Table 2.

Meta-analysis of studies in Shwachman–Diamond syndrome (SDS) patients who fulfil criteria for common variable immunodeficiency (CVID) and other immunological abnormalities.

Disease category	Age/sex	Recurrent infections	IgG levels (g/l)	IgM levels (g/l)	IgA levels (g/l)	Vaccine responses	CD19 cells (%)	CD4/ CD8 ratio	CD16/ CD56 cells (%)	Neutrophil chemotaxis	Reference
‘Possible’ CVID	7/M	Bacterial + viral	7·0*	0·3	0·8	Absent/††	24	2·2‡	1·0§	Abnormal	[4]
	18/M	Bacterial + viral	4·8§	0·3	0·7	Normal/††	1·2§	0·5§	1·8§	–	[4]
	41/F	Bacterial	5·8§ (6·3–12·9 g/l)	–	–	Absent (pneumococcal)	–	Normal	–	–	[5]
	59/M	Bacterial	1·3§	< 0·2§	< 0·2§	–	1·10§	1·4§	1·43§	–	Index case
Hypogamma- globulinaemia of other types	4 month/F 1·5/M	Bacterial Bacterial	3·8§ (6·0–15·75 g/l) 6·8	0·40§ (0·7–2·3 g/l) <0·1§	0·07§ (0·2–1·4 g/l) 0·3	Absent –	CD20–6 (10–30%) –	2·0 –	3 –	Abnormal –	[6] [7]
	2/M	Bacterial + viral	6·1§,¶	0·3	0·2§	–	–	–	–	–	[4]
	–	Bacterial	–	Low	–	–	Normal	Normal	–	Abnormal	[8]
	–	Bacterial	–	–	Low	–	Normal	Low	–	Abnormal	[8]
	–	Bacterial	–	–	Low	–	Normal	Normal	–	Abnormal	[8]
	–	Bacterial	–	Low	–	–	Normal	Normal	–	Abnormal	[8]
	16/M	Bacterial	0·36§	0·03§	0·12§	–	–	–	–	–	[9]
	3/M	Nil	4·8§	0·07§	Absent§	–	–	–	–	–	[10]
	6·5/F	Nil	3·8§	0·78	0·18§	–	–	–	–	–	[10]
Other immunological abnormalities	8/M	Bacterial + viral	9·8	0·3	0·7	–/††	9·3	2·3	1·5§	Abnormal	[4]
	17/M	Bacterial	8·2	0·4	11·1	–	–	–‡	–	Abnormal	[4]
	14/F	Bacterial	16·5	1·3	2·7	Normal/††	15	1·0§	3·5§	–	[4]

^*Low IgG1 levels;

^†Low anti-B isohaemagglutinin levels;

^‡low lymphocyte proliferation to concanavalin A/pokeweed mitogen;

^§low for age;

^¶low IgG1 and IgG3 levels; –, not studied/available.