Abstract:
BACKGROUND:Histomorphometry of endomyocardial biopsies is one component of arrhythmogenic right ventricular cardiomyopathy (ARVC) diagnosis, although there is a need for stricter diagnostic criteria for this disease in pediatrics. The clinical utility of biopsy analysis as a component of ARVC diagnosis was evaluated in pediatric patients. METHODS:Histomorphometric analysis of fibrofatty infiltrate was completed on pediatric right ventricular endomyocardial biopsy samples. Myocardial replacement by fat and fibrosis was quantified. ARVC diagnosis was established using the 2010 ARVC Task Force criteria, with the biopsy measures compared across various ARVC diagnoses (definite, borderline, possible, or no ARVC). Receiver-operating characteristic (ROC) curve analysis was also completed using biopsy measures. RESULTS:The greatest proportion of fat, fibrosis, and myocardial replacement was in the definite ARVC cohort, and was significantly larger than for the other diagnosis cohorts. ROC curve analysis (with the biopsy analysis removed from the diagnostic classification) produced cutoff values of 15 and 25% myocardial replacement, which is lower than current adult diagnosis criteria. CONCLUSION:We propose modifications in pediatric major and minor biopsy diagnosis criteria to allow for improved sensitivity. This study suggests that biopsy analysis in children is most significant for subjects with a more severe disease presentation.
journal_name
Pediatr Resjournal_title
Pediatric researchauthors
Sreetharan S,MacIntyre CJ,Fatah M,Warren AE,Wilson GJ,Hamilton RMdoi
10.1038/s41390-018-0093-xsubject
Has Abstractpub_date
2018-10-01 00:00:00pages
552-557issue
4eissn
0031-3998issn
1530-0447pii
10.1038/s41390-018-0093-xjournal_volume
84pub_type
杂志文章abstract::Infantile Refsum's disease is characterized by high levels of phytanic acid and the absence of normal hepatic peroxisomes. We investigated the in vivo influence of phytol, a precursor of phytanic acid, on peroxisomes by both biochemical and morphological methods. Enhanced supply of phytol in the diet of adult mice cau...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198605000-00007
更新日期:1986-05-01 00:00:00
abstract:BACKGROUND:Biliary atresia (BA) is an infantile disorder characterized by progressive sclerosing cholangiopathy leading to biliary obstruction. First-line treatment of BA is hepatoportoenterostomy, the prognosis of which is related to age at surgery and to histological variables such as extent of fibrosis and ductular ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2013.177
更新日期:2014-01-01 00:00:00
abstract::The regionalization of pediatric services has resulted in differential access to care, sometimes creating barriers to those living in underserved, rural communities. These disparities in access contribute to inferior healthcare outcomes among infants and children. We review the medical literature on telemedicine and i...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2015.192
更新日期:2016-01-01 00:00:00
abstract::The etiology of the tall stature almost invariably seen in homocystinuric patients is not known. The effect of metabolic control and the role of the GH-IGF system on growth were investigated in 10 patients with homocystinuria. There was a direct correlation between the plasma free homocyst(e)ine and growth velocity SD...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200106000-00014
更新日期:2001-06-01 00:00:00
abstract::To examine the levels and relationship of blood pressure and insulin during puberty, blood pressure and serum insulin were measured in 3596 subjects, aged 3-18 y, whose pubertal status was graded according to the Tanner classification. The same study protocol was repeated in two follow-up surveys 3 and 6 y later for 2...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1203/00006450-199702000-00019
更新日期:1997-02-01 00:00:00
abstract::To determine the influence of an altered carotid body function on the laryngeal chemoreflex (LCR) response, reflex apnea was induced by laryngeal water stimulation during normoxia or acute hypoxia in unanesthetized awake lambs in which the ventilatory response to acute hypoxia was attenuated by prolonged postnatal hyp...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199312000-00024
更新日期:1993-12-01 00:00:00
abstract::Genetic variants of surfactant protein B (SP-B) have been associated with respiratory distress syndrome (RDS) in the prematurely born infant. We wished to determine linkage between RDS and SP-B single nucleotide polymorphisms (SNPs) [-18 (A/C), 1013 (A/C), 1580 (C/T), and 9306 (A/G)] or SP-B-linked microsatellite [(D2...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.pdr.0000203145.48585.2c
更新日期:2006-04-01 00:00:00
abstract::The factors that mediate the postnatal fall in pulmonary vascular resistance, which is crucial for normal gas exchange, are not fully understood. The endothelium has been implicated in this phenomenon, through the release of vasorelaxant factors such as nitric oxide (NO). Human pulmonary expression of endothelial NO s...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/01.PDR.0000159575.58834.8D
更新日期:2005-05-01 00:00:00
abstract::Inflammatory phenomena seem to contribute to the occurrence of perinatal cerebral white matter damage and CP. The stimulus that initiates the inflammation remains obscure. One thousand two hundred forty-six infants born before the 28th postmenstrual week had a protocol ultrasound scan of the brain read concordantly by...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/PDR.0b013e3181bf5fab
更新日期:2010-01-01 00:00:00
abstract::Immunologic consequences of exposure to HIV-1 in utero are still poorly understood. This study investigates relationships between type-1 [interferon-gamma (IFN-gamma)] and type-2 (IL-10) cytokine production and maternal-infant HIV-1 transmission. Cord blood leukocytes from deliveries of 71 HIV-1-infected and 11 uninfe...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-200109000-00018
更新日期:2001-09-01 00:00:00
abstract::BackgroundPost-hemorrhagic ventricular dilatation (PHVD) is predictive of mortality and morbidity among very-low-birth-weight preterm infants. Impaired cerebral blood flow (CBF) due to elevated intracranial pressure (ICP) is believed to be a contributing factor.MethodsA hyperspectral near-infrared spectroscopy (NIRS) ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2017.131
更新日期:2017-10-01 00:00:00
abstract::We have evaluated the effect of galanin (Gal), a newly identified hypothalamic peptide, on growth hormone (GH) secretion in 10 children with normal stature (NS), nine with constitutional growth delay (CGD), and five with isolated GH deficiency (IGHD). Gal was infused intravenously at a rate of 8 or 15 micrograms/kg/h....
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198910000-00006
更新日期:1989-10-01 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
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181975ee4
更新日期:2009-04-01 00:00:00
abstract::IL-8/CXC ligand (CXCL) 8 is ingested in high concentrations by the human fetus/neonate with amniotic fluid and human milk, and is also produced constitutively by intestinal epithelial cells (IEC). We have shown that recombinant human IL-8/CXCL8 (rhIL-8/CXCL8) protects cultured IEC against tumor necrosis factor (TNF)-a...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000133196.25949.98
更新日期:2004-08-01 00:00:00
abstract::In spontaneously breathing newborn lambs, alveolar hypoxia increases lung microvascular pressure, which causes lung lymph flow to increase and the concentration of protein in lymph to decrease. To see if this response derives from hypoxia itself rather than from the change in breathing pattern that occurs during hypox...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198405000-00009
更新日期:1984-05-01 00:00:00
abstract::Neurological injury is the primary lethal mechanism of injury in children, and the primary etiology of long-term disability after trauma. Laboratories and clinical/translational teams have sought to develop stem/progenitor cell therapies to improve recovery in a clinical setting in which there is no significant repara...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1038/pr.2017.253
更新日期:2018-01-01 00:00:00
abstract::Several markers of growth and biochemical development in the rat were studied after administration of prostacyclin (PGI2) and 16, 16-dimethyl prostaglandin E2 (16, 16DM PGE2). Intermittent administration of PGI2 for 3 days to 10- and 19-day-old animals, with subsequent sacrifice at 14 and 23 days, resulted in signific...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198307000-00004
更新日期:1983-07-01 00:00:00
abstract:BACKGROUND:The aim was to identify susceptibility alleles for infantile hypertrophic pyloric stenosis (IHPS) in a pedigree previously linked to IHPS5 on chromosome 16q24. METHODS:We screened the positional and functional candidate gene FOXF1 by Sanger sequencing in a single affected individual. All family members for ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/pr.2016.244
更新日期:2017-04-01 00:00:00
abstract:BACKGROUND:Recent studies suggest that infantile hypertrophic pyloric stenosis (IHPS) and congenital heart defects (CHDs) may share some genetic risk factors, but little is known about the co-occurrence of the two conditions in patients. METHODS:Our study cohort included 2,212,756 persons born in Denmark 1977-2013. We...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1038/s41390-019-0369-9
更新日期:2019-06-01 00:00:00
abstract::Congenital Central Hypoventilation Syndrome (CCHS) patients show partial retention of peripheral chemoreception despite impaired ventilatory responses to CO2 and hypoxia. The condition allows examination of central responses to hyperoxia, which minimizes afferent traffic from peripheral chemoreceptors. We used functio...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000155763.93819.46
更新日期:2005-04-01 00:00:00
abstract::The mitochondria play a major role in the regulation of intracellular calcium. Despite the fact that the enterocytes receive the majority of absorbed calcium, the role of the intestinal mitochondria in calcium transport during maturation is not known. Therefore, the current studies were designed to characterize calciu...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198901000-00023
更新日期:1989-01-01 00:00:00
abstract::The mechanism by which dexamethasone (DEX) inhibits neutrophil (PMN) recruitment to a site of inflammation, such as the newborn lung with bronchopulmonary dysplasia, is not completely understood. The aim of our study was to determine whether DEX inhibits neutrophil-induced neutrophil recruitment by inhibition of inter...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199910000-00008
更新日期:1999-10-01 00:00:00
abstract::Mucosal samples from rabbit jejunum were incubated (30 min, 25 degrees C) with (125I)glycinin in the presence of buffer, soybean lectin (50 micrograms/ml) soyasaponins (1 mg/ml), or both lectin and saponins. The mucosal uptake of (125I)glycinin was negligible with buffer, and increased progressively with additions of ...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-198209000-00005
更新日期:1982-09-01 00:00:00
abstract::Tracheal aspirate IL-8 concentration and airway epithelial cell IL-8 expression are each increased in premature infants undergoing mechanical ventilation. We sought to determine the cytokines responsible for IL-8 expression in this context. Tracheal aspirates were collected from 18 mechanically ventilated premature in...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/01.PDR.0000145274.47221.10
更新日期:2004-12-01 00:00:00
abstract::Pyritinol-HCl was tested for its impact on the cognitive functions of children with learning disabilities. This study is a contribution to scientific discussion on the complicated methodologic problems in evaluating the clinical efficacy of psychopharmacologic agents. Sixty-seven pupils of slow learner classes between...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-197509000-00006
更新日期:1975-09-01 00:00:00
abstract::We have previously discovered that the adult Drosophila melanogaster is tolerant to a low O2 environment, withstanding hours of total O2 deprivation without showing any evidence of cell injury. Subsequently, our laboratory embarked on the study of hypoxia tolerance using a mutagenesis and overexpression screens to beg...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e3181b27275
更新日期:2009-09-01 00:00:00
abstract::The results of prior studies suggest that abnormalities of development and function of the fetal adrenal occur in pregnancies complicated by intrauterine fetal growth retardation (IUGR). In the present investigation, we sought to extend such studies by matching IUGR infants with normally grown infants of women in whom...
journal_title:Pediatric research
pub_type: 临床试验,杂志文章
doi:10.1203/00006450-199406000-00003
更新日期:1994-06-01 00:00:00
abstract::Values of IGF-I after extraction, its binding proteins, and the high affinity GH-binding protein (BP) are not well established in pediatric patients with insulin-dependent diabetes mellitus (IDDM). We report data for IGF-I, IGFBP-1, and -3, and GHBP in 92 Spanish children with IDDM, separated according to pubertal sta...
journal_title:Pediatric research
pub_type: 杂志文章
doi:10.1203/00006450-199606000-00011
更新日期:1996-06-01 00:00:00
abstract::Oxygen is critical for multicellular existence. Its reduction to water by the mitochondrial electron transport chain helps supply the metabolic demands of human life. The incompletely reduced, reactive oxygen byproducts of this reaction, however, can be quite toxic. In this review, we explore the mechanisms responsibl...
journal_title:Pediatric research
pub_type: 杂志文章,评审
doi:10.1203/PDR.0b013e31818fc83f
更新日期:2009-03-01 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