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This paper presents an adaptive load frequency control technique in both of isolated microgrid power system and two interconnected microgrids (MGs). A modified Jaya optimization method is used to make online tuning of the area controller. The Balloon Effect (BE) modification is designed to increase the sensitivity of the Jaya algorithm to both of the system load disturbance and parameters variations. In Jaya with BE technique, the objective function (OF) is affected by the on-time system problems. Communication delay time is considered in the dynamic model of interconnected MGs. System with proposed adaptive control method has been tested in several scenarios including step and variable load demand and parameters variations. Simulation results using Matlab software show that the proposed adaptive controller tuned by Jaya + BE technique provides better performance of frequency control than other methods such as conventional controller or adaptive one tuned by classical Jaya algorithm.

The Pervasive Computing is a new paradigm in information technology. Over the past two decades, many of Pervasive Computing and Internet of Things applications were developed to facilitate our life. The current study will mainly focus on pervasive computing applications at work settings and empirically validate the proposed model. The Mobile Business Intelligence application was chosen as an initial stage technology of pervasive computing. The study developed a proposed model which included the extended Unified Theory of Acceptance and Use of Technology (UTAUT) model along with the Trust and Perceived Convenience variables to investigate behavioral intention to use pervasive computing applications. Using Questionnaire survey, data was collected from 277 employees in some companies in Egypt. The correlation, regression and Structural Equation Modeling (SEM) were used for analysis. The results showed that effort expectancy explained 17.9% of the variations in Perceived Convenience, 28.9% of the variations in Performance Expectancy were justified by Perceived Convenience. The results also indicated that Performance Expectancy, Effort Expectancy, Social Influence, Facilitating Conditions, Perceived Convenience and Trust explained 46.7% of the variations in behavioral intention to use pervasive computing applications.

Desbuquois dysplasia type 1 (DBQD1) is a very rare skeletal dysplasia characterized by growth retardation, short stature, distinct hand features, and a characteristic radiological monkey wrench appearance at the proximal femur. We report on 2unrelated Egyptian patients having the characteristic features of DBQD1 with different expressivity. Patient 1 presented at the age of 45 days with respiratory distress, short limbs, faltering growth, and distinctive facies while patient 2 presented at 5 years of age with short stature and hypospadias. The 2 patients shared radiological features suggestive of DBQD1. Whole-exome sequencing revealed a homozygous frameshift mutation in the CANT1 gene (NM_001159772.1:c.277_278delCT; p.Leu93ValfsTer89) in patient 1 and a homozygous missense mutation (NM_138793.4:c.898C>T; p.Arg300Cys) in patient 2. Phenotypic variability and variable expressivity of DBQD was evident in our patients. Hypoplastic scrotum and hypospadias were additional unreported associated findings, thus expanding the phenotypic spectrum of the disorder. We reviewed the main features of skeletal dysplasias exhibiting similar radiological manifestations for differential diagnosis. We suggest that the variable severity in both patients could be due to the nature of the CANT1 gene mutations which necessitates the molecular study of more cases for phenotype-genotype correlations.

Here we report a consanguineous Egyptian family with two siblings presented with congenital microcephaly, early-onset epileptic encephalopathy, feeding difficulties, and early lethality. The condition was initially diagnosed as molybdenum cofactor deficiency as the brain imaging for one of them showed brain edema and intracranial hemorrhage in addition to the hypoplastic corpus callosum, vermis hypoplasia, and small-sized pons. Subsequently, whole exome sequencing identified a novel homozygous missense variant in exon 4 of ASNS gene c.397_398GT > CA (p.Val133Gln) confirming the diagnosis of asparagine synthetase deficiency syndrome. No discernible alternative cause for the intracranial hemorrhage was found. Our patient is the second to show asparagine synthetase deficiency and intracranial hemorrhage, thus confirming the involvement of ASNS gene. As such, it is important to consider asparagine synthetase deficiency syndrome in patients with microcephaly, brain edema, and neonatal intracranial hemorrhage.

Blepharophimosis-ptosis-intellectual disability syndrome (BPID) is an extremely rare recognizable blepharophimosis intellectual disability syndrome (BID). It is caused by biallelic variants in the UBE3B gene with only 24 patients described worldwide. Herein, we report on the clinical, brain imaging and molecular findings of additional nine patients from six unrelated Egyptian families. Patients presented with the characteristic features of the syndrome including blepharophimosis, ptosis, upslanted palpebral fissures with epicanthic folds, hypertelorism, long philtrum, high arched palate, micrognathia, microcephaly, and intellectual disability. Other findings were congenital heart disease (5 patients), talipes equinovarus (5 patients), genital anomalies (5 patients), autistic features (4 patients), cleft palate (2 patients), hearing loss (2 patients), and renal anomalies (1 patient). New or rarely reported findings were spherophakia, subvalvular aortic stenosis and hypoplastic nails, and terminal phalanges. Brain MRI, performed for 7 patients, showed hypogenesis or almost complete agenesis of corpus callosum. Genetic studies revealed five novel homozygous UBE3B variants. Of them, the c.1076G>A (p.W359*) was found in three patients from two unrelated families who shared similar haplotype suggesting a likely founder effect. Our results strengthen the clinical, dysmorphic, and brain imaging characteristic of this unique type of BID and extend the mutational spectrum associated with the disorder.

Cisplatin and oxaliplatin are widely used anticancer drugs. Their use is restricted by their dose-limiting side effects: nephrotoxicity and neurotoxicity, respectively. Cerium oxide nanoparticles (CONPs) are promising antioxidant and anti-inflammatory agent. To test the possible ameliorative impact of CONPs on the toxic effect of cisplatin and oxaliplatin in male albino rats. Forty eight rats were divided into 6 groups: control group, CONPs group, cisplatin group, cisplatin and CONPs group, oxaliplatin group, and oxaliplatin and CONPs group. After 4 weeks, serum urea and creatinine, renal tissue level of interleukin 10 (IL10), and total antioxidant (TAO) were measured in control, CONPs, and cisplatin groups. The other kidney was used for histopathological and immunohistochemical studies. The right sciatic nerves and the lumbar spinal cord of rats from control, CONPs, and oxaliplatin groups were used for immunohistochemical evaluations of nitrotyrosine, myelin basic protein (MBP), and glial fibrillary acidic protein (GFAP). Cisplatin significantly increased serum urea and creatinine levels, significantly decreased the kidney level of IL10 and TAO with marked tubular necrosis, hemorrhage and renal damage. Also, it decreased IL10 immunohistochemical expression. CONPs significantly decreased the serum urea and creatinine level and increased IL10 and TAO with lower renal damage and strong IL10 expression compared with cisplatin group. Oxaliplatin significantly decreased MBP immunoreactivity and increased nitrotyrosine immunoreactivity. In the lumbar spinal cord, GFAP immunoreactivity was significantly increased. CONPs significantly increased MBP and decreased nitrotyrosine immunoreactivity. GFAP immunoreactivity was significantly decreased. CONPs ameliorated cisplatin and oxaliplatin primary toxicities through anti-inflammatory and antioxidant characteristics.

AIM: To study the changes in choroidal thickness in central serous chorioretinopathy (CSCR) over a 3mo follow-up using spectral domain optical coherence tomography (SD-OCT). METHODS: This prospective study included 60 eyes, both eyes of 20 patients (mean age: 33.65±5.24y) with classic acute unilateral central serous chorioretinopathy and normal fellow eye and 20 eyes as healthy controls. Fluorescein angiography and OCT were done. The subfoveal choroidal thickness (SFCT), central macular thickness (CMT), 1000 μm temporal and nasal to the centre of the fovea and the subretinal fluid were measured. RESULTS: There was a statistically significant difference in SFCT among the three groups at the three different locations. SFCT in eyes with CSCR (372.40±34.39 μm) was significantly greater than that in each of the unaffected fellow eyes (302.10±8.9 μm) and control eyes (279.80±14.49 μm) at the base line and after 3mo follow-up. The mean CMT in CSCR was 317±141.86 μm, with a statistically significant positive correlation between SFCT and CMT. CONCLUSION: The increase in the choroidal thickness at different locations as well as hyper-dilated and hyper-permeable vessels known as “pachychoroid” seems to play an important role in a broad spectrum of diseases that includes central serous chorioretinopathy.

AimsCoronary artery disease (CAD) is the leading cause of death worldwide in both men and women. Accordingly, we retrospectively reviewed the effects of various risk factors on coronary angiographic outcomes.Methods and ResultsData were collected from the catheter lab through Tripoli university hospital records, whereas the team reviewed clinical data and coronary artery diagrams for one year from 01/04/2019 to 31/03/2020. In our study, the total number of cases was 666; 401 male and 265 female, ranging in age between 27 and 91 years. Considering the data, a significantly increased incidence of coronary artery disease (CAD) among the male who smokes, and who were less than 60 years of age. Furthermore, in the present study, the total number of normal patients was predominantly female. The most common risk factors for women were diabetes mellitus (DM), hypertension (HTN) (12%,13% respectively). While the men share the significant effects of smoking on coronary angiography (C. Angio) findings (40.52%), and most of them underwent a percutaneous coronary intervention (PCI).ConclusionsIn our study, there was evidence that CAD is a prevalent disease among the middle-aged populations with male gender preference. The risk factors including diabetes, hypertension, and smoking are the most contributing factors for the developing CAD in Libya.

SARS-CoV-2 infection is widely spread over people, from youth to the elderly. Vaccination against SARS-CoV-2 is an important preventive measurement to help end the SARS-CoV-2 pandemic. From 30 April to 15 July, we collected the number of people infected with SARS-COV-2 and the mortality rate from daily reports issued by the National Center for Disease Control of Libya (NCDC). Approximately 445000 doses have been administered in Libya since 10 April, and 5 thousand doses are now being administered during this period on a daily basis. To estimate the rate of breakthrough vaccine infection of the SARS-COV-2 in Libya. We found that one dose of the three different types of vaccines had decreased the virus transmission across people and mortality rate until 10 weeks after the first dose. This study highlights the dramatic success of the early months of the nation’s coronavirus vaccines rollout.

Hepatocellular carcinoma (HCC) is on the rise worldwide, and its incidence in diabetic patients is two to three times that of non-diabetics. Current therapeutic options fail to provide considerable survival benefits to patients with HCC. There is a strong possibility that the FDA-approved antidiabetic combination of empagliflozin and metformin could show complementary effects to control HCC progression. However, their multitarget effects have not yet been studied on HCC development. Therefore, the present study aims to evaluate the antitumorigenic activity of this combination in non-diabetic mice with diethylnitrosamine-induced HCC. Empagliflozin/metformin combination prolonged survival and improved histological features of mice livers. Additionally, Empagliflozin/metformin showed anti-inflammatory potential and relieved oxidative stress. On the one hand these effects are likely attributed to the ability of metformin to inactivate NF-κB in an AMPK-dependent mechanism and on the other hand to the ability of the empagliflozin to inhibit the MAPKs, p38 and ERK1/2. Empagliflozin also showed a less robust effect on AMPK than that of metformin. Moreover, empagliflozin enhanced the autophagy inducing activity of metformin. Furthermore, empagliflozin/metformin exhibited increased apoptotic potential. Consequently, empagliflozin augmented the antitumorigenic function of metformin by exerting better control of angiogenesis, and metastasis. To conclude, our findings suggest empagliflozin as an ideal adjunct to metformin for the inhibition of HCC progression. In addition, since the incidence of hypoglycemia is minimal due to insulin-independent mechanism of action of both treatments, empagliflozin/metformin could be a promising therapeutic modality for the management of diabetic patients with HCC; and even non diabetic ones.

WIPI2 is a member of the human WIPI protein family (seven-bladed b-propeller proteins binding phosphatidylinositols, PROPPINs), which play a pivotal role in autophagy and has been implicated in the pathogenesis of several neurological conditions. The homozygous WIPI2 variant c.745G>A; p.(Val249Met) (NM_015610.4) has recently been associated with a neurodevelopmental disorder in a single family. Using exome sequencing and Sanger segregation analysis, here, two novel homozygous WIPI2 variants [c.551T>G; p.(Val184Gly) and c.724C>T; p.(Arg242Trp) (NM_015610.4)] were identified in four individuals of two consanguineous families. Additionally, follow-up clinical data were sought from the previously reported family. Three non-ambulant affected siblings of the first family harbouring the p.(Val184Gly) missense variant presented with microcephaly, profound global developmental delay/intellectual disability, refractory infantile/childhood-onset epilepsy, progressive tetraplegia with joint contractures and dyskinesia. In contrast, the proband of the second family carrying the p.(Arg242Trp) missense variant, similar to the initially reported WIPI2 cases, presented with a milder phenotype, encompassing moderate intellectual disability, speech and visual impairment, autistic features, and an ataxic gait. Brain MR imaging in five patients showed prominent white matter involvement with a global reduction in volume, posterior corpus callosum hypoplasia, abnormal dentate nuclei and hypoplasia of the inferior cerebellar vermis. To investigate the functional impact of these novel WIPI2 variants, we overexpressed both in WIPI2-knockout HEK293A cells. In comparison to wildtype, expression of the Val166Gly WIPI2b mutant resulted in a deficient rescue of LC3 lipidation whereas Arg224Trp mutant increased LC3 lipidation, in line with the previously reported Val231Met variant. These findings support a dysregulation of the early steps of the autophagy pathway. Collectively, our findings provide evidence that biallelic WIPI2 variants cause a neurodevelopmental disorder of variable severity and disease course. Our report expands the clinical spectrum and establishes WIPI2-related disorder as a congenital disorders of autophagy., Maroofian et al. reported two novel homozygous WIPI2 variants [c.551T>G; p.(Val184Gly) and c.724C>T; p.(Arg242Trp) (NM_015610.4)] in four individuals of two consanguineous families with neurodevelopmental disorder and brain abnormalities. Expression of the Val184Gly mutant in WIPI2-knockout HEK293A cells resulted in a deficient LC3 lipidation rescue whereas Arg242Trp mutant increased LC3 lipidation., Graphical Abstract Graphical Abstract

Empagliflozin and metformin are widely used for the treatment of type 2 diabetes. These drugs showed marked anti-inflammatory effects in different animal models via enhancing AMPK activity. Yet, the protective anti-inflammatory effects of their combination against ulcerative colitis have not been previously investigated. The current study aimed to explore the potential of empagliflozin/metformin combination to mitigate the DSS-induced rat colitis model. The modulating effects of empagliflozin and metformin on the AMPK/mTOR/NLRP3 axis and T cell polarization were delineated. In this study, distal colons were examined for macroscopic and microscopic pathological alterations. ELISA, qRT-PCR, and immunohistochemistry techniques were applied to detect proteins and cytokines involved in AMPK/mTOR/NLRP3 axis and T Cell polarization. Oral administration of empagliflozin (10 mg/kg/day) and metformin (200 mg/kg/day) combination alleviated colitis as revealed by the reduced disease activity index, macroscopic damage index, colon weight/length ratio, and histopathologic scoring values. Interestingly, empagliflozin/metformin combination significantly enhanced AMPK phosphorylation and depressed mTOR and NLRP3 expression leading to a subsequent reduction in caspase-1 cleavage and inhibition of several inflammatory cytokines, including IL-1β, and IL-18. Reduced mTOR expression and reduced IL-6 levels led to a reduction in Th17 cell polarization and maintenance. Together, the current study reveals that the protective effects of empagliflozin and metformin against DSS-induced colitis are fundamentally mediated via enhancing AMPK phosphorylation. Since adult humans with diabetes mellitus are at greater risk for developing inflammatory bowel diseases, clinical application of empagliflozin/metformin combination represents a novel therapeutic approach for treating diabetic patients with ulcerative colitis.

Background and objectives The X-linked bleeding disorder, hemophilia A, is caused by defective production of factor VIII (FVIII). Hemophilic patients require regular FVIII infusions. Recombinant factor replacement poses the safest line of therapy. However, its main drawbacks are high expenses and the higher liability for formation of inhibitors. Recent studies confirmed the ability of bone marrow-derived stem cells to secrete FVIII. This study aims to generate bioscaffold from decellularized liver and subsequently seed it with trans-differentiated human stem cells into hepatic-like cells. This scaffold can then be implanted intraperitoneally or subcutaneously to provide FVIII. Methods After generation of the bioscaffold, seeding of discoid scaffolds with trans-differentiated human hepatocyte-like cells was performed. Then, the generated organoid was implanted into peritoneal cavity or subcutaneous tissue of experimental rats. Results Serum human FVIII was significantly increased in rats subjected to subcutaneous implantation compared intraperitoneal implantation. Immunostaining for detecting Cytokeratin 19 and human anti-globulin confirmed the presence of mature human hepatocytes that were significantly increased in subcutaneous implanted scaffold compared to the intraperitoneal one. Conclusion Implantation of decellularized bioscaffold seeded with trans-differentiated stem cells in rats was successful to establish production of FVIII. Subcutaneous implantation showed higher FVIII levels than intraperitoneal implantation.

Introduction  Intracranial pressure (ICP) monitoring is a vital element in the diagnosis and management of several neurological disorders, such as head injury, hydrocephalus, subarachnoid hemorrhage, and intracranial hematoma.  The optic nerve sheath is in direct contact with the subarachnoid space. This relationship provides the physiological foundation for using the optic nerve sheath to assess intracranial pressure. Since the optic nerve sheath is loosely attached to the nerve, the subarachnoid space in this region more distensible, and appears bulbous on ultrasound. Dilation of the optic nerve sheath, in contrast to papilloedema, occurs faster, and can be a sign of increased intracranial pressure.  The advancements of ultrasound modalities enabled researchers to enhance optic nerve sheath diameter (ONSD) measurements, with further research that concentrated on determining the best distance behind the globe to measure OSND. According to a 1996 study, ONSD increased by up to 60% at 3 mm behind the globe compared to only 35% at 10 mm. Later studies showed that the measuring should be performed 3 mm behind the globe, since ultrasound contrast is superior at this depth with a linear probe, allowing for reproducibility.

Sjögren-Larsson syndrome (SLS) is a rare neurocutaneous disorder characterized by congenital ichthyosis, spastic diplegia and intellectual disability. It is an inborn error of lipid metabolism caused by biallelic mutations in the ALDH3A2 gene encoding the fatty aldehyde dehydrogenase that plays a pivotal role in metabolism of long-chain aliphatic aldehydes and alcohols. In this report, we describe the clinical, neuro-radiological and molecular findings of 35 patients with SLS. All patients shared the typical clinical manifestations of SLS including spasticity, ichthyosis and intellectual disability. Brain MRI demonstrated deep while matter affection in all patients that varied in severity. Mutational analysis of the ALDH3A2 gene revealed 16 distinct mutations including 11 previously unreported ones. Three mutations (p.S365L, p.R9* and p.G400R) were recurrent in our patients with frequencies ranging from 12 to 24%. Interestingly, patients carrying the two new mutations p.R9* and p.G400R shared similar haplotypes suggesting possible founder effects in our population. In conclusion, we present a large cohort of patients from the same ethnicity with the characteristic clinical and brain imaging findings of SLS but with variable inter and intra familial severity and expressivity. We also identified many novel and founder ALDH3A2 mutations thus expanding the mutational spectrum of the disorder.

Background and study aim: Diabetes is a self managed disease and the participation of the individual, health care team and his family is very essential in managing the disease and preventing its complications.The aim of study was to evaluate the effectiveness of a self-management support program on improving Knowledge and practices of patients with diabetes mellitus at Zagazig University Hospital. Subjects and Methods: A quasi experimental research design was used in this study. A purposive sample of 70 patients with diabetic foot patients were randomly assigned to either the control (n= 35) or the study (n= 35) group using matched criteria of foot ulcer history and foot problems. Three tools were used for collection of data, patient's assessment questionnaire, questionnaire about patient knowledge about diabetes mellitus and foot care and self reported practice questionnaire. Results: The study finding revealed that sixty (60.0%) of patients in the study and control groups were males with more than 40 years of age with Mean±SD (52.7±8.8 and 53.5±7.8 respectively), there was statistical significant relationship between patient knowledge and practice where more than three quarters (89.6%) of patients who had unsatisfactory knowledge had inadequate practice. Also there was positive correlation between foot care score and knowledge score and practice score. Conclusion: The self-management support program showed an improvement in patients' knowledge which reflected an improvement on their practice and diabetic foot care behaviors in post phase. It is Recommended to increase awareness and prevent the diabetic foot and its complications by apply nursing intervention programs regarding importance of foot care and to impart knowledge on foot care, use media to motivate diabetic patient for monthly diabetic clinic visiting, use media to increase awareness about the diabetic foot, continues follow up for patients who is suffering from the diabetic foot and increase distributing free handbooks that are specific for diabetes in the public places.

The tremendous and astonishing technological progress in the modern era in all different fields has ledto a lack of human mobility and thus a lack of physical and physiological competence, which made himvulnerable to many diseases called hypokinetic diseases such as cardiovascular disease, obesity, lower backpain, diabetes, and high blood pressure. The Study Problem: Diabetes is one of the most common andwidespread diseases today, and failure to control this disease will lead to many complications, includingcardiovascular disease, high-fat content, in addition to limb muscle atrophy, high blood pressure andperipheral nerve inflammation, which sometimes leads to leg amputation. The cause of death and this is thesource of the problem. The Study Objectives: Knowing the blood sugar level of non-athletic patients aged40-55 years before the program. Designing an athletic program for diabetics aged 40-55 years who are nonathletes for the research group. To know the effect of the program on blood sugar, HDL cholesterol, and PCVblood viscosity for the research group. Identify the effect of the program on the nerve signals received fromthe upper and lower extremities of the research group. The study community the human field: people withdiabetes who are 40-55 years old who are not athletes. from 1/3/2020 to 1/7/2020. Hospitals and specializedcenters for treating this disease and the Physiotherapy Center for Sports Medicine in Egypt. Results: thevalues of the blood sugar test indicate the significance of the differences between the pre-and post-tests andin favor of the post-test, considering that the drop in blood sugar level is a positive case. blood HDL ratiotest indicates the significance of the differences between the pre-and post-tests and in favor of the post-test,considering that the drop in blood sugar level is a positive case. blood HDL ratio test considering the highlevel of HDL cholesterol is a positive case. Conclusions: 1-The diet and exercise program has a positiveeffect on low blood sugar levels. 2-The sports and nutritional program have a positive effect on the levelof high-density lipoprotein (HDL) cholesterol in the blood. 3-The sports and nutritional program have apositive effect on blood viscosity (PCV). 4-The sports and nutritional program have a positive effect on thenerve signals coming from the upper and lower extremities.

We evaluated the role of initial laparoscopy and optimized approach in cases of unilateral nonpalpable testis. Seventy-four patients with nonpalpable testes were presented. We excluded 9 patients, with palpable testes under anesthesia. Laparoscopy was offered to 65 patients. Contralateral testis hypertrophy with length ≥ 1.8 cm was confirmed in 47 patients. Ultrasound results were available for 35 patients. Age ranged from 1 to 10 years. Of 65 nonpalpable testes, right side comprised 23 (35.4%) and the left 42 (64.6%). Laparoscopy revealed intra-abdominal testis in 18 patients (27.7%), blind-ending vessels and vas in 8 (12.3%), and vas and vessels traversing the internal ring in 39 (60%). Treatment of intra-abdominal testes included Fowler–Stephens orchiopexy in 7 patients, laparoscopic orchiopexy in 9, and laparoscopic orchiectomy in 2. In 8 patients with blind-ending vas and vessels, laparoscopy was terminated. In 39 patients with vas and vessels traversing the internal ring, scrotal exploration was performed in 36 patients with closed internal ring and inguinal exploration in 3 with open internal ring. Vanished testes were present in 43/47(91.5%) of patients with contralateral testis hypertrophy ≥ 1.8 cm. Ultrasound detected the presence of a testis in only 4/11 (36.3%) of patients, although it could not identify vanished testis. Initial laparoscopy should be retained as one of the standard treatment for nonpalpable testis. It was the only required modality in 26 patients (40%) and optimized further treatment in 39 patients (60%) by evaluation of the condition of the internal ring.

Background: Injury of the anterior cruciate ligament (ACL) is the most frequently seen ligamentous injury of the knee joint. Diagnosis is based on history, physical examination and MRI findings and a definitive diagnosis is confirmed with arthroscopy. Many tests are used for diagnosis of ACL with different sensitivity and specificity. Objectives: To evaluate lever sign test as a diagnostic tool for ACL injury compared the other 3 tests (anterior drawer, Lachman, and pivot shift test) when performed before anesthesia and under anesthesia. Patients and Methods: The study included 100 patients in the age of 18 to 45 years complaining of knee instability and confirmed as ACL injury by MRI. The 4 special tests for ACL (the Lachman, the anterior drawer, the pivot shift test and the lever sign test) were performed on the injured knee and the contralateral non-injured knee as a control both preanesthesia and under anesthesia. The results were compared to the arthroscopic findings. Results: Lever sign test sensitivity is 72% on examination before anesthesia and 80% on examination under anesthesia with specificity 92%. The most sensitive test Preanesthesia was the Lachman test 88% and postanesthesia was the pivot shift test 89%. The most specefic test was highest for the pivot-shift test 100%. Conclusion: Lever sign test is a simple test which can be routinely used in evaluation of ACL function in both acute and chronic knee injury. The test is weak diagnostic tool in partial ACL injury as it uses pure translational displacement as its assessment method and does not assess the rotational component.

Biallelic mutations in the SLC30A10 gene cause an inborn error of Mn metabolism characterized by hypermanganesemia, polycythemia, early-onset dystonia, and liver cirrhosis (HMDPC). To date, only 14 families from various ethnic groups have been reported. Here, we describe 10 patients from 7 unrelated Egyptian families with HMDPC. Markedly elevated blood Mn levels, the characteristic basal ganglia hyperintensity on T1-weighted images, and variable degrees of extrapyramidal manifestations with or without liver disease were cardinal features in all patients. Eight patients presented with striking early diseased onset (≤2 years). Unexpectedly, early hepatic involvement before the neurological regression was noted in 3 patients. Mutational analysis of SLC30A10 gene revealed 6 novel homozygous mutations (c.77T > C (p.Leu26Pro), c.90C > G (p.Tyr30*), c.119A > C (p.Asp40Ala), c.122_124delCCT (p.Ser41del), c.780_782delCAT (p.Iso260del) and c.957 + 1G > C). Treatment using 2,3 dimercaptosuccinic acid as a manganese chelating agent showed satisfactory results with improvement of biochemical markers, hepatic manifestations and relative amelioration of the neurological symptoms. Our findings present a large cohort of patients with HMDPC from same ethnic group. The majority of our patients showed severe and early presentation with clear phenotypic variability among sibship. Moreover, we extend the phenotypic and mutational spectrum and emphasize the importance of early diagnosis and treatment of this potentially fatal disorder.

Background: mitochondrial appropriate function depends on ideal mitochondrial translocation machinery, of which translocase of outer mitochondrial membrane (TOMM) have a vital role in normal functioning cell. Any disturbance in the translocation machinery leads to either tumorigenesis or apoptosis. Aim of the work: This study aimed to investigate the expression of TOMM34 in renal cell carcinoma (RCC). TOMM34 expression was assessed in 42 samples of RCC patients using immunohistochemistry technique. Results: immunohistochemical staining revealed significant accumulation of TOMM34 protein in RCC cases compared to their corresponding non-cancerous renal tissues. TOMM34 protein expression was significantly associated with age and gender categories in contrast to the other clinicopathological features. Conclusion: according to protein expression level this study demonstrated that TOMM34 is a marker of poor outcome in RCC.

Aim Metformin and empagliflozin combined therapy may have complementary effects that go beyond the well-recognized targets of their monotherapy through AMPK activation. Therefore, the current study was designed to investigate for the first time the hepatoprotective effects of such combination therapy in the carbon tetrachloride (CCl4)-induced hepatic fibrosis model in mice. Materials and methods Determination of liver enzymes and the liver content of oxidative stress parameters, and hydroxyproline were performed biochemically. ELISA was performed to measure PDGF-BB, TNF-α, TGF-β, TIMP-1, AMPK, p-mTOR, NF-κB P65 binding activity, p38 MAPKα, JNK1/2 and ERK1/2. Real-time qPCR was conducted to determine Col1a1 and α-SMA. In addition, histopathological examination using H&E and Masson's trichrome stain were performed for determination of histopathological changes. Key findings Empagliflozin inhibited the activation of p38 MAPK and ERK1/2 and exhibited a weak AMPKα stimulation. On the other hand, metformin exerted a more robust stimulatory action on the AMPKα that was accompanied by a notable decrease in the NF-κB nuclear binding activity and a decline in the p-mTOR levels. Nevertheless, the effect of metformin on MAPK kinases was insignificant. Our results revealed that blunting p38 MAPKα and ERK1/2 activities by empagliflozin enhanced the antifibrotic effect of metformin and augmented its AMPK-induced NF-κB inactivation. Significance As diabetes is one of the most common risk factors for liver fibrosis, the use of antidiabetic drugs is expected to improve therapeutic outcome. Therefore, metformin/empagliflozin combined therapy could be promising in preventing hepatic inflammation and fibrosis via exhibiting complementary effects particularly in diabetic patients.

Background Fitness trackers allow users to collect, manage, track, and monitor fitness-related activities, such as distance walked, calorie intake, sleep quality, and heart rate. Fitness trackers have become increasingly popular in the past decade. One in five Americans use a device or an app to track their fitness-related activities. These devices generate massive and important data that could help physicians make better assessments of their patients’ health if shared with health providers. This ultimately could lead to better health outcomes and perhaps even lower costs for patients. However, sharing personal fitness information with health care providers has drawbacks, mainly related to the risk of privacy loss and information misuse. Objective This study investigates the influence of granting users granular privacy control on their willingness to share fitness information. Methods The study used 270 valid responses collected from Mtrurkers through Amazon Mechanical Turk (MTurk). Participants were randomly assigned to one of two groups. The conceptual model was tested using structural equation modeling (SEM). The dependent variable was the intention to share fitness information. The independent variables were perceived risk, perceived benefits, and trust in the system. Results SEM explained about 60% of the variance in the dependent variable. Three of the four hypotheses were supported. Perceived risk and trust in the system had a significant relationship with the dependent variable, while trust in the system was not significant. Conclusions The findings show that people are willing to share their fitness information if they have granular privacy control. This study has practical and theoretical implications. It integrates communication privacy management (CPM) theory with the privacy calculus model.

Background Hyperphosphatasia with mental retardation syndrome (HPMRS) is caused by recessive mutations in genes involved in the glycosylphosphatidylinsitol pathway, including PGAP3. Materials and Methods We describe 10 patients from 8 Egyptian families presenting with developmental delay, severe intellectual disability, distinct facial dysmorphism and increased alkaline phosphatase. Sanger sequencing of PGAP3 was performed. Results Eight patients had cleft palate, 4 had postnatal microcephaly and 5 had seizures. Neuro-imaging findings showed thin corpus callosum in 9 patients, mild ventriculomegaly in 3 patients and variable degrees of cerebellar vermis hypoplasia in 4 patients, a finding not previously reported in patients with HPMRS. Additional manifestations included double row teeth, hypogenitalism and congenital heart disease. Biallelic loss of function mutations in the PGAP3 gene were detected in all patients. Nine patients were homozygous for the c.402dupC (p.M135Hfs*28) mutation strongly suggesting a founder effect. On the other hand, 1 patient had a novel mutation, c.817_820delGACT (p.D273Sfs*37). Conclusion This is the largest series of patients with HPMRS from same ethnic group. Our results reinforce the distinct clinical and facial features of PGAP3-related HPMRS which are the clue for targeted genetic testing. Moreover, we present additional unreported clinical and neuro-imaging findings and a novel mutation thus expanding the phenotypic and mutational spectrum of this rare disorder.

Background Neoadjuvant chemotherapy (NAC) has clinically important outcome. Early evaluation of the treatment response is important to avoid unnecessary therapy in non-responders. MRI is the most sensitive imaging for monitoring NAC response. Aim of this study Is to test the ability of DWI to detect early response to NAC. Results The study was performed on 20 patients, proved pathologically to have invasive breast cancer. All patients underwent breast MRI before initiation of NAC, after one cycle and after completion of the NAC protocol. Regarding the mass size, results showed no significant change in maximum mass diameters occur after the 1st NAC cycle. Absolute value of the ADC show increase all along the treatment course which has no significant correlation with the pathological response. The relative increase ADC more than 20% calculated from subtraction of the pre-treatment ADC mean value from that after the first NAC, subdividing the result on the pre-treatment ADC mean value and multiply it by 100 reflected significantly on the pathological response (p value of 0.011). Conclusion ADC value can predict responder from non-responder as early as after the first cycle of chemotherapy.

Lenz-Majewski syndrome (LMS) is an extremely rare type of cutis laxa caused by dominant mutations in PTDSS1 gene. We report an Egyptian patient who presented with cutis laxa, brachydactyly, and progeroid features. LMS syndrome was suspected and a previously reported de novo heterozygous missense mutation (c.284G > T, p.R95L) in PTDSS1 was identified. To the best of our knowledge, nine molecularly proven patients with LMS from different ethnicities have been reported. Our patient is the first report from the Middle East and the tenth molecularly proven patient reported to date. His clinical features were in accordance with LMS syndrome. In addition, his hands X-ray images showed hypoplastic or absent middle and proximal phalanges but sparing the thumbs. This hand patterning was similarly observed before. Further, he had relatively large and convex fingernails. Our report highlights this unique hand patterning and suggests these signs should be considered among the diagnostic criteria of LMS. Further reports of patients with PTDSS1 mutations are necessary to further elucidate the detailed clinical features of LMS syndrome.

The innovation and evolution of technologies in smartphone industry has enabled users to efficiently achieve many tasks including utilizing search engines for instant information retrieval anytime and anywhere. Nonetheless, some users choose not to use these smartphone features including search engines to seek information. This study explores the factors that impact the likelihood of information seeking via smartphones. Privacy concern was found to be one of the main factors influencing the likelihood of seeking information. Android users were more likely to seek information compared to iPhone users, possibly due to the differences in the features of the operating systems of these phones. Motivation to seek information captured by technology ownership increases the likelihood of information seeking. The diversity of social network connections also plays a significant in information seeking behavior of the users.

Background Phishing is a cybercrime in which the attackers usually impersonate a trusted source. The attackers usually send an email that contains a link that allows them to steal the receiver’s personal information. In the United States, phishing is the number one cybercrime by victim count according to the Federal Bureau of Investigation’s 2019 internet crime report. Several studies investigated ways to increase awareness and improve employees’ resistance to phishing attacks. However, in 2019, successful phishing attacks continued to rise at a high rate Objective The objective of this study was to investigate the influence of personality-based antecedents on phishing susceptibility in a health care context. Methods Survey data were collected from participants through Amazon Mechanical Turk to test a proposed conceptual model using structural equation modeling. Results A total of 200 participants took part. Health concerns, disposition to trust, and risk-taking propensity yielded higher phishing susceptibility. This highlights the important of personality-based factors in phishing attacks. In addition, females had a higher phishing susceptibility than male participants Conclusions While previous studies used health concerns as a motivator for contexts such as sharing personal health records with providers, this study shed light on the danger of higher health concerns in enabling the number one cybercrime.

Background Chronic recurrent multifocal osteomyelitis (CRMO) is an uncommon inflammatory disease mostly affects the metaphyses of long bones. It can be distinguished from osteomyelitis by multifocality and recurrence. Hyperphosphatemic familial tumoral calcinosis (HFTC) is a rare genetic disorder characterised by increased re-absorption of phosphate through the renal proximal tubule leading to increased phosphate concentration and deposition of calcified deposits in cutaneous and subcutaneous tissues, as well as some visceral organs. HFTC is inherited in an autosomal recessive manner and is caused by mutations in three different genes, FGF23, GALNT3 and KLOTHO. CRMO has been associated with some chronic inflammatory diseases such as inflammatory bowel disease, palmoplantar pustulosis and SAPHO syndrome. The association of CRMO and HFTC is extremely rare and only three patients have been described so far in the literature. Objectives To report the clinical, radiological and molecular findings of two sibs with CRMO associated with HFTC. Methods In this report, we present two siblings; offspring of consanguineous parents. They presented with spontaneous bony pains not responding to NSAID and later on, that developed tender hard masses. There were no similarly affected family members, and they had a non-affected sibling. Clinical, laboratory, pathological and radiological examination was performed. Mutational analyses of the FGF23, GALNT3 and KLOTHO genes was carried out by Sanger sequencing of the entire coding region of each gene. Results Laboratory results including blood cultures and sensitivities were normal, apart from mildly elevated ESR. Serum calcium, 25(OH) vitamin D, renal functions, albumin, alkaline phosphatase, parathormone hormone, and phosphorous were normal apart from hyperphoshatemia in both siblings. Initial x-rays revealed lytic lesions with a sclerotic margin. Follow up x-rays showed healing with sclerosis and hyperostosis. After developing the hard masses, x-rays showed calcified masses. Resection pathological analysis revealed non-neoplastic inflammatory bone growth with prominent periosteal and bone proliferation, it was free from any granulomatous or malignant changes. We suggested a provisional diagnosis of CRMO associated with HFTC. Molecular studies confirmed the diagnosis by identifying a known pathogenic mutation in the donor splice site of exon 8 of the GALNT3 gene, c.1524+1G>A. The mutation was found in the homozygous form in the two sibs and both parents were heterozygous. Conclusions This study documents the first Egyptian family clinically diagnosed with CRMO associated with HFTC and confirmed by molecular studies, with the identification of a splice mutation in the GALNT3 gene. Acknowledgements This work was funded by the STDF grant number 5253. Disclosure of Interest None declared

Type I diabetes (TID) is generally assumed to be caused by an immune associated, if not directly immune-mediated, destruction of pancreatic β-cells. In patients with long-term diabetes, the pancreas lacks insulin-producing cells and the residual β-cells are unable to regenerate. Patients with TID are subjected to a lifelong insulin therapy which shows risks of hypoglycemia, suboptimal control and ketosis. In this study, we investigated the potential role of vildagliptin (Vilda) alone or in combination with pioglitazone (Pio), as treatment regimens for TID using streptozotocin (STZ)-induced TID model in rats. Daily oral administration of Vilda (5 mg/kg) alone or in combination with Pio (20 mg/kg) for 7 weeks significantly reduced blood glucose levels and HbA1c. It increased serum insulin levels and decreased serum glucagon. It also showed a strong antioxidant activity. Immunohistochemical analysis showed a marked improvement in β-cells in treated groups when compared with the diabetic group, which appeared in the normal cellular and architecture restoration of β-cells in the islets of Langerhans. Vilda alone or in combination with Pio has the ability to improve the overall glycemic control in type I diabetic rats and may be considered a hopeful and effective remedy for TID.

Deep Learning is an artificial intelligence function that imitates the mechanisms of the human mind in processing records and developing shapes to be used in selection construction. The objective of the paper is to improve the performance of the deep learning using a proposed algorithm called RFHTMC. This proposed algorithm is a merged version from Random Forest and HTM Cortical Learning Algorithm. The methodology for improving the performance of Deep Learning depends on the concept of minimizing the mean absolute percentage error which is an indication of the high performance of the forecast procedure. In addition to the overlap duty cycle which its high percentage is an indication of the speed of the processing operation of the classifier. The outcomes depict that the proposed set of rules reduces the absolute percent errors by using half of the value. And increase the percentage of the overlap duty cycle with 15%.

Health information exchanges (HIEs) are multisided platforms that facilitate the sharing of patient health information (PHI) between providers and payers across organizations within a region, community or hospital system. The benefits of HIEs to payers and providers include lower cost, faster services, and better health outcome. However, most HIEs have configured the patient healthcare consent process to give all providers who sign up with the exchange access to PHI for all consenting patients, leaving no control to patients in customized what information to share and with who. This research investigates the impact of granting greater control to patients in sharing their personal health information on consent rates and making them active participants in the HIEs system. This research utilizes a randomized experimental survey design study. The study uses responses from 388 participants and structural equation modeling (SEM) to test the conceptual model. The main findings of this research include that patients consent rate increases significantly when greater control in sharing PHI is offered to the patient. In addition, greater control reduces the negative impact of privacy concern on the intention to consent. Similarly, trust in healthcare professionals leads to higher consent when greater control is offered to the patient. Thus, greater control empowers the role of trust in engaging patients and sustaining HIEs. The paper makes a theoretical contribution to research by extending the unified theory of acceptance and use of technology (UTAUT) model. The findings impact practice by providing insights that will help sustain HIEs.

Background: The causes for thousands of individually rare recessive diseases have been discovered since the adoption of next generation sequencing (NGS). Following molecular diagnosis in a child, families could use this information to make informed decisions in planning future pregnancies. However, there are no population-level studies to measure the effect of applying this knowledge on recurrence risk. Methods: In a retrospective study involving 1172 families with a recessive pediatric brain disease (rPBD) that underwent NGS-based molecular diagnosis, prenatal diagnosis was offered to families seeking to prevent recurrence through fetal genotyping. Pregnancies that were carried to term were assessed for the health of child and mother. Findings: Between 2010 and 2016, 526 families received a molecular diagnosis following NGS, 91 families returned to the clinic with 101 subsequent pregnancies, and 84 opted for fetal genotyping. Of 59 fetuses that genotyped negative and were carried to term, all were unaffected at follow-up. Of 24 that genotyped positive, 16 were terminated, reducing the disease recurrence from the expected 25% to the observed 12% ([95% CI 0·04 to 0·20], p=0·011). The remaining 8 pregnancies were carried to term, and all demonstrated disease concordant with their affected sibling(s). Interpretation: Identification of causes through NGS, coupled with prenatal fetal genotyping in subsequent pregnancies, can empower families to make informed decisions and reduce recessive disease recurrence. Funding Statement: NIH R01NS048453, R01NS052455, UL1TR001442 of CTSA and HHMI. Declaration of Interests: All authors declare no competing interests. Ethics Approval Statement: This study was approved at the host institutions of the respective study sites. The University of California San Diego IRB approved recruitment of subjects for NGS and return of research results to the referring physician and family. The National Research Centre in Cairo approved the study for confirmation of research results, fetal genotyping and genetic counseling for families. Amniocentesis and eTOP utilized standard procedural consent according to local practice.

This is the first Egyptian study with detailed clinical and orodental evaluation of eight patients with pycnodysostosis and identification of four mutations in CTSK gene with two novel ones and a founder effect. Pycnodysostosis is a rare autosomal recessive skeletal dysplasia due to mutations in the CTSK gene encoding for cathepsin K, a lysosomal cysteine protease. We report on the clinical, orodental, radiological, and molecular findings of eight patients, from seven unrelated Egyptian families with pycnodysostosis. All patients were offspring of consanguineous parents and presented with the typical clinical picture of the disorder including short stature, delayed closure of fontanels, hypoplastic premaxilla, obtuse mandibular angle, and drum stick terminal phalanges with dysplastic nails. Their radiological findings showed increased bone density, acro-osteolysis, and open cranial sutures. Mutational analysis of CTSK gene revealed four distinct homozygous missense mutations including two novel ones, c.164A>C (p. K55T) and c.433G>A (p.V145M). The c.164A>C (p. K55T) mutation was recurrent in three unrelated patients who also shared similar haplotype, suggesting a founder effect. Our findings expand the mutational spectrum of CTSK gene and emphasize the importance of full clinical examination of all body systems including thorough orodental evaluation in patients with pycnodysostosis.

Swarm robots are characterized by its ability to perform a number of operations distributed in order to reach a single goal. So they need to communicate continuously and effectively. However, one of the most common obstacles facing these robots is the environment in which they work. It is possible that the common goal is widely distributed and in difficult places where communication is difficult. So the aim of the research is to use a modern technology that enables the squadrons to communicate at long distances. WiMAX changed into deliberate because of its numerous merits, collectively with its functionality to kilometers. Four factors were then analyzed to measure performance through data transmission through audio, video and e-mail as well as searching through browsing. The consequences indicated that performance reached excessive degrees beneath particular obstacles. WiMax generation demonstrates its capability to enhance the performance of both upload response time and download response time and lowering both of the traffic received and traffic sent between robots.

Medical Molecular Genetics, Division of HumanGenetics and Genome Research, Center of ScientificExcellence of Human Genetics, National ResearchCentre, Cairo, EgyptCorrespondence to Mohamed S. Abdel-Hamid, MSc,Department of Medical Molecular Genetics, Division ofHuman Genetics and Genome Research, Center ofScientific Excellence of Human Genetics, NationalResearch Centre, El-Buhouth St, Dokki, 12311 Cairo,EgyptTel: +20 1001667730; fax: +20 233 370 931;e-mail: mohamadnrc@hotmail.comReceived 10 October 2014Accepted 23 October 2014Middle East Journal of Medical Genetics2015, 4:7–12

Various endoscopic instruments used in treating renal stones have been used in managing bladder calculi. Our aim is to evaluate the use of transurethral ureteroscopic pneumatic cystolithotripsy for the management of large bladder calculi.In a retrospective study conducted between May 2005 and July 2011, 53 patients with solitary bladder stones were subjected to our technique, transurethral ureteroscopic pneumatic cystolithotripsy. The mean patient age was 54.3 years. The mean stone size was 4.8 cm. Diagnostic cystoscopy was performed first. A semirigid ureteroscope with a pneumatic probe was introduced through the cystoscope sheath, and pneumatic lithotripsy was performed. The cystoscope sheath works as a draining channel, allowing bladder evacuation. The bladder collapses over the stone and, subsequently, prevents stone migration, which shortens the lithotripsy's duration. Stone immobilization allows the transmission of full pneumatic power to the stone. The stone fragments were removed through periodic bladder irrigation using an Ellick evacuation-irrigation system, and a 16F Foley catheter was placed at the end of the procedure.Our technique was effective in all cases, including stone-free bladders. No surgical complications were detected. The mean operative time was 83 ± 21.0 minutes, which is comparable to that of standard management. The mean duration of lithotripsy and evacuation was 29.7 ± 18.4 minutes. In the postoperative period, patients were followed up for 18 months, with no urethral stricture being reported in any case.The transurethral ureteroscopic pneumatic cystolithotripsy procedure is a safe technique for the management of large bladder calculi. It allows the bladder to collapse over the stone, leading to stone immobilization and, subsequently, decreasing lithotripsy duration. Our procedure is associated with a minimal chance of postoperative urethral injury because all stone fragments are evacuated through the cystoscope sheath, without contact with the urethral urothelium.

Middle East Journal of Medical Genetics 2014, 3:37–41 Temtamy preaxial brachydactyly syndrome (TPBS; OMIM: 605282) is a rare autosomalrecessive skeletal disorder. Loss-of-function mutations in the human CHSY1 gene were found to cause TPBS. The syndrome has been reported and homozygous or compound heterozygous mutations have been confirmed in 16 patients worldwide from consanguineous families. Seven reported patients were Egyptians. Here we report on an additional Egyptian patient with TPBS, an offspring of consanguineous parents. Phenotype analysis and molecular studies were performed on the family. Clinical examination of the studied patient confirmed the facial dysmorphic features and typical digital anomalies. Molecular studies revealed a novel homozygous nonsense mutation in exon 2 of the CHSY1 gene, c.613G4T (p.E205X). The mutation was found in the heterozygous state in both parents and was not found in 200 normal chromosomes of Egyptian origin by PCR and restriction fragment length polymorphism (PCR-RFLP) analysis. This case report adds a new Egyptian patient with TPBS with a novel mutation in the CHSY1 gene. The total number of reported cases has now reached 17, including eight Egyptian patients. The study confirms our previous conclusion that the syndrome is an easily recognizable dysmorphic syndrome and that the rarity of worldwide reports could be due to underdiagnosis.

BACKGROUND Abdominal aortic aneurysm (AAA) is associated with a prothrombotic diathesis that may increase the risk of cardiovascular events. This diathesis is exacerbated in the short term by open aneurysm repair (OAR) and endovascular aneurysm repair (EVAR). However, the effect of EVAR and OAR on coagulation and fibrinolysis in the medium and long term is poorly understood. The purpose of this study was to investigate the medium-term effects of EVAR and OAR on thrombin generation, neutralization, and fibrinolysis. METHODS Prothrombin fragment (PF)1+2, thrombin antithrombin (TAT) complex, plasminogen activator inhibitor (PAI) activity, and tissue-plasminogen activator (t-PA) antigen were measured in eight age-matched controls (AMCs), 29 patients with AAA immediately before (preoperatively) and 12 months after EVAR (post-EVAR), and in 11 patients at a mean of 16 months after OAR (post-OAR). RESULTS Preoperatively, PF1+2 levels were significantly higher in patients with AAAs than in AMC. PF1+2 levels post-EVAR and post-OAR were significantly lower than preoperative values and similar to AMC. There was no significant difference in TAT, PAI, or t-PA between AMC, AAA preoperatively, and post-EVAR. Post-OAR, PAI activity was significantly higher than in preoperative patients. CONCLUSIONS AAA is associated with increased thrombin generation without upregulation of fibrinolysis. The prothrombotic, hypofibrinolytic diathesis observed in patients with AAA returns toward normal in the medium term after EVAR and OAR, although there is a trend toward decreased fibrinolysis post-OAR.

Background Endovascular aneurysm repair (EVAR) is associated with an improved perioperative mortality compared to open surgical repair. This benefit may reflect reduced incidence of microvascular and macrovascular thrombotic complications after EVAR. Purpose The purpose of this study was to review and compare the effects of abdominal aortic aneurysm (AAA), open surgical repair, and EVAR on coagulation, fibrinolysis, and platelet activation. Methods A MEDLINE (1966-2010) and Cochrane library search for articles relating to the effects of AAA, open surgical repair, and EVAR on hemostasis was performed utilizing and cross-linking terms such as clotting, fibrinolysis, AAA, EVAR, and open surgical repair. Studies with a small cohort of patients (less than 7) or in which values of assessed biomarkers were not included were rejected. Results AAA is associated with increased thrombin generation, activity, and fibrin turnover as evidenced by increased plasma levels of thrombin-antithrombin III-complex (TAT), activated protein C-protein C inhibitor (APC-PCI), fibrin-monomer-fibrinogen (FM-F), F1+2, fibrinogen, and D-dimer. The extent of hemostatic derangement correlates with the volume of intraluminal thrombus. This procoagulant state is exaggerated in the immediate perioperative period after both open surgical repair and EVAR, but is attenuated at medium-term follow-up although not normalized. Conclusion The resultant prothrombotic diathesis after open surgical repair and EVAR may account for the high level of perioperative thrombotic complications.

ObjectiveTo examine serum cytokine/chemokine profiles before and 6 months after endovascular repair (EVAR) of abdominal aortic aneurysm (AAA) and to determine whether they correlate with serum inflammatory activity using an in vitro model of leukocyte recruitment.MethodsSerum IL-1-α, IL-1β, IL-4, IL-6, IL-8, IL-10, IFN-γ, IP-10, MCP-1, TNF-α, and TNF-β were measured using a cytometry-based immunoassay. To test patient serum for direct inflammatory activity, human endothelial cells (EC) were stimulated with 30% patient serum for 24 hours. To test patient serum for the ability to prime EC for inflammatory responses, EC were incubated with 30% patient serum for 24 hours, followed by stimulation with low-dose (5 U/mL) TNF for 4 hours. Under both regimens of stimulation, the degree of EC activation was assessed by assaying neutrophil recruitment in a flow-based model.ResultsOnly IL-1α (67.9 ± 10.4 pg/mL vs 41.9 ± 7.4 pg/mL) and IL-8 (51.5 ± 5.1 vs 32.6 ± 4.7 pg/mL) changed significantly after surgery. Patient serum alone was unable to activate EC. However, serum from both time points could prime EC responses to low-dose TNF. Thus, after priming with preoperative serum, EC stimulated with TNF could recruit 76.7 ± 12.0 neutrophils/mm2 into the subendothelial cell space. Post-EVAR serum was significantly less effective (44.4 ± 10.2 neutrophils/mm2). This reduction in neutrophil recruitment correlated with reduced IL-1α in post-EVAR serum. The addition of a neutralizing antibody against IL-1α to pre-EVAR serum inhibited EC priming and neutrophil recruitment, strongly implying that this cytokine was the priming agent.ConclusionEVAR reduces serum IL-1α and its inflammatory activity in patient serum. IL-1α is, therefore, implicated in the molecular pathology of AAAs and may have potential as a clinically useful biomarker.Clinical RelevanceContinuing uncertainty exists over which patients will benefit from abdominal aortic aneurysm (AAA) repair in addition to medical therapy as well as which repair method, open or endovascular, is the most clinically and cost-effective. Greater understanding of AAA development and progression, and the subsequent identification of biomarkers for rupture risk, are likely to result in better selection of patients for intervention and provide better measures of successful repair. These novel data implicate interleukin (IL)-1α in the molecular pathology of AAA. Further, they suggest that IL-1α may be a predictive biomarker for AAA size as well as outcome after endovascular aneurysm repair.

Aim Occlusion or severe stenosis of pedal and plantar arteries limits surgical options for critical limb ischaemia (CLI). Below-the-ankle (BTA) angioplasty is potentially useful as an adjunct to proximal angioplasty. In this study, the feasibility and outcome of this procedure were explored, as they have not been evaluated previously. Methods Patients' demographics, indications, procedures and outcomes were recorded. Outcomes were determined by technical success, primary patency, limb salvage and amputation-free survival (AFS) rates. Results Between 2004 and 2008, 42 cases of BTA angioplasty were performed for 39 patients. Forty cases (95.2%) had CLI. Technical success was achieved in 88% of cases. At 6, 12 and 24 months, AFS was 70.7%, 60.9% and 57.1%, limb salvage was 84.9%, 81.9% and 81.9% and patient survival was 83.3%, 73.8% and 67.3, respectively. Seven major amputations (16.6%) were performed, four of which had failed angioplasty. Two patients required re-intervention. Univariate analysis showed insulin-dependent diabetics, occlusive lesions, failure of angioplasty and state of the run off to be the predictors of limb loss. Conclusions BTA angioplasty for pedal and plantar arterial occlusive disease is technically feasible. It has good medium-term clinical outcome and limb salvage in a group of patients with poor surgical options.

Aim of the study: To compare efficacy of tramadol injection as an opioid analgesia versus epidural analgesia on governing labor pain, progress and outcomes (maternal and fetal). Duration and place of study: Department of Obstetrics and Gynecology Zagazig University from November 2011 to December 2013. Methodology: One hundred fifty pregnant women primigravida had gestational age between 37 to 41 weeks (confirmed by early ultrasound) with vertex presentation without any risk factors, in established labor (cervical dilation >3cm with regular uterine contraction) were included in this study divided into two groups , tramadol group (A) and epidural group (B) . Subjects of group (A) received 1mg/kg tramadol intramuscular bolus and 100mg in 500 ml Ringer lactate at the rate of 8-24drops/min. and those of group (B) received 0.125% bupivacaine with fentanyl 5 mcg/ml 10- 15 mL to be repeated hourly throughout labor and continued until birth. Pain relief was assessed by visual analogue scale of 10 scores ranging from no pain to unbearable pain) before the administration of the drug at 0, 5min., 10min., 15min., 30min., 1 h then every 2 h until full dilatation. Maternal and neonatal out comes were determined. Results: Total number of patients was one hundred fifty, all were primigravida.The mean age of group A was 22.81 ± 1.89 years and 23.23 ± 1.28 in group B; Mode ofdelivery was spontaneous vaginal in 64 patients (85.3%) in group A and 53 patients (70.6%) in group B Instrumental vaginal delivery in 6 patients (8%) of group A and 13 Patients (17.3%) of group B. Cesarean section in 5 patients (6.6%) of group A and 9 patients12% of group B. At one minute majority of the babies of group A had mean Apgar score 8.7 ± 0. 52 versus 8.65 ± 4.1 at group B. At 5 minute, 9.40 ± 0.33 versus 9.54 ± 0.23. There were no significant differences.In the tramadol group, pain relief was excellent in 13.3%, good in 30.6% and average in 54.6% versus 29.3%, 48% and 22.6% in epidural group. In both the groups there was no significant effect on duration of 1st and 3rd stage of labor but Second stage of labor was prolonged in the epidural group. Conclusion: Epidural anesthesia and tramadol provided excellent pain relief in majority of the patients. Since, Tramadol administration is easy could be considered as a good alternative to epidural analgesia in lower source settings of the developing nations.

We investigated the association between c-3279TG and unconjugated neonatal hyperbilirubinemia. In all, 141 neonates were recruited; 63 had hyperbilirubinemia necessitating treatment, and 78 with bilirubin 7 mg/dl served as the control group. The frequency of occurrence of c-3279T G allele was significantly higher in the hyperbilirubinemic (49.2%) than in the control group (25.6%). The homozygous (p = 0.001, OR = 17.7 and CI = 3.9-79.3) rather than the heterozygous state (p = 0.3, OR = 0.7 and CI = 0.3-1.6) was associated with hyperbilirubinemia. Among the hyperbilirubinemic group, comparison between the three genotypes, homozygous mutation, heterozygous mutation and the normal allele, revealed that the former was associated with significantly higher mean peak total serum bilirubin [mean ± standard deviation (SD): 33.7 ± 8.2, 26.9 ± 2.8 and 21± 2.7, respectively, p-value = 0.0001], higher bilirubin/albumin ratio (p = 0.000) and a longer duration of hospital stay (p = 0.001). Homozygous c-3279T G mutation represents an important risk factor for the development of neonatal hyperbilirubinemia.

Abdominal aortic aneurysm (AAA) is associated with chronic mural inflammation and a pro-thrombotic diathesis. It has been suggested that both may be related to biologically active intra-sac thrombus. The aim of this study was to examine the relationship between thrombin generation, fibrinolysis, platelet activity and AAA sac thrombus volume.30 patients (29 men) of median (IQR) age 75 (71-82) years with an infra-renal AAA5.5 cm in antero-posterior diameter were prospectively studied. AAA, lumen and thrombus volumes were calculated using a CT workstation (Vitrea). Plasma thrombin-antithrombin (TAT), plasminogen activator inhibitor (PAI)-1, and soluble (s) P-selectin were measured as biomarkers of coagulation, fibrinolysis and platelet activity, respectivelyMedian (IQR) AAA total, lumen and thrombus volumes were 188 (147-247) cm(3), 80 (54.3-107) cm(3) and 97.6 (63-127) cm(3) respectively. TAT levels were significantly higher (median, QR, 7.15 [4.7-31.3] μg/L, p=0.001) and sP-selectin levels significantly lower (median, IQR, 80.5 [68-128] ng/ml, p=0.0001) than the normal range. PAI-1 levels (median, IQR, 20.9 [8.4-50.7] ng/ml) were normal. There was no correlation between AAA thrombus volume and PAI-1 (r=-0.25, p=0.47), sP-Selectin (r=0.26, p=0.43) or TAT plasma levels (r=-0.21, p=0.54).The present study confirms that patients with AAA demonstrate haemostatic derangement, but the extent of the haemostatic derangement does not correlate with AAA sac thrombus volume.

Objective: (1) To report the incidence of myocardial injury in patients undergoing endovascular aortic aneurysm repair (EVAR) through the routine measurement of perioperative cardiac troponin-T (cTnT) and (2) to investigate and correlate changes in perioperative cTnT levels with any concomitant hemostatic derangement. Methods: Prospective study of 30 patients undergoing elective EVAR for infrarenal abdominal aortic aneurysm. Cardiac TnT was assayed at 24 hours postoperatively. Plasma thrombin antithrombin III complex (TAT), plasminogen activator inhibitor 1 (PAI-1), tissue plasminogen activator (t-PA) activity, and soluble P-selectin (sP-selectin) were assayed preoperatively and at 24 hours postoperatively. Results: Five (17%) patients demonstrated elevated cTnT levels at 24 hours; 3 patients had no clinical evidence of myocardial injury. There was a positive correlation between cTnT and TAT levels at 24 hours post-EVAR ( r = .38, P = .039, Kendall-tau B = 0.26). Conclusions: Endovascular aortic aneurysm repair is associated with a significant risk of perioperative myocardial injury that is underdetected clinically and associated with a procoagulopathic state.

Nowadays, sub-45 nm designs are facing the challenges of parametric yield loss and reliability issues. Existing design practices increase the system's area/power penalty in order to cope with the growing number of design corners and their widening distributions. Our proposed solution is the Standardized Knobs and Monitors (SKM) framework, which enables monitoring and adjusting the circuits at run-time by utilizing power-delay trade-offs. More specifically, we focus on the systematic insertion of digital monitors at the RTL level of design abstraction and demonstrate our approach by using modified crystal ball delay monitor in a real-life wireless application