MRI screening of kindred at risk of developing paragangliomas: support for genomic imprinting in hereditary glomus tumours
1992

MRI Screening for Hereditary Paragangliomas

Sample size: 83 publication Evidence: high

Author Information

Author(s): A.P.G. van Gils, A.G.L. van der Mey, R.P.L.M. Hoogma, L.A. Sandkuijl, P.D. Maaswinkel-Mooy, T.H.M. Falkel, E.K.J. Pauwels

Primary Institution: University Hospital Leiden

Hypothesis

Is the development of hereditary glomus tumours determined by the sex of the transmitting parent?

Conclusion

The study found that MRI screening can detect previously unknown paragangliomas in family members at risk, supporting the theory of genomic imprinting.

Supporting Evidence

  • MRI detected 20 glomus tumours in the screened family members.
  • The study identified 10 previously unknown tumours in asymptomatic relatives.
  • Genomic imprinting theory was supported by the finding that no tumours were found in descendants of female gene carriers.

Takeaway

Doctors used special scans to find hidden tumors in family members who might get sick, and they learned that these tumors are more likely to show up if the dad carries the gene.

Methodology

Screening included MRI and urinary catecholamine testing for 90 family members at risk.

Potential Biases

Potential bias due to self-selection of participants who chose to undergo screening.

Limitations

Some family members declined to participate, which may affect the completeness of the data.

Participant Demographics

Family members aged over 18 years, including both males and females.

Statistical Information

P-Value

23375

Statistical Significance

p<0.05

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