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

Users Online : 211838

AbstractCase ReportDiscussionConclusionKey MessageAcknowledgementReferences
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

Dr Mohan Z Mani

"Thank you very much for having published my article in record time.I would like to compliment you and your entire staff for your promptness, courtesy, and willingness to be customer friendly, which is quite unusual.I was given your reference by a colleague in pathology,and was able to directly phone your editorial office for clarifications.I would particularly like to thank the publication managers and the Assistant Editor who were following up my article. I would also like to thank you for adjusting the money I paid initially into payment for my modified article,and refunding the balance.
I wish all success to your journal and look forward to sending you any suitable similar article in future"



Dr Mohan Z Mani,
Professor & Head,
Department of Dermatolgy,
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."



Dr Kalyani R
Professor and Head
Department of Pathology
Sri Devaraj Urs Medical College
Sri Devaraj Urs Academy of Higher Education and Research , Kolar, Karnataka
On Sep 2018




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.
‘Knowledge is treasure of a wise man.’ The free access of this journal provides an immense scope of learning for the both the old and the young in field of medicine and dentistry as well. The multidisciplinary nature of the journal makes it a better platform to absorb all that is being researched and developed. The publication process is systematic and professional. Online submission, publication and peer reviewing makes it a user-friendly journal.
As an experienced dentist and an academician, I proudly recommend this journal to the dental fraternity as a good quality open access platform for rapid communication of their cutting-edge research progress and discovery.
I wish JCDR a great success and I hope that journal will soar higher with the passing time."



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.
Their prompt and timely response to review's query and the manner in which they have set the reviewing process helps in extracting the best possible scientific writings for publication.
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

Images in Medicine
Year : 2010 | Month : April | Volume : 4 | Issue : 2 | Page : 2354 - 2357 Full Version

Cleidocranial Dysplasia – Report Of a Case


Published: April 1, 2010 | DOI: https://doi.org/10.7860/JCDR/2010/.699
TELKAR S R *, KERUDI V V**, *** DODAMANI A, ****PRASHANTH V K

*(MDS )Senior Lecturer Dept. of Oral Medicine and Radiology, **(MDS),Reader, Dept. of Orthodontics,*** (MDS),Professor& Head, Dept. of Preventive & Community Dentistry,****(MDS)Reader, Department of Preventive & Community Dentistry, Institution: A C P M Dental College, Dhule, Maharashtra,(India)

Correspondence Address :
TELKAR S R,(MDS )Senior Lecturer Department of Oral Medicine and Radiology A C P M Dental College, Dhule, Maharashtra,Pin code 424001.(India)Email swaroopmds@yahoo.co.in
Phone no:+919766745495,+919404184816 Fax no: 02562279924

Abstract

Cleidocranial dysplasia is a rare congenital disorder of the bone, characterized by abnormalities of the clavicles, skull and jaw bones. It was first described by Pierre Marie and Paul Sainton in 1898 and since then, over 1000 cases have been documented in medical reports. It is a rare syndrome which is usually caused by an autosomal dominant gene, although 40% of the cases of CCD appear spontaneously, with no apparent genetic cause. The bony and dental features of CCD may be visualized on the radiographical images of the face and the skull and they are characteristic. We report here, a case of Cleidocranial dysplasia in an 18 year old patient, with emphasis on radiological findings.

Keywords

Cleidocranial dysplasia, impacted teeth, Marie Sainton Disease, Clavicle, Mutational dysostosis

Introduction
Cleidocranial dysplasia is a well known, rare syndrome which is usually caused by an autosomal dominant gene, usually caused by a mutation of the Core Binding Factor – α 1 gene, located at chromosome 6p21 (1),(2),(3).

CCD is also known as the Marie and Sainton disease, Mutational dysostosis and Cleidocranial dysostosis (4). The disorder was originally thought to involve the bones of intramembranous origin only, namely the bones of the skull, clavicles and flat bones. However, it is now known that the bones of endochondral ossification are also affected and that it is a generalized disorder of many skeletal structures and therefore, the substituted term ‘cleidocranial dysostosis’ was given (5).

CCD presents with skeletal defects of several bones, the most striking of which are the partial or complete absence of the clavicles, which allows the patient to approximate the shoulders anteriorly in the midline (6).

Case Report

An 18 year old male patient reported to our department with a chief complaint of pain in the lower jaw.

The head and neck examination revealed frontal and occipital bossing and the presence of a metopic suture. The clavicles were absent on both the sides and the patient was able to get his shoulders anteriorly in the midline when asked to do so (Table/Fig 1).

Examination of the oral cavity revealed over retained deciduous teeth and the presence of the first, second permanent molars and lower incisors in the entire oral cavity (Table/Fig 2). Dental caries was noted in relation to right mandibular 2nd molar and the tooth was tender on percussion.

Based on the clinical findings, the patient was provisionally diagnosed as Cleidocranialdysplasia and was therefore, subjected to routine radiological examination.

The panoramic radiograph of the patient revealed the presence of a total number of 56 teeth, including both erupted and unerrupted impacted teeth (Table/Fig 3). The overall size of the maxillary sinuses was reduced. The ascending ramus of the mandible was parallel sided and the coronoid process was pointed and curved distally. The zygomatic arches on both sides were drooping downwards on the ramus of the mandible.

The chest radiograph was characteristic in appearance, i.e. a narrow bell shaped thoracic cage, the complete absence of bilateral clavicles, small scapulae and small glenoids and the ribs were directed obliquely downwards (Table/Fig 4). The skull radiographs revealed open sutures, multiple wormian bones in the occipital region and sphenoids which showed the hypoplastic presence of a metopic suture (Table/Fig 5), (Table/Fig 6). The overall shape of the skull was of a ‘light bulb’, with midface deficiency and mild prognathism of the mandible.

Discussion

CCD is a rare congenital defect of autosomal dominant inheritance, with skeletal defects of several bones (5). The clavicles may be rudimentary, or completely absent (6). Delayed ossification of the cranial sutures and fontanneles, coupled with raised hydrocephalic pressure results in expansion of the cranial vault, leading to bossing of the frontal, parietal and occipital bones (5). Similar findings were noted in our case too.

According to Jensen and Kreiborg, the zygomatic bone is hypoplastic with the arch being thin or even discontinuous and having a characteristic downward bend (6).

Characteristically, patients with CCD show prolonged retention of deciduous dentition and delayed eruption of permanent teeth. As many as 63 unerrupted teeth have been documented in one patient (5). The panoramic radiograph of our patient revealed 56 teeth. Jensen and Kreiborg have suggested that supernumerary teeth form as a result of the activation of the remnants of the dental lamina which are left unresorbed during odontogenesis (7).

In terms of the dental management of CCD, several approaches have been reported over the years. The option of no treatment was common in the past. The current “state of the art” management involves a multistaged treatment protocol of the timely extraction of deciduous teeth, staged surgical removal of supernumerary teeth, exposure of selected unerrupted permanent teeth and orthodontic forced eruption (8). Our case has also been referred to the department of Oral and Maxillofacial Surgery and Orthodontics for further needful.

Conclusion

CCD is a condition which is usually present since birth, but is often missed or diagnosed through incidental findings by the physician, the same as in our case.

Key Message

Oral radiologists play an important role in diagnosing systemic diseases which are manifested in the jaws.

Acknowledgement

Dr Tushar Nagle, A C P M Dental College, Dhule, Maharashtra,(India). Dr Patvardhan (MD), Department of Radiology and Imaging, A C P M Medical College and Hospital, Dhule, Maharashtra, (India)

References

1.
Jefferson L O Tanaka, Evelise Ono, Edmundo Medici Filho, Julio C. M. Castilho, Luiz C. Moraes and Mari E. L. Moraes. Cleidocranial dysplasia: importance of radiographic images in diagnosis of the condition. Journal of Oral Science 2006: 48(3): 161-66.
2.
Sapp JP, Eversole LR, Wysocki GP (1997). Contemporary Oral and Maxillofacial Pathology. Mosby, St. Louis, 30-31.
3.
Neville BW (1995). Oral and Mxillofacial Pathology. WB Saunders, Philadelphia, 445-46.
4.
Kalliala E, Takinen PJ. Cleidocranial dysostosis:repot of six typical cases and one atypical case. Oral Surg Oral Med Oral Pathol 1962; 14: 808.
5.
Rajeev Kumar Garg and Prachi Agarwal. Clinical spectrum of Cleidocranial dysplasia: a case report. Cases journal 2008; 1:377.
6.
CM McNamara, BC O’ Riordan, M Blake and JR Sandy. Cleidocranial dysplasia: radiological appearances on dental panoramic radiography. Dentomaxillofacial Radiology 1999; 28: 89-97.
7.
Hemalatha R and Balasubramaniam M R. Cleidocranial dysplasia: A case report. J Indian Soc Prevent Dent 2008, March, 40-43.
8.
John Daskalogiannakis, Luis Piedade, Tom C. Lindholm, George K.B.Sandor and Robrt P.Carmichael. J Can Dent Assoc 2006; 72(4):337-42.

JCDR is now Monthly and more widely Indexed .
  • 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
  • Google
  • Popline (reproductive health literature)
  • www.omnimedicalsearch.com