Evidence map›Paper›PMID 35681534›Full record

ReviewCells2022

Biomarkers in Nephropathic Cystinosis: Current and Future Perspectives.

Francesco Emma, Giovanni Montini, Marco Pennesi, Licia Peruzzi, Enrico Verrina, Bianca Maria Goffredo, Fabrizio Canalini, David Cassiman, Silvia Rossi, Elena Levtchenko

Abstract readReview
In one paragraph

Review in Cells, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed, 1 pooled it
–field-weighted citation impact
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

7 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Article
  3. Review
  4. Article
  5. Article
  6. Article
  7. Article
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.

Francesco EmmaDepartment of Pediatric Subspecialties, Division of Nephrology, Bambino Gesù Children's Hospital-IRCCS, 00165 Rome, Italy.ORCID 0000-0002-0383-3468
Giovanni MontiniPediatric Nephrology, Dialysis and Transplant Unit, Fondazione Ca' Grande IRRCS Ospedale Maggiore Policlinico, 20122 Milan, Italy.ORCID 0000-0002-7350-4475
Marco PennesiInstitute for Maternal and Child Health, IRCCS Burlo Garofolo, 34137 Trieste, Italy.
Licia PeruzziPediatric Nephrology Unit, Regina Margherita Children's Hospital, AOU Città della Salute e della Scienza di Torino, 10126 Turin, Italy.ORCID 0000-0002-5704-3867
Enrico VerrinaDialysis Unit, Department of Pediatrics, IRCCS Istituto Giannina Gaslini, 16147 Genoa, Italy.
Bianca Maria GoffredoDepartment of Pediatric Subspecialties, Division of Metabolic Diseases, Bambino Gesù Children's Hospital-IRCCS, 00165 Rome, Italy.
Fabrizio CanaliniMedical Department, Chiesi Pharmaceutics, 43100 Parma, Italy.
David CassimanDepartment of Metabolic Diseases, University Hospitals Leuven, 3000 Leuven, Belgium.
Silvia RossiMedical Department, Chiesi Pharmaceutics, 43100 Parma, Italy.
Elena LevtchenkoDepartment of Pediatric Nephrology and Development and Regeneration, University Hospitals Leuven, University of Leuven, 3000 Leuven, Belgium.ORCID 0000-0002-8352-7312

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Early diagnosis and effective therapy are essential for improving the overall prognosis and quality of life of patients with nephropathic cystinosis. The severity of kidney dysfunction and the multi-organ involvement as a consequence of the increased intracellular concentration of cystine highlight the necessity of accurate monitoring of intracellular cystine to guarantee effective treatment of the disease. Cystine depletion is the only available treatment, which should begin immediately after diagnosis, and not discontinued, to significantly slow progression of renal and extra-renal organ damage. This review aims to discuss the importance of the close monitoring of intracellular cystine concentration to optimize cystine depletion therapy. In addition, the role of new biomarkers in the management of the disease, from timely diagnosis to implementing treatment during follow-up, is overviewed.

Indexed as

CystinosisFanconi SyndromeBiomarkersCysteamineCystineHumansQuality of LifeBiomarkersCysteamineCystinebiomarkerscysteaminecystinekidneynephropathic cystinosistherapeutic monitoring

Identifiers

PMID35681534
PMCPMC9180050

What OpenQuestion holds

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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.