Table V.

Warning signs of PIDD for gastroenterologists

Clinical occurrences	PIDD	Laboratory tests
Chronic diarrhea	Antibody deficiencies	AMI
Inflammatory bowel disease	Combined immunodeficiencies (infants)	CMI, AMI
Chronic giardiasis
Autoimmune enteropathy + severe intractable diarrhea. Other diagnoses associated: hypothyroidism, eczema, thrombocytopenia, autoimmune hemolytic anemia, neonatal diabetes	Immunodysregulation, polyendocrinopathy and enteropathy, X-linked (IPEX)	CMI, Coombs, glycemia, and TSH
		ANA
Persistent candidiasis	Combined immunodeficiencies	CMI
		T cell Lymphoproliferative assay
	Chronic mucocutaneous candidiasis	Lymphoproliferation to Candida
	Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED)	Candidin test
		ANA and endocrine evaluation
Severe abdominal pain emulating an acute abdomen	Hereditary angioedema	Dosage and/or functional activity assay of C1INH, C4, C1q
Liver abscess mainly due to S aureus	Chronic granulomatous disease (CGD)	P
	Hyper IgE syndrome (HIES)	Serum IgE, eosinophilia
		Specific Scorea
Hepatobiliary infection due to C parvum	CD40 ligand (L) deficiency	AMI
Inflammatory bowel disease in infants	CGD	P
	Interleukin-10 (IL-10) or interleukin 10 receptor (IL-10R) deficiencies	II

Abbreviations: AMI antibody mediated immunity, ANA antinuclear antibodies, C1INH C1 esterase inhibitor, CMI cellular mediated immunity, CBCcomplete blood count, II innate immunity, P phagocytosis, TSH thyroid stimulating hormone

^aScore for classical Hyper IgE diagnosis [26]