AREAS COVERED: Published and unpublished studies from January 1981 to June 2020 were systematically reviewed on PubMed, Web of Science and Scopus. Additionally, the reference list of all studies was hand-searched for additional studies. This effort identified a total of 104614 registered patients and suggests identification of at least 10590 additional PID patients, mainly from countries located in Asia and Africa. Molecular defects in genes known to cause PID were identified and reported in 13852 (13.2% of all registered) patients.
EXPERT OPINION: Although these data suggest some progress in the identification and documentation of PID patients worldwide, achieving the basic requirement for the global PID burden estimation and registration of undiagnosed patients will require more reinforcement of the progress, involving both improved diagnostic facilities and neonatal screening.
METHODS: Collation and review of existing estimates of IDU prevalence and HIV prevalence from published and unpublished documents for the period 1998-2003. The strength of evidence for the information was assessed based on the source and type of study.
RESULTS: Estimates of IDU prevalence were available for 130 countries. The number of IDU worldwide was estimated as approximately 13.2 million. Over ten million (78%) live in developing and transitional countries (Eastern Europe and Central Asia, 3.1 million; South and South-east Asia, 3.3 million; East-Asia and Pacific, 2.3 million). Estimates of HIV prevalence were available for 78 countries. HIV prevalence among IDU of over 20% was reported for at least one site in 25 countries and territories: Belarus, Estonia, Kazakhstan, Russia, Ukraine, Italy, Netherlands, Portugal, Serbia and Montenegro, Spain, Libya, India, Indonesia, Malaysia, Myanmar, Nepal, Thailand, Viet Nam, China, Argentina, Brazil, Uruguay, Puerto Rico, USA and Canada.
CONCLUSIONS: These findings update previous assessments of the number of countries with IDU and HIV-infected IDU, and the previous quantitative global estimates of the prevalence of IDU. However, gaps remain in the information and the strength of the evidence often was weak.
METHODS: We analyzed the clinical, immunologic, and genetic data of IEI patients from 22 countries in the Middle East and North Africa (MENA) region. The data was collected from national registries and diverse databases such as the Asian Pacific Society for Immunodeficiencies (APSID) registry, African Society for Immunodeficiencies (ASID) registry, Jeffrey Modell Foundation (JMF) registry, J Project centers, and International Consortium on Immune Deficiency (ICID) centers.
RESULTS: We identified 17,120 patients with IEI, among which females represented 39.4%. Parental consanguinity was present in 60.5% of cases and 27.3% of the patients were from families with a confirmed previous family history of IEI. The median age of patients at the onset of disease was 36 months and the median delay in diagnosis was 41 months. The rate of registered IEI patients ranges between 0.02 and 7.58 per 100,000 population, and the lowest rates were in countries with the highest rates of disability-adjusted life years (DALY) and death rates for children. Predominantly antibody deficiencies were the most frequent IEI entities diagnosed in 41.2% of the cohort. Among 5871 patients genetically evaluated, the diagnostic yield was 83% with the majority (65.2%) having autosomal recessive defects. The mortality rate was the highest in patients with non-syndromic combined immunodeficiency (51.7%, median age: 3.5 years) and particularly in patients with mutations in specific genes associated with this phenotype (RFXANK, RAG1, and IL2RG).
CONCLUSIONS: This comprehensive registry highlights the importance of a detailed investigation of IEI patients in the MENA region. The high yield of genetic diagnosis of IEI in this region has important implications for prevention, prognosis, treatment, and resource allocation.
OBJECTIVE: To investigate the evidence available: 1) on government initiatives to mandate transparency in drug pricing worldwide, 2) on the reported effects of drug pricing transparency initiatives on drug price, and 3) on the limitations and barriers of the implementation of drug pricing transparency.
METHODS: Databases such as Medline-Ovid, Cochrane Central Register, PubMed, and Science Direct were used to search for relevant literature from inception to February 2018. A manual search of grey literature such as policy papers, governmental publications, and websites was also performed to obtain the information that was not available in the articles. Using narrative synthesis, the results were critically assessed and summarized according to its context of drug pricing approaches.
RESULTS: Of the 4382 relevant articles located, 12 studies met the inclusion criteria for drug price transparency initiatives. Only 3 studies reported the outcomes on the regulation of drug prices. Two studies in South Africa showed that price transparency initiatives did not necessarily reduce drug prices. Another study in the Philippines indicated a reduction in medicines' price based on the effects of government-mediated access prices. The limitations and barriers in price transparency initiatives include fragmentation of the healthcare system and nondisclosure of discounts and rebates by pharmaceutical companies.
CONCLUSION: Drug pricing transparency initiatives have been implemented in many countries and commonly coexist with a country's pricing policies. Nevertheless, due to sparse evidence, the effect of drug price transparency initiatives on price control is still inconclusive.
Objectives: The aim of this study was to assess the relationship between khat and occlusal caries progression.
Methods: A cohort study was carried out among 98 Yemeni khat chewers and 101 non-chewers aged 18-35 years old with early occlusal caries lesions. All participants answered questions on socio-demographic, khat , oral hygiene , sugar intake, and oral health knowledge at baseline. All posterior teeth with an early enamel lesion on occlusal surfaces detected by visual inspection at baseline were also subjected to DIAGNOdent assessment to confirm early lesion (DIAGNOdent reading 13-24). Participants were re-examined after 12 weeks. Caries progression was considered to occur when the DIAGNOdent reading was >25. Data were analyzed using Relative risk, Mann-Whitney U test, a Wilcoxon Signed-Rank test and logistic regression analysis.
Results: Occlusal caries progression incidence between khat chewers and non-chewers, with the relative risk was 1.68. There was no significant difference in occlusal caries progression on chewing side and non-chewing side among khat chewers. Khat chewing was a statistical predictor for those with low income.
Conclusion: Khat is a risk factor for occlusion caries progression among low income group.