Recent advances in primary immunodeficiencies: identification of novel genetic defects and unanticipated phenotypes.

abstract：:125I-Tyr insulin (125I-Tyr Ins) was injected into the vitelline vein of rat fetuses in utero after 17, 19, or 21 days of gestation. Three min later, the weight and radioactivity of various organs and of the remaining carcass were measured. A radioactivity concentration index (RCI) was calculated by dividing the specif...

journal_title：Pediatric research

authors： Sodoyez-Goffaux FR,Sodoyez JC,De Vos CJ

更新日期：1981-09-01 00:00:00

abstract：:Acute postasphyxial encephalopathy around the time of birth remains a major cause of death and disability. The possibility that hypothermia may be able to prevent or lessen asphyxial brain injury is a "dream revisited". In this review, a historical perspective is provided from the first reported use of therapeutic hyp...

journal_title：Pediatric research

authors： Gunn AJ,Laptook AR,Robertson NJ,Barks JD,Thoresen M,Wassink G,Bennet L

更新日期：2017-01-01 00:00:00

abstract：:The objective of this study was to establish longitudinal normative limits for home memory monitors during early infancy. Eighty-eight healthy infants were monitored overnight at 0.25-19 wk of age using the Healthdyne Smart Monitor. Apnea settings were 14 s for recording and 40 s for alarm; the bradycardia setting was...

journal_title：Pediatric research

authors： Hunt CE,Hufford DR,Bourguignon C,Oess MA

更新日期：1996-02-01 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

authors： Semama DS,Thonney M,Guignard JP,Gouyon JB

更新日期：1993-08-01 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：:Shigellosis in children can cause growth retardation, worsening of malnutrition, and hypoproteinemia. To assess the effects of ingestion of a protein-rich diet during convalescence, 22 children aged 2 to 4 y with culture-proven shigellosis were randomly assigned after 5 d of antibiotic treatment to 21-d feeding regime...

journal_title：Pediatric research

authors： Kabir I,Butler T,Underwood LE,Rahman MM

更新日期：1992-12-01 00:00:00

abstract：:Phototherapy results in the conversion of native bilirubin to more water-soluble configurational and structural isomers. The serum half-life for the configurational isomer, the principal photoproduct in vivo, was determined by high pressure liquid chromatography in six premature infants following cessation of photothe...

journal_title：Pediatric research

authors： Ennever JF,Knox I,Denne SC,Speck WT

更新日期：1985-02-01 00:00:00

abstract：:Acute hemolytic anemia associated with red blood cell (RBC) glucose-6-phosphate dehydrogenase (G6PD) deficiency is commonly encountered in the Mediterranean basin. Nevertheless, concomitant clinical evidence of white blood cell G6PD deficiency is extremely rare in Israel. This study sought to assess simultaneously lev...

journal_title：Pediatric research

authors： Wolach B,Ashkenazi M,Grossmann R,Gavrieli R,Friedman Z,Bashan N,Roos D

更新日期：2004-05-01 00:00:00

abstract：:The aim of this study was to evaluate the changes in Doppler transmitral flow patterns during the 1st d of life. Doppler echocardiography of the ascending aorta and mitral valve was performed serially in 20 normal neonates at 2, 12, and 24 h of age. A computer-interfaced digitizer pad was used to measure the following...

journal_title：Pediatric research

authors： Harada K,Shiota T,Takahashi Y,Tamura M,Toyono M,Takada G

更新日期：1994-04-01 00:00:00

abstract：:Sugar alcohols have been found to play an important osmoregulatory role both in unicellular organisms and, more recently, in multicellular organisms, including mammals. This study shows that myo-inositol accumulates in the brains of chronically hypernatremic mice, as had been earlier found in rats, and demonstrates fo...

journal_title：Pediatric research

authors： Thurston JH,Sherman WR,Hauhart RE,Kloepper RF

更新日期：1989-11-01 00:00:00

abstract：:Human autoimmune lymphoproliferative syndrome (ALPS) is caused by defective CD95-mediated apoptosis of lymphocytes. In most patients, heterozygous mutations within the CD95 gene are found. Mutated proteins interfere with CD95-signaling in a dominant-negative way. However, the penetrance of clinical disease is variable...

journal_title：Pediatric research

authors： Fuchs H,Posovszky C,Lahr G,van der Werff ten Bosch J,Boehler T,Debatin KM

更新日期：2009-02-01 00:00:00

abstract：:The effect of increased red blood cell aggregation on uteroplacental blood flow was studied in 11 awake, late-pregnant guinea pigs. The aggregation of the red cells was increased by administering high molecular weight dextran (HMWD) to the previously hemoconcentrated animal. The purpose of the hemoconcentration before...

journal_title：Pediatric research

authors： Verkeste CM,Boekkooi PF,Saxena PR,Peeters LL

更新日期：1992-01-01 00:00:00

abstract：BACKGROUND:Food allergy is common in children with prevalence up to 10%. We assessed the clinico-laboratory characteristics and frequency of food sensitization to the commonly consumed food among upper Egyptian preschool children with recurrent wheezy chest. METHODS:This cross-sectional descriptive study was conducted...

journal_title：Pediatric research

authors： Abdallah AM,Osman NS,Mohammad HA,Metwalley KA,Embaby M,ElMelegy TT

更新日期：2020-10-01 00:00:00

abstract：:We have developed a regional dual-threshold method for detecting endocrine pulses at variably low hormone concentrations such as those that occur in the pediatric age group. This algorithm uses the local intra-series coefficient of variation to test for a significant increase and decrease (peak or pulse) in the experi...

journal_title：Pediatric research

authors： Velduis JD,Weiss J,Mauras N,Rogol AD,Evans WS,Johnson ML

更新日期：1986-07-01 00:00:00

abstract：:Magnesium-deficient fetuses exhibited malformations (44%), anemia, and edema. Maternal plasma magnesium levels at day 21 of pregnancy reflected the level of dietary magnesium (2.43 +/- 0.09 mg Mg/100 ml, control; 0.74 +/- 0.02 mg Mg/100 ml, deficient). Plasma magnesium levels of deficient fetuses showed similar decrea...

journal_title：Pediatric research

authors： Cosens G,Diamond I,Theriault LL,Hurley LS

更新日期：1977-06-01 00:00:00

abstract：:Previous studies in mature fetal sheep have shown that alcohol depresses cerebral blood flow (CBF), cerebral O2 consumption (CMRO2), and cerebral glucose consumption (CMRglu). This effect earlier in gestation might contribute to the pathogenesis of fetal alcohol syndrome. Physiologic studies of immature fetal sheep ha...

journal_title：Pediatric research

authors： Gleason CA,Hotchkiss KJ

更新日期：1992-06-01 00:00:00

abstract：:Pesticides are chemicals that are designed specifically for the purpose of killing or suppressing another living organism. Human toxicity is possible with any pesticide, and a growing body of literature has investigated possible associations with neurodevelopmental disorders. Attention deficit disorder with or without...

journal_title：Pediatric research

authors： Roberts JR,Dawley EH,Reigart JR

更新日期：2019-01-01 00:00:00

abstract：:The secreted protein sonic hedgehog (Shh) is essential for normal development of many organs. Targeted disruption of Shh in mouse leads to near complete absence of craniofacial skeletal elements at birth, and mutation of SHH in human causes holoprosencephaly (HPE), frequently associated with defects of derivatives of ...

journal_title：Pediatric research

authors： Yamagishi C,Yamagishi H,Maeda J,Tsuchihashi T,Ivey K,Hu T,Srivastava D

更新日期：2006-03-01 00:00:00

abstract：BACKGROUND:The preterm newborn is at high risk of developing cardiovascular compromise during the first day of life and this is associated with increased risk of brain injury. Standard treatments are volume expansion and administration of inotropes, typically dopamine and/or dobutamine, but there is limited evidence th...

journal_title：Pediatric research

authors： Eiby YA,Shrimpton NY,Wright IM,Lumbers ER,Colditz PB,Duncombe GJ,Lingwood BE

更新日期：2016-12-01 00:00:00

abstract：:A substantial proportion of the "apparently normal" preterm infants exhibit minor and moderate dysfunctions in neuromotor outcome as they grow older. Birth characteristics, minor abnormalities on the neonatal ultrasound scan of the brain, and motor milestones have only limited value in the early detection of these chi...

journal_title：Pediatric research

authors： Fallang B,Øien I,Hellem E,Saugstad OD,Hadders-Algra M

更新日期：2005-08-01 00:00:00

abstract：:The effect of hypoxia-ischemia (HI) on IL-1, and IL-6 bioactivity in relation to expression of IL-1 alpha, IL-1 beta, and IL-6 mRNA was studied, and the neuroprotective efficacy of IL-1 receptor antagonist (IL-1ra) was evaluated in neonatal rats. HI was induced in 7-d-old rats by unilateral carotid artery ligation and...

journal_title：Pediatric research

authors： Hagberg H,Gilland E,Bona E,Hanson LA,Hahin-Zoric M,Blennow M,Holst M,McRae A,Söder O

更新日期：1996-10-01 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

authors： Yeager AM,Shinohara M,Shinn C

更新日期：1991-03-01 00:00:00

abstract：:BackgroundLactoferrin (LTF) could play a beneficial role in insulin resistance and diabetes, but the association of its gene variants with cardio-metabolic disorders in children has not been investigated. This study aimed to examine the relationship between LTF variants, plasma LTF concentrations, and cardio-metabolic...

journal_title：Pediatric research

authors： Marcil V,Mayeur S,Lamarche B,England J,Henderson M,Delvin E,Amre D,Levy E

更新日期：2017-11-01 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

authors： Kisker CT,Robillard JE,Clarke WR

更新日期：1982-01-01 00:00:00

abstract：BACKGROUND:In infants admitted to an ICU with respiratory failure, there is an association between the ratio of CD8+ to CD4+ T cells within the upper respiratory tract and disease severity. Whether this ratio is associated with respiratory disease severity within children presenting to a pediatric emergency department ...

journal_title：Pediatric research

authors： Cosgrove PR,Redhu NS,Tang Y,Monuteaux MC,Horwitz BH

更新日期：2021-01-27 00:00:00

abstract：:This study determines the inotropic effects of prostaglandin D2 (PGD2) and prostaglandin E1 (PGE1) in the isolated, arterial perfused newborn (NB) and adult (A) rabbit heart. Significant positive inotropism of PGD2 was observed at all concentrations studied (1 X 10(-17) to 1 X 10(-7) M) in the two age groups; the effe...

journal_title：Pediatric research

authors： Uemura S,Nakanishi T,Matsuoka S,Friedman WF,Jarmakani JM

更新日期：1984-12-01 00:00:00

abstract：BACKGROUND:Intranasal corticosteroids are the most efficacious anti-inflammatory medications for allergic rhinitis (AR). However, the efficacy and safety of intranasal corticosteroids in children have not yet been subject to specific research in China. The aim of this study was to investigate the efficacy and safety of...

journal_title：Pediatric research

authors： Zhang Y,Wei P,Chen B,Li X,Luo X,Chen X,Xiang M,Li L,Zhao S,Xiao X,Yang X,Chen J,Fu Y,Xiao S,Liu H,Cheng L,Yao H

更新日期：2020-10-02 00:00:00

abstract：BACKGROUND:Childhood body mass index (BMI) trajectory classes are rarely linked to early puberty risk, particularly among Chinese children. We estimated early puberty risk across BMI trajectory classes, investigated factors contributing to pubertal development, and examined differences in final adult height between chi...

journal_title：Pediatric research

authors： Fan HY,Lee YL,Hsieh RH,Yang C,Chen YC

更新日期：2020-07-01 00:00:00

abstract：:Left ventricular (LV) output nearly triples at birth, in association with increases in serum catecholamines. Similar increases in catecholamines in utero, however, do not increase output. We hypothesized that catecholamines increase contractility in utero, but that output cannot increase until LV loading conditions ar...

journal_title：Pediatric research

authors： Berning RA,Klautz RJ,Teitel DF

更新日期：1997-01-01 00:00:00

abstract：:The previous biochemical evidence had suggested that glutaric aciduria type II (GA II) is due to deficient dehydrogenation of multiple short-chain acyl coenzyme A's (CoA's), bu the precise biochemical mechanism underlying this disease was unknown. We investigated substrate oxidation and in vitro activities of isovaler...

journal_title：Pediatric research

authors： Rhead W,Mantagos S,Tanaka K

更新日期：1980-12-01 00:00:00