Research Article

Propionic and Methylmalonic Acidemias: Initial Clinical and Biochemical Presentation

Table 1

Laboratory parameters at initial presentation.

Lab. parameterRange (SI)Normal reference range

pH7.23-7.437.35-7.45
HCO34-25 mmol/l22-26
Anion gap10-3912-15
Ammonia45-212μmol/l9-33 μmol/l
Lactate1.1-6.5 mmol/l1-2.2 mmol/l
Blood glucose67-250 (3.7-13.8 mmol/l) mg/dl ( mmol/l)70-126 mg/dl
Uric acid2.5-11.2 (148.71-666.23 μmol/l) mg/dl (μmol/l)3.5-4.5 mg/dl
Glycine77.5-721μmol/l20-500 μmol/l
Alanine99.56-401μmol/l20-600 μmol/l
C39.98-27.7μmol/l0-7 μmol/l
C3 : C20.31-1.68μmol/l0-0.25 μmol/l
Urea12-50 (1.99-8.3 mmol/l) mg/dl ( mmol/l)10-45 mg/dl
Creatinine0.4-0.8 (35.37-70.74 μmol/l) mg/dl (μmol/l)0.6-1 mg/dl
AST17-74 U/l20-45
ALT20-69 U/l20-45
Albumin29-45 g/l35-50 g/dl
Hemoglobin87-120 g/l g/l
Platelets157,000-470,000
White blood cells2700-70003,500-5000/mm3