Validation and Reliability of Iris Cameras in Mucopolysaccharidoses

Validation and Reliability of Iris Recognition and Manual InfraRed Cameras Using Bespoke Image Analysis Software for the Quantification of Corneal Opacification in Mucopolysaccharidoses

To assess the reliablity and validity of 2 new iris cameras in the assement of corneal opacification in mucopolysaccharidoses

Study Overview

• Status: Recruiting
• Conditions: Mucopolysaccharidoses
• Intervention / Treatment: Other: Imaging

Detailed Description

The mucopolysaccharidoses (MPS) are a group of disorders resulting from accumulation of glycosaminoglycans within various tissues including the eye. This can result in visual loss from corneal opacification. The assessment of corneal opacification is extremely subjective and based on a clinician grading this during slit lamp examination. There can be a significant variability and can prove problematic particularly in clinical trials assessing if corneal opacification is affected by potential new treatments.

The use of imaging could facilitate objective measurement of corneal clouding. A previous study using an iris camera and image analysis to provide a corneal opacification measure score (COM score) demonstrated that it can be used in children with MPS, however it still had limitations- as the system is fully automated, if certain conditions are not met an image is not captured. This can be an issue in younger children who may not be cooperative, and limited its use in younger patient populations.

It is anticipated that these limitations can be addressed by employing a newer iris camera (IriShield MK2120UL). The new camera's advantage is that it takes images monocularly, therefore only the eye under question needs to be aligned. We will also assess a new manual system (IRcamNew) which takes images whenever the shutter is manually pressed. This would be useful in children who cannot fixate with one eye, and can also be used when the child is examined under general anaesthetic which is commonly done in paediatric ophthalmology.

If proven to be comparable to the existing iris camera (IriscamOrig) in terms of validity and reliability, the added benefits of these new iris cameras can open its use in a wider range of patient populations - for instance, in paediatric clinical trials-, as well as allowing longitudinal tracking of corneal clouding from early childhood well into adulthood.

• Study Type: Observational
• Enrollment (Anticipated): 60

Contacts and Locations

• Study Contact: Name: Jane Ashworth, MD; Phone Number: 0161 276 1234; Email: jane.ashworth@mft.nhs.uk

Study Locations

• United Kingdom
  • Manchester, United Kingdom, M13 9WL
    • Recruiting
    • Manchester University NHS Foundation Trust
    • Contact: Zaria Ali, MBChB; Phone Number: 01612765560; Email: Zaria.ali@mft.nhs.uk
    • Contact: Fiona Crawford, PhD; Email: mreh.research@mft.nhs.uk

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: Yes
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

MPS patients and healthy controls

Description

Inclusion Criteria:

• Any patient aged 0-100 with MPS type I, type III, type IV and type VI. Type VII MPS patients may also be eligible.

For the control group -

Participants of any age currently being followed at MREH that have an eye condition unrelated to the cornea as established by clinical examination.

Exclusion Criteria:

• MPS II

Study Plan

How is the study designed?

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
MPS patients; Any patient aged 0-100 with MPS type I, type III, type IV and type VI. Type VII MPS patients may also be eligible.	Other: Imaging; Imaging with 3 iris cameras
Control; For the control group - Participants of any age currently being followed at MREH that have an eye condition unrelated to the cornea as established by clinical examination.	Other: Imaging; Imaging with 3 iris cameras

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Comparison of reliability of images taken by two different operators using the two ne w iris cameras	Bland altman plots to compare reliability between images and COM scores obtained by 2 different operators	2 months
Correlation of COM scores between two new iris cameras and IrisCamOrig	Validity will be assessed by comparing the COM scores generated by the new iris cams to the previously validated iris camera	2 months

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Comparison of clinical grading to COM score obtained by image analysis	Compare clinical grading of corneal opacification on slit lamp by clinicians to corneal opacification measure generated by image analysis	1 month

Collaborators and Investigators

• Sponsor: Manchester University NHS Foundation Trust
• Collaborators: Orchard Therapeutics

Study record dates

Study Major Dates

• Study Start (Actual): March 8, 2023
• Primary Completion (Anticipated): September 8, 2023
• Study Completion (Anticipated): September 8, 2023

Study Registration Dates

• First Submitted: April 12, 2022
• First Submitted That Met QC Criteria: April 25, 2022
• First Posted (Actual): April 29, 2022

Study Record Updates

• Last Update Posted (Actual): March 22, 2023
• Last Update Submitted That Met QC Criteria: March 21, 2023
• Last Verified: March 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Metabolic Diseases; Genetic Diseases, Inborn; Connective Tissue Diseases; Carbohydrate Metabolism, Inborn Errors; Metabolism, Inborn Errors; Lysosomal Storage Diseases; Mucinoses; Mucopolysaccharidoses
• Other Study ID Numbers: IRAS 315339

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No