Evidence map›Paper›PMID 33691726›Full record

ArticleOrphanet journal of rare diseases2021

Growth in ataxia telangiectasia.

Valerie A I Natale, Tim J Cole, Cynthia Rothblum-Oviatt, Jennifer Wright, Thomas O Crawford, Maureen A Lefton-Greif, Sharon A McGrath-Morrow, Haley Schlechter, Howard M Lederman

Erratum issuedOpen access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Orphanet journal of rare diseases, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 11 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed, 1 pooled it
1.4field-weighted citation impact, top 19% 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

11 citing papers in PubMed, 1 synthesis or guideline pooled it, 17 citations in OpenAlex.

  1. Pooled it
  2. Article
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  6. Review
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  10. Correction to: Growth in ataxia telangiectasia.Orphanet journal of rare diseases · 2021
    Article
  11. Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

9 authors at 5 institutions in 3 countries.

Valerie A I NataleForgotten Diseases Research Foundation, Santa Clara, CA, USA. vnatale@forgottendiseases.org.ORCID http://orcid.org/0000-0003-0418-6423
Tim J ColeUCL Great Ormond Street Institute of Child Health, London, UK.
Cynthia Rothblum-OviattA-T Children's Project, Coconut Creek, FL, USA.
Jennifer WrightDivision of Pediatric Allergy and Immunology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Thomas O CrawfordDepartments of Pediatrics and Neurology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Maureen A Lefton-GreifDepartments of Pediatrics, Otolaryngology-Head and Neck Surgery, and Physical Medicine and Rehabilitation, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Sharon A McGrath-MorrowChildren's Hospital of Philadelphia Division of Pulmonary Medicine and Sleep, Philadelphia, PA, USA.
Haley SchlechterInstitute for Clinical and Translational Research, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Howard M LedermanDivision of Pediatric Allergy and Immunology, The Johns Hopkins Medical Institutions, Baltimore, MD, USA.
Johns Hopkins University · USA-T Children's Project · USChildren's Hospital of Philadelphia · USFondation Congolaise Pour La Recherche Médicale · CGGreat Ormond Street Hospital · GB

Funding

ZINC AND CA EDTA THERAPY OF PLUMBISMM01RR000052 · NCRR · JOHNS HOPKINS UNIVERSITY · PI BELCHER, HAROLYN MILLICENT EDITH · 1985 to 2007
$37.3M
Resolution of Diffuse Inflammatory Lung Injury in Neonatal MiceR01HL114800 · NHLBI · JOHNS HOPKINS UNIVERSITY · PI MCGRATH-MORROW, SHARON ANN · 2013 to 2021
$3.3M
Methods to Improve Clinical Trials for A-TR21TR003534 · NCATS · JOHNS HOPKINS UNIVERSITY · PI LEDERMAN, HOWARD M., LEFTON-GREIF, MAUREEN A · 2020 to 2021
$433k
NCATS NIH HHS R21 TR003534NCRR NIH HHS M01 RR000052NHLBI NIH HHS R01 HL114800
6 · The paper itself

Abstract

backgroundAtaxia telangiectasia (A-T) is a DNA repair disorder that affects multiple body systems. Neurological problems and immunodeficiency are two important features of this disease. At this time, two main severity groups are defined in A-T: classic (the more severe form) and mild. Poor growth is a common problem in classic A-T. An objective of this study was to develop growth references for classic A-T. Another objective was to compare growth patterns in classic A-T and mild A-T with each other and with the general population, using the CDC growth references. A final objective was to examine the effects of chronic infection on height.

resultsWe found that classic A-T patients were smaller overall, and suffered from height and weight faltering that continued throughout childhood and adolescence. When compared to the CDC growth references, the median heights and weights for both male and female patients eventually fell to or below the 3rd centile on the CDC charts. Height faltering was more pronounced in females. Birthweight was lower in the classic A-T group compared to mild A-T and the general population, whereas birth length was not. Finally, we investigated height and BMI faltering in relation to number of infections and found no association.

conclusionsClassic A-T appears to affect growth in utero. Although children appear to grow well in very early life, faltering begins early, and is unrelenting.

Indexed as

Ataxia TelangiectasiaAdolescentBody HeightBody WeightChildFemaleGrowth DisordersHumansMaleAtaxia telangiectasiaGrowthGrowth chartsInfections

Identifiers

PMID33691726
PMCPMC7945359
OpenAlexW3134658996

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.