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Interstitial emphysema

MedGen UID:
234586
Concept ID:
C1370824
Disease or Syndrome
Synonym: Interstitial emphysema of lung
SNOMED CT: Interstitial emphysema of lung (77690003); Interstitial emphysema (11211003); Interstitial pulmonary emphysema (77690003)
 
HPO: HP:0032965
Monarch Initiative: MONDO:0000923

Definition

Interstitial emphysema is characterized by air dissecting within the interstitium of the lung, typically in the peribronchovascular sheaths, interlobular septa, and visceral pleura. It is most commonly seen in neonates receiving mechanical ventilation. It is rarely recognized radiographically in adults and is infrequently seen on CT scans. It appears as perivascular lucent or low attenuating halos and small cysts. [from HPO]

Conditions with this feature

Chitayat syndrome
MedGen UID:
934646
Concept ID:
C4310679
Disease or Syndrome
Chitayat syndrome (CHYTS) is a rare condition characterized by respiratory distress presenting at birth, bilateral accessory phalanx resulting in shortened index fingers with ulnar deviation, hallux valgus, and characteristic facial features including prominent eyes, hypertelorism, depressed nasal bridge, full lips, and upturned nose (summary by Balasubramanian et al., 2017).
Gastrointestinal defects and immunodeficiency syndrome 2
MedGen UID:
1811526
Concept ID:
C5676901
Disease or Syndrome
PI4KA-related disorder is a clinically variable disorder characterized primarily by neurologic dysfunction (limb spasticity, developmental delay, intellectual disability, seizures, ataxia, nystagmus), gastrointestinal manifestations (multiple intestinal atresia, inflammatory bowel disease), and combined immunodeficiency (leukopenia, variable immunoglobulin defects). Age of onset is typically antenatal or in early childhood; individuals can present with any combination of these features. Rare individuals present with later-onset hereditary spastic paraplegia. Brain MRI findings can include hypomyelinating leukodystrophy, cerebellar hypoplasia/atrophy, thin or dysplastic corpus callosum, and/or perisylvian polymicrogyria.

Professional guidelines

PubMed

Ng G, da Silva O, Ohlsson A
Cochrane Database Syst Rev 2016 Dec 14;12(12):CD003214. doi: 10.1002/14651858.CD003214.pub3. PMID: 27960245Free PMC Article
Little DC, Pratt TC, Blalock SE, Krauss DR, Cooney DR, Custer MD
J Pediatr Surg 2003 Mar;38(3):492-6. doi: 10.1053/jpsu.2003.50086. PMID: 12632374
Yost CC, Soll RF
Cochrane Database Syst Rev 2000;(2):CD001456. doi: 10.1002/14651858.CD001456. PMID: 10796266

Recent clinical studies

Etiology

Kastein K, Coe K
Adv Neonatal Care 2023 Apr 1;23(2):160-166. Epub 2023 Jan 31 doi: 10.1097/ANC.0000000000001040. PMID: 36719092
Diaz A, Bujnowski D, McMullen P, Lysandrou M, Ananthanarayanan V, Husain AN, Freeman R, Vigneswaran WT, Ferguson MK, Donington JS, Madariaga MLL, Abdelsattar ZM
Ann Thorac Surg 2022 Jul;114(1):301-310. Epub 2021 Jul 31 doi: 10.1016/j.athoracsur.2021.06.076. PMID: 34343471Free PMC Article
El Shahed AI, Dargaville PA, Ohlsson A, Soll R
Cochrane Database Syst Rev 2014 Dec 14;2014(12):CD002054. doi: 10.1002/14651858.CD002054.pub3. PMID: 25504256Free PMC Article
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2004 Oct 18;(4):CD000456. doi: 10.1002/14651858.CD000456.pub2. PMID: 15494996
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2001;(1):CD000456. doi: 10.1002/14651858.CD000456. PMID: 11279692

Diagnosis

Diaz A, Bujnowski D, McMullen P, Lysandrou M, Ananthanarayanan V, Husain AN, Freeman R, Vigneswaran WT, Ferguson MK, Donington JS, Madariaga MLL, Abdelsattar ZM
Ann Thorac Surg 2022 Jul;114(1):301-310. Epub 2021 Jul 31 doi: 10.1016/j.athoracsur.2021.06.076. PMID: 34343471Free PMC Article
Xiao ZY, Tao Y, Tang XY, Chen GJ, Guo L
World J Pediatr 2009 Feb;5(1):68-70. Epub 2009 Jan 27 doi: 10.1007/s12519-009-0014-5. PMID: 19172338
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2004 Oct 18;(4):CD000456. doi: 10.1002/14651858.CD000456.pub2. PMID: 15494996
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2001;(1):CD000456. doi: 10.1002/14651858.CD000456. PMID: 11279692
Cyrlak D, Milne EN, Imray TJ
Crit Rev Diagn Imaging 1984;23(1):75-117. PMID: 6399015

Therapy

Kastein K, Coe K
Adv Neonatal Care 2023 Apr 1;23(2):160-166. Epub 2023 Jan 31 doi: 10.1097/ANC.0000000000001040. PMID: 36719092
El Shahed AI, Dargaville PA, Ohlsson A, Soll R
Cochrane Database Syst Rev 2014 Dec 14;2014(12):CD002054. doi: 10.1002/14651858.CD002054.pub3. PMID: 25504256Free PMC Article
Seger N, Soll R
Cochrane Database Syst Rev 2009 Apr 15;(2):CD007836. doi: 10.1002/14651858.CD007836. PMID: 19370695
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2004 Oct 18;(4):CD000456. doi: 10.1002/14651858.CD000456.pub2. PMID: 15494996
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2001;(1):CD000456. doi: 10.1002/14651858.CD000456. PMID: 11279692

Prognosis

Koo HJ, Do KH, Chae EJ, Kim HJ, Song JS, Jang SJ, Hong SB, Huh JW, Lee E, Hong SJ
Eur Radiol 2017 Jan;27(1):203-211. Epub 2016 May 5 doi: 10.1007/s00330-016-4367-6. PMID: 27147415
Banki F, Estrera AL, Harrison RG, Miller CC 3rd, Leake SS, Mitchell KG, Khalil K, Safi HJ, Kaiser LR
Am J Surg 2013 Dec;206(6):1001-6; discussion 1006. doi: 10.1016/j.amjsurg.2013.08.009. PMID: 24296102
Xiao ZY, Tao Y, Tang XY, Chen GJ, Guo L
World J Pediatr 2009 Feb;5(1):68-70. Epub 2009 Jan 27 doi: 10.1007/s12519-009-0014-5. PMID: 19172338
Dechant KL, Faulds D
Drugs 1991 Nov;42(5):877-94. doi: 10.2165/00003495-199142050-00009. PMID: 1723378
Unger JM, England DM, Bogust GA
J Thorac Imaging 1989 Jan;4(1):86-94. doi: 10.1097/00005382-198901000-00015. PMID: 2643717

Clinical prediction guides

Kastein K, Coe K
Adv Neonatal Care 2023 Apr 1;23(2):160-166. Epub 2023 Jan 31 doi: 10.1097/ANC.0000000000001040. PMID: 36719092
Diaz A, Bujnowski D, McMullen P, Lysandrou M, Ananthanarayanan V, Husain AN, Freeman R, Vigneswaran WT, Ferguson MK, Donington JS, Madariaga MLL, Abdelsattar ZM
Ann Thorac Surg 2022 Jul;114(1):301-310. Epub 2021 Jul 31 doi: 10.1016/j.athoracsur.2021.06.076. PMID: 34343471Free PMC Article
Lee E, Son SK, Yoon J, Cho HJ, Yang SI, Jung S, Do KH, Cho YA, Lee SY, Park DU, Hong SJ
J Korean Med Sci 2018 Apr 16;33(16):e119. doi: 10.3346/jkms.2018.33.e119. PMID: 29651817Free PMC Article
Cools F, Offringa M, Askie LM
Cochrane Database Syst Rev 2015 Mar 19;2015(3):CD000104. doi: 10.1002/14651858.CD000104.pub4. PMID: 25785789Free PMC Article
Banki F, Estrera AL, Harrison RG, Miller CC 3rd, Leake SS, Mitchell KG, Khalil K, Safi HJ, Kaiser LR
Am J Surg 2013 Dec;206(6):1001-6; discussion 1006. doi: 10.1016/j.amjsurg.2013.08.009. PMID: 24296102

Recent systematic reviews

El Shahed AI, Dargaville PA, Ohlsson A, Soll R
Cochrane Database Syst Rev 2014 Dec 14;2014(12):CD002054. doi: 10.1002/14651858.CD002054.pub3. PMID: 25504256Free PMC Article
Seger N, Soll R
Cochrane Database Syst Rev 2009 Apr 15;(2):CD007836. doi: 10.1002/14651858.CD007836. PMID: 19370695
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2004 Oct 18;(4):CD000456. doi: 10.1002/14651858.CD000456.pub2. PMID: 15494996
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2001;(1):CD000456. doi: 10.1002/14651858.CD000456. PMID: 11279692
Greenough A, Milner AD, Dimitriou G
Cochrane Database Syst Rev 2000;(2):CD000456. doi: 10.1002/14651858.CD000456. PMID: 10796368

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