Evidence map›Paper›PMID 37547314›Full record

SynthesisFrontiers in endocrinology2023

Kidney disease in adults with Prader-Willi syndrome: international cohort study and systematic literature review.

Denise H van Abswoude, Karlijn Pellikaan, Naomi Nguyen, Anna G W Rosenberg, Kirsten Davidse, Franciska M E Hoekstra, Ilse M Rood, Christine Poitou, Graziano Grugni, Charlotte Høybye and 6 more

Erratum issuedAbstract readSystematic Review
In one paragraph

Synthesis in Frontiers in endocrinology, 2023. 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 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–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

1 citing paper in PubMed.

  1. Review
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

16 authors.

Denise H van AbswoudeDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Karlijn PellikaanDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Naomi NguyenDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Anna G W RosenbergDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Kirsten DavidseDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Franciska M E HoekstraDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Ilse M RoodDepartment of Nephrology, Radboud University Medical Center, Radboud Institute for Health Sciences, Nijmegen, Netherlands.
Christine PoitouAssistance Publique-Hôpitaux de Paris, Rare Diseases Center of Reference 'Prader-Willi Syndrome and Obesity with Eating Disorders' (PRADORT), Nutrition Department, Institute of Cardiometabolism and Nutrition (ICAN), Pitié-Salpêtrière Hospital, Sorbonne Université, National Institute of Health and Medical Research (INSERM), Nutriomics, Paris, France.
Graziano GrugniInternational Network for Research, Management & Education on adults with Prader-Willi Syndrome (INfoRMEd-PWS).
Charlotte HøybyeInternational Network for Research, Management & Education on adults with Prader-Willi Syndrome (INfoRMEd-PWS).
Tania P MarkovicInternational Network for Research, Management & Education on adults with Prader-Willi Syndrome (INfoRMEd-PWS).
Assumpta CaixàsInternational Network for Research, Management & Education on adults with Prader-Willi Syndrome (INfoRMEd-PWS).
Antonino CrinòInternational Network for Research, Management & Education on adults with Prader-Willi Syndrome (INfoRMEd-PWS).
Sjoerd A A van den BergDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Aart J van der LelyDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.
Laura C G de GraaffDepartment of Internal Medicine, Division of Endocrinology, Erasmus Medical Center, University Medical Center Rotterdam, Rotterdam, Netherlands.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Prader-Willi syndrome (PWS) is a rare, complex, genetic disorder characterized by hyperphagia, hypotonia, delayed psychomotor development, low muscle mass and hypothalamic dysfunction. Adults with PWS often have obesity, hypertension and type 2 diabetes mellitus (DM2), known risk factors for cardiovascular disease (CVD) and chronic kidney disease (CKD). Early symptoms of CVD and CKD may be masked by intellectual disability and inability to express physical complaints. Furthermore, kidney diseases are often asymptomatic. Therefore, renal and cardiovascular disease might be missed in patients with PWS. Microalbuminuria is an early sign of microvascular damage in the kidneys and other vascular beds. Therefore, we screened our adult PWS cohort for the presence of elevated urinary albumin and (micro)albuminuria. Methods: We retrospectively collected anthropometric measurements, blood pressure, medical history, medication use, urine dipstick and biochemical measurements form electronic patient files. In addition, we performed a systematic literature review on kidney disease in PWS. Results: We included 162 adults with genetically confirmed PWS (56% male, median age 28 years), of whom 44 (27%) had DM2. None had known CVD. All subjects had normal estimated glomerular filtration rate (eGFR) according to non-PWS reference intervals. Elevated urinary albumin or (micro)albuminuria was present in 28 (18%); 19 out of 75 (25%) had an increased urinary albumin-to-creatinine ratio (UACR) and 10 out of 57 (18%) had an increased urinary protein-to-creatinine ratio. Elevated urinary albumin was present at a young age (median age 26 (IQR 24-32) years) and was associated with an significantly higher BMI and LDL-cholesterol levels and higher prevalence of DM2, hypertension and dyslipidemia than those with normal UACR ( Conclusion: Upon screening, one in every five adults with PWS had increased urinary albumin or (micro)albuminuria, early signs of microvascular disease. All had normal eGFR, according to non-PWS reference intervals, and none had a formal diagnosis of CVD. As muscle mass is low in PWS, creatinine levels and eGFR may be spuriously normal. Urinalysis in this patient group can be used as a screening tool for microvascular (kidney) disease. We propose an algorithm for the detection and management of microvascular disease in adults with PWS.

Indexed as

Cardiovascular DiseasesDiabetes Mellitus, Type 2HypertensionPrader-Willi SyndromeRenal Insufficiency, ChronicAdultAlbuminsAlbuminuriaCohort StudiesCreatinineFemaleHumansMaleRetrospective StudiesYoung AdultAlbuminsCreatininecardiovascular diseasekidney diseasekidney function testsPrader-Willi Syndromeproteinuriaurine tract infections

Identifiers

PMID37547314
PMCPMC10402738

What Socratic holds

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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 Socratic graph.