Evidence map›Paper›PMID 29343274›Full record

ArticlePediatric rheumatology online journal2018

Coexistent sickle-cell anemia and autoimmune disease in eight children: pitfalls and challenges.

Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert

Open access · goldAbstract read
In one paragraph

Article in Pediatric rheumatology online journal, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.

0numbers the graph read from it
0cells of the map it votes in
16citing papers in PubMed
2.1field-weighted citation impact, top 13% 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

16 citing papers in PubMed, 37 citations in OpenAlex.

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  13. Optimizing the management of chronic pain in sickle cell disease.Hematology. American Society of Hematology. Education Program · 2020
    Review
  14. Article
  15. Article
  16. Rheumatic Manifestations of Haemoglobinopathies.Current rheumatology reports · 2018
    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

7 authors at 3 institutions in 1 country.

Valerie Li-Thiao-TeOnco-Hématologie Pédiatrique, Centre Hospitalier Universitaire Amiens, Amiens, France. lithiaote.valerie@chu-amiens.fr.ORCID http://orcid.org/0000-0003-0767-1758
Florence UettwillerUnité d'Immunologie-Hématologie et Rhumatologie Pédiatrique, Centre de référence pour la Rhumatologie et les maladies Auto-immunes Systémiques de l'Enfant, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France.
Pierre QuartierUnité d'Immunologie-Hématologie et Rhumatologie Pédiatrique, Centre de référence pour la Rhumatologie et les maladies Auto-immunes Systémiques de l'Enfant, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France.
Florence LacailleHépatologie-Gastroentérologie-Nutrition Pédiatrique, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France.
Brigitte Bader-MeunierUnité d'Immunologie-Hématologie et Rhumatologie Pédiatrique, Centre de référence pour la Rhumatologie et les maladies Auto-immunes Systémiques de l'Enfant, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris, Paris, France.
Valentine BroussePédiatrie Générale, Centre de Référence des Hémoglobinopathies, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris Labex-GR-Ex, Paris, France.
Mariane de MontalembertPédiatrie Générale, Centre de Référence des Hémoglobinopathies, Hôpital Necker-Enfants Malades, Assistance Publique Hôpitaux de Paris Labex-GR-Ex, Paris, France.
Hôpital Necker-Enfants Malades · FRDélégation Paris 5 · FRCentre Hospitalier Universitaire Amiens-Picardie · FR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundPatients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY

designAmong 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID.

resultsWe identified 8 patients aged from 7 to 17 years diagnosed with AID; juvenile idiopathic arthritis (n = 3), systemic lupus erythematosus (n = 2), Sjögren's syndrome (n = 1) and autoimmune hepatitis (n = 2). The diagnosis of AID was often delayed due to similarities of the symptoms with those of SCD. Patients treated with steroids experienced multiple vaso-occlusive crises and received prophylactic chronic blood transfusions when it was possible. Tolerance to other immunosuppressive and biological treatments, such as anti-TNF agents, was good. A remission of AID was achieved in 4 patients, without worsening the course of the SCD. One patient underwent a geno-identical hematopoietic stem cell transplantation that cured both diseases. Another one underwent a successful liver transplantation.

conclusionCoexistence of AID and SCD generates diagnostic and therapeutic challenges. Early diagnosis of AID is important to define the best treatment, which may include targeted biological therapy.

Indexed as

AdolescentAnemia, Sickle CellAutoimmune DiseasesBiological FactorsChildComorbidityFemaleHumansImmunosuppressive AgentsMaleRetrospective StudiesBiological FactorsImmunosuppressive AgentsAutoimmune diseaseBiological therapyChildrenJuvenile idiopathic arthritisSickle cell diseaseSystemic lupus erythematosus

Identifiers

PMID29343274
PMCPMC5772694
OpenAlexW2787961971

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.