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)

In children with congenital heart disease pulmonary vascular disease can be fatal for a variety of reasons. Even before the classical changes of advanced pulmonary vascular obstructive disease have developed, a marked increase in pulmonary vascular smooth muscle can be fatal due to pulmonary hypertensive crises. After the Fontan procedure, a modest increase in muscularity can jeopardise the outcome since there is no subpulmonary ventricle to support the pulmonary circulation. Following heart transplantation, a slight increase in muscularity can cause failure of the donor right ventricle unless that heart is already hypertrophied as in the domino procedure. In all children with pulmonary hypertension, either persistent pulmonary hypertension of the newborn or secondary to congenital heart disease the pulmonary vasculature fails to remodel normally after birth. Newborn vessels are characterized by the immaturity of the smooth muscle cells and the paucity of connective tissue. In the hypertensive lung smooth muscle differentiation and connective tissue deposition is accelerated. In children with congenital heart disease intimal changes follow. In these children the potential reversibility of disease following intracardiac repair is determined by the type of pathological change present at the time of repair. However, pulmonary hypertensive crises can occur in young children with potentially reversible disease. Operability is not synonymous with the potential reversibility of pathological lesions. Correlations between structural findings at lung biopsy and haemodynamic findings at cardiac catheterization have improved the accuracy with which the natural and unnatural history of pulmonary vascular disease can be predicted, but is still inadequate because we do not understand the functional implications of the changes.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pathophysiological and metabolic manifestations of pulmonary vascular disease in children. 139 33

Six basic mechanisms facilitating development of chronic placental deficiency, hypotrophy and fetal death are distinguished: 1) insufficiency of invasion of the extra-villous cytotrophoblast into the placental bed resulting in incomplete gestational restructuring of spiral arteries and reduction of uteroplacental circulation; 2) rheological disturbances in the intervillous space due to ultrastructural pathology of the glycocalyx and microvilli of the cytiotrophoblast; 3) pathological immaturity of microvilli and retraction of the diffusion surface; 4) disturbances of the villi perfusion resulting from prevailing development of the connective tissue components, reduction of stroma capillary bed and obliteration angiopathy at the level of supporting villi and umbilical cord; 5) placental barrier pathology; 6) endocrine deficiency resulting from the deficiency in the synthesis of human chronic gonadotropin during the first and, in part, second trimesters and the shortage of placental lactogen at the end of pregnancy.
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PMID:[Basic pathogenetic mechanisms of chronic placental insufficiency]. 852 48

Central nervous system vascular disorders in the neonate comprise structural anomalies or malformations of arteries and veins and physiologic alterations of cerebral blood flow, which can mimic structural vascular disease. Clinical , imaging, and transcatheter therapeutic aspects of neonatal cerebral vascular malformations are described. Symptomatic high-flow vascular malformations characteristically present with cardiac failure and associated systemic problems in the neonate, whereas infants typically present with macrocephaly and hydrocephalus and older children with hemorrhage, developmental delay, or focal deficits. Neonatal cerebral hemorrhage is typically primary or associated with immaturity, parturitional trauma, or coagulopathy. Likewise, cerebral ischemic lesions are more likely secondary to hypoxic, ischemic events than to thromboembolic or structural cerebrovascular occlusive disease. The role of the current noninvasive imaging modalities in each of these clinical problems is reviewed and illustrated.
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PMID:Neonatal central nervous system vascular disorders. 940 72

Recent research has recognised new populations of non-hematopoietic cells in the blood. One of these, circulating endothelial cells (CECs), often defined by the expression of membrane glycoprotein CD146, are rarely found in the blood in health, but raised numbers are present in a wide variety of human conditions, including inflammatory, immune, infectious, neoplastic and cardiovascular disease, and seem likely to be evidence of profound vascular insult. An additional population are endothelial progenitor cells, defined by the co-expression of endothelial and immaturity cell surface molecules and also by the ability to form colonies in vitro. Although increased numbers of CECs correlate with other markers of vascular disease, questions remain regarding the precise definition, cell biology and origin of CECs. For example, they may be damaged, necrotic or apopototic, or alive, and could possess procoagulant and/or proinflammatory properties. However, since these cells seem to be representative of in situ endothelium, their phenotype may provide useful information. Indeed, whatever their phenotype, there is growing evidence that CECs may well be a novel biomarker, the measurement of which will have utility in various clinical settings related to vascular injury. Despite this promise, progress is impeded by the diversity of methodologies used to detect these cells. Accordingly, results are sometimes inconclusive and even conflicting. Nevertheless, increased CECs predict adverse cardiovascular events in acute coronary syndromes, suggesting they may move from being simply a research index to having a role in the clinic. The objective of the present communication is to condense existing data on CECs, briefly compare them with progenitor cells, and summarise possible mechanism(s) by which they may contribute to vascular pathology.
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PMID:Circulating endothelial cells. Biomarker of vascular disease. 1571 37

Due to transient gut immaturity, most very preterm infants receive parenteral nutrition (PN) in the first few weeks of life. Yet providing enough protein and energy to sustain optimal growth in such infants remains a challenge. Extrauterine growth restriction is frequently observed in very preterm infants at the time of discharge from hospital, and has been found to be associated with later impaired neurodevelopment. A few recent randomized trials suggest that intensified PN can improve early growth; whether or not such early PN improves long-term neurological outcome is still unclear. Several other questions regarding what is optimal PN for very preterm infants remain unanswered. Amino acid mixtures designed for infants contain large amounts of branched-chain amino acids and taurine, but there is no consensus on the need for some nonessential amino acids such as glutamine, arginine, and cysteine. Whether excess growth in the first few weeks of life, at a time when very preterm infants receive PN, has an imprinting effect, increasing the risk of metabolic or vascular disease at adulthood continues to be debated. Even though uncertainty remains regarding the long-term effect of early PN, it appears reasonable to propose intensified initial PN. The aim of the current position paper is to review the evidence supporting such a strategy with regards to the early phase of nutrition, which is mainly covered by parenteral nutrition. More randomized trials are, however, needed to further support this type of approach and to demonstrate that this strategy improves short- and long-term outcome.
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PMID:Parenteral nutrition for preterm infants: Issues and strategy. 2965 25