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Pulmonary interstitial glycogenosis

MedGen UID:
838075
•Concept ID:
C3161106
•
Disease or Syndrome
Synonyms: Infantile cellular interstitial pneumonitis; infantile cellular interstitial pneumonitis; PIG; pig; PIG - Pulmonary interstitial glycogenosis; pulmonary interstitial glycogenosis
SNOMED CT: Pulmonary interstitial glycogenosis (707551007); PIG - Pulmonary interstitial glycogenosis (707551007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016321
Orphanet: ORPHA217557

Definition

Pulmonary interstitial glycogenosis (PIG) is a rare non-lethal pediatric form of interstitial lung disease (ILD, see this term). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPulmonary interstitial glycogenosis

Recent clinical studies

Etiology

Congenital interstitial lung diseases: What the anesthesiologist needs to know.
Bertolizio G, Engelhardt T, Veyckemans F
Paediatr Anaesth 2022 Feb;32(2):138-147. Epub 2021 Nov 11 doi: 10.1111/pan.14325. PMID: 34738691
Early onset children's interstitial lung diseases: Discrete entities or manifestations of pulmonary dysmaturity?
Bush A, Griese M, Seidl E, Kerem E, Reu S, Nicholson AG
Paediatr Respir Rev 2019 Apr;30:65-71. Epub 2018 Oct 9 doi: 10.1016/j.prrv.2018.09.004. PMID: 30552058
Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course.
Liptzin DR, Baker CD, Darst JR, Weinman JP, Dishop MK, Galambos C, Brinton JT, Deterding RR
Pediatr Pulmonol 2018 Dec;53(12):1651-1658. Epub 2018 Jul 18 doi: 10.1002/ppul.24123. PMID: 30019520Free PMC Article
Pulmonary interstitial glycogenosis - A systematic analysis of new cases.
Seidl E, Carlens J, Reu S, Wetzke M, Ley-Zaporozhan J, Brasch F, Wesselak T, Schams A, Rauch D, Schuch L, Kappler M, Schelstraete P, Wolf M, Stehling F, Haarmann E, Borensztajn D, van de Loo M, Rubak S, Lex C, Hinrichs B, Reiter K, Schwerk N, Griese M
Respir Med 2018 Jul;140:11-20. Epub 2018 May 17 doi: 10.1016/j.rmed.2018.05.009. PMID: 29957271
Rare Lung Diseases: Interstitial Lung Diseases and Lung Manifestations of Rheumatological Diseases.
Ramamurthy MB, Goh DY, Lim MT
Indian J Pediatr 2015 Oct;82(10):956-61. Epub 2015 Aug 20 doi: 10.1007/s12098-015-1867-3. PMID: 26286176

Diagnosis

Congenital interstitial lung diseases: What the anesthesiologist needs to know.
Bertolizio G, Engelhardt T, Veyckemans F
Paediatr Anaesth 2022 Feb;32(2):138-147. Epub 2021 Nov 11 doi: 10.1111/pan.14325. PMID: 34738691
Interstitial lung disease in infancy.
Bush A, Gilbert C, Gregory J, Nicholson AG, Semple T, Pabary R
Early Hum Dev 2020 Nov;150:105186. Epub 2020 Sep 15 doi: 10.1016/j.earlhumdev.2020.105186. PMID: 32958330
Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course.
Liptzin DR, Baker CD, Darst JR, Weinman JP, Dishop MK, Galambos C, Brinton JT, Deterding RR
Pediatr Pulmonol 2018 Dec;53(12):1651-1658. Epub 2018 Jul 18 doi: 10.1002/ppul.24123. PMID: 30019520Free PMC Article
Pulmonary interstitial glycogenosis - A systematic analysis of new cases.
Seidl E, Carlens J, Reu S, Wetzke M, Ley-Zaporozhan J, Brasch F, Wesselak T, Schams A, Rauch D, Schuch L, Kappler M, Schelstraete P, Wolf M, Stehling F, Haarmann E, Borensztajn D, van de Loo M, Rubak S, Lex C, Hinrichs B, Reiter K, Schwerk N, Griese M
Respir Med 2018 Jul;140:11-20. Epub 2018 May 17 doi: 10.1016/j.rmed.2018.05.009. PMID: 29957271
Interstitial lung disease in children.
Cazzato S, di Palmo E, Ragazzo V, Ghione S
Early Hum Dev 2013 Oct;89 Suppl 3:S39-43. Epub 2013 Aug 22 doi: 10.1016/j.earlhumdev.2013.07.021. PMID: 23973003

Therapy

Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course.
Liptzin DR, Baker CD, Darst JR, Weinman JP, Dishop MK, Galambos C, Brinton JT, Deterding RR
Pediatr Pulmonol 2018 Dec;53(12):1651-1658. Epub 2018 Jul 18 doi: 10.1002/ppul.24123. PMID: 30019520Free PMC Article
Pulmonary interstitial glycogenosis - A systematic analysis of new cases.
Seidl E, Carlens J, Reu S, Wetzke M, Ley-Zaporozhan J, Brasch F, Wesselak T, Schams A, Rauch D, Schuch L, Kappler M, Schelstraete P, Wolf M, Stehling F, Haarmann E, Borensztajn D, van de Loo M, Rubak S, Lex C, Hinrichs B, Reiter K, Schwerk N, Griese M
Respir Med 2018 Jul;140:11-20. Epub 2018 May 17 doi: 10.1016/j.rmed.2018.05.009. PMID: 29957271
An infant with pulmonary interstitial glycogenosis: clinical improvement is associated with improvement in the pulmonary diffusion capacity.
Ehsan Z, Montgomery GS, Tiller C, Kisling J, Chang DV, Tepper RS
Pediatr Pulmonol 2014 Mar;49(3):E17-20. Epub 2013 Feb 8 doi: 10.1002/ppul.22738. PMID: 23401236
Histologic resolution of pulmonary interstitial glycogenosis.
Deutsch GH, Young LR
Pediatr Dev Pathol 2009 Nov-Dec;12(6):475-80. doi: 10.2350/08-12-0575.1. PMID: 19320535
Pulmonary interstitial glycogenosis: a new variant of neonatal interstitial lung disease.
Canakis AM, Cutz E, Manson D, O'Brodovich H
Am J Respir Crit Care Med 2002 Jun 1;165(11):1557-65. doi: 10.1164/rccm.2105139. PMID: 12045133

Prognosis

Congenital interstitial lung diseases: What the anesthesiologist needs to know.
Bertolizio G, Engelhardt T, Veyckemans F
Paediatr Anaesth 2022 Feb;32(2):138-147. Epub 2021 Nov 11 doi: 10.1111/pan.14325. PMID: 34738691
Interstitial lung disease in infancy.
Bush A, Gilbert C, Gregory J, Nicholson AG, Semple T, Pabary R
Early Hum Dev 2020 Nov;150:105186. Epub 2020 Sep 15 doi: 10.1016/j.earlhumdev.2020.105186. PMID: 32958330
Pulmonary interstitial glycogenosis: Diagnostic evaluation and clinical course.
Liptzin DR, Baker CD, Darst JR, Weinman JP, Dishop MK, Galambos C, Brinton JT, Deterding RR
Pediatr Pulmonol 2018 Dec;53(12):1651-1658. Epub 2018 Jul 18 doi: 10.1002/ppul.24123. PMID: 30019520Free PMC Article
Pulmonary interstitial glycogenosis associated with a spectrum of neonatal pulmonary disorders.
Cutz E, Chami R, Dell S, Langer J, Manson D
Hum Pathol 2017 Oct;68:154-165. Epub 2017 Sep 2 doi: 10.1016/j.humpath.2017.06.026. PMID: 28873355
Pulmonary interstitial glycogenosis.
Lanfranchi M, Allbery SM, Wheelock L, Perry D
Pediatr Radiol 2010 Mar;40(3):361-5. Epub 2009 Nov 10 doi: 10.1007/s00247-009-1455-7. PMID: 19902199

Clinical prediction guides

Isolated pulmonary interstitial glycogenosis associated with alveolar growth abnormalities: A long-term follow-up study.
Sardón O, Torrent-Vernetta A, Rovira-Amigo S, Dishop MK, Ferreres JC, Navarro A, Corcuera P, Korta-Murua J, Peña PG, Pérez-Belmonte E, Villares A, Camats N, Fernández-Cancio M, Carrascosa A, Pérez-Yarza EG, Moreno-Galdó A
Pediatr Pulmonol 2019 Jun;54(6):837-846. Epub 2019 Mar 25 doi: 10.1002/ppul.24324. PMID: 30912317
High-resolution CT findings of pulmonary interstitial glycogenosis.
Weinman JP, White CJ, Liptzin DR, Deterding RR, Galambos C, Browne LP
Pediatr Radiol 2018 Aug;48(8):1066-1072. Epub 2018 Apr 23 doi: 10.1007/s00247-018-4138-4. PMID: 29687227
An infant with pulmonary interstitial glycogenosis: clinical improvement is associated with improvement in the pulmonary diffusion capacity.
Ehsan Z, Montgomery GS, Tiller C, Kisling J, Chang DV, Tepper RS
Pediatr Pulmonol 2014 Mar;49(3):E17-20. Epub 2013 Feb 8 doi: 10.1002/ppul.22738. PMID: 23401236

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