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Secretory piece deficiency

MedGen UID:
140769
Concept ID:
C0398709
Disease or Syndrome
Synonyms: SECRETORY COMPONENT DEFICIENCY; Secretory IgA deficiency
SNOMED CT: Secretory piece deficiency (234554004); Secretory component deficiency (234554004)
 
HPO: HP:0004433
Monarch Initiative: MONDO:0010019
OMIM®: 269650

Definition

Deficiency of secretory IgA (polymers of 2-4 IgA monomers are linked by two additional chains) and is the primary antibody response at the mucosal level, where it forms immune complexes with pathogens and allergens. [from HPO]

Clinical features

From HPO
Intermittent diarrhea
MedGen UID:
66782
Concept ID:
C0239181
Sign or Symptom
Repeated episodes of diarrhea separated by periods without diarrhea.
Secretory piece deficiency
MedGen UID:
140769
Concept ID:
C0398709
Disease or Syndrome
Deficiency of secretory IgA (polymers of 2-4 IgA monomers are linked by two additional chains) and is the primary antibody response at the mucosal level, where it forms immune complexes with pathogens and allergens.
Chronic intestinal candidiasis
MedGen UID:
892549
Concept ID:
C4025199
Disease or Syndrome
Persistent overgrowth of Candida albicans in the gastrointestinal tract.

Conditions with this feature

Secretory piece deficiency
MedGen UID:
140769
Concept ID:
C0398709
Disease or Syndrome
Deficiency of secretory IgA (polymers of 2-4 IgA monomers are linked by two additional chains) and is the primary antibody response at the mucosal level, where it forms immune complexes with pathogens and allergens.
Deletion of long arm of chromosome 18
MedGen UID:
96605
Concept ID:
C0432443
Disease or Syndrome
Monosomy 18q is a partial deletion of the long arm of chromosome 18 characterized by highly variable phenotype, most commonly including hypotonia, developmental delay, short stature, growth hormone deficiency, hearing loss and external ear anomalies, intellectual disability, palatal defects, dysmorphic facial features, skeletal anomalies (foot deformities, tapering fingers, scoliosis) and mood disorders.

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