Evidence map›Paper›PMID 34422195›Full record

ArticleThe Pan African medical journal2021

Clinical, cytogenetic and molecular findings in nine Moroccan patients with Fanconi anemia.

Yassamine Doubaj, Abdelali Zrhidri, Siham Chafai Elalaoui, Jaber Lyahyai, Youssef El Kadiri, Nadia Elkassimi, Aziza Sbiti, Maria El Kababri, Laila Hessissen, Abdelaziz Sefiani

Open access · goldAbstract readCase Reports
In one paragraph

Article in The Pan African medical journal, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact, top 96% 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

1 citing paper in PubMed, 0 citations in OpenAlex.

  1. Review
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

10 authors at 1 institution in 1 country.

Yassamine DoubajCentre de Recherche en Génomique et Pathologies Humaines (Centre GENOPATH), Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc.
Abdelali ZrhidriCentre de Recherche en Génomique et Pathologies Humaines (Centre GENOPATH), Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc.
Siham Chafai ElalaouiCentre de Recherche en Génomique et Pathologies Humaines (Centre GENOPATH), Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc.
Jaber LyahyaiCentre de Recherche en Génomique et Pathologies Humaines (Centre GENOPATH), Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc.
Youssef El KadiriCentre de Recherche en Génomique et Pathologies Humaines (Centre GENOPATH), Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc.
Nadia ElkassimiDépartement de Génétique Médicale, Institut National d'Hygiène, Rabat, Maroc.
Aziza SbitiDépartement de Génétique Médicale, Institut National d'Hygiène, Rabat, Maroc.
Maria El KababriCentre d´Hématologie et Oncologie Pédiatrique, Hôpital d´Enfants, Rabat, Maroc.
Laila HessissenCentre d´Hématologie et Oncologie Pédiatrique, Hôpital d´Enfants, Rabat, Maroc.
Abdelaziz SefianiCentre de Recherche en Génomique et Pathologies Humaines (Centre GENOPATH), Faculté de Médecine et de Pharmacie, Université Mohammed V, Rabat, Maroc.
Institut National d'Hygiène du Maroc · MA

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionFanconi anemia (FA) is a rare inherited hematological disease due to a defect in the DNA repair pathway resulting in congenital abnormalities and high susceptibility to develop cancers. The cytogenetic analysis using alkylating agents is still a reference test to establish the diagnosis. Despite the genetic heterogeneity, the identification of the causal mutation is actually performed especially after the development of next generation sequencing (NGS).

methodswe report here nine Moroccan patients referred to the department of Medical Genetics for suspicion of FA. We realized a genetic consultation to establish a clinical record with biological data before carrying out the genetic analysis. Karyotyping with mitomycin was performed for all the probands before elaborating molecular study. We used massively parallel sequencing to analyse the three most frequent mutated genes FANCA, FANCC, and FANCG, representing 84% of all genes involved in FA.

resultsall the patients showed hematological signs associated with at least one extra-hematological congenital anomaly. The chromosomal breaks were significantly higher for the nine patients, compared to the controls. The molecular diagnosis was confirmed in 8 of the 9 families tested (88.8%) with 4 novel mutations. The next generation based sequencing identified 9 variations: 6 in the FANCA gene (66.6%), 3 in the FANCG gene (33.3%) and no FANCC variation was found. Of those, 7 were homozygous and 2 were compounds heterozygous.

conclusionto the best of our knowledge, this is the first molecular report of Moroccan patients with FA suggesting the predominance of two genes without any recurrent mutation. The molecular analysis of FANCA and FANCG genes should be offered first for all patients in Morocco.

Indexed as

ChildChild, PreschoolCytogenetic AnalysisFanconi AnemiaFanconi Anemia Complementation Group A ProteinFanconi Anemia Complementation Group C ProteinFanconi Anemia Complementation Group G ProteinFemaleGenetic TestingHigh-Throughput Nucleotide SequencingHumansMaleMoroccoMutationFANCA protein, humanFANCC protein, humanFANCG protein, humanFanconi Anemia Complementation Group A ProteinFanconi Anemia Complementation Group C ProteinFanconi Anemia Complementation Group G ProteinanemiacytogeneticdiagnosisFanconimolecularMoroccan

Identifiers

PMID34422195
PMCPMC8363957
OpenAlexW3164442495

What OpenQuestion holds

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LicenceCC BY
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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.