Abstract:
:In an effort to characterize the hepatic abnormality in patients with alpha1-antitrypsin deficiency, three unrelated children with the disorder (Pi types ZZ and SZ), two heterozygous parents (Pi type MZ), and three normal subjects (Pi type MM) were studied. As expected, the livers of the ZZ- and SZ-deficient subjects showed abnormal accumulation of alpha1-antitrypsin in the cisternae of the rough endoplasmic reticulum as judged by immunofluorescent and electron microscopic studies. Their parents (MZ phenotype) demonstrated identical although less extensive hepatic abnormalities. Short term cultures of liver tissue in the presence of radiolabeled amino acids showed both synthesis and release of alpha1-antitrypsin in normal control subjects and in the patients with the Z protein. Radiolabeled intracellular alpha1-antitrypsin could not be found. These studies demonstrate synthesis of alpha1-antitrypsin by the livers of normal and genetically deficient subjects in vitro, and suggest several possible mechanisms for alpha1-antitrypsin deficiency.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Bhan AK,Grand RJ,Colten HR,Alper CAdoi
10.1203/00006450-197601000-00007subject
Has Abstractpub_date
1976-01-01 00:00:00pages
35-40issue
1eissn
0031-3998issn
1530-0447journal_volume
10pub_type
杂志文章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:BACKGROUND:Adolescents born preterm have altered hypothalamic-pituitary-adrenal axis function with a blunted cortisol stress response, however, the influences of intrauterine growth restriction and race are unclear. METHODS:We measured salivary cortisol before and 20 min after a maximal-exercise stress test and calcul...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0682-3
更新日期:2020-05-01 00:00:00
abstract::To define respiratory effects of peripheral acidemia during early development, we injected lactic acid (LA) into the jugular vein of 32 1- to 7-d-old rabbit pups and four adult rabbits. The body weight of newborns varied between 67 +/- 17 g in 1-d-old and 119.8 +/- 33.7 g in 7-d-old animals versus 4 +/- 0.1 kg in adul...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199106010-00006
更新日期:1991-06-01 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::Studies have shown that human neonates who develop group B streptococcal sepsis usually lack opsonic antibody (Ab) to their infecting strain and that these neonates may also have impaired polymorphonuclear leukocyte (PMN) function. The present study was designed to determine the efficacy of administration of PMNs or o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198012000-00031
更新日期:1980-12-01 00:00:00
abstract::Energy and nitrogen balances were measured in growing low birthweight infants fed either mother's expressed breast milk or a 20 kcal per ounce formula to determine whether or not there were differences between the two dietary groups in (1) the partition of energy among excretion, expenditure, and storage and (2) the r...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198311000-00011
更新日期:1983-11-01 00:00:00
abstract:BACKGROUND:Serum caffeine concentrations >20 μg/ml (100 μmol/l) in infants treated for apnea of prematurity increases TNF-α and decreases IL-10, changes that perhaps are linked to comorbidities. We hypothesize that this proinflammatory cytokine profile may be linked to differential binding of caffeine to adenosine rece...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.50
更新日期:2016-07-01 00:00:00
abstract::This study was aimed at describing abdominal and laryngeal muscle responses to upper airway occlusion (UAO) in early life and the effect of sleep states on these responses. Twelve nonsedated, 9-26-d-old lambs were studied. We simultaneously recorded 1) airflow (pneumotachograph + face mask); 2) sleep states (electroco...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199706000-00011
更新日期:1997-06-01 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::This study aimed to characterize early neonatal microvascular function after preeclamptic pregnancy with respect to infant sex and in utero growth. Peripheral microvascular blood flow was examined prospectively from 6 to 72 h of age using laser Doppler flowmetry in a cohort of term infants of normotensive women and wo...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/pdr.0b013e318193edf1
更新日期:2009-03-01 00:00:00
abstract::There are substantial alterations in fuel homeostasis immediately after birth. Leptin is a putative regulator of energy metabolism. Consequently, the aim of this study was to examine whether there are changes in circulating leptin concentrations during the early postnatal period. Umbilical cord mixed blood samples wer...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199902000-00007
更新日期:1999-02-01 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::In view of the suggested relationship between substrate availability, fetal growth and circulating fetal IGF-I concentrations, we investigated the effect of maternal starvation on plasma IGF-I levels in the late gestation ovine fetus. Ten fetuses aged 125-130 d gestation were sampled daily from indwelling arterial cat...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199004000-00015
更新日期:1990-04-01 00:00:00
abstract::Long-term parenteral nutrition (TPN) in children is associated with sustained hyperinsulinemia due to a high nutriment infusion flow 12 h/24 h, with plausible lipotoxicity secondary to repeated lipid infusions and with changes in incretin hormone release. The aim of this study was to test whether long-term TPN can lea...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/PDR.0b013e3181559d5c
更新日期:2007-11-01 00:00:00
abstract::The objective of the present study is to compare the emergence of a circadian respiratory pattern in subsequent siblings of SIDS and controls infants to see whether the unique time and age of SIDS is correlated with altered circadian manifestations. During the first three months of life, a pattern in respiratory rates...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198004000-00018
更新日期:1980-04-01 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
pub_type: 杂志文章
doi:10.1203/00006450-199512000-00011
更新日期:1995-12-01 00:00:00
abstract:BACKGROUND:Early brain development is closely dictated by distinct neurobiological principles. Here, we aimed to map early trajectories of structural brain wiring in the neonatal brain. METHODS:We investigated structural connectome development in 44 newborns, including 23 preterm infants and 21 full-term neonates scan...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0138-1
更新日期:2018-12-01 00:00:00
abstract::Cerebral blood flow (CBF) measurement by near infrared spectroscopy (NIRS) using oxyhemoglobin (HbO2) as a tracer (CBF-HbO2) needs rapid changes in arterial oxygen saturation (SaO2) which often cannot be achieved in many sick infants. An alternative method based on the same adaptation of the Fick principle using i.v. ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199801000-00006
更新日期:1998-01-01 00:00:00
abstract::Epithelial hyperplasia is an early feature of the renal and biliary lesions in autosomal recessive polycystic kidney disease (ARPKD). To explore the cellular basis of this hyperplasia we isolated, cultured, and characterized biliary tract epithelium from common bile duct explants of mice with ARPKD (the BPK mouse) and...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199506000-00014
更新日期:1995-06-01 00:00:00
abstract::L-selectin, a surface adhesion glycoprotein expressed on leukocytes, has a well-established role in mediating inflammation and lymphocyte recirculation. Recent evidence suggests that L-selectin may also influence hematopoiesis. We observed that a greater proportion of CD34+ cells express L-selectin in cord blood compa...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199906000-00015
更新日期:1999-06-01 00:00:00
abstract::Our objective was to determine the quantitative changes of individual triacylglycerol (TG) species in liver and carcass during early postnatal growth. Pregnant rats were killed at d 21 of pregnancy, and neonatal rats were killed at d 3 or 9 after birth. Quantitative changes in fatty acids and TG species of fetal/neona...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199201000-00009
更新日期:1992-01-01 00:00:00
abstract::We investigated the developmental changes in the pattern of urinary metabolites of theophylline, a substrate for CYP1A2, to study when CYP1A2, which is absent in the perinatal period, fully develops during childhood. The urinary ratios of three metabolites (1-methyluric acid, 3-methylxanthine, and 1,3-dimethyluric aci...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199901000-00011
更新日期:1999-01-01 00:00:00
abstract:BACKGROUND:Oral propranolol, a nonselective β-blocker, is able to reduce the progression of retinopathy of prematurity in newborns, but it appeared unsafe. This study aimed to find, in rabbits, a propranolol eye drop concentration able to induce lower plasma but higher retinal concentrations than those obtained after o...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.108
更新日期:2014-10-01 00:00:00
abstract::Although the pathogenesis of necrotizing enterocolitis remains uncertain, ischemia appears to be an important contributing factor to the development of this disorder. Reperfusion plays a major role in ischemia-related injury, and oxygen free radicals produced during reperfusion most likely contribute to the injury. Th...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199708000-00009
更新日期:1997-08-01 00:00:00
abstract::Functional disturbances in microcirculation in juvenile type 1 diabetes (T1D) are believed to underlie, in part, the later occurrence of cardiovascular complications. Some epidemiologic studies suggested greater risk of microvascular complications in those with T1D-risk genotypes of human leukocyte antigen (HLA). We i...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e318165bfd4
更新日期:2008-04-01 00:00:00
abstract::The effects of fetal hypoxemia on blood coagulation (platelet count, prothrombin time, partial thromboplastin time, fibrinogen, factors II, V, VI, VIII, IX, X, XI, XII, XIII and von Willebrands activities, fibrin degradation products, and fibrin monomer) were evaluated in nine chronically catheterized fetal lambs earl...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198201001-00003
更新日期:1982-01-01 00:00:00
abstract::There is significant uncertainty over the role of assessment of long-term neurodevelopmental outcome (LTO) in neonatal clinical trials. A multidisciplinary working group was established to identify key issues in this area and to make recommendations about optimal approaches to evaluate LTO in therapeutic trials in new...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/s41390-019-0526-1
更新日期:2019-11-01 00:00:00
abstract::Blue and white phototherapy was given to infant (and weanling) homozygous Gunn rats treated with different doses of riboflavin-5-phosphate (ribofl.5'p.) During the first hours after a flavin-injection, the effect of phototherapy with both types of fluorescent lamps was enhanced. With equal radiant power applied, the s...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197912000-00001
更新日期:1979-12-01 00:00:00
abstract::Serum diamine oxidase (DAO) activities were measured in 20 control patients and in 24 patients with gastroenteritis. Results (mean +/- SE) were as follows: 1) control patients (n = 20), 36.2 +/- 5.7 pmol h-1 ml-1; 2) gastroenteritis (acute phase) (n = 11), 31 +/- 4.9 pmol h-1 ml-1; 3) gastroenteritis (healing phase) (...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198501000-00008
更新日期:1985-01-01 00:00:00
abstract::The renal effects of endothelin-1 were investigated in 16 anesthetized and mechanically ventilated newborn rabbits. Renal blood flow and glomerular filtration rate were determined by the clearance of para-aminohippuric acid and inulin, respectively. Each animal acted as its own control. In eight newborn rabbits, a bol...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199308000-00002
更新日期:1993-08-01 00:00:00