Abstract:
:A male child presented on the first day of life with metabolic acidosis with elevated blood lactate (15 mM), pyruvate (0.4 mM), and free fatty acid (1.3 mM) levels and a blood pH of 7.16. The severity of the acidosis was diminished by intravenous administration of glucose in large doses and by bicarbonate. On two occasions, when the acidosis was particularly severe, peritoneal dialysis using an acetate buffer was required. Restriction of the dietary intake of saturated fatty acids or treatment with nicotinic acid also appeared to diminish the severity of acidosis. No improvement was achieved by the administration of thiamine or biotin. Tissues taken at postmortem showed normal activity of gluconeogenic enzymes and pyruvate dehydrogenase. The activity of pyruvate dehydrogenase in tissue homogenates preincubated with ATP was reduced by 60-75% both in liver of the patient and of the controls because of the inactivation of the enzyme by pyruvate dehydrogenase kinase. Addition of Ca++ and Mg++ to the inactivated enzyme caused a prompt return of the activity to normal in controls but not in the patient. This defect, which was apparent in muscle and liver but not in brain, we attribute to a markedly reduced activity of pyruvate dehydrogenase phosphatase in the patient.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Robinson BH,Sherwood WGdoi
10.1203/00006450-197512000-00015subject
Has Abstractpub_date
1975-12-01 00:00:00pages
935-9issue
12eissn
0031-3998issn
1530-0447journal_volume
9pub_type
杂志文章abstract::We examined the effect of inspiratory resistive loaded breathing (IRL) on the electromyographic (EMG) activity of the costal and crural diaphragm in nine anesthetized spontaneously breathing piglets (age 10-23 days, weight 2.8-4.4 kg). Bipolar wire electrodes were inserted into the anterior paratendinous costal diaphr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198701000-00007
更新日期:1987-01-01 00:00:00
abstract::Several markers of growth and biochemical development in the rat were studied after administration of prostacyclin (PGI2) and 16, 16-dimethyl prostaglandin E2 (16, 16DM PGE2). Intermittent administration of PGI2 for 3 days to 10- and 19-day-old animals, with subsequent sacrifice at 14 and 23 days, resulted in signific...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198307000-00004
更新日期:1983-07-01 00:00:00
abstract::Prenatal exposure to infection and subsequent inflammatory responses have been implicated in the etiology of schizophrenia and autism. In this review, we summarize current evidence from human and animal studies supporting the hypothesis that the pathogenesis of these two disorders is linked via exposure to inflammatio...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e318212c196
更新日期:2011-05-01 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::Bubble continuous positive airway pressure (B-CPAP) applies small-amplitude, high-frequency oscillations in airway pressure (DeltaPaw) that may improve gas exchange in infants with respiratory disease. We developed a device, high-amplitude B-CPAP (HAB-CPAP), which provides greater DeltaPaw than B-CPAP provides. We stu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181dcd580
更新日期:2010-06-01 00:00:00
abstract::Human Bifidobacterium sp strain bifidum (B. bifidum) was administered to BALB/c lactating mice (n = 58) and their litters (n = 327 pups) to evaluate the ingested strain's adherent properties and ability to inhibit murine rotavirus (MRV) infection. ELISA and anaerobic bacteriologic techniques were used to measure MRV s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199406000-00014
更新日期:1994-06-01 00:00:00
abstract:BACKGROUND:Although cardiopulmonary bypass (CPB) has been previously studied as risking infection and inflammatory responses, few studies evaluate the relationship of preoperative high total immunoglobulin E (tIgE) to outcomes in pediatric patients predisposed to atopy undergoing cardiac surgery with CPB. METHODS:Seru...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0048-2
更新日期:2018-11-01 00:00:00
abstract::Preterm infants are exposed to frequent painful procedures and agitating stimuli over the many weeks of their hospitalization in the neonatal intensive care unit (NICU). The adverse neurobiological impact of pain and stress in the preterm infant has been well documented, including neuroimaging and neurobehavioral outc...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0744-6
更新日期:2020-08-01 00:00:00
abstract::The placenta is essential to nutrition before birth. Recent work has shown that a range of clearly defined alterations can be found in the placentas of infants with intrauterine growth restriction (IUGR). In the mouse, a placental specific knockout of a single imprinted gene, encoding IGF-2, results in one pattern of ...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/01.PDR.0000181381.82856.23
更新日期:2005-11-01 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::Hypoxic-ischemic injuries can evolve over several days, and recent studies suggest that further neuronal death may occur 6 to 72 h later. Because cerebral temperature is an important determinant of outcome during the primary injury, we investigated the effect of temperature, on outcome, during the later phases of inju...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199604000-00005
更新日期:1996-04-01 00:00:00
abstract::Escherichia coli which elaborate heat stable enterotoxin (ST) are a major cause of endemic diarrhea in infants. The reason(s) for this increased susceptibility of infants to ST-mediated diarrhea is unknown. We investigated the possibility that the immature (14 and 21 day old) rat small intestine is more sensitive to S...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198606000-00017
更新日期:1986-06-01 00:00:00
abstract:BACKGROUND:A lack of consensus exists as to the timing of kidney biopsy in children with steroid-dependent nephrotic syndrome (SDNS) where minimal change disease (MCD) predominates. This study aimed at examining the applicability of a biomarker-assisted risk score model to select SDNS patients at high risk of focal seg...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0277-z
更新日期:2019-03-01 00:00:00
abstract:BACKGROUND:No study has examined the association between prenatal phthalate exposure and intrauterine growth retardation (IUGR). This study aimed to investigate whether prenatal exposure to phthalates was associated with increased risk of IUGR. METHODS:A total of 126 mother-newborn pairs, including 42 IUGR cases and 8...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.103
更新日期:2014-10-01 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:BACKGROUND:Neonates with hypoxic-ischemic encephalopathy (HIE) frequently develop acute kidney injury (AKI). Aminophylline has been shown to reduce severe renal dysfunction in neonates after perinatal asphyxia. However, the effect of aminophylline on renal function in neonates undergoing hypothermia has not been studie...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-0999-y
更新日期:2020-06-05 00:00:00
abstract::An amendment to this paper has been published and can be accessed via a link at the top of the paper. ...
journal_title:Pediatric research
pub_type: 已发布勘误
doi:10.1038/s41390-019-0680-5
更新日期:2020-04-01 00:00:00
abstract::In this study, we have investigated the possible role of the pro-aggregatory arachidonic acid (AA) metabolite thromboxane, in the impaired function of neonatal platelets. In platelet-rich plasma thromboxane production (measured by radioimmunoassay of thromboxane B2) was not different between neonates and adults when s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198409000-00003
更新日期:1984-09-01 00:00:00
abstract::Epidermal growth factor (EGF), a potent mitogen present in milk, is postulated to play an important physiologic role in the ontogeny of the intestine. An artificial rat milk, free of EGF, was developed and fed to newborn rats in an effort to evaluate the physiologic role of EGF on intestinal glucose transport. Compari...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198704000-00017
更新日期:1987-04-01 00:00:00
abstract::Chondro-osseous tissue from five patients with diastropic dwarfism was studied by histologic, histochemical, and electron microscopic methods. The major abnormalities observed were: 1) irregular distribution of chondrocytes undergoing degeneration in the resting cartilage; 2) abnormal distribution of collagens in the ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197908000-00007
更新日期:1979-08-01 00:00:00
abstract::The objective of this study was to obtain insight into bone formation and resorption in children with newly diagnosed untreated acute lymphoblastic leukemia (ALL). In 23 consecutive children with ALL, a bone biopsy was taken from the crista iliaca posterior under ketamine anesthesia, together with the diagnostic marro...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000090929.16450.DE
更新日期:2003-12-01 00:00:00
abstract::The use of tissue engineering to develop new urinary bladder could have direct impact on children suffering from congenital bladder dysfunction. Current bladder tissue engineering protocols rely on scaffolds to support the ingrowth of new cells and the subsequent development of new tissues. The interaction between cel...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181d35034
更新日期:2010-05-01 00:00:00
abstract::The treatment of multiple acyl-CoA-dehydrogenase deficiency (MADD) includes a low-fat, low-protein, high-carbohydrate diet, avoiding long fasting periods. However, there is no useful biochemical marker to determine the response to different diets or fasting periods. The aims of this study are to report a patient with ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200107000-00013
更新日期:2001-07-01 00:00:00
abstract::One of two slightly different influenza A/ts-1[E] recombinant candidate live vaccines was given intranasally to each of 23 young children. Twelve of 15 children who had no serum HI antibody but who did have serum ANAB at the time of administration became infected and 1 had mild rhinitis. All eight who lacked both type...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197604000-00008
更新日期:1976-04-01 00:00:00
abstract::The transport of phosphate into jejunal brush border membrane vesicles isolated from 14- to 42-day-old rats was investigated using a rapid filtration technique. The presence of a sodium gradient enhanced phosphate uptake at all ages. Both sodium-dependent phosphate uptake and passive phosphate uptake declined with inc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198512000-00021
更新日期:1985-12-01 00:00:00
abstract:BACKGROUND:Vascular endothelial growth factor (VEGF), a well-characterized regulator of angiogenesis, has been mechanistically implicated in retinal neovascularization and in the pathogenesis of retinopathy of prematurity. However, the ontogeny of VEGF expression in the human fetal retina is not well known. Because ret...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2015.15
更新日期:2015-04-01 00:00:00
abstract::EBV has been associated with several malignancies in humans. EBV can also infect marmoset B lymphocytes, which, as opposed to human B cells, are permissive for lytic Epstein-Barr viral replication. Mice with a severe combined immunodeficiency phenotype (SCID mice) are extremely susceptible to EBV-induced lymphomagenes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199410000-00007
更新日期:1994-10-01 00:00:00
abstract::Serum insulin, growth hormone (GH), insulin-like growth factors (IGFs) I and II, cortisol, and albumin concentrations were measured in 15 children with kwashiorkor, 15 with marasmic-kwashiorkor, and 21 with marasmus, before and in the survivors, after nutritional rehabilitation, as well as in 10 underweight and eight ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198611000-00012
更新日期:1986-11-01 00:00:00
abstract::The association between lung injury and thrombocytopenia was investigated by comparing the megakaryocyte and platelet counts, and platelet activation using P-selectin as a marker, between the prepulmonary (right atrial) and postpulmonary (left atrial) blood in adult and neonatal (preterm and term) rats with and withou...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000079186.86219.29
更新日期:2003-09-01 00:00:00
abstract:BACKGROUND:Phytosterols in soybean oil (SO) lipids likely contribute to parenteral nutrition-associated liver disease (PNALD) in infants. No characterization of phytosterol metabolism has been done in infants receiving SO lipids. METHODS:In a prospective cohort study, 45 neonates (36 SO lipid vs. 9 control) underwent ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2015.78
更新日期:2015-08-01 00:00:00