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Trial registered on ANZCTR


Registration number
ACTRN12608000508370
Ethics application status
Approved
Date submitted
16/09/2008
Date registered
30/09/2008
Date last updated
3/07/2012
Type of registration
Retrospectively registered

Titles & IDs
Public title
Importance of dermatological examination of patients with Marfan Syndrome: A case-controlled prospective study
Scientific title
Importance of dermatological examination of patients with Marfan Syndrome to evaluate the diagnostic value of dermatological signs: A case-controlled prospective study
Universal Trial Number (UTN)
Trial acronym
Linked study record

Health condition
Health condition(s) or problem(s) studied:
skin symptoms in Marfan syndrome patients 3706 0
Condition category
Condition code
Skin 3871 3871 0 0
Other skin conditions

Intervention/exposure
Study type
Observational
Patient registry
Target follow-up duration
Target follow-up type
Description of intervention(s) / exposure
One structured interview and one complete standardized dermatological clinical examination: Interview included date of birth, age of rapid pubertal growth and/or weight gain(s) or loss(es) of more than 5 kg, age of striae appearence, history of traumata, surgery, relation to scars, medical history specially medication (cortisone), hernia. Dermatological examination included search for all visible skin manifestations having been described in literature as linked to Marfan Syndrome: striae, hypertrophic, hernia, large or atrophic surgical or post-traumatic scars, cutis laxa, elastosis perforans serpiginosa, fibroma, pterigion, poliosis, abnormal pigmentation, purpura, auricular dysplasia, or inter-digital membranes, number and localization of striae. Duration of interview and examination approximately 20 minutes. Patients were seen between april 2003 and november 2006.
Intervention code [1] 3418 0
Diagnosis / Prognosis
Comparator / control treatment
matched subjects do not have Marfan Syndrome. No treatment is given in the context of this study. Duration of interview and examination approximately 20 minutes. Patients were seen once between april 2003 and november 2006.
Control group
Active

Outcomes
Primary outcome [1] 4776 0
sensitivity and specificity of skin symptoms related in the above described standardised questionnaire and dermatological examination. Information from questionnaire and examination was related on a predefined spreadsheet. Striae were related with their number, and localisation.
Timepoint [1] 4776 0
During the complete standardized dermatological clinical examination.
Secondary outcome [1] 8070 0
sensitivity and specificity of others skin symptoms. Dermatological examination included search for all visible skin manifestations having been described in literature as linked to MS : striae, hypertrophic, hernia, large or atrophic surgical or post-traumatic scars, cutis laxa, elastosis perforans serpiginosa, fibroma, pterigion, poliosis, abnormal pigmentation, purpura, auricular dysplasia, or inter-digital membranes.
Timepoint [1] 8070 0
During the complete standardized dermatological clinical examination.

Eligibility
Key inclusion criteria
marfan syndrome and control group paired on sex, height and age
Minimum age
18 Years
Maximum age
65 Years
Gender
Both males and females
Can healthy volunteers participate?
Yes
Key exclusion criteria
children

Study design
Purpose
Screening
Duration
Cross-sectional
Selection
Case control
Timing
Prospective
Statistical methods / analysis

Recruitment
Recruitment status
Completed
Date of first participant enrolment
Anticipated
Actual
Date of last participant enrolment
Anticipated
Actual
Date of last data collection
Anticipated
Actual
Sample size
Target
Accrual to date
Final
Recruitment outside Australia
Country [1] 1200 0
France
State/province [1] 1200 0

Funding & Sponsors
Funding source category [1] 3906 0
Hospital
Name [1] 3906 0
Ambroise Pare Hospital
Address [1] 3906 0
9 avenue Charles de Gaulle
92100 Boulogne
Country [1] 3906 0
France
Primary sponsor type
Hospital
Name
Department of Dermatology
Address
Ambroise Paré Hospital
9 avenue Charles de Gaulle
92100 Boulogne
Country
France
Secondary sponsor category [1] 3488 0
None
Name [1] 3488 0
Address [1] 3488 0
Country [1] 3488 0

Ethics approval
Ethics application status
Approved
Ethics committee name [1] 6011 0
CPP = Comite de Protection des Personnes
Ethics committee address [1] 6011 0
Hopital Tarnier-Cochin
89 rue d'Assas
75006 Paris
Ethics committee country [1] 6011 0
France
Date submitted for ethics approval [1] 6011 0
17/09/2008
Approval date [1] 6011 0
17/09/2008
Ethics approval number [1] 6011 0
Ethics committee name [2] 6017 0
Ethics committee address [2] 6017 0
Ethics committee country [2] 6017 0
Date submitted for ethics approval [2] 6017 0
Approval date [2] 6017 0
16/09/2008
Ethics approval number [2] 6017 0

Summary
Brief summary
Various skin manifestations have been reported in literature for MS without knowing their real diagnostic value and are frequently incompletely described. Some of them are common in the general population and physicians may miss their diagnostic significance. For the moment, only striae atrophica (stretch marks) without marked weight gain, pregnancy or repetitive stress and recurrent or incisional herniae are considered as skin and integument minor criteria for diagnosis of MS. As they are frequent, even dermatologists may miss MS diagnosis. On the other hand, the precise characterization of dermatological signs of MS is the first step to refine the definition of the expanding spectrum of MS-related disorders, whatever their biological origin (fibrillinopathies and TGFbeta signalopathies).
The main objective of this carefully designed prospective case-control study was to characterize precisely the diagnostic value of the dermatological signs reported to date as linked to MS, and specially the value of striae.
Trial website
Trial related presentations / publications
Public notes

Contacts
Principal investigator
Name 28943 0
Address 28943 0
Country 28943 0
Phone 28943 0
Fax 28943 0
Email 28943 0
Contact person for public queries
Name 12100 0
Dr Monica Ledoux
Address 12100 0
Department of Dermatology
Ambroise Paré Hospital
9 avenue Charles de Gaulle
92100 Boulogne
Country 12100 0
France
Phone 12100 0
33 1 49 09 56 68
Fax 12100 0
Email 12100 0
monica.ledoux@wanadoo.fr
Contact person for scientific queries
Name 3028 0
Dr Monica Ledoux
Address 3028 0
Department of Dermatology
Ambroise Paré Hospital
9 avenue Charles de Gaulle
92100 Boulogne
Country 3028 0
France
Phone 3028 0
33 1 49 09 56 68
Fax 3028 0
Email 3028 0
monica.ledoux@wanadoo.fr

No information has been provided regarding IPD availability
Summary results
Have study results been published in a peer-reviewed journal?
Other publications
Have study results been made publicly available in another format?
Results – basic reporting
Results – plain English summary