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Table 1 Clinical and laboratory characteristics of the patient at diagnosis of SDS (2 years), diagnosis of liver disease (12 years), diagnosis of HPS (15 years), liver transplantation (18 years) and at the present follow-up

From: Liver transplantation for very severe hepatopulmonary syndrome due to vitamin A-induced chronic liver disease in a patient with Shwachman-Diamond syndrome

  Age 2 Age 12 Age 15 Age 18
Liver transplant
Age 22
Height 74.6 cm (< 3° %ile) 129 cm (< 3° %ile) 135 cm (< 3° %ile) 140 cm (< 3° %ile) 141 cm (< 3° %ile)
Weight 7.6 kg (< 3° %ile) 21 kg (< 3° %ile) 30 kg (< 3° %ile) 41 kg (< 3° %ile) 44.2 kg (< 3° %ile)
CMV-PCR NA Negative Negative Negative Negative
SatO2 at rest 100% 99% 88% 79% 99%
PaO2 arterial blood gas in room air 115 mmHg NA 50 mmHg 35 mmHg NA
A-a gradient in room air -6 mmHg NA 61 mmHg 75 mmHg NA
White blood count 8400/uL (5500-15,500) 3000/uL (4500-13,000) 1390/uL (4000-10,000) 1500/uL (4000-10,000) 4000/uL (4000-10,000)
Neutrophils 2200/uL (1500-9000) 1000/uL (1800-8000) 400/uL (2500-7800) 400/uL (2500-7800) 1400/uL (2500-7800)
Hemoglobin 13.9 g/dL (11.5-13.5) 10.9 g/dL (12-16) 9.4 g/dL (12-16) 10.4 g/dL (12-16) 16 g/dL (12-16)
Platelets 307,000/uL
(100000-450,000)
48,000/uL
(150000-450,000)
23,000/uL (150000-450,000) 19,000/uL (150000-450,000) 153,000/uL (150000-450,000)
Lymphocytes 4700/uL (1700-6900) 1700/uL (1200-3600) 840/uL (1200-3600) 800/uL (1200-3600) 1290/uL (1200-3600)
T cells (CD3+) NA NA 768/uL (800-3500) NA 1060/uL (798-2823)
B cells (CD19+) NA NA 49/uL (200-600) NA 120/uL (82-476)
NK cells (CD56+) NA NA 22/uL (70-1200) NA 79/uL (66-745)
IgG NA 15.60 g/L (5.30-13) 14.60 g/L (5.76-12.65) 12.20 g/L (7.51-15.60) 9.10 g/L (7.51-15.60)
IgA NA 3.23 g/L (0.60-2.70) 4.11 g/L (0.81-2.32) 4.10 g/L (0.82-4.53) 3.19 g/L (0.82-4.53)
IgM NA 0.85 g/L (0.43 - 1.73) 0.67 g/L (0.30-1.59) 1.13 g/L (0.46-3.04) 0.47 g/L (0.46-3.04)
AST 166 U/L (5-37) 46 U/L (≤ 32) 32 U/L (≤ 32) 27 U/L (≤ 32) 23 U/L (≤ 31)
ALT 187 U/L (5-37) 15 U/L (≤ 31) 20 U/L(≤ 31) 19 U/L (≤ 31) 14 U/L (≤ 31)
Gamma GT 27 U/L (7-32) 69 U/L (≤ 35) 13 U/L (≤ 35) 13 U/L (≤ 35) 7 U/L (≤ 40)
Total bilirubin 0.43 mg/dL (0.2-1) 1.58 mg/dL (≤ 1) 3.79 mg/dL (≤ 1) 5.17 mg/dL (≤ 1) 0.38 mg/dL (≤ 1.18)
INR NA 1.3 1.5 1.3 1
Albumin 45 g/L (32-52) 38 g/L (35-52) 35 g/L (35-52) 37 g/L (35-52) 39 g/L (35-52)
  1. A-a Alveolar-arterial, ALT Alanine transaminase, AST Aspartate transaminase, CMV Cytomegalovirus, Gamma GT Gamma-glutamyltransferase, HPS Hepatopulmonary syndrome, Ig Immunoglobulin, INR International normalized ratio, mmHg Millimeters of mercury, NA No data available, NK Natural killer, uL Microliter, %ile Percentile. Reference ranges for age in brackets