Evidence map›Paper›PMID 30949167›Full record

ArticleFrontiers in immunology2019

Natural Killer Cell Degranulation Defect: A Cause for Impaired NK-Cell Cytotoxicity and Hyperinflammation in Fanconi Anemia Patients.

Snehal Shabrish, Madhura Kelkar, Niranjan Chavan, Mukesh Desai, Umair Bargir, Maya Gupta, Priti Mehta, Akanksha Chichra, Chandrakala S, Prasad Taur and 3 more

Open access · goldAbstract readCase Reports
In one paragraph

Article in Frontiers in immunology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
0.7field-weighted citation impact, top 29% 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

4 citing papers in PubMed, 6 citations in OpenAlex.

  1. Article
  2. Article
  3. Red blood cell folate and cardiovascular deaths among hypertensive adults, an 18-year follow-up of a national cohort.Hypertension research : official journal of the Japanese Society of Hypertension · 2020
    Article
  4. 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

13 authors at 4 institutions in 1 country.

Snehal ShabrishDepartment of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.
Madhura KelkarDepartment of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.
Niranjan ChavanDepartment of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.
Mukesh DesaiDivision of Immunology, Bai Jerbai Wadia Hospital for Children, Mumbai, India.
Umair BargirDepartment of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.
Maya GuptaDepartment of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.
Priti MehtaSurya Hospitals, Mumbai, India.
Akanksha ChichraSurya Hospitals, Mumbai, India.
Chandrakala SDepartment of Haematology, Seth G. S. Medical College and KEM Hospital, Mumbai, India.
Prasad TaurDivision of Immunology, Bai Jerbai Wadia Hospital for Children, Mumbai, India.
Vinay SaxenaNational Institute of Virology, Mumbai Unit, Haffkine Institute, Mumbai, India.
Babu Rao VundintiDepartment of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.
Manisha MadkaikarDepartment of Paediatric Immunology and Leukocyte Biology, National Institute of Iummunohematology (ICMR), KEM Hospital, Mumbai, India.
National Institute of Immunohaematology · INBai Jerbai Wadia Hospital for Children · INSurya Hospitals · INICMR-National Institute of Virology · IN

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Fanconi anemia (FA) is a rare inherited syndrome characterized by progressive bone marrow failure (BMF), abnormal skin pigmentation, short stature, and increased cancer risk. BMF in FA is multifactorial and largely results from the death of hematopoietic stem cells due to genomic instability. Also, inflammatory pathology in FA has been previously reported, however the mechanism is still not clear. In literature, decreased NK-cell count and/or impaired NK-cell activity, along with other immunological abnormalities have been described in FA-patients (1). However, to the best of our knowledge, this is the first report showing a defective degranulation mechanism leading to abnormal NK-cell cytotoxicity in FA-patients, which may explain the development of a hyperinflammatory response in these patients. This may predispose some patients to develop Hemophagocytic lymphohistiocytosis (HLH) which manifests with prolonged fever, progressive cytopenias and organomegaly. Early diagnosis and initiation of immunosuppressive therapy in these patients will help to better manage these patients. We also propose FA genes to be listed as a cause of familial HLH.

Indexed as

AdolescentCell DegranulationChild, PreschoolFanconi AnemiaFemaleHumansInflammationKiller Cells, NaturalMaleFamilial Hemophagocytic lymphohistiocytosis (FHL)Fanconi anemiaHLH-targeted therapyNK cell cytotoxicityNK cell degranulation defect

Identifiers

PMID30949167
PMCPMC6438155
OpenAlexW2923273749

What OpenQuestion holds

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LicenceCC BY
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Registered trials

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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.