Abstract:
:Yesterday's immunodeficiencies emphasized the clinical and familial associations of the syndromes and date from the 1920s (ataxia-telangiectasia, chronic mucocutaneous candidiasis), the 1930s (Wiskott-Aldrich syndrome), skipping the 1940s, but blossoming in the 15-y period from 1950 to 1965. In this period, primary immunodeficiencies affecting all the major limbs of the immune system were first described (1950: severe combined immunodeficiency; 1952: X-linked agammaglobulinemia; 1957: chronic granulomatous disease; 1965: C2 deficiency). Today's immunodeficiencies, as detailed in Stiehm's Immunologic Disorders in Infants and Children (Edition 1, 1973; Edition 2, 1980; and Edition 3, 1989) emphasize the immunologic and genetic aspects of immunodeficiency. These increased from 43 syndromes in the 1973 edition (34 primary, nine secondary) to 94 syndromes in the 1989 edition (66 primary, 28 secondary). This means that about two primary and one secondary immunodeficiencies have been uncovered annually. Tomorrow's immunodeficiencies, to be covered in Edition 4, will include new clinical and immunologic observations and molecular and biochemical studies that characterize some unique immunodeficiencies. These include the following six groups of defects: 1) neutropenic syndromes with hypogammaglobulinemia, including the WHIM syndrome; 2) phenotypic genetic syndromes with immunodeficiency including Bloom's syndrome and Schimke's immuno-osseous dysplasia; 3) natural killer cell defects associated with a) other primary immunodeficiencies, b) other nonimmunologic illness, and c) primary natural killer defects; 4) T-cell membrane defects; 5) IL defects; and 6) miscellaneous phagocytic illnesses including periodontitis and the asplenia syndrome.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Stiehm ERdoi
10.1203/00006450-199305001-00007subject
Has Abstractpub_date
1993-01-01 00:00:00pages
S2-7; discussion S7-8issue
1 Suppleissn
0031-3998issn
1530-0447journal_volume
33pub_type
历史文章,杂志文章,评审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::Preterm infants are more prone to bone mineral deficiency the lower their birth weight. To achieve the intrauterine bone mineral accretion rate postnatally, 74 low-birth-weight infants (median birth weight, 980 g; range 430-1.580 g) were each supplemented enterally and/or parenterally with calcium and/or phosphorus in...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199401000-00027
更新日期:1994-01-01 00:00:00
abstract::Three members of a Virginia family with a bleeding disorder were found to have a serum defect in thromboplastin generation similar to the previously reported "Car. factor" deficiency. Sera from three members of the original Car. family did not correct the defect of affected members of the Virginia family. Partial thro...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-197504000-00009
更新日期:1975-04-01 00:00:00
abstract::The activity of lysozyme in saliva and serum was determined in 51 patients with cystic fibrosis. Measurements were made on two occasions at least 1 month apart and compared to those of 25 normal healthy individuals of the same ages, sex, and race. The mean serum lysozyme activity of normal individuals was 5.8 microgra...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198210000-00014
更新日期:1982-10-01 00:00:00
abstract::The effects of an emulsified intravenous fat preparation (Intralipid) on bilirubin transport were analyzed by cholestyramine extraction, spectrophotometric analysis, Sephadex gel filtration, peroxidase assay, bilirubin uptake by red blood cells, and by toxicity in tissue culture (L-929) cells. Intralipid is capable of...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197703000-00004
更新日期:1977-03-01 00:00:00
abstract:BACKGROUND:Maternal smoking impairs fetal growth; however, if postnatal growth differs between children born small for gestational age (SGA) with smoking and non-smoking mother is unknown. METHODS:Cohort-study of term born children born appropriate for gestational age with non-smoking mother (AGA-NS, n = 30,561), SGA ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0352-5
更新日期:2019-06-01 00:00:00
abstract:BACKGROUND:Human milk oligosaccharides (HMOs) shape the intestinal microbiota in term infants. In premature infants, alterations in the intestinal microbiota (dysbiosis) are associated with risk of necrotizing enterocolitis (NEC) and sepsis, and the influence of HMOs on the microbiota is unclear. METHODS:Milk, urine, ...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1038/pr.2015.162
更新日期:2015-12-01 00:00:00
abstract::Epidemiologic studies have linked intrauterine growth restriction (IUGR) with an increased incidence of cardiovascular disease later in life; reduced cardiomyocyte number in IUGR hearts may underlie such prenatal programming. Our aim was to examine the effect of IUGR, as a result of maternal protein restriction, on th...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000157726.65492.CD
更新日期:2005-06-01 00:00:00
abstract::Children treated for acute lymphoblastic leukemia may develop reduced bone mineral density during treatment, but there is little information on the mechanisms involved. In a prospective, longitudinal study on 15 children with ALL, we undertook serial measurements of markers of bone and collagen turnover, insulin-like ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200010000-00012
更新日期:2000-10-01 00:00:00
abstract:BACKGROUND:Angiotensin (ANG) II is involved in experimental hyperoxia-induced lung fibrosis. Angiotensin-converting enzyme-2 (ACE-2) degrades ANG II and is thus protective, but is downregulated in adult human and experimental lung fibrosis. Hyperoxia is a known cause of chronic fibrotic lung disease in neonates, but th...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2015.27
更新日期:2015-05-01 00:00:00
abstract:IMPACT:Raises the importance of Social Determinants of Health in pediatrics. Considers the impact of Social Determinants of Health on COVID-19. Offers opportunities for research in the future. ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-020-01253-0
更新日期:2020-11-10 00:00:00
abstract::In mice, secondary alveolar septal formation primarily occurs during a brief postnatal period and is accompanied by transient expansion of the interstitial lung fibroblast (LF) population. PDGF-A, which solely signals through PDGF-receptor-alpha (PDGF-Rα), is required for expansion, but the receptor's relevant downstr...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e31821cfb5a
更新日期:2011-07-01 00:00:00
abstract::Altered pulmonary vascular resistance might be a factor for delayed closure of the ductus arteriosus (DA) in preterm infants. Angiotensin II plays a central role in the elevation of pulmonary vascular resistance. Angiotensin II exerts its vasoconstrictor effect on the angiotensin II type 1 receptor (AT1R). Homozygous ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000088016.67117.39
更新日期:2003-11-01 00:00:00
abstract::Pulmonary hypoplasia has been associated with absent or hypoplastic pulmonary artery in four cases in humans. Despite these reports, the effects of decreased pulmonary arterial flow on fetal lung growth have not been adequately studied. This study defines the effects of left pulmonary artery (LPA) ligation on fetal lu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199002000-00004
更新日期:1990-02-01 00:00:00
abstract::BackgroundPoor aerobic fitness is associated with worsening of asthma symptoms, and fitness training may improve asthma control. The mechanism linking fitness with asthma is not known. We hypothesized that repeated bouts of exercise would lead to a downregulation of glucocorticoid receptor (GR) expression on circulati...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.66
更新日期:2017-08-01 00:00:00
abstract:BACKGROUND:Near-infrared spectroscopy (NIRS) is an upcoming clinical method for monitoring regional cerebral oxygen saturation (rScO2) in neonates. There is a growing market offering different devices and sensors. Even though this technique is increasingly clinically applied, little is known about the similarities and/...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.133
更新日期:2013-11-01 00:00:00
abstract::Twenty-four samples of breast milk from nine mothers of infants suffering from breast milk jaundice were studied. Eight samples of milk from mothers of nonjaundiced infants, along with five formula milks enriched with polyunsaturated fatty acids, served as controls. Milks from mothers with jaundiced infants had no inh...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197606000-00007
更新日期:1976-06-01 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::The IQ's of Jamaican boys aged 6-10 were associated significantly with the presence or absence of severe malnutrition in infancy, with height at time of IQ testing, and with a measure of the boys' social background. A multiple correlation coefficient of 0.674 was obtained between IQ and the three factors. Social backg...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197601000-00011
更新日期:1976-01-01 00:00:00
abstract:BACKGROUND:Infantile hemangioma (IH) is the most frequent vascular tumor of early childhood. Recently, propranolol, a nonselective β1- and β2-adrenoceptor inhibitor, was introduced into the therapy of severe proliferating IH with excellent results. However, the underlying mechanism of action of propranolol is still unc...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.16
更新日期:2013-04-01 00:00:00
abstract:BACKGROUND:Bronchopulmonary dysplasia (BPD) is an important cause of morbidity. The aim of this study was to evaluate the preventive effect of cytidine 5'-diphosphocholine (CDP-choline) treatment on hyperoxic lung injury in a neonatal rat model. METHODS:A total of 30 newborn pups were divided into control, hyperoxia, ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.68
更新日期:2013-07-01 00:00:00
abstract::Although fetal asphyxia, i.e. hypoxemia, acidosis, and hypercapnia, increases plasma arginine vasopressin (AVP) greater than 40-fold, hypoxemia and metabolic acidosis occurring independently cause only 5-fold and 2-fold increases, respectively. To determine the effects of hypercapnia on AVP release, we examined the ef...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199110000-00014
更新日期:1991-10-01 00:00:00
abstract::Newborn Sprague-Dawley rat pups were found to have reduced net tubular reclamation of the beta-amino acid taurine in vivo. The reabsorption of this compound increased between the 2nd and 4th week of life and the excreted taurine fell despite a rise in glomerular filtration rate indicative of increased transport with m...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-197907000-00012
更新日期:1979-07-01 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: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::We evaluated the effectiveness of administration of high-dose busulfan (BU), an alkylating agent with substantial myeloablative but negligible immunosuppressive properties, on the engraftment of congenic normal bone marrow and spleen cells (hematopoietic cell transplantation; HCT) in murine galactosylceramidase defici...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199103000-00016
更新日期:1991-03-01 00:00:00
abstract:BACKGROUND:Secreted frizzled-related protein 5 (SFRP5) is an adipokine protecting against obesity-related insulin resistance and diabetes. SFRP5 binds to wingless type mouse mammary tumor virus (MMTV) integration site family member 5A (WNT5A) to improve insulin sensitivity. We performed the first study of SFRP5 and WNT...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2014.29
更新日期:2014-06-01 00:00:00
abstract:OBJECTIVES:(1) Examine associations of a branched-chain amino acid (BCAA) metabolite pattern with metabolic risk across adolescence; (2) use Least Absolute Shrinkage and Selection Operator (LASSO) to identify novel metabolites of metabolic risk. METHODS:We used linear regression to examine associations of a BCAA score...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-018-0195-5
更新日期:2019-02-01 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
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181f85a20
更新日期:2010-12-01 00:00:00
abstract::A longitudinal study describes the pattern of appearance of eight primitive reflexes in a population of 47 viable extremely premature infants, beginning as early as 25 wk postconceptional age (PCA). Infants were examined weekly, from 1 wk of age until discharge from the neonatal intensive care unit. Primitive reflexes...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198612000-00018
更新日期:1986-12-01 00:00:00