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A simple clinical model to estimate the probability of Marfan syndrome

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A simple clinical model to estimate the probability of Marfan syndrome. / Sheikhzadeh, S; Kusch, M L; Rybczynski, M; Kade, C; Keyser, B; Bernhardt, A M; Hillebrand, M; Mir, T S; Fuisting, B; Robinson, P N; Berger, J; Lorenzen, V; Schmidtke, J; Blankenberg, S; von Kodolitsch, Y.

in: QJM-INT J MED, Jahrgang 105, Nr. 6, 6, 06.2012, S. 527-535.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Sheikhzadeh, S, Kusch, ML, Rybczynski, M, Kade, C, Keyser, B, Bernhardt, AM, Hillebrand, M, Mir, TS, Fuisting, B, Robinson, PN, Berger, J, Lorenzen, V, Schmidtke, J, Blankenberg, S & von Kodolitsch, Y 2012, 'A simple clinical model to estimate the probability of Marfan syndrome', QJM-INT J MED, Jg. 105, Nr. 6, 6, S. 527-535. https://doi.org/10.1093/qjmed/hcs008

APA

Sheikhzadeh, S., Kusch, M. L., Rybczynski, M., Kade, C., Keyser, B., Bernhardt, A. M., Hillebrand, M., Mir, T. S., Fuisting, B., Robinson, P. N., Berger, J., Lorenzen, V., Schmidtke, J., Blankenberg, S., & von Kodolitsch, Y. (2012). A simple clinical model to estimate the probability of Marfan syndrome. QJM-INT J MED, 105(6), 527-535. [6]. https://doi.org/10.1093/qjmed/hcs008

Vancouver

Sheikhzadeh S, Kusch ML, Rybczynski M, Kade C, Keyser B, Bernhardt AM et al. A simple clinical model to estimate the probability of Marfan syndrome. QJM-INT J MED. 2012 Jun;105(6):527-535. 6. https://doi.org/10.1093/qjmed/hcs008

Bibtex

@article{f7554ea692e24c2cade48e6c8b8b2472,
title = "A simple clinical model to estimate the probability of Marfan syndrome",
abstract = "BACKGROUND: Marfan syndrome is a heritable connective tissue disease. Definitive diagnosis is complex, and requires sequencing of a large gene, FBN1.AIM: We aimed to develop a simple model to estimate the pre-test probability of Marfan syndrome.DESIGN: Prospective cross-sectional study.METHODS: We applied diagnostic standards for definitive diagnosis or exclusion of Marfan syndrome in 329 consecutive persons. In 208 persons with random assignment to our derivation group, we performed multivariate logistic regression to assess 14 clinical variables for inclusion in a prediction model with derivation of score points from the estimated coefficients. We created cut-offs to classify low, moderate and high probability of Marfan syndrome. For validation, we applied the model to the remaining 121 persons.RESULTS: We identified seven variables for inclusion in the final model, where we assigned four score points to ectopia lentis, two points to a family history of Marfan syndrome, and one point to previous thoracic aortic surgery, to pectus excavatum, to a wrist and thumb sign, to previous pneumothorax, and to skin striae. In the derivation group 12, 42 and 92% of persons with low (≤1 point), moderate (>1-3.5 points) or high pre-test probability (>3.5 points) had Marfan syndrome, compared to 12, 57 and 91%, respectively, in the validation group. Positive likelihood ratios were 13.96 and 8.54 in the high probability group of the derivation and validation group, respectively.CONCLUSION: A simple prediction model provides evidence for Marfan syndrome. This model can be used to identify patients who require definitive diagnostic work-up.",
keywords = "Adult, Humans, Male, Aged, Female, Middle Aged, Adolescent, Young Adult, Prospective Studies, Cross-Sectional Studies, Predictive Value of Tests, Mutation/genetics, *Decision Support Techniques, Microfilament Proteins/genetics, Marfan Syndrome/*diagnosis/genetics, Adult, Humans, Male, Aged, Female, Middle Aged, Adolescent, Young Adult, Prospective Studies, Cross-Sectional Studies, Predictive Value of Tests, Mutation/genetics, *Decision Support Techniques, Microfilament Proteins/genetics, Marfan Syndrome/*diagnosis/genetics",
author = "S Sheikhzadeh and Kusch, {M L} and M Rybczynski and C Kade and B Keyser and Bernhardt, {A M} and M Hillebrand and Mir, {T S} and B Fuisting and Robinson, {P N} and J Berger and V Lorenzen and J Schmidtke and S Blankenberg and {von Kodolitsch}, Y",
year = "2012",
month = jun,
doi = "10.1093/qjmed/hcs008",
language = "English",
volume = "105",
pages = "527--535",
journal = "QJM-INT J MED",
issn = "1460-2725",
publisher = "Oxford University Press",
number = "6",

}

RIS

TY - JOUR

T1 - A simple clinical model to estimate the probability of Marfan syndrome

AU - Sheikhzadeh, S

AU - Kusch, M L

AU - Rybczynski, M

AU - Kade, C

AU - Keyser, B

AU - Bernhardt, A M

AU - Hillebrand, M

AU - Mir, T S

AU - Fuisting, B

AU - Robinson, P N

AU - Berger, J

AU - Lorenzen, V

AU - Schmidtke, J

AU - Blankenberg, S

AU - von Kodolitsch, Y

PY - 2012/6

Y1 - 2012/6

N2 - BACKGROUND: Marfan syndrome is a heritable connective tissue disease. Definitive diagnosis is complex, and requires sequencing of a large gene, FBN1.AIM: We aimed to develop a simple model to estimate the pre-test probability of Marfan syndrome.DESIGN: Prospective cross-sectional study.METHODS: We applied diagnostic standards for definitive diagnosis or exclusion of Marfan syndrome in 329 consecutive persons. In 208 persons with random assignment to our derivation group, we performed multivariate logistic regression to assess 14 clinical variables for inclusion in a prediction model with derivation of score points from the estimated coefficients. We created cut-offs to classify low, moderate and high probability of Marfan syndrome. For validation, we applied the model to the remaining 121 persons.RESULTS: We identified seven variables for inclusion in the final model, where we assigned four score points to ectopia lentis, two points to a family history of Marfan syndrome, and one point to previous thoracic aortic surgery, to pectus excavatum, to a wrist and thumb sign, to previous pneumothorax, and to skin striae. In the derivation group 12, 42 and 92% of persons with low (≤1 point), moderate (>1-3.5 points) or high pre-test probability (>3.5 points) had Marfan syndrome, compared to 12, 57 and 91%, respectively, in the validation group. Positive likelihood ratios were 13.96 and 8.54 in the high probability group of the derivation and validation group, respectively.CONCLUSION: A simple prediction model provides evidence for Marfan syndrome. This model can be used to identify patients who require definitive diagnostic work-up.

AB - BACKGROUND: Marfan syndrome is a heritable connective tissue disease. Definitive diagnosis is complex, and requires sequencing of a large gene, FBN1.AIM: We aimed to develop a simple model to estimate the pre-test probability of Marfan syndrome.DESIGN: Prospective cross-sectional study.METHODS: We applied diagnostic standards for definitive diagnosis or exclusion of Marfan syndrome in 329 consecutive persons. In 208 persons with random assignment to our derivation group, we performed multivariate logistic regression to assess 14 clinical variables for inclusion in a prediction model with derivation of score points from the estimated coefficients. We created cut-offs to classify low, moderate and high probability of Marfan syndrome. For validation, we applied the model to the remaining 121 persons.RESULTS: We identified seven variables for inclusion in the final model, where we assigned four score points to ectopia lentis, two points to a family history of Marfan syndrome, and one point to previous thoracic aortic surgery, to pectus excavatum, to a wrist and thumb sign, to previous pneumothorax, and to skin striae. In the derivation group 12, 42 and 92% of persons with low (≤1 point), moderate (>1-3.5 points) or high pre-test probability (>3.5 points) had Marfan syndrome, compared to 12, 57 and 91%, respectively, in the validation group. Positive likelihood ratios were 13.96 and 8.54 in the high probability group of the derivation and validation group, respectively.CONCLUSION: A simple prediction model provides evidence for Marfan syndrome. This model can be used to identify patients who require definitive diagnostic work-up.

KW - Adult

KW - Humans

KW - Male

KW - Aged

KW - Female

KW - Middle Aged

KW - Adolescent

KW - Young Adult

KW - Prospective Studies

KW - Cross-Sectional Studies

KW - Predictive Value of Tests

KW - Mutation/genetics

KW - Decision Support Techniques

KW - Microfilament Proteins/genetics

KW - Marfan Syndrome/diagnosis/genetics

KW - Adult

KW - Humans

KW - Male

KW - Aged

KW - Female

KW - Middle Aged

KW - Adolescent

KW - Young Adult

KW - Prospective Studies

KW - Cross-Sectional Studies

KW - Predictive Value of Tests

KW - Mutation/genetics

KW - Decision Support Techniques

KW - Microfilament Proteins/genetics

KW - Marfan Syndrome/diagnosis/genetics

U2 - 10.1093/qjmed/hcs008

DO - 10.1093/qjmed/hcs008

M3 - SCORING: Journal article

C2 - 22301820

VL - 105

SP - 527

EP - 535

JO - QJM-INT J MED

JF - QJM-INT J MED

SN - 1460-2725

IS - 6

M1 - 6

ER -