Table 2.

Summary of pre and post fast plasma metabolites and hormones in 6 children with type II SMA after a 20 hour fast

	Fed State (Pre Fast)					Fasted State (Post Fast)
Metabolite/hormone	median	Q1	Q3	Min	Max	median	Q1	Q3	Min	Max	P valueA
Glucose (mg/dL)	111.5	102	118	98.9	130	79.9	74.8	85.8	74.1	86	0.03*
Insulin (uIU/mL)	119	49	242	12	254	9.5	8	13	4	17	0.03*
Cortisol (ug/dL)	6.9	1.8	12	1.6	14.1	4.7	4.5	4.9	3.6	9.7	0.56
Free fatty acids(mmol/L)	0.24	0.14	0.37	0.12	0.65	0.94	0.93	1.07	0.91	1.39	0.03*
Glutamine (umol/L)	421.5	348	500	273	562	397	356	519	317	531	1
Alanine (umol/L)	508	378	723	314	773	185	167	240	151	275	0.03*
Arginine (umol/L)	97	87	104	84	134	52.5	49	66	39	70	0.03*
Asparagine (umol/L)	44	39	63	34	67	26.5	23	34	23	39	0.03*
Citrulline (umol/L)	25.5	15	35	14	39	17.5	17	21	16	26	0.1
Cystine (umol/L)	29.5	23	34	19	42	35.5	32	39	24	41	0.31
Glutamic acid (umol/L)	69	40	93	26	110	44	19	84	13	85	0.03*
Glycine (umol/L)	197	172	245	158	257	183.5	174	272	159	309	0.68
Histidine (umol/L)	72.5	67	77	57	87	62.5	59	69	58	88	0.43
Isoleucine (umol/L)	104	94	122	43	211	51.5	41	64	35	67	0.03*
Methionine (umol/L)	38.5	21	42	17	43	14	14	15	12	20	0.03*
Leucine (umol/L)	171	146	208	73	335	95	87	103	59	110	0.03*
Lysine (umol/L)	204.5	153	226	123	289	107	97	111	85	148	0.03*
Ornithine (umol/L)	57	52	77	36	88	29	25	35	21	37	0.03*
Phenylalanine (umol/L)	80	58	87	52	88	40.5	38	50	30	51	0.03*
Proline (umol/L)	331	241	444	213	533	99.5	96	119	86	126	0.03*
Serine (umol/L)	134.5	121	144	109	147	117	106	128	100	130	0.07
Taurine (umol/L)	39	34	51	33	54	45.5	34	57	33	58	0.32
Threonine (umol/L)	135	111	165	93	182	64.5	59	85	45	104	0.03*
Tryptophan (umol/L)	54.5	43	69	39	79	42	31	45	24	46	0.06
Tyrosine (umol/L)	113.5	65	125	52	127	35	33	43	29	52	0.03*
Valine (umol/L)	376.5	276	415	156	602	171.5	158	206	114	249	0.03*

^AWilcoxon Sign Rank test pre and post fasting

^*P <0.05