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Table 2 Variables of our patient at the time of admission to our hospital, when he started the second line therapy with Sirolimus and after three months since the begging of this therapy

From: Late-onset of immunodysregulation, polyendocrinopathy, enteropathy, x-linked syndrome (IPEX) with intractable diarrhea

Variables

Reference range, age and sex-adjusted

Admission

Start SIROLIMUS

3 months after SIROLIMUS

White-cell count — per mm 3

4.5 - 13.5

15.01

4.04

5.01

Hemoglobin — g/dl

11.5 - 14.5

16.3

11.7

11.5

Hematocrit —%

35 - 42

46.0

34.4

35.7

Differential count —%

    

Neutrophils

40.0 - 74.0

89.6

51.2

48.0

Lymphocytes

19.0 - 48.0

6.6

30.3

38.0

Monocytes

3.0 - 9.0

2.4

13.5

7.6

Eosinophils

0.0 - 6.0

0.4

1.7

4.4

Basophils

0.0 - 1.5

0.3

1.1

0.7

Platelet count — per mm 3

250 - 550

522

247

273

Glucose — mg/dl

60 - 100

125

107

77

Insulinemia — microU/mL

7 - 24

 

6.8

 

C-peptide — ng/mL

1.1 - 4.4

2.7

 

Islet cell autoantibodies

Neg

Neg

Neg

Glutamic acid decarboxylase— UI/ml

<10 Neg

Neg

Neg

 

>10 Pos

   

UREA — mg/dl

15 - 50

72

40

18

Creatinine — mg/dl

0.5 - 1

0.91

0.54

0.35

Uric Ac. — mg/dl

2.2 - 6.6

8.6

5.2

3.4

Total Colesterol — mg/dl

130 - 204

 

121

 

TG — mg/dl

31 - 108

40

HDL — mg/dl

> 35

62

LDL — mg/dl

< 170

50

Electrolytes — mmol/L

    

Sodium

136 - 146

128

139

142

Potassium

3.5 - 5.3

5.5

4.3

4.3

Chlorine

98 - 106

85

103

105

Calcium

8.8 - 10.8

9.6

9.3

9.2

Phosphorus — mg/dl

2.9 - 5.4

7.6

5

4.4

Magnesium — mg/dl

1.6 - 2.6

2.2

1.6

2.1

Plasma Osmolarity — mOsm/L

278 - 305

266

  

Protein — g/dl

    

Total

6,4 - 8.1

4.1

6.2

6.7

Albumin

3.5 - 5

2.4

4.2

4.3

γ–Globulin —%

11.1 - 18.8

10.5

11.4

13.4

Bilirubin — mg/dl

    

Total

0.20 - 1.10

1.54

0.44

0.3

Direct/Indirect

0.00-0.30/< 0.80

0.48/1.06

0.21/0.23

0.1/0.2

AST/ALT — U/L

< 38/< 41

44/34

16/10

22/17

Total Amylase — U/L

30 - 100

50

60

 

Iron — μg/dl

53 - 119

 

47

52

U.I.B.C./T.I.B.C. — μg/dl

110-330/250-400

300/347

273/325

Ferritin — ng/mL

7 - 140

22

16

TSH — microU/mL

0.6 - 6.3

 

1.93

1.02

FT3 — pg/mL

2.5 - 5.5

3.6

4.1

FT4 — pg/mL

9.0 - 17.0

20.7

12.9

ATA — UI/mL

< 115

23

16

Anti TPO Ab — UI/mL

< 34

12

13

ESR — mm

< 15

6

15

9

CRP — mg/dl

< 0.5

0.05

2.05

0.09

Ab anti harmonine IgG — U.A.

< 3.0 absent

 

>100

 

> 0.3 present

ANA

< 1:80

 

< 1:80

 

AMA

< 1:40

 

< 1:40

ENA

< 0,7 Neg

 

Neg

 

0.7 - 1-0 Bl

  
 

> 1.0 Pos

  
  1. ALT Alanine aminotransferase, AMA Anti-mitochondrial antibodies, ANA Antinuclear antibodies, anti-TPO Ab Anti-ThyroidPeroxidase Antibodies, AST aspartate aminotransferase, ATA Anti-Thyroglobulin Antibodies, Bl Borderline, CRP C-reactive protein, ENA Extractable Nuclear Antigens, ESR erythrocyte sedimentation rate, FT3 Free Triiodothyronine, FT4 Free Thyroxine, HDL High-Density Lipoprotein, LDL Low-Density Lipoprotein, Neg Negative, Pos Positive, T.I.B.C. Total iron-binding capacity, TG triglycerides, TSH Thyroid-Stimulating Hormone, U.I.B.C. Unsaturated Iron Binding Capacity.