Table 1.

Diagnostic parameters and pancreatitis associated protein (PAP) measurements in patients with 3-hydroxy-3-methylglutaric aciduria

Case	Organic acids	HMGCL mutation	PAP (ng/mL)	Reference
P1	+	c.109G>T	6.72	Casale et al. 1998
P2	+	c.109G>T	8.49	Casale et al. 1998
P3	+	c.109G>T/c504_5delCT	20.12a	Menao et al. 2009
			31.87
P4	+	c.144G>T/c.504_5delCT	5.61	Menao et al. 2009
P5	+	c.202_207delCT	9.42	Menao et al. 2009
P6	+	c.575T>C	6.17	Menao et al. 2009
P7	+	c.109G>T	5.39	Menao et al. 2009
P8	+	c.109G>T	7.85	Menao et al. 2009
P9	+	c.109G>T/c.825C>G	5.80a	-----
			7.37
P10	+	c.109G>T	2.13a	Menao et al. 2009
P11	+	c.109G>T/c.504_5delCT	5.35a	-----
P12	+	c.242G>A	5.93a	-----
P13	+	-----	13.63	-----
P14	+	-----	26.92a	-----
			10.46
P15	+	-----	6.16	-----

PAP values in controls (17.46 ± 8.27 ng/ml, mean ± SD, n = 11)

+ Elevated organic acids (3-hydroxy-3-methylglutaric, 3-methylglutaconic, 3-methylglutaric and 3-hydroxy-isovaleric); ----- no data; PAP values measured in periods intercrisis and

^ain acute crisis. PAP values < 50 ng/mL are considered normal