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Histologic features of bladder cancer in Boston, USA, Manchester, UK, and Nagoya, Japan.

Histologic characteristics of bladder cancer in Boston, USA, Manchester, UK, and Nagoya, Japan, were evaluated. In each of these areas broadly-based series of cases were assembled during a collaborative case-control study. The present analysis was based on 589 cases in Boston, 484 cases in Manchester, and 241 cases in Nagoya. A single pathologist reviewed a slide of the primary tumor without reference to identifying information or other data. The primary histologic type of nearly all tumors was transitional-cell, and there was little variation in the proportion of transitional-cell tumors among the study areas. Nor was there much variation in the distribution of histologic grade, the proportion of tumors showing submucosal invasion, or the proportion of tumors with a papillary surface. Age at diagnosis was strongly correlated with histologic grade. The proportion of grade III (most malignant) tumors was about twice as high among patients 80 years of age and over as among those aged less than 50. An apparent association between age and submucosal invasion was explained in large part by the relationships of histologic grade to submucosal invasion and to age. Other histologic features had only weak and inconsistent relations with age. None of the features evaluated showed consistent associations with history of cigarette-smoking or with sex.

Adenocarcinoma↗

Hb Lepore-Baltimore (delta 68Leu-beta 84Thr) and Hb Lepore-Washington-Boston (delta 87Gln-beta IVS-II-8) in central Portugal and Spanish Alta Extremadura.

Hb Lepore is one of the most common abnormal haemoglobins in Caucasians in Central Portugal and in the Spanish Alta Extremadura (0.28% in a survey of school children). A group of 19 Portuguese and 14 Spanish Hb Lepore carriers (all unrelated) was characterised at the molecular level by the polymerase chain reaction, sequencing and restriction enzyme analysis. The Portuguese and one Spanish carrier were heterozygous for Hb Lepore-Baltimore, whereas all other Spanish subjects were Hb Lepore-Washington-Boston carriers. Sequencing of the Hb Lepore-Baltimore gene further established the crossover at delta 68-beta 84, a region two codons (CDs) shorter than that previously described and easily confirmed by digestion with MaeI and BanI. Data from haplotype analysis suggest that this crossover occurred as an independent event on the Iberian Peninsula. The haematological data were similar in both groups except for the levels of Hb F and the G gamma chain, which were significantly higher in the Hb Lepore-Baltimore heterozygotes. Quantification of the globin chains and the mRNA transcripts showed that the delta beta gene is transcribed at a higher level than the delta gene with levels of translation giving rise to 10%-15% of Hb Lepore. The different levels of Hb F observed in the two groups are the results of the higher transcription rate of the gamma genes in Hb Lepore-Baltimore heterozygotes and an apparently less efficient translation of G gamma genes in Hb Lepore-Washington-Boston heterozygotes.

Base Sequence↗

Performance of normal elderly on the Boston Naming Test.

The Boston Naming Test has enjoyed increasing use in many research studies since its introduction. However, there is little normative data on the age group above 60 years of age. This study presents data from a sample of 58 well-defined healthy elderly males and females between the ages of 60 and 85. In comparison with published normative data, our sample has higher means, smaller standard deviations, and narrower ranges. These results suggest that aging alone does not significantly alter recognition-cued word-finding ability as defined by the Boston Naming Test. Also there is remarkably consistent performance throughout our age range.

Aged↗

The Boston Naming Test in Swedish: normative data.

The purpose of the present study was to introduce a Swedish version of the Boston Naming Test and to offer normative data based on a sample of native Swedish-speaking healthy adults stratified concerning age, gender, and length of education. The subjects were assessed with other lexical tests and half of the group also performed tests of global cognitive function. A semantic analysis of the responses was performed and the typical Swedish naming of the pictures of BNT was investigated. The results showed that long education, high performance on tests of global cognitive ability (FSIQ), verbal fluency, and other lexical tests had a significant positive association to a good performance on BNT whereas age and gender had a minor influence. The response analysis showed that the more difficult an item was, the more variations in the response pattern. To conclude, the Boston Naming Test is appropriate for use in a Swedish-speaking context and an effective test for assessment of naming ability.

Adult↗

The independent role of cytomegalovirus as a risk factor for invasive fungal disease in orthotopic liver transplant recipients. Boston Center for Liver Transplantation CMVIG-Study Group. Cytogam, MedImmune, Inc. Gaithersburg, Maryland.

PURPOSE: To assess impact of cytomegalovirus (CMV) donor-recipient serostatus, infection, or disease on development of invasive fungal infection in orthotopic liver transplant recipients. PATIENTS AND METHODS: An analysis of prospectively collected data in 146 liver transplant recipients (intention to treat cohort) from 4 tertiary care, university-affiliated transplant centers in Boston (Boston Center for Liver Transplantation). Patients were observed for 1 year after transplantation for the development of CMV infection, CMV disease, CMV pneumonia, as well as for the development of opportunistic fungal infections, graft survival, and mortality. Weekly cultures were taken of urine and throat and every other week of buffy coat for CMV for 2 months, then monthly for 6 months, at 1 year, and at the time of any clinical illness. Pre- and posttransplant variables including CMV-serostatus of donor and recipient, fungal isolation from sterile body sites, fungemia, bacteremia, antibiotic use, immunosuppression, treatment for rejection, and volumes of blood products were measured. RESULTS: Survival analysis demonstrated that 36% of patients with CMV disease developed invasive fungal disease within the first year post-transplant compared with 8% of those without CMV disease (P < 0.0001). One-year mortality in patients with invasive fungal disease was 15 of 22 (68%) compared with 23 of 124 (19%) in those without invasive fungal disease (P < 0.001). A multivariable, time-dependent analysis demonstrated that being a CMV-seronegative recipient of a CMV-seropositive donor organ (P < 0.001), having bacteremia (P = 0.001), UNOS (United Network for Organ Sharing) status 4 (need for life support measures) at transplant (P = 0.002), and volume of platelets (P = 0.002) were independently associated with invasive fungal disease. Restriction of cases of invasive fungal disease to those that occurred more than 2 weeks after transplant demonstrated an association with CMV disease (P = 0.003), bacteremia (P = 0.003), need for life support (P = 0.03), and volume of blood products transfused (P = 0.02). CONCLUSION: CMV disease or being a CMV-seronegative recipient of a CMV-seropositive donor organ is an important predictor for invasive fungal disease following orthotopic liver transplantation.

Adolescent↗

Role of heat-labile toxigenic Escherichia coli and Reovirus-like agent in diarrhoea in Boston children.

61 Boston children aged five years or less with acute diarrhoea were studied for evidence of infection with Escherichia coli strains that produce heat-labile enterotoxin (L.T.) or with a reovirus-like agent associated with childhood gastroenteritis. This represented the first evaluation of the prevalence of disease produced by these two agents in the same population. E. coli, isolated from acute-phase stool specimens, were tested in adrenal-cell tissue-culture and adult-rabbit ileal-loop assays for L.T. Acute and convalescent phase sera, collected from 31 children, were tested by the adrenal-cell assay for anti-L.T. activity. None of the 61 children demonstrated evidence of infection with L.T.-positive E. coli. Paired sera from 31 of the children studied were also tested for evidence of recent infection with the reovirus-like agent by determining titres of immunofluorescent-staining antibody to the serologically related Nebraska calf diarrhoea virus. 11 of the children (35%) had evidence of recent infection. These results suggest that an important proportion of endemic acute diarrhoea of young children in Boston is caused by the reovirus-like agent, and that disease caused by L.T.-producing E. coli is uncommon.

Antibodies, Bacterial↗

Changes in the abnormal alpha-subunit upon CO-binding to the normal beta-subunit of Hb M Boston: resonance Raman, EPR and CD study.

Heme-heme interaction in Hb M Boston (His alpha 58-->Tyr) was investigated with visible and UV resonance Raman (RR), EPR, and CD spectroscopies. Although Hb M Boston has been believed to be frozen in the T quaternary state, oxygen binding exhibited appreciable co-operativity (n=1.4) and the near-UV CD spectrum indicated weakening of the T marker at pH 9.0. Binding of CO to the normal beta-subunit gave no change in the EPR and visible Raman spectra of the abnormal alpha-subunit at pH 7.5, but it caused an increase of EPR rhombicity and significant changes in the Raman coordination markers as well as the Fe(III)-tyrosine related bands of the alpha-subunit at pH 9.0. The UVRR spectra indicated appreciable changes of Trp but not of Tyr upon CO binding to the alpha-subunit at pH 9.0. Therefore, we conclude that the ligand binding to the beta heme induces quaternary structure change at pH 9.0 and is communicated to the alpha heme, presumably through His beta 92-->Trp beta 37-->His alpha 87.

Amino Acids↗

Determination of Fe-CO geometry in the subunits of carbonmonoxy hemoglobin M Boston using femtosecond infrared spectroscopy.

We have undertaken ultrafast infrared (IR) spectroscopic studies in order to elucidate the geometry of bound CO in the alpha and beta subunits of hemoglobin (Hb) M Boston 13CO. Hb M Boston is a mutant human Hb in which the distal histidine in the alpha subunits is replaced by a tyrosine. The IR absorptions of bound 13CO fall at 1925 cm-1 for the alpha subunits and 1907 cm-1 for the beta subunits. Despite a difference of nearly 20 cm-1 in these peaks, the measured anisotropies of the bound 13CO depletions following 30% photolysis are nearly identical, with values of -0.142 +/- 0.002 obtained for the alpha subunits and -0.140 +/- 0.003 obtained for the beta subunits. These translate to values of 20 degrees +/- 1 degree and 21 degrees +/- 1 degree for the values of the average angles between the CO bond and the normal to the heme planes in the alpha and beta subunits, respectively. Our present results and the work of previous investigators [Nagai, M., Yoneyama, Y., & Kitagawa, T. (1991) Biochemistry 30, 6495-6503] suggest that a change in the polar interactions of the bound CO with the heme pocket environment upon substitution of tyrosine for the distal histidine and a less bent structure for the Fe-C-O unit in the alpha subunits are responsible for the difference in the bound CO absorption frequencies in the alpha and beta subunits. A spectrum of the depletion of the bound 13CO peaks following photolysis indicates that both subunits photodissociate CO with the same quantum yield and neither subunit exhibits significant recombination within 1 ns.

Binding Sites↗

Boston University Doctor of Science Degree Program: clinical doctorate in audiology.

This article describes the development of the Boston University Clinical Doctoral Program in Audiology dating from its inception in 1985 to the present. The issues surrounding the establishment of the clinical doctorate, entering the program, and the curriculum are discussed. The unique features of this program and associated resources available within the clinical and scientific community of the greater Boston area are highlighted.

Audiology↗

Normative data on the Korean version of the Boston Naming Test.

The purpose of this study is to introduce the Korean version of the Boston Naming Test (K-BNT) and to present the normative data. The K-BNT contains the same number of test items and uses the identical general administration method as the original Boston Naming Test (BNT). However, most of the items had to be altered due to the linguistic and cultural differences between Korean and English speakers. We administered 60 line-drawing pictures to six hundred normal participants in eight age groups (15-19, 20-24, 25-34, 35-44, 45-54, 55-64, 65-74, and 75 years and older), five educational levels (0, 1-6, 7-9, 10-12, and 13 years and more), and gender. Since the K-BNT scores were not affected by gender and found not to be significantly different among some age groups, we finalized each cell based on four age groups (15-44, 45-54, 55-74, and 75 years and older) and five education levels (0, 1-6, 7-9, 10-12, and 13 years and more). The highest score was achieved by the 15-44 age group with 13 and more years of education (M = 53.93, SD = 3.06), and the lowest by those of 75 years and older with no education (M = 27.42, SD = 8.22).

Adolescent↗

Further crossvalidation of regression-based neuropsychological norms with an update for the Boston Naming Test.

Regression-based norms for the Trail Making Test, Boston Naming Test, and Wisconsin Card Sorting Test, which we published in 1991 and 1993, have been criticized by Fastenau (1998) as having overcorrected for demographic influences in a sample of 63 older adults. We present data from new, independent participant samples that are consistent with expectations from the regression-based norms. We propose that Fastenau's findings in this instance resulted from the nonrepresentative nature of his relatively small sample, rather than from statistical deficiencies of regression based norms. Our currently published norms on one of the four tests considered here, the Boston Naming Test, are based upon a participant sample that was small and had inadequate representation of young adults. We address this by providing updated norms based on a much larger and more representative sample (N = 531).

Adult↗

The effect of a modified Boston brace with anti-rotatory blades on the progression of curves in idiopathic scoliosis: aetiologic implications.

UNLABELLED: The effect of a modified Boston brace with anti-rotatory blades on idiopathic scoliotic curves, mainly right thoracic with a compensatory left lumbar, was studied. METHOD AND MATERIAL: Twenty-eight scoliotic children divided into three sub-groups according to the curve type were included in the study. Cobb angle and rotation was measured on posteroanterior spinal radiographs taken during the first examination and also during the follow-up with the children in and out of the brace. RESULTS: Ten curves improved, 13 remained stable and 5 increased (Cobb angle change >5 degrees compared with the initial measurement). The brace treatment had more affect on the double curves, while single curves remained unaffected. Rotation remained unchanged in all curve types except in the lumbar component of double (right thoracic-left lumbar) curves. DISCUSSION: These findings indicate that in curves with a compensatory component (e.g. main thoracic with compensatory lumbar curve), a deforming rotatory force, which is blocked by the de-rotatory action of the blades of the above-modified Boston brace, is present and seems to be more active in the lumbar spine. It is hypothesized that this deforming rotatory force seems to be a major aetiological factor for double curves. In conclusion the conservative treatment using this brace is beneficially affecting the natural history of IS in children.

Adolescent↗

The molecular basis of Boston-type craniosynostosis: the Pro148-->His mutation in the N-terminal arm of the MSX2 homeodomain stabilizes DNA binding without altering nucleotide sequence preferences.

Craniosynostosis, Boston type is an autosomal dominant disorder that results in the premature fusion of calvarial bones and ensuing abnormalities in skull shape. We showed previously that this disorder is tightly linked to the Msx2 homeobox gene on the long arm of chromosome 5, and that affected individuals bear a mutated copy of Msx2. In addition, transgenic mice in which either mutant or wild-type mouse Msx2 is overexpressed in the developing skull also exhibit craniosynostosis. That both mutant and wild-type Msx2 elicit craniosynostosis in transgenic mice and that the Boston type mutation is dominant led us to hypothesize that the mutation might enhance the normal function of Msx2. The mutation is located in position 7 of the N-terminal arm of the homeodomain, a region implicated in both target sequence recognition and protein-protein interactions. Here we test the hypothesis that the Pro148-->His mutation alters the DNA binding properties of Msx2. Using gel shift and binding site selection analyses, we show that the mutation enhances the affinity of Msx2 for a set of known Msx2 target sequences but has little or no effect on the site specificity of Msx2 binding. The enhancement of Msx2 binding is due largely if not entirely to an increased stability of the mutant Msx2-DNA complex. These data provide a molecular-level explanation of how the Pro148-->His mutation enhances Msx2 function and thus leads to the dominant craniosynostosis phenotype.

Animals↗

Tracheobronchial amyloidosis. The Boston University experience from 1984 to 1999.

Tracheobronchial amyloidosis (TBA), an idiopathic disorder characterized by deposition of fibrillar proteins in the tracheobronchial tree, occurred in 10 patients referred to the Amyloid Program at Boston University over the past 15 years. Fewer than 100 cases of TBA have been described; only 1 series encompassed more than 3 patients. We analyzed our experience with biopsy-proven TBA to define better its natural history. Follow-up averaged approximately 8 years and was obtained in all cases, making this outcome reporting the largest and most complete to date. Three of these patients were prospectively studied for up to 24 months to examine the utility of bronchoscopy, computerized tomography (CT) imaging, and pulmonary function tests (PFTs) in monitoring disease progression. No patient with TBA developed signs or symptoms of systemic amyloidosis during the period reviewed. Conversely, tracheobronchial disease was not diagnosed in 685 patients with primary systemic (AL) amyloidosis during the 15-year study period at Boston University. Bronchoscopy proved most useful in establishing the diagnosis by biopsy. Narrowing of major airways limited its inspection of the tracheobronchial tree, however. In contrast, CT imaging provided quantitative assessment of airway narrowing and mural thickening--2 major consequences of amyloid infiltration. These CT features, in the presence of mural calcifications sparing the posterior tracheal membrane, have been reported in few disorders other than TBA. The ability of CT to map airway involvement and identify extraluminal manifestations of TBA made it the study of choice for establishing disease extent. Three patterns of disease were evident by CT imaging and bronchoscopic examination: proximal, mid, and distal airways involvement. Those with severe proximal disease had significantly decreased air flows, air trapping, and fixed upper airway obstruction on PFTs. Patients with distal disease had normal airflows. PFTs could not clearly distinguish proximal from severe mid airways disease. Thirty percent of patients died within 7-12 years after diagnosis, all having proximal or severe mid airways disease. Repeated rigid bronchoscopic debridement and laser treatments did not prevent progressive airways narrowing in patients dying from TBA. Most patients with mid airways involvement, and all distal airway cases, had either stagnant disease or slowly increasing amyloid deposits when followed for up to 14 years. In a small subset of patients followed prospectively, serial PFTs were most sensitive to disease progression. CT-derived measures of airway lumen diameter and wall thickness did not change significantly despite marked improvements in airflow after rigid bronchoscopy. Our experience suggests that serial PFTs and CT imaging together offer the best assessment of airway involvement and disease progression in patients with TBA. In the future, radiation therapy may provide more definitive treatment of TBA than debulking procedure have to date.

Adult↗

Flexure and residual astigmatism with Paraperm O2 and Boston II lenses on toric corneas.

It has been shown previously that thin polymethyl methacrylate (PMMA), cellulose acetate butyrate (CAB), and Polycon I contact lenses flex on toric corneas. We investigated the flexure and induced residual astigmatism of two gas permeable contact lens materials, Paraperm O2 and Boston II, and compared the results with those of other materials. Six subjects (12 eyes) with corneal toricities ranging from 1.75 to 3.25 D wore five Paraperm O2 lenses and five Boston II lenses with center thicknesses ranging from 0.10 to 0.20 mm. Flexure and induced residual astigmatism were measured on all corneas wearing all lenses in a double-masked fashion. All lenses studied showed flexure and induced residual astigmatism, which increased as center thickness decreased. For both lens types, lenses thinner than 0.15 mm flexed significantly more than thicker lenses. This critical center thickness should be considered when fitting these lens types on toric corneas.

Adult↗

The Boston bracing system for idiopathic scoliosis. Follow-up results in 295 patients.

A total of 295 patients treated with the Boston bracing system with follow-up of at least 1 year after completion of bracing are reviewed. Pre-brace curves ranged from 20-59 degrees Cobb. Mean age at brace initiation was 13.2 years with a mean treatment time of 2.9 years and mean follow-up of 1.4 years. Mean best in-brace correction averaged 50% with correction averaging 23% at the initiation of weaning from the brace. By the time of brace discontinuance, average curve correction was 15%. At follow-up, average correction was 11%. A comparison of follow-up with pre-brace values of major curves showed that 49% were unchanged +/- 5 degree, 39% achieved final correction of 5-15 degrees, 4% achieved final correction of 15 degrees or more, 4% of patients lost 5-15 degrees, and 3% lost more than 15 degrees by the time of follow-up. Eleven percent of patients underwent surgery during the period of bracing; 1% had surgery during follow-up period. Correction and control of major curves with apexes below T8 and above L2 were best. A strong correlation between best, or initial in-brace correction, and follow-up correction was noted. Young age at the initiation of bracing and higher degrees of pre-brace curvature increased the incidence of surgery. Those curves that had corrected most at the end of bracing were most at risk for loss of correction after bracing. Partial compliance with brace wear appeared as effective as full-time wear. Boston braces without superstructure appeared to be as effective as braces with superstructure for curves with apexes below T7.

Adolescent↗

The biomechanical effectiveness of the Boston brace in the management of adolescent idiopathic scoliosis.

The present study assesses the biomechanical effectiveness of the Boston brace on a group of 14 patients with adolescent scoliosis. Interface pressure measurements were performed at the appropriate compression pads within the brace when it was first applied and on two further sessions within the 6-month period of brace treatment. A corresponding series of Cobb angle measurements also were performed. Mean values of total force exerted through the brace of 58 +/- 18 N were associated with an initial curve correction of 37 +/- 21%. This mean force level was maintained throughout the study, although the mean curve correction at 6 months had reduced to 15 +/- 14%. In addition to biomechanical parameters, factors relating to the nature of the individual curve were important in determining the overall effectiveness of the Boston brace system.

Adolescent↗

Ultrasonographic appearance of Dandy Walker-like Syndrome in a Boston Terrier.

Congenital cerebellar disorders can be caused by in utero or neonatal infections, genetic aberrations causing malformations, and neurodegenerative processes. Most congenital cerebellar disorders are diagnosed definitively with histopathology. However, antemortem diagnosis of cerebellar malformations can be made by imaging the brain. This paper describes the antemortem appearance of a congenital cerebellar malformation in a Boston Terrier puppy on ultrasound images. This appearance was compared with the postmortem findings that were comparable to Dandy Walker Syndrome in humans. A previous report of this syndrome in Boston Terriers suggests the problem may be inherited in this breed.

Animals↗