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Recurrent meningitis

MedGen UID:
152879
Concept ID:
C0746495
Disease or Syndrome
Synonym: Meningitis, recurrent
 
HPO: HP:0006946

Definition

An increased susceptibility to meningitis as manifested by a medical history of recurrent episodes of meningitis. [from HPO]

Conditions with this feature

Wiskott-Aldrich syndrome
MedGen UID:
21921
Concept ID:
C0043194
Disease or Syndrome
The WAS-related disorders, which include Wiskott-Aldrich syndrome, X-linked thrombocytopenia (XLT), and X-linked congenital neutropenia (XLN), are a spectrum of disorders of hematopoietic cells, with predominant defects of platelets and lymphocytes caused by pathogenic variants in WAS. WAS-related disorders usually present in infancy. Affected males have thrombocytopenia with intermittent mucosal bleeding, bloody diarrhea, and intermittent or chronic petechiae and purpura; eczema; and recurrent bacterial and viral infections, particularly of the ear. At least 40% of those who survive the early complications develop one or more autoimmune conditions including hemolytic anemia, immune thrombocytopenic purpura, immune-mediated neutropenia, rheumatoid arthritis, vasculitis, and immune-mediated damage to the kidneys and liver. Individuals with a WAS-related disorder, particularly those who have been exposed to Epstein-Barr virus (EBV), are at increased risk of developing lymphomas, which often occur in unusual, extranodal locations including the brain, lung, or gastrointestinal tract. Males with XLT have thrombocytopenia with small platelets; other complications of Wiskott-Aldrich syndrome, including eczema and immune dysfunction, are usually mild or absent. Males with XLN have congenital neutropenia, myeloid dysplasia, and lymphoid cell abnormalities.
Pyogenic bacterial infections due to MyD88 deficiency
MedGen UID:
383023
Concept ID:
C2677092
Disease or Syndrome
Immunodeficiency-68 (IMD68) is an autosomal recessive primary immunodeficiency characterized by severe systemic and invasive bacterial infections beginning in infancy or early childhood. The most common organisms implicated are Streptococcus pneumoniae, Staphylococcus aureus, and Pseudomonas, although other organisms may be observed. IMD68 is life-threatening in infancy and early childhood. The first invasive infection typically occurs before 2 years of age, with meningitis and upper respiratory infections being common manifestations. The mortality rate in early childhood is high, with most deaths occurring before 8 years of age. Affected individuals have an impaired inflammatory response to infection, including lack of fever and neutropenia, although erythrocyte sedimentation rate (ESR) and C-reactive protein may be elevated. General immunologic workup tends to be normal, with normal levels of B cells, T cells, and NK cells. However, more detailed studies indicate impaired cytokine response to lipopolysaccharide (LPS) and IL1B (147720) stimulation; response to TNFA (191160) is usually normal. Patients have good antibody responses to most vaccinations. Viral, fungal, and parasitic infections are generally not observed. Early detection is critical in early childhood because prophylactic treatment with IVIg or certain antibiotics is effective; the disorder tends to improve naturally around adolescence. At the molecular level, IMD68 results from impaired function of selective Toll receptor (see TLR4, 603030)/IL1R (see IL1R1; 147810) signaling pathways that ultimately activate NFKB (164011) to produce cytokines (summary by Picard et al., 2010). See also IMD67 (607676), caused by mutation in the IRAK4 gene (602170), which shows a similar phenotype to IMD68. As the MYD88 and IRAK4 genes interact in the same intracellular signaling pathway, the clinical and cellular features are almost indistinguishable (summary by Picard et al., 2010).
Factor I deficiency
MedGen UID:
483045
Concept ID:
C3463916
Disease or Syndrome
C3 glomerulopathy (C3G) is a complex ultra-rare complement-mediated renal disease caused by uncontrolled activation of the complement alternative pathway (AP) in the fluid phase (as opposed to cell surface) that is rarely inherited in a simple mendelian fashion. C3G affects individuals of all ages, with a median age at diagnosis of 23 years. Individuals with C3G typically present with hematuria, proteinuria, hematuria and proteinuria, acute nephritic syndrome or nephrotic syndrome, and low levels of the complement component C3. Spontaneous remission of C3G is uncommon, and about half of affected individuals develop end-stage renal disease (ESRD) within ten years of diagnosis, occasionally developing the late comorbidity of impaired visual acuity.

Professional guidelines

PubMed

Cho TA, Venna N
Neurol Clin 2010 Nov;28(4):1061-88. doi: 10.1016/j.ncl.2010.03.023. PMID: 20816277
Roos KL
Semin Neurol 1998;18(2):185-96. doi: 10.1055/s-2008-1040872. PMID: 9608616
Hermans PE, Goldstein NP, Wellman WE
Am J Med 1972 Jan;52(1):128-40. doi: 10.1016/0002-9343(72)90014-9. PMID: 4111896

Recent clinical studies

Etiology

Snoek L, van Kassel MN, Koelman DLH, van der Ende A, van Sorge NM, Brouwer MC, van de Beek D, Bijlsma MW
BMJ Open 2023 Dec 30;13(12):e077887. doi: 10.1136/bmjopen-2023-077887. PMID: 38159962Free PMC Article
Ter Horst L, Brouwer MC, van der Ende A, van de Beek D
Clin Infect Dis 2021 Nov 2;73(9):e2545-e2551. doi: 10.1093/cid/ciaa1623. PMID: 33751028Free PMC Article
Masri A, Alassaf A, Khuri-Bulos N, Zaq I, Hadidy A, Bakri FG
Childs Nerv Syst 2018 Aug;34(8):1541-1547. Epub 2018 May 4 doi: 10.1007/s00381-018-3815-9. PMID: 29728757
Rosenberg J, Galen BT
Curr Pain Headache Rep 2017 Jul;21(7):33. doi: 10.1007/s11916-017-0635-7. PMID: 28551737
Onwubalili JK
J Infect 1983 Jul;7(1):2-20. doi: 10.1016/s0163-4453(83)90863-0. PMID: 6313809

Diagnosis

Masri A, Alassaf A, Khuri-Bulos N, Zaq I, Hadidy A, Bakri FG
Childs Nerv Syst 2018 Aug;34(8):1541-1547. Epub 2018 May 4 doi: 10.1007/s00381-018-3815-9. PMID: 29728757
Rosenberg J, Galen BT
Curr Pain Headache Rep 2017 Jul;21(7):33. doi: 10.1007/s11916-017-0635-7. PMID: 28551737
Cho TA, Venna N
Neurol Clin 2010 Nov;28(4):1061-88. doi: 10.1016/j.ncl.2010.03.023. PMID: 20816277
Ginsberg L, Kidd D
Pract Neurol 2008 Dec;8(6):348-61. doi: 10.1136/jnnp.2008.157396. PMID: 19015295
Coyle PK
Neurol Clin 1999 Nov;17(4):691-710. doi: 10.1016/s0733-8619(05)70162-6. PMID: 10517924Free PMC Article

Therapy

Masri A, Alassaf A, Khuri-Bulos N, Zaq I, Hadidy A, Bakri FG
Childs Nerv Syst 2018 Aug;34(8):1541-1547. Epub 2018 May 4 doi: 10.1007/s00381-018-3815-9. PMID: 29728757
Rosenberg J, Galen BT
Curr Pain Headache Rep 2017 Jul;21(7):33. doi: 10.1007/s11916-017-0635-7. PMID: 28551737
Gordon MF, Allon M, Coyle PK
Neurology 1990 Jan;40(1):163-4. doi: 10.1212/wnl.40.1.163. PMID: 2296366
Kushnick T
Clin Pediatr (Phila) 1972 May;11(5):308-9. doi: 10.1177/000992287201100518. PMID: 4555039
Lorber J
Proc R Soc Med 1969 Nov;62(11 Part 1):1093-4. PMID: 5356856Free PMC Article

Prognosis

Snoek L, van Kassel MN, Koelman DLH, van der Ende A, van Sorge NM, Brouwer MC, van de Beek D, Bijlsma MW
BMJ Open 2023 Dec 30;13(12):e077887. doi: 10.1136/bmjopen-2023-077887. PMID: 38159962Free PMC Article
Ter Horst L, Brouwer MC, van der Ende A, van de Beek D
Clin Infect Dis 2021 Nov 2;73(9):e2545-e2551. doi: 10.1093/cid/ciaa1623. PMID: 33751028Free PMC Article
Rosenberg J, Galen BT
Curr Pain Headache Rep 2017 Jul;21(7):33. doi: 10.1007/s11916-017-0635-7. PMID: 28551737
Coyle PK
Neurol Clin 1999 Nov;17(4):691-710. doi: 10.1016/s0733-8619(05)70162-6. PMID: 10517924Free PMC Article
Kavaliotis J, Tsiaousi A, Papavasiliou D, Kansouzidou A
Scand J Infect Dis 1994;26(4):403-5. doi: 10.3109/00365549409008612. PMID: 7984971

Clinical prediction guides

Snoek L, van Kassel MN, Koelman DLH, van der Ende A, van Sorge NM, Brouwer MC, van de Beek D, Bijlsma MW
BMJ Open 2023 Dec 30;13(12):e077887. doi: 10.1136/bmjopen-2023-077887. PMID: 38159962Free PMC Article
Ter Horst L, Brouwer MC, van der Ende A, van de Beek D
Clin Infect Dis 2021 Nov 2;73(9):e2545-e2551. doi: 10.1093/cid/ciaa1623. PMID: 33751028Free PMC Article
Chen B, Shi Y, Gong Y, Chen J, Li Y
Int J Pediatr Otorhinolaryngol 2019 Sep;124:147-151. Epub 2019 Jun 1 doi: 10.1016/j.ijporl.2019.05.045. PMID: 31195308
Wang J, Li Y, Chen S, Hao X
Int J Pediatr Otorhinolaryngol 2016 Aug;87:185-9. Epub 2016 May 20 doi: 10.1016/j.ijporl.2016.05.026. PMID: 27368469
Coyle PK
Neurol Clin 1999 Nov;17(4):691-710. doi: 10.1016/s0733-8619(05)70162-6. PMID: 10517924Free PMC Article

Recent systematic reviews

Singh R, Thorwarth RM, Bendok BR, Rath TJ, Bhuskute AA, Gnagi SH, Lal D
J Neurosurg Pediatr 2022 Apr 1;29(4):379-386. Epub 2022 Jan 14 doi: 10.3171/2021.11.PEDS21388. PMID: 35171832
Lovin BD, Appelbaum EN, Makoshi L, Whitehead WE, Sweeney AD
Ann Otol Rhinol Laryngol 2021 Dec;130(12):1360-1368. Epub 2021 Apr 9 doi: 10.1177/00034894211007242. PMID: 33834882

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