1 and 2 1 in benign lesions (P < 0 02) In patients with metas

1 and 2.1 in benign lesions (P < 0.02). In patients with metastatic solid tumors, the mean standardized uptake value was 7.3. Stratification of solid tumor lesions according to whether they had a sclerotic appearance on computed tomographic scans showed that FDG-PET was a significantly better predictor of cancer status in lytic or mixed lesions. In patients with a history of solid tumors, there was 100% concordance between the FDG-PET and needle-biopsy diagnoses in nonsclerotic lesions, when the standardized uptake value cutoff of 2 was used.

CONCLUSION: FDG-PET is an accurate screening test for vertebral metastases in cancer

patients. It is especially accurate in patients with nonsclerotic vertebral lesions and a history of solid malignancy.”
“Influenza Bleomycin datasheet is a serious global public health problem and an economic burden. With the continual emergence of new influenza Capmatinib mw A virus strains, new antiviral drugs are needed urgently. In this study, an improved embryonated chicken egg

model for evaluating antiviral activity against Influenza A virus was developed. In the model, the influenza A virus was injected into the allantoic cavity and ribavirin was injected into the albumen of the egg. The levels of influenza A virus in the allantoic fluid was titrated by the hemagglutination test after incubation for 72 h at 35.5 degrees C and 12 h at 4 degrees C. Ribavirin treatment at a dose of 25 mg/kg to 100 mg/kg decreased significantly the hemagglutination titers both of Influenza virus A/FM1, H1N1 (IVA1) (p < 0.01) and influenza virus A/Wuhan/359/95, BCKDHA H3N2 (IVA3) (p < 0.01). In a time-dependent drug addition assay, significant efficacy of ribavirin against both IVA1 and IVA3 was observed when the drug was administered before and shortly after viral inoculation

(p < 0.01 or p < 0.05). In conclusion, ribavirin treatment showed significant antiviral activity against IVA1 and IVA3 in this model, suggesting that the improved model would be useful for evaluating the anti-influenza virus activity of potential inhibitors. (C) 2008 Published by Elsevier B.V.”
“OBJECTIVE: To analyze pre- and postoperative x-rays of sagittal spines and to review the surgical results of 21 patients with lumbar degenerative kyphosis whose spines were sagittally well compensated by compensatory mechanisms but who continued to suffer from intractable back pain

METHODS: We performed a retrospective review of 21 patients treated with combined anterior and posterior spinal arthrodesis. Inclusion criteria were: lumbar degenerative kyphosis patients with intractable back pain and whose spines were sagittally well compensated by a compensatory mechanism, defined as a C7 plumb line to the posterior aspect of the L5-S1 disc of less than 5 cm.

Furthermore, their oligomeric state was determined by sedimentati

Furthermore, their oligomeric state was determined by sedimentation through sucrose gradients and by separation by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) under nonreducing conditions. In sucrose gradient analyses, HCV envelope glycoproteins were associated with fractions containing the most infectious viral particles. Importantly, besides maturation of some of their glycans, HCV envelope PF2341066 glycoproteins showed a dramatic change in their oligomeric state after incorporation into the viral

particle. Indeed, virion-associated E1 and E2 envelope glycoproteins formed large covalent complexes stabilized by disulfide bridges, whereas the intracellular forms of these proteins assembled as noncovalent heterodimers. Furthermore, the virion-associated glycoprotein complexes were recognized by the large extracellular loop of CD81 as well as conformation-sensitive antibodies, indicating that these proteins are in a functional conformation. Overall, our study fills a gap in the description of HCV outer morphology and should guide further investigations into virus entry and assembly.”
“Recent work identified novel progestin signaling molecules, including progesterone receptor membrane

component 1 (Pgrmc1), Pgrmc2, serpine see more mRNA binding protein 1 (Serbp1), progestin and adiponectin receptors 7 (Paqr7) and Paqr8. These molecules mediate rapid progesterone (P(4)) effects in non-neural tissue and we recently mapped their expression in the brain. Many rapid effects of P(4) require 17 beta-estradiol (E(2)) and P(4) priming; therefore, we examined the effects of ovarian hormones on the expression of these nonclassical progestin signaling molecules. We focused specifically on the anteroventral periventricular nucleus (AVPV), the sexually dimorphic nucleus of the preoptic area (SDN-POA) and the ventrolateral portion of

the DNA ligase ventromedial nucleus (VMNvl). These brain nuclei are important for female reproduction. Ovariectomized adult female rats were implanted with capsules containing sesame oil or E(2), and injected 48 h later with sesame oil or P(4). Brains were collected 8 h later and RNA was isolated from the AVPV, SDN-POA and VMNvl. We assessed the effects of ovarian hormones on mRNA levels using quantitative polymerase chain reaction (QPCR). In the AVPV, Serbp1 mRNA levels were increased by P(4) in the presence of E(2), and Paqr8 was downregulated by P(4) alone. In the SDN-POA, combined E(2) and P(4) increased Pgrmc1 and Serbp1 mRNA levels, and E(2) alone increased Paqr8 mRNA levels. Finally, in the VMNvl, P(4) increased mRNA levels encoding Pgrmc1, Pgrmc2 and Serbp1, and the combination of E(2) and P(4) increased Pgrmc1 and Serbp1 mRNA levels. Paqr7 was not regulated by E(2) or P(4) in any brain region examined.

V All rights reserved “
“The implementation

of cost

V. All rights reserved.”
“The implementation

of cost effective HIV-1 RNA quantitation assays in resource-poor settings is Of paramount importance for monitoring HV-1 infection. A study comparing the analytical performance of three HIV-1 RNA assays (Generic HIV Viral Load (R), Amplicor Autophagy activator inhibitor (TM) v1.5 and Nuclisens EasyQ (R) v1.2) was performed on 160 plasma samples from 160 consecutive antiretroviral treatment naive HIV-1-infected pregnant women assessed for eligibility in the Kesho Bora trial aimed at prevention of mother-to-child transmission of HIV-1 in three African countries (Burkina Faso, Kenya and South Africa). Correlation and agreement of results of the three assays were assessed for plasma HIV-1 RNA quantitation in specimens harbouring

mainly sub-subtype All, subtype C, and circulating recombinant form (CRF) 02_AG and CRF06_cpx.

Good degrees of correlation and agreement were observed between these HIV-1 RNA assays. However, nine (9/160, 5.6%) strains detectable with the Generic HIV Viral Load (R) assay were not detected by either the Amplicor (TM) (n = 7) or EasyQ (R) (n = 2) test. One strain (0.6%) was missed with the Generic HIV Viral Load (R) assay. Further, concordantly positive plasma samples harbouring CRF02_AG and CRF06_cpx yielded significantly higher HIV-1 RNA concentrations when tested by Generic HIV Viral Load (R), as compared to Amplicor (TM) v1.5 learn more (mean differences, +033 and NADPH-cytochrome-c2 reductase +0.67 log(10) copies/ml; P = 0.0004 and P = 0.002, respectively). The Generic HIV Viral Load (R) assay accurately quantified the majority of the non-B HIV-1 subtypes assessed in this study. Due to its low cost (similar to 10 US $/test), this assay performed with open real-time PCR instruments is now used routinely in the Kesho Bora trial and may be recommended in other African settings. (C) 2009 Elsevier B.V. All rights reserved.”
“A seemingly novel

siadenovirus species was detected by PCR and sequencing in the sample of a great tit (Parus major) found dead in Hungary. Since the genus Siadenovirus has very few known members so far, further study of the virus was intriguing not only from epizootiological but also from taxonomical aspects. The sample, which had been tested in another PCR survey previously. consisted of less than 50 mu 1 of extracted nucleic acid. To ensure sufficient target DNA for an extended study, the viral genome had to be preserved. To this end, the sample was subjected to a novel method of non-specific DNA amplification. Using the amplified DNA as target, different PCR and sequencing strategies were applied with consensus or specific primers for the study of the central genome part of the putative tit adenovirus. The sequence of supposedly one half (13,628 bp) of the genome was determined including eight full genes between the genes of the IVa2 and hexon proteins.

(C) 2009 Elsevier Ltd All rights reserved “
“The fruit fly,

(C) 2009 Elsevier Ltd. All rights reserved.”
“The fruit fly, Drosophila melanogaster, displays a scale-free behavior in foraging,

i.e., the dwell time on food exhibits a power law distribution. The scaling exponent is generally believed to be stable and the significance of the exponent itself with respect to the scale-free behavior remains elusive. We propose a model where by the scaling exponent of the scale-free behavior of an animal depends on the memory of the individual. The proposed model is based on the premise that animal behaviors are associated with internal states of the animal. The changes in the scaling exponent are derived by considering losing memory as increasing uncertainty, which is expressed in terms of information entropy of the internal states. Predicted model behaviors agree with experimental Selleck Ricolinostat results of foraging behavior in wild-type and learning/memory Drosophila mutants. The concept of changes in the scaling exponent due to the amount of memory provides a novel insight into the emergence of a scale-free behavior and the meaning of the Galunisertib cost scaling exponent. (C) 2009 Elsevier Ltd. All rights reserved.”
“Recent evidence using a modified Simon

task suggests that hand processing involves implicit coding of the spatial position of the hand relative to the side of the body to which it is attached from the viewer’s reference point. This effect, called the Sidedness effect, has been found to emerge only when at least the forearm is present (the forearm thus providing the spatial reference for representing the rest of body) and

it has been interpreted within the framework of the structural representation of the body. In this study we use the same modified Simon task to investigate whether hand processing involves the implicit access to a spatially and bio-mechanically organized structural Adenosine body representation. In a first experiment the hand stimuli were attached to a body inappropriately without respecting the bio-mechanical constraints and no Sidedness effect was found. in Experiment 2 where the hand stimuli were presented attached to a non-bodily shape the Sidedness effect was observed only when they were attached appropriately. Whilst previous research has involved explicit representational processes, our results suggest that we can implicit access to a ‘structural description of the body’ and elaborate the anatomical and bio-mechanical plausibility. (C) 2009 Elsevier Ltd. All rights reserved.”
“Glioblastoma is the most common and the most aggressive type of brain cancer. The median survival time from the time of diagnosis is approximately one year.

35;

95% CI, 1 61-11 7)

CONCLUSION: In the mic

35;

95% CI, 1.61-11.7).

CONCLUSION: In the microsurgical treatment of supratentorial Luminespib datasheet AVMs, hemorrhage, male sex, and frontotemporal location are associated with higher rates of preoperative seizures, whereas deep artery perforators are associated with postoperative seizures. Achieving freedom from seizure is an important goal that can be achieved in the surgical treatment of AVMs because epilepsy can significantly diminish patient quality of life.”
“There is evidence that anterior cingulate cortex (ACC) function is related to individual differences in temperament. An important question regards how early such brain-behavior associations emerge. We examined the relationship between cortical folding patterns of the ACC, which are functionally relevant and primarily determined by birth, and individual differences in four core temperament dimensions (Effortful check details Control, Negative Affectivity, Surgency, and Affiliation).

Magnetic resonance imaging was used to classify 153 (81 male) early adolescents as displaying a leftward asymmetric, rightward asymmetric, or symmetric pattern of ACC folding, as indexed by the incidence and extent of the paracingulate sulcus (PCs). A leftward asymmetric pattern of ACC folding was associated with significantly higher temperamental Effortful Control and lower Negative Affectivity than a rightward asymmetric pattern. Further, this difference was significant only for males. Across males and females, a symmetric pattern was associated with higher temperamental Affiliation

than was a rightward asymmetric pattern of ACC folding. These findings suggest that early neurodevelopmental processes contribute to individual differences in temperament They also illustrate sexual dimorphisms in the neural underpinnings of temperament. (C) 2008 Elsevier Ireland Ltd. All rights reserved.”
“The identification of T cell co-inhibition as a central mechanism in the regulation of adaptive immunity during infectious diseases provides new opportunities for immunotherapeutic interventions. However, the fact that T cell activity is frequently downregulated Galeterone during pathogen-directed responses suggests a pivotal physiological role of co-inhibitory pathways during infectious disease. Reports of exacerbated immunopathology in conditions of impaired co-inhibition foster the view that downregulation of T cell activity is an essential negative feedback mechanism that protects from excessive pathogen-directed immunity. Thus, targeting co-inhibitory pathways can bear detrimental potential through the deregulation of physiological processes. Here, we summarize recent preclinical and clinical interventions that report immune-related adverse events after targeting co-inhibitory pathways.”
“Background/Aims: beta(2)-adrenoceptor (beta(2)-AR) activation induces smooth muscle relaxation and endothelium-derived nitric oxide (NO) release.

Results Compared with participants with dementia, patients with

Results. Compared with participants with dementia, patients with delirium demonstrated higher CSF lactate (1.87 vs 1.48 mmol/L, p < .001) and protein levels (0.62 vs 0.44 g/L, p = .036) and lower levels of neuron-specific enolase (4.84 vs 8.98 ng/mL, p < .001)

but no difference in S100B. The changes correlated with clinical indices and outcomes.

Conclusion. Older patients with delirium experience significant metabolic disturbance in the brain, which requires further investigation.”
“BACKGROUND: Cerebrospinal fluid (CSF) drainage serves an important role in the management of patients with established or potential CSF fistulae. Classically, a lumbar CSF drain has been used for this purpose and has been shown to be safe and effective. In certain cases, such as extensive previous lumbar surgery, a lumbar drain cannot be used. In such instances, a cervical CSF drain can be inserted via a lateral C1-2 puncture and provides an excellent and selleck products safe alternative.

OBJECTIVE: To describe the technique, safety, and effectiveness of placing a cervical drain for CSF drainage. Pitfalls and possible complications and their avoidance are also discussed.

METHODS: Twenty-seven cervical drains were JSH-23 cell line placed in 24 patients with a mean age of 56.1 years (range,

19-82 years). There were 13 women and 11 men. All cervical drains were placed via a lateral C1-2 puncture under direct fluoroscopic Ureohydrolase vision. A standard Hermetic closed-tip lumbar catheter was used in all cases. The drains were in place for an average of 5.96 days (range, 3-11 days). CSF surveillance was performed on the day of placement as well as every 48 hours that the drain was in place.

RESULTS: Cervical drain placement was achieved in all cases, allowing for continuous CSF drainage. No permanent procedural complications occurred. There were no instances of meningitis.

CONCLUSIONS: Placement of a cervical intrathecal catheter for CSF drainage is a safe and effective alternative when lumbar access is contraindicated or not achievable.”
“Urinary excretion of water and all major electrolytes exhibit robust circadian oscillations. The 24-h

periodicity has been well documented for several important determinants of urine formation, including renal blood flow, glomerular filtration, tubular reabsorption, and tubular secretion. Disturbance of the renal circadian rhythms is increasingly recognized as a risk factor for hypertension, polyuria, and other diseases and may contribute to renal fibrosis. The origin of these rhythms has been attributed to the reactive response of the kidney to circadian changes in volume and/or in the composition of extracellular fluids that are entrained by rest/activity and feeding/fasting cycles. However, numerous studies have shown that most of the renal excretory rhythms persist for long periods of time, even in the absence of periodic environmental cues.

Imaging is Usually carried Out a day or more after administration

Imaging is Usually carried Out a day or more after administration Selleckchem CB-839 of the radiotracer, and serial and tomographic imaging may be necessary for optimal delineation.

MIBG imaging is more useful

for detecting pheochromocytoma, with reported accuracies greater than 80%, than for detecting carcinoid tumors, where the accuracy has been similar to 70% and is reportedly higher in mid-gut tumors. MIBG imaging has been invaluable in the accurate staging of children with neuroblastoma, a lethal childhood tumor of the sympathetic nervous system.

An important application of MIBG imaging is to demonstrate targeting of therapeutic I-131 MIBG. Imaging is thus useful in file detection of disease as well as in the demonstration of adequate targeting for therapy – either qualitatively or quantitatively

with dosimetry. The latter will probably be feasible with PET using isotopes like iodine-124, and perhaps with single photon emission computed tomography/computed tomography. Imaging with MIBG will continue to be the mainstay selleck chemicals for detection and staging of GEP-NET. More importantly, perhaps, imaging with MIBG will form part of an imaging continuum, including assessment of glycolytic rate and somatostatin receptor Status, that will enable assessment Of tumor phenotype and guide management. (C) 2008 Elsevier Inc. All rights reserved.”
“Kaposi’s sarcoma-associated herpesvirus (KSHV)-encoded glycoprotein B (gB) is an important determinant of viral infectivity and virion egress. A small interfering RNA (siRNA)-based strategy was devised to inhibit

KSHV gB gene expression. Transient cotransfection of plasmids constitutively expressing gB and anti-gB siRNAs in 293 cells Guanylate cyclase 2C substantially inhibited gB mRNA levels and protein production. Similarly, transient expression of siRNAs into the primary effusion lymphoma cell line BCBL-1 caused a substantial reduction of gB transcripts and protein synthesis. TaqMan real-time PCR assays against the lytic KSHV gene ORF59 and infectivity assays on 293 cells were employed to assess the effect of inhibiting gB synthesis on virion egress from BCBL-1 cells and infectivity on 293 cells, respectively. These experiments showed that gB was essential for virion egress and infectivity. Transfection of a codon-optimized gB gene with the first 540 nucleotides altered, and therefore not recognized by anti-gB siRNAs that target the native but not the codon-optimized sequence, efficiently rescued virion egress and infectivity in BCBL-1 cells in the presence of siRNAs inhibiting wild-type gB expression. To assess the role of the cytoplasmic domain of gB in virion egress, mutant gB genes were generated specifying carboxyl terminal truncations of 25 and 58 amino acids disrupting two prominent predicted alpha-helical domains associated with virus-induced cell fusion.

Interestingly, our results also suggest that it will not always b

Interestingly, our results also suggest that it will not always be the cold-adapted phenotypes that drive polewards range expansion. Instead, range shifts may be driven by phenotypes conferring adaptation

to conditions prevalent towards the centre of a species’ equilibrium distribution. This may have important consequences for the conservation method termed predictive provenancing. These initial results highlight the potential importance of local adaptation in determining how species will respond to climate change and we argue that this AG-120 is an area requiring urgent theoretical and empirical attention. (C) 2010 Elsevier Ltd. All rights reserved.”
“Huntingtin, the protein that when mutated causes Huntington disease (HD), has many known interactors and participates in diverse cellular functions. Mutant Htt (mHtt) engages in a variety of aberrant interactions that lead to pathological gain of toxic functions as well as loss of normal functions. The broad symptomatology of HD, including diminished voluntary motor control,

cognitive decline, and psychiatric disturbances, reflects the Multifaceted KPT-8602 datasheet neuropathology. Although currently available therapies for HD focus on symptom management, the autosomal dominant cause and the adult onset make this disease an ideal candidate for genetic intervention. A variety of gene therapy approaches have been tested in mouse models of HD, ranging from those aimed at ameliorating downstream pathology or replacing lost neuronal populations to more upstream strategies to reduce mHtt levels. Here the authors review the results of these preclinical trials.”
“Although density-dependent dispersal and

relative dispersal (the difference in dispersal rates between species) have been documented in natural systems, their effects on the stability of metacommunities are poorly understood. Here we investigate the effects before of intra- and interspecific density-dependent dispersal on the regional stability in a predator-prey metacommunity model. We show that, when the dynamics of the populations reach equilibrium, the stability of the metacommunity is not affected by density-dependent dispersal. However, the regional stability, measured as the regional variability or the persistence, can be modified by density-dependent dispersal when local populations fluctuate over time. Moreover these effects depend on the relative dispersal of the predator and the prey. Regional stability is modified through changes in spatial synchrony. Interspecific density-dependent dispersal always desynchronizses local dynamics, whereas intraspecific density-dependent dispersal may either synchronize or desynchronize it depending on dispersal rates. Moreover, intra- and interspecific density-dependent dispersal strengthen the top-down control of the prey by the predator at intermediate dispersal rates.

Our discussion focuses primarily on B lymphoma Mo-MLV1 insertion

Our discussion focuses primarily on B lymphoma Mo-MLV1 insertion region proteins of the polycomb repressive complex, alpha thalassemia/mental retardation syndrome X-linked chromatin remodeling factors, and the best known and most dynamic gene repressors, histone deacetylases. We will highlight exciting current data associated with these proteins and provide promising speculation

about how they can be manipulated Adavosertib manufacturer by drugs, biologics, or noninvasive stimulation for stroke recovery.”
“Background. Human aging is associated with heightened risk of diabetes and cardiovascular disease. Increased fat mass may contribute to age-related diseases by harboring inflammatory macrophages Vactosertib ic50 that produce metabolically important proteins such as plasminogen activator inhibitor-1 (PAI-1). Elevated PAI-1 concentrations have been implicated in the pathogenesis of such aging-related conditions as insulin resistance, obesity, and atherosclerosis. We have previously reported that increased plasma free fatty acid (FFA) concentrations augment both circulating PAI-1 concentrations and PAI-1 production by adipose tissue macrophages (ATMs).

Methods. Because increasing age is associated with increased infiltration and reactivity of adipose macrophages, we performed euglycemic-hyperinsulinemic

clamp studies and adipose tissue biopsies with and without elevated FFA concentrations in 31 nondiabetic participants stratified by age, to determine whether middle-aged individuals manifest heightened insulin resistance and PAI-1 production by ATMs in response to elevated nutrient signals relative to their young adult peers.

Results. We observed that elevating FFA concentrations under euglycemic-hyperinsulinemic clamp conditions induced

the same degree of insulin resistance in both middle-aged and younger body mass index-matched adults, whereas systemic PAI-1 Staurosporine datasheet concentrations were significantly increased in the middle-aged group. Likewise, elevated FFA and insulin concentrations induced larger increases in PAI-1 gene expression in the whole fat and ATMs of middle-aged compared with younger adult participants.

Conclusions. These studies reveal a heightened adipose inflammatory response to increased FFA and insulin availability in middle-aged individuals relative to younger adults, suggesting that increased susceptibility to the effects of fatty acid excess may contribute to the pathogenesis of age-related diseases.”
“There has long been an interest in examining the involvement of opioid neurotransmission in nicotine rewarding process and addiction to nicotine. Over the past 3 decades, however, clinical effort to test the effectiveness of nonselective opioid antagonists (mainly naloxone and naltrexone) for smoking cessation has yielded equivocal results.

001) Ureters with a higher reflux grade also showed a higher hyd

001). Ureters with a higher reflux grade also showed a higher hydrodistention selleck compound grade. The normal control group (mean

+/- SEM hydrodistention grade 0.62 +/- 0.07) showed a statistically lower hydrodistention grade than the reflux groups (overall mean hydrodistention grade 2.26 +/- 0.01). Mean posttreatment hydrodistention grade in the failed reflux surgery group was statistically higher than that in the mean successful reflux surgery group (2.03 +/- 0.09 vs 1.33 +/- 0.08). By defining the degree of hydrodistention as normal (H0-H1) and abnormal (H2-H3) the concordance between observers was 95% and 96%.

Conclusions: The dynamic hydrodistention classification is a reliable method of evaluating the presence or absence of vesicoureteral reflux as it correlates significantly with radiographic reflux grade. It has high interobserver

concordance.”
“Recent evidence suggests that trace amines such as tyramine and octopamine, alternative products of tyrosine metabolism (an aminoacid parent of dopamine and noradrenaline), play a role in the homeostasis of the extrapyramidal system. However, the relevance of these trace amines in the pathogenesis of Parkinson’s disease buy OSI-906 is still largely unknown. Here, we assessed the plasma levels of octopamine and noradrenaline in three sub-groups of PD patients, namely de novo, non-fluctuating and fluctuating patients, versus age-matched control subjects. We show that octopamine is detectable in plasma of all subjects, the mean levels of which are significantly lower in PD patients, including de nova patients, when compared to controls (p < 0.001). Unlike this, no changes

in plasmatic noradrenaline levels were found in the de novo patients, but only in plasma of fluctuating and non-fluctuating PD patients. These findings raise the possibility that Parkinson’s disease is firstly characterized by abnormalities of tyrosine decarboxylase, rather than tyrosine hydroxylase, enzyme activity. Given the role of this enzyme in the production of trace amines, circulating octopamine levels may hold promise as a biomarker of early Parkinson’s disease. (C) 2010 Elsevier Ireland Ltd. All rights reserved.”
“Purpose: We investigated factors affecting the breakthrough urinary tract infection Chloroambucil rate during prophylactic antibiotic treatment in children with primary vesicoureteral reflux.

Materials and Methods: Medical charts were retrospectively reviewed in children with primary vesicoureteral reflux diagnosed at age 12 months or less who received prophylactic antibiotics and underwent (99m)Tc-dimercapto-succinic acid scan. Parameters assessed for their relation to breakthrough urinary tract infection were gender, presenting symptoms, age at presentation, prophylactic antibiotic type, reflux grade at presentation and scan findings.

Results: Enrolled in the study were 52 boys and 6 girls with a mean age at presentation of 3.7 months and a mean followup of 42.5 months.