Fibrous dysplasia (FD) is a non-malignant condition caused by post-zygotic, activating mutations of the GNAS gene that results in inhibition of the differentiation and proliferation of bone-forming ...stromal cells and leads to the replacement of normal bone and marrow by fibrous tissue and woven bone. The phenotype is variable and may be isolated to a single skeletal site or multiple sites and sometimes is associated with extraskeletal manifestations in the skin and/or endocrine organs (McCune-Albright syndrome). The clinical behavior and progression of FD may also vary, thereby making the management of this condition difficult with few established clinical guidelines. This paper provides a clinically-focused comprehensive description of craniofacial FD, its natural progression, the components of the diagnostic evaluation and the multi-disciplinary management, and considerations for future research.
Purpose: The forced mid-expiratory flow (FEF25-75%) value is a potentially sensitive marker of obstructive peripheral airflow. We aimed to assess whether FEF25-75% can be an early predictor of ...chronic obstructive pulmonary disease (COPD). Patients and Methods: Between July 1, 2007 and June 31, 2009, we identified 3624 patients who underwent a pulmonary function test (PFT) in Gangnam Severance Hospital. We selected 307 patients aged over 40 years without COPD who had normal PFT results at baseline and who had follow-up PFT records more than 1 year later. A FEF25-75% z-score less than − 0.8435 was considered low. We defined COPD as a forced expiratory volume in one second/forced vital capacity value of less than 0.7 before July 31, 2019. Results: Among 307 patients, 91 (29.6%) had low FEF25-75% at baseline. After 10 years, the incidence rate of COPD in the low FEF25-75% group was significantly higher than that in the normal FEF25-75% group (41.8% vs 7.4%; P-value< 0.001). The Cox proportional hazard model showed that age (hazard ratio HR 1.09; P-value< 0.001), smoking status (occasional smoker HR, 4.59; P-value< 0.001 and long-term smoker HR, 2.18; P-value=0.023), and low FEF25-75% (HR, 3.31; P-value< 0.001) were predictive factors for the development of COPD. Conclusion: The FEF25-75% value in patients with normal lung function is a useful predictor for the development of COPD. We should carefully monitor patients who present with low FEF25-75% values, even if they have normal lung function.
Muscle wasting and chronic inflammation are predominant features of patients with COPD. Systemic inflammation is associated with an accelerated decline in lung function. In this study, the prevalence ...of sarcopenia and the relationships between sarcopenia and systemic inflammations in patients with stable COPD were investigated.
In a cross-sectional design, muscle strength and muscle mass were measured by handgrip strength (HGS) and bioelectrical impedance analysis in 80 patients with stable COPD. Patients (≥40 years old) diagnosed with COPD were recruited from outpatient clinics, and then COPD stages were classified. Sarcopenia was defined as the presence of both low muscle strength (by HGS) and low muscle mass (skeletal muscle mass index SMMI). Levels of circulating inflammatory biomarkers (IL-6 and high-sensitivity TNFα hsTNFα) were measured.
Sarcopenia was prevalent in 20 (25%) patients. Patients with sarcopenia were older, had lower body mass index, and a higher percentage of cardiovascular diseases. In addition, they had significantly higher modified Medical Research Council scores and lower 6-minute walk distance than those without sarcopenia. HGS was significantly correlated with age, modified Medical Research Council score, and COPD Assessment Test scores. Both HGS and SMMI had associations with IL-6 and hsTNFα (HGS,
=-0.35,
=0.002; SMMI,
=-0.246,
=0.044) level. In multivariate analysis, old age, lower body mass index, presence of cardiovascular comorbidities, and higher hsTNFα levels were significant determinants for sarcopenia in patients with stable COPD.
Sarcopenia is very common in patients with stable COPD, and is associated with more severe dyspnea-scale scores and lower exercise tolerance. Systemic inflammation could be an important contributor to sarcopenia in the stable COPD population.
In patients with acute abdomen, especially during pregnancy, it is difficult to suspect myoma torsion initially due to its rarity. However, when these patients have a solid mass in the pelvis, ...torsion of uterine fibroids or adnexa and large infarct inside leiomyoma are the main diagnoses that must be differentiated. We report a case of uterine myoma torsion mimicking ovarian torsion in the second trimester of pregnancy that was successfully managed by laparoscopic myomectomy. A 31-year-old pregnant woman with severe right lower abdominal pain at 21 + 2 weeks of gestation was transferred. On ultrasonography, the appendix appeared normal but a solid mass (7.0×4.0 cm) containing a cystic portion was observed on the right side of the uterus. The mass had no blood flow and tenderness appeared on the transducer pressure. Pelvic magnetic resonance imaging revealed a 7 cm edematous mass with low signal intensity on T2-weighted image, instead of a normal-looking right ovary. These findings led to suspicion of right ovarian torsion. One day after admission, the patient's symptoms worsened with acute elevation in C-reactive protein (CRP) (4.55 mg/dL) and neutrophile-to-lymphocyte ratio (NLR) (10.1), despite the use of analgesics and tocolytics, and uterine contractions were not controlled. Emergency surgery was performed, and the final diagnosis was a uterine leiomyoma torsion. Laparoscopic myomectomy was successfully performed, and symptoms were relieved after operation. She had full term vaginal delivery without complication. The differential diagnosis between myoma torsion and ovarian torsion is challenging in patients with acute abdomen during pregnancy. To exclude ovarian torsion, a comparison with an asymptomatic contralateral normal ovary will be very helpful. When fibroids are found in mothers with an acute abdomen, follow-up of NLR and CRP is related to preterm labor and can be a reference point for considering surgery.
A 3-year-old castrated male ferret was suspected to have lymphoma based on a markedly enlarged mesenteric lymph node. Hodgkin’s-like lymphoma (HLL) was suspected because numerous Reed-Sternberg-like ...cells were observed in the fine-needle aspiration biopsy of the mesenteric lymph node. In the post-mortem histopathology, neoplastic cells were invasively proliferated not only in the mesenteric lymph nodes, but also in the liver and spleen. Immunohistochemically, most neoplastic cells were positive for vimentin and BLA-36, weakly positive for CD20, and negative for Pax5, CD79a, CD3, CD45, and Iba-1, demonstrating similarity to human Hodgkin’s lymphoma (HL). In addition, a prominent programmed cell death protein 1 (PD-1) expression was observed at the sites showing malignant tumour proliferation. HLL in non-human beings can be morphologically and immunophenotypically similar to human HL, but the prognosis and clinical outcome may differ. This is the first study analysing the PD-1 expression in a ferret, suggesting that PD-1 can be a novel diagnostic and prognostic factor in a similar manner to humans.
Single to multiple pulse packs of bipolar, alternating current radiofrequency (RF) oscillations have been used for various medical purposes using invasive microneedle electrodes. This study was ...designed to evaluate the effects of pulse widths and cycles of RF pulse packs on immediate and delayed thermal tissue reactions in in vivo rat skin.
RF energy at the frequency of 1 MHz and power of 70 W was delivered at each experimental setting into in vivo rat skin at 1.5-mm microneedle penetration, and then, tissue samples were obtained after 1 h and 3, 7, 14, and 21 days and histologically analyzed.
A single-pulse-pack RF treatment generated coagulative necrosis zones in the dermal peri-electrode area and zones of non-necrotic thermal reactions in the dermal inter-electrode area. Multiple pulse-pack, RF-treated rat skin specimens revealed that the number and size of peri-electrode coagulative necrosis were markedly decreased by increasing the number of pulse packs and accordingly decreasing the conduction time of each pulse pack. The microscopic changes in RF-induced non-necrotic thermal reaction in the inter-electrode area were more remarkable in specimens treated with RF of 7 or 10 pulse packs than in specimens treated with RF of 1-4 pulse packs.
The gated delivery of multiple RF pulse packs using a bipolar, alternating current, 1-MHz RF system using insulated microneedle electrodes efficiently generates non-necrotic thermal tissue reactions over the upper, mid, and deep dermis and subcutaneous fat in the inter-electrode areas.
Vitamin C, as an antioxidant, has recently been suggested to provide protection against COPD; however, only few national cohort studies have investigated these effects. We aimed to confirm the ...protective effects of vitamin C against COPD in Korean patients.
We analyzed the data of 3,283 adults aged ≥40 years (representing 23,541,704 subjects) who underwent pulmonary function tests and responded to questionnaires on smoking history and vitamin C intake, with stratification variables and sampling weight designated by the Korea 2012 National Health and Nutrition Examination Survey.
Among all the subjects, 512 (representing 3,459,679 subjects; 15.6%) were diagnosed as having COPD based on pulmonary function test results. Male gender, old age, residence in suburban/rural regions, low household income, low educational level, an occupation in agriculture or fisheries, and heavy smoking were significantly associated with COPD. Low intake of nutrients, including potassium, vitamin A, carotene, retinol, and vitamin C, was significantly associated with COPD. The prevalence of COPD in heavy smokers with the lowest quartile (Q1, <48.50 mg; 63.0%) and low-middle quartile (Q2, 48.50-84.38 mg; 56.4%) of vitamin C intake was significantly higher than that in subjects with the high-middle quartile (Q3, 84.38-141.63 mg; 29.5%) and highest quartile (Q4, >141.63 mg; 32.6%) of vitamin C intake (
=0.015). In multivariate analysis, male gender, old age, heavy smoking, and a low intake of vitamin C were significant independent risk factors for COPD. A significant reduction of 76.7% in COPD risk was observed with a Q3 vitamin C intake compared to Q1 vitamin C intake (odds ratio, 0.233; 95% confidence interval, 0.094-0.576) in heavy smokers.
This large-scale national study suggests that dietary vitamin C provides protection against COPD, independent of smoking history, in the general Korean population.
Aim: To evaluate the effects of a combination probiotic on symptoms and colonic transit in patients with irritable bowel syndrome (IBS) and significant bloating.
Methods: Forty‐eight patients with ...Rome II IBS were randomized in a parallel group, double‐blind design to placebo or VSL# 3 twice daily (31 patients received 4 weeks and 17 patients 8 weeks of treatment). Pre‐ and post‐treatment colonic transit measurements were performed using scintigraphy with 111In charcoal. Symptoms were summarized as an average daily score for the entire period of treatment and separately for the first 4 weeks of treatment. Weekly satisfactory relief of abdominal bloating was assessed.
Results: Treatment with VSL# 3 was associated with reduced flatulence over the entire treatment period (placebo 39.5 ± 2.6 vs VSL# 3 29.7 ± 2.6, P = 0.011); similarly, during the first 4 weeks of treatment, flatulence scores were reduced (placebo 40.1 ± 2.5 vs VSL# 3 30.8 ± 2.5, P = 0.014). Proportions of responders for satisfactory relief of bloating, stool‐related symptoms, abdominal pain and bloating scores were not different. Colonic transit was retarded with VSL# 3 relative to placebo (colon geometric center 2.27 ± 0.20 vs 2.83 ± 0.19, P = 0.05 respectively).
Conclusion: VSL# 3 reduces flatulence scores and retards colonic transit without altering bowel function in patients with IBS and bloating.
We developed three-dimensional electrospun silk fibroin (ESF) scaffolds with controllable pore size. The purpose of this study was to evaluate ESF scaffolds with pores (P-ESF) for bone regeneration ...via in vitro and in vivo studies, with a comparison to a commercially available porous three-dimensional polylactic acid (PLA) scaffold. P-ESF supported significantly higher proliferation and alkaline phosphatase activity of osteoblasts than PLA in vitro (p < 0.05). Moreover, higher expression levels of activated adhesion-related proteins, including focal adhesion kinase, were observed in the P-ESF than in PLA, as confirmed by western blot analyses. Microcomputed tomography revealed that 78.30% of the original bone volume was attained in the P-ESF implantation group at 7 weeks after critical bone defect formation in rat calvaria. Comparatively, the PLA implantation group showed only 49.31%. Histological evaluation also showed new bone tissue formation upon P-ESF implantation. Taken together, the P-ESF scaffold may be a good bone substitute for bone regeneration.
Background: Glioma stem cells (GSCs) have been reported to contribute to tumor initiation and relapse, therapy resistance, and intra-tumoral heterogeneity of glioblastoma multiforme. Therefore, ...inhibiting GSCs presents a critical therapeutic tactic to suppress the aggressiveness of tumors. Methods: In this study, we examined the effects of 7β-22 dihydroxyhopane (AP 18), isolated from the sub-Antarctic lichen, Pseudocyphellaria freycinetii. The cytotoxic effect of AP 18 and its effects on cell proliferation were assessed by alamarBlue assay and 5-ethynyl-2′-deoxyuridine (EdU) assay. Real-time confluence analysis was performed with a Celloger automatic live cell imaging system. Western Blotting and 3-D optical diffraction tomography (ODT) imaging were performed to determine whether apoptosis was triggered by AP 18. A Limiting dilution assay and qRT-PCR were performed to investigate the impact of AP 18 on GSC stemness. Results: AP 18 significantly reduced GSCs viability and proliferation, inducing programmed cell death identified by Annexin V/PI staining and had effects on morphologic features determined by 3-D ODT. Interestingly, treatment with AP 18 suppressed stemness features. Conclusion: Taken together, our results suggest that AP 18 might be a potential therapeutic agent to target GSCs.
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