Abstract:
:Severe tetrahydrobiopterin (BH4) deficiency is a naturally occurring model of cerebral catecholamine and serotonin shortage. Examination of the stimulated release and physiologic secretion pattern of several hormones in affected individuals permits certain conclusions concerning the involvement of these neurotransmitters in hormone regulation. Treatment, moreover, permits the ranking of the quality of the therapeutic regimens in use according to the degree of hormonal alteration. The 24-h secretion pattern of prolactin, GH, cortisol, and melatonin and the stimulated release of prolactin, GH, TSH, and gonadotropins were studied in an affected girl. Severe hyperprolactinemia with disruption of the pulsatile and circadian secretion pattern was the prevailing feature. The GH physiologic secretion pattern was not affected, but its stimulation was impaired. Melatonin displayed a normal circadian secretion pattern; the rhythm, however, was advanced by several hours. Conventional treatment of BH4 deficiency, i.e. BH4, 5-hydroxytryptophan, and L-DOPA/carbidopa (the last named given in three doses per day), suppresses prolactin levels merely for a few hours. L-DOPA/carbidopa given at shorter intervals or, even better, as a slow release preparation, is more effective in suppressing prolactin levels. Our data indicate immense hyperprolactinemia but few other hormonal disturbances in severe BH4 deficiency. Prolactin secretion may serve as an extremely sensitive marker for the hypothalamic dopamine content under different therapeutic regimens. Treatment with an L-DOPA/carbidopa slow release preparation produces virtually normal prolactin levels.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Birnbacher R,Scheibenreiter S,Blau N,Bieglmayer C,Frisch H,Waldhauser Fdoi
10.1203/00006450-199804000-00006subject
Has Abstractpub_date
1998-04-01 00:00:00pages
472-7issue
4 Pt 1eissn
0031-3998issn
1530-0447journal_volume
43pub_type
杂志文章abstract::Extracellular glutathione peroxidase (E-GPx) is a selenoenzyme that reduces hydrogen peroxide and organic peroxides. All plasma glutathione peroxidase (GPx) activity in humans is attributable to E-GPx. The gastrointestinal (GI) tract also synthesizes and secretes E-GPx into the extracellular milieu. Endogenously gener...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200205000-00016
更新日期:2002-05-01 00:00:00
abstract::Three patients with Menkes' disease, an inherited disorder of copper transport, were studied to determine whether the copper deficiency was associated with a lipoprotein disorder. Hypocuprinemia was documented in all three cases. Two patients had severe copper and ceruloplasmin deficiencies, whereas the third patient ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198409000-00012
更新日期:1984-09-01 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::We hypothesized that fetal pancreatic digestive enzymes play a role in the lung damage after meconium aspiration. We studied the effect of meconium on the A549 alveolar epithelial cell line. The exposure of the cells to 0.5 to 5% meconium resulted in significant disruption of connections between A549 cells and caused ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181ebd4c3
更新日期:2010-09-01 00:00:00
abstract::Adverse perinatal events affecting cerebral functions are a major cause of neonatal mortality, morbidity, and long-term neurologic deficit. Intrapartum fetal EEG, which records fetal brain electrical activity, provides a monitoring modality for evaluating the fetal CNS during labor. In this study, we describe a new ap...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200009000-00013
更新日期:2000-09-01 00:00:00
abstract::Jejunal biopsies obtained from 45 children referred for a variety of clinical conditions were examined histologically and by standard immunofluorescence methods. The number of plasma cells was recorded per "mucosal tissue unit". The results showed that most of the immunoglobulin A (IgA) counts in infants under 3 yr wi...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197906000-00002
更新日期:1979-06-01 00:00:00
abstract::Prenatal maternal therapy with glucocorticoid reduces the incidence of respiratory distress syndrome (RDS) in premature infants. To investigate the effects of this treatment on the fetal endocrine system, we determined serum concentrations of betamethasone, cortisol, dehydroepiandrosterone sulfate, growth hormone, and...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198002000-00011
更新日期:1980-02-01 00:00:00
abstract::Blood mononuclear cells from newborns and from adults, immune or nonimmune to herpes simplex virus, were cultured with IL 2 and herpes simplex virus and the amount of gamma-interferon in the supernatant measured after 3 days. The newborn and nonimmune adult cells made equivalent trace amounts of gamma-interferon in cu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198605000-00004
更新日期:1986-05-01 00:00:00
abstract::We studied the effects of anemia and ouabain administration on cardiac function, oxygen physiology, and blood catecholamine levels in nine newborn lambs. We measured oxygen consumption continuously, along with traditional hemodynamic variables. Oxygen transport was calculated. Following baseline measurements, lambs we...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198705000-00005
更新日期:1987-05-01 00:00:00
abstract::Neonatal hypoxic-ischemic encephalopathy (HIE) is a clinically defined neurological condition after lack of oxygen and often associated with cardiac dysfunction in term infants. Therapeutic hypothermia (HT) after birth is neuroprotective in infants with HIE. However, it is not known whether HT is also cardioprotective...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31822941ee
更新日期:2011-10-01 00:00:00
abstract::The purpose of this study is to determine whether the oxidative injury markers, protein carbonyls and malondialdehyde (MDA), are elevated in tracheal aspirates from very low birth weight (< 1500 g) infants; to determine whether levels correlate with myeloperoxidase as a marker of neutrophil inflammation; and to assess...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-200005000-00014
更新日期:2000-05-01 00:00:00
abstract::In the human fetus, liver macrophages appear to be the primary source of erythropoietin (Epo). Epo production shifts from the liver to peritubular cells in the kidney sometime during the 3rd trimester. Some preterm infants experience a hyporegenerative anemia that appears to be caused by inadequate Epo production. It ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199402000-00008
更新日期:1994-02-01 00:00:00
abstract::Indomethacin, because of its prostaglandin inhibition, may cause constriction of the ductus arteriosus and affect the pulmonary circulation. To study this possibility, we gave indomethacin daily by gavage to two groups of pregnant rats from the 17th day of pregnancy through delivery (group 1, 2 mg/kg/day; group 2, 4 m...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198102000-00013
更新日期:1981-02-01 00:00:00
abstract::We compared sex-specific growth attainment of a population-based cohort of 147/166 (89%) extremely low birthweight (ELBW < 1000 g) and 131/145 (90%) sociodemographically comparable normal birthweight (NBW) cohort at young adulthood, and examined the pattern of growth trajectories and correlates of growth at ages 1, 2,...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000246201.93662.8e
更新日期:2006-12-01 00:00:00
abstract::Chronically catheterized fetal lambs (n = 11, gestational age 111-139 days) and neonatal lambs (n = 20, postnatal age 4-30 days) were studied to explore during development the relationship of renal hemodynamic responses during hypoxemia to plasma epinephrine concentration (E), plasma norepinephrine concentration (NE),...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198802000-00004
更新日期:1988-02-01 00:00:00
abstract::The activity of UDP-D-xylose:proteoglycan core protein beta-D-xylosyltransferase (EC 2.4.2.26), the enzyme that catalyzes the initiation of the polysaccharide chain linkage to the core protein of proteoglycans, was measured in costal cartilage from 20 fetal sheep of 65-138 days gestation. Activity of the enzyme was es...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198512000-00003
更新日期:1985-12-01 00:00:00
abstract::It has been shown that glucocorticoids accelerate lung development by limiting alveolar formation resulting from a premature maturation of the alveolar septa. Based on these data, the aim of the present work was to analyze the influence of dexamethasone on cell cycle control mechanisms during postnatal lung developmen...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200202000-00008
更新日期:2002-02-01 00:00:00
abstract::Although tyrosine is considered indispensable during the neonatal period, its poor solubility has limited its inclusion in parenteral amino acid solutions to less than 1% of total amino acids. Dipeptides of tyrosine are highly soluble, have been shown to be well used and safe in animal models and humans, and, therefor...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1203/00006450-200101000-00022
更新日期:2001-01-01 00:00:00
abstract::Mucolipidosis 4 (ML4) is an autosomal recessive disorder with both lipid and mucopolysaccharide storage. The disease is characterized by severe visual impairment and psychomotor retardation. In our effort to find a phenotypic marker for ML4 fibroblasts, living cells were stained with fluorescent compounds. The stainin...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199506000-00003
更新日期:1995-06-01 00:00:00
abstract:BACKGROUND:Social determinants of health (SDH) can substantially impact health outcomes. A systematic review, however, has never been conducted on associations of SDH with congenital heart disease (CHD) outcomes. The aim, therefore, was to conduct such a systematic review. METHODS:Seven databases were searched through...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-020-01196-6
更新日期:2020-10-17 00:00:00
abstract::Infection with human immunodeficiency virus (HIV) causes a progressive immunodeficiency, often manifested in adults by the development of infections with relatively rare, opportunistic organisms. However, in HIV-infected children, recurrent serious infections with common encapsulated bacteria, as well as recurrent min...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验,评审
doi:10.1203/00006450-199305001-00464
更新日期:1993-01-01 00:00:00
abstract::Animal models with complex cortical development are useful for improving our understanding of the wide spectrum of neurodevelopmental challenges facing human preterm infants. MRI techniques can define both cerebral injury and alterations in cerebral development with translation between animal models and the human infa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181a291d9
更新日期:2009-07-01 00:00:00
abstract::BackgroundThe mechanisms underlying aberrant activation of intestinal Toll-like receptor 4 (TLR4) signaling in necrotizing enterocolitis (NEC) remain unclear. In this study, we examined the role of single-immunoglobulin interleukin-1 receptor-related molecule (SIGIRR), an inhibitor of TLR signaling, in modulating expe...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.211
更新日期:2018-01-01 00:00:00
abstract::Simultaneous measurements of serum immunoreactive thyrocalcitonin (iTCT), immunoreactive parathyroid hormone (iPTH), calcium, inorganic phosphate, magnesium, and alkaline phosphatase were made in 37 normal children whose ages ranged from 6-12 years. Between the ages of 6 and 12 there is a statistically significant ris...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197702000-00006
更新日期:1977-02-01 00:00:00
abstract::The odds risk of vertical transmission of human immunodeficiency virus (HIV) to preterm infants is almost four times that of term infants and may relate to maternal and neonatal factors. We characterized the competence of early nonspecific cellular immunity, namely natural killer cytotoxicity (NKC) and antibody-depend...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199609000-00021
更新日期:1996-09-01 00:00:00
abstract::Alveolar hypoxia increases pulmonary artery pressure in lambs and sheep but increases lung lymph flow only in lambs; the reasons for this are unknown. To test the hypothesis that hypoxia-induced pulmonary venous constriction could in part explain the fluid filtration response in young lambs, eight young lambs (13 +/- ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198712000-00013
更新日期:1987-12-01 00:00:00
abstract::Thoracoabdominal asynchrony (TAA) and chest wall distortion (CWD) are commonly seen in preterm infants secondary to a highly compliant rib cage and poor compensation of distorting forces by inspiratory rib cage muscles. Continuous positive airway pressure (CPAP) reduces TAA and CWD by stenting the chest wall. We hypot...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200102000-00008
更新日期:2001-02-01 00:00:00
abstract:BACKGROUND:To investigate the relationship between unexplained indirect hyperbilirubinemia of Vietnamese newborns and the polymorphism of the promoter TATA box and exon 1 of bilirubin uridine diphosphate glucuronosyltransferase (UGT1A1) gene. METHODS:A total of 149 neonates were divided into the hyperbilirubinemia gro...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0825-6
更新日期:2020-12-01 00:00:00
abstract::The interactions between CD40 and CD40L (CD154) are critical for effective humoral immune response. CD40 signaling facilitates T lymphocyte dependent B cell proliferation and immunoglobulin isotype switch. The objective of our study was to investigate the CD40 and CD40L expression on peripheral blood mononuclear cells...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000219298.96471.18
更新日期:2006-06-01 00:00:00
abstract::Carbohydrate and lipid intakes have both been found to modulate the metabolism of long-chain fatty acids. To define the respective influence of these two energy substrates on plasma fatty acid concentrations, 32 studies were performed in 16 parenterally fed newborn infants (mean +/- SEM, birth wt: 2.15 +/- 0.1 kg, age...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198910000-00002
更新日期:1989-10-01 00:00:00