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Record W2044194932 · doi:10.1159/000103365

Outcomes in CAH: Need for Evidence-Based Estimates

2007· article· en· W2044194932 on OpenAlexaff
Scott D. Grosse, Guy Van Vliet

Bibliographic record

VenueHormone Research in Paediatrics · 2007
Typearticle
Languageen
FieldMedicine
TopicNeonatal Health and Biochemistry
Canadian institutionsUniversité de MontréalCentre Hospitalier Universitaire Sainte-Justine
Fundersnot available
KeywordsMedicinePediatricsNewborn screeningDemography

Abstract

fetched live from OpenAlex

The statement that among children born in United States jurisdictions that screened for CAH in 2006 ‘Over 56% of presumed cases of CAH went undetected’ is incorrect. The sensitivity of newborn screening for classical CAH is extremely high, essentially 100% for salt-wasting CAH [2] . The ‘National Newborn Screening and Genetics Resource Center records’ cited by Ms. Leight come from the US National Newborn Screening Information System (NNSIS) database to which states voluntarily report data and which is regularly updated [3] . The NNSIS as of April 6, 2007, included a total of 150 children born in 2006 with confirmed classical CAH. Because a number of states had either not yet reported data or reported incomplete data for CAH diagnoses in 2006, the ultimate number will be substantially larger. NNSIS data for 2005 included 180 children confirmed with classical CAH born in 30 states with a total of 2.8 million births that were screened for CAH, or 1 in 15,700 births. We share Ms. Leight’s concern about the plight of children with CAH born in low-income countries, among whom the rate of mortality is likely to be quite high. On the other hand, the most cost-effective approach to reducing CAH mortality in such countries needs to be carefully considered along with the availability of treatment following identification of CAH. This requires data on outcomes with and without screening. Migeon and Donohoue We appreciate the many efforts of the CARES Foundation, Inc. on behalf of children with CAH and recognize that the death of even one child with this preventable outcome is a tragedy. Preventive programs and policies can be most effectively developed on an evidence-based foundation, and the available data support our conclusion that infant mortality in classical CAH is 4% in North America and Europe. Additional supporting data come from a recent study of stored blood spot specimens for unexplained infant deaths in Austria and the Czech Republic that found about one fifth as many cases of CAH as had been expected on the basis of expert opinion [1] . Out of more than 2 million births during a 13-year period, there were 3 unexplained deaths due to CAH, or 2% of the expected number of children with CAH. We agree with Ms. Leight that there should also be a systematic study of a sample of all infant deaths to determine the frequency of death due to CAH. As stated in our article: ‘One way to ascertain such deaths in cohorts not screened for CAH at birth is to retrieve stored dried blood spot filter paper cards for children who died in infancy or early childhood and test for the presence of CAH, including analysis of CYP21 mutations specific for SW-CAH.’ We would be pleased to collaborate with CARES Foundation, Inc. to identify other research needs as well. Published online: June 4, 2007 HORMONE RESEARCH

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 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)0.005
metaresearch head score (Gemma)0.005
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 score0.033
Threshold uncertainty score0.550

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0050.005
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0010.002
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.001
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 head0.167
GPT teacher head0.464
Teacher spread0.297 · 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 teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
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

Citations3
Published2007
Admission routes1
Has abstractyes

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