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Primary immunodeficiency syndrome due to p14 deficiency

MedGen UID:
372135
Concept ID:
C1835829
Disease or Syndrome
Synonym: Immunodeficiency due to defect in mapbp-interacting protein
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Gene (location): LAMTOR2 (1q22)
 
Monarch Initiative: MONDO:0012559
OMIM®: 610798
Orphanet: ORPHA90023

Definition

Primary immunodeficiency syndrome due to p14 deficiency is characterised by short stature, hypopigmentation, coarse facies and frequent bronchopulmonary <i>Streptococcus pneumoniae</i> infections. [from ORDO]

Clinical features

From HPO
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Recurrent bronchopulmonary infections
MedGen UID:
488990
Concept ID:
C2169795
Finding
An increased susceptibility to bronchopulmonary infections as manifested by a history of recurrent bronchopulmonary infections.
Immunodeficiency
MedGen UID:
7034
Concept ID:
C0021051
Disease or Syndrome
Failure of the immune system to protect the body adequately from infection, due to the absence or insufficiency of some component process or substance.
Decreased circulating total IgM
MedGen UID:
116095
Concept ID:
C0239989
Finding
An abnormally decreased level of immunoglobulin M (IgM) in blood.
Neutropenia
MedGen UID:
163121
Concept ID:
C0853697
Finding
An abnormally low number of neutrophils in the peripheral blood.
Coarse facial features
MedGen UID:
335284
Concept ID:
C1845847
Finding
Absence of fine and sharp appearance of brows, nose, lips, mouth, and chin, usually because of rounded and heavy features or thickened skin with or without thickening of subcutaneous and bony tissues.
Hypopigmentation of the skin
MedGen UID:
102477
Concept ID:
C0162835
Disease or Syndrome
A reduction of skin color related to a decrease in melanin production and deposition.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary immunodeficiency syndrome due to p14 deficiency
Follow this link to review classifications for Primary immunodeficiency syndrome due to p14 deficiency in Orphanet.

Professional guidelines

PubMed

Zamani R, Shahkarami S, Rezaei N
Allergol Immunopathol (Madr) 2021;49(2):178-190. Epub 2021 Mar 1 doi: 10.15586/aei.v49i2.61. PMID: 33641308

Recent clinical studies

Etiology

Tajik S, Fazlollahi MR, Alizadeh Z, Badalzadeh M, Houshmand M, Razaghian A, Bahram S, Molitor A, Carapito R, Shariat M, Hamidieh AA, Behniafard N, Abdolkarimi B, Rostami T, Moin M, Pourpak Z
Pediatr Allergy Immunol 2024 Nov;35(11):e14264. doi: 10.1111/pai.14264. PMID: 39485047
Ünsal H, Caka C, Bildik HN, Esenboğa S, Kupesiz A, Kuşkonmaz B, Cetinkaya DU, van der Burg M, Tezcan İ, Çağdaş D
Scand J Immunol 2024 Jan;99(1):e13335. Epub 2023 Oct 15 doi: 10.1111/sji.13335. PMID: 38441205
Zamani R, Shahkarami S, Rezaei N
Allergol Immunopathol (Madr) 2021;49(2):178-190. Epub 2021 Mar 1 doi: 10.15586/aei.v49i2.61. PMID: 33641308
Soyak Aytekin E, Çağdaş D, Tan C, Tezcan İ
Eur Ann Allergy Clin Immunol 2021 Mar;53(2):75-79. Epub 2021 Mar 1 doi: 10.23822/EurAnnACI.1764-1489.146. PMID: 32351099
Rezaei N, Moazzami K, Aghamohammadi A, Klein C
Int Rev Immunol 2009;28(5):335-66. doi: 10.1080/08830180902995645. PMID: 19811314

Diagnosis

Tajik S, Fazlollahi MR, Alizadeh Z, Badalzadeh M, Houshmand M, Razaghian A, Bahram S, Molitor A, Carapito R, Shariat M, Hamidieh AA, Behniafard N, Abdolkarimi B, Rostami T, Moin M, Pourpak Z
Pediatr Allergy Immunol 2024 Nov;35(11):e14264. doi: 10.1111/pai.14264. PMID: 39485047
Ünsal H, Caka C, Bildik HN, Esenboğa S, Kupesiz A, Kuşkonmaz B, Cetinkaya DU, van der Burg M, Tezcan İ, Çağdaş D
Scand J Immunol 2024 Jan;99(1):e13335. Epub 2023 Oct 15 doi: 10.1111/sji.13335. PMID: 38441205
Zamani R, Shahkarami S, Rezaei N
Allergol Immunopathol (Madr) 2021;49(2):178-190. Epub 2021 Mar 1 doi: 10.15586/aei.v49i2.61. PMID: 33641308
Soyak Aytekin E, Çağdaş D, Tan C, Tezcan İ
Eur Ann Allergy Clin Immunol 2021 Mar;53(2):75-79. Epub 2021 Mar 1 doi: 10.23822/EurAnnACI.1764-1489.146. PMID: 32351099

Clinical prediction guides

Tajik S, Fazlollahi MR, Alizadeh Z, Badalzadeh M, Houshmand M, Razaghian A, Bahram S, Molitor A, Carapito R, Shariat M, Hamidieh AA, Behniafard N, Abdolkarimi B, Rostami T, Moin M, Pourpak Z
Pediatr Allergy Immunol 2024 Nov;35(11):e14264. doi: 10.1111/pai.14264. PMID: 39485047

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