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Peripheral neuropathy in Krabbe disease

2006· article· en· W2092822136 on OpenAlex

Why this work is in the frame

A frame that forgets how it found something cannot be audited. These are the routes that admitted this work.

affAt least one author lists a Canadian institution in the pinned OpenAlex snapshot.

Bibliographic record

VenueNeurology · 2006
Typearticle
Languageen
FieldMedicine
TopicLysosomal Storage Disorders Research
Canadian institutionsUniversity of Alberta Hospital
Fundersnot available
KeywordsMedicineHematopoietic stem cell transplantationNerve conduction velocityKrabbe diseaseSural nerveMotor nerveTransplantationPeripheralPeripheral neuropathySensory nerveInternal medicineSurgeryCardiologyDiseaseSensory systemAnatomyNeuroscienceEndocrinologyLeukodystrophy

Abstract

fetched live from OpenAlex

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) may slow the progression of Krabbe disease (KD) if performed early in the disease. The authors' studies indicate that more than 90% of patients with KD have severe abnormalities in peripheral nerve conduction. OBJECTIVE: To assess the effect of HSCT on nerve conduction in patients with KD. METHODS: The authors performed serial nerve conduction studies (NCS) in 12 patients with KD after HSCT. The average follow-up was 18 months (6 months to 3 years) after HSCT. Pretransplant NCS were not available in two patients; all others (10 of 12) had significant pretransplant abnormalities. RESULTS: After HSCT, previously absent F-waves (1 patient) and sural sensory responses (SNR) (3 patients) were found recordable. All patients continued to have recordable SNR after HSCT, and these became normal in 7 of 12 patients. Distal motor nerve latency became normal in 6 of 17 and motor nerve conduction velocity (CV) in 2 of 17 nerves; F-wave latencies (FWL) improved in 6 of 17 nerves, but did not become normal in any. There was greater improvement in nerve conduction abnormalities if the transplant was performed earlier in life. After an initial improvement, there was subsequent worsening of motor latencies (2 of 12), motor CV (2 of 12), FWL (3 of 12), and SSR (1 of 12), indicating that benefit from HSCT may be temporary. CONCLUSIONS: Serial nerve conduction studies are useful in following the course of peripheral neuropathy in Krabbe disease. Hematopoietic stem cell transplantation is followed by improvement in peripheral nerve conduction abnormalities in these patients, suggesting remyelination of the nerves.

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.

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.015
Threshold uncertainty score0.409

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.000
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.000
Insufficient payload (model declined to judge)0.0000.000

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 head0.012
GPT teacher head0.271
Teacher spread0.259 · 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