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Table 3 Systemic involvement and underline disease

From: Clinical study of children with cryofibrinogenemia: a retrospective study from a single center

Case No.

1

2

3

4

5

6

7

8

No. Abnormal/Total

%

Skin biopsy

(+)

(−)

ND

ND

(+)

ND

ND

(−)

2/4

50

Renal involvement

3/8

37.5

 Proteinuria

(−)

(−)

(−)

(−)

(+)

(+)

(−)

(−)

2/8

25

 Hematuria

(−)

(−)

(−)

(+)

(+)

(+)

(−)

(−)

3/8

37.5

 Renal insufficiency

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(+)

1/8

12.5

Arthritis

(+)

(+)

(+)

(+)

(+)

(+)

(+)

(+)

8/8

100

Thrombotic events

(−)

(−)

(+)

(+)

(−)

(+)

(+)

(+)

5/8

62.5

 Angiogram

ND

ND

(+)

(+)

ND

(+)

ND

(+)

4/4

100

Respiratory involvement

 Pulmonary embolism

(+)

ND

(−)

ND

(−)

ND

(−)

ND

1/4

25

 ARDS

(+)

(−)

(+)

(+)

(−)

(−)

(+)

(−)

4/8

50

Neurological involvement

 Multineuritis

(+)

(−)

(+)

ND

(−)

(−)

(−)

(+)

3/7

42.9

 Brain MRI

(−)

(−)

(+)

(+)

ND

(−)

(+)

(+)

4/7

57.1

 Spine MRI

ND

ND

(−)

ND

ND

ND

ND

(−)

0/2

0

 SSEP

ND

(−)

(−)

ND

ND

(−)

ND

ND

0/3

0

 EMG

ND

(−)

ND

ND

ND

ND

(+)

ND

1/2

50

 EEG

ND

(+)

(−)

ND

ND

ND

(+)

(+)

3/4

75

 BAEP

ND

(−)

ND

ND

ND

(−)

(−)

(+)

1/4

25

 VEP

ND

ND

ND

ND

ND

ND

(+)

(+)

2/2

100

Associated disease

 Autoimmune disease

CG

(−)

JIA

JIA

JIA, JDM, SLE

JIA

B, SLE, JIA

CM, JIA, B

6/8

75

 HCV/HBV infection

(−)

(−)

(−)

(−)

(−)

(−)

(−)

(−)

0

0

  1. (+) = abnormal; (−) = normal; ND = not done
  2. ARDS acute respiratory distress syndrome, NCV nerve conduction velocity, EMG electromyogram, SSEP somatosensory evoked potential, BAEP brainstem auditory evoked potential, VEP visual evoked potential, HCV hepatitis C virus infection, HBV hepatitis B virus infection, CG cryoglobulinemia, JIA juvenile idiopathic arthritis, JDM juvenile dermatomyositis, SLE systemic lupus erythematosus, B Behcet’s disease, CM Cutaneous mastocytosis