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排序方式: 共有1135条查询结果,搜索用时 15 毫秒
101.
EM Maier J Pongratz AC Muntau B Liebl U Nennstiel-Ratzel U Busch R Fingerhut B Olgemöller AA Roscher W Röschinger 《Clinical genetics》2009,76(2):179-187
Medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD) represents a potentially fatal fatty acid β‐oxidation disorder. Newborn screening (NBS) by tandem mass spectrometry (MS/MS) has been implemented worldwide, but is associated with unresolved questions regarding population heterogeneity, burden on healthy carriers, cut‐off policies, false‐positive and negative rates. In a retrospective case‐control study, 333 NBS samples showing borderline acylcarnitine patterns but not reaching recall criteria were genotyped for the two most common mutations (c.985A>G/c.199C>T) and compared with genotypes and acylcarnitines of 333 controls, 68 false‐positives, and 34 patients. c.985A>G was more frequently identified in the study group and false‐positives compared to controls (1:4.3/1:2.3 vs. 1:42), whereas c.199C>T was found more frequently only within the false‐positives (1:23). Biochemical criteria were devised to differentiate homozygous (c.985A>G), compound heterozygous (c.985A>G/c.199C>T), and heterozygous individuals. Four false‐negatives were identified because our initial algorithm required an elevation of octanoylcarnitine (C8) and three secondary markers in the initial and follow‐up sample. The new approach allowed a reduction of false‐positives (by defining high cut‐offs: 1.4 μmol/l for C8; 7 for C8/C12) and false‐negatives (by sequencing the ACADM gene of few suspicious samples). Our validation strategy is able to differentiate healthy carriers from patients doubling the positive predictive value (42→88%) and to target NBS to MCADD‐subsets with potentially higher risk of adverse outcome. It remains controversial, if NBS programs should aim at identifying all subsets of all diseases included. Because the natural course of milder variants cannot be assessed by observational studies, our strategy could serve as a general model for evaluation of MS/MS‐based NBS. 相似文献
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Mazzucchelli R Lopez-Beltran A Cheng L Scarpelli M Kirkali Z Montironi R 《BJU international》2008,102(10):1369-1374
We review the clinicopathological features of the following unusual histological variants of prostatic carcinoma: small cell carcinoma, ductal adenocarcinoma, sarcomatoid (carcinosarcoma), basal cell, squamous cell and adenosquamous, and urothelial carcinoma. These variants are rare and account for 5-10% of carcinomas that originate in the prostate. Some develop from acinar adenocarcinoma after hormonal or radiation therapy. They are usually aggressive tumours that often present with secondary deposits. The outcome is generally poor. Only basal cell carcinoma is seen as a low-grade carcinoma. 相似文献
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Effects of cholinergic modulation on responses of neocortical neurons to fluctuating input 总被引:1,自引:0,他引:1
Neocortical neurons in vivo are spontaneously active and intracellular
recordings have revealed strongly fluctuating membrane potentials arising
from the irregular arrival of excitatory and inhibitory synaptic
potentials. In addition to these rapid fluctuations, more slowly varying
influences from diffuse activation of neuromodulatory systems alter the
excitability of cortical neurons by modulating a variety of potassium
conductances. In particular, acetylcholine, which effects learning and
memory, reduces the slow alterhyperpolarization, which contributes to spike
frequency adaptation. We used whole-cell patch-clamp recordings of
pyramidal neurons in neocortical slices and computational simulations to
show, first, that when fluctuating inputs were added to a constant current
pulse, spike frequency adaptation was reduced as the amplitude of the
fluctuations was increased. High- frequency, high-amplitude fluctuating
inputs that resembled in vivo conditions exhibited only weak spike
frequency adaptation. Second, bath application of carbachol, a cholinergic
agonist, significantly increased the firing rate in response to a
fluctuating input but minimally displaced the spike times by < 3 ms,
comparable to the spike jitter observed when a visual stimulus is repeated
under in vivo conditions. These results suggest that cholinergic modulation
may preserve information encoded in precise spike timing, but not in
interspike intervals, and that cholinergic mechanisms other than those
involving adaptation may contribute significantly to cholinergic modulation
of learning and memory.
相似文献
106.
Abstract Protein-chemical and molecular studies were conducted on all osteogenesis imperfecta (OI) type III/IV patients referred to our hospital during the last 15 y. Of a total of 16 OI type III/IV patients studied, 15 patients were heterozygous for a mutation in one of the two genes coding for collagen I, COL1A1 or COL1A2. Cultured fibroblasts from these 15 patients produced both normal and abnormal collagen I molecules, pointing to a dominant-negative effect of the mutation. Nine mutations had not been described previously. Parental mosaicism was demonstrated in three families. In the 16th child the causative mutation was not found. In conclusion, OI type III/IV in most patients of Western European ancestry is caused by dominant mutations in the genes for collagen I, and recurrence of OI is caused in most cases by parental gonadal mosaicism. 相似文献
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Impact of Early Initiation of Enteral Nutrition on Survival During Pediatric Extracorporeal Membrane Oxygenation
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Kristin C. Greathouse MS CPNP‐AC Kelly T. Sakellaris MS RD Dmitry Tumin PhD Jacob Katsnelson MD Joseph D. Tobias MD Don Hayes MD Jr. Andrew R. Yates MD 《JPEN. Journal of parenteral and enteral nutrition》2018,42(1):205-211
Introduction: Pediatric data related to safety, tolerance, and outcomes of enteral nutrition (EN) for patients requiring extracorporeal membrane oxygenation (ECMO) are lacking. The objectives of this study were to evaluate early nutrition status and timing of EN initiation on survival during pediatric ECMO. Methods: A single center institutional review board–approved retrospective chart review was performed on all pediatric patients requiring ECMO from October 2008 through December 2013. Demographics, ECMO variables, laboratory values, vasoactive inotropic score (VIS), and nutrition data on day 5 (d5) were collected. Patients receiving parenteral nutrition (PN) were compared with those receiving any EN on d5. Analyses were conducted to identify factors influencing survival to completion of ECMO and to discharge. Results: Forty‐nine patients aged 53 ± 76 months met inclusion criteria. Kaplan‐Meier curves demonstrated greater survival to discharge in patients receiving any EN, compared with only receiving PN (P = .031). EN on d5 of ECMO support (P = .040) and a higher percentage of daily energy intake achieved (P = .013) were protective, whereas a higher VIS was associated with increased mortality (P = .010). Multivariable analysis demonstrated EN was no longer associated with survival to discharge (P = .139), whereas energy intake (P = .021) and VIS (P = .013) remained significant. Conclusions: Pediatric patients who received nutrition that was closer to goal energy intake, as well as those who received any EN early during ECMO, had improved survival to hospital discharge. 相似文献