Immune tolerance induction (ITI) in haemophilia B patients with inhibitor should be carefully considered because of the relatively poor (25%) overall success rate and the high risk of complications. ITI in combination with an immunosuppressive treatment was started in two children with haemophilia B with factor IX (FIX) inhibitor. To avoid anaphylactic reactions and inhibitor boost, the FIX replacement therapy was stopped and patients received a treatment with recombinant activated factor VII (rFVIIa).
View Article and Find Full Text PDFObjectives: Our goal was to analyze the association between human milk intake and severe retinopathy of prematurity in extremely low birth weight infants.
Patients And Methods: This study is a secondary analysis of data collected for a trial of glutamine supplementation in extremely low birth weight infants (birth weight <1000 g). Among the 1433 participants in that trial, data are available regarding human milk intake and the occurrence of severe retinopathy of prematurity (defined in this study as retinopathy of prematurity treated surgically) for 1057 infants.
Background: The relative importance of previous diagnosis and hereditary prothrombotic risk factors for cerebral venous thrombosis (CVT) in children in determining risk of a second cerebral or systemic venous thrombosis (VT), compared with other clinical, neuroimaging, and treatment variables, is unknown.
Methods: We followed up the survivors of 396 consecutively enrolled patients with CVT, aged newborn to 18 years (median 5.2 years) for a median of 36 months (maximum 85 months).
The prevalence of Escherichia coli O157:H7 on beef subprimal cuts intended for mechanical tenderization was evaluated. This evaluation was followed by the assessment of five antimicrobial interventions at minimizing the risk of transferring E. coli O157:H7 to the interior of inoculated subprimal cuts during blade tenderization (BT) or moisture enhancement (ME).
View Article and Find Full Text PDFFor years, patients who were treated successfully with carpal tunnel release were told there was nothing that could be done about their lower extremity symptoms. Now that lower extremity nerve decompression has been accepted as an option to treat appropriate patients, the authors looked for correlations between a successful outcome with carpal tunnel syndrome and its predictive value of success for lower extremity nerve decompression. Data from a recent study demonstrate that a good result from upper extremity peripheral nerve surgery predicts the outcome for lower extremity peripheral nerve surgery in 88% of patients, and is, therefore, information valuable for prognosis and clinical decision-making.
View Article and Find Full Text PDFIn lieu of traditional training of examiners to identify cerebral palsy on a neurologic examination at age 1 year, we proposed an alternative approach using a multimedia training video and CD-ROM we developed after a two-step validation process. We hypothesized that use of CD-ROM interactive training will lead to reliable and valid performance of the neurologic examination by both pediatric neurologists and nonpediatric neurologists. All examiners were asked to take one of six interobserver variability tests found on the CD-ROM on two occasions.
View Article and Find Full Text PDFThe goal of the Complex Trait Consortium is to promote the development of resources that can be used to understand, treat and ultimately prevent pervasive human diseases. Existing and proposed mouse resources that are optimized to study the actions of isolated genetic loci on a fixed background are less effective for studying intact polygenic networks and interactions among genes, environments, pathogens and other factors. The Collaborative Cross will provide a common reference panel specifically designed for the integrative analysis of complex systems and will change the way we approach human health and disease.
View Article and Find Full Text PDFIsolated ischemia of the cervical spinal cord is an uncommon but increasingly recognized complication of vertebral artery dissection (VAD). The authors report a young patient with congenital afibrinogenemia, who developed VAD with extensive unilateral spinal cord infarction, probably caused by local compression of spinal radicular feeders at their origin by vertebral artery hematoma.
View Article and Find Full Text PDFThe present study was designed to evaluate prothrombotic risk profiles in 59 consecutively recruited white neonates with renal venous thrombosis (RVT). The rates of prothrombotic risk factors (PRs)-for example, the factor V (FV) 1691G> A mutation, the factor II (FII) 20210G> A variant, antithrombin (AT), protein C (PC), protein S (PS), elevated lipoprotein(a) (Lp(a)), total fasting plasma homocysteine (tHcy) levels, and anticardiolipin antibodies (ACAs)-were compared with those of 118 healthy control children. At onset, 32 (54.
View Article and Find Full Text PDFElevated total homocysteine (tHcy) concentrations are an independent risk factor for thromboembolic events in adults. In children with moderate hyperhomocysteinemia data are sparse. Therefore, between 1995 and 2002 we consecutively recruited 163 white pediatric patients with a first symptomatic thromboembolic event and 255 healthy controls (mean age: 6.
View Article and Find Full Text PDFBackground And Purpose: The present study was performed to evaluate the rate of recurrent symptomatic thromboembolism with respect to prothrombotic risk factors and underlying clinical conditions.
Methods: In a series of 215 consecutively enrolled neonates with arterial ischemic stroke (AIS), the factor V G1691A mutation, factor II G20210A variant, methylenetetrahydrofolate reductase (MTHFR) T677T genotype, lipoprotein (Lp) (a), antithrombin, protein C, protein S, and anticardiolipin antibodies (ACA) were investigated. Patient median follow-up was 3.
Background: The present study was performed to assess the association of prothrombotic risk factors and underlying conditions (infections, vascular trauma, immobilization, malignancies, autoimmune diseases, renal diseases, metabolic disorders, obesity, birth asphyxia, cardiac malformations, and use of prothrombotic drugs) with cerebral venous thrombosis (CVT) in children.
Methods And Results: From 1995 to 2002, 149 pediatric patients aged newborn to <18 years (median 6 years) with CVT were consecutively enrolled. In patients and in 149 age- and gender-matched children with similar underlying clinical conditions but without CVT, the factor V G1691A mutation, the factor II G20210A variant, lipoprotein(a) [Lp(a)], protein C, protein S, antithrombin, and antiphospholipid antibodies, as well as associated clinical conditions, were investigated.
Over 225,000 independent Agrobacterium transferred DNA (T-DNA) insertion events in the genome of the reference plant Arabidopsis thaliana have been created that represent near saturation of the gene space. The precise locations were determined for more than 88,000 T-DNA insertions, which resulted in the identification of mutations in more than 21,700 of the approximately 29,454 predicted Arabidopsis genes. Genome-wide analysis of the distribution of integration events revealed the existence of a large integration site bias at both the chromosome and gene levels.
View Article and Find Full Text PDFThe development of inhibitors is currently one of the most serious complications in the treatment of hemophilic children. Prospective studies of previously untreated patients (PUP) showed that up to 52% of patients with severe hemophilia A developed inhibitors during the first 50 exposure days (ED) (>100 for outliers). Inhibitor development is influenced by the type of hemophilia, the severity and the type of mutation.
View Article and Find Full Text PDFIn neonates and infants, numerous clinical and environmental conditions lead to elevated thrombin generation and subsequent thrombus formation. Genetic prothrombotic defects (protein C, protein S and antithrombin deficiency, mutations of coagulation factor V and factor II, elevated lipoprotein (a)) have been established as risk factors of thromboembolic events in neonates and infants. The interpretation of the laboratory evaluation relies on age-dependent normal reference values.
View Article and Find Full Text PDFTo resolve ambiguity in the literature about the anatomy of the "trochanteric bursa" or trochanteric subgluteus maximus bursa, this study examines the constancy, structure, and relationships of this bursa in a series of anatomical dissections of the hip. Sixteen embalmed hip specimens, from subjects aged 63-91 years, were examined. Subgluteus maximus bursae were demonstrated in 13 hips.
View Article and Find Full Text PDFGroin dissection was performed in adult male post-mortem subjects to establish the prevalence of inguinal canal "lipoma." Thirty-six body halves (age range 24-92 years) were studied. Of these, 27 (75%) contained a discrete mass of fat within the inguinal canal.
View Article and Find Full Text PDFWe compare diverging evolution of a two-component (gas+stars) galactic disk embedded in a "live" halo with that of an identical pure stellar disk. Our modeling supports the conjecture that the growth of central concentration in galaxies dissolves the main family of regular orbits in the stellar bar and assists in the formation of a galactic bulge.
View Article and Find Full Text PDFJ Neurol Neurosurg Psychiatry
July 2002
Background: Traumatic stroke usually occurs after dissection of large extracranial or intracranial vessels, leading to disseminated cerebral embolism. Stretching and distorting forces in cerebral intraparenchymal end arteries can cause intimal lesions followed by an occluding thrombus.
Objective: To investigate the importance of traumatic endothelial lesions in intraparenchymal end arteries after minor head injuries.
Background And Purpose: We sought to compare different antithrombotic secondary treatments (mainly medium-dose aspirin with low-dose low-molecular-weight heparin [LMWH]) in pediatric patients with a first ischemic stroke onset with regard to the risk of stroke recurrence.
Methods: The population comprised 135 consecutively recruited children aged >/=6 months to =18 years with a first episode of ischemic stroke (idiopathic, n=79; cardiac, n=15; vascular, n=30; infectious, n=11). The stroke patients enrolled received prophylactic antithrombotic therapy (aspirin, n=49; LMWH, n=86) in a nonrandomized fashion and were prospectively followed up for a median (range) of 36 (8 to 48) months.
High and monotonically declining levels of EEG slow wave activity (SWA) occur following arousal from hibernation. Similar profiles of SWA occur in mammals including humans during sleep following periods of prolonged wakefulness, and have been interpreted as reflecting a homeostatic process regulating NREM sleep. It was proposed that even though hibernation appears to be an evolutionary extension of NREM sleep, the low brain temperatures during hibernation are not compatible with sleep restorative processes, and therefore sleep debt accumulates during hibernation and may be a factor triggering periodic arousal.
View Article and Find Full Text PDFDuring the last decade, capillary electrophoresis (CE) of DNA has undergone rapid development. This improvement was especially important for DNA sequencing, where CE has now become a standard method facilitating to decipher several genomes within a very short time. Here, we give a review of the fundamentals of DNA separation in CE and the major factors influencing the performance.
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