The value of simultaneous determination of blood large neutral amino acids and tetrahydrobiopterin metabolites in the diagnosis of atypical hyperphenylalaninemia

Egyptian Liver Journal

Table 6 Correlation between various amino acids and either neopterin or biopterin in the classic and atypical hyperphenylalanemia

nmol/L	Classic PKU, n = 46				Atypical PKU, n = 14				Control, n = 50
	Biopterin		Neopterin		Biopterin		Neopterin		Biopterin		Neopterin
	r	p	r	p	r	p	r	p	r	p	r	p
Cit	0.01	0.97	− 0.01	0.96	− 0.10	0.73	− 0.14	0.63	0.09	0.56	0.14	0.33
Orn	− 0.29*	0.04	− 0.21	0.17	− 0.38	0.18	− 0.34	0.23	0.05	0.74	− 0.08	0.59
Met	0.07	0.63	0.12	0.44	− 0.15	0.62	− 0.07	0.82	0.11	0.46	0.08	0.56
Arg	− 0.21	0.17	− 0.35*	0.02	− 0.28	0.33	− 0.19	0.51	0.29*	0.04	0.20	0.16
Val	0.00	0.98	− 0.12	0.44	− 0.04	0.90	− 0.22	0.46	0.16	0.26	0.10	0.48
Leu	0.17	0.27	0.08	0.61	− 0.16	0.59	− 0.10	0.74	− 0.27	0.06	− 0.23	0.11

Spearman correlation between neopterin/biopterin and LNAAs
r Spearman coefficient, PKU phenylketonuria, Tyr tyrosine, PT phenylalanine to tyrosine ratio, Cit citrulline, Orn ornethine, Met methionine, Age arginine, Val valine, Leu leucine
*P-value < 0.01 indicates a highly significant correlation