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Record W4238349297 · doi:10.1002/9781118981559.ch25

Prenatal Diagnosis of Miscellaneous Biochemical Disorders

2015· other· en· W4238349297 on OpenAlex
David S. Rosenblatt, David Watkins

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

Venuenot available
Typeother
Languageen
FieldMedicine
TopicBiomedical Research and Pathophysiology
Canadian institutionsMcGill University
Fundersnot available
KeywordsCystinosisMethylenetetrahydrofolate reductaseHomocystinuriaPrenatal diagnosisMedicineMaple syrup urine diseaseMalabsorptionPregnancyBiologyGeneticsInternal medicineMethionineBiochemistryCystineGeneFetusEnzymeAllele

Abstract

fetched live from OpenAlex

This chapter discusses disorders of folate metabolism and those of cobalamin metabolism, and summarizes advances in the prenatal diagnosis of these disorders. The genes for all of these disorders have been identified, and prenatal diagnosis by molecular genetic analysis is possible when the causal mutations segregating in the family are known. Six genetic disorders affecting folate metabolism are generally recognized: hereditary folate malabsorption, cerebral folate deficiency, methylenetetrahydrofolate reductase deficiency, glutamate formiminotransferase deficiency, dihydrofolate reductase deficiency, and MTHFD1 deficiency. All of the inborn errors affecting folate metabolism are inherited as autosomal recessive traits. The chapter considers the disorders that affect activity of methionine synthase (cblE and cblG), which uses 5-methyltetrahydrofolate as a methyl donor. Cystinosis can be diagnosed by examining the cystine content of cultured fibroblasts or leukocytes. Treatment in cystinosis includes the management of the renal disease and dialysis or transplantation after end-stage renal disease develops.

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 categoriesInsufficient payload (model declined to judge)
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Not applicable · Consensus signal: Not applicable
GenreCandidate signal: Other · Consensus signal: Other
Teacher disagreement score0.033
Threshold uncertainty score0.968

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)0.0330.000

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 head0.015
GPT teacher head0.293
Teacher spread0.278 · 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

Quick stats

Citations0
Published2015
Admission routes1
Has abstractyes

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