X
Search Filters
Format Format
Format Format
X
Sort by Item Count (A-Z)
Filter by Count
Journal Article (1734) 1734
Publication (117) 117
Book Chapter (29) 29
Book Review (12) 12
Magazine Article (7) 7
Dissertation (2) 2
Book / eBook (1) 1
Conference Proceeding (1) 1
Reference (1) 1
more...
Subjects Subjects
Subjects Subjects
X
Sort by Item Count (A-Z)
Filter by Count
humans (1277) 1277
maple syrup urine disease (616) 616
infant, newborn (496) 496
female (493) 493
male (486) 486
syrup-urine-disease (364) 364
infant (360) 360
index medicus (356) 356
maple syrup urine disease - diagnosis (302) 302
child (292) 292
pediatrics (277) 277
animals (251) 251
child, preschool (239) 239
maple syrup urine disease - genetics (222) 222
maple syrup urine disease - metabolism (200) 200
genetics & heredity (185) 185
biochemistry & molecular biology (161) 161
metabolism (156) 156
maple syrup urine disease - therapy (152) 152
endocrinology & metabolism (140) 140
amino acids (131) 131
maple syrup urine disease - blood (129) 129
leucine (128) 128
adult (126) 126
mutation (126) 126
rats (122) 122
neurosciences (120) 120
3-methyl-2-oxobutanoate dehydrogenase (119) 119
maple syrup urine disease - enzymology (115) 115
maple syrup urine disease - complications (112) 112
maple syrup urine disease - diet therapy (107) 107
metabolic diseases (105) 105
leucine - blood (98) 98
adolescent (97) 97
branched chain amino acids (97) 97
brain (96) 96
diagnosis (95) 95
medicine, research & experimental (92) 92
phenylketonuria (91) 91
leucine - metabolism (90) 90
multienzyme complexes - genetics (90) 90
urine (90) 90
children (89) 89
medicine & public health (89) 89
ketone oxidoreductases - genetics (86) 86
molecular sequence data (85) 85
amino acids, branched-chain - metabolism (81) 81
base sequence (81) 81
metabolism, inborn errors - diagnosis (80) 80
pregnancy (80) 80
biochemistry, general (79) 79
maple syrup urine disease - pathology (79) 79
analysis (78) 78
amino acids - blood (76) 76
physiological aspects (74) 74
inborn-errors (71) 71
oxidative stress (70) 70
cells, cultured (69) 69
internal medicine (68) 68
amino acid metabolism, inborn errors - diagnosis (67) 67
diet therapy (63) 63
phenylketonurias - diagnosis (63) 63
amino acids, branched-chain - blood (62) 62
brain - pathology (62) 62
inborn errors of metabolism (62) 62
branched-chain amino acids (61) 61
clinical neurology (61) 61
isoleucine - blood (59) 59
research (59) 59
keto acids - metabolism (57) 57
time factors (57) 57
genetic aspects (56) 56
isoleucine (56) 56
magnetic resonance imaging (56) 56
syrup urine disease (56) 56
human genetics (55) 55
mass spectrometry (54) 54
rats, wistar (54) 54
brain - metabolism (53) 53
infants (53) 53
maple syrup urine disease - physiopathology (53) 53
metabolic disorders (53) 53
msud (53) 53
neurology (52) 52
article (51) 51
kinetics (51) 51
biomedicine (50) 50
diagnosis, differential (50) 50
radiology, nuclear medicine & medical imaging (50) 50
chain amino-acids (49) 49
diet (49) 49
transplantation (49) 49
amino-acids (48) 48
mice (48) 48
multienzyme complexes - metabolism (48) 48
fibroblasts - enzymology (47) 47
care and treatment (46) 46
enzymes (46) 46
liver (46) 46
valine - blood (46) 46
more...
Library Location Library Location
Language Language
Language Language
X
Sort by Item Count (A-Z)
Filter by Count
English (1529) 1529
German (83) 83
French (37) 37
Spanish (29) 29
Italian (20) 20
Japanese (20) 20
Polish (13) 13
Chinese (8) 8
Portuguese (5) 5
Russian (4) 4
Dutch (3) 3
Czech (2) 2
Hebrew (2) 2
Hungarian (2) 2
Norwegian (2) 2
Turkish (2) 2
Danish (1) 1
Finnish (1) 1
Romanian (1) 1
Serbian (1) 1
Swedish (1) 1
more...
Publication Date Publication Date
Click on a bar to filter by decade
Slide to change publication date range


Transplantation, ISSN 0041-1337, 03/2019, Volume 103, Issue 3, pp. 536 - 543
BACKGROUND.Data describing the technical aspects of living donor (LD) domino liver transplantation (DLT) in maple syrup urine disease (MSUD) are limited. The... 
SURGERY | CASE SERIES | IMMUNOLOGY | TRANSPLANTATION | Case studies | Maple syrup urine disease | Usage | Transplantation | Research | Liver
Journal Article
International Journal of Developmental Neuroscience, ISSN 0736-5748, 11/2019, Volume 78, pp. 222 - 226
Journal Article
Chemistry Letters, ISSN 0366-7022, 6/2015, Volume 44, Issue 6, pp. 812 - 814
Keto acids play important roles in vivo. In this paper, a novel one-pot keto acid detection system has been developed by the combination of the oxime-click... 
SYRUP-URINE-DISEASE | REAGENT | CHEMISTRY | FLUORESCENT | CHEMISTRY, MULTIDISCIPLINARY | PROBES
Journal Article
Liver Transplantation, ISSN 1527-6465, 06/2019, Volume 25, Issue 6, pp. 827 - 828
Liver transplantation (LT) for various metabolic diseases, which in most cases have a structurally normal liver, has increased the interest in the use of... 
SURGERY | GASTROENTEROLOGY & HEPATOLOGY | TRANSPLANTATION
Journal Article
Archives of Disease in Childhood, ISSN 0003-9888, 05/2019, Volume 104, Issue Suppl 2, p. A106
AimsMSUD is an inherited metabolic condition, caused by BCKD (branched-chain ketoacid dehydrogenase) complex deficiency. Although rare, mimicry of presentation... 
Urine | Neonates | Demography | Maple syrup urine disease | Leucine | Metabolism | Medical screening | Mimicry | Neurological complications | Encephalopathy | Children | Diagnosis | Age
Journal Article
Experimental and Clinical Transplantation, ISSN 1304-0855, 12/2016, Volume 14, Issue 6, pp. 691 - 692
Maple syrup urine disease (MSUD), an inherited autosomal recessive disorder of branched-chain amino acid metabolism, is characterized by potentially... 
SYRUP-URINE-DISEASE | TRANSPLANTATION | T?p
Journal Article
Journal of Pediatric Endocrinology and Metabolism, ISSN 0334-018X, 01/2019, Volume 32, Issue 1, pp. 83 - 84
Journal Article
Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, ISSN 1003-9406, 10/2018, Volume 35, Issue 5, pp. 679 - 682
Journal Article
Analytical Chemistry (Washington), ISSN 0003-2700, 03/2016, Volume 88, Issue 5, pp. 2871 - 2871
We developed a whole-cell surface plasmon resonance (SPR) sensor based on a leucine auxotroph of Escherichia coli displaying a gold-binding protein (GBP) in... 
Proteins | Escherichia coli | Maple syrup urine disease | Amino acids | Plasmons | Diagnosis | Sensors | Leucine
Journal Article
The Journal of Maternal-Fetal & Neonatal Medicine, ISSN 1476-7058, 06/2018, Volume 31, Issue 12, pp. 1663 - 1666
Maple syrup urine disease (MSUD) is an autosomal metabolic condition that can cause significant morbidity in pregnant women and their infants. Advances in... 
metabolic disease | Obstetrics | maple syrup urine disease | pregnancy | DIAGNOSIS | MANAGEMENT | WOMAN | OBSTETRICS & GYNECOLOGY
Journal Article
Liver Transplantation, ISSN 1527-6465, 06/2019, Volume 25, Issue 6, pp. 889 - 900
Liver transplantation (LT) has been shown to be a feasible treatment in patients with severe forms of maple syrup urine disease (MSUD). Because of a sufficient... 
SURGERY | GASTROENTEROLOGY & HEPATOLOGY | CHILDREN | TRANSPLANTATION
Journal Article