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. Author manuscript; available in PMC: 2020 Oct 1.
Published in final edited form as: J Clin Immunol. 2019 Aug 2;39(7):653–667. doi: 10.1007/s10875-019-00659-8

Table 1.

Demographics and co-morbidities in patients with and without IBD.

IBD
N=49
No IBD
N=96
P
Median Age (Years) at CGD Diagnosis (Range) 1.5# (<1–16) 1# (<1–18) 0.452
Median Age (Years) at Allogeneic HCT (Range) 11 (1–26) 5 (<1–21) <0.001
Genetic Mutation (Protein Deficiency)
CYBA (p22phox) 2 (4.1%) 6 (6.3%) 0.172
NCF4 (p40phox) 1 (2.0%)
NCF1 (p47phox) 2 (4.1%) 15 (15.6%)
NCF2 (p67phox) 1 (2.0%) 3 (3.1%)
CYBB (gp91phox) 40 (81.6%) 66 (68.8%)
 Unknown 3 (6.1%) 6 (6.2%)
Oxidase Function
 Unable to Be Determined 6 (12.2%) 16 (16.7%) 0.718
 Oxidase Null 28 (57.1%) 55 (57.3%)
 Oxidase Positive 15 (30.6%) 25 (26.0%)
History of Infection(s) Within 1 Year Prior to Allogeneic HCT 38 (77.6%) 64 (66.7%) 0.175
  Staphylococcus Aureus 1 (2.0%) 8 (8.3%) 0.137
  Nocardia Species Infection 1 (2.0%) 4 (4.2%) 0.507
  Aspergillus Species Infection 7 (14.3%) 17 (17.7%) 0.600
History of Pneumonia Within 1 Year Prior to Allogeneic HCT 23 (46.9%) 37 (38.5%) 0.332
History of Lung Resection 3 (6.1%) 8 (8.4%) 0.623
History of Liver Resection 1 (2.0%) 0 (0%) 0.162
History of Gastrointestinal Resection 0 0 N/A
Median Albumin g/dL (Range) 3.5@ (1.7–4.4) 3.8@ (2.5–5.0) <0.001
Weight <5th Percentile for Age 17* (35.4%) 14 (14.6%) 0.004
Height <5th Percentile for Age 24 (51.1%) 25 (26.0%) 0.003
Lansky or Karnofsky Score <90 Prior to Allogeneic HCT 10+ (20.4%) 5+ (5.2%) 0.007
Received IBD Therapy 47 (97.9%)
IBD Controlled Prior to Allogeneic HCT 32$ (65%)
#

Age at diagnosis was reported for 24 IBD patients and 71 patients without IBD

@

Albumin data was reported for 48 IBD patients and 92 patients without IBD

*

Weight data was reported for 48 IBD patients

Height data was reported for 47 IBD patients

+

Lansky or Karnofsky was unknown for 1 IBD patient and 9 patients without IBD

IBD therapy was reported for 48 IBD patients

$

IBD status was reported for 48 IBD patients (and reported as unknown for 1 IBD Patient)