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.
CASE DESCRIPTION: A 25-years old Chinese female, with no history of systemic or ocular disease, presented to the eye clinic with one-month history of right eye (RE) blurring of vision with foreign body sensation. On examination, there were dense white crystalline needle-like projections over inferior paracentral corneal stroma with intact epithelium. There was also presence of lower eyelid epiblepharon with lashes rubbing against the diseased area. Corneal scraping cultures were suggestive of bacterial infection. Patient responded well with corneal epithelium debridement, intensive topical antibiotics and epiblepharon correction to prevent further microtrauma.
CONCLUSIONS: The only contributing factor for ICK in our patient was trichiasis from epiblepharon. Repetitive microtrauma caused by the eyelashes lead to direct penetration and inoculation of normal ocular flora into the corneal stroma. Clinicians need to be vigilant in ruling out other possible causes such as lid abnormalities when managing an ICK patient without apparent risk factors.
METHODS: A cross-sectional, self-reported, web-based questionnaire was conducted among 500 adults between February and March 2020. Questionnaire items pertained to the knowledge and attitudes toward liver-related health and diseases.
RESULTS: Half of the respondents were aged ≥35 years and 52.0% were males. Gaps in knowledge included the lack of awareness of different types of hepatitis, including the potential transmission risks and complications of HBV and HCV. About half acknowledged liver fibrosis and cirrhosis as key determinants of liver-related disease progression. A higher proportion rightly recognized the diagnostic test for HCV (40.8%) than HBV (30.0%) despite more being aware of HBV than HCV. Less than one-third were aware of the risk factors, screening tests, and complications of NAFLD. Despite the majority (92.8%) agreeing that regular screening was important for liver health, only 67.0% attended recent health screening and one-fifth were unlikely to seek medical consultation upon exposure to viral hepatitis risk factors. Reasons for this low urgency included the perception of being healthy, cost-related concerns, and societal discrimination.
CONCLUSION: Robust education efforts are needed to raise awareness and empower the community with knowledge of liver-related diseases, particularly viral hepatitis and NAFLD in Malaysia.
DISCUSSION: Although crucial guidance has been released on how to maintain TB and HIV services during the pandemic, it is acknowledged that what was considered normal service pre-pandemic needs to improve to ensure that we rebuild person-centred, inclusive and quality healthcare services. The threat that the pandemic may reverse gains in the response to TB and HIV may be turned into an opportunity by pivoting to using proven differentiated service delivery approaches and innovative technologies that can be used to maintain care during the pandemic and accelerate improved service delivery in the long term. Models of care should be convenient, supportive and sufficiently differentiated to avoid burdensome clinic visits for medication pick-ups or directly observed treatments. Additionally, the pandemic has highlighted the chronic and short-sighted lack of investment in health systems and the need to prioritize research and development to close the gaps in TB diagnosis, treatment and prevention, especially for children and people with HIV. Most importantly, TB-affected communities and civil society must be supported to lead the planning, implementation and monitoring of TB and HIV services, especially in the time of COVID-19 where services have been disrupted, and to report on legal, policy and gender-related barriers to access experienced by affected people. This will help to ensure that TB services are held accountable by affected communities for delivering equitable access to quality, affordable and non-discriminatory services during and beyond the pandemic.
CONCLUSIONS: Successfully reaching the related targets of ending TB and AIDS as public health threats by 2030 requires rebuilding of stronger, more inclusive health systems by advancing equitable access to quality TB services, including for people with HIV, both during and after the COVID-19 pandemic. Moreover, services must be rights-based, community-led and community-based, to ensure that no one is left behind.
METHODS: In this systematic review and individual participant data meta-analysis, we updated a search of PubMed (MEDLINE), Embase, the Cochrane Library, and conference abstracts for publications from Jan 1, 2011, to March 12, 2018, done in a previous systematic review to include the period up to Aug 2, 2019. We screened the reference lists of identified pieces and contacted experts in the field. We included prospective cross-sectional, observational studies and randomised trials among adult and adolescent (age ≥10 years) ambulatory people living with HIV, irrespective of signs and symptoms of tuberculosis. We extracted study-level data using a standardised data extraction form, and we requested individual participant data from study authors. We aimed to compare the W4SS with alternative screening tests and strategies and the WHO-recommended algorithm (ie, W4SS followed by Xpert) with Xpert for all in terms of diagnostic accuracy (sensitivity and specificity), overall and in key subgroups (eg, by antiretroviral therapy [ART] status). The reference standard was culture. This study is registered with PROSPERO, CRD42020155895.
FINDINGS: We identified 25 studies, and obtained data from 22 studies (including 15 666 participants; 4347 [27·7%] of 15 663 participants with data were on ART). W4SS sensitivity was 82% (95% CI 72-89) and specificity was 42% (29-57). C-reactive protein (≥10 mg/L) had similar sensitivity to (77% [61-88]), but higher specificity (74% [61-83]; n=3571) than, W4SS. Cough (lasting ≥2 weeks), haemoglobin (<10 g/dL), body-mass index (<18·5 kg/m2), and lymphadenopathy had high specificities (80-90%) but low sensitivities (29-43%). The WHO-recommended algorithm had a sensitivity of 58% (50-66) and a specificity of 99% (98-100); Xpert for all had a sensitivity of 68% (57-76) and a specificity of 99% (98-99). In the one study that assessed both, the sensitivity of sputum Xpert Ultra was higher than sputum Xpert (73% [62-81] vs 57% [47-67]) and specificities were similar (98% [96-98] vs 99% [98-100]). Among outpatients on ART (4309 [99·1%] of 4347 people on ART), W4SS sensitivity was 53% (35-71) and specificity was 71% (51-85). In this population, a parallel strategy (two tests done at the same time) of W4SS with any chest x-ray abnormality had higher sensitivity (89% [70-97]) and lower specificity (33% [17-54]; n=2670) than W4SS alone; at a tuberculosis prevalence of 5%, this strategy would require 379 more rapid diagnostic tests per 1000 people living with HIV than W4SS but detect 18 more tuberculosis cases. Among outpatients not on ART (11 160 [71·8%] of 15 541 outpatients), W4SS sensitivity was 85% (76-91) and specificity was 37% (25-51). C-reactive protein (≥10 mg/L) alone had a similar sensitivity to (83% [79-86]), but higher specificity (67% [60-73]; n=3187) than, W4SS and a sequential strategy (both test positive) of W4SS then C-reactive protein (≥5 mg/L) had a similar sensitivity to (84% [75-90]), but higher specificity than (64% [57-71]; n=3187), W4SS alone; at 10% tuberculosis prevalence, these strategies would require 272 and 244 fewer rapid diagnostic tests per 1000 people living with HIV than W4SS but miss two and one more tuberculosis cases, respectively.
INTERPRETATION: C-reactive protein reduces the need for further rapid diagnostic tests without compromising sensitivity and has been included in the updated WHO tuberculosis screening guidelines. However, C-reactive protein data were scarce for outpatients on ART, necessitating future research regarding the utility of C-reactive protein in this group. Chest x-ray can be useful in outpatients on ART when combined with W4SS. The WHO-recommended algorithm has suboptimal sensitivity; Xpert for all offers slight sensitivity gains and would have major resource implications.
FUNDING: World Health Organization.
MATERIALS AND METHOD: This study was conducted in Malaysia. Six students were observed twice each in their classroom and their classroom interactions were video recorded. The video recordings were transcribed and coded for the presence of a communication event, the student's mode of communication and communication function, the communication partner involved, and access to the AAC system.
RESULTS: Contrary to past studies, most students in this study spontaneously initiated interaction almost as many times as they responded. They primarily communicated with gestures and verbalizations/vocalizations despite having been introduced to an AAC system. When students communicated using their AAC system, they mainly interacted with the teachers, and for the function of either behavioral regulation or joint attention. It was found that for 39% of communicative events, the student's aided AAC system was not within arm's reach.
CONCLUSION: The findings highlight the need for efforts to encourage students with complex communication needs to use AAC more frequently in their classroom to be able to communicate more effectively and for a wider range of communicative functions. Speech-language pathologists can work closely with teachers to provide the necessary support to these students.
METHODS: This cross-sectional seroprevalence study with a two-stage stratified random cluster sampling design included 5,131 representative community dwellers in Malaysia aged ≥1 year. Data collection lasted from 7 August to 11 October 2020 involving venous blood sampling and interviews for history of COVID-19 symptoms and diagnosis. Previous SARS-CoV-2 infection was defined as screened positive using the Wantai SARS-CoV-2 Total Antibody enzyme-linked immunosorbent assay and confirmed positive using the GenScript SARS-CoV-2 surrogate Virus Neutralization Test. We performed a complex sampling design analysis, calculating sample weights considering probabilities of selection, non-response rate and post-stratification weight.
RESULTS: The overall weighted prevalence of SARS-CoV-2 infection was 0.49% (95%CI 0.28-0.85) (N = 150,857). Among the estimated population with past infection, around 84.1% (95%CI 58.84-95.12) (N = 126 826) were asymptomatic, and 90.1% (95%CI 67.06-97.58) (N = 135 866) were undiagnosed.
CONCLUSIONS: Our study revealed a low pre-variant and pre-vaccination seroprevalence of SARS-CoV-2 infection in Malaysia up to mid-October 2020, with a considerable proportion of asymptomatic and undiagnosed cases. This led to subsequent adoption of SARS-CoV-2 antigen rapid test kits to increase case detection rate and to reduce time to results and infection control measures.
METHOD: Surveys (Study 1) and semi-structured interviews (Study 2) were used to collect data. Thirty-eight respondents completed the Affiliate Stigma Scale (ASS), Caregiver Burden Inventory (CBI), and Care-related Quality of Life (CarerQol) scales (GMFCS). Eleven respondents took part in semi-structured interviews, which were then thematically evaluated.
RESULTS: Parents did not feel stigmatized because they had a child with CP. However, parents needed some form of short-term relief from caregiving. After controlling for demographics, high stress respondents experienced high affiliate stigma whereas low stress respondents had better quality of life. Although the respondents with less stigma had a better quality of life, this effect was moderate. Three major themes emerged from the interviews.
CONCLUSION: Counseling, support groups, and helper services should be offered to parents. Also, healthcare professionals participating in CP rehabilitation, community-based rehabilitation, and health institutions should be educated on how to better help parents of children with CP.
MATERIAL AND METHOD: 25 healthy electronic cigarette smokers and 25 age- and gender-matched healthy non-smokers were included in the study. RNFL, GCL, IPL and choroidal thickness were measured by SD-OCT using an automated programme. After normality tests, an independent sample t-test was used to analyse the differences in RNFL, GCL, IPL, and choroidal thickness values between the groups.
RESULTS: The mean age of electronic cigarette smokers and non-smokers was 33.68 and 33.64 years, respectively. The mean smoking history was 6.6 years (range 5-8 years). Most of the participants smoked 2-5 ml of e-liquid per day (52%), while 36% smoked more than 5 ml and 12% smoked less than 2 ml per day. The mean intraocular pressure in the electronic cigarette smoker group was 15.0 mmHg, while the non-smoker group was 15.32 mmHg. The mean axial length in the electronic cigarette smoker group and non-smoker group was 23.36 and 23.63 mm, respectively. No significant difference was observed regarding RNFL, GCL, IPL or choroidal thickness between both groups.
CONCLUSION: The thickness of the RNFL, GCL, IPL, and choroid was found to be similar in both the healthy electronic cigarette smokers and non-smokers groups.