Applied Evidence

Validation of the Q87.11 ICD Code for Prader-Willi Syndrome.

Journal of intellectual disability research : JIDR · 2026 · Cohort

James Luccarelli, Theresa V Strong, Thomas H McCoy

PMID 42340009

The Q87.11 billing code flags the right patient 96.8% of the time, making it a reliable way for researchers to pull PWS cohorts from claims data.

It reports no treatment outcomes and does not change what a clinician does with a patient.

Key findings

1A single Q87.11 code in a patient's record has a positive predictive value of 96.8% (95% CI 92.0–99.1%) for that person actually having Prader-Willi syndrome, with specificity and negative predictive value both at 100%.

2Per-encounter sensitivity drops substantially: 79.4% (95% CI 62.6–91.1%) for inpatient visits but only 46.0% (95% CI 44.1–47.9%) for outpatient visits, because clinicians often code the acute problem rather than the underlying PWS.

3Among 2,147 patients whose notes mentioned PWS-related terms but who lacked the Q87.11 code, a 10% sample review found only 1 true missed case, yielding an any-time sensitivity of 92.4% (95% CI 68.9–100%).

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How it was doneWhat they found


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What it does not show

Single US health system; coding and documentation practices may differ elsewhere, and the authors explicitly caution against extrapolating outside the US. The PWS cohort is small (122 confirmed cases), and the false-negative estimate rests on a 10% sample of 2,147 text-matched records, giving a wide confidence interval on sensitivity (68.9% to 100%). Full genetic subtyping was unavailable for a significant fraction of patients, so some clinical diagnoses of PWS could not be confirmed molecularly. Retrospective design with a waiver of informed consent; no prospective follow-up or outcome tracking. The 4 false-positive cases include one where the code was applied to a fetus during a genetics evaluation, suggesting the code can be attached to the wrong person in the record.

Declared interests

No funding source or conflict-of-interest declaration is stated in the supplied text.

The easy way to misread this

Do not read the 96.8% positive predictive value as meaning the code is equally reliable on every visit. Per-encounter sensitivity falls to 46% for outpatient encounters, so a patient with PWS may not carry the code on a given clinic visit. Also, the validation was done in one large US academic health system; the authors themselves warn that coding patterns may differ in other settings, so the accuracy figures should not be assumed to hold in a different country or a smaller community hospital.

Summarised by AI from the full paper, without a clinician reviewing it. Check it against the source before it changes what you do. Read it on PubMed →


The study

Participants
2,718,218 eligible individuals; 126 with the Q87.11 code; 122 confirmed PWS
Certainty of evidence
Low

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    Cite

    James Luccarelli, Theresa V Strong, Thomas H McCoy Validation of the Q87.11 ICD Code for Prader-Willi Syndrome. Journal of intellectual disability research : JIDR. 2026.

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