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Record W2171543283

Moebius and Kallmann syndromes with diabetes insipidus:a phenotype of Fibroblast Growth Factor (FGF) 8 mutation

2014· article· en· W2171543283 on OpenAlexvenueno aff
Ahmad Marzuki Omar, Mohammad Arif Shahar, Norasyikin Abd Wahab, Norlaila Mustafa, Norlela Sukor, Nor Azmi Kamaruddin

Bibliographic record

VenueJournal of Endocrinology and Metabolism · 2014
Typearticle
Languageen
FieldMedicine
TopicPectus Deformity Diagnosis and Treatment
Canadian institutionsnot available
Fundersnot available
KeywordsMedicineKallmann syndromeHypogonadotropic hypogonadismAnosmiaDiabetes insipidusEndocrinologyHyposmiaAgenesisRenal agenesisInternal medicineDelayed pubertyAnatomy
DOInot available

Abstract

fetched live from OpenAlex

Moebius syndrome is a rare congenital condition typically characterised by congenital facial and abducens nerve palsies. Dysfunction of other cranial nerves, malformations of orofacial structures, and limb anomalies may also be present. The aetiology is not well understood, but may relate to chromosomal abnormalities and exposure to teratogens and vascular events during gestation. Kallmann syndrome, on the other hand, consists of congenital hypogonadotropic hypogonadism and anosmia. It represents a form of isolated GnRH deficiency. There have been several reported cases of Moebius syndrome associated with Kallmann syndrome, with one reported case of Moebius syndrome with diabetes insipidus and another of Kallmann syndrome with diabetes insipidus. We report a 30-year-old man who was born with left lower motor neuron facial, abducens and oculomotor nerve palsies, dental agenesis, hypogonadotropic hypogonadism with bilateral cryptorchidism and micropenis. His developmental milestones were delayed. At the age of 10 years old, he had progressive hearing loss following recurrent bilateral otitis media with effusion. A year later he was diagnosed with central diabetes insipidus and noted to have anosmia and bilateral optic disc coloboma. The pituitary MRI findings were normal. This case illustrates a rare combination of Moebius and Kallmann syndromes with diabetes insipidus, which may well describe the FGF8 mutation in this patient.

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)0.000
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 score0.104
Threshold uncertainty score0.332

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0010.000
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 head0.011
GPT teacher head0.240
Teacher spread0.228 · 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

Citations0
Published2014
Admission routes1
Has abstractyes

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