Progressive familial leukodystrophy of late onset

We report a family in which three siblings developed dementia between the ages of 40 and 70 years. Two of the siblings developed symptoms of depression, abnormal behavior, and an inability to function, progressing to severe dementia. The third sibling had a severe dementia, the clinical details of which are not available. In the two deceased siblings neuropathologic examinations demonstrated severe demyelination, axon loss, and gliosis in cerebral white matter. Cerebellar and brainstem white matter were unaffected. Cerebral gray matter was negligibly affected. The disorder, histopathologically classified as a pigmented orthochromatic leukodystrophy, is extremely rare. Its etiology is unknown, but the pathology and familial occurrence imply that it represents a genetic defect in a function localized in the cerebral white matter.     

Cyclosporin A in patients receiving renal allografts from cadaver donors. 1978

A psychrotrophic yeast, Rhodotorula glutinis KUJ 2731, isolated from soil, effectively produced an extracellular endo-beta-glucanase (EC 3.2.1.4). The enzyme was monomeric, and the molecular mass was about 40,000 Da. The N-terminal amino acid sequence was H-Ser-Leu-Pro- Lys-Leu-Gly-Gly-Val-Asp-Leu-Ala-Gly-Leu-Asp-Ile-Gly-Lys-Asp-Lys-Asn-. alpha-Helix content was calculated to be about 32.6%. The isoelectric point was 8.57. The activation energy was 20.9 kJ/mol, which was much smaller than that of mesophilic enzymes. The enzyme was active at temperatures from 0 to 70 degrees C, with a highest initial velocity at 50 degrees C similar to other psychrotrophic enzymes. The enzyme was inhibited by Hg2+. The enzyme catalyzed hydrolysis of carboxymethyl cellulose with an apparent K(m) of 1.1% and Vmax of 556 mumol/min/mg. Products from the enzymatic hydrolysis of carboxymethyl cellulose by the enzyme were glucose, cellobiose, and cellotriose. The enzyme also catalyzed the transglycosylation of p-nitrophenyl-beta-cellotrioside to cellotetraose.     

Noninvasive measurement of distal radius instability

Except for subjective clinical criteria, there is no formal definition of distal radius fracture instability in the literature. The purposes of this ex vivo biomechanical study were (1) to provide an objective mechanical definition of fracture instability and (2) to demonstrate a noninvasive method that allows for direct measurement of instability. The following 3 questions are addressed: (1) Can the stability of distal radius fractures be measured using computed tomography (CT)? (2) Are the stability measurements reproducible? (3) How does external fixation change stability? A CT technique is described that was used to measure displacement of fracture fragments and measure the compliance of ex vivo distal radius fractures before and after external fixation. Validation studies of the CT technique revealed a mean coefficient of variation of 0.38. There was a linear relationship between measured and known displacements for all 3 orthogonal planes (coefficient of determination 0.99; p < .01). There was significant fracture displacement with loads as small as 20 N. The slope of the load-displacement curve (structural compliance) provided a quantitative measure of fracture instability. Fracture compliance decreased up to 69% after application of an external fixator.     

Growth kinetics and treatment response of the intracerebral rat 9L brain tumor model: a quantitative in vivo study using magnetic resonance imaging

We report the use of magnetic resonance imaging (MRI) for in situ tumor growth rate studies of experimental intracranial 9L tumors. T2-weighted spin-echo coronal magnetic resonance images of rat brains with 9L tumors were obtained every 2 days beginning at 8-11 days postimplantation using a 7 tesla MRI system. Tumors were clearly delineated in the images as a hyperintense region with a relatively well-demarcated border and minimal peritumoral edema. Tumor volumes from individual slices were summed together to yield the total tumor volume. The accuracy of this methodology for volumetric determination was verified by MRI phantom studies. Tumor growth rates determined from sequential MRI measurements of tumor volumes were quantitated in terms of volumetric doubling time. Tumor doubling times were found to range from 50 to 81 h, with an average of 66 +/- 8 h (n = 10). Intracranial 9L tumors were found to grow exponentially over the entire life span of the animal, allowing treated animals to serve as their own controls since the volumetric doubling time could be determined from three to four MRI scans before treatment administration. The intracerebral tumor growth delay following a single injection of 1, 3-bis(2-chloroethyl)-1-nitrosourea (13.3 mg/kg i.p.) allowed for noninvasive determination of in vivo log cell kill. A 2.0 +/- 0.2 (n = 3) log cell kill from 1,3-bis(2-chloroethyl)-1-nitrosourea treatment was found from post-treatment MRI volume measurements. These results demonstrate that MRI provides a powerful and sensitive method for assessing the growth and treatment response of intracranial 9L tumors in the rat.     

p53 tumour suppressor gene mutations in benign prostatic hyperplasia and prostate cancer

OBJECTIVE: Seminal vesicle cysts combined with ipsilateral renal agenesis represent a rare urological anomaly. We searched the literature to review the clinical presentation, diagnosis and therapeutic treatment options of this anomaly. METHODS: A pooled analysis was performed of 52 cases of seminal vesicle cysts combined with ipsilateral renal agenesis, including our own observation. The evaluation included: patient age at diagnosis, race, laterality (R/L), presence of ureteral remnant in the cyst, presenting symptoms, diagnostic examinations, treatment and outcome. RESULTS: The mean age at diagnosis was 30.2 years. The majority presented in the 2nd, 3rd and 4th decade of their lives. Only 2 patients (4%) were of African origin, all others were Caucasians. The distribution R:L was 2:1. Ureteral remnants were present in 14 patients (27%). The most common symptoms were: dysuria (37%), frequency (33%), perineal pain (29%), epididymitis (27%), pain following ejaculation (21%) and scrotal pain (13%). Infertility was found in 9 patients (17%). The cyst was palpable by digital rectal examination in 79%. All patients underwent intravenous urography, and 88% underwent cystoscopy. Other frequently performed investigations are: ultrasonography (27%), CT scanning (27%), vasovesiculography (46%) and urethrocystography (23%). The final treatment was open surgery in 74%, aspiration in 6%, transurethral deroofing of the cyst in 6% and spontaneous rupture in 4%. In 6% no treatment was given and in 4% the treatment is unknown. All patients were free of symptoms after open exploration. The success rates after transurethral deroofing and aspiration were 75 and 30% respectively. CONCLUSION: Seminal vesicle cysts combined with ipsilateral renal agenesis are a rare urological anomaly, occurring in men in the 2nd to 4th decade of their life. They present with symptoms of bladder irritation and obstruction and with pain in the perineum and scrotum. Epididymitis is frequently found. The diagnostic work-up consists of a digital rectal examination, transrectal and abdominal ultrasonography, CT scan and a cystoscopy. Open surgery and transurethral deroofing of the cyst give excellent results (100 and 75% cure respectively). Aspiration of the cyst should only be used for diagnostic purposes.     

Networking,Storytelling and Knowledge Production in First-Year Writing

As writing teachers, we seek to validate the knowledge that students bring to the classroom while introducing and cultivating new methods, strategies, techniques and technologies that may serve them now and in the future. In this article we describe an approach we take that integrates curricular and extracurricular co-apprenticeship strategies using public writing models in conjunction with social networking tools. With publication as a final goal, we urge students to move from expressing personal problems to addressing social issues, using a private, non-commercial learning network NoDiff <http://NoDiff.com> as a safe zone for skills development, knowledge production and social inquiry. In taking this approach, we resist dichotomies that place professional skills training in strict opposition to critical pedagogy. We argue that many of the so-called “extracurricular” composing activities that most of today's students engage in lay the groundwork for both professional and civic participation. Acknowledging that we as composition teachers are also beginners in the world of 21^st century communication, we emphasize both (1) the continual transference of skills and knowledge among teachers and learners; and (2) the importance of providing what is often lacking in skills-based ‘new literacy’ training, namely a critical pedagogical approach to learning as social engagement and critical intervention.     

The effective thermal conductivity of a composite material with spherical inclusions

A new method is presented for calculating the effective thermal conductivity of a composite material containing spherical inclusions. The surface of a large body is assumed kept at a uniform temperature. This body is in contact with a composite material of infinite extent having a lower temperature far from the heated body. Green's theorem is then used to calculate the rate of heat transfer from the heated body to the composite material, yielding $$k_e /k = 1 + \frac{{3(\alpha - 1)}}{{[\alpha + 2 - (\alpha - 1)\phi ]}}\{ \phi + f(\alpha )\phi ^2 + 0(\phi ^3 )\} $$ where k _e is the effective thermal conductivity, k is the thermal conductivity of the continuous phase, α is the ratio of the thermal conductivity of the spherical inclusions to k, and φ is the volume fraction occupied by the dispersed phase. The function f(α) is presented in this work. Although a similar result has been found previously by renormalization techniques, the method presented in this paper has merit in that a decaying temperature field is used. As a result, only convergent integrals are encountered, and a renormalization factor is not needed. This method is more straightforward than its predecessors and sheds additional light on the basic properties of two-phase materials.     

Use of hydration inhibitors to improve bond durability of aluminium adhesive joints

The adsorption of selected organic hydration inhibitors onto Forest Products Laboratory (FPL)-etched aluminium surfaces and the subsequent hydration of the treated surfaces have been studied using X-ray photoelectron spectroscopy (XPS) and surface behaviour diagrams (SBDs) supplemented by Fourier Transform Infra-red Spectroscopy (FTIR). Wedge tests were used to evaluate performance of these inhibitors in improving bond durability and the locus of failure was identified by XPS and high resolution scanning electron microscopy (X-SEM). The results indicate that nitrilotris methylene phosphonic acid (NTMP) and related compounds adsorb to the alumina surface via the POH bonds of the phosphonic acid groups, resulting in a displacement of water normally adsorbed onto the surface. A model of adsorption was developed which suggests that after treatment with very low concentrations of inhibitor (1 ppm), only one leg of the NTMP molecule adsorbs onto the surface although at higher concentrations (100 ppm) all three legs adsorb. Hydration is a three-step process: (i) reversible physisorption of water; (ii) slow dissolution of the inhibitor followed by rapid hydration of the freshly exposed Al₂O₃ to boehmite (AlOOH); and (iii) further hydration of the AlOOH to bayerite [Al(OH)₃]. Analysis of the adsorption, hydration, and wedge test results using different inhibitors suggests the following five inhibitor characteristics that promote good bond performance: (i) displacement of water and occupation of all active sites on the Al₂O₃ surface; (ii) formation of strong inhibitor surface bonds; (iii) insolubility of the resulting inhibitor-aluminium complex in aqueous solutions; (iv) compatibility with the adhesive or primer; (v) coupling of the inhibitor to the adhesive.     

Soluble Epoxide Hydrolase Blockade after Stroke Onset Protects Normal but Not Diabetic Mice

Soluble epoxide hydrolase (sEH) is abundant in the brain, is upregulated in type 2 diabetes mellitus (DM2), and is possible mediator of ischemic injury via the breakdown of neuroprotective epoxyeicosatrienoic acids (EETs). Prophylactic, pre-ischemic sEH blockade with 4-[[trans-4-[[(tricyclo[3.3.1.13,7]dec-1-ylamino)carbonyl]amino]cyclohexyl]oxy]-benzoic acid (tAUCB) reduces stroke-induced infarct in normal and diabetic mice, with larger neuroprotection in DM2. The present study tested whether benefit occurs in normal and DM2 mice if tAUCB is administered after stroke onset. We performed 60 min middle cerebral artery occlusion in young adult male C57BL mice divided into four groups: normal or DM2, with t-AUCB 2 mg/kg or vehicle 30 min before reperfusion. Endpoints were (1) cerebral blood flow (CBF) by laser Doppler, and (2) brain infarct at 24 h. In nondiabetic mice, t-AUCB reduced infarct size by 30% compared to vehicle-treated mice in the cortex (31.4 ± 4 vs. 43.8 ± 3 (SEM)%, respectively) and 26% in the whole hemisphere (26.3 ± 3 vs. 35.2 ± 2%, both p < 0.05). In contrast, in DM2 mice, tAUCB failed to ameliorate either cortical or hemispheric injury. No differences were seen in CBF. We conclude that tAUCB administered after ischemic stroke onset exerts brain protection in nondiabetic but not DM2 mice, that the neuroprotection appears independent of changes in gross CBF, and that DM2-induced hyperglycemia abolishes t-AUCB-mediated neuroprotection after stroke onset.     

Segregation Neutralised Steels: Microstructural Banding Elimination from Dual-Phase Steel Through Alloy Design Accounting for Inherent Segregation

Metallurgical and Materials Transactions A - Banding in commercial dual-phase steels, such as banded ferrite and pearlite or ferrite and martensite microstructures, is inherited from segregation...