Epidemiology of thromboembolic events in children and adolescents with antiphospholipid syndrome: A systematic review with meta-analysis.

BACKGROUND AND AIM: This was a systematic review and meta-analysis of the prevalence of thromboembolic events in children and adolescents with antiphospholipid syndrome (APS). METHODS: We searched PubMed, EMBASE and Web of Science to select relevant articles published between 1 January 2000 and 27 February 2022. We used the random-effects meta-analysis to estimate pooled point prevalence rates of thromboembolic events in studies with a minimum sample size of 30. RESULTS: We included five studies reporting data of 336 children and adolescents with primary APS and secondary APS (SAPS). Pooled point prevalence rates of initial general thrombosis, arterial thrombosis, venous thrombosis and stroke in individuals with seropositive APS were 98.2% (95% confidence interval [CI] 87.5-100), 27.6% (95% CI 21.4-34.2), 51.1% (95% CI 38.2-63.9) and 13.4% 95% CI (6.3-22.7), respectively. Pooled point prevalence rates of initial arterial and venous thromboses in children and adolescents with SAPS were 45.7% (95% CI 21.1-71.6) and 29.2% (95% CI 14.8-46), respectively. CONCLUSION: Arterio-venous thromboembolism is highly frequent in children and adolescents with SAPS. More studies using thrombotic and non-thrombotic APS classification criteria are warranted to better assess the frequency and predictors of thromboembolism in age- and ancestry-diverse pediatric populations affected by different types of APS.

Epidemiology of thromboembolic events in children and adolescents with antiphospholipid syndrome: A systematic review with meta-analysis / Epidemiología de los acontecimientos tromboembólicos en niños y adolescentes con síndrome antifosfolípido: una revisión sistemática con metaanálisis

Background and aim: This was a systematic review and meta-analysis of the prevalence of thromboembolic events in children and adolescents with antiphospholipid syndrome (APS). Methods: We searched PubMed, EMBASE and Web of Science to select relevant articles published between 1 January 2000 and 27 February 2022. We used the random-effects meta-analysis to estimate pooled point prevalence rates of thromboembolic events in studies with a minimum sample size of 30. Results: We included five studies reporting data of 336 children and adolescents with primary APS and secondary APS (SAPS). Pooled point prevalence rates of initial general thrombosis, arterial thrombosis, venous thrombosis and stroke in individuals with seropositive APS were 98.2% (95% confidence interval [CI] 87.5–100), 27.6% (95% CI 21.4–34.2), 51.1% (95% CI 38.2–63.9) and 13.4% 95% CI (6.3–22.7), respectively. Pooled point prevalence rates of initial arterial and venous thromboses in children and adolescents with SAPS were 45.7% (95% CI 21.1–71.6) and 29.2% (95% CI 14.8–46), respectively. Conclusion: Arterio-venous thromboembolism is highly frequent in children and adolescents with SAPS. More studies using thrombotic and non-thrombotic APS classification criteria are warranted to better assess the frequency and predictors of thromboembolism in age- and ancestry-diverse pediatric populations affected by different types of APS.(AU)

Antecedentes y objetivo: Se trata de una revisión sistemática y un metaanálisis de la prevalencia de acontecimientos tromboembólicos en niños y adolescentes con síndrome antifosfolípido (SAF). Métodos: Se realizaron búsquedas en PubMed, EMBASE y Web of Science para seleccionar los artículos pertinentes publicados entre el 1 de enero de 2000 y el 27 de febrero de 2022. Se utilizó el metaanálisis de efectos aleatorios para estimar las tasas de prevalencia puntual agrupadas de eventos tromboembólicos en estudios con un tamaño muestral mínimo de 30. Resultados: Se incluyeron cinco estudios con datos de 336 niños y adolescentes con APS primario y APS secundario (SAPS). Las tasas de prevalencia puntual agrupadas de trombosis general inicial, trombosis arterial, trombosis venosa e ictus en individuos con SAF seropositivo fueron de 98,2% (intervalo de confianza [IC] 95%: 87,5-100), 27,6% (IC 95%: 21,4-34,2), 51,1% (IC 95%: 38,2-63,9) y 13,4% (IC 95%: 6,3-22,7), respectivamente. Las tasas de prevalencia puntual agrupadas de trombosis arteriales y venosas iniciales en niños y adolescentes con SAF secundario fueron de 45,7% (IC 95%: 21,1-71,6) y de 29,2% (IC 95%: 14,8-46), respectivamente. Conclusión: La tromboembolia arteriovenosa es muy frecuente en niños y adolescentes con SAF. Se justifica la realización de más estudios que utilicen criterios de clasificación del SCA trombótico y no trombótico para evaluar mejor la frecuencia y los factores predictivos de la tromboembolia en poblaciones pediátricas de edad y ascendencia diversas afectadas por distintos tipos de SCA.(AU)

Autoimmune inflammatory myopathy biomarkers.

The autoimmune inflammatory myopathy disease spectrum, commonly known as myositis, is a group of systemic diseases that mainly affect the muscles, skin and lungs. Biomarker assessment helps in understanding disease mechanisms, allowing for the implementation of precise strategies in the classification, diagnosis, and management of these diseases. This review examines the pathogenic mechanisms and highlights current data on blood and tissue biomarkers of autoimmune inflammatory myopathies.

Oral antiviral treatments for COVID-19: opportunities and challenges.

The use of antiviral COVID-19 medications can successfully inhibit SARS-CoV-2 replication and prevent disease progression to a more severe form. However, the timing of antiviral treatment plays a crucial role in this regard. Oral antiviral drugs provide an opportunity to manage SARS-CoV-2 infection without a need for hospital admission, easing the general burden that COVID-19 can have on the healthcare system. This review paper (i) presents the potential pharmaceutical antiviral targets, including various host-based targets and viral-based targets, (ii) characterizes the first-generation anti-SARS-CoV-2 oral drugs (nirmatrelvir/ritonavir and molnupiravir), (iii) summarizes the clinical progress of other oral antivirals for use in COVID-19, (iv) discusses ethical issues in such clinical trials and (v) presents challenges associated with the use of oral antivirals in clinical practice. Oral COVID-19 antivirals represent a part of the strategy to adapt to long-term co-existence with SARS-CoV-2 in a manner that prevents healthcare from being overwhelmed. It is pivotal to ensure equal and fair global access to the currently available oral antivirals and those authorized in the future.

Epidemiology of Digital Clubbing and Hypertrophic Osteoarthropathy: A Systematic Review and Meta-analysis.

ABSTRACT: Digital clubbing and hypertrophic osteoarthropathy (HOA) are long-standing clinical entities, but their prevalence have not been synthesized. We aimed to estimate the prevalence of digital clubbing and HOA in people with existing medical conditions.We comprehensively searched PubMed, Embase, and Web of Science to select studies addressing HOA or digital clubbing and published through March 23, 2021. Summary estimates of the prevalence were derived through random-effects meta-analysis and narrative synthesis. The review protocol has been registered with PROSPERO, CRD42021243934.Of 3973 records, we included 142 studies. In adults, the pooled prevalence of digital clubbing was 33.4% (95% confidence interval [CI], 16.6-52.8), 31.3% (95% CI, 22.4-41.1), 27% (95% CI, 9.4-49.5), and 22.8% (95% CI, 10.8-37.6) in subjects with intestinal diseases, interstitial lung diseases, infective endocarditis, and hepatic diseases, respectively. In children and adolescents, the pooled prevalence of digital clubbing was 29.1% (95% CI, 19.4-39.9), 23% (95% CI, 9.0-41.1), 19.5% (95% CI, 4.1-42.4), and 17.1% (95% CI, 9.5-26.5) in subjects with human immunodeficiency virus infection, hemoglobinopathies, cystic fibrosis, and tuberculosis. The pooled prevalence of HOA was 10.1% (95% CI, 2.0-23.1) in adults with cancers, and 5% (95% CI, 2.5-8.2) in children and adolescents with cystic fibrosis.In conclusion, the prevalence of digital clubbing varied across disease groups in both adults and children. Full-spectrum HOA was mostly reported in adults with liver disease and cancers, and in children and adolescents with cystic fibrosis.

Epidemiology of Sjögren Syndrome in Africa: A Scoping Review.

BACKGROUND: The epidemiology of Sjögren syndrome (SS) has been extensively studied in America, Europe, and Asia. OBJECTIVE: To summarize available data on the epidemiology of SS in Africa. METHODS: MEDLINE, EMBASE, and African Journals Online were searched from inception up to May 17, 2020, to identify relevant articles. Data gleaned from these reports have been summarized narratively in this review. RESULTS: Twenty-one hospital-based studies were included. These studies reported 744 cases of SS. The mean age at diagnosis varied between 28 and 73.6 years, and the female proportion ranged from 83.3% to 100%. There was no population-based incidence or prevalence. Among people with autoimmune and other rheumatic conditions, the frequency of primary SS was in the range 1.9% to 47.6%, whereas that of rheumatoid arthritis-associated secondary SS was in the range 4.3% to 100%. Sicca symptoms were the commonest features, with most frequently involved organs being joints, lungs, and neurological structures. Main autoantibodies were anti-Ro/SS antigen A, anti-La/SS antigen B, and antinuclear antibodies. CONCLUSIONS: The epidemiology of SS is poorly characterized in Africa. Available data are broadly consistent with those from other populations. Extensive and high-quality research is urgently needed.

Epidemiology of Idiopathic Inflammatory Myopathies in Africa: A Contemporary Systematic Review.

BACKGROUND: The epidemiology of idiopathic inflammatory myopathies (IIMs) has been extensively studied in America, Europe, and Asia, but remains unclear in Africa. OBJECTIVE: The aim of this review was to summarize available data on the epidemiology of IIMs in Africa. METHODS: We searched MEDLINE, EMBASE, and African Journals Online for studies published up to December 30, 2020, and reporting epidemiological data on IIMs in Africa. Data were combined through narrative synthesis. The review protocol was registered with PROSPERO, CRD42020186781. RESULTS: We included 39 studies reporting 683 cases (71.7% adults) of IIMs. Incidence rates of ~7.5/1,000,000 person-years and 1.2/1,000,000 person-years were estimated for dermatomyositis (DM), whereas polymyositis (PM) had an incidence rate of 8.8/1,000,000 person-years. Prevalence estimates of 11.49/100,000 and 11/100,000 (95% confidence interval, 0-32) were provided for IIMs and the PM subtype, respectively. Mean age at diagnosis ranged from 7.9 to 57.2 years, and 50% to 100% of the patients were females. Main subtypes of adult-onset IIMs were DM (21%-93%) and PM (12%-79%), whereas the commonest juvenile subtype was juvenile DM (5.8%-9%). Skeletal muscle involvement (56%-100%) was the main disease feature, and esophagus was the most commonly affected internal organ (6%-65.2%). Anti-Jo1/histidyl tRNA synthetase (7%-100%) and anti-Mi2 (17%-45%) antibodies were the most frequent myositis specific antibodies. Early mortality was high (7.8%-45%), and main death causes were infections, cancers and organ damage in respiratory and cardiovascular domains. CONCLUSIONS: Apart from a potential younger age at onset of adult IIMs in Africa, current sparse data mostly suggest a similar epidemiology between Africa and other regions. Further high-quality studies are required to validate these findings.

Assessment of the efficacy of hemodialysis on uric acid clearance in a sub-Saharan African population at the end stage kidney disease.

BACKGROUND: Uricemia dramatically rises with the stage of chronic kidney disease (CKD) and correlates with its mortality. Hemodialysis (HD) being the most used treatment at the end stage in sub-Saharan Africa, we sought to evaluate its efficacy on the clearance of uric acid (UAc) when used alone and twice per week. METHODS: A cross-sectional study of all consenting patients with CKD stage 5 recruited at random during HD sessions in a reference Centre in Cameroon from January to April 2017. We collected socio-demographic data, relevant clinical information, HD related variables, and measured serum uric acid (SUA) levels before and after the dialysis to assess the uric acid clearance. A clearance between 65 and 80% and above 80% was considered as low and good efficacy of HD respectively. Statistical analysis was performed using SPSS version 21.0. Factors associated with HD efficacy were assessed using Fisher's exact test and are presented with their odds ratios (OR) and 95% confidence levels. RESULTS: One hundred four patients (53 females) were included. The mean age was 49.9 ± 13.3 years. Hypertension (25%) and chronic glomerulonephritis (16%) were the main suspected etiologies of CKD. The median time on renal replacement therapy by HD was 3 years [1; 6]. The prevalence of hyperuricemia was 81.9%. The means of SUA levels were 78.8 ± 13.8 mg/L and 26.4 ± 6.6 mg/L respectively before and after dialysis. Mean SUA clearance was 66% ± 10%. The efficacy of HD on UAc was moderate in 92 (63.9%) and good in 2 (1.4%) patients. Excess weight (OR 0.4 [0.2; 0.9]) and Kt/Vurea < 1.2 (OR 0.1 [0.04; 0.2]) significantly reduces the efficacy of HD. CONCLUSION: HD used alone for 2 sessions per week has a moderate efficacy on uric acid clearance in CKD. Therefore, we should improve the Kt/V (> 1.2), and combine HD to uric acid lowering drugs and diet modifications to increase its efficacy.

Coronavirus Disease 2019 (COVID-19) as a Multi-Systemic Disease and its Impact in Low- and Middle-Income Countries (LMICs).

Currently, the coronavirus disease 2019 (COVID-19) is the priority of the global health agenda. Since the first case was reported in Wuhan, China, this infection has continued to spread and has been considered as a pandemic by the World Health Organization (WHO) within 3 months of its outbreak. Several studies have been done to better understand the pathogenesis and clinical aspects of the disease. It appears that COVID-19 affects almost all body organs due to the direct effect of the virus and its induced widespread inflammatory response. This multi-systemic aspect of the disease has to be inculcated in COVID-19 management by health providers to improve patient outcomes. This strategy could help curb the burden of the disease especially in low- and middle-income countries (LMICs) like most African countries where the pandemic is at an "embryonic" stage.

Outcomes of pregnancy and associated factors in sub-Saharan African women with systemic lupus erythematosus: a scoping review.

OBJECTIVE: To scope and summarise available literature on the outcomes of pregnancy and associated factors in sub-Saharan African women with SLE. METHODS: Electronic databases and reference lists of retrieved articles were searched to identify relevant studies published from 1 January 2000 to 28 October 2019. Data were combined through narrative synthesis. RESULTS: We included four studies retrospectively reporting a total of 137 pregnancies in 102 women over a 26-year period. Mean age at conception ranged from 27.2 to 39.9 years. Kidney damage, the predominant organ manifestation before conception, was reported in 43 (42.2%) patients. Ninety-seven (70.8%) pregnancies resulted in 98 live births. SLE flares occurred in 44 (32.2%) pregnancies, mainly skin (20.4%) and renal (18.2%) flares. Major adverse pregnancy outcomes (APOs) were preterm birth 38.8%, low birth weight 29.8%, pregnancy loss 29.2% and pre-eclampsia 24.8%. The main factors associated with APOs were nephritis and SLE flares. CONCLUSION: Over two-thirds of pregnancies resulted in live birth in this cohort of sub-Saharan African women with SLE. The main APOs and associated factors described in other parts of the world are also seen in this region, but with high rates of APOs. A large prospective multinational study is warranted for more compelling evidence.

Epidemiology of rheumatoid arthritis in sub-Saharan Africa: a systematic review and meta-analysis protocol.

BACKGROUND: So far, only one meta-analysis has estimated the prevalence of rheumatoid arthritis (RA) in Africa. Out of 10 studies included in that meta-analysis, nine came from sub-Saharan African countries and had been published between 1968 and 1988. We will conduct a new systematic review and meta-analysis to update their estimates and provide more consistent prevalence data on RA in sub-Saharan Africa. METHODS: We will comprehensively search electronic databases to select observational studies addressing RA in sub-Saharan Africa and published as from 1 January 2000: PubMed, EMBASE, African Journals Online, Web of Science, and Global Index Medicus. Summary estimates will be derived through random-effects meta-analysis whenever possible. Alternatively, estimates will be reported through narrative synthesis when the random-effects meta-analysis will be impossible. The risk of bias will be assessed using standard methods. DISCUSSION: This systematic review and meta-analysis shall quantify the magnitude of RA morbidity and mortality in sub-Saharan Africa. Results from this review will be disseminated in peer-reviewed journals, conferences and on social media platforms. SYSTEMATIC REVIEW REGISTRATION: PROSPERO CRD42020153483.

Systemic lupus erythematosus in Native sub-Saharan Africans: A systematic review and meta-analysis.

BACKGROUND: The prevalence and phenotype of systemic lupus erythematosus (SLE) have not been thoroughly investigated in Native sub-Saharan Africans despite knowledge that the highest burden of SLE occurs in people with an African trait. Through this systematic review of literature and meta-analysis, we wished to fill this gap. METHODS: PubMed, EMBASE, Web of Science, African Journals Online, and Global Index Medicus as well as references of retrieved papers were searched to select studies addressing SLE in Native sub-Saharan Africans and published between January 1, 2008 and October 7, 2018. The prevalence of SLE and its characteristics were pooled through narrative review and random-effects model. Heterogeneity (I2) was assessed via the χ2 test. Pooled estimates are expressed with 95% confidence intervals. This study is registered with PROSPERO: registration number CRD42019139226. RESULTS: Out of 1502 papers, 15 hospital-based studies were included. The pooled prevalence of SLE among 28,575 participants was 1.7% (0.8-2.9), with substantial heterogeneity between studies (I2 = 96.9% [94.8%; 98.1%], τ2 = 0.0020, p < 0.0001). The mean age at diagnosis ranged from 28.8 to 39.2 years. The female proportion varied from 88% to 100%. Rheumatological (5.1%-99.9%), dermatological (4.3%-100%) and hematological (1.4-86.9%) manifestations were the commonest clinical features of SLE. Patients had a high seroprevalence for anti-ribonucleoprotein 57.9% (36.4-77.9), anti-Smith 53.5% (40.4-66.2), anti-Sjogren syndrome antigen A 45.6% (19.2-73.4) and anti-Sjogren syndrome antigen B 33.7% (13.6-57.6) autoantibodies. The most used treatments were corticosteroids 99% (94.9-100) and antimalarials 62. 8% (23.3-94.1). The pooled mortality rate was 10.3% (3.3-20.6) and death was mainly due to infections, kidney disease and neurological involvement. CONCLUSION: Over the last 30 years, SLE was not rare among Native sub-Saharan Africans and its featured characteristics were earlier onset, female predominance, and high seropositivity for extractable nuclear antigen autoantibodies. Corticosteroids and antimalarials were the standard treatments. The mortality rate was high. Population prevalence and incidence as well as full description of SLE characteristics in Native sub-Saharan Africans are needed.

Epidemiological data on systemic lupus erythematosus in native sub-Saharan Africans.

Multiethnic studies conducted outside sub-Saharan Africa identify African Black people as the highest-risk group for morbidity and mortality among the 5,000,000 people who are affected by lupus globally. In the meantime, there have bee few attempts to summarize lupus data from sub-Saharan africa. We therefore conducted a systematic review and meta-analysis addressing systemic lupus erythematosus in Native sub-Saharan Africans. This paper both serves as repository for and describes the data obtained by qualitative and quantitative synthesis, notably the pooled prevalence of autoantibodies, the pooled frequency of cumulative drug use, the prevalence of comorbidities/complications and the mortality rate in Native sub-Saharan Africans with systemic lupus erythematosus. These data are interpreted in the research article titled "Systemic lupus erythematosus in Native sub-Saharan Africans: a systematic review and meta-analysis" (Essouma et al., 2019) [1].

Adult T-type lymphoblastic lymphoma presenting as hypercalcemic crisis and aplastic anemia: a case report.

BACKGROUND: Hypercalcemia and aplastic anemia are two uncommon presentations of non-Hodgkin lymphoma that potentially worsen the disease prognosis. Although hypercalcemia has been reported in the B-cell subtypes and some T-cell subtypes of non-Hodgkin lymphoma, it has not been described in T-cell lymphoblastic lymphoma. The same applies to aplastic anemia, which is also not described in T-type lymphomas. CASE PRESENTATION: We report a case of a 52-year-old Cameroonian man with acute kidney injury who presented with confusion, abdominal pain, constipation, polyuria, polydipsia, calciphylaxis, enlarged lymph nodes, tachycardia, and a blood pressure of 170/88 mmHg. Laboratory investigations revealed hypercalcemia (total/ionized 199.5/101.75 mg/L), normal serum phosphorus (40.20 mg/L), and a low intact parathyroid hormone (9.70 pg/ml). Complete blood count revealed pancytopenia. Peripheral blood smear confirmed thrombocytopenia but showed neither blasts nor flower cells. Bone marrow aspirate revealed hypocellularity with no blasts or fibrosis. Lymph node biopsy was suggestive of T-cell precursor lymphoma. T-lymphoblastic lymphoma presenting with hypercalcemic crisis and aplastic anemia was diagnosed, and the patient received the cyclophosphamide-doxorubicin-vincristine-prednisone protocol of chemotherapy together with filgrastim and whole-blood transfusion for aplastic anemia. The short-term outcome was fatal, however. CONCLUSIONS: Severe hypercalcemia and aplastic anemia are potential paraneoplastic syndromes of adult T-type lymphoblastic lymphoma, with fatal short-term outcome.