Evidence map›Paper›PMID 28612151›Full record

ArticleJournal of genetic counseling2017

The Genetics Journey: A Case Report of a Genetic Diagnosis Made 30 Years Later.

Linford A Williams, Shane C Quinonez, Wendy R Uhlmann

Open access · bronzeAbstract read
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In one paragraph

Article in Journal of genetic counseling, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
0.9field-weighted citation impact, top 21% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

5 citing papers in PubMed, 10 citations in OpenAlex.

  1. Article
  2. [Clinical case analysis and literature review of mandibulofacial dysostosis with microcephaly syndrome].Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgery · 2022
    Review
  3. Article
  4. Article
  5. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

3 authors at 2 institutions in 1 country.

Linford A WilliamsGenomic Medicine Institute, Cleveland Clinic, Cleveland, OH, USA.
Shane C QuinonezDepartment of Pediatrics, Division of Genetics, Metabolism and Genomic Medicine, University of Michigan, Ann Arbor, MI, USA.
Wendy R UhlmannDepartment of Internal Medicine, Division of Molecular Medicine and Genetics, University of Michigan, 300 North Ingalls, NI3 A03, SPC 5419, Ann Arbor, MI, 48109, USA. wuhlmann@umich.edu.
University of Michigan–Ann Arbor · USCleveland Clinic · US

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Mandibulofacial dysostosis with microcephaly (MFDM) is a rare autosomal dominant condition that was first described in 2006. The causative gene, EFTUD2, identified in 2012. We report on a family that initially presented to a pediatric genetics clinic in the 1980s for evaluation of multiple congenital anomalies. Re-evaluation of one member thirty years later resulted in a phenotypic and molecularly confirmed diagnosis of MFDM. This family's clinical histories and the novel EFTUD2 variant identified, c.1297_1298delAT (p.Met433Valfs*17), add to the literature about MFDM. This case presented several genetic counseling challenges and highlights that "the patient" can be multiple family members. We discuss testing considerations for an unknown disorder complicated by the time constraint of the patient's daughter's pregnancy and how the diagnosis changed previously provided recurrence risks. Of note, 1) the 1980s clinic visit letters provided critical information about affected family members and 2) the patient's husband's internet search of his wife's clinical features also yielded the MFDM diagnosis, illustrating the power of the internet in the hands of patients. Ultimately, this case emphasizes the importance of re-evaluation given advances in genetics and the value of a genetic diagnosis for both patient care and risk determination for family members.

Indexed as

Genetic TestingPhenotypeAbnormalities, MultipleFemaleGenetic CounselingHumansMaleMandibulofacial DysostosisMicrocephalyPeptide Elongation FactorsPregnancyRibonucleoprotein, U5 Small NuclearEFTUD2 protein, humanPeptide Elongation FactorsRibonucleoprotein, U5 Small Nuclearc.1297_1298delATEFTUD2Genetic counselingInternet searchMandibulofacial dysostosis with microcephaly (MFDM)Patient lettersp.Met433Valfs*17Tracheoesophageal fistula (TEF)

Identifiers

PMID28612151
OpenAlexW2626414360

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.