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Progressive pulmonary function impairment

MedGen UID:
341487
Concept ID:
C1849570
Finding
HPO: HP:0006520

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProgressive pulmonary function impairment

Conditions with this feature

PULMONARY ALVEOLAR MICROLITHIASIS
MedGen UID:
56374
Concept ID:
C0155912
Disease or Syndrome
Pulmonary alveolar microlithiasis (PULAM) is a rare disease characterized by the deposition of calcium phosphate microliths throughout the lungs. Most patients are asymptomatic for several years or even for decades, and, generally, the diagnosis is incidental to clinical investigations unrelated to the specific disorder. Cases with early onset or rapid progression are rare. A 'sandstorm-appearing' chest roentgenogram is a typical diagnostic finding. The onset of this potentially lethal disease varies from the neonatal period to old age, and the disease follows a long-term progressive course, resulting in a slow deterioration of lung functions. About one-third of the reported cases are said to be familial (summary by Corut et al., 2006).

Professional guidelines

PubMed

Frank AJ, Dagogo-Jack I, Dobre IA, Tait S, Schumacher L, Fintelmann FJ, Fingerman LM, Keane FK, Montesi SB
Oncologist 2023 Jan 18;28(1):12-22. doi: 10.1093/oncolo/oyac226. PMID: 36426803Free PMC Article
Bosi A, Tonelli R, Castaniere I, Clini E, Beghè B
Minerva Med 2021 Oct;112(5):605-614. Epub 2021 Feb 26 doi: 10.23736/S0026-4806.21.07372-9. PMID: 33634676
Arvanitaki A, Giannakoulas G, Baumgartner H, Lammers AE
Heart 2020 Nov;106(21):1638-1645. Epub 2020 Jul 20 doi: 10.1136/heartjnl-2020-316665. PMID: 32690623

Recent clinical studies

Diagnosis

Oltvai ZN, Smith EA, Wiens K, Nogee LM, Luquette M, Nelson AC, Wikenheiser-Brokamp KA
Cold Spring Harb Mol Case Stud 2020 Jun;6(3) Epub 2020 Jun 12 doi: 10.1101/mcs.a005074. PMID: 32532878Free PMC Article

Prognosis

Oltvai ZN, Smith EA, Wiens K, Nogee LM, Luquette M, Nelson AC, Wikenheiser-Brokamp KA
Cold Spring Harb Mol Case Stud 2020 Jun;6(3) Epub 2020 Jun 12 doi: 10.1101/mcs.a005074. PMID: 32532878Free PMC Article

Clinical prediction guides

Oltvai ZN, Smith EA, Wiens K, Nogee LM, Luquette M, Nelson AC, Wikenheiser-Brokamp KA
Cold Spring Harb Mol Case Stud 2020 Jun;6(3) Epub 2020 Jun 12 doi: 10.1101/mcs.a005074. PMID: 32532878Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

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