Abstract:
:Carbonic anhydrase II (CA II) is the only soluble isozyme of CA which is known to be expressed in kidney. We recently identified a deficiency of this enzyme as the basis for the autosomal recessive syndrome of osteopetrosis with renal tubular acidosis and cerebral calcification. In order to explore the physiological importance of CA II in the kidney, we studied the renal response to intravenously infused acetazolamide in two CA II-deficient patients and two control subjects. Following acetazolamide infusion, the CA II-deficient patients exhibited a prompt rise in urinary pH and HCO3- excretion similar to the response seen in control subjects. These findings indicate that CA II-deficient patients, who lack detectable CA II in their erythrocytes, still expressed an acetazolamide-inhibitable CA activity in their kidneys. These results can be explained in three ways: 1) the CA II deficiency which is profound in the erythrocytes of these patients may not be expressed in their kidney. 2) An acetazolamide-sensitive CA other than CA II, such as CA I and CA III, which is not normally expressed in kidney, is expressed in kidneys of CA II-deficient patients. 3) The CA II deficiency is expressed in kidney in these patients but the acetazolamide response is due to inhibition of the luminal, membrane-bound CA which is the product of a different gene and unaffected by the CA II deficiency mutation. We favor the third possibility.(ABSTRACT TRUNCATED AT 250 WORDS)
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Sly WS,Whyte MP,Krupin T,Sundaram Vdoi
10.1203/00006450-198510000-00017subject
Has Abstractpub_date
1985-10-01 00:00:00pages
1033-6issue
10eissn
0031-3998issn
1530-0447journal_volume
19pub_type
杂志文章abstract::Skin fibroblasts derived from 6 patients with cystic fibrosis (CF), 1-6 months old, and from 6 age matched donors were investigated for their ability to accumulate cyclic adenosine 3':5'-monophosphate (c-AMP) in response to isoproterenol and prostaglandin E1 (PGE1) using strictly defined culture conditions. In order t...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198003000-00018
更新日期:1980-03-01 00:00:00
abstract::Women who are at risk of preterm delivery are treated with antenatal steroids to facilitate fetal lung maturation. During this period, there is a potential for fetal or maternal hypoxemia to occur. Fetal responses to hypoxemia in sheep are well documented. However, less is known regarding maternal responses to hypoxem...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000117847.59343.B2
更新日期:2004-05-01 00:00:00
abstract::Necrotizing enterocolitis (NEC) is a devastating intestinal disease of premature infants. Maternal milk has been suggested to be partially protective against NEC; however, the mechanisms of this protection are not defined. The aim of this study was to examine the effect(s) of artificial feeding of rat milk (RM)-versus...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000050657.56817.E0
更新日期:2003-03-01 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::Although the use of antenatal glucocorticoids has resulted in decreased neonatal morbidity/mortality, recent animal studies have raised concerns regarding adverse effects of these medications on postnatal cardiovascular function. We hypothesized that antenatal betamethasone (Beta) exposure alters cerebral vascular rea...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181edb9fa
更新日期:2010-10-01 00:00:00
abstract::Breast-fed infants have higher bilirubin levels than formula-fed infants, possibly because of variations in the composition of the breast milk. The aim of this study was to investigate whether there is a relationship between cytokine levels in the colostrum of nursing mothers and neonatal jaundice (NJ). Breast milk sa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31809871c9
更新日期:2007-08-01 00:00:00
abstract::BackgroundHyperoxic lung injury is characterized by cellular damage from high oxygen concentrations that lead to an inflammatory response and it disrupts normal alveolarization in the developing newborn lung. Adenosine is a signaling molecule that is generated extracellularly by ecto-5'-nucleotidase (CD73) in response...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.176
更新日期:2017-08-23 00:00:00
abstract::We investigated the relative contribution of the two pathways of phosphatidylcholine (PC) synthesis in lungs from chick embryos of 12 to 20 days of incubation as well as from hatched chicks and adult chickens. Lung "blocks" were incubated for 60 min in vitro in the presence of [3H]methionine and [14C]choline. PC was s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198105000-00008
更新日期:1981-05-01 00:00:00
abstract::The effect of alpha-adrenergic blockade by phentolamine on the pulmonary vascular response to dopamine was studied in chronically prepared newborn lambs. Dopamine was administered at doses of 2.7 micrograms . kg-1 . min-1, 27 micrograms . kg-1 . min-1 and 270 micrograms . kg-1 . min-1 with and without alpha-adrenergic...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198306000-00007
更新日期:1983-06-01 00:00:00
abstract::Developmental disability, intrauterine growth retardation, renal anomalies, and dysmorphic features have been described in offspring of women who abuse toluene during pregnancy. A Sprague-Dawley rat model was developed to study this clinical syndrome. During d 6-19 of gestation, 11 treated dams received daily gavage d...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199412000-00023
更新日期:1994-12-01 00:00:00
abstract::The relationship between cerebral blood flow (CBF) and the evolution of brain edema was investigated in an experimental model of perinatal hypoxic-ischemic brain damage. Seven-d postnatal rats were subjected to unilateral common carotid artery ligation followed by 3 h of hypoxia with 8% oxygen at 37 degrees C. This in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199005000-00007
更新日期:1990-05-01 00:00:00
abstract::Progress has been made in the reduction of morbidity and mortality from neonatal sepsis. However, diagnosis continues to rely primarily on conventional microbiologic techniques, which can be inaccurate. The objective of this review is to provide the clinician with an overview of the current information available on di...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2017.134
更新日期:2017-10-01 00:00:00
abstract::We established an experimental animal model of the gastrointestinal colonization and systemic disease following oral challenge of type III group B streptococcal strain in 3-day-old newborn rats. Two type III group B streptococcal strains isolated from the cerebrospinal fluid of septic newborn infants produced coloniza...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198412000-00022
更新日期:1984-12-01 00:00:00
abstract::Sudden infant death syndrome is the most common cause of postneonatal infant mortality in the developed world. It is a diagnosis of exclusion with peak age of incidence between 2 and 6 mo. Fifty to 63% of these infants have a preexisting upper respiratory tract infection before death. We hypothesized that the immature...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200210000-00005
更新日期:2002-10-01 00:00:00
abstract::A study using fractional clearances during orally induced water diuresis was designed to delineate the mechanism underlying defective tubular reabsorption of sodium in very low-birth-weight neonates. The use of clearance methodology during maximal water diuresis may give an indirect estimate of distal sodium delivery ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198312000-00017
更新日期:1983-12-01 00:00:00
abstract::Gluconeogenesis decreases during septic shock, but its mechanism is not well known. Tumor necrosis factor alpha (TNF-alpha), which is a key cytokine in septic shock, can increase GLUT1 gene expression and glucose uptake in muscles and fatty tissues. TNF-alpha does not alter the metabolism of hepatocytes in which GLUT2...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200104000-00018
更新日期:2001-04-01 00:00:00
abstract::Tracheal aspirate IL-8 concentration and airway epithelial cell IL-8 expression are each increased in premature infants undergoing mechanical ventilation. We sought to determine the cytokines responsible for IL-8 expression in this context. Tracheal aspirates were collected from 18 mechanically ventilated premature in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000145274.47221.10
更新日期:2004-12-01 00:00:00
abstract:BACKGROUND:The objective was to determine the association between perioperative risk factors and brain imaging abnormalities on neurologic outcome in neonates with hypoplastic left heart syndrome (HLHS) or d-Transposition of the great arteries (d-TGA) who underwent cardiac surgery including cardiopulmonary bypass. MET...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-1085-1
更新日期:2020-07-25 00:00:00
abstract::Hypoxia and other adverse conditions are commonly encountered by rapidly growing cells. The RNA-binding protein RBM3 (RNA-binding motif protein 3), which is transcriptionally induced by low temperature and hypoxia, has recently been implicated in survival of colon cancer cells by mechanisms involving cyclooxygenase-2 ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181c13326
更新日期:2010-01-01 00:00:00
abstract::Two infants with disorders of propionate metabolism were studied at 7 months of age to determine optimum levels of intake of protein and calories to meet the requirements for essential amino acid for growth in infancy, and at the same time minimize the accumulation of toxic intermediates. An effect of alanine was foun...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198501000-00023
更新日期:1985-01-01 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::This study investigated the effects of intraventricular/periventricular blood on cerebral cAMP production and cortical cerebrovascular reactivity. Under halothane and N2O anesthesia, 3 mL of either autologous blood or artificial cerebrospinal fluid (CSF) were injected into the left caudate nucleus; volume was adequate...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199709000-00010
更新日期:1997-09-01 00:00:00
abstract::Human and ovine placental tissue homogenates were assayed for dipeptidase activity in vitro. Glycyl-L-leucine, L-leucyl glycine, glycyl-L-lysine, and L-lysyl glycine were hydrolyzed by placental homogenates. The pH optimum for the reaction was 8.0. The relationship between enzyme activity and concentration was linear ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198104000-00013
更新日期:1981-04-01 00:00:00
abstract:BACKGROUND:Short forms of IQ (S-IQ) assessments are time efficient and highly predictive of the full IQ (F-IQ) in healthy individuals. To investigate the validity of S-IQs for patients with neurodevelopmental impairments, this study tested a well-established S-IQ version in patients with congenital heart disease (CHD)....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0667-2
更新日期:2020-04-01 00:00:00
abstract::The purpose of this study was to investigate the effect of constant light in a neonatal care unit on the development of the sleep-and-wakefulness rhythm in preterm infants. Two groups of infants (57 preterm infants without other complications and 58 healthy term infants) were prospectively studied over infancy by a da...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199302000-00014
更新日期:1993-02-01 00:00:00
abstract::An alteration in head position, which effects cerebral blood flow, may increase the risk for intraventricular hemorrhage in the critically ill infant. The purpose of this study was to evaluate in vivo cerebral oxidative metabolism as an index of tissue oxygen delivery reflecting brain blood flow, in healthy preterm an...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198709000-00013
更新日期:1987-09-01 00:00:00
abstract::A new model has been developed for the study of maternal phenylketonuria. Beginning on the 12th day of gestation the diet of pregnant rats was supplemented with 0.5% alpha-methylphenylalanine and 3% phenylalanine. This resulted in an 83% reduction of hepatic phenylalanine hydroxylase activity. The maternal plasma phen...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198205000-00014
更新日期:1982-05-01 00:00:00
abstract::Tracheal epithelial cells obtained from adult and infant ferrets were grown in primary culture in vitro. Cells from adult animals grew readily, and many ciliated cells were observed in the cultures. Successful cultures were derived from infant animals, but cell number in infant and adult cultures began to decrease aft...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199107000-00016
更新日期:1991-07-01 00:00:00
abstract::Carlo Agostoni, MD, and Kwang Sik Kim, MD, are the Guest Editors for this annual review issue on Nutrition and the Microbiome. Dr Agostoni is a Professor of Pediatrics at the Department of Clinical Sciences and Community Health, University of Milan, within the Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, ...
journal_title:Pediatric research
pub_type: 历史文章
doi:10.1038/pr.2014.195
更新日期:2015-01-01 00:00:00
abstract::The purpose of this study was to investigate atrial myocardial blood flow in the fetus under conditions of acute right ventricular pressure load and adenosine infusion. Late gestation fetal sheep were instrumented for acute right ventricular pressure loading or adenosine infusion, and regional myocardial blood flow wa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199403000-00010
更新日期:1994-03-01 00:00:00