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Hypoproteinemia, hypercatabolic(IMD43)

MedGen UID:
343422
Concept ID:
C1855796
Disease or Syndrome
Synonyms: B2M DEFICIENCY; BETA-2-MICROGLOBULIN DEFICIENCY; IMD43; IMMUNODEFICIENCY 43; MHC CLASS I DEFICIENCY 4
 
Gene (location): B2M (15q21.1)
 
Monarch Initiative: MONDO:0009434
OMIM®: 241600

Definition

Immunodeficiency-43 (IMD43) is an autosomal recessive immunologic disorder characterized by decreased or absent expression of MHC class I molecules on the cell surface. Most affected individuals develop recurrent bacterial respiratory tract infections in childhood or adulthood, which may progress to bronchiectasis, and about half develop ulcerating or necrotizing granulomatous inflammatory skin lesions. Laboratory studies show decreased numbers of B cells, hypogammaglobulinemia, hypoproteinemia, and decreased alpha-beta CD8+ T cells with increased gamma-delta CD8+ T cells. The severity is variable, and some individuals may be asymptomatic (summary by Ardeniz et al., 2015). For a discussion of genetic heterogeneity of MHC class I deficiency, see MHC1D1 (604571). [from OMIM]

Clinical features

From HPO
Radial bowing
MedGen UID:
347136
Concept ID:
C1859399
Anatomical Abnormality
A bending or abnormal curvature of the radius.
Hypoplasia of the ulna
MedGen UID:
395934
Concept ID:
C1860614
Congenital Abnormality
Underdevelopment of the ulna.
Bronchiectasis
MedGen UID:
14234
Concept ID:
C0006267
Disease or Syndrome
Persistent abnormal dilatation of the bronchi owing to localized and irreversible destruction and widening of the large airways.
Organizing pneumonia
MedGen UID:
538531
Concept ID:
C0264383
Disease or Syndrome
Organizing pneumonia manifests as a histologic pattern characterized by loose plugs of connective tissue in the airspaces and distal airways. Interstitial inflammation and fibrosis are minimal or absent. Cryptogenic organizing pneumonia, or COP, is a distinctive clinical disorder among the idiopathic interstitial pneumonias, but the histologic pattern of organizing pneumonia is encountered in many different situations, including pulmonary infection, hypersensitivity pneumonitis, and collagen vascular diseases. Airspace consolidation is the cardinal feature of organizing pneumonia on chest radiographs and CT scans. In COP, the distribution is typically subpleural and basal and sometimes bronchocentric. Other manifestations of organizing pneumonia include groundglass opacity, tree-in-bud pattern, and nodular opacities.
Recurrent respiratory infections
MedGen UID:
812812
Concept ID:
C3806482
Finding
An increased susceptibility to respiratory infections as manifested by a history of recurrent respiratory infections.
Lung abscess
MedGen UID:
7397
Concept ID:
C0024110
Disease or Syndrome
A circumscribed area of pus or necrotic debris in lung parenchyma, which leads to a cavity, and after formation of bronchopulmonary fistula, can manifest as an air-fluid level inside the cavity.
B lymphocytopenia
MedGen UID:
340780
Concept ID:
C1855067
Finding
An abnormal decrease from the normal count of B cells.
Reduced natural killer cell count
MedGen UID:
383765
Concept ID:
C1855767
Finding
Less than normal number of natural killer cells, a type of lymphocyte in the innate immune system with an ability to mediate cytotoxicity and produce cytokines after the ligation of a germline-encoded activation receptor.
Abnormal circulating IgA concentration
MedGen UID:
1706440
Concept ID:
C5139420
Finding
An abnormal deviation from normal levels of IgA immunoglobulin in blood.
Abnormal circulating IgM concentration
MedGen UID:
1688699
Concept ID:
C5139423
Finding
An abnormal deviation from normal levels of IgM immunoglobulin in blood.
Decreased specific antibody response to polysaccharide vaccine
MedGen UID:
1708273
Concept ID:
C5139462
Finding
A reduced ability to synthesize postvaccination antibodies against polysaccharides in vaccines, as measured by antibody titer determination following vaccination.
Decreased circulating IgG concentration
MedGen UID:
1720114
Concept ID:
C5234937
Finding
An abnormally decreased level of immunoglobulin G (IgG) in blood.
Hypoalbuminemia
MedGen UID:
68694
Concept ID:
C0239981
Finding
Reduction in the concentration of albumin in the blood.
Hypoproteinemia
MedGen UID:
581229
Concept ID:
C0392692
Finding
A decreased concentration of protein in the blood.
Decreased circulating beta-2-microglobulin level
MedGen UID:
1371004
Concept ID:
C4476717
Finding
Reduced concentration of beta-2-microglobulin in the blood.
Subcutaneous nodule
MedGen UID:
101803
Concept ID:
C0151811
Pathologic Function
Slightly elevated lesions on or in the skin with a diameter of over 5 mm.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

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