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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