Evidence map›Paper›PMID 29986738›Full record

ArticleOrphanet journal of rare diseases2018

Challenges of palliative care in children with inborn metabolic diseases.

Jessica I Hoell, Jens Warfsmann, Felix Distelmaier, Arndt Borkhardt, Gisela Janßen, Michaela Kuhlen

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

Article in Orphanet journal of rare diseases, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.

0numbers the graph read from it
0cells of the map it votes in
12citing papers in PubMed
1.1field-weighted citation impact, top 18% 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

12 citing papers in PubMed, 18 citations in OpenAlex.

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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

6 authors at 1 institution in 1 country.

Jessica I HoellMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany.
Jens WarfsmannMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany.
Felix DistelmaierMedical Faculty, Department of General Paediatrics, Neonatology and Paediatric Cardiology, Centre for Child and Adolescent Health, University of Duesseldorf, Moorenstr. 5, 40225 Duesseldorf, Germany.
Arndt BorkhardtMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany.
Gisela JanßenMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany.
Michaela KuhlenMedical Faculty, Department of Paediatric Oncology, Haematology and Clinical Immunology, Centre for Child and Adolescent Health, University of Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany. Michaela.Kuhlen@med.uni-duesseldorf.de.ORCID 0000-0003-4577-0503
Heinrich Heine University Düsseldorf · DE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundOur objective was to evaluate children with metabolic diseases in paediatric palliative home care (PPC) and the process of decision-making. This study was conducted as single-centre retrospective cohort study of patients in the care of a large specialized PPC team.

resultsBetween 01/2013 and 09/2016, 198 children, adolescents and young adults were in the care of our PPC team. Twenty-nine (14.6%) of these patients had metabolic conditions. Median age at referral was 2.6 years (0-24), median duration of care 352 days (3-2248) and median number of home visits 13 (1-80). Most patients are still alive (16; 55.2%). Median number of drugs administered was 5 (range 0-12), antiepileptics were given most frequently. Symptom burden was high in all children with metabolic disorders at referral and remained high throughout care. Predominant symptoms were gastrointestinal, respiratory and neurologic symptoms. Children with metabolic conditions, who were referred to PPC younger than 1 year of age had a shorter period of care and died earlier compared to those children, who were referred to PPC later in their lives (older than 10 years of age). Eleven (37.9%) of the children initially had no resuscitation restrictions and 7 (53.8%) of those who died, did so on ICU.

conclusionsAbout 15% of children with life-limiting conditions in PPC present with metabolic diseases. Symptom burden is high with neurologic, respiratory and gastrointestinal symptoms being the most frequent and most of those being difficult to treat. In these children, particular attention needs to be addressed to advance care planning.

Indexed as

Metabolism, Inborn ErrorsAdolescentAdultChildChild, PreschoolDecision MakingFemaleHumansInfantInfant, NewbornMaleMetabolic DiseasesNervous System DiseasesPalliative CareYoung AdultChildrenEnd-of-life careMetabolic disease, neurological diseasePalliative care

Identifiers

PMID29986738
PMCPMC6038293
OpenAlexW2881037939

What OpenQuestion holds

Textmetadata
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.