scholarly journals Diagnosis and management of X-linked hypophosphatemia in children and adolescent in the Gulf Cooperation Council countries

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Fahad Al Juraibah ◽  
Elham Al Amiri ◽  
Mohammed Al Dubayee ◽  
Jamal Al Jubeh ◽  
Hessa Al Kandari ◽  
...  

Abstract Introduction X-linked hypophosphatemia (XLH) is a rare inherited cause of hypophosphatemic rickets and osteomalacia. It is caused by mutations in the phosphate-regulating endopeptidase homolog, X-linked (PHEX). This results in increased plasma fibroblast growth factor-23 (FGF23), which leads to loss of renal sodium-phosphate co-transporter expression leading to chronic renal phosphate excretion. It also leads to low serum 1,25-dihydroxyvitamin D (1,25(OH)2D), resulting in impaired intestinal phosphate absorption. Chronic hypophosphatemia in XLH leads to impaired endochondral mineralization of the growth plates of long bones with bony deformities. XLH in children and adolescents also causes impaired growth, myopathy, bone pain, and dental abscesses. XLH is the most frequent inherited cause of phosphopenic rickets/osteomalacia. Hypophosphatemia is also found in calcipenic rickets/osteomalacia as a result of secondary hyperparathyroidism. Thus, chronic hypophosphatemia is a common etiologic factor in all types of rickets. Results There is considerable overlap between symptoms and signs of phosphopenic and calcipenic rickets/osteomalacia. Wrong diagnosis leads to inappropriate treatment of rickets/osteomalacia. Nutritional rickets and osteomalacia are common in the Gulf Cooperation Council countries which include Saudi Arabia, United Arab Emirates, Kuwait, Qatar, Bahrain, and Oman. Due to high levels of consanguinity in the region, genetic causes of phosphopenic and calcipenic rickets/osteomalacia are also common. Conclusion This guideline was developed to provide an approach to the diagnosis of XLH, especially where there is no family history of the disease, and that other related conditions are not mistaken for XLH. We also guide the medical management of XLH with conventional treatment and with burosumab, a recombinant human IgG1 monoclonal antibody to FGF23.

Author(s):  
Nurul Nadirah Razali ◽  
Ting Tzer Hwu ◽  
Karuppiah Thilakavathy

AbstractHypophosphatemic rickets (HR) is a syndrome of hypophosphatemia and rickets that resembles vitamin D deficiency, which is caused by malfunction of renal tubules in phosphate reabsorption. Phosphate is an essential mineral, which is important for bone and tooth structure. It is regulated by parathyroid hormone, 1,25-dihydroxyvitamin D and fibroblast-growth-factor 23 (FGF23). X-linked hypophosphatemia (XLH), autosomal dominant HR (ADHR), and autosomal recessive HR (ARHR) are examples of hereditary forms of HR, which are mainly caused by mutations in the phosphate regulating endopeptidase homolog, X-linked (


2020 ◽  
Vol 39 (7) ◽  
pp. 430-436
Author(s):  
Laura Lucchetti ◽  
Francesco Emma ◽  
Danilo Fintini ◽  
Marco Cappa

X-linked hypophosphatemia (XLH) is an X-linked disorder with dominant penetration, caused by mutations in the PHEX gene, which encodes for an endopeptidase that is predominantly expressed in osteoblasts, osteocytes and odontoblasts. PHEX mutations cause increased production of fibroblast growth factor 23 (FGF23) that in turn leads to hypophosphatemia by causing inhibition of the renal phosphate reabsorption and of the synthesis of active 1,25-dihydroxyvitamin D. In children XLH is characterised by rickets, bone pain, physical dysfunction, impaired growth, disproportionate short stature, lowerlimb deformities, pathological fractures, dental malposition and dental abscesses. Although phenotype may be variable in severity, early diagnosis and treatment are critical to improve outcome. Laboratory tests show hypophosphatemia associated with hyperphosphaturia and elevated alkaline phosphatase, while parathormone and calcium levels are normal. For decades, patients have been treated with conventional therapy, including active vitamin D supplementation and fractionated daily doses of oral phosphate salts. However, these therapies rarely normalise the phenotype. More recently, burosumab, a recombinant human IgG1 monoclonal antibody against FGF-23, has been introduced for the treatment of XLH. In phase 2 trials, burosumab has been shown to improve significantly clinical symptoms, as well as biological and radiological signs of rickets.


2018 ◽  
Vol 15 (1) ◽  
pp. 16-38 ◽  
Author(s):  
Samir Srairi

The paper develops a framework to explore the risk disclosure practices of 29 Islamic banks operating in the Gulf Cooperation Council countries over the period of 2013-2016 and examines the potential factors which might be affecting risk disclosure. To analyze the level of risk disclosure, the paper develops a composite index by using the content analysis technique. We also employ OLS technique to examine factors affecting Islamic banks’ risk disclosure. The results indicate a very high difference in risk disclosure between countries. Only two countries, the United Arab Emirates and Bahrain, have a higher level of risk disclosure. The findings also suggest that reporting on some risk disclosure types especially displaced commercial risk and rate of return risk is very low. The regression results show that Islamic banks with a stronger set of corporate governance mechanisms and an active Shariah board appear to disclose more risk information. Other factors that influence risk disclosure practices of Islamic banks are bank size, leverage, cross-border listings and the level of political and civil regression. The study recommends that Islamic banks have to revise their communication strategies and provide more risk information related to rate of return risk and display commercial risk. In addition, GCC regulators should establish risk disclosure regulations which have to become mandatory for all Islamic banks. To the best of our knowledge, the paper provides the first analysis related to the determinants of corporate risk disclosures of Islamic banks in the Arab Gulf region.


Author(s):  
Fahad M Al-Anezi

Abstract Background Electronic health (e-health) approaches such as telemedicine, mobile health, virtual healthcare and electronic health records are considered to be effective in increasing access to healthcare services, reducing operational costs and improving the quality of healthcare services during the coronavirus disease 2019 (COVID-19) outbreak, a pandemic resulting from the spread of a novel coronavirus discovered in December 2019. In this context, the aim of this study was to identify the most important factors influencing decision making on the implementation of e-health in Gulf Cooperation Council (GCC) member states (Bahrain, Kuwait, Oman, Qatar, Saudi Arabia and the United Arab Emirates), which are in the process of digitizing healthcare services. Methods This study reviewed the literature to identify the important factors influencing decision making on e-health. In addition, a questionnaire-based survey was conducted in order to identify the most important criteria to be considered in decision making on e-health. The survey link was forwarded to 978 healthcare practitioners and 42 experts (purposive sampling), from which a final sample of 892 (864 practitioners and 28 experts) was achieved, reflecting a response rate of 87.45%. Results Of the 44 factors identified under seven themes (strategic, quality, management, technology, function characteristics, economic, sociocultural and demographic factors), 22 factors were identified to be the most important criteria. Conclusions Findings from this study suggest that decision making in relation to e-health is a complex process that requires consideration of various factors. It was also found that attention should be paid to sociocultural and demographic factors, which may need to be considered in increasing healthcare access during the COVID-19 outbreak.


2017 ◽  
Vol 9 (4) ◽  
pp. 29
Author(s):  
Sarah G. Alzahrani ◽  
Lauren Copeland

Understanding differences among consumers across varying cultures is of great importance to the success of international retailers. Ignoring the influence of culture and centralized marketing has led to a decline in profits for some international companies. Studying the culture of Middle East countries, particularly the Gulf Cooperation Council Countries (GCCC), Saudi Arabia, United Arab Emirates, Kuwait, Bahrain, Qatar, and Oman, is essential before marketing in these countries. Additionally, the GCCC is one of the top 10 luxury markets in the world. Hofstede model of national culture is crucial for GCCC due to the fact culture norms regarding dress and appearance are nationally adopted. A sample of 170 participants from the GCCC was collected using an online questionnaire of 45 items measuring national culture dimensions and need for uniqueness when shopping for luxury goods. It was found that power distance in all GCCC countries was a significant predictor of having a need for uniqueness, as well as indulgence. Power distance had a positive relationship with the need for uniqueness while indulgence had a negative relationship with the need for uniqueness. For other dimensions, findings indicated that long term vs short term orientation, masculinity, uncertainty avoidance, and individualism were not significant predictors leading to uniqueness. Additionally, the important construct for uniqueness among GCCC consumers is unpopular choice followed by avoiding similarity. Creative choice is less important among the three constructs of uniqueness for GCCC participants. 


Author(s):  
Ali Mustafa Qamar ◽  
Rehan Ullah Khan ◽  
Suliman Alsuhibany

COVID-19 was declared a pandemic by World Health Organization in March 2020. Since then, it has attracted the enormous attention of researchers from around the world. The world has gone through previous instances of corona-viruses such as Severe Acute Respiratory Syndrome and Middle Eastern Respiratory Syndrome. Nevertheless, none was of these were of this serious nature as COVID-19. In this research, we carry out a bibliometric analysis of coronavirus research using the Scopus database. However, we restricted ourselves to the Gulf Cooperation Council countries, comprising Bahrain, Kuwait, Oman, Qatar, Saudi Arabia, and the United Arab Emirates. The analysis was performed using Biblioshiny software. We analyzed 4288 articles written by 24226 researchers from 1994 till 2021, published in 1429 sources. The number of authors per publication is 5.65. A bulk of the research (more than 68%) appeared in the form of articles. More than 43% of the publications appeared in 2020 and more than 44% in 2021. Saudi Arabia appears the most-cited country, followed by Qatar. Journal of Infection and Public Health published the most number of papers, whereas New England Journal of Medicine is the most-cited one. Memish, Z.A. wrote the maximum number of papers. The top source, according to the H-index, is the Journal of Virology. Furthermore, the two most prolific universities are King Saud University and King Abdulaziz University, both from Saudi Arabia. The research uncovered deep learning as a niche theme used in recent publications. The research landscape continues to alter as the pandemic keeps on evolving.


Author(s):  
Simon Chadwick

This chapter presents an overview of sports business in the six Gulf Cooperation Council (GCC) states. GCC member states stage mega-sports events and invest in global sports through the acquisition of football clubs, for example. Shirt sponsorship and stadium naming rights deals of the region’s national airlines aim to create favorable perceptions of the companies and their nations as well as to diversify economies beyond oil and gas. This chapter also provides a statistical profile of sport in each GCC member state and shows that Bahrain, Kuwait, and Oman are lagging far behind Saudi Arabia, the United Arab Emirates, and Qatar in terms of sport industry size. Fluctuating oil prices, political tensions between GCC states, and weak attendance at games are serious threats to the future growth of the sport industry. Our conclusion is that the private sector needs to develop extensively in order to replace the state as the industry’s central focus.


Author(s):  
Ali AlSahow ◽  
Daniel Muenz ◽  
Mohammed A Al-Ghonaim ◽  
Issa Al Salmi ◽  
Mohamed Hassan ◽  
...  

Abstract Background Dialysis adequacy, as measured by single pool Kt/V, is an important parameter for assessing hemodialysis (HD) patients’ health. Guidelines have recommended Kt/V of 1.2 as the minimum dose for thrice-weekly HD. We describe Kt/V achievement, its predictors and its relationship with mortality in the Gulf Cooperation Council (GCC) (Bahrain, Kuwait, Oman, Qatar, Saudi Arabia and the United Arab Emirates). Methods We analyzed data (2012–18) from the prospective cohort Dialysis Outcomes and Practice Patterns Study for 1544 GCC patients ≥18 years old and on dialysis >180 days. Results Thirty-four percent of GCC HD patients had low Kt/V (<1.2) versus 5%–17% in Canada, Europe, Japan and the USA. Across the GCC countries, low Kt/V prevalence ranged from 10% to 54%. In multivariable logistic regression, low Kt/V was more common (P < 0.05) with larger body weight and height, being male, shorter treatment time (TT), lower blood flow rate (BFR), greater comorbidity burden and using HD versus hemodiafiltration. In adjusted Cox models, low Kt/V was strongly related to higher mortality in women [hazard ratio (HR) = 1.91, 95% confidence interval (CI) 1.09–3.34] but not in men (HR = 1.16, 95% CI 0.70–1.92). Low BFR (<350 mL/min) and TT (<4 h) were common; 41% of low Kt/V cases were attributable to low BFR or TT (52% for women and 36% for men). Conclusion Relatively large proportions of GCC HD patients have low Kt/V. Increasing BFR to ≥350 mL/min and TT to ≥4 h thrice weekly will reduce low Kt/V prevalence and may improve survival in GCC HD patients—particularly among women.


Author(s):  
Adrienne A. Reynolds

The countries of the Middle East are undergoing rapid change in many areas, and the field of education is illustrative. This is particularly the case for the members of the Gulf Cooperation Council (GCC): Bahrain, Kuwait, Oman, Qatar, Saudi Arabia, and the United Arab Emirates (UAE). The governments of the GCC are actively striving to lessen their dependence on both their petroleum-based economies as well as the large expatriate populations that make up the majority of human resources in the workplace. One of the solutions to greater independence is through upgrading the national educational systems. Educational technology plays a key role, both as a tool used in education as well as being a conduit toward embedding technological facility into every aspect of their economic development, and thus is an end in itself.


2020 ◽  
Vol 2020 ◽  
pp. 1-15
Author(s):  
Amani Abu-Shaheen ◽  
Humariya Heena ◽  
Abdullah Nofal ◽  
Doaa A. Abdelmoety ◽  
Abdulrahman Almatary ◽  
...  

Background. Thalassemia has a burden on the healthcare systems of many countries. About 56000 conceptions result in thalassemia, globally. Objective. To assess the epidemiological profile of thalassemia in the Gulf Cooperation Council (GCC) countries. Methods. A systematic search was conducted in MEDLINE/PubMed (National Library of Medicine), CINAHL, and Embase. Relevant observational studies reporting the epidemiology of thalassemia among the GCC population were selected. Data on the prevalence, frequency, and complications of thalassemia were extracted. The quality of the retrieved studies was assessed according to the Newcastle–Ottawa Quality Assessment Scale. Results. Eighteen studies (14 cross-sectional studies, two retrospective observational studies, and two retrospective analysis) with a total of 3343042 participants were included in this systematic review. Of the 18 studies, 11 studies were conducted in Saudi Arabia, two in the Kingdom of Bahrain, one in Kuwait, three in the United Arab Emirates (UAE), and one in Qatar. The prevalence of thalassemia among children below five years of age ranged from 0.25% to 33%, while it was 0.9% in children above five years and from 0.035% to 43.3% among adult thalassemia patients. The most-reported risk factors were consanguineous marriage and high-risk marriage. There was a lack of data regarding mortality rates in thalassemia. Conclusions. Despite the premarital screening and genetic counseling (PMSGC) program for thalassemia, the incidence of high-risk couple marriages in GCC countries cannot be effectively diminished. This study suggested that the PMSGC program should adopt more attention for the high-risk areas, to enhance the level of consciousness about the hemoglobinopathy diseases and the consequences of consanguinity among the at-risk couple.


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