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)

Extremely low birth-weight newborns (<1000g) experience low levels of thyroid hormone that vary inversely with the severity of neonatal illness and the extent of developmental immaturity with levels reaching a nadir at approximate, equals7 days after birth; this phenomenon can persist for several weeks. In the absence of transplacental passage, 30-50% of these neonates cannot generate sufficient quantities of thyroid hormone to meet postnatal demands, placing them at an increased risk for developmental delay and cerebral palsy. Population surveys and interventional trials suggest that a therapeutic opening exists during a 'window of opportunity' corresponding to this period of diminished capacity. Variables to consider before intervention focus on the consideration that supplementation of both the substrate thyroxine and the active hormone triiodothyronine may be necessary in quantities that do not suppress thyroid-stimulating hormone release, yet overcome the persistence of increased conversion to 3,3'5'-triodo-L-thyronine, terminal deiodination, and activity of the sulfation inactivation pathways, as well as the diminished capacity of the newborn to accommodate postnatal physiologic changes. Single daily replacement doses may suppress levels of converting enzymes in the brain, suggesting that physiologic 'mimicry' provided by a constant infusion may be the preferred dosing option. Properly powered clinical trials targeting long-term developmental outcomes are needed to discern whether these interventions will do more than simply elevate blood levels of thyroid hormones to the target values of either the fetus or developing neonate. Identifying the appropriate indications for supplementation may alleviate individual pain and distress due to disability for several hundred extremely low birth-weight neonates each year in the US alone, and save society a pro-rated lifetime cost of nearly $US1 million per child.
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PMID:Neonatal thyroxine supplementation for transient hypothyroxinemia of prematurity : beneficial or detrimental? 1710 19

Our aim was to correlate an immunohistochemical pattern of selected podocyte cytoskeleton-associated proteins in children diagnosed with focal segmental glomerulosclerosis (FSGS) and diffuse mesangial proliferation accompanied by glomerular immaturity (Im-DMP) with the clinical courses of both diseases. The material included 33 renal biopsies obtained from children diagnosed with DMP with or without signs of glomerular immaturity (ten and 15 participants, respectively) or FSGS (eight patients). Ezrin, podocalyxin, synaptopodin and nephrin expression was evaluated by immunohistochemical assay. A positive reaction for ezrin, podocalyxin, synaptopodin and nephrin was observed in the most superficial, continuous 'layer' of podocytes in Im-DMP patients. This distribution closely mimicked the immunohistochemical pattern observed in FSGS. The severe initial course of Im-DMP was transient. Resistance to steroids (six children) and renal insufficiency (two patients) in these subjects subsided, whilst, in the FSGS patients, the resistance to steroids recognized in all the children and the renal insufficiency diagnosed in three of them were still present. Mimicry between the immunohistochemical pattern of glomerular immaturity in DMP and focal segmental glomerulosclerosis might explain the severe initial course of this nephrotic syndrome in children. The transient clinical character of the former may also indicate that it is not a variant of FSGS.
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PMID:Is mesangial hypercellularity with glomerular immaturity a variant of glomerulosclerosis? 1723 49

The potential for vaccines to act as triggers of autoimmune reactions has received much recent attention. Such an association is very poorly defined mechanistically, but may potentially involve epitope mimicry between vaccinal and self antigen, or the immuno-stimulatory effects of vaccine adjuvant. If such reactions occur, they are more likely to involve adults than infants in early life, as a reflection of the immunological immaturity of the newborn. There has been a recent focus in immunology on the link between innate and adaptive immunity provided by dendritic cells and the range of Toll-like receptors (TLRs) that are the point of first contact of these cells with microbial antigen. These interactions appear to determine the nature of the subsequent adaptive immune response and whether it may be mediated by Th1, Th2, Th17 or T regulatory populations. TLR interactions may also be significant in the induction of vaccinal immunity and agonists of these receptors are being developed as potential vaccine adjuvants. There are differences in cytokine production of adult and newborn dendritic cells, and these differences must be considered in the application of such novel adjuvants to products intended for either age group.
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PMID:From tolerance to autoimmunity: is there a risk in early life vaccination? 1754 92