Evidence map›Paper›PMID 27514596›Full record

ReviewClinics in chest medicine2016

Hermansky-Pudlak Syndrome.

Souheil El-Chemaly, Lisa R Young

Abstract readReview
In one paragraph

Review in Clinics in chest medicine, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers.

0numbers the graph read from it
0cells of the map it votes in
50citing papers in PubMed
5.9field-weighted citation impact, top 4% 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

50 citing papers in PubMed, 113 citations in OpenAlex.

  1. Article
  2. Anandamide Is a Potential Blood Biomarker of Hermansky-Pudlak Syndrome Pulmonary Fibrosis.American journal of respiratory and critical care medicine · 2025
    Article
  3. Review
  4. Article
  5. Article
  6. Article
  7. Article
  8. Review
  9. Rare interstitial lung diseases: a narrative review.Journal of thoracic disease · 2024
    Review
  10. Article
  11. Article
  12. Review
  13. Article
  14. Review
  15. A case of Hermansky-Pudlak with dyspnea.Oxford medical case reports · 2023
    Article
  16. Article
  17. Review
  18. Article
  19. Article
  20. 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

2 authors at 2 institutions in 1 country.

Souheil El-ChemalyDivision of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.
Lisa R YoungDivision of Pulmonary Medicine, Department of Pediatrics, Vanderbilt University School of Medicine, 2200 Children's Way, Doctor's Office Tower 11215, Nashville, TN 37232, USA; Division of Allergy, Pulmonary, and Critical Care, Department of Medicine, Vanderbilt University School of Medicine, 1161 21st Avenue South, B-1220 Medical Center North, Nashville, TN 37232, USA. Electronic address: lisa.young@vanderbilt.edu.
Brigham and Women's Hospital · USVanderbilt University Medical Center · US

Funding

RLDC: Molecular Pathway-Driven Diagnostics & Therapeutics for Rare Lung DiseasesU54HL127672 · NHLBI · CINCINNATI CHILDRENS HOSP MED CTR · PI TRAPNELL, BRUCE C · 2014 to 2018
$3.7M
Mechanisms of Pulmonary Fibrosis in Hermansky-Pudlak SyndromeR01HL119503 · NHLBI · VANDERBILT UNIVERSITY MEDICAL CENTER · PI YOUNG, LISA R. · 2013 to 2021
$3.6M
NHLBI NIH HHS R01 HL119503NHLBI NIH HHS U54 HL127672
6 · The paper itself

Abstract

Hermansky-Pudlak syndrome (HPS) is an autosomal recessive disorder that is associated with oculocutaneous albinism, bleeding diatheses, granulomatous colitis, and highly penetrant pulmonary fibrosis in some subtypes, including HPS-1, HPS-2, and HPS-4. HPS pulmonary fibrosis shows many of the clinical, radiologic, and histologic features found in idiopathic pulmonary fibrosis, but occurs at a younger age. Despite knowledge of the underlying genetic defects, there are currently no definitive therapeutic or preventive approaches for HPS pulmonary fibrosis other than lung transplant.

Indexed as

AlbinismAlbinism, OculocutaneousArteriovenous MalformationsBlood Coagulation DisordersCrohn DiseaseEpistaxisGastrointestinal DiseasesHemorrhagic DisordersHermanski-Pudlak SyndromeHumansHypertension, PulmonaryIntracranial Arteriovenous MalformationsLiver DiseasesPulmonary ArteryPulmonary FibrosisPulmonary VeinsAdaptor protein 3AlbinismBiogenesis of lysosome-related organelle complexHermansky-Pudlak syndromeInterstitial lung diseasePulmonary fibrosis

Identifiers

PMID27514596
PMCPMC4987498
OpenAlexW262736855

What OpenQuestion holds

Textmetadata
LicenceTDM
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.