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Hypophosphatasia (HPP): Suspect-and-test checklist for clinicians

A one-page worksheet that turns the full Hypophosphatasia (HPP) diagnosis guide into something you can use at the point of care: what to look for, what to order, which results should not end the workup, and who else to test or refer.

Who it is forClinicians and care teams who may see Hypophosphatasia (HPP) before it is diagnosed, including primary care, the specialists named in the guide, and referral or genetics coordinators.
When to use itWhen Hypophosphatasia (HPP) is on the differential, when a patient has several red flags, or when you are preparing a referral or a second opinion.

How to use it, section by section

  1. Red flags present: Tick each finding the patient has. They are grouped by body system so you can scan quickly.
  2. Already labeled as: Tick earlier diagnoses on the chart that the sources say are often given first.
  3. Family to test: Note relatives who may need testing, and record when symptoms began and which specialists have already been seen.
  4. Testing, in order: Tick each test as you order it and write the result on its line. The order follows the published sources.
  5. Results that do not rule it out: A reminder of findings that can look reassuring but do not exclude Hypophosphatasia (HPP).
  6. Specialists: Tick the referral, then write who it went to and any notes.
Two ways to fill it inPrint it blank and write on it, or tick boxes and type on this screen first, then print or save as a PDF. Keep it with the chart or send it with the referral.
PrivacyNothing you type is sent or saved. It stays in this browser tab and disappears when you close it. Use patient initials, not full identifiers.

What it is not: Not a diagnostic score, a protocol or coding advice. It summarizes published sources, and clinical judgment and the treating clinician govern. Every point is sourced in the full Hypophosphatasia (HPP) guide.

One Letter page. Use your browser's print or save as PDF.Full guide with sources
Suspect-and-test checklist
Hypophosphatasia (HPP)

Inherited ALPL disorder. Start from a persistently low ALP for age and sex with no other cause.

ICD-10-CME83.39Trial Friend for Clinicians

1Red flags present

Labs

Teeth

Adult bones and joints

Children

Family

2Already labeled as

3Family to test

4Testing, in the order the sources describe

  1. Repeat ALP, age and sex rangeLithium heparin tube, not EDTA or oxalate. Pediatric ranges for children.
    Result
  2. Exclude other causes of low ALPAntiresorptives, hypothyroidism, myeloma, celiac, Wilson disease, low magnesium or zinc.
    Result
  3. Plasma PLP and urine PEAStop pyridoxine 1 week before PLP, per IWG.
    Result
  4. ALPL sequencing with deletion/duplicationSupports diagnosis; a negative result does not exclude HPP.
    Result
  5. ImagingChildren: skeletal survey. Adults: films for pseudofractures, chondrocalcinosis; renal ultrasound.
    Result
  6. 2024 IWG criteriaLow ALP plus 2 major, or 1 major and 2 minor criteria.
    Result

5Results the sources say do not rule it out

  • Low ALP on a bisphosphonate. Usually drug effect, not HPP.
  • A child's ALP read against an adult range.
  • Normal ALP during pregnancy or after a recent long bone fracture.
  • ALP above 25IU/L. Higher values do not exclude HPP.

6Specialists the sources name

Diagnosis code: E83.39 Other disorders of phosphorus metabolismCodes patients often carry before diagnosis are listed in the online guide.
Full guide and sourcesEvery point here is sourced in the online guide, which also has a patient note to print.
Trial Friend, trialfriend.com/for-clinicians. Key sources: HPP IWG diagnostic criteria (2023); HPP IWG pediatric criteria (2023); HPP IWG adult review (2023); Italian pediatric expert opinion (2023); Tornero 2020 Madrid low ALP study; Tornero 2024 chondrocalcinosis study; all 24 with links in the online guide. Checked November 10, 2026. A reference checklist for licensed clinicians that summarizes published sources. It is not a diagnostic tool, a score or a protocol, and it does not replace clinical judgment, local practice or the prescribing information. Not coding advice. Trial Friend never receives anything written or typed on this sheet.