A wide spectrum of phenotype of deficiency of deaminase 2 (DADA2): a systematic literature review

Maccora, Ilaria; Maniscalco, Valerio; Campani, Silvia; Carrera, Simona; Abbati, Giulia; Marrani, Edoardo; Mastrolia, Maria Vincenza; Simonini, Gabriele

doi:10.1186/s13023-023-02721-6

Orphanet Journal of Rare Diseases

Table 2 Table of the identified correlations among the different clinical presentations

From: A wide spectrum of phenotype of deficiency of deaminase 2 (DADA2): a systematic literature review

	Haematological	Neurological	Fever	Skin	Immunological	Abdominal	Recurrent infections	Kidney	Failure to thrive	Splenomegaly	Hepatomegaly
Haematological	213	106	108	135 ρ. 282 p < .001	135 ρ. 417 p < .001	61 ρ. 142 p 0.008	66 ρ. 344 p < 0.001	37	28 ρ. 171 P 0.001	116 ρ. 557 P < 0.001	89 ρ. 465 P < .001
Neurological	106	193	126 ρ. 192 p < 0.001	165 ρ. 183 p < 0.001	80 ρ. 107 P0.045	99	22 ρ. 141 p0.009	51 ρ. 134 P0.013	19	58	52
Fever	108	126 ρ. 192 p < 0.001	194	167 ρ. 341 p < 0.001	86	87 ρ. 218 p < 0.001	37	56	31 ρ. 253 P < 0.001	68	59
Skin	135 ρ. 282 p < .001	167 ρ. 183 P < 0.001	167 ρ. 341 p < 0.001	257	109	88 ρ. 195 P < 0.001	40 ρ. 113 p 0.035	67 ρ. 144 P0.007	32 ρ. 125 p0.02	80 ρ. 110 P0.041	67
Immunological	135 ρ. 417 p < .001	82	75	92	160	41	58 ρ. 338 p < 0.001	32	16	73 ρ. 322 P < 0.001	46 ρ. 166 p.003
Abdominal	61 ρ. 142 p 0.008	87 ρ. 107 P0.045	87 ρ. 218 p < 0.001	88 ρ. 195 P < 0.001	49	113	15	42 P < 0.001	13	34 ρ. 127 P0.018	34
Recurrent infections	66 ρ. 344 P < 0.001	22 ρ. 141 p0.009	33	32 ρ. 113 p 0.035	58 ρ. 338 p < 0.001	14	70	11	14 ρ. 207 p < 0.001	42 ρ. 299 P < 0.001	27 ρ. 171 P < 0.001
Kidney	37	51 ρ. 134 P0.013	42	67 ρ. 144 P0.007	28	42 ρ. 229 P < 0.001	6	67	11 ρ. 137 p0.011	24	21
Failure to thrive	28 ρ. 171 p 0.001	19	31 ρ. 253 p < 0.001	32 ρ. 125 P0.02	16	13	14 ρ. 207 p < 0.001	11 ρ. 229 p0.011	36	15	10
Splenomegaly	116 ρ. 505 p < 0.001	53	57	80 ρ. 110 P0.041	73 ρ .322 P < 0.001	73 ρ. 127 P < 0.001	42 ρ. 299 P < 0.001	24	15	116	81 ρ. 766 p < 0.001
Hepatomegaly	89 ρ. 465 P < .001	46	45	67	46 ρ. 160 P0.003	34	27 ρ. 215 P < 0.001	21	10	81 ρ. 702 P < 0.001	89

Values highlighted in bold are statistically significant
Numbers into the table represent the number of children exhibiting the identified manifestations. The value of the ρ correlation has been specifically reported for each one correlation, properly adjusted for multiple correlations
p < 0.05 was considered as significant

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ISSN: 1750-1172

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