Hyperprolactinemia, a tool in treatment control of tetrahydrobiopterin deficiency: endocrine studies in an affected girl.

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 Res

journal_title

Pediatric research

authors

Birnbacher R,Scheibenreiter S,Blau N,Bieglmayer C,Frisch H,Waldhauser F

doi

10.1203/00006450-199804000-00006

subject

Has Abstract

pub_date

1998-04-01 00:00:00

pages

472-7

issue

4 Pt 1

eissn

0031-3998

issn

1530-0447

journal_volume

43

pub_type

杂志文章
  • Increased expression of extracellular glutathione peroxidase in mice with dextran sodium sulfate-induced experimental colitis.

    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

    authors: Tham DM,Whitin JC,Cohen HJ

    更新日期:2002-05-01 00:00:00

  • Studies of lipids, lipoproteins, and apolipoproteins in Menkes' disease.

    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

    authors: Blackett PR,Lee DM,Donaldson DL,Fesmire JD,Chan WY,Holcombe JH,Rennert OM

    更新日期:1984-09-01 00:00:00

  • Transient intrauterine hypotension: effect on newborn rat brain.

    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

    authors: Apak RA,Anlar B,Atilla P,Cakar N

    更新日期:2001-01-01 00:00:00

  • A new look at the pathogenesis of the meconium aspiration syndrome: a role for fetal pancreatic proteolytic enzymes in epithelial cell detachment.

    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

    authors: Ivanov VA,Gewolb IH,Uhal BD

    更新日期:2010-09-01 00:00:00

  • Real-time spectral analysis of the fetal EEG: a new approach to monitoring sleep states and fetal condition during labor.

    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

    authors: Thaler I,Boldes R,Timor-Tritsch I

    更新日期:2000-09-01 00:00:00

  • Histopathologic changes and the immune response within the jejunal mucosa in infants and children.

    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

    authors: Maffei HV,Kingston D,Hill ID,Shiner M

    更新日期:1979-06-01 00:00:00

  • Steroid and growth hormone levels in premature infants after prenatal betamethasone therapy to prevent respiratory distress syndrome.

    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

    authors: Ballard PL,Gluckman PD,Liggins GC,Kaplan SL,Grumbach MM

    更新日期:1980-02-01 00:00:00

  • Herpes simplex virus-stimulated gamma-interferon production by newborn mononuclear cells.

    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

    authors: Hayward AR,Herberger M,Saunders D

    更新日期:1986-05-01 00:00:00

  • Ouabain effects on oxygen physiology in anemic lambs.

    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

    authors: Berman W Jr,Lister G Jr,Alverson D,Olsen S

    更新日期:1987-05-01 00:00:00

  • Immediate hypothermia reduces cardiac troponin I after hypoxic-ischemic encephalopathy in newborn pigs.

    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

    authors: Liu X,Tooley J,Løberg EM,Suleiman MS,Thoresen M

    更新日期:2011-10-01 00:00:00

  • Elevated protein carbonyls and lipid peroxidation products correlating with myeloperoxidase in tracheal aspirates from premature infants.

    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

    authors: Buss IH,Darlow BA,Winterbourn CC

    更新日期:2000-05-01 00:00:00

  • Erythropoietin production by macrophages from preterm infants: implications regarding the cause of the anemia of prematurity.

    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

    authors: Ohls RK,Li Y,Trautman MS,Christensen RD

    更新日期:1994-02-01 00:00:00

  • Effects of indomethacin on fetal rat lungs: a possible cause of persistent fetal circulation (PFC).

    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

    authors: Harker LC,Kirkpatrick SE,Friedman WF,Bloor CM

    更新日期:1981-02-01 00:00:00

  • Growth trajectories of extremely low birth weight infants from birth to young adulthood: a longitudinal, population-based study.

    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

    authors: Saigal S,Stoskopf B,Streiner D,Paneth N,Pinelli J,Boyle M

    更新日期:2006-12-01 00:00:00

  • Renal hemodynamic responses to hypoxemia during development: relationships to circulating vasoactive substances.

    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

    authors: Weismann DN,Robillard JE

    更新日期:1988-02-01 00:00:00

  • Fetal cartilage xylosyltransferase activity and skeletal growth in sheep.

    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

    authors: Morriss FH Jr,Fitzgerald B,Riddle LM

    更新日期:1985-12-01 00:00:00

  • Impairment of rat postnatal lung alveolar development by glucocorticoids: involvement of the p21CIP1 and p27KIP1 cyclin-dependent kinase inhibitors.

    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

    authors: Corroyer S,Schittny JC,Djonov V,Burri PH,Clement A

    更新日期:2002-02-01 00:00:00

  • The effect of graded intake of glycyl-L-tyrosine on phenylalanine and tyrosine metabolism in parenterally fed neonates with an estimation of tyrosine requirement.

    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

    authors: Roberts SA,Ball RO,Moore AM,Filler RM,Pencharz PB

    更新日期:2001-01-01 00:00:00

  • Cultured skin fibroblasts derived from patients with mucolipidosis 4 are auto-fluorescent.

    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

    authors: Goldin E,Blanchette-Mackie EJ,Dwyer NK,Pentchev PG,Brady RO

    更新日期:1995-06-01 00:00:00

  • Social determinants of health and outcomes for children and adults with congenital heart disease: a systematic review.

    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

    authors: Davey B,Sinha R,Lee JH,Gauthier M,Flores G

    更新日期:2020-10-17 00:00:00

  • Intravenous immune globulin for the prevention of infections in children with symptomatic human immunodeficiency virus infection.

    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

    authors: Mofenson LM,Moye J Jr

    更新日期:1993-01-01 00:00:00

  • Characterization of brain development in the ferret via MRI.

    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

    authors: Barnette AR,Neil JJ,Kroenke CD,Griffith JL,Epstein AA,Bayly PV,Knutsen AK,Inder TE

    更新日期:2009-07-01 00:00:00

  • Single-Immunoglobulin Interleukin-1-Related Receptor regulates vulnerability to TLR4-mediated necrotizing enterocolitis in a mouse model.

    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

    authors: Fawley J,Cuna A,Menden HL,McElroy S,Umar S,Welak SR,Gourlay DM,Li X,Sampath V

    更新日期:2018-01-01 00:00:00

  • Serum calcitonin and blood mineral interrelationships in normal children aged six to twelve years.

    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

    authors: Shainkin-Kerstenbaum R,Funkenstein B,Conforti A,Shani S,Berlyne GM

    更新日期:1977-02-01 00:00:00

  • Characterization of natural killer and antibody-dependent cellular cytotoxicity of preterm infants against human immunodeficiency virus-infected cells.

    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

    authors: Merrill JD,Sigaroudinia M,Kohl S

    更新日期:1996-09-01 00:00:00

  • Alveolar hypoxia increases small pulmonary wedge pressure in awake young lambs.

    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

    authors: Hazinski TA,Kennedy KA

    更新日期:1987-12-01 00:00:00

  • Proportional assist ventilation decreases thoracoabdominal asynchrony and chest wall distortion in preterm infants.

    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

    authors: Musante G,Schulze A,Gerhardt T,Everett R,Claure N,Schaller P,Bancalari E

    更新日期:2001-02-01 00:00:00

  • The relationship between hyperbilirubinemia and the promoter region and first exon of UGT1A1 gene polymorphisms in Vietnamese newborns.

    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

    authors: Nguyen TT,Zhao W,Yang X,Zhong DN

    更新日期:2020-12-01 00:00:00

  • The expression of CD40 on monocytes of children with primary humoral immunodeficiencies.

    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

    authors: Kowalczyk D,Macura-Biegun A,Zembala M

    更新日期:2006-06-01 00:00:00

  • Total parenteral nutrition in the newborn: energy substrates and plasma total fatty acids.

    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

    authors: Pineault M,Lepage G,Bisaillon S,Roy CC,Chessex P

    更新日期:1989-10-01 00:00:00