Article in PDF
How to Cite
Citation Manager
Readers' Comments (0)
Audio Visual Article Statistics
Link
to PUBMED
Print this Article
Send to a Friend
Advertisers
Access Statistics Resources
Authors are the souls of any journal, and deserve much respect. To publish a journal manuscripts are needed from authors. Authors have a great responsibility for producing facts of their work in terms of number and results truthfully and an individual honesty is expected from authors in this regards. Both ways its true "No authors-No manuscripts-No journals" and "No journals–No manuscripts–No authors". Reviewing a manuscript is also a very responsible and important task of any peer-reviewed journal and to be taken seriously. It needs knowledge on the subject, sincerity, honesty and determination. Although the process of reviewing a manuscript is a time consuming task butit is expected to give one's best remarks within the time frame of the journal.
Salient features of the JCDR: It is a biomedical, multidisciplinary (including all medical and dental specialities), e-journal, with wide scope and extensive author support. At the same time, a free text of manuscript is available in HTML and PDF format. There is fast growing authorship and readership with JCDR as this can be judged by the number of articles published in it i e; in Feb 2007 of its first issue, it contained 5 articles only, and now in its recent volume published in April 2011, it contained 67 manuscripts. This e-journal is fulfilling the commitments and objectives sincerely, (as stated by Editor-in-chief in his preface to first edition) i e; to encourage physicians through the internet, especially from the developing countries who witness a spectrum of disease and acquire a wealth of knowledge to publish their experiences to benefit the medical community in patients care. I also feel that many of us have work of substance, newer ideas, adequate clinical materials but poor in medical writing and hesitation to submit the work and need help. JCDR provides authors help in this regards.
Timely publication of journal: Publication of manuscripts and bringing out the issue in time is one of the positive aspects of JCDR and is possible with strong support team in terms of peer reviewers, proof reading, language check, computer operators, etc. This is one of the great reasons for authors to submit their work with JCDR. Another best part of JCDR is "Online first Publications" facilities available for the authors. This facility not only provides the prompt publications of the manuscripts but at the same time also early availability of the manuscripts for the readers.
Indexation and online availability: Indexation transforms the journal in some sense from its local ownership to the worldwide professional community and to the public.JCDR is indexed with Embase & EMbiology, Google Scholar, Index Copernicus, Chemical Abstracts Service, Journal seek Database, Indian Science Abstracts, to name few of them. Manuscriptspublished in JCDR are available on major search engines ie; google, yahoo, msn.
In the era of fast growing newer technologies, and in computer and internet friendly environment the manuscripts preparation, submission, review, revision, etc and all can be done and checked with a click from all corer of the world, at any time. Of course there is always a scope for improvement in every field and none is perfect. To progress, one needs to identify the areas of one's weakness and to strengthen them.
It is well said that "happy beginning is half done" and it fits perfectly with JCDR. It has grown considerably and I feel it has already grown up from its infancy to adolescence, achieving the status of standard online e-journal form Indian continent since its inception in Feb 2007. This had been made possible due to the efforts and the hard work put in it. The way the JCDR is improving with every new volume, with good quality original manuscripts, makes it a quality journal for readers. I must thank and congratulate Dr Hemant Jain, Editor-in-Chief JCDR and his team for their sincere efforts, dedication, and determination for making JCDR a fast growing journal.
Every one of us: authors, reviewers, editors, and publisher are responsible for enhancing the stature of the journal. I wish for a great success for JCDR."
Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
E-mail: drrajendrak1@rediffmail.com
On May 11,2011
Dr. Shankar P.R.
"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
Most of the manuscripts are published within 3 to 4 months of their submission if they are found to be suitable after the review process. JCDR is published bimonthly and the accepted articles were usually published in the next issue. Recently, due to the increased volume of the submissions, the review process has become slower and it ?? Section can take from 4 to 6 months for the articles to be reviewed. The journal has an extensive author support system and it has recently introduced a paid expedited review process. The journal also mentions the average time for processing the manuscript under different submission systems - regular submission and expedited review.
Strengths of the journal: The journal has an online first facility in which the accepted manuscripts may be published on the website before being included in a regular issue of the journal. This cuts down the time between their acceptance and the publication. The journal is indexed in many databases, though not in PubMed. The editorial board should now take steps to index the journal in PubMed. The journal has a system of notifying readers through e-mail when a new issue is released. Also, the articles are available in both the HTML and the PDF formats. I especially like the new and colorful page format of the journal. Also, the access statistics of the articles are available. The prepublication and the manuscript tracking system are also helpful for the authors.
Areas for improvement: In certain cases, I felt that the peer review process of the manuscripts was not up to international standards and that it should be strengthened. Also, the number of manuscripts in an issue is high and it may be difficult for readers to go through all of them. The journal can consider tightening of the peer review process and increasing the quality standards for the acceptance of the manuscripts. I faced occasional problems with the online manuscript submission (Pre-publishing) system, which have to be addressed.
Overall, the publishing process with JCDR has been smooth, quick and relatively hassle free and I can recommend other authors to consider the journal as an outlet for their work."
Dr. P. Ravi Shankar
KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
E-mail: ravi.dr.shankar@gmail.com
On April 2011 Anuradha
Dear team JCDR, I would like to thank you for the very professional and polite service provided by everyone at JCDR. While i have been in the field of writing and editing for sometime, this has been my first attempt in publishing a scientific paper.Thank you for hand-holding me through the process.
Dr. Anuradha
E-mail: anuradha2nittur@gmail.com
On Jan 2020
Original article / research
Full Version
Association of HbE Haemoglobinopathy in Patients with Systemic Lupus Erythematosus: A Cross-sectional Study
Correspondence Address :
Anuradha Kamanna,
RIMS Girls Hostel, Lamphelpat, Imphal-795004, Manipur, India.
E-mail: anuradhamaths13@gmail.com
Introduction: Systemic Lupus Erythematosus (SLE) is a chronic inflammatory disease of autoimmune origin that affects multiple systems, with the haematologic system being commonly involved. However, the co-existence of haemoglobinopathies and connective tissue disorders has rarely been investigated, and the available data on this matter are primarily anecdotal.
Aim: To determine the prevalence of Haemoglobin E (HbE) haemoglobinopathy in adult SLE patients and to assess the association of HbE haemoglobinopathy and SLE with disease activity.
Materials and Methods: A hospital-based cross-sectional study was conducted at the Department of General Medicine, Regional Institute of Medical Sciences (RIMS) Hospital, Imphal, Manipur, India, from April 2021 to July 2022. The study included SLE patients diagnosed during the study period who attended the rheumatology Outpatient Department (OPD). The independent variables were age, gender, occupation, religion, and family history, while HbE haemoglobinopathy, Mean Corpuscular Volume (MCV), Mean Corpuscular Haemoglobin (MCH), Red Blood Cell (RBC) count, and Systemic Lupus Activity Measure Revised Index (SLAM-R index) score were the dependent variables. Data were analysed using Statistical Package for Social Sciences (SPSS) version 21.0, with proportions analysed using the Chi-square test and Fisher’s-exact test, and means compared using Analysis of Variance (ANOVA) and independent t-test. A p-value of <0.05 was considered significant.
Results: Of the 105 SLE patients included in the study, 93% were females. The majority of participants (36.2%) were in the age group of 21-30 years. Twenty-five patients (23.8%) had HbE haemoglobinopathy. Anaemia and MCV were significantly associated with HbE patients. Among the 25 HbE patients, 24 (96%) had active SLE disease. Among the HbE negative patients, 55 (68.7%) had active disease, while 25 (31.3%) did not have any active disease. Active SLE disease was significantly associated with HbE haemoglobinopathy (p-value=0.002).
Conclusion: The overall prevalence of HbE haemoglobinopathy in SLE patients was found to be 24%. Hb levels and MCV levels were significantly lower in HbE patients. There was a significant association between active SLE disease and HbE haemoglobinopathy.
Anaemia, Disease activity, Haemoglobin E, Systemic lupus activity measure revised index score
The SLE is a chronic autoimmune illness characterised by various immunological abnormalities and the production of autoantibodies, which result in widespread inflammation and tissue damage to organs. Globally, the prevalence of SLE ranges from 6.5 to 178.0 per 100,000 people, depending on epidemiological data (1). Asians and individuals of African ancestry are more likely than Caucasians to develop SLE and experience more severe clinical symptoms of the disease (2). The clinical presentation and course of the disease vary among populations, indicating the importance of genetics in disease development (2). In India, the reported prevalence of SLE is 3.2 per 100,000 (3).
Haemoglobin is a tetramer composed of two alpha-like and two beta-like globin chains. Haemoglobinopathies are a group of inherited disorders affecting the globin portion of haemoglobin (4). HbE is the most common haemoglobin variant in Southeast Asia and Northeast India (5). It is an abnormal haemoglobin with a single point mutation where glutamic acid is replaced by lysine at position 26 of the beta-globin chain. HbE is the most prevalent abnormal haemoglobin variant in Southeast Asia, Northeast India, and the four different populations of Manipur, India. The Meitei population has the highest incidence of HbE, with 18.207% of individuals possessing HbβE (5). Among them, 15.81% are heterozygous, 1.917% are homozygous, and 0.48% are compound heterozygous. The incidence of HbE among the Hill Kabui population is 6.67%, with 6.30% being heterozygous and only 0.37% being homozygous. No homozygous individuals have been found in the remaining two populations. The percentage frequencies of heterozygous individuals in the latter are 5.75% and 2.44% among the Kiren and Smite populations, respectively (6).
The SLE is a chronic inflammatory disease of autoimmune origin that affects multiple systems, with the haematologic system being involved in most cases. SLE can cause anaemia through various mechanisms, including haemolytic anaemia. It can also cause thrombocytopenia, especially when associated with antiphospholipid antibody syndrome involving autoantibodies against platelets, glycoprotein IIb/IIIa, or the thrombopoietin receptor (6). Additionally, some patients with SLE may have another condition that can cause anaemia. It has been found that the prevalence of beta-thalassemia in patients with SLE is lower than that in the general population, but if co-existence occurs, SLE seems to have a more severe course (7).
Haematologic abnormalities are common in SLE, and all three blood cell lines can be affected. Anaemia of chronic disease is the most common type of anaemia in SLE patients, while autoimmune haemolytic anaemia is relatively rare, and severe thrombocytopenia is also rare (8). The co-existence of haemoglobinopathies and connective tissue disorders has rarely been investigated, and published data on this matter are only anecdotal. Although varying degrees of anaemia are a common finding in SLE, the issue of concomitant haemoglobinopathies has rarely been addressed (7). SLE associated with beta-thalassemia trait is rare, and concomitant disease tends to exhibit more severe systemic symptoms (9).
Complications appear to be more severe in SLE with beta-thalassemia (7). The combination of HbE/beta-thalassemia and SLE is even rarer, as reported in cases worldwide (10). Therefore, the purpose of the present study was to document the prevalence of HbE haemoglobinopathy in SLE and its association with SLE disease activity.
A hospital-based cross-sectional study was conducted from April 2021 to July 2022 in the Department of General Medicine, Regional Institute of Medical Sciences (RIMS) Hospital, Imphal, Manipur, India. Ethical approval was obtained from the Institutional Research Ethics Board before the study commenced (Ref No. A/206/REB-Comm(SP)/RIMS/2015/699/41/2020).
Inclusion and Exclusion criteria: Patients aged 18 years and above who attended the Medicine OPD, Rheumatology OPD, or were admitted to the medicine ward at RIMS Imphal and diagnosed with SLE during the study period were included. Those who refused to participate were excluded.
Sample size calculation: A sample size of 105 was calculated using the formula: n=4×PQ/L² (where P is prevalence, Q= 100-P, and L is the Absolute Allowable error of 6), based on the prevalence of HbE haemoglobin type in the Manipur population as 10.53%, from a study by Singh MR et al., (5).
Study Procedure
A consecutive sampling method was used to recruit participants. The independent variables were age, gender, occupation, religion, and duration of the disease. The dependent variables were HbE haemoglobinopathy, mean corpuscular volume, mean corpuscular haemoglobin, RBC count, and SLAM-R Index score. All eligible patients were informed about the study, and after obtaining informed written consent, a thorough clinical history was taken along with a complete physical examination and investigations. Modified Systemic Lupus International Collaborating Centres (SLICC) Criteria (11) were used to diagnose SLE, where the patient must satisfy four criteria out of 11 clinical criteria and six immunologic criteria, including atleast one clinical criterion and one immunologic criterion. Alternatively, if the patient has biopsy-proven nephritis compatible with SLE and positive Antinuclear Antibody (ANA) or anti double-stranded Deoxyribonucleic Acid (dsDNA) antibodies, they are also considered to have SLE. Disease activity was measured using the SLAM-R Index. The haemoglobin types were screened using high-performance liquid chromatography (12), along with known control samples.
Measurement of disease activity: Disease activity was measured using the SLAM-R Index, which assesses global disease activity within the previous month. It includes 23 clinical manifestations in nine organs/systems and seven laboratory features, with a possible range of 0 to 81. Each organ item may score 0 to 3 points if any of the clinical manifestations were present within the previous month (severity is incorporated into a higher score per item). Most items can score a maximum of three points, while a few items can score a maximum of one point. The laboratory category can score a maximum of 21 points. A score of seven or more was considered indicative of active disease activity (13).
Statistical Analysis
The collected data were entered and analysed using SPSS (IBM) version 21.0. Data summarisation was performed using descriptive statistics such as mean, median, standard deviation, and percentages. Categorical variables were analysed using either the Chi-square test or Fisher’s-exact test. A p-value <0.05 was considered statistically significant.
A total of 105 patients with SLE were included in the study. Ninety-eight participants (93%) were females, with a female:male ratio of 14:1. The mean age of the participants was 35.26 years, and the median age was 35 (31-40) years. As shown in (Table/Fig 1), the majority of participants (36.2%) were in the age group of 21-30 years, and only 6.6% were in the age group of 20 years or younger. As shown in (Table/Fig 2), the maximum number of patients (41%) had a duration of SLE between 2.1 to 5 years. The mean duration of SLE was 5.6 years and 59% of patients had a duration of SLE less than five years. The majority of patients were females (93.3%). The majority of the participants in the present study were Hindu (88.6%) and homemakers (73.3%). Twenty-five patients (24%) had HbE haemoglobinopathy, as shown in (Table/Fig 3). (Table/Fig 4) demonstrates a significant association between MCV (fL) and Hb% with HbE haemoglobinopathy. Hb% and MCV were significantly lower in HbE patients. The mean Hb% was 10.58% in HbE patients and 11.9% in non HbE patients, with a statistically significant difference (p-value=0.02). The mean MCV (fL) was 83 fL in HbE patients and 87.7 fL in non HbE patients, also with a statistically significant difference (p-value <0.001). The mean difference in all other parameters was not statistically significant. Anaemia was significantly associated with HbE positive patients, as shown in (Table/Fig 5). (Table/Fig 6) shows a significant association between active SLE disease and HbE haemoglobinopathy (based on SLAM-R index score). Out of 25 HbE patients, 24 (96%) had active SLE disease, while among the HbE negative patients, 55 (68.7%) had active disease and 25 (31.3%) did not have any active disease.
The SLE is a systemic autoimmune disease with variable multisystem involvement and heterogeneous clinical features (14). In the present study, 25 out of 105 patients (23.8%) with SLE were found to have HbE haemoglobinopathy. This prevalence rate is higher than that in the general population of the same areas, which was reported as 10.53% and 11.69% (5),(15). It is also twice the expected prevalence rate of 11.69%. However, a study by Castellino G et al., reported a lower prevalence of beta-thalassemia minor (9.6%) among SLE patients in Italy (7). This difference in findings suggests the need for further research in this area.
The mean age of the study population in the present study was 35.26 years, with a median age of 35 (31-40) years. In contrast, a study by Castellino G et al., reported a higher mean age of 54 years (7). In the present study, out of 105 patients, 98 were female and seven were male, accounting for 93% female patients and 7% male patients. This finding of female predominance is consistent with a study by Castellino G et al., where out of 177 SLE patients, 145 were females and 32 were males (7). The disease activity of SLE was measured using the SLAM-R Index. Among the 25 HbE patients, 24 (96%) had active SLE disease, while one (4%) did not. Among the HbE negative patients, 55 (68.7%) had the disease and 25 (31.3%) did not have any active disease. The association between active SLE disease and HbE haemoglobinopathy was statistically significant. Of the 25 HbE patients, 16 (64%) had anaemia, and the association between anaemia and HbE haemoglobinopathy was statistically significant (p=0.01). Most HbE patients were detected within 10 years of the duration of SLE. The mean duration of SLE was 6.32 years. The difference in age (in years) and duration of SLE (in years) between HbE patients and non HbE patients was not statistically significant.
In the present study, the MCV level was significantly lower in HbE patients. Parameters such as Total Leucocyte Count (TLC), platelet count, Erythrocyte Sedimentation Rate (ESR), Mean Corpuscular Haemoglobin (MCH), and Mean Corpuscular Haemoglobin Concentration (MCHC) did not show significant differences compared to non HbE patients. These variations may be influenced by the duration of the disease and treatment status. Further studies that take into account parameters such as disease duration and treatment status could provide more insight.
Based on these results, it is possible to suggest a relationship between HbE heterozygote subjects and SLE. However, caution is needed before drawing conclusions that HbE trait may have a facilitating effect on the development of SLE. When investigating a small number of patients, the association may be coincidental. Nevertheless, if confirmed, this finding may contribute to a better understanding of the biological aspects of HbE haemoglobinopathy and SLE. The mechanism through which the HbE trait may interfere with the development of SLE remains speculative. The immunological reactivity of HbE heterozygous subjects has not been studied, and the increased prevalence of HbE haemoglobinopathy in SLE patients may be related to genetically determined susceptibility or the influence of environmental factors that alter host susceptibility and facilitate SLE development. Further investigation of HbE heterozygotes with autoimmunity may provide valuable insights into the pathobiology of SLE and HbE haemoglobinopathy.
Limitation(s)
Limitation of the present study include the lack of measurement of anti-dsDNA titre, C3, and C4 complement levels. These factors, which directly affect the disease activity of SLE, may confound the findings of the present study. Additionally, the use of the SLAM-R index, which relies on subjective reporting of symptoms rather than objective documentation, may introduce bias. The index also gives equal weight to mild and severe organ disease activity without considering the significance of the organ involved.
The prevalence of HbE haemoglobinopathy in SLE patients was found to be 24%, with a higher proportion of female patients. There was a significant association between anaemia and HbE haemoglobinopathy. Hb levels and MCV levels were also significantly lower in HbE patients. The present study found a significant association between HbE haemoglobinopathy and SLE disease activity using the SLAM-R index, but further research is needed to explore these relationships using other scoring systems and considering other confounding factors.
DOI: 10.7860/JCDR/2023/65202.18395
Date of Submission: May 10, 2023
Date of Peer Review: May 23, 2023
Date of Acceptance: Jul 26, 2023
Date of Publishing: Sep 01, 2023
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was Ethics Committee Approval obtained for this study? Yes
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. No
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: May 11, 2023
• Manual Googling: Jun 17, 2023
• iThenticate Software: Jul 14, 2023 (11%)
ETYMOLOGY: Author Origin
EMENDATIONS: 6
- Emerging Sources Citation Index (Web of Science, thomsonreuters)
- Index Copernicus ICV 2017: 134.54
- Academic Search Complete Database
- Directory of Open Access Journals (DOAJ)
- Embase
- EBSCOhost
- Google Scholar
- HINARI Access to Research in Health Programme
- Indian Science Abstracts (ISA)
- Journal seek Database
- Popline (reproductive health literature)
- www.omnimedicalsearch.com