Lysosomal cystine transport in cystinosis variants and their parents.

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

authors： Wallen LD,Perry SF,Alston JT,Maloney JE

更新日期：1990-02-01 00:00:00

abstract：:Branching morphogenesis, defined as growth and branching of epithelial tubules during embryogenesis, is a fundamental feature of renal, lung, mammary gland, submandibular gland, and pancreatic morphogenesis in mammals. Disruption of branching morphogenesis has been demonstrated to result in maldevelopment of some of t...

journal_title：Pediatric research

authors： Hu MC,Rosenblum ND

更新日期：2003-10-01 00:00:00

abstract：:Insulin administration is the primary therapy for type 1 diabetes mellitus (T1DM). Current available insulin therapies do not successfully enable children with T1DM to reach glycemic goals without side effects such as hypoglycemia and weight gain. Pramlintide is a synthetic analog of human amylin that acts in conjunct...

journal_title：Pediatric research

authors： Raman VS,Heptulla RA

更新日期：2009-04-01 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：:Bilirubin-IX alpha photooxidation products were detected by high performance liquid chromatography in the urine of neonates undergoing phototherapy for hyperbilirubinemia. The in vivo photoproducts were identified by chromatographic comparison with authentic synthetic standards using two complementary methods. Bilirub...

journal_title：Pediatric research

authors： Lightner DA,Linnane WP 3rd,Ahlfors CE

更新日期：1984-08-01 00:00:00

abstract：:This study tested the hypothesis that the sexually dimorphic adrenocortical response to stress is already established before birth. Chronically instrumented late gestation pregnant sheep carrying 16 male and 15 female age-matched singleton fetuses were subjected to an acute episode of hypoxic stress. Maternal and feta...

journal_title：Pediatric research

authors： Giussani DA,Fletcher AJ,Gardner DS

更新日期：2011-02-01 00:00:00

abstract：:The auditory brainstem response (ABR) was monitored in nine infant rhesus monkeys during the intravenous infusion of 50-168 mg/kg of unconjugated bilirubin. Sulfisoxazole (200 mg/kg) was sometimes given near the end of or just before the bilirubin infusion if no obvious ABR change had yet occurred. Five of the animals...

journal_title：Pediatric research

authors： Ahlfors CE,Bennett SH,Shoemaker CT,Ellis WG,Davis SL,Wennberg RP,Goetzman BW

更新日期：1986-06-01 00:00:00

abstract：:Clinical trials demonstrated decreasing rates of bronchopulmonary dysplasia in preterm infants with hypoxic respiratory failure treated with inhaled nitric oxide (iNO). However, the molecular and biochemical effects of iNO on developing human fetal lungs remain vastly unknown. By using a well-characterized model of hu...

journal_title：Pediatric research

authors： Johnston LC,Gonzales LW,Lightfoot RT,Guttentag SH,Ischiropoulos H

更新日期：2010-05-01 00:00:00

abstract：:To study the normal development of blood coagulation factor activities in a growing fetus while avoiding the effects of labor and delivery, a chronic fetal lamb model was developed in which serial blood samples from 10 fetuses were studied during the third trimester of pregnancy and 24 hr after birth. Under operating ...

journal_title：Pediatric research

authors： Kisker CT,Robillard JE,Clarke WR

更新日期：1981-07-01 00:00:00

abstract：:The present study investigates the effects of polycythemia on renal hemodynamics and function in 15 anesthetized newborn dogs, 2-10 days of age. Microspheres were used to study renal blood flow. Experimental animals received an exchange transfusion with adult dog packed red blood cells resulting in an increase in hema...

journal_title：Pediatric research

authors： Kotagal UR,Kleinman LI

更新日期：1982-02-01 00:00:00

abstract：:In the original article, the legend within Fig. 3 incorrectly read as '*p < 0.10, **p < 0.05, ***p > 0.01'. This has now been changed to '*p < 0.10, **p < 0.05, ***p < 0.01'. This has been corrected in both the PDF and HTML versions of the Article. The authors would like to apologise for this error. ...

journal_title：Pediatric research

authors： Benavides A,Metzger A,Tereshchenko A,Conrad A,Bell EF,Spencer J,Ross-Sheehy S,Georgieff M,Magnotta V,Nopoulos P

更新日期：2019-04-01 00:00:00

abstract：:Our goal for this study was to determine whether the maturation of fat absorption in neonatal life is functionally related to an increased ability to hydrolyze dietary fat, to absorb long-chain fatty acids, or to do both. In 16 preterm and in eight term neonates, the intestinal ability to hydrolyze triacylglycerols an...

journal_title：Pediatric research

authors： Rings EH,Minich DM,Vonk RJ,Stellaard F,Fetter WP,Verkade HJ

更新日期：2002-01-01 00:00:00

abstract：:Children with cystic fibrosis excreted elevated urinary levels of all three polyamines--putrescine, spermidine, and spermine. Heterozygote parents excreted intermediate concentrations of the polyamines, but not levels significantly different from levels in normal controls. Patients with cystic fibrosis who were admini...

journal_title：Pediatric research

authors： Russell DH,Rosenblum MG,Beckerman RC,Durie BG,Taussig LM,Barnett DR

更新日期：1979-10-01 00:00:00

abstract：:The microchemical study of growth cartilage biopsies may improve the classification and the genetic advice of some types of growth disturbances and contribute to the understanding of biochemical defects. Small tibial growth cartilage biopsies were performed during orthopedic surgery in cases with achondroplasia, pseud...

journal_title：Pediatric research

authors： Stanescu V,Maroteaux P

更新日期：1975-10-01 00:00:00

abstract：:Animal models with complex cortical development are useful for improving our understanding of the wide spectrum of neurodevelopmental challenges facing human preterm infants. MRI techniques can define both cerebral injury and alterations in cerebral development with translation between animal models and the human infa...

journal_title：Pediatric research

authors： Barnette AR,Neil JJ,Kroenke CD,Griffith JL,Epstein AA,Bayly PV,Knutsen AK,Inder TE

更新日期：2009-07-01 00:00:00

abstract：:Kinetic data are presented for the decarboxylation of branched chain alpha-ketoacids (BCKA) by intact human fibroblasts. Cultured cells of normal individuals and nine patients with different clinical pictures of maple syrup urine disease (MSUD) are studied with both alpha-ketoisocaproic acid (2-oxo-4-methylpentanoic a...

journal_title：Pediatric research

authors： Wendel U,Wentrup H,Rüdiger HW

更新日期：1975-09-01 00:00:00

abstract：:A four-year-old Israeli Arab boy was found to have glycogen accumulation in both liver and muscle without clinical symptoms. Liver phosphorylase kinase (PK) activity was 20% of normal, resulting in undetectable activity of phosphorylase a. Muscle PK activity was about 25% of normal, resulting in a marked decrease of p...

journal_title：Pediatric research

authors： Bashan N,Iancu TC,Lerner A,Fraser D,Potashnik R,Moses SW

更新日期：1981-04-01 00:00:00

abstract：BACKGROUND:Maternal thyroid deficiency can increase Rho-kinase procontractile influence in arteries of 2-week-old progeny. Here we hypothesized that augmented role of Rho-kinase persists in arteries from adult progeny of hypothyroid rats. METHODS:Dams were treated with 6-propyl-2-thiouracil (PTU) in drinking water (0....

journal_title：Pediatric research

authors： Gaynullina DK,Sofronova SI,Shvetsova AA,Selivanova EK,Sharova AP,Martyanov AA,Tarasova OS

更新日期：2018-07-01 00:00:00

abstract：BACKGROUND:Adult human airway smooth muscle (ASM) produce cytokines involved in recruitment and survival of leukocytes within airway walls. Cytokine generation by adult ASM is glucocorticoid-sensitive. Whether developing lung ASM produces cytokines in a glucocorticoid-sensitive fashion is unknown. METHODS:Cultured fet...

journal_title：Pediatric research

authors： Pearson H,Britt RD Jr,Pabelick CM,Prakash YS,Amrani Y,Pandya HC

更新日期：2015-12-01 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

authors： Keunen K,van der Burgh HK,de Reus MA,Moeskops P,Schmidt R,Stolwijk LJ,de Lange SC,Išgum I,de Vries LS,Benders MJ,van den Heuvel MP

更新日期：2018-12-01 00:00:00

abstract：:Sudden infant death syndrome (SIDS) is multifactorial and may result from the interaction of a number of environmental, genetic, and developmental factors. We studied three major genes causing long QT syndrome in 42 Japanese SIDS victims and found five mutations, KCNQ1-K598R, KCNH2-T895M, SCN5A-F532C, SCN5A-G1084S, an...

journal_title：Pediatric research

authors： Otagiri T,Kijima K,Osawa M,Ishii K,Makita N,Matoba R,Umetsu K,Hayasaka K

更新日期：2008-11-01 00:00:00

abstract：:Mutations in multidrug resistance 3 gene (MDR3 or ABCB4) underlie progressive familial intrahepatic cholestasis type 3 (PFIC3), a severe pediatric liver disease progressing to cirrhosis. Abcb4-/- mice exhibit slowly developing hepatic lesions that can be accelerated by feeding a cholic acid (CA)-supplemented diet. We ...

journal_title：Pediatric research

authors： Lamireau T,Bouchard G,Yousef IM,Clouzeau-Girard H,Rosenbaum J,Desmoulière A,Tuchweber B

更新日期：2007-02-01 00:00:00

abstract：OBJECTIVE:To determine associations of low superior vena cava (SVC) flow (≤55 ml/kg/min) and low right ventricular output (RVO) (≤150 ml/kg/min) in preterm infants. DESIGN/METHODS:An observational study in infants <30 weeks gestation randomized to receive immediate (<10 s) or delayed cord clamping (DCC) (≥60 s). RESU...

journal_title：Pediatric research

authors： Popat H,Robledo KP,Kirby A,Sebastian L,Evans N,Gill A,Kluckow M,Sinhal S,de Waal K,Tarnow-Mordi W,Osborn D

更新日期：2019-07-01 00:00:00

abstract：BACKGROUND:To understand the increased susceptibility of preterm neonates to infection. METHODS:A murine listeriosis model using immunohistochemistry, microarray technology, and real-time polymerase chain reaction (PCR). RESULTS:We report that recombinant serum amyloid A (SAA) administered prophylactically 18 h befor...

journal_title：Pediatric research

authors： Hawkins JS,Wu Q,Wang Y,Lu CY

更新日期：2013-12-01 00:00:00

abstract：BACKGROUND:Hurricane Maria struck Puerto Rico on 20 September 2017 causing catastrophic devastation. Prolonged shortage of food had been a substantial challenge to the residents after Maria. Experiencing food insecurity in utero has been associated with negative health outcomes later in life. We aim to examine whether ...

journal_title：Pediatric research

authors： Wang L,de Ángel Solá D,Acevedo Flores M,Schriefer A,Wang L,Gerónimo López K,Chang A,Warner B,Shan L,Holtz LR,Rosario Matos N

更新日期：2020-12-01 00:00:00

abstract：:Because minimal information is available about surfactant metabolism in bronchopulmonary dysplasia, we measured half-lives and pool sizes of surfactant phosphatidylcholine in very preterm baboons recovering from respiratory distress syndrome and developing bronchopulmonary dysplasia, using stable isotopes, radioactive...

journal_title：Pediatric research

authors： Janssen DJ,Carnielli VP,Cogo PE,Seidner SR,Luijendijk IH,Wattimena JL,Jobe AH,Zimmermann LJ

更新日期：2002-11-01 00:00:00

abstract：:Factors affecting bone turnover in premature infants are not entirely clear but certainly are different from those influencing bones of adults and children. To identify fetal and maternal factors that might influence bone turnover, we prospectively studied 50 infants (30 preterm and 20 full-term) born at Ain Shams Uni...

journal_title：Pediatric research

authors： Aly H,Moustafa MF,Amer HA,Hassanein S,Keeves C,Patel K

更新日期：2005-05-01 00:00:00

abstract：:Antigenic analysis of neuroblastoma cells was performed by autologous typing and indirect immunofluorescent test combined with absorption tests. Serum of a patient with neuroblastoma before treatment showed weak reactivity with cultured autologous neuroblastoma cells, while serum obtained from the same patient 3 month...

journal_title：Pediatric research

authors： Kawai K,Sakatoku H,Matsuda T,Kazuoka K,Kubo S,Kamiya H,Sakurai M

更新日期：1986-10-01 00:00:00

abstract：:The ontogenetic renal responsiveness to exogenous cortisol was examined in the chronically cannulated ovine fetus. The contribution of effects at proximal and distal tubule of the kidney were studied also. Cortisol (81.5 micrograms/h) was infused into immature ovine fetuses (mean gestational age -113.9 days) on five o...

journal_title：Pediatric research

authors： Towstoless MK,McDougall JG,Wintour EM

更新日期：1989-07-01 00:00:00

abstract：:Uteroplacental insufficiency and subsequent intrauterine growth retardation (IUGR) increase the risk of insulin resistance in humans and rats. Aberrant skeletal muscle lipid metabolism contributes to the pathogenesis of insulin resistance. Peroxisome proliferator-activated receptor-gamma co-activator-1 (PGC-1) is a tr...

journal_title：Pediatric research

authors： Lane RH,Maclennan NK,Daood MJ,Hsu JL,Janke SM,Pham TD,Puri AR,Watchko JF

更新日期：2003-06-01 00:00:00