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Airway obstruction

MedGen UID:
1387
Concept ID:
C0001883
Disease or Syndrome
Synonym: Obstructive lung disease
SNOMED CT: Embarrassed airway (79688008); Respiratory obstruction (79688008)
 
HPO: HP:0006536
Monarch Initiative: MONDO:0002267

Definition

Obstruction of conducting airways of the lung. [from HPO]

Conditions with this feature

Fabry disease
MedGen UID:
8083
Concept ID:
C0002986
Disease or Syndrome
Fabry disease is the most common of the lysosomal storage disorders and results from deficient activity of the enzyme alpha-galactosidase A (a-Gal A), leading to progressive lysosomal deposition of globotriaosylceramide and its derivatives in cells throughout the body. The classic form, occurring in males with less than 1% a-Gal A enzyme activity, usually has its onset in childhood or adolescence with periodic crises of severe pain in the extremities (acroparesthesia), the appearance of vascular cutaneous lesions (angiokeratomas), sweating abnormalities (anhidrosis, hypohidrosis, and rarely hyperhidrosis), characteristic corneal and lenticular opacities, and proteinuria. Gradual deterioration of renal function to end-stage renal disease (ESRD) usually occurs in men in the third to fifth decade. In middle age, most males successfully treated for ESRD develop cardiac and/or cerebrovascular disease, a major cause of morbidity and mortality. Heterozygous females typically have milder symptoms at a later age of onset than males. Rarely, females may be relatively asymptomatic throughout a normal life span or may have symptoms as severe as those observed in males with the classic phenotype. In contrast, late-onset forms occur in males with greater than 1% a-Gal A activity. Clinical manifestations include cardiac disease, which usually presents in the sixth to eighth decade with left ventricular hypertrophy, cardiomyopathy, arrhythmia, and proteinuria; renal failure, associated with ESRD but without the skin lesions or pain; or cerebrovascular disease presenting as stroke or transient ischemic attack.
Mucopolysaccharidosis, MPS-II
MedGen UID:
7734
Concept ID:
C0026705
Disease or Syndrome
Mucopolysaccharidosis type II (MPS II; also known as Hunter syndrome) is an X-linked multisystem disorder characterized by glycosaminoglycan (GAG) accumulation. The vast majority of affected individuals are male; on rare occasion heterozygous females manifest findings. Age of onset, disease severity, and rate of progression vary significantly among affected males. In those with early progressive disease, CNS involvement (manifest primarily by progressive cognitive deterioration), progressive airway disease, and cardiac disease usually result in death in the first or second decade of life. In those with slowly progressive disease, the CNS is not (or is minimally) affected, although the effect of GAG accumulation on other organ systems may be early progressive to the same degree as in those who have progressive cognitive decline. Survival into the early adult years with normal intelligence is common in the slowly progressing form of the disease. Additional findings in both forms of MPS II include: short stature; macrocephaly with or without communicating hydrocephalus; macroglossia; hoarse voice; conductive and sensorineural hearing loss; hepatosplenomegaly; dysostosis multiplex; spinal stenosis; and carpal tunnel syndrome.
Mucopolysaccharidosis type 7
MedGen UID:
43108
Concept ID:
C0085132
Disease or Syndrome
Mucopolysaccharidosis type VII is an autosomal recessive lysosomal storage disease characterized by the inability to degrade glucuronic acid-containing glycosaminoglycans. The phenotype is highly variable, ranging from severe lethal hydrops fetalis to mild forms with survival into adulthood. Most patients with the intermediate phenotype show hepatomegaly, skeletal anomalies, coarse facies, and variable degrees of mental impairment (Shipley et al., 1993). MPS VII was the first autosomal mucopolysaccharidosis for which chromosomal assignment was achieved.
Marshall-Smith syndrome
MedGen UID:
75551
Concept ID:
C0265211
Disease or Syndrome
The Marshall-Smith syndrome (MRSHSS) is a malformation syndrome characterized by accelerated skeletal maturation, relative failure to thrive, respiratory difficulties, mental retardation, and unusual facies, including prominent forehead, shallow orbits, blue sclerae, depressed nasal bridge, and micrognathia (Adam et al., 2005).
Keutel syndrome
MedGen UID:
383722
Concept ID:
C1855607
Disease or Syndrome
Keutel syndrome (KTLS) is an autosomal recessive disorder characterized by multiple peripheral pulmonary stenoses, brachytelephalangy, inner ear deafness, and abnormal cartilage ossification or calcification (summary by Khosroshahi et al., 2014).

Professional guidelines

PubMed

Smith DK, McDermott AJ, Sullivan JF
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Recent clinical studies

Etiology

Kuriyama A, Jackson JL, Kamei J
Crit Care 2020 Nov 7;24(1):640. doi: 10.1186/s13054-020-03358-8. PMID: 33160405Free PMC Article
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Diagnosis

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Indian J Pediatr 2015 Aug;82(8):737-44. Epub 2015 Jun 25 doi: 10.1007/s12098-015-1811-6. PMID: 26104110
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Therapy

Moraa I, Sturman N, McGuire TM, van Driel ML
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Cochrane Database Syst Rev 2020 Jan 17;1(1):CD007074. doi: 10.1002/14651858.CD007074.pub3. PMID: 31978261Free PMC Article
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Med Intensiva 2015 Aug-Sep;39(6):359-72. Epub 2015 Jan 17 doi: 10.1016/j.medin.2014.11.003. PMID: 25599942

Prognosis

Salari N, Khazaie H, Abolfathi M, Ghasemi H, Shabani S, Rasoulpoor S, Mohammadi M, Rasoulpoor S, Khaledi-Paveh B
Neurol Sci 2022 Jan;43(1):219-231. Epub 2021 Nov 19 doi: 10.1007/s10072-021-05765-3. PMID: 34797460
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Clinical prediction guides

Van den Bossche K, Van de Perck E, Kazemeini E, Willemen M, Van de Heyning PH, Verbraecken J, Op de Beeck S, Vanderveken OM
Sleep Med Rev 2021 Dec;60:101534. Epub 2021 Aug 3 doi: 10.1016/j.smrv.2021.101534. PMID: 34418668
Kuriyama A, Jackson JL, Kamei J
Crit Care 2020 Nov 7;24(1):640. doi: 10.1186/s13054-020-03358-8. PMID: 33160405Free PMC Article
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Int J Chron Obstruct Pulmon Dis 2016;11:3007-3013. Epub 2016 Nov 30 doi: 10.2147/COPD.S54927. PMID: 27942210Free PMC Article
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Int J Chron Obstruct Pulmon Dis 2016;11 Spec Iss(Spec Iss):13-20. Epub 2016 Feb 19 doi: 10.2147/COPD.S85977. PMID: 26937186Free PMC Article

Recent systematic reviews

Bergamini M, Simeone G, Verga MC, Doria M, Cuomo B, D'Antonio G, Dello Iacono I, Di Mauro G, Leonardi L, Miniello VL, Palma F, Scotese I, Tezza G, Caroli M, Vania A
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Couper K, Abu Hassan A, Ohri V, Patterson E, Tang HT, Bingham R, Olasveengen T, Perkins GD; International Liaison Committee on Resuscitation Basic and Paediatric Life Support Task Force Collaborators
Resuscitation 2020 Nov;156:174-181. Epub 2020 Sep 16 doi: 10.1016/j.resuscitation.2020.09.007. PMID: 32949674
Zhang L, Mendoza-Sassi RA, Wainwright C, Klassen TP
Cochrane Database Syst Rev 2017 Dec 21;12(12):CD006458. doi: 10.1002/14651858.CD006458.pub4. PMID: 29265171Free PMC Article
Magis-Escurra C, Reijers MH
BMJ Clin Evid 2015 Feb 25;2015 PMID: 25715965Free PMC Article
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