Title: Navigating the Complexity of a Rheumatic Heart Disease Literature Review

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Whilst it is clear that RHD can be triggered by recurrent group A streptococcal infections, the
mechanisms driving clinical progression are still poorly understood. However, both populations are
indigenous, and genetic heterogeneity may have affected the results. The overall kappa between the
first and second echo reviews for the presence of RHD was 0.89. Detailed echocardiographic
findings are provided in Table 2. Ficolin-3 was found highly expressed in the liver and lung tissues,
indicating its significance in both activation of the lectin pathway and pulmonary host defense ( 80,
104 ). Indeed, studies have shown significant differences in the prevalence of RHD, when the WHO
diagnostic criteria of 2006 is compared with the WHF criteria of 2012 76. When plaque builds up,
they harden and narrow your coronary arteries, decreasing blood flow to your heart. Treatments that
reduce these risk factors include angiotensin-converting enzyme inhibitors, statins, and, in some
patients, metformin. This article is an open access article distributed under the terms and conditions
of the Creative Commons Attribution (CC BY) license ( ). Voices behind the Statistics: A Systematic
Literature Review of the Lived Experience of Rheumatic Heart Disease. The three chains are
associated by disulfide bonds and form the structural unit of MBL, which, in turn, polymerize into
higher-order MBL oligomers ( 19 ) (Figure 3 ). Clinical profile of paediatric patients with rheumatic
heart disease at moi t. One publication provides an example of negative language used by healthcare
providers with regard to compliance and illness. The inadequacy of world health organization criteria
for optimizing. Mayosi Authors Jean Jacques Noubiap View author publications You can also search
for this author in. Located in the literature to assess relevance to other settings. After the removal of
duplicates, 528 were assessed for inclusion. Conflicts of Interest The authors declare no conflict of
interest. Like C1s, MASP-2 leads to the formation of the C3 convertase, C4b2a, but its activation is
dependent on MASP-1. Therefore, we primarily report here on the 15 Australian publications, and
only include findings from the New Zealand literature where they are notably different or,
conversely, add depth to themes from the Australian literature. These findings could signify that the
true prevalence of RHD is underrated or overrated by studies employing the WHO or WHF criteria,
respectively. This is contrary to the hypothesis that school-based studies are likely to underrate the
actual burden of RHD due to the association between school attendance and socioeconomic status;
which in turn, is a principal risk factor for RHD 100. Interleukin ( IL ) 10 is another immune
modulatory gene that has been studied in RHD patients from different populations and other
autoimmune diseases. All three pathways are initiated by multiple stimuli independently from each
other and subsequently the proteolytic cascades converge toward the activation of the major
component C3, which results in the assembly of the membrane-attack complex (MAC) (Figure 2 ) ( 5
). These social and cultural strengths within Indigenous groups may mitigate some of the difficulties
of living with RHD (see also sub-theme: Factors contributing to positive experiences of health-care).
In fact, both C1q and MBL facilitate the binding of apoptotic cells to immature dendritic cells as
well as to macrophages ( 27 ). Haynes, E.; Mitchell, A.; Enkel, S.; Wyber, R.; Bessarab, D. The
incidence of acute rheumatic fever (ARF) and of its. Association of ficolin-3 with severity and
outcome of chronic heart failure. Mannan-binding lectin enhances susceptibility to visceral
leishmaniasis. On the other hand, MASP2 polymorphisms were associated with the susceptibility to
leprosy ( 136 ), human T lymphotropic virus infection ( 137 ), malaria ( 138 ), Chagas disease ( 139 ),
bacterial infections ( 140 ), and hepatitis C ( 141 ).
Ficolin 2 (FCN2) functional polymorphisms and the risk of rheumatic fever and rheumatic heart
disease. Assignment page only contains the topic title, eg.“Influenza” or “Rheumatic Heart Disease”.
Mannan-binding-lectin-associated serine proteases, characteristics and disease associations. Effective
antibiotic treatment acutely (starting less than 10 days.)almost completely eliminates risk of the dis.
The authors proposed that alleles associated with increased production of FCN1 may help to
eliminate the infection; however, prolonged inflammation under the influence of these alleles may
cause tissue damage. Another patient had an indication of commissurotomy for MV stenosis at the
time of diagnosis ( Figure 2 ). Prof Dr Bashir Ahmed Dar 5.Rheumatic heart disease pathology
5.Rheumatic heart disease pathology PNK SINGH More Related Content What's hot Acute
rheumatic fever-definition,pathophysiology,clinical presentation and ma. Genetic fine mapping and
functional studies in extreme phenotypes, together with large-scale omics studies including
genomics, transcriptomics, epigenomics, and metabolomics, are expected to provide new information
not only on RHD but also on the mechanisms of other autoimmune diseases and facilitate future
clinical translation. To tackle the burden of RHD, the World Heart Federation (WHF) released in
2013 a position statement on the prevention and control of RHD, with the ambitious goal of
achieving a 25% reduction in premature deaths from ARF and RHD among individuals aged 7.
However, both populations are indigenous, and genetic heterogeneity may have affected the results.
For this reason, we have also not given subtheme and theme totals in this section. Next Article in
Journal Contextual and Psychosocial Factors Influencing the Use of Safe Water Sources: A Case of
Madeya Village, uMkhanyakude District, South Africa. The mannan-binding lectin pathway and
lung disease in cystic fibrosis-disfunction of mannan-binding lectin-associated serine protease 2
(MASP-2) may be a major modifier. Complement: a key system for immune surveillance and
homeostasis. Visit our dedicated information section to learn more about MDPI. We found that only
one sub-theme relating to medication costs was specific to New Zealand. DNA typing of HLA class
II genes in Japanese patients with rheumatic heart disease. Noteworthy omissions in the reviewed
literature include: the experiences of children and adolescents; evidence of Indigenous priorities and
perspectives related to policy and practice; nuanced discussions of power relations (for example
regarding medication compliance); recognition of Indigenous knowledges and strengths as central;
and a lack of critical reflection regarding the impacts of a dominant biomedical approach. All 11
patients had mild-to-moderate MV regurgitation, four with associated MV stenosis and abnormal
morphology (including two bioprostheses). Molecular determinants of oligomer formation and
complement fixation in mannose-binding proteins. In: Ferretti JJ, Stevens DL, Fischetti VA, editors.
Activation of the lectin pathway occurs through the binding of the complex of mannose-binding
lectin (MBL), CL-K1 or ficolins, and MBL-associated serine proteases 1 and 2 (MASP-1 and
MASP-2, respectively) to various carbohydrates or acetylated residues on the surface of pathogens
(PAMP, pathogen-associated molecular pattern). Some polymorphisms on MASP1 and MASP2 genes
lead to changes in serum levels and functions of MASPs, thereby influencing complement activation
by the lectin pathway ( 128, 129 ). In systemic lupus erythematosus (SLE), higher expression MIF
alleles ( 173C and 794 extended alleles) were associated with a lower disease risk ( 57 ). Work-role
of Radiation Therapists in the Consequences of Adaptive Radiotherap. In an initial study in 2001,
there was no association between the A, B, C, and D alleles and RHD in Chinese patients ( 164 ).
Located in the literature to assess relevance to other settings. Streptococcus pyogenes: Basic Biology
to Clinical Manifestations. Painful, migratory, and limited to the major joint of extremities
Inflammation in 1 joint lasts for 1-2 week and polyarthritis as whole resolve in 1 month or less. Note
that from the first issue of 2016, this journal uses article numbers instead of page numbers.
In addition to covering the above sections, you must also cover the below points. The first screening
program, including 16 developing. However, appropriate comparison across cohorts is limited by
small sample sizes, the use of non-standardized criteria for diagnosis of RHD, short duration of
follow up, varying proportion of participants on penicillin prophylaxis and rates of adherence to
penicillin prophylaxis. This might explain the heterogeneity of RHD observed across studies. In 16
months, 226 relatives and 47 controls of 121 patients were screened, including 129 children, 77
siblings and 20 parents. We conceived and applied a search strategy based on the combination of
terms related to RHD, ARF and echocardiography. Tropical Medicine and Infectious Disease
(TropicalMed). The association between mannan-binding lectin gene polymorphism and clinical
leprosy: new insight into an old paradigm. Record a range of illustrative quotes including themes
from the accounts of “many,” “most,” or “some” study participants. This resulted not only from
socioeconomic development, but also reflects the expansion of the public health system and
improved access to basic RHD care (e.g., the treatment of pharyngitis and acute rheumatic fever,
primary and secondary prophylaxis, and clinical follow-up), provided at the primary level. This
systematic review presents a critical, interpretive analysis of publications that include lived
experiences of RHD. Common risk alleles for inflammatory diseases are targets of recent positive
selection. A 3D confirmatory standard echocardiogram showing severe mitral valve stenosis,
associated with marked morphological abnormalities in a first-degree relative with the indication of
mitral commissurotomy at the time of diagnosis. RHD remains a common cause of acquired heart
disease in young adults and children in many developing countries and, to a lesser extent, in
developed countries ( 4, 5 ). Treatment of ARF First line of symptomatic therapy is antiinflammatory
agent ranging from salicylates to steroid. Journal of Theoretical and Applied Electronic Commerce
Research (JTAER). MASP-2 levels have been related also with a number of diseases, including
schizophrenia ( 142 ), septic shock ( 143 ), acute lymphoblastic leukemia, non-Hodgkin lymphoma,
central nervous system tumors ( 144 ), colorectal cancer ( 145, 146 ), among others. PM, Assis RV, et
al. Human heart-infiltrating t-cell clones from. The Pacific Islands Rheumatic Heart Disease Genetics
Network performed the first GWAS in RHD in 2017 ( 39 ), which examined 1006 indigenous people
in different Oceanian countries using the 300k Illumina HumanCore platform, which is considered a
low-density GWAS chip. The first page of the PDF of this article appears above. Mannose-binding
lectin binds to a range of clinically relevant microorganisms and promotes complement deposition.
For that reason, Ficolin-3 is considered to play an important role in both systemic and local innate
immune responses ( 80, 105, 106 ). Cellular mechanisms leading autoimmune reactivity and disease.
J. Full size table Discussion This systematic review and meta-analysis provides a critical summary of
the global prevalence of RHD based on data pooled from 82 observational community- and school-
based studies involving 1,090,792 individuals. Haynes, Emma, Alice Mitchell, Stephanie Enkel,
Rosemary Wyber, and Dawn Bessarab. Recent study involving all MASPs showed that MASP-1
levels were highest in subacute myocardial infarction and lowest in acute stroke patients, while
MASP-2 levels were low in both conditions and MASP-3 and MAp44 levels did not differ between
the groups. It is initiated by the binding of pattern-recognition plasma molecules such as mannose-
binding lectin (MBL), collectin 11 (CL-K1), or ficolins to carbohydrates or acetylated residues
present on microorganisms or to aberrant glycocalyx patterns on apoptotic, necrotic, or malignant
cells ( 7 ). A 3D confirmatory standard echocardiogram showing severe mitral valve stenosis,
associated with marked morphological abnormalities in a first-degree relative with the indication of
mitral commissurotomy at the time of diagnosis. Plasma levels of mannan-binding lectin (MBL)-
associated serine proteases (MASPs) and MBL-associated protein in cardio- and cerebrovascular
diseases. Acute rheumatic heart disease (RHD) is the cardiac manifestation of RF and is associated
with inflammation of the valves, myocardium, or pericardium.
Acute rheumatic fever-definition,pathophysiology,clinical presentation and ma. The inadequacy of
world health organization criteria for optimizing. Moreover, MASP-2 levels were found significantly
reduced in myocardial infarction, probably due to the activation of the lectin pathway during acute
myocardial ischemia ( 159 ). Detection of structural gene mutations and promoter polymorphisms in
the mannan-binding lectin (MBL) gene by polymerase chain reaction with sequence-specific primers.
In addition, cost-effectiveness analyses in these different scenarios are also essential to guide
discussions about the implementation of the strategy. 6. Limitations Our study has several
limitations, most of them inherent to the difficulties in enrolling a large sample of RHD patients
which are representative of the Brazilian population in terms of age, gender and race distribution.
The impact of FCN2 polymorphisms and haplotypes on the ficolin-2 serum levels. Full size image
Characteristics of included studies reporting on the prevalence of RHD Regarding methodological
quality, 51 (62%) had low risk of bias, 30 (37%) had moderate risk of bias, and one (1%) had high
risk of bias. The funder did not have any relationship with the conduct of the study, data collection,
analysis, and interpretation, and the preparation, review, or approval of this manuscript. Over the last
few decades, genome-wide association studies (GWAS) have been the major tool used to identify
genetic loci that predispose to different complex diseases. An Echo Doppler Study in 5-15-Years-Old
School Children. In addition to the effect of chronic diseases on mortality and morbidity, these
diseases also present macroeconomic and developmental challenges. 18:50 Zoloktilar: A number of
cardiovascular disease management strategies in the literature reported promising results. The innate
immune component ficolin 3 (Hakata antigen) mediates the clearance of late apoptotic cells. The
realities of injection pain for children and adolescents demonstrates, for example, the overlapping
theme domains impacting on the lived experience of RHD. IL-1, and IL-17, have been shown to be
associated with disease. Consenting first-degree relatives were invited for echo screening using
handheld devices (GE VSCAN) by non-physicians, with remote interpretation. Conventional
systematic review inclusion criteria were broadened to include publications that might otherwise
have been omitted (such as editorials and case studies). Tropical Medicine and Infectious Disease
(TropicalMed). Funding support: This study was partly funded by FAPEMIG, CNPq and and. ARF
uncommon Incidence of ARF: Population-based Studies What is the prevalence of rheumatic heart
disease. Clinical implications of shared genetics and pathogenesis in autoimmune diseases. The
European League Against Rheumatism published their recommendation on estimating cardiovascular
risk in RA; cardiovascular, this has not been validated yet. Nevertheless, the last two decades have
seen a huge amount of “big” omics data generated in many other rheumatic autoimmune diseases (
70 ) sharing many features with RHD such as symptoms and progression. Although limited, this
sample consists of a high-risk population selected in a tertiary specialized outpatient clinic—a
referral center for the state—allowing for novel insights about familial predisposition among the
individuals with the most severe phenotypes. Subsequent analysis on MBL2 polymorphisms in the
same cohort of patients showed that MBL2 genotypes associated with high MBL levels were also
significantly associated with RHD, if compared with controls, suggesting a role for high-producing
MBL2 genotypes in the susceptibility to RHD. It is noteworthy that the clinical significance of
borderline RHD in individuals with no prior history of ARF remains obscure. Assignment page only
contains the topic title, eg.“Influenza” or “Rheumatic Heart Disease”. Plasma levels of mannan-
binding lectin (MBL)-associated serine proteases (MASPs) and MBL-associated protein in cardio-
and cerebrovascular diseases. Age-dependent variation in the serum concentration of mannan-binding
protein. MAp44, a human protein associated with pattern recognition molecules of the complement
system and regulating the lectin pathway of complement activation. This reflects the extent to which
Indigenous priorities are often not those of biomedical research.
Oligomeric structures required for complement activation of serum mannan-binding proteins. A
recent meta-analysis of nine studies including 1333 RHD cases and 1212 controls from seven
different populations showed no correlation with the disease or disease subtypes ( 52 ). FIGURE 3:
Transthoracic echocardiography from 14-year-old child with rheumatic involvement of aortic valve.
The innate immune component ficolin 3 (Hakata antigen) mediates the clearance of late apoptotic
cells. The authors suggested that high MBL levels could be a cause of undesirable complement
activation in RHD patients, contributing to the pathogenesis of rheumatic cardiomyopathy ( 166 ).
The major flaw of this meta-analysis was to pool together estimates from studies which used various
echocardiographic screening approaches and criteria. Funding support: This study was partly funded
by FAPEMIG, CNPq and and. L, et al. Status of research and development of vaccines for.
Prevalence of rheumatic heart disease in children and young adults. Echocardiography has been
reported to be far more sensitive than cardiac auscultation in screening for RHD 43, 50, 56. Work-
role of Radiation Therapists in the Consequences of Adaptive Radiotherap. Journal of
Pharmaceutical and BioTech Industry (JPBI). The realities of injection pain for children and
adolescents demonstrates, for example, the overlapping theme domains impacting on the lived
experience of RHD. Approximately 60% of ARF patients develop RHD ( 1 ), and although there is a
proven association between GAS infection and RHD, the triggered autoimmune process in RHD can
occur autonomously after removing the stimulus ( 10 ), suggesting that after initiation of the
autoimmune response via molecular mimicry, host factors, most likely genetic factors, play an
important role in disease progression in susceptible individuals. ARF is a multisystem disease
resulting from an autoimmune reaction to group A streptococcal (GAS) pharyngitis in genetically
susceptible individuals 1. This loss of oxygen in the blood can lead to ischemia and later, myocardial
infarction Cardiovascular disease or heart disease is a cardiovascular of diseases that involve the
heart or blood vessels. RHD causes inflammation of the cardiac valves, initially leading to clinically
silent valvular disease and ultimately severe permanent damage. Arguably, part of the problem is a
lack of attention to the voices, opinions and understandings of the people behind these statistics.
Journal of Functional Morphology and Kinesiology (JFMK). Download citation Received: 17
February 2019 Accepted: 21 October 2019 Published: 19 November 2019 DOI: Share this article
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a shareable link is not currently available for this article. Figure 2, Flow chart of literature searches
and screening results - Types of Dietary Fat and Cardiovascular Disease: A Systematic Review Your
browsing activity is empty. However, the authors suggested a putative role for MBL deficiency in
the progression of RHD, by considering the age of onset of heart disease. Since MBL is a key
molecule in the innate host defense against bacterial infection, the authors postulated that MBL
could be considered as a double-edged sword molecule in the physiopathology of RF and RHD, on
one hand conferring protection against initial infection by rheumatogenic streptococci, but on the
other hand eliciting inflammation and complement tissue damage in the chronic stage of the disease (
167 ). In conclusion, first-degree relatives of individuals with clinical RHD are at greater risk of
having RHD, on top of socioeconomic conditions. UNSD of countries by continent was not
associated with RHD prevalence. In an initial study in 2001, there was no association between the A,
B, C, and D alleles and RHD in Chinese patients ( 164 ). Neutrophils and macrophages express
complement receptors (CR1, CR2, and CR4), which bind C3b, C4b, and iC3b. Generalized
secondary amyloidosis was observed in 32 (18.93%) of the 169 patients with RA. This study reveals
a high prevalence of RHD among WHO regions, with the highest rates recorded in Africa. Mice
deficient in ficolin, a lectin complement pathway recognition molecule, are susceptible to
Streptococcus pneumoniae infection.
Outcomes assessed in the review Primary outcomes of interest were not detailed other than a general
statement about impact on disease disease. Please note these are examples only and may not meet all
the requirements of this assignment. Five amino acid exchanges were described, all with allele
frequencies below 5%: p.Leu12Val, p.Leu117fs, p.Thr125Ala, p.Glu166Asp, and p.Val287Ala ( 80 ).
This high conservation indicates that Ficolin-3 might exert crucial function in the immune response.
Another study reported lower MBL serum levels in RHD patients from Yemen, compared with blood
donors ( 165 ). Decoding the role of regulatory element polymorphisms in complex disease.
Interestingly, after conditional analysis, the HLA class III signal remained associated with RHD,
indicating that the effect of rs201026476 is independent of class I and class II. Generalized
secondary amyloidosis was observed in 32 (18.93%) of the 169 patients with RA. L-ficolin is a
pattern recognition molecule specific for acetyl groups. Recognising these interactions necessitates
creating change at every level of experience, addressing sociological as well as disease-specific and
health system factors. Dengue Fever-Related Cardiac manifestation in Ibn-Sina Hospital Mukalla,
Hadh. Second, also due to sample limitations, no stratified sampling procedures were carried out;
thus, the findings cannot be extrapolated to the Brazilian population. Abbreviations: GE, general
electric; RHD, rheumatic heart disease. Nursing Care of Patients with Life Threatening Conditions,
High Acuity Situat. Population genome project initiatives in developing countries and the Middle
East, such as Qatar Genome ( 91 ), are expected to provide good reference data to help with these
efforts. Polymorphisms in the MASP1 gene are associated with serum levels of MASP-1, MASP-3,
and MAp44. In Brazil, studies about the association between genetic polymorphisms and cytokine
expression, and unfavorable outcomes of latent and clinical RHD (progression to clinical disease and
severe valve involvement requiring intervention, respectively) have been conducted. Same as above
2minor manifestation plus evidence of GAS infection. Even though it has been generally accepted
that systemic activity is related to chronic inflammation and accelerated pathogenic processes
leading to cardiovascular compromise, it is important to assess other novel factors in patients that
may also contribute. The inadequacy of world health organization criteria for optimizing.
Calcification-competent extracellular vesicles derived from. Mannan-binding lectin-associated serine
protease (MASP)-1 is crucial for lectin pathway activation in human serum, whereas neither MASP-1
nor MASP-3 is required for alternative pathway function. B cell activation and differentiation: the
recognition of C3-tagged antigen plays an important role in B cell activation and differentiation.
Globally, GAS upper respiratory tract infections are only exceeded by HIV, tuberculosis, and malaria
in terms of consequent morbidity and mortality ( 1 ). However, the authors suggested a putative role
for MBL deficiency in the progression of RHD, by considering the age of onset of heart disease.
Wyber R, et al. Rheumatic heart disease worldwide: Jacc scientific. This is an open-access article
distributed under the terms of the Creative Commons Attribution License (CC BY). A 3D
confirmatory standard echocardiogram showing severe mitral valve stenosis, associated with marked
morphological abnormalities in a first-degree relative with the indication of mitral commissurotomy
at the time of diagnosis. This entails management of heart failure, control of arrhythmias. The global
burden of group A streptococcal diseases. Systemic vasculitis was observed in 26 cases (15.38%)
among 169 patients with RA.