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Patients with inborn errors of immunity (IEI) are prone to develop infections, either due to a broad spectrum of pathogens or to only one microbe. Since skin is a major barrier tissue, cutaneous infections are among the most prevalent in patients with IEI due to high exposures to many microbes. In the general population, human papillomaviruses (HPVs) cause asymptomatic or self-healing infections, but, in patients with IEI, unusual clinical expression of HPV infection is observed ranging from epidermodysplasia verruciformis (EV) (a rare disease due to β-HPVs) to profuse, persistent, and recalcitrant warts (due to α-, γ-, and μ-HPVs) or even tree man syndrome (due to HPV2). Mutations in EVER1, EVER2, and CIB1 are associated with EV phenotype; GATA2, CXCR4, and DOCK8 mutations are typically associated with extensive HPV infections, but there are several other IEI that are less frequently associated with severe HPV lesions. In this review, we describe clinical, immunological, and genetic patterns of IEI related to severe HPV cutaneous infections and propose an algorithm for diagnosis of IEI with severe warts associated, or not, with lymphopenia.

The term of intellectual property rights refers to the rights in copyright, designs, patents, circuit layouts, trademarks and plant breeder’s rights. It also includes the doctrines of passing off and breach of confidence. The role of intellectual property rights is significant in the global economy; they are the only assets that some corporations possess. In the most cases, labourers create such inventions in the course of their work. Therefore, modern laws around the world state specific provisions for regulating the ownership of such rights when the work is made by an employee. The present research aims at analysing legal provisions of Iraqi law in the field and comparing them to those of the United States of America (USA) and the United Kingdom (UK). It focuses on four main intellectual creations: copyright, patents, designs and plant varieties. Finally, the study points out some suggestions and updates to be made in the relevant Iraqi laws.

Data acquisition problem in large-scale distributed Wireless Sensor Networks (WSNs) is one of the main issues that hinder the evolution of Internet of Things (IoT) technology. Recently, combination of Compressive Sensing (CS) and routing protocols has attracted much attention. An open question in this approach is how to integrate these techniques effectively for specific tasks. In this paper, we introduce an effective deterministic clustering based CS scheme (DCCS) for fog-supported heterogeneous WSNs to handle the data acquisition problem. DCCS employs the concept of fog computing, reduces total overhead and computational cost needed to self-organize sensor network by using a simple approach, and then uses CS at each sensor node to minimize the overall energy expenditure and prolong the IoT network lifetime. Additionally, the proposed scheme includes an effective algorithm for CS reconstruction called Random Selection Matching Pursuit (RSMP) to enhance the recovery process at the base station (BS) side with a complete scenario using CS. RSMP adds random selection process during the forward step to give opportunity for more columns to be selected as an estimated solution in each iteration. The results of simulation prove that the proposed technique succeeds to minimize the overall network power expenditure, prolong the network lifetime and provide better performance in CS data reconstruction.

Objectives Coronary microvascular dysfunction (CMD) is considered to cause angina pectoris in a large proportion of women with no obstructive coronary artery disease (CAD). However, data supporting a relation between angina pectoris and CMD are limited. We compared CMD in women with angina with asymptomatic women and evaluated the relation between presence of CMD, angina characteristics, cardiovascular risk factors and results of stress testing.Methods In a cross-sectional study, we included 1684 women with angina and

Severe combined immunodeficiency (SCID) comprises a heterogeneous group of inherited immunologic disorders with profound defects in cellular and humoral immunity. SCID is the most severe PID and constitutes a pediatric emergency. Affected children are highly susceptible to bacterial, viral, fungal, and opportunistic infections with life-threatening in the absence of hematopoietic stem cell transplantation. We report here two cases of SCID. The first case is a girl diagnosed with SCID at birth based on her family history and lymphocyte subpopulation typing. The second case is a 4-month-old boy with a history of recurrent opportunistic infections, BCGitis, and failure to thrive, and the immunology workup confirms a SCID phenotype. The genetic study in the two cases revealed a novel mutation in the RAG2 gene, c.826G > A (p.Gly276Ser), in a homozygous state. The novel mutation in the RAG2 gene identified in our study may help the early diagnosis of SCID.

Antibiotic resistance has become a major world health challenge and has limited the ability of physician's treatment. Staphylococcus aureus the most notorious pathogens causes morbidity and mortality especially in burn patients. However, Staphylococcus aureus rapidly acquired resistance to multiple antibiotics. Vancomycin, a glycopeptide antibiotic remains a drug of choice for treatment of severe Methicillin Resistance S. aureus infections. This study aimed to detect the emergence of beta-lactam and glycopeptide resistance genes. 50 clinical specimens of S. aureus collected from burn patients in burn and plastic surgery units in Sulaimani-Iraq city. All specimens were confirmed to be positive for S. aureus. All the isolates were assessed for their susceptibility to different antibiotics depending on NCCL standards, followed by Extended Spectrum Beta Lactamase detection by double disk diffusion synergy test. The production of ?- lactamases was evaluated in the isolated strains by several routine methods and polymerase chain reaction. Among the isolates 94% were Methicillin resistance and 34.28% were Extended Spectrum Beta Lactamase producer. PCR based molecular technique was done for the bla genes related to ?- lactamase enzymes by the specific primers, as well as genes which related to reduced sensitivity to Vancomycin were detected. The results indicated that all isolated showed the PBP1, PBP2, PBP3, PBP4, trfA and trfB, graSR, vraS except the vraR gene and the prolonged therapy of Methicillin resistance infection with teicoplanin have been associated with progress of resistance and the rise of tecoplanin resistance may be a prologue to evolving Vancomycin resistance. In conclusion, beta-lactam over taking can rise Vancomycin- Intermediate S. aureus strains leading to appearance of Vancomycin resistance although the treatment of Vancomycin resistant infections is challenging.

The interpretation of subsurface fluid flow features in seismic reflection data is a key part of identifying the presence of hydrocarbon and active petroleum systems. Currently, this kind of study is mainly conducted utilizing offshore seismic reflection data with very limited cases utilizing onshore seismic reflection data. In addition, the Alamein basin is an area of prolific study in onshore Egypt, with most related studies concentrating on basin analysis and reservoir characterization. Therefore, in our study we aimed to make practical and effective use of onshore seismic reflection data with seismic attribute analysis to describe seismic facies, delineating and interpreting subsurface fluid flow features. The relatively vertical V-shaped and pipe or concave-up-shaped features with distorted reflections inside them are revealed through the analysis of variance, sweetness, chaos, instantaneous frequency and the RMS amplitude of seismic attributes. These subsurface fluid flow features are a product of mature source rock that migrates hydrocarbon vertically through faults (especially deep-seated faults) and fracture systems (fault and fracture-related subsurface fluid flow features). The classification scheme presented in our study could be implemented in the onshore case worldwide.

The number of genetically defined Primary Immunodeficiency Diseases (PID) has increased exponentially, especially in the past decade. The biennial classification published by the IUIS PID expert committee is therefore quickly expanding, providing valuable information regarding the disease-causing genotypes, the immunological anomalies, and the associated clinical features of PIDs. These are grouped in eight, somewhat overlapping, categories of immune dysfunction. However, based on this immunological classification, the diagnosis of a specific PID from the clinician's observation of an individual clinical and/or immunological phenotype remains difficult, especially for non-PID specialists. The purpose of this work is to suggest a phenotypic classification that forms the basis for diagnostic trees, leading the physician to particular groups of PIDs, starting from clinical features and combining routine immunological investigations along the way. We present 8 colored diagnostic figures that correspond to the 8 PID groups in the IUIS Classification, including all the PIDs cited in the 2011 update of the IUIS classification and most of those reported since.

Background and study aims Hands-on training for per-oral endoscopic myotomy (POEM) in the United States is limited and without a structured curriculum or assessment tool. Training to competency in POEM is critical and POEM trainees must acquire multiple cognitive and technical skills to achieve proficiency. The aim of this study was to develop a POEM training and skills evaluation tool. Patients and methods The training protocol included initial explant porcine models followed by live human cases, proctored by a single endoscopist experienced in POEM who prospectively graded trainees for each step (“skill”) of the procedure on a 5-point scale. Procedural skills were divided into cognitive and technical skills. Acceptable passing level was considered a score ≥ 4 for each skill. Results Three trainees completed a total of 18 cases (8 cases on animal explant models and 10 human cases). Overall, cognitive skills were acquired early in training with scores of ≥ 4 achieved by ≤ 3 cases. Technical skills required more cases and direction with scores ≥ 4 in technical skills achieved by three porcine and eight human cases. Entry of the endoscope into the submucosal space and submucosal tunneling were the most challenging steps followed by myotomy. Conclusion This pilot study introduces a POEM training and skills evaluation tool for training to competency. Submucosal entry, tunneling, and myotomy were the most challenging to learn while cognitive skills were learned early in training. Evaluation of more trainees at multiple sites will be necessary to further validate the utility of this tool.

Objective: the present study was aimed to evaluate the role of pharmaceutical services in improving the outcome of mineral bone disorder in patients with advanced chronic kidney disease. Methodology: One hundred and twenty patients with chronic kidney disease-mineral bone disorder (CKD-MBD) screened for eligibility, seventy-six patients enrolled in the study and randomly allocated into two groups: pharmaceutical care and usual care, both groups interviewed by the pharmacist using specific questionnaire for assessing the quality of life (QoL). All the drug related problems (DRPs) including drug-drug interactions (DDIs) were recorded by the pharmacist. Blood samples were collected and utilized for analyzing the levels of vitamin D, phosphorous, calcium, albumin and parathyroid hormone at baseline and three months after. The pharmaceutical care group received all the educations about their medications and how to minimize DRPs; improve the QoL. Additionally, the pharmaceutical intervention included correcting the biochemical parameters. Results: Pharmaceutical care significantly improved patients QoL and minimized DRPs and DDIs. It was also effective in improving the biochemical parameters. Conclusion: Pharmaceutical care has a positive impact on improving the outcome of patients with CKD-MBD through attenuating DRPs, improving the biochemical parameters and the QoL.

Alpha-1 antitrypsin deficiency is an autosomal, codominant disorder caused by mutations of the SERPINA1 gene. This genetic disorder is mainly associated with development of pulmonary emphysema and/or chronic liver disease and cirrhosis.Here we report a very rare alpha-1 antitrypsin Null Q0cairo homozygous mutation characterized by a complete absence of alpha-1 antitrypsin in the plasma, in a non-consanguineous Moroccan family. This mutation has been previously described in heterozygosis in only three cases worldwide: an Italian/Egyptian family and two Italian families (Zorzetto et al., 2005). The main clinical features in two members of this Moroccan family were the severity and precocity of bronchiectasis, quickly spreading and seriously limiting respiratory function and physical activity by the second decade of age. Moreover, the index case presented with many episodes of pulmonary infections concomitant with severe neutropenia. The third member of the family presented with ankylosing spondyloarthritis and developed panniculitis later but had no respiratory symptoms.The presence of this alpha-1-antitrypsin Q0cairo homozygous mutation could explain the severity of clinical manifestations. Moreover, our observations highlight a great variability of clinical expression for the same mutation: early severe bronchiectasis, panniculitis, rheumatologic manifestations. This study further underlines the importance of genotyping by whole SERPINA1 gene sequencing in addition to serum alpha-1 antitrypsin determination, to enable detection of alpha-1 antitrypsin deficiency due to rare genotypes. Keywords: Alpha-1 antitrypsin deficiency, Bronchiectasis, Panniculitis, Null mutation, SERPINA1 genotyping

Context: Spontaneous forniceal rupture is one of the possible complications of urolithiasis. The mechanism of forniceal rupture is not well explained in the literature. Most of the cases presented with sudden onset of acute renal colic and diagnosed by noncontrast CT (NCCT). Until now there is no solid consensus about the ideal management of such a condition. Aim: To study indications and validity of conservative management of spontaneous caliceal rupture. Settings and Design: This is an observational prospective study. Materials and Methods: All cases diagnosed as spontaneous forniceal rupture in our departments from 2011 to 2015 were enrolled. All cases were diagnosed with NCCT or CT-Urography.. Non complicated patients were primarily managed conservatively. Intervention (DJ stent insertion, nephrostomy tube insertion, or urgent ureteroscopy) was reserved to complicated cases or solitary kidney. Drainage of urinoma was done in cases with sizable urinoma (more than 100 ml3). Results: A total of 40 patients: Twenty eight (70%) patients had normal serum creatinine level at presentation, while 12 (30%) patients had elevated serum creatinine. Twenty three (57.5%) patients were successfully managed conservatively, their mean size of stone was 6.2 ± 2 mm and the hospital stay was 3.1 ± 1.9 days. Eighteen patients (42.5%) underwent interventions. Their stone size was 4.5 ± 2.1 mm and the hospital stay for the interventional group was 4.2 ± 1.8 days. Statistical Analysis Used: Mann-Whitney test was used for testing difference between means because the data groups are not parametric. Otherwise, simple descriptive statestics were used. Conclusion: Spontaneous forniceal rupture is more likely to occur with smaller distal ureteric stones. Conservative management is a valid option in non-complicated cases. Intervention should be reserved to complicated cases or cases with sizable urinoma.

The recent advances in compressive sensing (CS) based solutions make it a promising technique for signal acquisition, image processing and other types of data compression needs. In CS, the most challenging problem is to design an accurate and efficient algorithm for reconstructing the original data. Greedy-based reconstruction algorithms proved themselves as a good solution to this problem because of their fast implementation and low complex computations. In this paper, we propose a new optimization algorithm called grey wolf reconstruction algorithm (GWRA). GWRA is inspired from the benefits of integrating both the reversible greedy algorithm and the grey wolf optimizer algorithm. The effectiveness of GWRA technique is demonstrated and validated through rigorous simulations. The simulation results show that GWRA significantly exceeds the greedy-based reconstruction algorithms such as sum product, orthogonal matching pursuit, compressive sampling matching pursuit and filtered back projection and swarm based techniques such as BA and PSO in terms of reducing the reconstruction error, the mean absolute percentage error and the average normalized mean squared error.

Background: X-linked agammaglobulinemia is an inherited immunodeficiency recognized since 1952. In spite of seven decades of experience, there is still a limited understanding of regional differences in presentation and complications. This study was designed by the Primary Immunodeficiencies Committee of the World Allergy Organization to better understand regional needs, challenges and unique patient features. Methods: A survey instrument was designed by the Primary Immunodeficiencies Committee of the World Allergy Organization to collect both structured and semi-structured data on X-linked agammaglobulinemia. The survey was sent to 54 centers around the world chosen on the basis of World Allergy Organization participation and/or registration in the European Society for Immunodeficiencies. There were 40 centers that responded, comprising 32 countries. Results: This study reports on 783 patients from 40 centers around the world. Problems with diagnosis are highlighted by the reported delays in diagnosis>24 months in 34% of patients and the lack of genetic studies in 39% of centers Two infections exhibited regional variation. Vaccine-associated paralytic poliomyelitis was seen only in countries with live polio vaccination and two centers reported mycobacteria. High rates of morbidity were reported. Acute and chronic lung diseases accounted for 41% of the deaths. Unusual complications such as inflammatory bowel disease and large granular lymphocyte disease, among others were specifically enumerated, and while individually uncommon, they were collectively seen in 20.3% of patients. These data suggest that a broad range of both inflammatory, infectious, and autoimmune conditions can occur in patients. The breadth of complications and lack of data on management subsequently appeared as a significant challenge reported by centers. Survival above 20 years of age was lowest in Africa (22%) and reached above 70% in Australia, Europe and the Americas. Centers were asked to report their challenges and responses (n = 116) emphasized the difficulties in access to immunoglobulin products (16%) and reflected the ongoing need for education of both patients and referring physicians. Conclusions: This is the largest study of patients with X-linked agammaglobulinemia and emphasizes the continued morbidity and mortality of XLA despite progress in diagnosis and treatment. It presents a world view of the successes and challenges for patients and physicians alike. A pivotal finding is the need for education of physicians regarding typical symptoms suggesting a possible diagnosis of X-linked agammaglobulinemia and sharing of best practices for the less common complications. Keywords: XLA, Agammaglobulinemia, Infection, Autoimmunity, Outcomes, Immunoglobulin, Therapy