Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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Dr Mohan Z Mani

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Believers Church Medical College,
Thiruvalla, Kerala
On Sep 2018




Prof. Somashekhar Nimbalkar

"Over the last few years, we have published our research regularly in Journal of Clinical and Diagnostic Research. Having published in more than 20 high impact journals over the last five years including several high impact ones and reviewing articles for even more journals across my fields of interest, we value our published work in JCDR for their high standards in publishing scientific articles. The ease of submission, the rapid reviews in under a month, the high quality of their reviewers and keen attention to the final process of proofs and publication, ensure that there are no mistakes in the final article. We have been asked clarifications on several occasions and have been happy to provide them and it exemplifies the commitment to quality of the team at JCDR."



Prof. Somashekhar Nimbalkar
Head, Department of Pediatrics, Pramukhswami Medical College, Karamsad
Chairman, Research Group, Charutar Arogya Mandal, Karamsad
National Joint Coordinator - Advanced IAP NNF NRP Program
Ex-Member, Governing Body, National Neonatology Forum, New Delhi
Ex-President - National Neonatology Forum Gujarat State Chapter
Department of Pediatrics, Pramukhswami Medical College, Karamsad, Anand, Gujarat.
On Sep 2018




Dr. Kalyani R

"Journal of Clinical and Diagnostic Research is at present a well-known Indian originated scientific journal which started with a humble beginning. I have been associated with this journal since many years. I appreciate the Editor, Dr. Hemant Jain, for his constant effort in bringing up this journal to the present status right from the scratch. The journal is multidisciplinary. It encourages in publishing the scientific articles from postgraduates and also the beginners who start their career. At the same time the journal also caters for the high quality articles from specialty and super-specialty researchers. Hence it provides a platform for the scientist and researchers to publish. The other aspect of it is, the readers get the information regarding the most recent developments in science which can be used for teaching, research, treating patients and to some extent take preventive measures against certain diseases. The journal is contributing immensely to the society at national and international level."



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Professor and Head
Department of Pathology
Sri Devaraj Urs Medical College
Sri Devaraj Urs Academy of Higher Education and Research , Kolar, Karnataka
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Dr. Saumya Navit

"As a peer-reviewed journal, the Journal of Clinical and Diagnostic Research provides an opportunity to researchers, scientists and budding professionals to explore the developments in the field of medicine and dentistry and their varied specialities, thus extending our view on biological diversities of living species in relation to medicine.
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Dr Saumya Navit
Professor and Head
Department of Pediatric Dentistry
Saraswati Dental College
Lucknow
On Sep 2018




Dr. Arunava Biswas

"My sincere attachment with JCDR as an author as well as reviewer is a learning experience . Their systematic approach in publication of article in various categories is really praiseworthy.
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It's a honour and pride to be a part of the JCDR team. My very best wishes to JCDR and hope it will sparkle up above the sky as a high indexed journal in near future."



Dr. Arunava Biswas
MD, DM (Clinical Pharmacology)
Assistant Professor
Department of Pharmacology
Calcutta National Medical College & Hospital , Kolkata




Dr. C.S. Ramesh Babu
" Journal of Clinical and Diagnostic Research (JCDR) is a multi-specialty medical and dental journal publishing high quality research articles in almost all branches of medicine. The quality of printing of figures and tables is excellent and comparable to any International journal. An added advantage is nominal publication charges and monthly issue of the journal and more chances of an article being accepted for publication. Moreover being a multi-specialty journal an article concerning a particular specialty has a wider reach of readers of other related specialties also. As an author and reviewer for several years I find this Journal most suitable and highly recommend this Journal."
Best regards,
C.S. Ramesh Babu,
Associate Professor of Anatomy,
Muzaffarnagar Medical College,
Muzaffarnagar.
On Aug 2018




Dr. Arundhathi. S
"Journal of Clinical and Diagnostic Research (JCDR) is a reputed peer reviewed journal and is constantly involved in publishing high quality research articles related to medicine. Its been a great pleasure to be associated with this esteemed journal as a reviewer and as an author for a couple of years. The editorial board consists of many dedicated and reputed experts as its members and they are doing an appreciable work in guiding budding researchers. JCDR is doing a commendable job in scientific research by promoting excellent quality research & review articles and case reports & series. The reviewers provide appropriate suggestions that improve the quality of articles. I strongly recommend my fraternity to encourage JCDR by contributing their valuable research work in this widely accepted, user friendly journal. I hope my collaboration with JCDR will continue for a long time".



Dr. Arundhathi. S
MBBS, MD (Pathology),
Sanjay Gandhi institute of trauma and orthopedics,
Bengaluru.
On Aug 2018




Dr. Mamta Gupta,
"It gives me great pleasure to be associated with JCDR, since last 2-3 years. Since then I have authored, co-authored and reviewed about 25 articles in JCDR. I thank JCDR for giving me an opportunity to improve my own skills as an author and a reviewer.
It 's a multispecialty journal, publishing high quality articles. It gives a platform to the authors to publish their research work which can be available for everyone across the globe to read. The best thing about JCDR is that the full articles of all medical specialties are available as pdf/html for reading free of cost or without institutional subscription, which is not there for other journals. For those who have problem in writing manuscript or do statistical work, JCDR comes for their rescue.
The journal has a monthly publication and the articles are published quite fast. In time compared to other journals. The on-line first publication is also a great advantage and facility to review one's own articles before going to print. The response to any query and permission if required, is quite fast; this is quite commendable. I have a very good experience about seeking quick permission for quoting a photograph (Fig.) from a JCDR article for my chapter authored in an E book. I never thought it would be so easy. No hassles.
Reviewing articles is no less a pain staking process and requires in depth perception, knowledge about the topic for review. It requires time and concentration, yet I enjoy doing it. The JCDR website especially for the reviewers is quite user friendly. My suggestions for improving the journal is, more strict review process, so that only high quality articles are published. I find a a good number of articles in Obst. Gynae, hence, a new journal for this specialty titled JCDR-OG can be started. May be a bimonthly or quarterly publication to begin with. Only selected articles should find a place in it.
An yearly reward for the best article authored can also incentivize the authors. Though the process of finding the best article will be not be very easy. I do not know how reviewing process can be improved. If an article is being reviewed by two reviewers, then opinion of one can be communicated to the other or the final opinion of the editor can be communicated to the reviewer if requested for. This will help one’s reviewing skills.
My best wishes to Dr. Hemant Jain and all the editorial staff of JCDR for their untiring efforts to bring out this journal. I strongly recommend medical fraternity to publish their valuable research work in this esteemed journal, JCDR".



Dr. Mamta Gupta
Consultant
(Ex HOD Obs &Gynae, Hindu Rao Hospital and associated NDMC Medical College, Delhi)
Aug 2018




Dr. Rajendra Kumar Ghritlaharey

"I wish to thank Dr. Hemant Jain, Editor-in-Chief Journal of Clinical and Diagnostic Research (JCDR), for asking me to write up few words.
Writing is the representation of language in a textual medium i e; into the words and sentences on paper. Quality medical manuscript writing in particular, demands not only a high-quality research, but also requires accurate and concise communication of findings and conclusions, with adherence to particular journal guidelines. In medical field whether working in teaching, private, or in corporate institution, everyone wants to excel in his / her own field and get recognised by making manuscripts publication.


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

Important Notice

Case report
Year : 2012 | Month : February | Volume : 6 | Issue : 1 | Page : 128 - 129

Eagle- Barrett Syndrome: A Case Report

Chapay Soren, Paramananda Pujhari, P.V. Subbarao

1. Assistant Professor of Pediatrics, 2. Assistant professor of Pediatrics, 3. Professor & Head of Pediatrics, Konaseema Institute of Medical Sciences, Chaitanya Nagar Amalapuram, East Godavari dist, Andhra Pradesh-533201, India.

Correspondence Address :
DR. Chapay Soren MD, Assistant Professor of Pediatrics
Konaseema Institute of Medical Sciences, Chaitanya Nagar
Amalapuram, East Godavari dist, Andhra Pradesh-533201, India.
Phone: 09440106344, E-mail: drcsoren55@yahoo.co.in

Abstract

Eagle-Barrett syndrome is a rare congenital anomaly of uncertain aetiology almost exclusive to males. It is characterized by the triad of absent or incomplete abdominal musculature, undescended testes, and urinary tract abnormalities. A male baby with above characteristics triad was born in our hospital. A diagnosis of Eagle-Barrett Syndrome was made. This was undertaken in order to highlight the occurrence of this rare syndrome in our environment and to review its pathogenesis, presentation and management approach.

Keywords

Urinary, Paediatrics, Renal Failure

INTRODUCTION
Eagle-Barrett Syndrome, also known as triad syndrome or Prune belly syndrome, represents a spectrum of anomalies with variable degrees of severity predominantly affecting males. It is a rare condition characterized by the triad of absent or incomplete abdominal musculature, bilateral cryptorchidism and urinary tract abnormalities. It is caused by urethral obstruction early in development resulting in massive bladder distension and urinary ascites, leading to degeneration of the abdominal wall musculature and failure of testicular descent. The impaired elimination of urine from the bladder leads to oligohydramnios, pulmonary hypoplasia, and Potter’s facies. The urinary tract may have variable degrees of hydronephrosis, renal dysplasia, dilated tortuous ureters, an enlarged bladder, and a dilated prostatic urethra. The exact aetiology of EBS is unknown.The case is reported for its rare congenital abnormality.

Case Report

A 3.5kg male baby was delivered in our hospital by an un-booked 29- year old Gravida 2, Para 1, Live 1 mother with an APGAR score of 5 at 1minutes and 10 at 5 minutes after birth. Abdominal examination revealed huge distension with thin and wrinkled skin protruding most prominently in the right side with visible bowel loops.(Table/Fig 1) Both the kidneys are palpable. Perineal examination showed bilateral cryptorchidism and hypospadiasis. Cardiac examination was normal by clinical examination and echocardiography. Investigations showed hyponatremia with Na of 130meq/l, urea of 65mg/ dl, and creatinine of 1.5mg/dl. Ultrasound imaging showed mild hydronephrosis with gross dilatation pelvicalyceal system of right kidney and moderate hydronephrosis of left kidney with grossly dilated ureters, and distended bowel loops.

Discussion

Eagle-Barrett syndrome is a rare congenital disorder predominantly affecting males with male:female ratio being 2.0:1 (1). The incidence has been reported to be ranged 1 in 29,000 to 1 in 40,000 live births (2). Parker et al. first recognized the three components of this syndrome (3). The characteristic triad consists of absent or incomplete abdominal musculature, bilateral cryptorchidism and urinary tract abnormalities (4), (5). The exact aetiology is not known, however some of the studies reveal the possibility of genetic inheritance (6). Along with the classical triad broad spectrum of defects including musculoskeletal, cardiovascular, pulmonary, gastrointestinal and genital malformations have been documented (7). When the urinary tract maldevelopment is associated with severe obstructive uropathy, this syndrome can lead to oligohydramnios and pulmonary hypoplasia. The pathogenesis of Eagle-Barrett syndrome is not clearly known. The mesodermal defect theory suggests that a defect exists in the mesoderm of the anterior abdominal wall and urinary tract. Between 6 and 10 weeks of gestation, aberrant development of the derivatives of the first lumbar myotome leads to a patchy muscular deficiency or hypoplasia of the abdominal wall as well as to urinary tract abnormalities (8), (9). An alternate theory, the urethral obstruction malformation complex, proposes that pressure atrophy of the abdominal wall muscles occurs when urethral obstruction leads to massive distension of the bladder and ureters. Bladder distension would also interfere with descent of the testes and thus be responsible for the bilateral cryptorchidism. This mechanism is responsible for the urinary tract dilatation and distension (10). The higher incidence of this syndrome in males has been explained on the basis of the more complex morphogenesis of the male urethra, possibly resulting in obstructive anomalies at several levels. Ultrasound, plain X-ray, and intravenous pyelogram are more useful investigations to diagnose the condition. Many neonates with Eagle-Barrett syndrome have difficulty with effective bladder emptying because the bladder musculature is poorly developed, and the urethra may be narrowed. When no obstruction is present, the goal of treatment is the prevention of urinary tract infection with antibiotic prophylaxis. When obstruction of the ureters or urethra is demonstrated, temporary drainage procedures, such as a vesicostomy, may help to preserve renal function until the child is old enough for surgery. Urinary tract infections occur often and should be treated promptly. Correction of the undescended testes by orchidopexy can be difficult in these children because the testes are located high in the abdomen and is best accomplished in the first 6months of life. Reconstruction of the abdominal wall (abdominoplasty) offers cosmetic and functional benefits. The prognosis ultimately depends on the degree of pulmonary hypoplasia and renal dysplasia. The most common complication is chronic renal failure that occurs in 25–30% of cases. Many infants are either stillborn or die within the first few weeks of life from severe lung or kidney problems, or a combination of congenital anomalies. There are cases of Eagle-Barrett syndrome who survived into adult life after abdominal reconstruction and urinary tract repair (11). There is no known prevention but the routine use of screening for fetal anomalies is helpful. If an antenatal diagnosis of urinary obstruction is made it may be possible to perform intra-uterine surgery to prevent the development of Eagle-Barrett syndrome (12). Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course. These patients need multidisciplinary management of a neonatologist, nephrologists, and pediatric urologist for an optimal outcome.

References

1.
Rabinowitz R, Schillinger JF. Prune Prune belly syndrome in the female subject. J Urol 1977;115:454-56.
2.
Greskovich FJ 3rd, Nyberg LM Jr. The prune belly syndrome: a review of its aetiology, defects, treatment and prognosis. J Urol 1988;140:707-12.
3.
Parker RW. Case of an infant in whom some of the abdominal muscles were absent. Trans Clin Soc Lond 1895;28:201-03.
4.
Manivel JC, Pettinato G, Reinberg Y, Gonzalez R, Burke B, Dehner LP, “Prune belly syndrome: clinicopathologic study of 29 cases,” Paediatric Pathology,1989;9(6):691-711.
5.
Eagle JF, Barrett GS. Congenital deficiency of abdominal musculature with associated genitourinary abnormalities: A syndrome. Report of 9 cases. Paediatrics. Nov 1950;6(5):721-36.
6.
Ramasamy R, Haviland M., Woodard JR., and Barone JG., “Patterns of inheritance in familial prune belly syndrome,” Urology.2005;65(6):1227.
7.
Salihu HM, Tchuinguem G, Aliyu MH, and Kouam L, “Prune belly syndrome and associated malformations: a 13- year experience from a developing country,” West Indian Medical Journal.2003;52(4):281- 84.
8.
Stephens FD, Gupta D. Pathogenesis of the prune belly syndrome. J Urol 1994;152:2328-31
9.
Greskovich F. J. and Nyberg L. M., “The prune belly syndrome: a review of itsaetiology, defects, treatment and prognosis,” Journal of Urology.1988;140(4):395-98.
10.
Reinberg Y, Shapiro E, Manivel JC, Manley CB, Pettinato G, Gonzalez R, “Prune belly syndrome in females: a triad of abdominal musculature deficiency and anomalies of the urinary and genital systems,” Journal of Paediatrics.1991;118(3):395-98.
11.
Woodhouse CRJ, “Prospects for fertility in patients born with genitourinary anomalies,” Journal of Urology.2001;165(6):2354-60.
12.
Leeners B, Sauer I, Schefels J, Cotarelo CL, and Funk A, “Prunebelly syndrome: therapeutic options including in utero placement of a vesicoamniotic shunt,” Journal of Clinical Ultrasound. 2000;28(9):500- 07.

Tables and Figures
[Table / Fig - 1]
DOI and Others

ID: JCDR/2012/3823:1878

FINANCIAL OR OTHER COMPETING INTERESTS: NONE.

Date of Submission: Nov 30, 2011
Date of Peer Review: Dec 26, 2011
Date of Acceptance: Jan 15, 2012
Date of Publishing: Feb 15, 2012

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