Medium chain acyl-CoA dehydrogenase deficiency in Pennsylvania: neonatal screening shows high incidence and unexpected mutation frequencies.

abstract：:Chenodeoxycholic acid labeled with 2H in the 11 and positions was prepared by catalytic reduction of delta 11-12 unsaturated derivatives of cholic acid. To validate the use of this stable isotope for the determination of bile acid kinetics by isotope dilution, it was administered to seven normal male volunteers simult...

journal_title：Pediatric research

authors： Balistreri WF,Cowen AE,Hofmann AF,Szczepanik PA,Klein PD

更新日期：1975-10-01 00:00:00

abstract：:The major variation in effect between undiluted cystic fibrosis and heterozygote sera on rabbit tracheal epithelium was in terms of the time after exposure at which changes occurred. Exposure to both types of sera for 3 min caused release of goblet cell mucus and ciliary resorption possibly by apical swelling of colum...

journal_title：Pediatric research

authors： Kennedy JR,Allen PL

更新日期：1980-11-01 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：BACKGROUND:Bicuspid aortic valve (BAV) is the most common type of congenital heart disease (CHD) and has a proposed genetic etiology. BAV is categorized by cusp fusion, with right-left (R-L) cusp fusion being associated with additional CHD, and right-noncoronary cusp (R-NC) fusion being associated with aortic valve dys...

journal_title：Pediatric research

authors： Bonachea EM,Chang SW,Zender G,LaHaye S,Fitzgerald-Butt S,McBride KL,Garg V

更新日期：2014-08-01 00:00:00

abstract：:The gastrointestinal (GI) barrier function is immature in the preterm neonate and might thus facilitate translocation of enteric bacteria and gut-derived septicemia. Circumstantial evidence suggests that bacterial uptake from the intestine may be further enhanced by an alteration of the host nutritional status. To tes...

journal_title：Pediatric research

authors： Steinwender G,Schimpl G,Sixl B,Kerbler S,Ratschek M,Kilzer S,Hollwarth ME,Wenzl HH

更新日期：1996-03-01 00:00:00

abstract：:Increased expression of CD38 on CD8(+) T cells is associated with activation of the immune system, progression of HIV disease, and death in adults. The prognostic significance of these cells in HIV-infected children, where the picture is complicated by age-related differences in CD38 expression, remains controversial....

journal_title：Pediatric research

authors： Sherman GG,Scott LE,Galpin JS,Kuhn L,Tiemessen CT,Simmank K,Meddows-Taylor S,Meyers TM

更新日期：2002-06-01 00:00:00

abstract：BACKGROUND:Cardiovascular instability immediately after birth is associated with intraventricular hemorrhage (IVH) in very-low-birth-weight (VLBW) infants. For circulatory management, evaluation of organ blood flow is important. In this study, the relationship between peripheral perfusion within 48 h after birth and IV...

journal_title：Pediatric research

authors： Ishiguro A,Suzuki K,Sekine T,Sudo Y,Kawasaki H,Itoh K,Kanai M,Kato I,Sobajima H,Tamura M

更新日期：2014-02-01 00:00:00

abstract：:Tonsillectomy and adenoidectomy (T&A) is a frequent surgical procedure in children with obstructive sleep apnea (OSA). Many symptomatic children who do not fulfill the currently recommended criteria for T&A may benefit from topical intranasal steroid therapy. However, the expression of glucocorticoid receptor (GCR) ex...

journal_title：Pediatric research

authors： Goldbart AD,Veling MC,Goldman JL,Li RC,Brittian KR,Gozal D

更新日期：2005-02-01 00:00:00

abstract：:Blood volume (BV), red cell mass (RCM; Cr-51) and plasma volume (125I-labeled albumin) were measured in 205 piglets from 28 litters shortly after birth. Spontaneous cord rupture in healthy piglets occurred during delivery (n = 25) or within 190 sec of birth (n = 82). Spontaneous and induced delay of cord rupture resul...

journal_title：Pediatric research

authors： Linderkamp O,Betke K,Güntner M,Jap GH,Riegel KP,Walser K

更新日期：1981-01-01 00:00:00

abstract：:The fetal and maternal concentration of various plasma proteins alters during pregnancy. Cells in the livers of fetal hamsters accumulate serum amyloid A (SAA) and C-reactive protein (CRP) mRNA, major acute phase reactants, when lipopolysaccharide is administered to the fetal circulation. No fetal SAA or CRP mRNA resp...

journal_title：Pediatric research

authors： Dowton SB,Waggoner DJ,Mandl KD

更新日期：1991-11-01 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

authors： Underwood MA,Gaerlan S,De Leoz ML,Dimapasoc L,Kalanetra KM,Lemay DG,German JB,Mills DA,Lebrilla CB

更新日期：2015-12-01 00:00:00

abstract：BACKGROUND:Protein intake may modulate cardiac structure and function in pathological conditions, but there is a lack of knowledge on potential effects in healthy infants. METHODS:Secondary analysis of an ongoing randomized clinical trial comparing two groups of infants receiving a higher (HP) or lower (LP) protein co...

journal_title：Pediatric research

authors： Collell R,Closa-Monasterolo R,Ferré N,Luque V,Koletzko B,Grote V,Janas R,Verduci E,Escribano J

更新日期：2016-06-01 00:00:00

abstract：:Very young children with organic brain damage, intractable seizures, and developmental retardation are at particular risk of developing fatal hepatic dysfunction coincident with valproate therapy, especially if the children are also receiving other anticonvulsant drugs. The mechanism of valproate-associated hepatic fa...

journal_title：Pediatric research

authors： Thurston JH,Hauhart RE

更新日期：1992-04-01 00:00:00

abstract：BACKGROUND:Following our clinical observation of tonic responses in response to the knee jerk in infants at very high risk for cerebral palsy (VHR infants), we systematically studied tonic responses, clonus, and reflex irradiation. We questioned (i) whether these responses occurred more often in VHR infants than in typ...

journal_title：Pediatric research

authors： Hamer EG,Dijkstra LJ,Hooijsma SJ,Zijdewind I,Hadders-Algra M

更新日期：2016-09-01 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

authors： Dix LM,van Bel F,Baerts W,Lemmers PM

更新日期：2013-11-01 00:00:00

abstract：:Zidovudine (azidothymidine, AZT) is used in pregnancy to reduce mother to infant transmission of HIV. Understanding the disposition of AZT in the fetus is necessary to optimize therapeutic regimens directed toward the fetus. Recent studies in primates found similar steady-state levels of the glucuronide metabolite of ...

journal_title：Pediatric research

authors： Garland M,Szeto HH,Daniel SS,Tropper PJ,Myers MM,Stark RI

更新日期：1998-07-01 00:00:00

abstract：:Critically ill infants are treated with dexamethasone (Dx) and other glucocorticoids to reduce inflammation and to promote lung and cardiac function. The neonatal immune system is immature, so neonatal dendritic cells (DCs) might be especially sensitive to glucocorticoid-mediated immunosuppression. To test this, we co...

journal_title：Pediatric research

authors： Mainali ES,Kikuchi T,Tew JG

更新日期：2005-07-01 00:00:00

abstract：:The perinatal ontogeny of preprotachykinin-A gene expression was assessed in central respiration-related structures. On the day of birth, there was an enhanced expression of preprotachykinin-A mRNA in the region of the nucleus tractus solitarii, the primary relay station for respiratory and cardiovascular reflexes. Th...

journal_title：Pediatric research

authors： Srinivasan M,Yamamoto Y,Persson H,Lagercrantz H

更新日期：1991-04-01 00:00:00

abstract：:The incidence and prevalence of celiac disease vary internationally. We studied the incidence of identified cases of childhood celiac disease in six Dutch provinces that cover 47.9% of the surface area and 67.6% of the total population of The Netherlands. Children with celiac disease aged 0-14 y, diagnosed from Januar...

journal_title：Pediatric research

authors： George EK,Mearin ML,van der Velde EA,Houwen RH,Bouquet J,Gijsbers CF,Vandenbroucke JP

更新日期：1995-02-01 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：:We have investigated the impaired secretion response of neonatal platelets. We compared the response of washed neonatal and adult platelets to thrombin and collagen, and to specific activators of calcium flux (inositol trisphosphate) and protein kinase C activation (oleoyl-acetyl glycerol). Neonatal platelets show no ...

journal_title：Pediatric research

authors： Israels SJ,Daniels M,McMillan EM

更新日期：1990-04-01 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 neonate conserves sodium avidly, and sodium intake is normally limited to that present in maternal milk. To evaluate the role of atrial natriuretic peptide (ANP) in this adaptation, preweaned rat pups were artificially reared and fed a formula with either normal sodium (25 mEq/L) or high sodium (145 mEq/L) for 7-8...

journal_title：Pediatric research

authors： Chevalier RL,Muchant DG,Thornhill BA,Belmonte DC,Pence RA,Baertschi AJ

更新日期：1996-08-01 00:00:00

abstract：:Kawasaki disease (KD) is an acute vasculitis of young childhood predominantly affecting the coronary arteries. IgA plasma cells have been found to infiltrate vascular and nonvascular tissues in fatal acute KD. To determine whether IgA B-lymphocytes were increased in the peripheral blood of patients with KD, we perform...

journal_title：Pediatric research

authors： Shingadia D,O'Gorman M,Rowley AH,Shulman ST

更新日期：2001-10-01 00:00:00

abstract：:The objective was to investigate any possible relationship between functional and structural vascular changes in women with low gestational age and/or low birth weight by analyzing the retinal vascular pattern in women with thoroughly documented blood pressure. Retinal vessel morphology was evaluated by digital image ...

journal_title：Pediatric research

authors： Kistner A,Jacobson L,Jacobson SH,Svensson E,Hellstrom A

更新日期：2002-06-01 00:00:00

abstract：:Rats were fed a 50% galactose diet during pregnancy and nursing, and the testes were later examined and hormone levels determined in male offspring. Exposure to galactose for various periods during pregnancy, throughout the entire gestation, or postnatally to nursing mother until pups were 5 wk of age produced no sign...

journal_title：Pediatric research

authors： Chen YT,Mattison DR,Bercu BB,Schulman JD

更新日期：1984-04-01 00:00:00

abstract：:In view of their known high incidence of noninsulin dependent diabetes (NIDDM), we sought to determine whether Native American (Plains Indian) children and adolescents show evidence of risk factors for both NIDDM and cardiovascular disease. Children and adolescents between the ages of 4 and 19 y were recruited, and fi...

journal_title：Pediatric research

authors： Blackett PR,Taylor T,Russell D,Lu M,Fesmire J,Lee ET

更新日期：1996-07-01 00:00:00

abstract：:Insulin treatment of prepubertal children with insulin-dependent diabetes improves body protein balance by decreasing the rate of protein degradation without stimulating protein synthesis. However, insulin also causes hypoaminoacidemia, so the inability of insulin to stimulate protein synthesis may have been limited b...

journal_title：Pediatric research

authors： Godil MA,Wilson TA,Garlick PJ,McNurlan MA

更新日期：2005-08-01 00:00:00

abstract：:The use of auditory brainstem response (ABR) for assessment of hearing in the neonate has not been without challenge. Although numerous articles have appeared, agreement regarding the utility of neonatal ABR testing does not exist. In review of the current studies and commentaries, a clear majority are favorable to ne...

journal_title：Pediatric research

authors： Cox LC

更新日期：1984-08-01 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