Abstract:
:Kinetic data are presented for the decarboxylation of branched chain alpha-ketoacids (BCKA) by intact human fibroblasts. Cultured cells of normal individuals and nine patients with different clinical pictures of maple syrup urine disease (MSUD) are studied with both alpha-ketoisocaproic acid (2-oxo-4-methylpentanoic acid (KIC)) and alpha-ketoisovaleric acid (2-oxo-3-methylbutanoic acid (KIVA)) as substrates. One normal cell strain and one patient cell strain is analyzed with alpha-keto-beta-methyl-n-valeric acid (2-oxo-3-methylpentanoic acid (MEVA)) as a substrate. A biphasic degradation kinetic for each BCKA is obtained for normal control subjects. The component with higher substrate affinity is affected in MSUD: for KIC the normally hyperbolic substrate curve is changed to sigmoid shape, for KIVA and MEVA as substrates this component is not detectable at all. Considering qualitative aspects of the BCKA decarboxylation kinetics intact fibroblasts yield the same results as our recent studies with the decarboxylase moieties of partially purified kidney BCKA dehydrogenase of normal individuals and one patient with classic MSUD (27). The decarboxylation velocities for normal and patient fibroblasts with one exception differ widely at low but not at high substrate concentrations of BCKA. To get meaningful data on the residual substrate degradation activities with intact fibroblasts of different phenotypes of MSUD physiologically low substrate concentrations are required in the assay.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Wendel U,Wentrup H,Rüdiger HWdoi
10.1203/00006450-197509000-00005subject
Has Abstractpub_date
1975-09-01 00:00:00pages
709-17issue
9eissn
0031-3998issn
1530-0447journal_volume
9pub_type
杂志文章abstract::To examine the levels and relationship of blood pressure and insulin during puberty, blood pressure and serum insulin were measured in 3596 subjects, aged 3-18 y, whose pubertal status was graded according to the Tanner classification. The same study protocol was repeated in two follow-up surveys 3 and 6 y later for 2...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1203/00006450-199702000-00019
更新日期:1997-02-01 00:00:00
abstract::Bacillus Calmette-Guerin (BCG) vaccination can protect animals from asthma, but the effect of BCG on childhood asthma prevention is controversial in humans. To verify the hypothesis that the BCG anti-asthma effect in childhood might be reversed by a respiratory virus infection, newborn BALB/c mice were divided into fi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000196368.08210.5c
更新日期:2006-02-01 00:00:00
abstract::Because minimal information is available about surfactant metabolism in bronchopulmonary dysplasia, we measured half-lives and pool sizes of surfactant phosphatidylcholine in very preterm baboons recovering from respiratory distress syndrome and developing bronchopulmonary dysplasia, using stable isotopes, radioactive...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200211000-00019
更新日期:2002-11-01 00:00:00
abstract::Growth hormone (GH) regulates growth and development in the postnatal period but lacks somatotropic activity in the fetus. In contrast, the placental hormone placental lactogen (PL) stimulates amino acid transport, DNA synthesis, and somatomedin production in isolated fetal tissues, suggesting that PL may function as ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199208000-00015
更新日期:1992-08-01 00:00:00
abstract::An association between chorioamnionitis and periventricular leukomalacia has been reported in human preterm infants. However, whether this link is causal has not been convincingly established, and the underlying molecular mechanisms remain unclear. The objective of this study was to establish a reproducible model of c...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200006000-00009
更新日期:2000-06-01 00:00:00
abstract::The pathophysiology of kwashiorkor, a severe edematous manifestation of malnutrition, is still poorly understood. The syndrome is, however, known to be associated with alterations in redox metabolism. To further elucidate the role of oxidative stress in kwashiorkor, we carried out a longitudinal study on the major blo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200102000-00018
更新日期:2001-02-01 00:00:00
abstract::Pups delivered by rats fed during pregnancy a choline-deficient (CD) diet containing 1% N,N-dimethylaminoethanol (DME) die within 36 hr of birth. The concentrations of sphingomyelins, phosphatidyl cholines, and disaturated phosphatidyl cholines in the lungs of these pups are lower than those in the lungs of pups deliv...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197809000-00014
更新日期:1978-09-01 00:00:00
abstract::Several lines of evidence suggest that formula with protein content of 3.0 g/100 kcal does not fully meet the protein needs of very-low-birth weight infants. Our purpose was to compare nitrogen balance, metabolic status and growth in infants fed a standard (3.0 g/100 kcal; RegPro) and high (3.6 g/100 kcal; HiPro) prot...
journal_title:Pediatric research
pub_type: 杂志文章,随机对照试验
doi:10.1203/01.pdr.0000196376.99101.34
更新日期:2006-02-01 00:00:00
abstract::Ambulatory blood pressure (ABP) monitoring offers a reliable method for determining blood pressure (BP) in children. The aim of this cohort study was to examine whether maternal preeclampsia is associated with elevated BP in an offspring. The study population consisted of 57 children born to preeclamptic mothers (PRE)...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000196734.54473.e3
更新日期:2006-02-01 00:00:00
abstract::A number of neuromuscular diseases are associated with molecular defects in the mitochondrial DNA (mtDNA). These include: 1) a missense mutation at nucleotide 11778 in the mtDNA of Leber's hereditary optic neuropathy patients; 2) a heterogeneous array of deletions in the mtDNA of ocular myopathy patients; and 3) small...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199011000-00023
更新日期:1990-11-01 00:00:00
abstract::The carbohydrate-deficient glycoprotein syndromes are a recently individualized group of genetic multisystemic disorders. A predominant feature is a severe involvement of the central and peripheral nervous system resulting in psychomotor retardation, seizures, ataxia, and, mostly after infancy, stroke-like episodes. T...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199305000-00024
更新日期:1993-05-01 00:00:00
abstract::The neuroprotective effect of hypothermia instituted after resuscitation from asphyxic cardiac arrest has not been studied in immature brain, particularly in a large animal model with recovery periods greater than 4 d. Moreover, protection from severe hypoxia seen with 3 h of hypothermia was reported to be lost when h...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000072783.22373.FF
更新日期:2003-08-01 00:00:00
abstract:BACKGROUND:To investigate relationships between infant body composition (BC) and human milk (HM) immunomodulatory proteins (IMPs) during the first 12 months of lactation. METHODS:BC of breastfeeding dyads (n = 20) was measured with ultrasound skinfolds (infants) and bioimpedance spectroscopy (infants/mothers) at 2, 5,...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0961-z
更新日期:2020-05-21 00:00:00
abstract::Infants dying with bronchopulmonary dysplasia (BPD) demonstrate increased numbers of pulmonary neuroendocrine cells (PNEC). These infants also possess altered airway epithelial and smooth muscle dimensions reminiscent of oxygen-exposed animals. Because the pathogenesis of BPD involves oxygen toxicity, we hypothesized ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199710000-00020
更新日期:1997-10-01 00:00:00
abstract::Infants with sudden infant death syndrome have higher hypoxanthine (Hx) concentrations in their vitreous humor than infants with respiratory distress syndrome and other infant control populations. However, previous research on piglets and pigs applying continuous hypoxemia has not been able to reproduce the concentrat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199312000-00013
更新日期:1993-12-01 00:00:00
abstract::Pesticides are chemicals that are designed specifically for the purpose of killing or suppressing another living organism. Human toxicity is possible with any pesticide, and a growing body of literature has investigated possible associations with neurodevelopmental disorders. Attention deficit disorder with or without...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-018-0200-z
更新日期:2019-01-01 00:00:00
abstract::Accurate interpretation of the results of GH stimulation tests is of pivotal importance not only in the evaluation of the etiology of growth retardation in children but also in the selection of the best candidates for GH therapy. We performed this study to test a novel immunofunctional GH ( IFGH) assay that makes use ...
journal_title:Pediatric research
pub_type: 评论,杂志文章
doi:10.1203/00006450-200011000-00010
更新日期:2000-11-01 00:00:00
abstract::We report a study on the effect of herpes simplex virus 1 (HSV-1) infection on apoptosis of neutrophils from both adults and neonates and present evidence showing that HSV-1 enhances apoptosis in neonatal, but not adult, neutrophils. HSV-1 enhanced the expression of both Fas and Fas ligand on the surface of neonatal n...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000191816.57544.b4
更新日期:2006-01-01 00:00:00
abstract::Vascular dopamine (DA1) receptors may modulate circulatory hemodynamics in lambs. We evaluated resting dopaminergic tone in lambs by pharmacologically manipulating peripheral DA1 receptors with i.v. SCH 23390, (a highly selective, competitive DA1 receptor antagonist) and i.v. fenoldopam, (a highly selective DA1 recept...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199302000-00018
更新日期:1993-02-01 00:00:00
abstract::BackgroundAdults born preterm have higher levels of cardiometabolic risk factors than their term-born peers. Studies have suggested that at least those born smallest eat less healthily. We examined the association between early (<34 weeks) and late (34-36 weeks) preterm birth and diet and food preferences in adult age...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.301
更新日期:2018-03-01 00:00:00
abstract::Intrauterine perfusion failure can cause cerebral malformations. We investigated the effect of transient maternal hypotension on newborn rat brain by inducing hypovolemic hypotension for 2.5 h during early embryonic day 7 (E7) or late (E15) gestation in pregnant rats. We found an increase in the number of TUNEL-positi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200101000-00012
更新日期:2001-01-01 00:00:00
abstract::The effects of ascorbic acid on the excretion of homogentisic acid and its derivative benzoquinone acetic acid were studied in two adults and three infants. The administration of relatively large amounts of ascorbic acid to the adults was followed by a disappearance of benzoquinone acetic acid from the urine, whereas ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198908000-00015
更新日期:1989-08-01 00:00:00
abstract::Genetic factors are implicated in pathogenesis of neonatal hyperbilirubinemia. In this nested case-control study, we determined 1) frequency of thymine-adenine (TA)n promoter polymorphism and Gly71Arg mutation in uridine diphosphoglucuronate-glucuronosyltransferase 1A1 (UGT1A1) gene in neonates > or =35-wk gestation p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31819ed5de
更新日期:2009-06-01 00:00:00
abstract::We have previously shown that milk sensitization aggravates intestinal dysfunction in the malnourished guinea pigs, suggesting that it may also impair the recovery from malnutrition. To test this hypothesis, the growing guinea pigs were malnourished by feeding only maize for 7 d and then were refed for 21 d with a bal...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199812000-00018
更新日期:1998-12-01 00:00:00
abstract::We studied the effects of exogenously administered adrenomedullin on fetal pulmonary arterial blood flow in near-term fetal sheep. The hemodynamic effects of a single injection of adrenomedullin into the left pulmonary artery were compared with those of acetylcholine; the effects of repeated injections of adrenomedull...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199704000-00007
更新日期:1997-04-01 00:00:00
abstract:BACKGROUND:Biliary atresia (BA) is an infantile disorder characterized by progressive sclerosing cholangiopathy leading to biliary obstruction. First-line treatment of BA is hepatoportoenterostomy, the prognosis of which is related to age at surgery and to histological variables such as extent of fibrosis and ductular ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.177
更新日期:2014-01-01 00:00:00
abstract::Plasma antioxidant status and total radical-trapping antioxidant potential (TRAP) of children (n = 24) with cystic fibrosis (CF) were compared with those of children (n = 21) without the disease. Children with CF were found to have elevated plasma concentrations of ascorbic acid (94.6 +/- 58.2 mumol/L), with respect t...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199303000-00008
更新日期:1993-03-01 00:00:00
abstract::Antenatal cocaine administration is associated with increases in catecholamines and glucocorticoids and results in fetal hypoxemia, with the speculated potential for increased free radical formation in the fetus. Because of this, we hypothesized that antenatal cocaine exposure would produce not only accelerated pulmon...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199304000-00004
更新日期:1993-04-01 00:00:00
abstract::A four-year-old Israeli Arab boy was found to have glycogen accumulation in both liver and muscle without clinical symptoms. Liver phosphorylase kinase (PK) activity was 20% of normal, resulting in undetectable activity of phosphorylase a. Muscle PK activity was about 25% of normal, resulting in a marked decrease of p...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198104000-00002
更新日期:1981-04-01 00:00:00
abstract::As the mechanisms controlling the amount and timing of growth saltations are not well understood, the identification of physiologic coupling in weight and length growth are important for further understanding normal growth biology. Thirty-four healthy infants (13 males, 21 females) participated in a longitudinal growt...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000184327.65102.a6
更新日期:2005-12-01 00:00:00