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Intraalveolar nodular calcifications

MedGen UID:
870579
Concept ID:
C4025028
Finding
HPO: HP:0006514

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIntraalveolar nodular calcifications

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).

Recent clinical studies

Diagnosis

Hartel PH, Shilo K, Klassen-Fischer M, Neafie RC, Ozbudak IH, Galvin JR, Franks TJ
Am J Surg Pathol 2010 May;34(5):730-4. doi: 10.1097/PAS.0b013e3181d9f16a. PMID: 20414100

Prognosis

Hartel PH, Shilo K, Klassen-Fischer M, Neafie RC, Ozbudak IH, Galvin JR, Franks TJ
Am J Surg Pathol 2010 May;34(5):730-4. doi: 10.1097/PAS.0b013e3181d9f16a. PMID: 20414100

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