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Table 2 Etiology of conjugated hyperbilirubinemia in infancy by study

From: Etiologies of conjugated hyperbilirubinemia in infancy: a systematic review of 1692 subjects

 

Extrahepatic BA

Ideopathic Neonatal Hepatitis

TPN associated cholestasis

Alagille Syndrome

Interlobular bile duct paucity

Alpha 1 Antitrypsin deficiency

Cystic Fibrosis

Infection

Hypopituitarism hypothyroid

Progressive familial intrahepatic cholestasis

Perinatal hypoxia-ischemia

Inspissated bile syndrome

Choledochal cyst

Hemolysis

Metabolic disease

Other a

 

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

N/%

Hitch et. al [18]

9

7

6

-

1

1

-

-

-

-

-

-

1

  

3

32.14%

25.00%

21.43%

 

3.57%

3.57%

      

3.57%

  

10.71%

Tolia et. al [11]

6

2

6

-

2

1

-

2

-

-

-

-

-

-

-

1

30.00%

10.00%

30.00%

 

10.00%

5.00%

 

10.00%

       

5.00%

Tiker et. al [12]

1

2

3

-

-

-

-

16

1

-

7

-

-

5

1

6

2.38%

4.76%

7.14%

    

38.10%

2.38%

 

16.67%

  

11.90%

2.38%

14.29%

Humphrey et. al [17]

30

22

-

4

2

4

3

2

2

2

-

1

1

-

-

17

33.33%

24.44%

 

4.44%

2.22%

4.44%

3.33%

2.22%

2.22%

2.22%

 

1.11%

1.11%

  

18.89%

Bazlul Karim et. al [5]

16

15

-

-

1

1

-

22

3

-

-

-

4

-

-

-

25.81%

24.19%

  

1.61%

1.61%

 

35.48%

4.84%

   

6.45%

   

Jiang et. al [10]

23

22

-

-

-

-

-

-

-

1

-

-

2

-

2

-

46.00%

44.00%

       

2.00%

  

4.00%

 

4.00%

 

Fischler et. al [9]

30

15

-

3

-

11

1

7

1

11

-

-

-

-

-

6

35.29%

17.65%

 

3.53%

 

12.94%

1.18%

8.24%

1.18%

12.94%

     

7.06%

Spivak et. al [13]

7

6

5

-

6

-

-

1

-

-

-

3

-

-

-

5

21.21%

18.18%

15.15%

 

18.18%

  

3.03%

   

9.09%

   

15.15%

Motala et. al [14]

41

52

Excludeds

-

-

6

1

35

-

-

-

-

5

-

5

-

28.28%

35.86%

4.14%

0.69%

24.14%

    

3.45%

 

3.45%

 

Ipek et. al [16]

4

7

8

-

2

-

-

9

4

-

28

6

-

15

6

3

4.35%

7.61%

8.70%

 

2.17%

  

9.78%

4.35%

 

30.43%

6.52%

 

16.30%

6.52%

3.26%

Rafeey et. al [2]

30

44

-

1

13

-

1

4

-

1

-

-

2

-

25

1

24.59%

36.07%

 

0.82%

10.66%

 

0.82%

3.28%

 

0.82%

  

1.64%

 

20.49%

0.82%

Johnson et. al [15]

31

33

-

-

-

1

-

12

-

-

-

3

2

-

-

19

30.69%

32.67%

   

0.99%

 

11.88%

   

2.97%

1.98%

  

18.81%

Stormon et. al [7]

34

18

35

6

1

13

7

23

10

2

21

9

3

-

17

6

16.59%

8.78%

17.07%

2.93%

0.49%

6.34%

3.41%

11.22%

4.88%

0.98%

10.24%

4.39%

1.46%

 

8.29%

2.93%

Mowat et. al [6]

32

61

-

-

1

24

2

9

-

-

-

-

2

-

2

4

23.36%

44.53%

  

0.73%

17.52%

1.46%

6.57%

    

1.46%

 

1.46%

2.92%

Yachha et. al [4]

33

7

-

-

2

-

-

5

-

-

-

-

-

-

2

11

55.00%

11.67%

  

3.33%

  

8.33%

      

3.33%

18.33%

Aanpreung et. al [3]

56

58

46

2

-

-

-

25

12

-

6

1

14

4

8

17

22.49%

23.29%

18.47%

0.80%

   

10.04%

4.82%

 

2.41%

0.40%

5.62%

1.61%

3.21%

6.83%

Danks et. al [19]

55

69

-

-

11

8

-

22

-

-

-

-

Excluded

-

6

-

32.16%

40.35%

  

6.43%

4.68%

 

12.87%

      

3.51%

 
  1. a See text for complete list and count of “other” diagnoses
  2. ♦TPN associated cholestasis was excluded from Motala et. al. See text for further detail.
  3. ✧Choledochal cysts were excluded from Danks et. al. See text for further detail