Pfeiffer Type I Syndrome: A Genetically Proven Case Report

ObjectivePfeiffer Syndrome is as rare as Apert syndrome in the Western population. This condition is very rare in the Asian population. At the best of our knowledge this is the first genetically proven case report from Iran. The authors report with a review of literature, the case of a infant with Pfeiffer syndrome, manifested by Lacunar skull, ventriculomegaly, bicoronal craniosynostosis,frontal bossing, shallow orbits, parrot-like nose, umbilical hernia, broad and medially deviated great toes.

Pareto Optimization of a Five-Degree of Freedom Vehicle Vibration Model Using a MultiObjective Uniform-Diversity Genetic Algorithm (MUGA), Engineering

a b s t r a c t In this paper, a new multi-objective uniform-diversity genetic algorithm (MUGA) with a diversity preserving mechanism called the e-elimination algorithm is used for Pareto optimization of a fivedegree of freedom vehicle vibration model considering the five conflicting functions simultaneously. The important conflicting objective functions that have been considered in this work are, namely, seat acceleration, forward tire velocity, rear tire velocity, relative displacement between sprung mass and forward tire and relative displacement between sprung mass and rear tire. Further, different pairs of these objective functions have also been selected for 2-objective optimization processes. The comparison of the obtained results with those in the literature demonstrates the superiority of the results of this work. It is shown that the results of 5-objective optimization include those of 2-objective optimization and, therefore, provide more choices for optimal design of a vehicle vibration model

Effects of feeding frequency on growth in hatchery reared beluga sturgeon (Huso huso)

The effects of feeding frequency on growth rate, food conversion ratio (FCR) and survival in juvenile beluga sturgeon were studied through two phases of rearing. 360 beluga fingerlings with an average weight of 20.51 ±0.33g were reared through a period of 60 days in the first phase and about 180 fingerlings with an average weight of 77.55±1.18g were reared through a period of 95 days in the second phase in fiberglass tanks (500 L) with a water flow rate of 0.2 L/sec under similar conditions of rearing (dissolved oxygen, light, water velocity, etc.). Three different feeding frequencies were used for each phase (3, 5 and 8 times feeding per day) with three replicates. It was evident from the results obtained from the first experimental phase that with an increase in feeding frequency there was an increase in growth rate, weight increase percentage and specific growth rate as well as a decrease in FCR value. However no significant differences were observed in the three group,) studied (P >0.05). Whereas in the second phase of study significant differences (P<0.05) were observed in the parameters studied (length, weight and FCR) in the third study group (with 8 times feeding per day) at the end of study period. We may therefore conclude that growth rate, nutritional uptake and social behavior of each fish species are dependent on its feeding frequency

Rearing Huso huso in earthen ponds and fiberglass tanks

Juvenile Huso huso with average weight of 28.76 plus or minus 0.35 were reared in fiberglass tanks and earthen ponds for 29 weeks in order to evaluate potential of rearing of the fish for meat production purposes. Fish in fiberglass tanks showed weight increase up to 584.17 grams in 200 days and produced 6 kg of biomass per metric square. Survival ratio, food conversion ratio and specific growth rate were measured as 98.4%, 2.21 and 1.41 (% per day) for the fish respectively. During the experiment period, H.huso reared in the earthen ponds reached a weight of 708 grams and produced 1.5 kg of biomass per metric square. The survival rate, food conversion ratio and specific growth rate for the fish cultured in the earthen ponds were 85.7%, and 1.32 and 1.69 (% per day). Based on the results, the fiberglass tanks provided better growth condition in the beginning of the experiment. However, in later stages of rearing the trend was reversed and fish reared in earthen ponds showed more suitable condition although no statistically significant differences between treatments were found (p>0.05)

Molecular mechanisms of vitamin D plus Bisphenol A effects on adipogenesis in human adipose-derived mesenchymal stem cells

Background: Obesity is considered a major health concern and mounting evidence suggests that the exposure to environmental endocrine disruptors, including Bisphenol-A (BPA), may enhance the risk to develop the disease. Moreover, growing documents propose that the vitamin D may contribute to adipogenic signaling and lipid accumulation during adipocyte differentiation. We focused on the molecular mechanism of vitamin D and BPA in human adipose-derived mesenchymal stem cells (hADMSCs) which vitamin D and BPA may influence adipose tissue development and function. Methods: Human adipose-derived mesenchymal stem cells were cultured for 14 days in lipogenic differentiation media containing continuous concentrations of vitamin D plus BPA (0.1 nM or 10 nM). The expression of adipogenic markers including the peroxisome proliferator-activated receptor γ (PPARγ), CCAAT-enhancer-binding protein α (C/EBP α) CCAAT-enhancer-binding protein β (C/EBP β), fatty acid synthase (FASN), lipoprotein lipase (LPL), sterol regulatory element-binding protein-1c (SREBP1c), insulin-induced gene-2 (INSIG2), vitamin D receptor (VDR), estrogen receptor-beta (ER-β), fatty acid-binding protein-4 (FABP4), and glucose transporter-4 (GLUT4) was measured using Quantitative polymerase chain reaction (qPCR) and enzyme-linked immunosorbent assay (ELISA). Lipid accumulation was visualized with staining with Oil Red O. Results: In the morphological assessment of mesenchymal stem cells treated with a concentration of 10 nM vitamin D plus BPA, more lipid accumulations were observed in comparison with the group with 0.1 nM concentration. Treatment of hADMSCs with vitamin D plus BPA (0.1 nM) significantly inhibited the induction of PPARγ, C/EBP β, C/EBP α, and FASN related to adipocyte differentiation and development. However, the exposure of cells to the concentration of 10 nM vitamin D plus BPA induced the expression of these genes associated to the adipogenesis. The remarkable increase in the level of SREBP1c was associated to the suppression of INSIG2 in treated preadipocytes with 10 nM vitamin D plus BPA. Our findings showed that the expression of VDR, ERβ, GLUT4, and FABP4 were upregulated through differentiation with the highest concentrations in 0.1 nM vitamin D plus BPA group for VDR, ERβ, and GLUT4. Conclusions: Vitamin D plus BPA at concentration of 10 nM boosted the adipogenesis during the critical stages of adipocytes development, whereas it seems to inhibit this process at concentration of 0.1 nM. © 2021, The Author(s)

Correction to: 1,25-Dihydroxyvitamin D3 modulates adipogenesis of human adipose-derived mesenchymal stem cells dose-dependently (Nutrition & Metabolism, (2021), 18, 1, (29), 10.1186/s12986-021-00561-4)

Following publication of the original article 1, the authors identified an error in the affiliation of Dr. Mehdi Hedayati. © 2021, The Author(s)

Bisphenol A enhances adipogenic signaling pathways in human mesenchymal stem cells

Background: The endocrine disruptor Bisphenol-A (BPA), has been involved in dysregulating adipose tissue development and increasing the risk of obesity. The objective of this experiment was to investigate whether treatment of human mesenchymal stem cells with BPA could modulate adipogenesis and adipocyte differentiation. Methods: In this experimental study, the human adipose-derived mesenchymal stem cells (hASCs) were cultured for 2 weeks with continuous exposure to 10- 10 M or 10- 8 M concentrations of BPA. The extent of triglyceride accumulation was visualized by Oil Red O staining. To evaluate BPA effect on the expression levels of key adipogenic trascripotion factors and proteins, we used Quantitative reverse transcriptase-polymerase chain reaction (qRT-PCR) and ELISA. Results: The results presented a dose-dependent triglyceride accumulation in treated cells with BPA. Additionally, we observed that BPA induced transcription of the Peroxisome proliferator-activated receptor-gamma (PPARγ), CCAAT-enhancer-binding protein-alpha (C/EBPα), CCAAT-enhancer-binding protein-beta (C/EBPβ), sterol regulatory element-binding protein-1c (SREBP1c), Fatty acid synthase (FASN), and lipoprotein lipase (LPL); BPA suppressed the expression of Fatty acid binding protein-4 (FABP4) and Estrogen receptor-beta (ERβ). Conclusions: Our findings supported the hypothesis that BPA enhances adipogenic differentiation thereby may play a role in development of obesity and dysregulation of metabolic homoeostasis. © 2020 The Author(s)

Long-term outcome of posterior spinal fusion for the correction of adolescent idiopathic scoliosis

Background: Adolescent idiopathic scoliosis (AIS) is the most common form of idiopathic scoliosis, and surgery is considered as one of the therapeutic options. However, it is associated with a variety of irreversible complications, in spite of the benefits it provides. Here, we evaluated the long-term outcome of posterior spinal fusion (PSF) of AIS to shed more light on the consequences of this surgery. Methods: In a cross-sectional study, a total of 42 AIS patients who underwent PSF surgery were radiographically and clinically inspected for the potential post-operative complications. Radiographic assessments included the device failure, union status, and vertebral tilt below the site of fusion. Clinical outcomes were evaluated using the Oswestry disability index (ODI) and visual analogue scale (VAS). Results: The mean age of the surgery was 14.4 ± 5.1 years. The mean follow-up of the patients was 5.6 ± 3.2 years. Complete union was observed in all patients, and no device failure was noticed. Pre- and post-operative vertebral tilt below the site of fusion were 11.12° ± 7.92° and 6.21° ± 5.73°, respectively (p < 0.001). The mean post-operative ODI was 16.7 ± 9.8. The mean post-operative VAS was 2.1 ± 0.7. ODI value was positively correlated with follow-up periods (p = 0.04, r = 0.471). New degenerative disc disease (DDD) was observed in 6 out of 37 (16) patients. Conclusion: In spite of the efficacy and safety of PSF surgery of AIS, it might result in irreversible complications such as DDD. Moreover, the amount of post-operative disability might increase over the time and should be discussed with the patients. © 2018 The Author(s)