-
1
-
-
0035142619
-
Current strategies for the management of neonatal urea cycle disorders
-
M. Summar Current strategies for the management of neonatal urea cycle disorders J. Pediatr. 138 2001 S30 S39
-
(2001)
J. Pediatr.
, vol.138
-
-
Summar, M.1
-
2
-
-
0035139967
-
Proceedings of a consensus conference for the management of patients with urea cycle disorders
-
M. Summar, and M. Tuchman Proceedings of a consensus conference for the management of patients with urea cycle disorders J. Pediatr. 138 2001 S6 S10
-
(2001)
J. Pediatr.
, vol.138
-
-
Summar, M.1
Tuchman, M.2
-
3
-
-
0025874776
-
Hyperammonaemia does not impair brain function in the absence of net glutamine synthesis
-
R.A. Hawkins, and J. Jessy Hyperammonaemia does not impair brain function in the absence of net glutamine synthesis Biochem. J. 277 Pt. 3 1991 697 703
-
(1991)
Biochem. J.
, vol.277
, Issue.3 PART
, pp. 697-703
-
-
Hawkins, R.A.1
Jessy, J.2
-
4
-
-
0026665643
-
Plasma glutamine concentration: A guide in the management of urea cycle disorders
-
N.E. Maestri, K.D. McGowan, and S.W. Brusilow Plasma glutamine concentration: a guide in the management of urea cycle disorders J. Pediatr. 121 1992 259 261
-
(1992)
J. Pediatr.
, vol.121
, pp. 259-261
-
-
Maestri, N.E.1
McGowan, K.D.2
Brusilow, S.W.3
-
5
-
-
0026004760
-
Inhibition of brain glutamine accumulation prevents cerebral edema in hyperammonemic rats
-
H. Takahashi, R.C. Koehler, S.W. Brusilow, and R.J. Traystman Inhibition of brain glutamine accumulation prevents cerebral edema in hyperammonemic rats Am. J. Physiol. 261 1991 H825 H829
-
(1991)
Am. J. Physiol.
, vol.261
-
-
Takahashi, H.1
Koehler, R.C.2
Brusilow, S.W.3
Traystman, R.J.4
-
6
-
-
0035140623
-
Long-term management of patients with urea cycle disorders
-
G.T. Berry, and R.D. Steiner Long-term management of patients with urea cycle disorders J. Pediatr. 138 2001 S56 S60
-
(2001)
J. Pediatr.
, vol.138
-
-
Berry, G.T.1
Steiner, R.D.2
-
7
-
-
0035145675
-
The nutritional management of urea cycle disorders
-
J.V. Leonard The nutritional management of urea cycle disorders J. Pediatr. 138 2001 S40 S44
-
(2001)
J. Pediatr.
, vol.138
-
-
Leonard, J.V.1
-
8
-
-
0020057470
-
Treatment of inborn errors of urea synthesis: Activation of alternative pathways of waste nitrogen synthesis and excretion
-
M.L. Batshaw, S. Brusilow, L. Waber, W. Blom, A.M. Brubakk, B.K. Burton, H.M. Cann, D. Kerr, P. Mamunes, R. Matalon, D. Myerberg, and I.A. Schafer Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion N. Engl. J. Med. 306 1982 1387 1392
-
(1982)
N. Engl. J. Med.
, vol.306
, pp. 1387-1392
-
-
Batshaw, M.L.1
Brusilow, S.2
Waber, L.3
Blom, W.4
Brubakk, A.M.5
Burton, B.K.6
Cann, H.M.7
Kerr, D.8
Mamunes, P.9
Matalon, R.10
Myerberg, D.11
Schafer, I.A.12
-
9
-
-
0025157218
-
A new case of hyperargininaemia: Neurological and biochemical findings prior to and during dietary treatment
-
M. Brockstedt, L.M. Smit, A.J. de Grauw, J.M. van der Klei-van Moorsel, and C. Jakobs A new case of hyperargininaemia: neurological and biochemical findings prior to and during dietary treatment Eur. J. Pediatr. 149 1990 341 343
-
(1990)
Eur. J. Pediatr.
, vol.149
, pp. 341-343
-
-
Brockstedt, M.1
Smit, L.M.2
De Grauw, A.J.3
Van Der Klei-van Moorsel, J.M.4
Jakobs, C.5
-
12
-
-
0029614698
-
Long-term survival of patients with argininosuccinate synthetase deficiency
-
N.E. Maestri, D.B. Clissold, and S.W. Brusilow Long-term survival of patients with argininosuccinate synthetase deficiency J. Pediatr. 127 1995 929 935
-
(1995)
J. Pediatr.
, vol.127
, pp. 929-935
-
-
Maestri, N.E.1
Clissold, D.B.2
Brusilow, S.W.3
-
14
-
-
0036834458
-
Dietary reference intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids
-
P. Trumbo, S. Schlicker, A.A. Yates, and M. Poos Dietary reference intakes for energy, carbohydrate, fiber, fat, fatty acids, cholesterol, protein and amino acids J. Am. Diet. Assoc. 102 2002 1621 1630
-
(2002)
J. Am. Diet. Assoc.
, vol.102
, pp. 1621-1630
-
-
Trumbo, P.1
Schlicker, S.2
Yates, A.A.3
Poos, M.4
-
15
-
-
5344249425
-
Nutrient intakes and physical growth of children with phenylketonuria undergoing nutrition therapy
-
P.B. Acosta, S. Yannicelli, R.H. Singh, S. Mofidi, R. Steiner, E. DeVincentis, E. Jurecki, L. Bernstein, S. Gleason, M. Chetty, and B. Rouse Nutrient intakes and physical growth of children with phenylketonuria undergoing nutrition therapy J. Am. Diet. Assoc. 103 2003 1167 1173
-
(2003)
J. Am. Diet. Assoc.
, vol.103
, pp. 1167-1173
-
-
Acosta, P.B.1
Yannicelli, S.2
Singh, R.H.3
Mofidi, S.4
Steiner, R.5
Devincentis, E.6
Jurecki, E.7
Bernstein, L.8
Gleason, S.9
Chetty, M.10
Rouse, B.11
-
16
-
-
0022881229
-
Use and interpretation of anthropometric indicators of nutritional status
-
WHO Working Group. Use and interpretation of anthropometric indicators of nutritional status. Bull. World Health Organ 64 (1986) 929-941.
-
(1986)
Bull. World Health Organ
, vol.64
, pp. 929-941
-
-
-
17
-
-
0024496675
-
Laboratory tests and nutritional assessment: Protein-energy status
-
D.R. Benjamin Laboratory tests and nutritional assessment: protein-energy status Pediatr Clin. North Am. 36 1989 139 161
-
(1989)
Pediatr Clin. North Am.
, vol.36
, pp. 139-161
-
-
Benjamin, D.R.1
-
18
-
-
0014035240
-
The determination of tryptophan in plasma, liver, and urine
-
W.D. Denckla, and H.K. Dewey The determination of tryptophan in plasma, liver, and urine J. Lab. Clin. Med. 69 1967 160 169
-
(1967)
J. Lab. Clin. Med.
, vol.69
, pp. 160-169
-
-
Denckla, W.D.1
Dewey, H.K.2
-
19
-
-
0016168683
-
Error in the determination of tryptophan by the method of Denckla and Dewey. a revised procedure
-
D.L. Bloxam, and W.H. Warren Error in the determination of tryptophan by the method of Denckla and Dewey. A revised procedure Anal. Biochem. 70 1974 621 625
-
(1974)
Anal. Biochem.
, vol.70
, pp. 621-625
-
-
Bloxam, D.L.1
Warren, W.H.2
-
23
-
-
0027397949
-
Prospective management of a child with neonatal citrullinemia
-
A.R. Melnyk, R. Matalon, B.W. Henry, W.P. Zeller, and C. Lange Prospective management of a child with neonatal citrullinemia J. Pediatr. 122 1993 96 98
-
(1993)
J. Pediatr.
, vol.122
, pp. 96-98
-
-
Melnyk, A.R.1
Matalon, R.2
Henry, B.W.3
Zeller, W.P.4
Lange, C.5
-
24
-
-
0030883916
-
Argininemia. a treatable genetic cause of progressive spastic diplegia simulating cerebral palsy. Case reports and literature review
-
A.N. Prasad, J.C. Breen, M.G. Ampola, and P. Rosman Argininemia. A treatable genetic cause of progressive spastic diplegia simulating cerebral palsy. Case reports and literature review J. Child Neurol. 12 1997 301 309
-
(1997)
J. Child Neurol.
, vol.12
, pp. 301-309
-
-
Prasad, A.N.1
Breen, J.C.2
Ampola, M.G.3
Rosman, P.4
-
25
-
-
0142089740
-
Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food
-
S. Yannicelli, P.B. Acosta, A. Velazquez, H.G. Bock, B. Marriage, T.W. Kurczynski, M. Miller, M. Korson, R.D. Steiner, and L. Rutledge Improved growth and nutrition status in children with methylmalonic or propionic acidemia fed an elemental medical food Mol. Genet. Metab. 80 2003 181 188
-
(2003)
Mol. Genet. Metab.
, vol.80
, pp. 181-188
-
-
Yannicelli, S.1
Acosta, P.B.2
Velazquez, A.3
Bock, H.G.4
Marriage, B.5
Kurczynski, T.W.6
Miller, M.7
Korson, M.8
Steiner, R.D.9
Rutledge, L.10
-
26
-
-
1642424401
-
Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients
-
F. Scaglia, S. Carter, W.E. O'Brien, and B. Lee Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients Mol. Genet. Metab. 81 Suppl. 1 2004 S79 S85
-
(2004)
Mol. Genet. Metab.
, vol.81
, Issue.1 SUPPL.
-
-
Scaglia, F.1
Carter, S.2
O'Brien, W.E.3
Lee, B.4
-
27
-
-
0016312163
-
Relevance of free tryptophan in serum to tissue tryptophan concentrations
-
B.K. Madras, E.L. Cohen, R. Messing, H.N. Munro, and R.J. Wurtman Relevance of free tryptophan in serum to tissue tryptophan concentrations Metabolism 23 1974 1107 1116
-
(1974)
Metabolism
, vol.23
, pp. 1107-1116
-
-
Madras, B.K.1
Cohen, E.L.2
Messing, R.3
Munro, H.N.4
Wurtman, R.J.5
-
29
-
-
0019364765
-
Therapy of urea cycle enzymopathies: Three case studies
-
M.L. Batshaw, M.J. Painter, G.T. Sproul, I.A. Schafer, G.H. Thomas, and S. Brusilow Therapy of urea cycle enzymopathies: three case studies Johns Hopkins Med. J. 148 1981 34 40
-
(1981)
Johns Hopkins Med. J.
, vol.148
, pp. 34-40
-
-
Batshaw, M.L.1
Painter, M.J.2
Sproul, G.T.3
Schafer, I.A.4
Thomas, G.H.5
Brusilow, S.6
-
30
-
-
0018895662
-
Neonatal argininosuccinic aciduria-survival after early diagnosis and dietary management
-
F.S. Collins, G.K. Summer, R.P. Schwartz, and J.C. Parke Neonatal argininosuccinic aciduria-survival after early diagnosis and dietary management J. Pediatr. 96 1980 429 431
-
(1980)
J. Pediatr.
, vol.96
, pp. 429-431
-
-
Collins, F.S.1
Summer, G.K.2
Schwartz, R.P.3
Parke, J.C.4
-
31
-
-
0026448020
-
Intercurrent illness in inborn errors of intermediary metabolism
-
M.A. Dixon, and J.V. Leonard Intercurrent illness in inborn errors of intermediary metabolism Arch. Dis. Child. 67 1992 1387 1391
-
(1992)
Arch. Dis. Child.
, vol.67
, pp. 1387-1391
-
-
Dixon, M.A.1
Leonard, J.V.2
-
32
-
-
0021041397
-
Hyperargininemia: Clinical course and treatment with sodium benzoate and phenylacetic acid
-
N. Mizutani, M. Maehara, C. Hayakawa, T. Kato, K. Watanabe, and S. Suzuki Hyperargininemia: clinical course and treatment with sodium benzoate and phenylacetic acid Brain Dev. 5 1983 555 563
-
(1983)
Brain Dev.
, vol.5
, pp. 555-563
-
-
Mizutani, N.1
Maehara, M.2
Hayakawa, C.3
Kato, T.4
Watanabe, K.5
Suzuki, S.6
|