TABLE 3.

Summary of biochemical findings in CD patients from clinical reports using sodium pyruvate as a treatment.

Patient age (years)	NaPy dose (g/day)/duration (days)	Biochemical findings a
		Improved but not normalized	Normalized	Unchanged	Worsened
Mutoh et al. 37 (Phenotype: AACD)
13	6/1095	T/S	TG, PSTI	T. bil, AST, ALT, Amy, T. chol, HDL‐C, BUN, UA, NH3, TP, Lac, Pyr, Na+, Cit, Ala, FR	Arg, Gln
Yazaki et al. 34 (Phenotype: AACD)
34	3–15/77	Exact values not provided (results in graph format)
41	3/23
Yazaki et al. 58 (Phenotype: AACD)
72	3–9/121	ALB, Arg, FR	RBC, Hb, TP, T. chol, ALP, GGT, Amy, PSTI	WBC, PLT, Cr, TG, ALT, LDH, T. bil, prothrombin time, activated partial prothromboplasmin time, NH3	BUN, AST, Cit
Kogure et al. 44 (Phenotype: AACD)
68	4.5–8/1825	Cit	NH3, PSTI
Nagasaka et al. 45 (Phenotype: silent period) b
3–13	300 c /91–182		Carn	Citr, ICitr, ɑ‐KG, ACarn, BHB, FFA, FBG, HbA1c, Lac, Pyr, L/P	Suc, Fum, Mal, FI

Abbreviations: α‐KG, alpha‐ketoglutarate; AACD, adolescent and adult citrin deficiency (formerly CTLN2); ACarn, acetylcarnitine; Ala, alanine; ALB, albumin; ALP, alkaline phosphatase; ALT, alanine aminotransferase; Amy, amylase; Arg, arginine; aPTT, activated partial thromboplastin time; ASS, argininosuccinate synthetase; AST, aspartate aminotransferase; BHB, β‐hydroxybutyrate; BUN, blood urea nitrogen; Carn, carnitine; CD, citrin deficiency; ChE, cholinesterase; Citr, citrate; Cit, citrulline; Cr, creatinine; D. bil, direct bilirubin; FBG, fasting blood glucose; Fe, iron; FFA, free fatty acids; FI, fasting insulin: FIB, fibrinogen; FR, fischer ratio; Fum, fumarate; Gln, glutamine; GGT, gamma‐glutamyl transpeptidase; Glu, glutamate; GS, glutamine synthetase; HE, hpatic encephalopathy; Hb, hemoglobin; HbA1c, hemoglobin A1c; HDL‐C, high‐density lipoprotein cholesterol; HYA, hyaluronic acid; ICitr, isocitrate; L/P, lactate to pyruvate ratio; Lac, lactate; LDH, lactate dehydrogenase; Mal, malate; MCT, medium chain triglycerides; Met, methionine; Na+, sodium ions; NaPy, sodium pyruvate; NICCD, neonatal intrahepatic cholestasis caused by citrin deficiency; NH3, ammonia; OGTT, oral glucose tolerance test; PSTI, pancreatic secretory trypsin inhibitor; PLT, platelet count; Pyr, pyruvate; RBC, red blood cell count; Suc, succinate; T/S, threonine to serine ratio; TG, triglycerides; T. bil, total bilirubin; T. chol, total cholesterol; TBA, total bile acid; TCA, tricarboxylic acid; Thr, threonine; TIBC, total iron‐binding capacity; TP, total protein; Tyr, tyrosine; UA, uric acid; WBC, white blood cell count.

^a Based on reference ranges provided in citations.

^b Reference ranges not provided. Data are compared against control values obtained from healthy volunteers.

^c Units in mg/kg/day.