Steroid sulfatase deficiency.

abstract：BACKGROUND:Risk factors for diastolic dysfunction in hypertrophic cardiomyopathy (HCM) are poorly understood. We investigated the association of variants in hypoxia-response genes with phenotype severity in pediatric HCM. METHODS:A total of 80 unrelated patients <21 y and 14 related members from eight families with HC...

journal_title：Pediatric research

authors： Alkon J,Friedberg MK,Manlhiot C,Manickaraj AK,Kinnear C,McCrindle BW,Benson LN,Addonizio LJ,Colan SD,Mital S

更新日期：2012-12-01 00:00:00

abstract：:Recently a soybean oil emulsion (Intralipid) (IL) has been released in the United States for use as a parenteral nutrient. The study reported here was undertaken to determine the effect of ingestion of IL on the synthesis and secretion of the second (C2) and fourth (C4) components of complement by guinea pig peritonea...

journal_title：Pediatric research

authors： Strunk RC,Kunke K,Nagle RB,Payne CM,Harrison HR

更新日期：1979-03-01 00:00:00

abstract：:Early childhood overweight and obesity have alarmingly increased over the years. Adulthood obesity is a well demonstrated significant independent predictor of cardiovascular risk (CVR) and/or mortality, which predisposes to the major components of metabolic syndrome (MS). Evidence of MS in obese children has been also...

journal_title：Pediatric research

authors： Aggoun Y

更新日期：2007-06-01 00:00:00

abstract：:After the infusion of fructose, 0.25 g/kg body wt, the mean peak plasma uric acid level was 5.4 +/- 0.7 (SEM) mg/100 ml in six normal children and was not significantly increased compared with that of the mean basal value of 4.1 +/- 0.5 mg/100 ml. The mean blood inorganic phosphate (Pi) levels were significantly less ...

journal_title：Pediatric research

authors： Kogut MD,Roe TF,Ng W,Nonnel GN

更新日期：1975-10-01 00:00:00

abstract：:Television viewing (TVV) is considered a contributing factor to the development of childhood obesity yet it is unclear whether obesity results, in part, from increased energy intake during TVV. The objective of this study was to determine the effect of TVV on food intake (FI) of boys at a meal and its effect on calori...

journal_title：Pediatric research

authors： Bellissimo N,Pencharz PB,Thomas SG,Anderson GH

更新日期：2007-06-01 00:00:00

abstract：:Endothelin-1 can cause pulmonary vasoconstriction via endothelin-A (ET(A)) receptor activation. We hypothesized that ET(A) blockers (EMD 122946 and BQ 610) would reduce hypoxia-induced (HYP) but not group B streptococcal infusion (GBS)-induced pulmonary hypertension in a juvenile whole animal model. Pulmonary hyperten...

journal_title：Pediatric research

authors： Ambalavanan N,Philips JB 3rd,Bulger A,Oparil S,Chen YF

更新日期：2002-12-01 00:00:00

abstract：:Preterm infants are exposed to frequent painful procedures and agitating stimuli over the many weeks of their hospitalization in the neonatal intensive care unit (NICU). The adverse neurobiological impact of pain and stress in the preterm infant has been well documented, including neuroimaging and neurobehavioral outc...

journal_title：Pediatric research

authors： McPherson C,Miller SP,El-Dib M,Massaro AN,Inder TE

更新日期：2020-08-01 00:00:00

abstract：:A double-label, double-substrate incubation technique has been developed and used to study the conversion of progesterone to testosterone in testes extracts from incompletely virilized males. The procedure involves separation of the microsomes from a testicular homogenate, incubating the microsomes with 1 microM [7-3H...

journal_title：Pediatric research

authors： Chasalow FI,Blethen SL,Marr HB,French FS

更新日期：1984-08-01 00:00:00

abstract：BACKGROUND:A lack of consensus exists as to the timing of kidney biopsy in children with steroid-dependent nephrotic syndrome (SDNS) where minimal change disease (MCD) predominates. This study aimed at examining the applicability of a biomarker-assisted risk score model to select SDNS patients at high risk of focal seg...

journal_title：Pediatric research

authors： Chan CY,Resontoc LP,Qader MA,Chan YH,Liu ID,Lau PY,Than M,Yeo WS,Loh AH,Tan PH,Wei C,Reiser J,Biswas SK,Ng KH,Yap HK

更新日期：2019-03-01 00:00:00

abstract：BACKGROUND:A growing body of evidence defines inflammation as a hallmark feature of disease pathogenesis of Duchenne muscular dystrophy. To tailor potential immune modulatory interventions, a better understanding of immune dysregulation in Duchenne muscular dystrophy is needed. We now asked whether dystrophin deficienc...

journal_title：Pediatric research

authors： Kranig SA,Tschada R,Braun M,Patry C,Pöschl J,Frommhold D,Hudalla H

更新日期：2019-08-01 00:00:00

abstract：:The immunological competence of neonatal rabbits inoculated intradermally with Treponema pallidum was examined. Both cellular responses and the production of humoral antibody to specific T. pallidum antigens and to nonspecific antigens or mitogens were investigated. In blast transformation assays, splenic and poplitea...

journal_title：Pediatric research

authors： Gamboa D,Miller JN,Lukehart SA,Baker-Zander SA,Sell S

更新日期：1984-10-01 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

authors： Crofton PM,Ahmed SF,Wade JC,Elmlinger MW,Ranke MB,Kelnar CJ,Wallace WH

更新日期：2000-10-01 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：:Nitric oxide plays a major role in vascular tone control. Increased blood levels of bradykinin (BK), which stimulates nitric oxide biosynthesis, occur at birth. BK effects on ductus arteriosus (DA) tone were investigated in fetal rabbit under fetal (2.5% O2 "low PO2") and neonatal (30% O2 "high PO2") conditions using ...

journal_title：Pediatric research

authors： Bateson EA,Schulz R,Olley PM

更新日期：1999-04-01 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:Pediatric myocarditis is a rare disease with substantial mortality. Little is known regarding its prognostic factors. We hypothesize that certain comorbidities and procedural needs may increase risks of poor outcomes. This study aims to identify prognostic factors for pediatric myocarditis. METHODS:The nati...

journal_title：Pediatric research

authors： Chou FS,Ghimire LV

更新日期：2020-11-18 00:00:00

abstract：:To date, many mutations, including intronic nucleotide changes, in the SLC12A3 gene encoding the thiazide-sensitive sodium-chloride cotransporter (NCCT) have been reported in Gitelman's syndrome (GS) patients. However, it has not been clarified whether intronic nucleotide changes affect mRNA content. Since mRNA analys...

journal_title：Pediatric research

authors： Kaito H,Nozu K,Fu XJ,Kamioka I,Fujita T,Kanda K,Krol RP,Suminaga R,Ishida A,Iijima K,Matsuo M

更新日期：2007-04-01 00:00:00

abstract：:The mononuclear cells of newborn infants compared to those of normal adults contain diminished activity of pyruvate kinase (PK) (14.9 +/- 1.2 vs. 22.3 +/- 1.3 mumol/10(10) cells/min) and phosphoglycerate kinase (PGK) (195 +/- 10 vs. 415 +/- 19). These deficiencies are accentuated in the cells of premature infants (4.9...

journal_title：Pediatric research

authors： Das M,Klein W,Feig SA

更新日期：1977-10-01 00:00:00

abstract：:Active compression-decompression (ACD) cardiopulmonary resuscitation (CPR) with the inspiratory threshold valve (ITV) has been recently recommended by the American Heart Association for treatment of adults in cardiac arrest (class IIb: alternative, useful intervention), but this new technique has never been used in a ...

journal_title：Pediatric research

authors： Voelckel WG,Lurie KG,Sweeney M,McKnite S,Zielinski T,Lindstrom P,Peterson C,Wenzel V,Lindner KH

更新日期：2002-04-01 00:00:00

abstract：BACKGROUND:Pulmonary hypertension (PH) in patients with bronchopulmonary dysplasia (BPD) results from vasoconstriction and/or vascular remodeling, which can be regulated by mitogen-activated protein kinases (MAPKs). MAPKs are deactivated by dual-specificity phosphatases (DUSPs). We hypothesized that single-nucleotide p...

journal_title：Pediatric research

authors： Chen LL,Zmuda EJ,Talavera MM,Frick J,Brock GN,Liu Y,Klebanoff MA,Trittmann JK

更新日期：2020-01-01 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

authors： Treszl A,Szabo M,Dunai G,Nobilis A,Kocsis I,Machay T,Tulassay T,Vasarhelyi B

更新日期：2003-11-01 00:00:00

abstract：:An increase in polymorphonuclear leukocytes (PMNs) and proinflammatory chemokines, such as IL-8 and macrophage inflammatory protein-1 alpha (MIP), are found in the airways during early stages of bronchopulmonary dysplasia. We determined whether IL-10 produces a dose-related inhibition of proinflammatory chemokine rele...

journal_title：Pediatric research

authors： Tryzmel J,Miskolci V,Castro-Alcaraz S,Vancurova I,Davidson D

更新日期：2003-09-01 00:00:00

abstract：:Necrotizing enterocolitis (NEC) is a devastating intestinal disease of premature infants. Maternal milk has been suggested to be partially protective against NEC; however, the mechanisms of this protection are not defined. The aim of this study was to examine the effect(s) of artificial feeding of rat milk (RM)-versus...

journal_title：Pediatric research

authors： Dvorak B,Halpern MD,Holubec H,Dvorakova K,Dominguez JA,Williams CS,Meza YG,Kozakova H,McCuskey RS

更新日期：2003-03-01 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：: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：:Superoxide anion generation during severe asphyxia and reventilation was assessed in newborn pigs. Using closed cranial windows over the parietal cortices, superoxide dismutase (SOD)-inhibitable nitroblue tetrazolium (NBT) reduction was determined during asphyxia/reventilation. Asphyxia was induced by turning off the ...

journal_title：Pediatric research

authors： Pourcyrous M,Leffler CW,Mirro R,Busija DW

更新日期：1990-12-01 00:00:00

abstract：BACKGROUND:Gender is increasingly recognized as an important influence on brain development, disease susceptibility, and response to pharmacologic/rehabilitative treatments. In regenerative medicine, it remains entirely unknown whether there is an interaction between transplanted stem cells and host gender that might b...

journal_title：Pediatric research

authors： Ashwal S,Ghosh N,Turenius CI,Dulcich M,Denham CM,Tone B,Hartman R,Snyder EY,Obenaus A

更新日期：2014-05-01 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：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：:We investigated whether obese children and adolescents have early echocardiographic signs of subclinical cardiac dysfunction and evaluated the respective influence of obesity per se versus parameters of carbohydrate and lipid metabolism that are frequently abnormal in obese subjects. The role of tissue Doppler imaging...

journal_title：Pediatric research

authors： Van Putte-Katier N,Rooman RP,Haas L,Verhulst SL,Desager KN,Ramet J,Suys BE

更新日期：2008-08-01 00:00:00