Table 2.

Clinical and serological profiles of TV NR.

Patients	#1	#2	#3	#4	#5	#6	#7
Gender	F	F	F	F	M	M	M
Age	3	16	6	4	10	9	14
Diagnosis/follow up	IgM deficiency	Bechet's disease	Primary antibody deficiency	Partial IgA deficiency	Common Variable Immunodeficieny	Selective IgA deficiency	Familial Cold Autoinflamamatory síndrome.
Notable clinical features	Recurrent pneumonias CT scan: atelectasis and bronchiectasis	Neutropenia, recurrent respiratory and urinary tract infection, vaginal candidiasis infection	Recurrent bronchitis, bronchiectasis, laryngomalacia, bronchomalacia.	Recurrent respiratory infections, atopic dermatitis and moderate bronchial asthma	Chronic diarrhea, recurrent ear infections. Intracranial hypertension.	Recurrent higher and lower respiratory tract infections (Sinusitis, ear infections, and pneumonia). Bronchiectasis.	Recurrent episodes of fever and systemic inflammation cold-related, higher, and lower respiratory tract infections (Sinusitis, pharyngitis, ear infections, and pneumonia), recurrent bronchiolitis. Bronchiectasis.
TV FI	1	1	0.3	1	1	2.1	1.7
PPV FI	14	0.9	1.2	0.4	2.6	8.4	7
IgG* (g/L)	6.66	10.8227	10.18	11.44	3.10	16.20	7.87
IgG1* (g/L)	4.76	4.34	6.53	7.43	0.34	7.75	4.65
IgG2*(g/L)	0.96	4.74	1.59	2.49	0.03	5.63	2.27
IgA*(g/L)	0.54	2.12	0.93	0.009	<0.002	<0.002	1.06
IgM*(g/L)	0.004	1.84	1.23	1.72	<0.09	0.07	1.1

^*(NV. Parameters were established according to age range).

NA, data not available.

All diagnoses were made according the ESID diagnostic criteria.