CHOP Lab Reference Ranges | Children's Hospital of ... · atp lymph 0 ‐ 2 0 ‐ 22 ‐ 42 ‐ 4 2...

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Transcript of CHOP Lab Reference Ranges | Children's Hospital of ... · atp lymph 0 ‐ 2 0 ‐ 22 ‐ 42 ‐ 4 2...

Core Laboratory Procedure Manual

THE CHILDREN’S HOSPITAL OF PHILADELPHIA

Page 1 of 2 Effective Date:

Hematology Normal Values Book 21 – 21.010P

ParameterMale Female Male Female Male Female Male Female Male Female Male Female Male Female Male Female Male Female Male Female Male Female

WBC 7.80‐15.91 8.36‐14.42 8.14‐14.99 7.05‐14.68 6.51‐13.32 6.00‐13.25 5.98‐13.51 6.48‐13.02 5.14‐13.38 4.86‐13.18 4.31‐11.00 4.27‐11.40 3.84‐9.84 4.19‐9.43 3.91‐8.77 4.37‐9.68RBC 4.50‐5.30 4.10‐5.10 4.50‐5.90 4.00‐5.20HGB 13.0‐16.0 12.0‐16.0 13.5‐17.5 12.0‐16.0HCT 37.0‐49.0 36.0‐46.0 41.0‐53.0 36.0‐46.0MCV 78‐98 78‐102 80‐100 80‐100MCH 25‐35 25‐35 26‐34 26‐34MCHC 31‐37 31‐37 31‐37 31‐37RDW STDV 46.3‐57.3 47.2‐59.8 43.9‐52.8 43.0‐55.0 35.3‐45.7 35.2‐45.1 35.3‐42.8 34.9‐42.4 35.1‐41.7 34.9‐42.0 35.1‐41.7 35.5‐41.8 36.7‐43.8 37.1‐44.2 37.8‐46.1 38.4‐47.7RDW CV 14.3‐16.8 14.4‐16.2 13.8‐16.1 13.6‐15.8 12.4‐15.3 12.2‐14.3 12.9‐15.6 12.7‐15.1 125‐14.9 12.4‐14.9 12.3‐14.1 12.2‐14.4 12.4‐14.5 12.3‐14.6 12.3‐14.3 12.4‐15.1PLTMPV 10.1‐12.1 10.0‐12.2 9.2 ‐ 10.8 9.4‐11.1 8.9‐10.6 9.0‐10.9 8.7‐10.5 8.8‐10.6 9.0‐10.9 8.9‐11.0 9.2‐11.4 9.3‐11.3 9.6‐11.8 9.6‐11.7 9.7‐11.9 9.6‐12.0IPF 1.4 ‐ 3.8 1.4‐4.5% 1.1‐3.6% 1.0‐3.6% 1.0‐4.9% 1.0‐4.7% 1.6‐6.1% 1.4‐6.4% 1.6‐7.1% 1.6‐4.9%BLASTPROMYMYELOMETAIGEige abSEG 14.0‐54.6 10.6‐57.3 10.2‐48.7 8.9‐68.2 10.9‐47.8 14.1‐76.0 17.5‐69.5 16.9‐74.0 22.4‐69.0 22.4‐69.0 28.6‐74.5 29.8‐71.4 32.5‐74.7 39.0‐73.6 40.3‐74.8 42.5‐73.2seg ab 1108‐5450 1230‐4800 830‐4230 1000‐4680 970‐5450 1040‐7200 1190‐7210 1270‐7180 1540‐7920 1600‐8290 1630‐7550 1640‐7870 1540‐7040 1820‐7470 1820‐7420 2000‐7150EOS . 0.0‐5.3 0.0‐4.5 0.0‐4.1 0.0‐4.0 0.0‐3.6 0.0‐3.7 0.0‐3.2 0.0‐4.1 0.0‐3.3 0.0‐4.7 0.0‐4.0 0.0‐4.0 0.0‐3.4 0.0‐4.4 0.0‐3.0eos ab 80 ‐ 800 60‐750 50‐570 40‐630 30‐610 20‐740 20‐820 20‐580 30‐530 30‐460 30‐520 30‐470 40‐380 20‐320 30‐440 30‐270BASO 0.0‐0.6 0.0‐0.5 0.0‐0.6 0.0‐0.5 0.0‐0.6 0.0‐0.6 0.0‐0.6 0.0‐0.6 0.1‐0.6 0.0‐0.6 0.0‐0.7 0.0‐0.6 0.0‐0.7 0.0‐0.6 0.0‐0.7 0.0‐0.7baso ab 10 ‐ 70 10 ‐ 60 10 ‐ 70 10 ‐ 50 10 ‐ 60 10 ‐ 70 10 ‐ 60 10 ‐ 60 10 ‐ 60 10 ‐ 60 10 ‐ 60 10 ‐ 50 10 ‐ 50 10 ‐ 50 10 ‐ 50 10 ‐ 50LYMPH 33.6 ‐ 76.8 31.9‐82.7 42.5‐85.7 37.8‐86.7 40.7‐83.7 30.4‐85.6 26.0‐79.6 27.4‐79.9 18.4‐66.6 18.1‐68.6 15.5‐56.6 16.7‐57.8 16.4‐52.7 18.2‐49.8 12.2‐47.1 18.2‐47.4lymph ab 2110‐8380 2420‐8200 2470‐7950 2290‐9142 2140‐8990 2140‐8990 1560‐7830 1520‐8090 1130‐5520 1250‐5770 970‐3960 1160‐4280 970‐3260 1160‐3330 850‐3000 1160‐3180ATP LYMPH 0 ‐ 2  0 ‐ 2 2 ‐ 4 2 ‐ 4  2 ‐ 4 2 ‐ 3 2 ‐ 4 2 ‐ 3 2 ‐ 3  2 ‐ 3 2 ‐ 3 2 ‐ 4 2 ‐ 4 2 ‐ 4 2 ‐ 4 2 ‐ 4 2 ‐ 3 2 ‐ 3 0 ‐ 2 0 ‐ 2 0 ‐ 2 0 ‐ 2MONO 4.3‐18.3 5.6‐13.8 4.4‐14.0 3.8‐15.5 3.8‐13.4 3.8‐12.6 4.4‐13.4 3.8‐12.8 4.2‐12.2 4.1‐11.4 4.2‐12.3 4.2‐11.3 4.4‐12.3 4.1‐10.9 4.4‐12.3 4.3‐11.0mono ab 280 ‐ 1380 420‐1210 280‐1050 280‐1210 280‐1070 240‐1170 250‐1150 260‐1080 190‐940 240‐920 190‐850 190‐810 180‐780 190‐720 190‐770 290‐710NRBCnrbc abRETICretic abIRFRET‐HE 28.7‐35.7 30.1‐35.7 27.7‐37.8 29.3‐37.3 32.4‐37.6 30.4‐39.7 30.3‐40.4 29.9‐38.4 36.0‐38.6 30.6‐40.7ESR 0 ‐ 20 mm/hr

31 ‐ 37

147.5 ‐ 216.4 4 ‐ 30 days:  Males and Feamles = 51.3 ‐ 110.4 51.8 ‐ 77.9 48.2 ‐ 88.2 43.5 ‐ 111.1 36.4 ‐ 68.0

30 ‐ 36

0 ‐ 14 days:  Males = 0.12‐0.66, Females = 0.09 ‐ 0.640 ‐ 14 days:  Males = 0.1 ‐ 0.8, Females = 0.1 ‐ 0.6

0 ‐ 14 days :  Males = 20‐110, Feamles = 20‐700 ‐ 14 days:  Males = 33.7 ‐ 67.6, Females = 24.9‐68.5

0 ‐ 14 days:  Males = 2070 ‐ 7530, females = 1750 ‐ 8000

0 ‐ 14 days:  Males = 6.7 ‐ 19.9, Females = 5.2 ‐ 20.6

29 ‐ 37 31 ‐ 37

M = 0.0 ‐ 0.0, F = 0.0 ‐ 0.0

30 ‐ 36

3.47 ‐ 5.40 2.12 ‐ 3.47 1.55 ‐ 2.70 0.99 ‐ 1.82 0.82 ‐ 1.45

42.0 ‐ 67.098 ‐ 12131 ‐ 3730 ‐ 37 29 ‐ 37

39.0 ‐ 63.086 ‐ 12428 ‐ 4028 ‐ 38

4 ‐ 30 days  Males and Females = 1.06 ‐ 2.37

28 ‐ 4028 ‐ 38

11.5 ‐ 15.53.60 ‐ 6.20 3.00 ‐ 5.40 2.70 ‐ 4.90 3.10 ‐ 4.50 3.70 ‐ 5.30

34.0 ‐ 40.075 ‐ 8724 ‐ 30

31.0 ‐ 55.085 ‐ 12328 ‐ 40

28.0 ‐ 42.077 ‐ 11526 ‐ 34

33.0 ‐ 39.070 ‐ 8623 ‐ 31

29.0 ‐ 41.074 ‐ 10825 ‐ 35

35.0 ‐ 45.077 ‐ 9525 ‐ 33

0 ‐ 180 days M = 27.6 ‐ 38.7, F = 29.2 ‐ 37.5

M = 0.0 ‐ 0.0, F = 0.0 ‐ 0.0

M = 0.0 ‐ 0.0, F = 0.0 ‐ 0.0M = 0.0 ‐ 0.0, F = 0.0 ‐ 0.0

0 ‐ 90

4‐30d M/F 0.0‐0.01‐3d M/F 0.06‐1.30 4‐30d M/F 0.04‐0.11 M/F 0.03‐0.09 M/F 0.03‐0.13 0.03‐0.12 0.03‐0.32 0.03‐0.15 0.03‐0.13

≤ 2 days 0 ‐ 280 2‐14 days 0 ‐ 270 14‐30 days 0‐220 31‐90days 0‐90 91‐180 days 0‐600.0‐0.9 0.0‐0.8 0.0 ‐ 0.3 0.0 ‐ 0.3 0.0 ‐ 0.6

8.9 ‐ 24.1 9.0 ‐ 18.7 9.3 ‐ 17.4

0 ‐ 140 0 ‐ 60

8.4 ‐ 21.7

M/F 0.0‐0.0 M/F 0.0‐0.0 M/F 0.0 ‐ 0.0

0 ‐ 40 0 ‐ 30

M/F 0.0‐0.0 M/F 0.0‐0.00.03‐0.11

0.98 ‐ 1.94 0.90 ‐ 1.49 0.86 ‐ 1.3642.4 ‐ 70.2 41.6 ‐ 65.1 39.1 ‐ 57.0

2 ‐ <6 years 6 ‐ <12 years 12 ‐ <18 years ≥ 18 years

Males = 2.0‐6.8%  Females = 1.3‐6.8%

≤ 3 days 4 ‐ 7 days 8 ‐ 14 days 15 ‐ 30 days 31 ‐ 60 days 61 ‐ 180 days

0 ‐ 14 days:  M = 51.0 ‐ 61.7, F = 51.4 ‐ 65.7

3.90 ‐ 6.60 3.90 ‐ 6.30 3.90 ‐ 5.30 4.00 ‐ 5.2013.5 ‐ 22.5 13.5 ‐ 19.5 12.5 ‐ 20.5 10.0 ‐ 18.0 9.0 ‐ 13.5 9.5 ‐ 13.5 10.5 ‐ 13.5 11.5 ‐ 13.5

150 ‐ 400

13.4 ‐ 23.3% 11.4 ‐ 25.8

6 months ‐ <2 years

Males 520 ‐ 1770        Females 570 ‐ 1720

0‐14 days:  Males = 20.2 ‐ 46.2, Females = 15.2 ‐ 66.1

1‐3d M/F 0.1‐8.3 M/F 0.0‐0.0 M/F 0.0‐0.0

0 ‐ 14 days:  Males ‐ 1600 ‐ 6060, Females = 1730 ‐ 67500 ‐ 14 days:  Males = 0.3 ‐ 5.2, Females = 0.4‐4.6

≤ 2 days 0.0 ‐ 1.7 2 ‐ 14 days 0.0 ‐ 1.9 14 ‐ 30 days 0.0 ‐ 1.3 31‐90days0.0 ‐ 0.9 91‐180 days0.0‐0.5

1‐3D,M/F 30.5‐35.1%  Male/Female 14.5‐24.6% 19.1 ‐ 28.9

0‐14 days  M = 8.04 ‐15.40, F = 8.16 ‐ 14.56

0 ‐ 14 days:  M = 14.8 ‐ 17.0, F = 14.6 ‐ 17.3

0 ‐ 14 days:  M = 10.2 ‐ 11.9, F = 10.4 ‐ 12.0

42.0 ‐ 66.088 ‐ 126

Hematology Normal Values Page 2 of 2

Book 21 – Hematology Normal Values Book 21 – 21.010P

Urinalysis: Sugar negative Bilirubin negative Ketones negative Spec.Gravity 1.003 –1.035 Blood negative pH 4.8 – 7.8 Protein negative Urobilinogen 0.2 – 1.0 E.U. Nitrite negative Leukocyte negative RBCs negative WBCs negative Urine Casts negative Urine Epi. negative Bacteria negative Yeast negative Mucus negative Amorphous negative Ur. Crystals negative Enhanced UA, WBC ≤ 9/cumm Fluids CSF: WBC RBC Neutrophils Lymphocytes Monocytes Neonates 0 – 30 /µl 0/ µl 0 – 8% 5 – 35% 50 – 90 % (30 days and younger) Adults 0 – 5/µl 0/ µl 0 - 6% 40 - 80% 15 – 45% References:

1. Nathan, David G, Orkin, Stuart H.: Nathan and Oski’s Hematology of Infancy and Childhood. 5th Edition, Volume 1 and II, W.B. Saunders Company, Philadelphia 1998.

2. Nathan, David G, Orkin, Stuart H.: Nathan and Oski’s Hematology of Infancy and Childhood. 6th Edition, Volume II, W.B. Saunders Company, Philadelphia 2003.

3. Soldin, Steven J, Wong, Edward C, Brugnara, Carlo, Soldin, Offie P. Pediatric Refernce Invervals. AACC Press, 2011.

4. Graff, Sister Laurine: A Handbook of Routine Urinalysis, J.B. Lippincott Company, 1983. 5. Galagan, Blomberg, Cornbleet, Glassy.Color Atlas of Body Fluids. College of American

Pathologists 2006. 6. Shaw KN, McGowan KL, Gorelick MH, Sanford Schwartz J., Screening for Urinary

Tract Infection in Infants in the Emergency Department: Which Test is Best? J Pediatrics, June 1998.

Children's Hospital of PhiladelphiaClinical Immunology Lab

Normal Ranges

Prepared 10/13/2017 Page 1 of 5

Analyte Age Normal Range UnitsAnti-Cardiolipin - IgG 0 - 14 GPLAnti-Cardiolipin - IgM 0 - 11 MPL

Anti-β2 Glycoprotein II - IgG 0 - 20 SGUAnti-β2 Glycoprotein II - IgM 0 - 20 SMUAnti-Diphtheria Antibodies > 0.1 IU/ml

Anti-dsDNA 0 - 9.9 IU/mlAnti-Endomysial Antibodies - IgA Negative

Anti-F-Actin 0 - 20 UnitsAnti-Gliadin (Deamidated - DGP) - IgA 0 - 19.9 UnitsAnti-Gliadin (Deamidated - DGP) - IgG 0 - 19.9 UnitsAnti-Liver/Kidney Microsome 1 (LKM-1) 0 - 20 Units

Anti-Neutrophil Cytoplasmic Antibodies (ANCA) NegativeAnti-Nuclear Antibodies (ANA) Negative

Anti-RNP 0 - 4.9 EliA U/mlAnti-S. cerevisiae (ASCA) - IgA 0 - 20 UnitsAnti-S. cerevisiae (ASCA) - IgG 0 - 20 Units

Anti-Sm 0 - 6.9 EliA U/mlAnti-Smooth Muscle Antibodies (ASMA) Negative

Anti-SS-A (Ro) 0 - 6.9 EliA U/mlAnti-SS-B (La) 0 - 6.9 EliA U/ml

Anti-Tetanus Antibodies > 0.15 IU/mlAnti-Tissue Transglutaminase (tTG) - IgA 0 - 20 Units

Calprotectin - Fecal < 15.6 - 50 µg/g< 2 days 60 - 158

2 - 7 days 67 - 1618 days - 5 months 73 - 165

6 - 24 months 78 - 1692 - 5 years 83 - 1746 - 9 years 87 - 181

10 - 14 years 90 - 187

Complement C3 mg/dl

Children's Hospital of PhiladelphiaClinical Immunology Lab

Normal Ranges

Prepared 10/13/2017 Page 2 of 5

Analyte Age Normal Range Units> 14 years 86 - 233

Complement C4 16 - 45 mg/dl< 1 month 0 - 5

1 - 6 months 20 - 727 - 9 months 27 - 73

10 - 23 months 27 - 1692 years 35 - 2223 years 40 - 251

4 -6 years 48 - 3367 -9 years 70 - 393

10 -12 years 70 - 45613 - 14 years 70 - 498

> 14 years 70 - 4860 - 11 months 0 - 15

1 - 5 years 0 - 606 - 9 years 0 - 90

10 - 15 years 0 - 200> 15 years 0 - 100< 1 month 598 - 1672

1 - 6 months 218 - 6367 - 9 months 292 - 816

10 - 23 months 383 - 10702 years 423 - 11843 years 477 - 1334

4 -6 years 539 - 15977 -9 years 583 - 1783

10 -12 years 511 - 165713 - 14 years 562 - 1607

> 14 years 635 - 1775IgG - CSF 0.5 - 6.0 mg/dl

Complement C3

IgA

IgE

IgG

mg/dl

mg/dl

IU/ml

mg/dl

Children's Hospital of PhiladelphiaClinical Immunology Lab

Normal Ranges

Prepared 10/13/2017 Page 3 of 5

Analyte Age Normal Range Units< 1 month 240 - 1060

1 - 5 months 180 - 7006 - 11 months 200 - 770

1 year 250 - 8502 years 320 - 9003 years 350 - 940

4 - 5 years 370 - 10006 - 8 years 400 - 10809 - 11 years 400 - 1150

12 - 17 years 370 - 1280> 17 years 490 - 1140< 1 month 87 - 410

1 - 5 months 34 - 2106 - 11 months 34 - 230

1 year 38 - 2602 years 52 - 2803 years 63 - 300

4 - 5 years 72 - 3406 - 8 years 85 - 4109 - 11 years 98 - 480

12 - 17 years 106 - 610> 17 years 150 - 640

IgG2 mg/dl

IgG1 mg/dl

Children's Hospital of PhiladelphiaClinical Immunology Lab

Normal Ranges

Prepared 10/13/2017 Page 4 of 5

Analyte Age Normal Range Units< 1 month 14 - 55

1 - 5 months 14 - 806 - 11 months 15 - 97

1 year 15 - 1132 years 14 - 1203 years 13 - 126

4 - 5 years 13 - 1336 - 8 years 13 - 1429 - 11 years 15 - 149

12 - 17 years 18 - 163> 17 years 20 - 110< 1 month 4 - 55

1 - 5 months 1 - 366 - 11 months 1 - 43

1 year 1 - 792 years 1 - 1063 years 1 - 127

4 - 5 years 1 - 1586 - 8 years 1 - 1899 - 11 years 3 - 210

12 - 17 years 4 - 230> 17 years 8 - 140< 1 month 6 - 15

1 - 6 months 11 - 607 - 9 months 37 - 124

10 - 23 months 43 - 1132 years 49 - 1793 years 51 - 194

4 -6 years 50 - 1947 -9 years 50 - 198

10 -12 years 64 - 20813 - 14 years 70 - 212

> 14 years 71 - 237Lyme Antibodies - IgG (ELISA) 0 - 0.79 OD RatioLyme Antibodies - IgM (ELISA) 0 - 0.79 OD Ratio

IgM mg/dl

IgG3 mg/dl

IgG4 mg/dl

Children's Hospital of PhiladelphiaClinical Immunology Lab

Normal Ranges

Prepared 10/13/2017 Page 5 of 5

Analyte Age Normal Range UnitsLyme Antibodies - IgG (Western Blot) NegativeLyme Antibodies - IgM (Western Blot Negative

Rheumatoid Factor (RF) NegativeRPR Non-reactive

Palmieri Metabolic Diagnostic Laboratory Reference Ranges by Test

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PLASMA ACYLCARNITINE PROFILE ACETYL C2 4.21 - 20.60 µmol/L PROPENOYL C3:1 0 - 0.02 µmol/L PROPIONYL C3 0 - 1.60 µmol/L BUTYRYL/ISOB 0 - 1.00 µmol/L TIGL/MECROT C5:1 0 - 0.20 µmol/L 3-OH-BUT C4-OH 0 - 0.40 µmol/L HEXANOYL C6 0.01 - 0.22 µmol/L 3-OH-ISOVAL C5-OH 0 - 0.11 µmol/L OCTENOYL C8:1 0 - 0.70 µmol/L OCTANOYL C8 0 - 0.50 µmol/L MALONYL C3 -DC 0 - 0.13 µmol/L DECADIEN C10:2 0 - 0.200 µmol/L CIS-4-DEC C10:1 0.01 - 0.45 µmol/L DECANOYL C10 0 - 0.90 µmol/L MEMAL/SUCC C4-DC 0 - 0.20 µmol/L GLUTARYL C5-DC 0 - 0.09 µmol/L DODECADIE C12:2 0 - 0.20 µmol/L DODECENYL C12:1 0 - 0.60 µmol/L DODECANOYL C12 0 - 0.60 µmol/L ADIP/MEGLU C6-DC 0 -0.10 µmol/L TETRADEC C14:2 0 - 0.15 µmol/L TETRADECEN C14:1 0 - 0.30 µmol/L TETRADECAN C14:0 0 - 0.30 µmol/L SUBERYL C8-DC 0 - 0.08 µmol/L 3-OH-C12:1 0 - 0.05 µmol/L 3-OH-C12:0 0 - 0.08 µmol/L 3-OH-C14:1 0 - 0.05 µmol/L 3-OH-C14:1 0 - 0.04 µmol/L HEXADECADIE 16:2 0 - 0.2 µmol/L HEXADECENOY 16:1 0 - 0.2 µmol/L PALMITOYL C16 0 - 1.0 µmol/L 3-OH-C16 0 - 0.06 µmol/L LINOLEOYL C18:2 0 - 0.30 µmol/L OLEOYL C18:1 0 - 0.50 µmol/L OCTADECANOY 18:0 0 - 0.11 µmol/L 3OH-LIN C18:20H 0 - 0.05 µmol/L 3OH-OLEOYL C18:1 0 - 0.03 µmol/L C16-DC 0 - 0.03 µmol/L C18:1-DC 0 - 0.03 µmol/L FREE AND TOTAL CARNITINE ANALYSIS PLASMA URINE TOTAL CARNITINE 25 - 69 nmol/mL 50 - 200 nmol/mg creatinine FREE CARNITINE 17 - 59 nmol/mL 34 - 114 nmol/mg creatinine ACYLCARNITINE 4 - 14 nmol/mL 11 - 91 nmol/mg creatinine RATIO (FREE/TOTAL) 0.68 - 0.86 DISACCHARIDASE ENZYME ANALYSIS ENZYME NORMAL DEFICIENT Lactase 14 – 33 nmol/min/mg protein < 14 nmol/min/mg protein Sucrase 25 – 66 nmol/min/mg protein < 25 nmol/min/mg protein Maltase 135 – 205 nmol/min/mg protein < 110 nmol/min/mg protein Palatinase 8.5 – 22 nmol/min/mg protein < 8.5 nmol/min/mg protein

Palmieri Metabolic Diagnostic Laboratory Reference Ranges by Test

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GALACTOSE -1- PHOSPHATE ANALYSIS Normal < 1.5mg% (mg/dL) Classic Galactosemics on lactose free diet: 1.5mg% – 6 mg% URINE GALACTITOL ANALYSIS Age Range Normal Classic Galactosemia < 1 year < 2 – 78 183 – 800 1 – 6 year < 2 – 36 194 – 620 > 6 year < 2 – 19 98 – 282 GALACTOSEMIA QUANTITATION (GALACTOSE-1-PHOSPHATE URIDYLTRANSFERASE ENZYME ACTIVITY) Normal: 15.9 – 26.4 nmol/hr/g hemoglobin Duarte Heterozygote: 11.8 – 21.1 nmol/hr/g hemoglobin Classic Galactosemia Heterozygote: 6.5 – 12.8 nmol/hr/g hemoglobin Duarte Homozygote: 8.3 – 9.4 nmol/hr/g hemoglobin Duarte/Classic Galactosemia Heterozygote: 3.0 – 5.4 nmol/hr/g hemoglobin Classic Galactosemia: 0.0 – 1.8 nmol/hr/g hemoglobin GALACTOKINASE ENZYME ACTIVITY Normal: 0.85 – 2.09 µmol/hr/g hemoglobin UDP-GALACTOSE-4-EPIMERASE ENZYME ACTIVITY Normal: > 3.0 mmol/hr/g hemoglobin Epimerase Deficient: < 0.5 mmol/hr/g hemoglobin LYSOSOMAL ENZYME ACTIVITY WHITE BLOOD CELL b-GALACTOSIDASE 65 – 120 nmol/hr/mg protein a-GALACTOSIDASE 44 – 75 nmol/hr/mg protein b-HEXOSAMINIDASE 490 – 855 nmol/hr/mg protein PERCENT A 50 – 64 nmol/hr/mg protein a-MANNOSIDASE 103 – 225 nmol/hr/mg protein a-FUCOSIDASE 38 – 73 nmol/hr/mg protein ARYLSULFATASE A 49 – 82 nmol/hr/mg protein b-GLUCOSIDASE 5.5 - 9.7 nmol/hr/mg protein b-MANNOSIDASE 80 – 110 nmol/hr/mg protein a-NEURAMINIDASE 32 – 90 nmol/hr/mg protein SERUM/PLASMA b-GALACTOSIDASE 15 – 55 nmol/hr/mL b-HEXOSAMINIDASE 480 – 860 nmol/hr/mL a-FUCOSIDASE 204 – 825 nmol/hr/mL b-GLUCURONIDASE 110 – 378 nmol/hr/mL METHYLMALONIC ACID ANALYSIS Normal: 50 - 300 nmol/L Methylmalonic Aciduria Patients: > 100,000 nmol/L URINE OROTIC ACID ANALYSIS Age Range Normal < 14 days 2.27 – 4.13 mmol/mol creatinine 15 days – 12 months 1.15 – 3.09 mmol/mol creatinine 13 months – 10 years 1.12 – 2.52 mmol/mol creatinine > 10 year 0.5 – 0.98 mmol/mol creatinine

Palmieri Metabolic Diagnostic Laboratory Reference Ranges by Test

Page 3 of 3

PYRUVATE/LACTATE ANALYSIS Sample Pyruvate Lactate L/P Ratio Blood 0.8 – 2.0mM 0.5 – 0.14mM 10 – 20 CSF 0.7 – 2.0mM 0.5 – 1.17mM 10 – 20 AMINO ACID ANALYSIS Units for Plasma and CSF Samples: µmol/L for Urine samples: µmol/g creatinine NRE = No range established

Amino Acid Plasma 1d-3wk

Plasma 4wk-adult CSF Urine

1d - 6d Urine

7d - 2m Urine

2m - 1y Urine

1y - 3 y Urine

4y - Adult Phosphoserine 10 - 40 8 - 35 5 - 25 200 - 970 300 - 1400 200 - 700 200 - 600 175 - 450

Taurine 49 - 115 55 - 204 NRE 511 - 4010 194 - 2394 116 -2639 168 -2446 66 - 1454

Phosphoethanolamine 5-50 5 - 20 4 - 16 200 - 550 260 - 1200 150 - 375 100 - 350 0 - 150

Aspartic 8-38 0 - 31 0 - 4 75 - 500 150 - 400 150 - 450 50 - 275 0 - 175

Hydroxyproline 0-82 0 - 68 0 - 10 148 - 1287 497 - 1479 107 -1280 0 - 594 0 - 193

Threonine 120-285 86 - 321 17 - 80 149 - 355 175 - 915 130 - 580 79 - 451 44 - 322

Serine 46-253 50 - 215 13 - 80 376 - 1323 737 - 1565 590 -1751 345 - 1200 126 - 585

Asparagine 30-83 19 - 106 0 - 22 21 - 173 27 - 368 84 - 325 27 - 443 23 - 286

Glutamic Acid 55-166 24 - 163 0 - 12 0 - 132 0 - 233 0 - 102 0 - 173 0 - 83

Glutamine 317 - 1051 345 - 1006 228 - 1008 264 - 727 899 - 1813 597 - 1955 170 - 1666 188 - 836

Sarcosine NRE NRE NRE 0 - 286 0 - 371 0 - 221 0 - 260 0 - 260

a-Amino Adipic Acid NRE NRE NRE 0 - 135 0 - 74 0 - 135 0 - 225 0 - 134

Proline 67-270 83 - 346 NRE 0 - 880 0 - 551 0 - 445 0 - 72 NRE

Glycine 192-491 103 - 424 1.2 - 15.7 1026-15250 3539 - 12622 974 - 8046 354 - 6313 388 - 2261

Alanine 120-449 88 - 440 16 - 57 165 - 1120 379 - 1621 258 - 1422 189 - 1370 73 - 604

Citrulline 2 - 38 4 - 46 0 - 3 0 - 54 0 - 141 0 - 110 0 - 84 0 - 52

a-Aminobutyric Acid 0 - 20 0 - 43 0 - 5 0 - 30 0 - 81 0 - 96 0 - 48 0 - 34

Valine 71-236 93 - 321 0 - 34 27 - 235 11 - 326 6 - 246 12 - 227 5 - 130

Cystine 3 - 59 2 - 47 NRE 109 - 530 46 - 654 29 - 308 21 - 127 5 - 130

Methionine 18 - 48 8 - 49 0 - 24 34 - 486 697 - 296 76 - 546 20 - 179 18 - 130

Cystathionine 0 - 12 0 - 12 NRE NRE NRE NRE NRE NRE

Isoleucine 11 - 87 17 - 106 2 - 15 80 - 325 35 - 362 59 - 245 15 -188 17 - 74

Alloisoleucine 0 0 0 0 0 0 0 0

Leucine 29 - 152 42 - 188 20 - 200 20 - 200 20 - 240 30 - 305 20 - 225 10 -110

Tyrosine 32 - 109 22 - 102 4 - 26 32 - 221 113 - 408 135 - 557 71 - 602 73 - 416

Phenylalanine 38 - 85 23 - 95 5 - 25 0 - 61 37 - 363 110 - 309 47 - 259 17 - 100

b-Alanine NRE NRE NRE 0 - 135 0 - 38 0 - 112 0 - 117 0 - 79

b-Amino Isobutyric Acid NRE NRE NRE 0 - 920 0 - 1211 0 - 916 38 - 628 0 - 409

Homocystine 0 0 NRE 0 - 12 0 - 13 0 - 21 0 - 28 0 - 20

GABA NRE NRE NRE 0 - 68 0 - 81 0 - 83 0 - 56 0 - 19

Ethanolamine 0-50 0-50 NRE NRE NRE NRE NRE NRE

Hydroxylysine Pk 1 NRE NRE NRE NRE NRE NRE NRE NRE

Hydroxylysine Pk 2 NRE NRE NRE NRE NRE NRE NRE NRE

Ornithine 26-164 13 - 151 4 - 19 25 - 415 57 - 317 30 - 186 16 - 138 9 - 116

Lysine 67-230 71 - 217 12 - 39 189 - 923 191 - 1198 161 -721 138 - 599 73 - 270

1-Methylhistidine NRE NRE NRE 0 - 1889 0 - 1889 0 - 1889 0 - 1889 0 - 1889

Histidine 37-111 38 - 132 12 - 39 18 - 952 757 - 2744 742 - 2794 491 - 2345 303 - 1820

3-Methylhistidine NRE NRE NRE 81 - 384 75 - 430 142 - 377 134 - 647 93 - 323

Anserine NRE NRE NRE NRE NRE NRE NRE NRE

Carnosine NRE NRE NRE 60 - 350 75 - 650 200 - 800 50 - 400 10 - 200

Arginine 23-120 15 - 120 10 - 47 16 - 104 5 - 155 28 - 126 13 - 95 6 - 37

Tryptophan NRE NRE NRE NRE NRE NRE NRE NRE