Literature DB >> 1327364

Predictive diagnosis of familial adenomatous polyposis with linked DNA markers: population based study.

F MacDonald1, D G Morton, P M Rindl, J Haydon, R Cullen, J Gibson, J P Neoptolemos, M R Keighley, C M McKeown, M Hultén.   

Abstract

OBJECTIVES: To evaluate the use of polymorphic DNA probes linked to the APC gene in the presymptomatic diagnosis of familial adenomatous polyposis.
DESIGN: Four DNA probes were tested on an unselected population of patients at risk of familial adenomatous polyposis.
SUBJECTS: The first 47 families notified to the West Midlands familial adenomatous polyposis register. Plus five families sent to our hospital as part of the West of Britain DNA consortium. MAIN OUTCOME MEASURES: The proportion of families and family members in whom DNA testing could be used to adjust the estimate of risk.
RESULTS: Only 17 families on the register (containing 46% (74/162) of the population at risk) had a suitable pedigree structure for DNA analysis. DNA was analysed in 12 of these families plus the five families from the West of Britain consortium. At least one probe was informative in 27 of the 33 subjects born with 50% risk, but the most informative probe (pi 227) was the one with the highest recombination rate (10%). Flanking markers were informative in only four of the 33 subjects.
CONCLUSIONS: These findings confirm the potential for accurate predictive diagnosis of familial adenomatous polyposis with polymorphic DNA probes, but such an approach is currently limited to about one third of affected families. A combined approach to presymptomatic diagnosis, which includes DNA testing and indirect ophthalmoscopy, is advocated.

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Year:  1992        PMID: 1327364      PMCID: PMC1882825          DOI: 10.1136/bmj.304.6831.869

Source DB:  PubMed          Journal:  BMJ        ISSN: 0959-8138


  19 in total

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3.  Localization of the genetic defect in familial adenomatous polyposis within a small region of chromosome 5.

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Journal:  Cell       Date:  1991-08-09       Impact factor: 41.582

6.  Congenital hypertrophy of retinal pigment epithelium: a sign of familial adenomatous polyposis.

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Journal:  BMJ       Date:  1989-02-11

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  14 in total

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Authors:  A J Macpherson; I Bjarnason; I C Forgacs
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2.  Uptake of genetic testing for cancer predisposition.

Authors:  D G Evans; E R Maher; R Macleod; D R Davies; D Craufurd
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3.  The genetics of inherited colon cancer.

Authors:  Y Wallis; F Macdonald
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5.  Cost comparison of predictive genetic testing versus conventional clinical screening for familial adenomatous polyposis.

Authors:  B Bapat; H Noorani; Z Cohen; T Berk; A Mitri; B Gallie; K Pritzker; S Gallinger; A S Detsky
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6.  Non-penetrance and late appearance of polyps in families with familial adenomatous polyposis.

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7.  The role of congenital hypertrophy of the retinal pigment epithelium in screening for familial adenomatous polyposis.

Authors:  W J Campbell; R A Spence; T G Parks
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8.  Evaluation of molecular genetic diagnosis in the management of familial adenomatous polyposis coli: a population based study.

Authors:  E R Maher; D E Barton; R Slatter; D J Koch; M H Jones; H Nagase; S J Payne; S J Charles; A T Moore; Y Nakamura
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9.  Analysis of faecal neutral sterols in patients with familial adenomatous polyposis by gas chromatography-mass spectrometry.

Authors:  G M Barker; S Radley; A Davis; K D Setchell; N O'Connell; I A Donovan; M R Keighley; J P Neoptolemos
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10.  Molecular analysis of the APC gene in 205 families: extended genotype-phenotype correlations in FAP and evidence for the role of APC amino acid changes in colorectal cancer predisposition.

Authors:  Y L Wallis; D G Morton; C M McKeown; F Macdonald
Journal:  J Med Genet       Date:  1999-01       Impact factor: 6.318

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