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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
Case report
Full Version
Collagenofibrotic Glomerulopathy with Thrombotic Microangiopathy: A Rare Case Report
Correspondence Address :
Dr. Khushbu Agarwal,
D-104 Vishwanath Sopan Shela, Near Club 07, Ahmedabad, Gujarat, India.
E-mail: khushbuagarwal82@gmail.com
Collagenofibrotic Glomerulopathy (CG) is a very rare glomerular disease characterised by the massive accumulation of atypical type III collagen fibrils within the mesangial matrix and subendothelial space, as well as a marked increase in serum type III procollagen peptide levels. Owing to its rarity, the cause and pathogenesis of CG remain uncertain. While initial reports of CG were primarily in patients of Asian descent, subsequent cases in patients of European descent have also been published. Clinically, patients present with oedema and hypertension, often progressing to end-stage renal disease. Proteinuria is a cardinal manifestation of this disease. A definitive diagnosis is contingent upon recognising this entity, maintaining a high index of suspicion, and confirming it through immunohistochemistry and Electron Microscopy (EM), due to its rarity, non specific presenting signs and symptoms, and various differential diagnoses on histology. Here, a case of a 44-year-old non diabetic, hypertensive female who presented with pedal oedema and nephrotic range proteinuria. The biopsy showed nodular expansion of the mesangium by homogeneous, congo red-negative, eosinophilic material, along with vascular changes indicative of acute thrombotic microangiopathy. Many conditions, such as amyloidosis, fibrillary glomerulonephritis, diabetic nephropathy, fibronectin glomerulopathy, light chain deposition disease, and thrombotic microangiopathy, can cause nodular or membranoproliferative glomerular changes. The biopsy was initially reported as nodular glomerulosclerosis, which, upon EM, showed mesangial and subendothelial space expansion by banded fibrillary material. Further immunohistochemistry for type III collagen was performed, which yielded positive results. Thus, the diagnosis of CG was confirmed.
Electron microscopy, Proteinuria, Type III collagen
A 44-year-old female presented with the chief complaints of generalised weakness, persistent pedal oedema, and facial oedema for three months. The patient was hypertensive and non diabetic, with no other co-morbidities reported. There were no bony deformities noted. On investigations, the serum creatinine level was 4.02 mg/dL (normal range: 0.4-1.4 mg/dL). A 24-hour protein test revealed 6.22 grams of protein in 24 hours. Routine urine microscopy showed no sediments. Complement levels were normal. Serum lactate dehydrogenase levels were elevated at 354 U/L (normal range: 100-190 U/L). Serum haptoglobin levels were <0.0078 g/L (normal range: 0.5-2.2 g/L). Serum Antinuclear Antibodies (ANA), dsDNA, p-ANCA, c-ANCA (Antineutrophil cytoplasmic autoantibodies), Antibodies to the phospholipase A2 receptor (PLA2R), and anti-glomerular basement membrane (Anti-GBM) antibodies were all within normal limits. The patient was sero-negative for Human Immunodeficiency Virus (HIV), Hepatitis B surface Antigen (HbsAg) and Hepatitis C Virus (HCV). A peripheral smear revealed evidence of haemolysis. Thrombocytopenia was noted, with a platelet count of 108,000/cumm (normal range: 150,000-450,000/cumm). Serum protein electrophoresis results were normal. On ultrasonography, the right kidney measured 9.2×3.6 cm, and the left kidney measured 9.8×4.7 cm.
A renal biopsy was performed and showed 110 glomeruli, none of which were sclerosed. All glomeruli exhibited nodular mesangial expansion by an acellular eosinophilic material (Table/Fig 1)a, which was negative for Periodic Acid Schiff (PAS) and Jones Methenamine Silver (JMS) staining. There was no duplication of capillary membranes. Some of the tubules showed hyaline and cellular casts. The sampled cortex demonstrated approximately 35-40% interstitial fibrosis and tubular atrophy. A few small-calibre arteries were thrombosed, with segmental fibrinoid necrosis of the vessel wall; however, the glomeruli did not show any features of thrombotic microangiopathy. A few small-calibre arteries also revealed nodular/circumferential hyalinosis, while medium-calibre arteries revealed mild fibrointimal thickening.
In light of the nephrotic range proteinuria and nodular mesangial expansion by an acellular eosinophilic material, a histologic differential diagnosis of amyloidosis was considered first. However, the congo red stain was negative. Other differential diagnoses considered included diabetic glomerulopathy and light chain deposition disease. The absence of any history of diabetes and the lack of PAS positivity in the mesangial nodules ruled out diabetic nephropathy. Direct immunofluorescence was performed to exclude light chain deposition disease. Direct immunofluorescence tests showed negativity for all immunoglobulins, complements, and light chains; therefore, light chain deposition disease or any other immune complex deposition disease was excluded.
In view of nodular mesangial expansion and the negativity for congo red, PAS, and JMS, a diagnosis of CG was suggested after ruling out all other differential diagnoses. Additionally, findings of acute thrombotic microangiopathy involving only the vessels were also present. Immunohistochemistry was positive for Type III collagen within the glomerulus (Table/Fig 1)b. EM revealed marked expansion of the lamina rara interna and mesangium due to the massive accumulation of curvilinear bundles of collagen fibrils with frayed edges, exhibiting periodicity (Table/Fig 1)c,d. Thus, CG was confirmed as the final diagnosis.
Arakawa M et al., initially reported a novel glomerular condition involving collagen fibrils in the subendothelial region and mesangial matrix in 1979 (1). Later, in 1990, Ikeda K et al., used IHC staining to demonstrate that the collagen fibrils in the renal glomerulus were type III collagen fibrils (2). The formal designation of collagen type III glomerulopathy was given to this novel glomerular illness by Imbasciati E et al., in 1991 (3).
Collagen type III is not found in normal glomeruli; it is a component of the extracellular of many diverse glomerular lesions as they undergo fibrosis (4), which includes organising crescents, fibrosis surrounding the afferent arteriole as it enters the tufts (especially in older individuals), and the urinary spaces of ischaemic glomeruli. Additionally, it may occasionally be found in the mesangial matrix in segmental glomerulosclerosis and diabetic glomerulosclerosis (5).
Many conditions, such as amyloidosis and/or fibrillary glomerulonephritis, diabetic nephropathy, fibronectin glomerulopathy, light chain deposition disease, and thrombotic microangiopathy, can cause nodular or membranoproliferative glomerular changes. Additional conditions that cause collagen deposition include Nail Patella Syndrome (NPS) and hereditary multiple exostoses. For the diagnosis to be confirmed, EM is required.
There is no gender predilection for this illness; symptoms can appear at any age, but adults make up the majority of recorded cases. Nephrotic-range proteinuria is present in more than half of the patients. Upon presentation, hypertension and microscopic haematuria are often observed. Renal function gradually deteriorates, and approximately 60% of cases can advance to end-stage renal disease (3),(4),(6).
A significant number of cases have been reported from Japan and other Asian countries, with about 21 cases reported from India (7). The largest series has been reported by Kurien AA et al., from India and Gubler MC et al., from France, comprising eight adult cases and ten paediatric cases, respectively (7),(8). The latest case reports published in India were in 2020—one by Modi SS et al., and another by Manocha A et al., (9),(10). The classic histopathological features of CG include double contour formation by PAS-negative flocculent material extending along the luminal side of the peripheral capillary loops and mesangial enlargement. Immunofluorescence shows no staining. Furthermore, the diagnosis of CG is confirmed by EM, which reveals electron-dense, banded, curvilinear structures characteristic of collagen, exhibiting periodicity in the range of 58-61 nm within the enlarged mesangium and subendothelial region. This was similar to present case, and collagen III IHC further confirmed the diagnosis.
Present case also exhibited vascular changes indicative of acute thrombotic microangiopathy. The peripheral smear showed features of haemolysis, serum haptoglobin was low, and serum LDH was elevated. Gubler MC et al., described that three of the ten paediatric patients also had thrombotic microangiopathy (4). Vogt BA et al., described one patient with persistent hypocomplementemia and inherited factor H deficiency associated with collagen type III glomerulopathy, suggesting that factor H deficiency may be linked to collagen III glomerulopathy (5),(8). Nevertheless, the relationship between these abnormalities and CG remains uncertain.
In present case, testing for factor H was not performed, and the cause of thrombotic microangiopathy was unknown. The patient did not present with any bony deformities; therefore, no genetic workup was conducted for NPS. The patient is currently on steroids, and the last recorded serum creatinine was 8.51 mg/dL. The patient was lost to follow-up thereafter.
Diabetic glomerulosclerosis, light chain deposition disease, and amyloidosis were our primary differential diagnoses based on light microscopy. There was no history of diabetes or PAS positivity indicative of diabetic glomerulopathy. The Congo red stain was negative for amyloidosis in this instance, and there was no kappa or lambda positivity on immunofluorescence.
A clinical history of longstanding diabetes, the distribution of nodular lesions (whether uniform or variable), staining characteristics, immunofluorescence findings for immunoglobulins/complements/kappa-lambda light chains, and ultrastructural studies aid in differentiating among almost all differential diagnoses (11).
There is no established course of treatment for this illness. It may be helpful to treat symptoms like oedema and hypertension. Dialysis or kidney transplantation may be advised in cases of end-stage renal disease. As of now, no known recurrences have been reported in the literature (12).
The pathologist must maintain a high degree of suspicion and awareness of CG due to its extreme rarity. Before this diagnosis is established, several distinct histopathological differentials must be ruled out. Type III collagen deposits must be identified using IHC and EM. Furthermore, in order to precisely classify and validate the diagnosis, any deposition disease needs to be investigated using EM.
DOI: 10.7860/JCDR/2024/74756.20345
Date of Submission: Aug 23, 2024
Date of Peer Review: Sep 17, 2024
Date of Acceptance: Sep 26, 2024
Date of Publishing: Nov 01, 2024
AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Aug 23, 2024
• Manual Googling: Sep 19, 2024
• iThenticate Software: Sep 25, 2024 (14%)
ETYMOLOGY: Author Origin
EMENDATIONS: 5
- 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
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- HINARI Access to Research in Health Programme
- Indian Science Abstracts (ISA)
- Journal seek Database
- Popline (reproductive health literature)
- www.omnimedicalsearch.com