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Severe viral infection

MedGen UID:
479058
Concept ID:
C3277428
Disease or Syndrome; Finding
Synonym: Severe viral infections
 
HPO: HP:0031691

Definition

An unusually severe viral infection. [from HPO]

Conditions with this feature

Immunodeficiency 19
MedGen UID:
816477
Concept ID:
C3810147
Disease or Syndrome
Immunodeficiency-19 (IMD19) is an autosomal recessive form of severe combined immunodeficiency (SCID) characterized by onset in early infancy of recurrent bacterial, viral, and fungal infections. Patients usually have chronic diarrhea, recurrent respiratory infections, and failure to thrive. Immunologic work-up shows a T cell-negative, B cell-positive, natural killer (NK) cell-positive phenotype. The disorder is lethal in early childhood without bone marrow transplantation (summary by Yu et al., 2011).
Severe combined immunodeficiency due to CTPS1 deficiency
MedGen UID:
863054
Concept ID:
C4014617
Disease or Syndrome
IMD24 is an autosomal recessive immunodeficiency characterized by the impaired capacity of activated T and B cells to proliferate in response to antigen receptor-mediated activation. Patients have early onset of severe chronic viral infections, mostly caused by herpesviruses, including Epstein-Barr virus (EBV) and varicella zoster virus (VZV); they also suffer from recurrent encapsulated bacterial infections, a spectrum typical of a combined deficiency of adaptive immunity (CID) (summary by Martin et al., 2014).
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
MedGen UID:
863730
Concept ID:
C4015293
Disease or Syndrome
IMD38 predisposes individuals to severe clinical disease upon infection with weakly virulent mycobacteria, including Mycobacterium bovis Bacille Calmette-Guerin (BCG) vaccines (Bogunovic et al., 2012). Patients do not experience severe disease in response to viral infection. Affected individuals have intracranial calcification (Zhang et al., 2015).
Primary immunodeficiency with post-measles-mumps-rubella vaccine viral infection
MedGen UID:
904009
Concept ID:
C4225260
Disease or Syndrome
Immunodeficiency-44 (IMD44) is an autosomal recessive primary immunodeficiency characterized by increased susceptibility to viral infections and adverse multisystemic reaction to vaccination in some patients. Affected individuals appear to have defects in mitochondrial fission and fusion (summary by Shahni et al., 2015).
Combined immunodeficiency due to DOCK8 deficiency
MedGen UID:
1648410
Concept ID:
C4722305
Disease or Syndrome
Hyper-IgE syndrome-2 with recurrent infections (HIES2) is an autosomal recessive immunologic disorder characterized by recurrent staphylococcal infections of the skin and respiratory tract, eczema, elevated serum immunoglobulin E, and hypereosinophilia. It is distinguished from autosomal dominant HIES1 (147060) by the lack of connective tissue and skeletal involvement (Renner et al., 2004). For a discussion of genetic heterogeneity of hyper-IgE syndrome, see 147060. See also TYK2 deficiency (611521), a clinically distinct disease entity that includes characteristic features of both autosomal recessive HIES2 and mendelian susceptibility to mycobacterial disease (MSMD; 209950) (Minegishi et al., 2006).
Immunodeficiency 74, COVID-19-related, X-linked
MedGen UID:
1768360
Concept ID:
C5435745
Disease or Syndrome
Immunodeficiency-74 (IMD74) is an X-linked recessive specific immunologic disorder characterized by the development of severe respiratory insufficiency in response to infection with the COVID-19 coronavirus, also known as SARS-CoV-2 ssRNA coronavirus. Affected individuals usually require mechanical ventilation in the ICU in order to survive. Laboratory studies show activation of the immune response and may show perturbation of some values, such as increased D-dimers and fibrinogen. In vitro functional studies of patient immune cells show impaired signaling through the TLR7 pathway, resulting in defective type I and type II interferon (IFN) responses. The patients reported to date did not have a history of immunodeficiency or chronic disease (summary by van Der Made et al., 2020).
Immunodeficiency 91 and hyperinflammation
MedGen UID:
1794283
Concept ID:
C5562073
Disease or Syndrome
Immunodeficiency-91 and hyperinflammation (IMD91) is an autosomal recessive complex immunologic disorder characterized by both immunodeficiency and recurrent infections, often to viruses or mycobacteria, as well as by hyperinflammation with systemic involvement. Affected individuals present in infancy with variable features, including fever, infection, thrombocytopenia, renal or hepatic dysfunction, recurrent infections, or seizures. Most patients eventually develop hepatic or renal failure, compromised neurologic function, lymphadenopathy or hepatosplenomegaly, and multiorgan failure resulting in death. More variable features may include intermittent monocytosis, features of hemophagocytic lymphohistiocytosis (HLH), and serologic evidence of hyperinflammation. The disorder is thought to result from dysregulation of the interferon response to viral stimulation in the innate immune system (summary by Le Voyer et al., 2021; Vavassori et al., 2021).

Professional guidelines

PubMed

Delavari S, Abolhassani H, Abolnezhadian F, Babaha F, Iranparast S, Ahanchian H, Moazzen N, Nabavi M, Arshi S, Fallahpour M, Bemanian MH, Shokri S, Momen T, Sadeghi-Shabestari M, Molatefi R, Shirkani A, Vosughimotlagh A, Safarirad M, Sharifzadeh M, Pashangzadeh S, Salami F, Shirmast P, Rezaei A, Moeini Shad T, Mohraz M, Rezaei N, Hammarström L, Yazdani R, Aghamohamamdi A
J Clin Immunol 2021 Feb;41(2):345-355. Epub 2020 Dec 1 doi: 10.1007/s10875-020-00928-x. PMID: 33263173Free PMC Article
Oh CS, Stratta RJ, Fox BC, Sollinger HW, Belzer FO, Maki DG
Transplantation 1988 Jan;45(1):68-73. doi: 10.1097/00007890-198801000-00016. PMID: 3276066

Recent clinical studies

Etiology

Marr KA, Platt A, Tornheim JA, Zhang SX, Datta K, Cardozo C, Garcia-Vidal C
Emerg Infect Dis 2021 Jan;27(1):18-25. Epub 2020 Oct 21 doi: 10.3201/eid2701.202896. PMID: 33084566Free PMC Article
Gautam S, Cohen AJ, Stahl Y, Valda Toro P, Young GM, Datta R, Yan X, Ristic NT, Bermejo SD, Sharma L, Restrepo MI, Dela Cruz CS
Thorax 2020 Nov;75(11):974-981. Epub 2020 Aug 21 doi: 10.1136/thoraxjnl-2020-214896. PMID: 32826284
Finelli C, Parisi S
Adv Biol Regul 2020 Aug;77:100742. Epub 2020 Jul 16 doi: 10.1016/j.jbior.2020.100742. PMID: 32773103Free PMC Article
Perricone C, Triggianese P, Bartoloni E, Cafaro G, Bonifacio AF, Bursi R, Perricone R, Gerli R
J Autoimmun 2020 Jul;111:102468. Epub 2020 Apr 17 doi: 10.1016/j.jaut.2020.102468. PMID: 32317220Free PMC Article
Olijve L, Jennings L, Walls T
Clin Microbiol Rev 2018 Jan;31(1) Epub 2017 Nov 15 doi: 10.1128/CMR.00047-17. PMID: 29142080Free PMC Article

Diagnosis

Sabulski A, Nehus EJ, Jodele S, Ricci K
J Pediatr Hematol Oncol 2022 Jan 1;44(1):e237-e240. doi: 10.1097/MPH.0000000000002036. PMID: 33369997
Marr KA, Platt A, Tornheim JA, Zhang SX, Datta K, Cardozo C, Garcia-Vidal C
Emerg Infect Dis 2021 Jan;27(1):18-25. Epub 2020 Oct 21 doi: 10.3201/eid2701.202896. PMID: 33084566Free PMC Article
Walker C, Deb S, Ling H, Wang Z
J Pharm Pharm Sci 2020;23:396-405. doi: 10.18433/jpps31501. PMID: 33086028
Olijve L, Jennings L, Walls T
Clin Microbiol Rev 2018 Jan;31(1) Epub 2017 Nov 15 doi: 10.1128/CMR.00047-17. PMID: 29142080Free PMC Article
Fischer GB, Sarria EE, Mattiello R, Mocelin HT, Castro-Rodriguez JA
Paediatr Respir Rev 2010 Dec;11(4):233-9. Epub 2010 Aug 19 doi: 10.1016/j.prrv.2010.07.005. PMID: 21109182

Therapy

Feng E, Balint E, Poznanski SM, Ashkar AA, Loeb M
Cells 2021 Mar 23;10(3) doi: 10.3390/cells10030708. PMID: 33806810Free PMC Article
Finelli C, Parisi S
Adv Biol Regul 2020 Aug;77:100742. Epub 2020 Jul 16 doi: 10.1016/j.jbior.2020.100742. PMID: 32773103Free PMC Article
Perricone C, Triggianese P, Bartoloni E, Cafaro G, Bonifacio AF, Bursi R, Perricone R, Gerli R
J Autoimmun 2020 Jul;111:102468. Epub 2020 Apr 17 doi: 10.1016/j.jaut.2020.102468. PMID: 32317220Free PMC Article
Mantero M, Rogliani P, Cazzola M, Blasi F, Di Pasquale M
Expert Opin Emerg Drugs 2018 Sep;23(3):185-199. Epub 2018 Jul 30 doi: 10.1080/14728214.2018.1504020. PMID: 30032674
Henter JI, Chow CB, Leung CW, Lau YL
Lancet 2006 Mar 11;367(9513):870-3. doi: 10.1016/S0140-6736(06)68232-9. PMID: 16530581

Prognosis

Kellnar A, Hoppe JM, Brunner S, Stremmel C
Clin Nutr ESPEN 2021 Oct;45:499-502. Epub 2021 Aug 28 doi: 10.1016/j.clnesp.2021.07.033. PMID: 34620361Free PMC Article
Delavari S, Abolhassani H, Abolnezhadian F, Babaha F, Iranparast S, Ahanchian H, Moazzen N, Nabavi M, Arshi S, Fallahpour M, Bemanian MH, Shokri S, Momen T, Sadeghi-Shabestari M, Molatefi R, Shirkani A, Vosughimotlagh A, Safarirad M, Sharifzadeh M, Pashangzadeh S, Salami F, Shirmast P, Rezaei A, Moeini Shad T, Mohraz M, Rezaei N, Hammarström L, Yazdani R, Aghamohamamdi A
J Clin Immunol 2021 Feb;41(2):345-355. Epub 2020 Dec 1 doi: 10.1007/s10875-020-00928-x. PMID: 33263173Free PMC Article
Finelli C, Parisi S
Adv Biol Regul 2020 Aug;77:100742. Epub 2020 Jul 16 doi: 10.1016/j.jbior.2020.100742. PMID: 32773103Free PMC Article
Arvin AM, Fink K, Schmid MA, Cathcart A, Spreafico R, Havenar-Daughton C, Lanzavecchia A, Corti D, Virgin HW
Nature 2020 Aug;584(7821):353-363. Epub 2020 Jul 13 doi: 10.1038/s41586-020-2538-8. PMID: 32659783
Fischer GB, Sarria EE, Mattiello R, Mocelin HT, Castro-Rodriguez JA
Paediatr Respir Rev 2010 Dec;11(4):233-9. Epub 2010 Aug 19 doi: 10.1016/j.prrv.2010.07.005. PMID: 21109182

Clinical prediction guides

Kellnar A, Hoppe JM, Brunner S, Stremmel C
Clin Nutr ESPEN 2021 Oct;45:499-502. Epub 2021 Aug 28 doi: 10.1016/j.clnesp.2021.07.033. PMID: 34620361Free PMC Article
Arvin AM, Fink K, Schmid MA, Cathcart A, Spreafico R, Havenar-Daughton C, Lanzavecchia A, Corti D, Virgin HW
Nature 2020 Aug;584(7821):353-363. Epub 2020 Jul 13 doi: 10.1038/s41586-020-2538-8. PMID: 32659783
Perricone C, Triggianese P, Bartoloni E, Cafaro G, Bonifacio AF, Bursi R, Perricone R, Gerli R
J Autoimmun 2020 Jul;111:102468. Epub 2020 Apr 17 doi: 10.1016/j.jaut.2020.102468. PMID: 32317220Free PMC Article
Safain MG, Roguski M, Kryzanski JT, Weller SJ
Clin Neurol Neurosurg 2015 Jan;128:10-6. Epub 2014 Nov 4 doi: 10.1016/j.clineuro.2014.10.015. PMID: 25462089
Cheuk DK, Chiang AK, Lee TL, Chan GC, Ha SY
Cochrane Database Syst Rev 2011 Mar 16;(3):CD006505. doi: 10.1002/14651858.CD006505.pub2. PMID: 21412895

Recent systematic reviews

Walker C, Deb S, Ling H, Wang Z
J Pharm Pharm Sci 2020;23:396-405. doi: 10.18433/jpps31501. PMID: 33086028
Cheuk DK, Chiang AK, Lee TL, Chan GC, Ha SY
Cochrane Database Syst Rev 2011 Mar 16;(3):CD006505. doi: 10.1002/14651858.CD006505.pub2. PMID: 21412895

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