Pediatric Selective IgM Immunodeficiency

Table 1

Characteristics of previously reported pediatric cases of SIgMID. WNL: within normal limits, GN: glomerulonephritis, CMV: cytomegalovirus, AD: atopic dermatitis, N/A: not available, OM: otitis media, URI: upper respiratory infection.

Reference	Age of diagnosis (year)/sex	Presenting history	Serum IgM mg/dL	Serum IgG mg/dL	Serum IgA mg/dL	IgE IU/mL	Comment
[19]	9 male	Pyoderma, AD, diarrhea, cutaneous candidiasis, rudimentary auricles	1/4 of normal controls	WNL	WNL	N/A	⊕ Isohemagglutinin; Lack of IgM response to S. typhi; “O” antigen
[9]	4.5 mo female	Autoimmune anemia, GN, failure to thrive	13	2500	390	N/A	Died of PCP; diffuse lymphoid hypoplasia, thymus dysplasia
[6]	8 male	Meningococcal sepsis, Waterhouse- Friderichsen syndrome	4	560	70		Died from infection
[6]	5 male	Meningococcal meningitis	12	1120	190		Died from infection
[20]	3	Celiac disease	33	N/A	N/A	N/A	IgM level normalized after gluten free diet
[20]	8	Celiac disease	30	N/A	N/A	N/A	IgM level normalized after gluten free diet
[20]	10	Celiac disease	35	N/A	N/A	N/A	IgM level normalized after gluten free diet
[20]	13	Celiac disease	40	N/A	N/A	N/A	IgM level normalized after gluten free diet
[20]	2	Celiac disease	20	N/A	N/A	N/A	IgM level normalized after gluten free diet
[21]	5 male	Asymptomatic	35	820	224	N/A	Familial cases
[2]	3.75 male	N/A	7	495	172	N/A	Partial deletion of long arm of chromosome 18
[12]	6 male	Recurrent pneumonia, OM, AD, sinusitis	6	1250	950		Received IM gammaglobulin treatment, salmonella enteritis, pneumococcal sepsis
[16]	8.5 mo male	Pseudomonas pneumonia, recurrent OM	0	WNL	Mildly reduced		Died from infection
[18]	13 male	CMV hepatitis	26–29	N/A	N/A	N/A
[14]	4	Meningitis	34	WNL	WNL	N/A	Low IgM hemagglutinin to meningococcus
[14]	1	Asymptomatic	36	WNL	WNL	N/A	Low IgM hemagglutinin to meningococcus
[22]	2.5 female	Recurrent OM, laryngitis, meningitis	8	530	48	N/A	Absent isohemagglutinin, chromosome #1 defect
[1]	3 patients, female <17	N/A	<23	WNL	WNL	N/A
[1]	7 patients, male <16	N/A	<23	WNL	WNL	N/A
[23]	12 female	Recurrent bronchitis, OM, pneumonia, bronchiectasis	34	686	87	N/A	No response to salmonella “H” antigen
[24]	Young male	N/A	8	1500	72	555
[10]	3 female	erythrodermic psoriasis	<4	680	125	340	OM, diarrhea, failure to thrive, recurrent respiratory infection, eosinophilia
[10]	9 male	AD	<4	900	153	8000	Failure to thrive, recurrent respiratory tract infection and pneumonia, skin infections, giardiasis, eosinophilia
[17]	16 female	Disseminated molluscum contagiosum	<4	2305	688	8900	AD, aphthous stomatitis, varicella, recurrent respiratory infections, eosinophilia, bronchiectasis
[25]	2.5 female	Failure to thrive, recurrent URI	37	869	45	N/A
[25]	5.5 female	Failure to thrive, recurrent OM, eyelid and GI infections	38	1064	42	N/A
[25]	8 male	Failure to thrive, recurrent OM, eyelid and GI infections	42	488	100	N/A
[26]	1 mo	Pneumonia	<6	<350	N/A	N/A	Nonspecific IgG subclass deficiency
[26]	11 mo	Bronchitis, OM	<4	<350	N/A	N/A
[26]	3	Bronchitis, OM	5	780	N/A	N/A
[26]	2	OM	11	~2200	N/A	N/A
[13]	1 mo	Meningitis, pneumonia, OM	6	717	27	<10	Allergic rhinitis
[13]	3	Bronchitis, OM	5	768	108	<10	Allergic rhinitis
[13]	2	OM	11	608	33	12	Allergic rhinitis
[13]	11 mo	OM, pneumonia	5	462	25	<10
[13]	11 mo	Bronchitis, OM	5	345	88	<10	Wheezing
[13]	8	OM, pneumonia	11	925	134	297	Allergic rhinitis, wheezing
[27]	10 male	Recurrent sinusitis, OM, pneumonia, chronic staphylococci blepharitis	23	900	86	N/A
[28]	15 female	Chronic OM	<6	1170	356	N/A	22q11.2 chromosome deletion
[3]	11 male	Asthma	<5	NL	NL	N/A	Father with SIgMID
[29]	17 male	Russell-Silver Syndrome with recurrent URI	1	NL	NL	N/A	No response to pneumococcal vaccine
[30]	1.5 male	Recurrent OM	28	N/A	N/A	N/A
[31]	7	Recurrent OM, pneumococcal pneumonia with emphysema	Deficient	NL	NL	Elevated	Lack of protective antibody response to polysaccharide and protein antigen, responded to IVIg
[31]	18 month	Asthma, recurrent OM, pseudomonal pneumonia, sepsis	Deficient	NL	NL	Elevated
Mean	6±4.7		16.5±13.8	939±580	183±226	1814±3509