Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This study describes 5 infants who were diagnosed with auditory neuropathy (AN) associated with severe to profound neural hearing loss shortly after birth. However, on repetition of the tests 7-12 months later, all infants showed full or partial recovery. The follow-up electrophysiological patterns were characterized by the appearance of wave I, followed by wave III and V, reflecting synchronization of auditory pathways and improvement in auditory nerve function. Suspected causative or contributory factors were neonatal hyperbilirubinemia, hypoxia, ischemia, and central nervous system immaturity, alone or in combination. These findings indicate that lack of an auditory brain stem response does not necessarily mean no hearing and that the situation where AN exists can improve. Thus, clinicians should be made aware that although cochlear implants may yield better auditory performance when applied early, they should be considered a therapeutic option only after repeated measures have proved persistent AN, and no child should be considered for an implant until a behavioral measure of hearing has been obtained.
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PMID:Transient deafness in young candidates for cochlear implants. 1753 1

Periventricular white matter (PVWM) injury is the leading cause of neurologic disability in survivors of prematurity. To address the role of ischemia in PVWM and cerebral cortical injury, we hypothesized that immaturity of spatially distal vascular 'end zones' or 'border zones' predisposes PVWM to greater decreases in cerebral blood flow (CBF) than more proximal structures. We quantified regional CBF with fluorescently labeled microspheres in 0.65 gestation fetal sheep in histopathologically defined three-dimensional regions by post hoc digital dissection and coregistration algorithms. Basal flow in PVWM was significantly lower than in gyral white matter and cortex, but was equivalent in superficial, middle, and deep PVWM. Absolute and relative CBF (expressed as percentage of basal) did not differ significantly during ischemia or reperfusion between PVWM, gyral white matter, or cortex. Moreover, CBF during ischemia-reperfusion was equivalent in three adjacent PVWM levels and was not consistent with the magnitude of severity of PVWM injury, defined by TUNEL (terminal deoxynucleotidyltransferase-mediated dUPT nick end labeling) staining. However, the magnitude of ischemia was predicted by the severity of discrete cortical lesions. Hence, unlike cerebral cortex, unique CBF disturbances did not account for the distribution of PVWM injury. Previously defined cellular maturational factors, thus, appear to have a greater influence on PVWM vulnerability to ischemic injury than the presence of immature vascular boundary zones.
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PMID:Cerebral blood flow heterogeneity in preterm sheep: lack of physiologic support for vascular boundary zones in fetal cerebral white matter. 1809 57

Necrotizing enterocolitis (NEC) is a clinical syndrome of ischemic necrosis of the bowel of multiple etiological factors that include the presence of intestinal ischemia, abnormal bacterial flora, and intestinal mucosal immaturity. Numerous reports have implied that the fecal microflora may contribute to the pathogenesis of NEC. A broad range of organisms generally found in the distal gastrointestinal tract have been recovered from the peritoneal cavity and blood of infants with NEC. The predominant organisms include Enterobacteriaceae (i.e., Escherichia coli, Klebsiella pneumoniae) , Clostridium spp., enteric pathogens (salmonellae, Coxsackie B2 virus, coronavirus, rotavirus), and potential pathogens (Bacteroides fragilis). The goals of the initial management is preventing ongoing damage, restoring hemostasis, and minimizing complications. Medical management includes withholding oral feeding, placement of nasogastric tube, abdominal decompression, paracentesis, vigorous intravenous hydration containing electrolytes and calories, support of the circulation, administration of antibiotics, and surveillance for deterioration or complications that require surgical intervention. Indications for surgery include clinical deterioration, perforation, peritonitis, obstruction, and abdominal mass. Prevention remains crucial to decrease the incidence of NEC. Preventive methods include cautious feeding regimens, the use of maternal breast milk, and the use of probiotics.
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PMID:Microbiology and management of neonatal necrotizing enterocolitis. 1823 62

Cerebral white matter injury, characterised by loss of premyelinating oligodendrocytes (pre-OLs), is the most common form of injury to the preterm brain and is associated with a high risk of neurodevelopmental impairment. The unique cerebrovascular anatomy and physiology of the premature baby underlies the exquisite sensitivity of white matter to the abnormal milieu of preterm extrauterine life, in particular ischaemia and inflammation. These two upstream mechanisms can coexist and amplify their effects, leading to activation of two principal downstream mechanisms: excitotoxicity and free radical attack. Upstream mechanisms trigger generation of reactive oxygen and nitrogen species. The pre-OL is intrinsically vulnerable to free radical attack due to immaturity of antioxidant enzyme systems and iron accumulation. Ischaemia and inflammation trigger glutamate receptor-mediated injury leading to maturation-dependent cell death and loss of cellular processes. This review looks at recent evidence for pathogenetic mechanisms in white matter injury with emphasis on targets for prevention and treatment of injury.
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PMID:Pathogenesis of cerebral white matter injury of prematurity. 1829 74

Oxidative stress (OS) is defined as an unbalance between prooxidant and antioxidant factors that can lead to cellular and tissue damage.The newborn, especially if preterm, is highly prone to OS and to the toxic effect of free radicals (FR). At birth, the newborn is exposed to a relatively hyperoxic environment caused by an increased oxygen bioavailability with greatly enhanced generation of FR. Additional sources (inflammation, hypoxia, ischemia, glutamate, and free iron release) occur magnifying OS. In the preterm baby, the perinatal transition is accompanied by the immaturity of the antioxidant systems and the reduced ability to induce efficient homeostatic mechanisms designed to control overproduction of cell-damaging FR. Improved understanding of the pathophysiological mechanism involved in perinatal brain lesions helps to identify potential targets for neuroprotective interventions, and the knowledge of these mechanisms has enabled scientists to develop new therapeutic strategies that have confirmed their neuroprotective effects in animal studies. Considering the growing role of OS in preterm newborn morbidity in respect to the higher risk of FR damage in these babies, a strict control of oxygen administration, lutein, melatonin, and hypothermia show great promise as potential neuroprotectants. This review provides an overview of the pathogenesis of free radical-mediated diseases of the newborn and the antioxidant strategies for now tested to reduce the OS and its damaging effects.
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PMID:Oxidative stress and antioxidant strategies in newborns. 2080 55

Cell transplantation has been proposed as a potential approach to the treatment of neurological disorders. One cell population of interest consists of human umbilical cord blood (hUCB) cells, which have previously been shown to be useful for reparative medicine in haematological diseases. However, hUCB cells are also capable of differentiating into various non-haematopoietic cells, including those of the neural lineage. Moreover, hUCB cells can secrete numerous neurotrophic factors and modulate immune function and inflammatory reaction. Several studies on animal models of ischemic brain injury have demonstrated the potential of hUCB cells to minimize damage and promote recovery after ischemic brain injury.This review focuses on the treatment of both stroke and perinatal hypoxic-ischemic brain injury using hUCB cells. We discuss the therapeutic effects demonstrated after hUCB cell transplantation and emphasize possible mechanisms counteracting pathophysiological events of ischemia, thus leading to the generation of a regenerative environment that allows neural plasticity and functional recovery. The therapeutic functional effects of hUCB cells observed in animal models make the transplantation of hUCB cells a promising experimental approach in the treatment of ischemic brain injury. Together with its availability, low risk of transplantation, immaturity of cells, and simple route of application, hUCB transplantation may stand a good chance of being translated into a clinical setting for the therapy of ischemic brain injury.
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PMID:Umbilical cord blood cell transplantation after brain ischemia--from recovery of function to cellular mechanisms. 2151 22

Progress in the development of rat models of human periventricular white matter injury (WMI) has been hampered by uncertainty about the developmental window in different rodent strains that coincides with cerebral white matter development in human premature infants. To define strain-specific differences in rat cerebral white matter maturation, we analyzed oligodendrocyte (OL) lineage maturation between postnatal days (P)2 and P14 in three widely studied strains of rat: Sprague-Dawley, Long-Evans and Wistar (W). We previously reported that late OL progenitors (preOL) are the major vulnerable cell type in human periventricular WMI. Strain-specific differences in preOL maturation were found at P2, such that the W rat had the highest percentage and density of preOL relative to the other strains. Overall, at P2, the state of OL maturation was similar to preterm human cerebral white matter. However, by P5, all three strains displayed a similar magnitude and extent of OL maturation that persisted with progressive myelination between P7 and P14. PreOL were the predominant OL lineage stage present in the cerebral cortex through P14, and thus OL lineage maturation occurred latter than in white matter. The hippocampus also displayed a later onset of preOL maturation in all three strains, such that OL lineage maturation and early myelination was not observed to occur until about P14. This timing of preOL maturation in rat cortical gray matter coincided with a similar timing in human cerebral cortex, where preOL also predominated until at least 8 months after full-term birth. These studies support that strain-specific differences in OL lineage immaturity were present in the early perinatal period at about P2, and they define a narrow window of preterm equivalence with human that diminishes by P5. Later developmental onset of preOL maturation in both cerebral cortex and hippocampus coincides with an extended window of potential vulnerability of the OL lineage to hypoxia-ischemia in these gray matter regions.
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PMID:Strain-specific differences in perinatal rodent oligodendrocyte lineage progression and its correlation with human. 2186 55

Necrotizing enterocolitis (NEC) is one of the most critical morbidities in preterm infants. The incidence of NEC is 7% in very-low-birth-weight infants, and its mortality is 15 to 30%. Infants who survive NEC have various complications, such as nosocomial infection, malnutrition, growth failure, bronchopulmonary dysplasia, retinopathy of prematurity, and neurodevelopmental delays. The most important etiology in the pathogenesis of NEC is structural and immunological intestinal immaturity. In preterm infants with immature gastrointestinal tracts, development of NEC may be associated with a variety of factors, such as colonization with pathogenic bacteria, secondary ischemia, genetic polymorphisms conferring NEC susceptibility, anemia with red blood cell transfusion, and sensitization to cow milk proteins. To date, a variety of preventive strategies has been accepted or attempted in clinical practice with regard to the pathogenesis of NEC. These strategies include the use of breast feeding, various feeding strategies, probiotics, prebiotics, glutamine and arginine, and lactoferrin. There is substantial evidence for the efficacy of breast feeding and the use of probiotics in infants with birth weights above 1,000 g, and these strategies are commonly used in clinical practice. Other preventive strategies, however, require further research to establish their effect on NEC.
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PMID:An update on necrotizing enterocolitis: pathogenesis and preventive strategies. 2223 29

Bacterial translocation as a direct cause of sepsis is an attractive hypothesis that presupposes that in specific situations bacteria cross the intestinal barrier, enter the systemic circulation, and cause a systemic inflammatory response syndrome. Critically ill children are at increased risk for bacterial translocation, particularly in the early postnatal age. Predisposing factors include intestinal obstruction, obstructive jaundice, intra-abdominal hypertension, intestinal ischemia/reperfusion injury and secondary ileus, and immaturity of the intestinal barrier per se. Despite good evidence from experimental studies to support the theory of bacterial translocation as a cause of sepsis, there is little evidence in human studies to confirm that translocation is directly correlated to bloodstream infections in critically ill children. This paper provides an overview of the gut microflora and its significance, a focus on the mechanisms employed by bacteria to gain access to the systemic circulation, and how critical illness creates a hostile environment in the gut and alters the microflora favoring the growth of pathogens that promote bacterial translocation. It also covers treatment with pre- and pro biotics during critical illness to restore the balance of microbial communities in a beneficial way with positive effects on intestinal permeability and bacterial translocation.
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PMID:Gut microbial translocation in critically ill children and effects of supplementation with pre- and pro biotics. 2293 15

Necrotizing enterocolitis (NEC) is a disease primarily of prematurity characterized by partial or entire gut necrosis and is associated with significant mortality and morbidity. Recent studies report that approximately 25% to 35% of very low-birth-weight infants less than 1500 g receiving packed red blood cell transfusions develop temporally associated NEC, known as transfusion-related NEC (TR-NEC). Although there are many known risk factors for NEC, this article focuses on 3 contributing factors: packed red blood cell transfusions, enteral feedings, and gastrointestinal immaturity. Previous data suggest that these factors may interact to affect neonatal intestinal tissue oxygenation, which may lead to tissue ischemia, resulting in intestinal injury. This article presents a conceptual framework that combines current theoretical perspectives for TR-NEC, and reviews previous research examining related variables and how their interaction may increase the risk for TR-NEC development. In addition, incorporation of the proposed framework to guide future research and nursing care in this area is discussed.
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PMID:Transfusion-related necrotizing enterocolitis: a conceptual framework. 2372 87


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