Adenosine deaminase deficiency without immunodeficiency: clinical and metabolic studies.

abstract：:To examine uridine diphosphate hexose (UDPhexose) content of cells that have more complete metabolic patterns than erythrocytes, which have been commonly used in the study of galactosemia, the concentrations of uridine diphosphate galactose (UDPgalactose) and uridine diphosphate glucose (UDPglucose) were determined in...

journal_title：Pediatric research

authors： Gibson JB,Reynolds RA,Palmieri MJ,States B,Berry GT,Segal S

更新日期：1994-11-01 00:00:00

abstract：:Increased concentrations of carcinoembryonic antigen (CEA) have been reported in the sera and sputum of patients with cystic fibrosis. We have found significantly higher concentrations of CEA in both extracted and unextracted saliva of patients with cystic fibrosis compared to age-matched controls and a comparable gro...

journal_title：Pediatric research

authors： MacSween JM,Gillespie CT

更新日期：1980-03-01 00:00:00

abstract：:A dietary-related deficiency of docosahexaenoic acid [C22:6(n-3)] in infant cerebral cortex has been identified. Absence or very low rates of hepatic synthesis from the essential fatty acid precursor, alpha-linolenic acid [C18:3(n-3)], in early life may have been a contributory factor. We have analyzed liver total lip...

journal_title：Pediatric research

authors： Farquharson J,Jamieson EC,Logan RW,Patrick WJ,Howatson AG,Cockburn F

更新日期：1995-09-01 00:00:00

abstract：:Despite the availability of successful prevention strategies to prevent excessive hyperbilirubinemia, the neurological sequelae of bilirubin neurotoxicity (BNTx) still occur throughout the world. Kernicterus, encephalopathy due to BNTx, is now understood to be a spectrum of severity and phenotypes known as kernicterus...

journal_title：Pediatric research

authors： Riordan SM,Shapiro SM

更新日期：2020-01-01 00:00:00

abstract：:Children with inborn errors of urea synthesis who survive neonatal hyperammonemic coma commonly exhibit cognitive deficits and neurologic abnormalities. Yet, there is evidence that ammonia is not the only neurotoxin. Hyperammonemia appears to induce a number of neurochemical alterations. In rodent models of hyperammon...

journal_title：Pediatric research

authors： Robinson MB,Heyes MP,Anegawa NJ,Gorry E,Djali S,Mellits ED,Batshaw ML

更新日期：1992-10-01 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

authors： Hazinski TA,Kennedy KA

更新日期：1987-12-01 00:00:00

abstract：BACKGROUND:Fetal hypoxia-ischemia (H-I) results in significant morbidity and mortality. Little is known about the timing of death in human stillbirths. The vulnerability of the fetus varies with age at the time of insult, but it is unknown what happens to the timing of fetal death in relation to a fetal insult. We aske...

journal_title：Pediatric research

authors： Derrick M,Englof I,Drobyshevsky A,Luo K,Yu L,Tan S

更新日期：2012-08-01 00:00:00

abstract：BACKGROUND:Aberrant immune responses play a key role in the pathogenesis of inflammatory bowel disease (IBD). Most studies conducted to delineate the underlying molecular mechanisms focus on adults; an understanding of these mechanisms in children remains to be determined. Here, cytokines and transcription factors prod...

journal_title：Pediatric research

authors： Busch MA,Gröndahl B,Knoll RL,Pretsch L,Doganci A,Hoffmann I,Kullmer U,Bähner V,Zepp F,Meyer CU,Gehring S

更新日期：2020-04-01 00:00:00

abstract：:The purpose of this project was to study the role of lipid peroxidation in oxygen-induced lung injury in the newborn lamb. It was our hypothesis that injury to the microvascular bed of the lung by oxygen would coincide with a burst of peroxidative activity and would be accompanied by an increased rate of excretion of ...

journal_title：Pediatric research

authors： Hansen TN,Smith CV,Gest AL,Smith HW,Giesler M

更新日期：1990-12-01 00:00:00

abstract：:A series of in vivo experiments is described in which [1-13C]phytanic acid was given as an oral substrate to a healthy subject and two patients showing an impairment in phytanic acid degradation, one with Refsum's disease and one with chondrodysplasia punctata. After intake of the substrate by the control in a dose of...

journal_title：Pediatric research

authors： ten Brink HJ,Schor DS,Kok RM,Stellaard F,Kneer J,Poll-The BT,Saudubray JM,Jakobs C

更新日期：1992-11-01 00:00:00

abstract：BACKGROUND:The basal ganglia are vulnerable to injury during cardiac arrest. Movement disorders are a common morbidity in survivors. Yet, neuronal motor network changes post-arrest remain poorly understood. METHODS:We compared function of the motor network in adult rats that, during postnatal week 3, underwent 9.5 min...

journal_title：Pediatric research

authors： Aravamuthan BR,Shoykhet M

更新日期：2015-10-01 00:00:00

abstract：:Cancer stem cells (CSC) are recently proposed to be the cancer initiating cells responsible for tumorigenesis and contribute to cancer resistance. Advances have been made in identifying and enriching CSC in leukemia and several solid tumors, including breast, brain and lung cancers. These studies suggest that, like no...

journal_title：Pediatric research

authors： Guo W,Lasky JL 3rd,Wu H

更新日期：2006-04-01 00:00:00

abstract：:Changes in high-density lipoprotein (HDL) subclass distribution were evaluated in a group of premature infants during the early postnatal period to ascertain whether enteral feeding brought about a rapid shift from neonatal to adult-like distributions. All infants were fed a combination of breast milk and formula. Cor...

journal_title：Pediatric research

authors： Genzel-Boroviczény O,D'Harlingue AE,Kao LC,Scott C,Forte TM

更新日期：1988-06-01 00:00:00

abstract：:Cardiac malformations (pulmonary trunk stenosis, ventricular septal defect, and double outlet right ventricle) were induced by the administration of two doses of retinoic acid (RA) to Wistar rats on d 13 of pregnancy. Contractile performance of the isolated perfused rat heart and its inotropic response to Ca2+ (0.6-10...

journal_title：Pediatric research

authors： Ostádalová I,Pexieder T,Ostádal B,Kolár F

更新日期：1995-12-01 00:00:00

abstract：:A son of related Turkish parents had grossly elevated serum tyrosine concentration and excreted tyrosine and p-hydroxyphenolic acids into the urine, whereas neither succinylacetone nor succinylacetoacetate could be demonstrated. The tyrosine concentration was normalized by a proper diet. This was not followed strictly...

journal_title：Pediatric research

authors： Andersson S,Nemeth A,Ohisalo J,Strandvik B

更新日期：1984-07-01 00:00:00

abstract：:Nutrition in early life, growth, and subsequent health over a lifetime are significantly interrelated. The aim of this study was to assess body composition changes in exclusively breast-fed infants from birth up to 6 mo of age. An observational, prospective, cohort study was conducted. Fifty-nine full-term, healthy, e...

journal_title：Pediatric research

authors： Roggero P,Giannì ML,Orsi A,Piemontese P,Amato O,Liotto N,Morlacchi L,Taroni F,Fields DA,Catalano PM,Mosca F

更新日期：2010-12-01 00:00:00

abstract：:The aim of the present study was to determine the proportion of gamma/delta T-lymphocytes and CD16/CD56 (CD3- and CD3+) cells in the peripheral blood of children and adolescents with Graves' disease (GD; n = 27; mean age, 15.5 +/- 5.1 y) and nontoxic nodular goiter (NTNG; n = 25; mean age, 15.2 +/- 5.7 y), in comparis...

journal_title：Pediatric research

authors： Bossowski A,Urban M,Stasiak-Barmuta A

更新日期：2003-09-01 00:00:00

abstract：:Docosahexaenoic acid (DHA) is an omega-3 fatty acid that is highly concentrated in CNS tissues. Although breast milk contains the fatty acids DHA and arachidonic acid, infant formulas marketed in North America do not contain these nutrients. The potential deleterious effects of rearing infants with formulas devoid of ...

journal_title：Pediatric research

authors： Champoux M,Hibbeln JR,Shannon C,Majchrzak S,Suomi SJ,Salem N Jr,Higley JD

更新日期：2002-03-01 00:00:00

abstract：:The incidence of allergic diseases has increased, and a relation between allergy and dietary fatty acids has been proposed. Modulation of the maternal immune function during pregnancy may have an impact on future clinical outcomes in the child. The aim of this study was to determine the effects of omega (omega)-3 long...

journal_title：Pediatric research

authors： Warstedt K,Furuhjelm C,Duchén K,Fälth-Magnusson K,Fagerås M

更新日期：2009-08-01 00:00:00

abstract：:Lipase activities were measured at pH 4 and pH 8 in the placentas of rats made diabetic by streptozotocin treatment and also in the placentas of women classified as having 1) impaired glucose tolerance or type 2 diabetes, 2) type 1 diabetes with no associated vascular complication, and 3) type 1 diabetes with associat...

journal_title：Pediatric research

authors： Kaminsky S,Sibley CP,Maresh M,Thomas CR,D'Souza SW

更新日期：1991-12-01 00:00:00

abstract：:Prematurity is associated with delayed postnatal activation of mitochondrial oxidative phosphorylation and impaired switch from glycolytic to oxidative metabolism. Fatty acids (FA), which represent a major energy substrate in mature muscle cells, are engaged in the postnatal activation of genes of energy metabolism an...

journal_title：Pediatric research

authors： Brauner P,Kopecký P,Flachs P,Ruffer J,Sebron V,Plavka R,Vítková I,Vorlícek J,Kopecký J

更新日期：2003-04-01 00:00:00

abstract：:Iron is essential for fetal organ development, but the effect of isolated iron deficiency on nephrogenesis is unknown. Human premature infants are at risk for disrupted nephrogenesis because glomerular development is incomplete until 36-wk gestation. We modeled the effects of iron on postnatal glomerulogenesis in four...

journal_title：Pediatric research

authors： Drake KA,Sauerbry MJ,Blohowiak SE,Repyak KS,Kling PJ

更新日期：2009-12-01 00:00:00

abstract：:Basal and pentagastrin-stimulated gastric acid secretion was measured in 26 healthy newborn infants on the 1st and 2nd days of life. Basal acid outputs (BAO) on day 1 (0.378 mEq/hr) and day 2 (0.388 mEq/hr) were similar and not significantly different from the maximal acid outputs (MAO) on day 1 (0.413 mEq/hr) and day...

journal_title：Pediatric research

authors： Euler AR,Byrne WJ,Meis PJ,Leake RD,Ament ME

更新日期：1979-01-01 00:00:00

abstract：:Previous studies have demonstrated metabolic dysfunction in the mononuclear cells of some children with abnormal cell-mediated immunity. Interpretation of these observations has been complicated by the extreme heterogeneity of cell types examined. The glycolytic metabolism of relatively enriched T-cells, non-T mononuc...

journal_title：Pediatric research

authors： Storch S,Klein W,Feig SA,Henderson T

更新日期：1980-02-01 00:00:00

abstract：BACKGROUND:Early recognition of patients at risk for sepsis is paramount to improve clinical outcomes. We hypothesized that subtle signatures of illness are present in physiological and biochemical time series of pediatric-intensive care unit (PICU) patients in the early stages of sepsis. METHODS:We developed multivar...

journal_title：Pediatric research

authors： Spaeder MC,Moorman JR,Tran CA,Keim-Malpass J,Zschaebitz JV,Lake DE,Clark MT

更新日期：2019-11-01 00:00:00

abstract：:Staphylococcus aureus has been isolated at an increasing rate from infants' stools during the last decades, but it is not known whether this species can colonize and persist in the intestinal microflora. To investigate this, 49 Swedish infants were followed prospectively from birth until 12 months of age. S. aureus wa...

journal_title：Pediatric research

authors： Lindberg E,Nowrouzian F,Adlerberth I,Wold AE

更新日期：2000-12-01 00:00:00

abstract：BACKGROUND:Cognitive impairment has been documented in adult diabetes but is unclear in pediatric diabetes. No study had been conducted to explore the relationship between attention-deficit/hyperactivity disorder (ADHD) and diabetes. Using a population-based data set, we aimed to examine the association between ADHD an...

journal_title：Pediatric research

authors： Chen HJ,Lee YJ,Yeh GC,Lin HC

更新日期：2013-04-01 00:00:00

abstract：:The etiology of the tall stature almost invariably seen in homocystinuric patients is not known. The effect of metabolic control and the role of the GH-IGF system on growth were investigated in 10 patients with homocystinuria. There was a direct correlation between the plasma free homocyst(e)ine and growth velocity SD...

journal_title：Pediatric research

authors： Topaloglu AK,Sansaricq C,Snyderman SE

更新日期：2001-06-01 00:00:00

abstract：:IL-8/CXC ligand (CXCL) 8 is ingested in high concentrations by the human fetus/neonate with amniotic fluid and human milk, and is also produced constitutively by intestinal epithelial cells (IEC). We have shown that recombinant human IL-8/CXCL8 (rhIL-8/CXCL8) protects cultured IEC against tumor necrosis factor (TNF)-a...

journal_title：Pediatric research

authors： Maheshwari A,Lacson A,Lu W,Fox SE,Barleycorn AA,Christensen RD,Calhoun DA

更新日期：2004-08-01 00:00:00

abstract：:Plasma aldosterone (A), corticosterone (B), deoxycorticosterone (DOC), progesterone (P), 17-hydroxyprogesterone (17-OHP), cortisol (F), and cortisone (E) were measured simultaneously by specific radioimmunoassays in small plasma samples obtained from 174 normal infants and children between 2 hr and 15 yr of age. The s...

journal_title：Pediatric research

authors： Sippell WG,Dörr HG,Bidlingmaier F,Knorr D

更新日期：1980-01-01 00:00:00