MétaCan
Menu
Back to cohort
Record W4285026337 · doi:10.15690/pf.v19i3.2438

Clinical Case of Drug Allergy to Enzyme Replacement Therapy in a Patient with Mucopolysaccharidosis Type II

2022· article· en· W4285026337 on OpenAlexaff
Julia Levina, Nato D. Vashakmadze, Leyla S. Namazova-Baranova, Еlena A. Vishneva, Mariya S. Karaseva, Natalia V. Zhurkova, Kamilla E. Efendieva, Anna A. Alekseeva, Vera G. Kalugina, Artur V. Zaz’yan

Bibliographic record

VenueПедиатрическая фармакология · 2022
Typearticle
Languageen
FieldMedicine
TopicLysosomal Storage Disorders Research
Canadian institutionsChildren’s Health Research Institute
Fundersnot available
KeywordsEnzyme replacement therapyHunter syndromeMucopolysaccharidosis type IIMedicineDesensitization (medicine)PharmacotherapyMucopolysaccharidosisDrugDiseasePharmacologyInternal medicine

Abstract

fetched live from OpenAlex

Background. Enzyme replacement therapy (ERT) with iduronate-2-sulfatase recombinant forms (idursulfase and idursulfase beta) is effective for the management of mucopolysaccharidosis type II (MPS II). Patients with Hunter syndrome require lifelong ERT that can negate endogenous enzyme deficiency. However, hypersensitivity reactions may occur during ERT, and they significantly complicate the implementation of vital therapy. Clinical case description. This article describes clinical case of a child with hypersensitivity reaction to ERT. The patient with confirmed diagnosis of MPS II was administrated with idursulfase. Then, the drug was replaced with idursulfase beta due to the allergic reaction. Thus, even after the drug change, side effects maintained without sustained improvement with underlying glucocorticosteroids (GCS), antihistamines and with decreased infusion rate. Concerning the vital need to continue ERT, this patient with drug allergy to this pharmacotherapeutic group was further administered with combined therapy of cyclosporine and omalizumab. Personalised protocol for the administration of idursulfase beta with desensitization was developed. Such experience was firstly described In Russian patient. Conclusion. The presented personalised combination therapy made it possible to prevent hypersensitivity reactions during ERT in the patient with MPS II.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame machine prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.002
Version: metacan-v3-hybrid-931329e0061cValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Case report · Consensus signal: Case report
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.004
Threshold uncertainty score0.010

Distilled classifier scores by category (both heads)

CategoryCodexGemma
Metaresearch0.0000.002
Meta-epidemiology (narrow)0.0020.001
Meta-epidemiology (broad)0.0010.001
Bibliometrics0.0020.001
Science and technology studies0.0020.001
Scholarly communication0.0010.001
Open science0.0010.001
Research integrity0.0040.003
Insufficient payload (model declined to judge)0.0030.001

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.036
GPT teacher head0.350
Teacher spread0.314 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designCase report
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations1
Published2022
Admission routes1
Has abstractyes

Explore more

Same venueПедиатрическая фармакологияSame topicLysosomal Storage Disorders ResearchFrench-language works237,207