Evidence map›Paper›PMID 38238766›Full record

ArticleOrphanet journal of rare diseases2024

A position statement on the post gene-therapy rehabilitation of aromatic I-amino acid decarboxylase deficiency patients.

Hui-Min Lee, Saadet Mercimek-Andrews, Gabriella Horvath, Diana Marchese, Richard E Poulin, Alexis Krolick, Kati-Lyn Tierney, Jasmine Turna, Judy Wei, Wuh-Liang Hwu

Open access · goldAbstract readConsensus Statement
In one paragraph

Article in Orphanet journal of rare diseases, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
1.0field-weighted citation impact, top 28% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed, 3 citations in OpenAlex.

  1. Trial
  2. Article
  3. Current clinical applications of AAV-mediated gene therapy.Molecular therapy : the journal of the American Society of Gene Therapy · 2025
    Review
  4. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors at 7 institutions in 3 countries.

Hui-Min LeeDepartment of Medical Genetics and Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, No. 8, Chung-Shan South Road, Taipei, 100226, Taiwan.
Saadet Mercimek-AndrewsDepartment of Medical Genetics, University of Alberta, 8613 114 Street, Edmonton, AB, T6G 2H7, Canada.
Gabriella HorvathDivision of Biochemical Genetics, Department of Pediatrics, University of British Columbia, 4480 Oak Street, Vancouver, BC, V6H 3V4, Canada.
Diana MarcheseDepartment of Pediatric Rehabilitation, Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA.
Richard E PoulinThai-Chinese International School, 101/177 Moo 7 Soi Mooban Bangpleenives, Prasertsin Road, Bangplee Yai, Samut Prakan, 10540, Thailand.
Alexis KrolickPTC Therapeutics Inc, 100 Corporate Ct #2400, South Plainfield, NJ, 07080, USA.
Kati-Lyn TierneyTexas Children's Hospital, 6621 Fannin Street, Houston, TX, 77030, USA.
Jasmine TurnaPTC Therapeutics Inc, 100 Corporate Ct #2400, South Plainfield, NJ, 07080, USA.
Judy WeiRuamrudee International School, 6 Soi Ramkhamhaeng 184, Khwaeng Min Buri, Min Buri, Bangkok, 10510, Thailand.
Wuh-Liang HwuDepartment of Medical Genetics and Pediatrics, National Taiwan University Hospital and National Taiwan University College of Medicine, No. 8, Chung-Shan South Road, Taipei, 100226, Taiwan. hwuwlntu@ntu.edu.tw.ORCID 0000-0001-6690-4879
PTC Therapeutics (United States) · USChildren's Mercy Hospital · USChina Medical University · TWNational Yang Ming Chiao Tung University · TWTexas Children's Hospital · USUniversity of Alberta · CAUniversity of British Columbia · CA

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Aromatic L-amino acid decarboxylase (AADC) deficiency is a rare genetic disorder of monoamine neurotransmitter synthesis that presents with a range of symptoms, including motor dysfunction and limited attainment of developmental motor milestones. The approval of eladocagene exuparvovec, a gene therapy for AADC deficiency with demonstrated efficacy for motor improvements, now expands the range of motor outcomes possible for patients with this disorder. However, recommendations and guidelines for therapy following treatment with gene therapy are lacking. To ensure patients can reach their full potential following treatment with gene therapy, it is essential they receive rehabilitation therapies designed specifically with their impairments and goals in mind. Therefore, we highlight specific rehabilitative needs of patients following gene therapy and propose a set of recommendations for the post-treatment period based on collective experiences of therapists, physicians, and caregivers treating and caring for patients with AADC deficiency who have been treated with gene therapy. These recommendations include a focus on periods of intensive therapy, facilitating active movements, training for functional abilities, cognitive and communication training, parent/caregiver empowerment, collaboration between therapists and caregivers to develop in-home programs, and the incorporation of supplemental forms of therapy that patients and their families may find more enjoyable and engaging. Many of these rehabilitative strategies may be employed prior to gene therapy. However, these recommendations will be valuable for therapists, caregivers, and wider treatment teams as they prepare for the post-treatment journey with these patients. Furthermore, the considerations and recommendations presented here may prove beneficial outside the AADC deficiency community as gene therapies and other treatments are developed and approved for other rare diseases.

Indexed as

Amino Acid Metabolism, Inborn ErrorsAmino AcidsAromatic-L-Amino-Acid DecarboxylasesGenetic TherapyHumansAmino AcidsAromatic-L-Amino-Acid DecarboxylasesAADC deficiencyFine motor developmentGene therapyGross motor developmentNeurotransmitter disorderPhysical therapyRehabilitation outcomes

Identifiers

PMID38238766
PMCPMC10797739
OpenAlexW4390972669

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.