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Evidence Library · What screening finds

Where the numbers collapse: sex chromosomes and microdeletions

For some conditions on the panel, a positive result is more likely to be wrong than right.

The blood test's reputation is built on Down syndrome. The rest of the panel does not perform anything like as well, and the further down the list you go, the worse it gets.

Sex chromosomes. In a planned analysis of the same large confirmed cohort:

ResultNumber analysedDetectionChance a positive result is correct
Monosomy X (Turner syndrome)17,29783.3%22.7%
Sex chromosome trisomies combined10,33370.4%82.6%

A positive result for Turner syndrome from this test is wrong roughly three times out of four. It also misses about one in six cases that are there. The authors say so directly: The PPV for the SCTs was similar to the autosomal trisomies, whereas the PPV for MX was substantially lower.

Microdeletions. A microdeletion is a small missing piece of a chromosome. The most-marketed one is called 22q11.2 deletion syndrome. In a study of 20,887 pregnancies with genetic outcomes for 18,289 of them, there were 12 confirmed cases, a rate of about 1 in 1,524.

23.7%
22q11.2 deletion
Chance a positive result is correct (11.4 to 40.2)
52.6%
Updated method
Same study, revised algorithm (28.9 to 75.6)
1 in 1,524
How common it was
In that study population

With the original method, more than three quarters of positive results were false. With an updated algorithm the test did better, but the range still runs from 28.9% to 75.6%, which is to say that even now it might be right most of the time or wrong most of the time.

What happened at one referral laboratoryA large US genetic diagnostic laboratory looked at 712 samples sent to it specifically to confirm a positive screening result. This is a referral caseload, not a screening population, and the numbers describe that caseload only. Among those referrals, the proportion of positive screens that turned out to be real was 21% for 22q11.2 deletion, 14% for 1p36 deletion, 0% for Cri-du-Chat syndrome and 0% for Prader-Willi and Angelman syndrome.

Zero is not a rounding artefact. Every single flagged case of those two that came for confirmation was a false alarm. The laboratory's own explanation: it happens likely because of the low prevalence of the individual targeted microdeletion syndromes in the general population.
The guidance already says thisCurrent society guidance, endorsed by ACOG: We do not recommend routine general population screening for any microdeletion condition. It adds that anyone who does choose 22q11.2 screening should do so only after proper counselling first, and that a woman who wants information about missing or extra pieces of chromosome should be offered a diagnostic test instead of this screen.

If a microdeletion panel was added to your blood test without a conversation, that is a departure from current guidance, and it is reasonable to say so.
Say this out loud"Does my panel include microdeletions? If so, why?"

"If this comes back positive for a microdeletion, how likely is it to be a false alarm?"

"I would like to opt out of any part of this test that ACOG does not recommend routinely."
Where this comes from:
472. Society for Maternal-Fetal Medicine; Rink BD, Dugoff L, Kuller JA. SMFM Consult Series #74: cell-free DNA screening for aneuploidies: updated guidance. Pregnancy (Hoboken). 2025;1(6):e70139.
475. Martin K, Dar P, MacPherson C, Egbert M, Demko Z, Parmar S, et al. Performance of prenatal cfDNA screening for sex chromosomes. Genet Med. 2023;25(8):100879.
476. Dar P, Jacobsson B, Clifton R, Egbert M, Malone F, Wapner RJ, et al. Cell-free DNA screening for prenatal detection of 22q11.2 deletion syndrome. Am J Obstet Gynecol. 2022;227(1):79.e1-79.e11.
150. Petersen AK, Cheung SW, Smith JL, Bi W, Ward PA, Peacock S, et al. Positive predictive value estimates for cell-free noninvasive prenatal screening from data of a large referral genetic diagnostic laboratory. Am J Obstet Gynecol. 2017;217(6):691.e1-691.e6.

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