympathetic nerve index, were higher than in the control group. This suggests that in SSc patients, sympathetic nerve activity was increased and parasympathetic nerve activity was decreased throughout the day.
One of the difficulties in laparoscopic gastrectomy is the identification of the feeding artery of the stomach. Recently, 3-dimensional computed tomographic angiography has enabled the noninvasive visualization of arteries surrounding the stomach. Preoperative 3-dimensional computed tomographic angiography may facilitate laparoscopic gastrectomy by obtaining a road map of the arteries of the stomach. Twenty-nine cases of gastric cancer were evaluated using 3-dimensional computed tomographic angiography before surgery. Three-dimensional computed tomographic angiography showed the left gastric artery in 29 patients (100%), the right gastroepiploic artery in 29 patients (100%), the right gastric artery in 24 patients (82.8%), and the left gastroepiploic artery in 21 patients (72.4%). The aberrant hepatic artery was detected in 6 patients, and the variant of the right gastric artery and the variant of the left gastric artery were detected in 5 cases and 1 case, respectively. All laparoscopic gastrectomy procedures were performed successfully without conversion to open surgery. Preoperative 3-dimensional computed tomographic angiography was considered to be useful for laparoscopic gastrectomy.
Periostin was originally identified as an osteoblast-specific factor and is highly expressed in the embryonic periosteum, cardiac valves, placenta, and periodontal ligament as well as in many adult cancerous tissues. To investigate its role during development, we generated mice that lack the periostin gene and replaced the translation start site and first exon with a lacZ reporter gene. Surprisingly, although periostin is widely expressed in many developing organs, periostin-deficient (peri(lacZ)) embryos are grossly normal. Postnatally, however, approximately 14% of the nulls die before weaning and all of the remaining peri(lacZ) nulls are severely growth retarded. Skeletal analysis revealed that trabecular bone in adult homozygous skeletons was sparse, but overall bone growth was unaffected. Furthermore, by 3 months, the nulls develop an early-onset periodontal disease-like phenotype. Unexpectedly, these mice also show a severe incisor enamel defect, although there is no apparent change in ameloblast differentiation. Significantly, placing the peri(lacZ) nulls on a soft diet that alleviated mechanical strain on the periodontal ligament resulted in a partial rescue of both the enamel and periodontal disease-like phenotypes. Combined, these data suggest that a healthy periodontal ligament is required for normal amelogenesis and that periostin is critically required for maintenance of the integrity of the periodontal ligament in response to mechanical stresses.
Periostin was originally isolated as an osteoblast-specific factor that functions as a cell adhesion molecule for preosteoblasts and is thought to be involved in osteoblast recruitment, attachment, and spreading. The protein was renamed "periostin" because of its expression in the periosteum and periodontal ligament, indicating a potential role in bone and maintenance of tooth structure. Periostin has structural similarity to insect fasciclin-I and can be induced by TGF-beta and Bmp2. Because tooth and periodontium development is a well-described genetic model for organogenesis governed by a reciprocal set of epithelial-mesenchymal interactions, thought to be controlled by various TGF-beta superfamily members, we investigated whether periostin is present during tooth morphogenesis. Both periostin mRNA and protein expression were analyzed throughout normal tooth development (embryonic day [E] 9.5-newborn) and within both Bmp4- and Msx2-null embryos. Periostin mRNA is initially present within the E9.5 first branchial arch epithelium and then shifts to underlying ectomesenchyme. Both mRNA and protein are asymmetrically localized to the lingual/palatal and buccal side during the early epithelial-mesenchymal interactions. Periostin is also present in dental papilla cells and within the trans-differentiating odontoblasts during the bell and hard tissue formation stages of tooth development. We suggest that periostin plays multiple roles as a primary responder molecule during tooth development and may be linked to deposition and organization of other extracellular matrix adhesion molecules during maintenance of the adult tooth, particularly at the sites of hard-soft tissue interface.
The chemokine stromal-derived factor-1 (SDF-1, also known as CXCL12) and its receptor CXCR4 have been implicated in homing of stem cells to the bone marrow and the homing of bone marrow-derived cells to sites of injury. Bone marrow cells infiltrate brain and give rise to long-term resident cells following injury. Therefore, SDF-1 and CXCR4 expression patterns in 40 mice were examined relative to the homing of bone marrow-derived cells to sites of ischemic injury using a stroke model. Mice received bone marrow transplants from green fluorescent protein (GFP) transgenic donors and later underwent a temporary middle cerebral artery suture occlusion (MCAo). SDF-1 was associated with blood vessels and cellular profiles by 24 hours through at least 30 days post-MCAo. SDF-1 expression was principally localized to the ischemic penumbra. The majority of SDF-1 expression was associated with reactive astrocytes; much of this was perivascular. GFP+ cells were associated with SDF-1-positive vessels and were also found in the neuropil of regions with increased SDF-1 immunoreactivity. Most vessel-associated GFP+ cells resemble pericytes or perivascular microglia and the majority of the GFP+ cells in the parenchyma displayed characteristics of activated microglial cells. These findings suggest SDF-1 is important in the homing of bone marrow-derived cells, especially monocytes, to areas of ischemic injury.
The purpose of this study is to evaluate the cardiovascular function in patients with incomplete type of SSc (SSSD; Scleroderma Sjögren syndrome associated Spectrum Disorders), SSc (Systemic Scleroderma) and SLE (Systemic Lupus Erythematosus) by using ECG and digital plethysmograph. We also preformed ambulatory ECG monitoring for cases of SSSD and SSc with QT interval prolongation. 1) In ECG findings, ischemic myocardial damage, left ventricular hypertrophy or QT interval prolongation was observed in 33% cases of SSSD and 32% cases of SSc, respectively. In addition, parasympathetic disorders were observed in approximately 20% cases of SSSD, SSc and SLE. 2) Digital plethysmogram findings suggested that the peripheral vascular damage was present in both cases of SSSD and SSc, and its severity was lower in SSSD than SSc. The incidence of abnormal cardiovascular function tests in SSSD was relatively less than that in SSc. The cardiovascular function tests are useful to find cardiovascular abnormalities in these collagen diseases.
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BACKGROUND: The spring ligament has been reported to be composed of the inferior calcaneonavicular and superomedial calcaneonavicular ligaments. We investigated the lower layer of the spring ligament fibrocartilage complex under the fibrocartilaginous surface, identified three distinct structures, and examined the morphology and running patterns of their fiber bundles. METHODS: Forty-eight cadaveric feet were examined. After ablation of the ankle joint and extirpation of the talus, the surface cartilage of the spring ligament fibrocartilage complex was carefully removed with forceps, and the length, width, and thickness of the three components of the complex were measured with calipers. RESULTS: The three components of the spring ligament complex are the superomedial calcaneonavicular ligament, the inferior calcaneonavicular ligament, and a structure that we termed the third ligament, which comprises fibers running from the notch between the calcaneal facets to the navicular tuberosity. CONCLUSIONS: We demonstrated a third component of the spring ligament; this component runs from the notch between the anterior and middle calcaneal facets to the tubercle of the navicular in the lower layer of the spring ligament complex, lying beneath the cartilaginous surface of the complex.
It is unknown whether diabetes mellitus is a risk factor of the hepatocarcinogenesis in patients with chronic hepatitis C. Three hundred eleven anti-hepatitis C virus (HCV) -positive patients who had undergone liver biopsies were studied. Patients with histologically proven cirrhosis or withdrawing within 12 months were excluded. Thus, the remaining 279 patients were followed-up for 65.9 +/- 29.4 months until the occurrence of hepatocellular carcinoma (HCC). During the observation period, HCC developed in 13 patients. Diabetes, age, sex, habitual alcohol intake, history of blood transfusion, serum alpha-fetoprotein level, histological findings, HCV genotype, viral load, and interferon therapy were assessed as potential risk factors. The Cox proportional hazard model identified that diabetes mellitus, histological staging, and age were independently associated with the occurrence of HCC. With multivariate analysis, only diabetes mellitus and age were associated with the occurrence of HCC. Diabetes mellitus may be associated with hepatocarcinogenesis in patients with chronic hepatitis C.
A 25 year-old male, a dairy farmer, had noticed an annular scaly erythema on the left cheek since 3 weeks, and visited a dermatological clinic for the eruption. Diagnosis of tinea faciei was made and he was treated with oral anti-histamine medicine and by topical application of anti-fungal ointment. However, the eruption worsened and enlarged so that he visited the department of dermatology of Kumiai Hospital on October 19, 1997. He was in good general health. Physical examination disclosed papules and pustules with swelling and erythema on the chin and cheeks. The results of routine laboratory investigations were within normal limits except for white blood cell (9,800/mm(3)) and C reactive protein (2+). A small white-yellowish colony was grown on brain heart infusion agar culture of the biopsied specimen of the lower jaw. Histopathological features showed epidermal hyperplasia with elongation of rate ridges and granulomatous changes around hair follicles in the dermis with many mononuclear cells and giant cells, where many positive spores and fine filamentous structures with PAS and Grocott stains were seen. Based on clinical, histopathological and mycological findings, a diagnosis of Trichophyton verrucosum was made. The patient was treated with oral itraconazole (100 mg/day) for two months. There was a good clinical response and no recurrence during three years and six months.