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B H Cohen

Publications and source records attributed to B H Cohen.

At least 19 recordsLinked to original sources
PubMed1992

Progressive vision loss. A rare manifestation of familial cavernous angiomas.

We studied four generations of a family in which the index case had progressive loss of vision secondary to a cavernous angioma of the optic nerve and chiasm. Magnetic resonance imaging of the brain revealed multiple, asymptomatic intracerebral cavernous angiomas. Brain magnetic resonance imaging scans of the family members revealed multiple cavernous angiomas in the brother and paternal grandfather, but none in the father or his siblings. Autopsy reports of the paternal great grandfather noted multiple cavernous angiomas in the brain and abdominal viscera. We believe our patient to be the sixth reported case in which a cavernous angioma involved the optic chiasm and optic nerve. Magnetic resonance imaging is a sensitive and specific method of detecting cavernous angiomas. Cavernous angiomas have an autosomal dominant pattern of inheritance with variable penetrance. Surgical intervention in patients with symptomatic cavernous angiomas depends on the location and size of the lesion and associated surgical risks.

Adolescent↗
PubMed1992

Muscle tension patterns during auditory attention.

Although there is much evidence demonstrating muscle tension changes during mental work, there are few data concerning muscle tension patterns during effortful attention to simple sensory stimuli. In the present study, sensory attention was evoked by a pitch discrimination task at three levels of difficulty, with a digit retention task administered for comparison. Twenty-four females each performed both tasks at all levels of difficulty, while the EKG, and the corrugator supercilii, frontalis, lip, jaw, chin, and forearm area EMG were recorded. As expected, heart rate decreased significantly with increasing difficulty of the pitch task. A pattern of facial EMG responses accompanied the pitch task, which included significant increases in corrugator and frontalis, and decreases in the jaw as a function of difficulty, and time within trials. The tension pattern observed during sensory intake is discussed in terms of its relation to emotional expressions and motor theories of attention.

Acoustic Stimulation↗
PubMed1992

Estimating familial aggregation while adjusting for covariates. Application to pulmonary function data from black and white sibships.

Although crude correlations are useful in family studies, some adjustment for effects of risk factors that vary both within and among families if often needed. A linear model for estimating sibship correlations while simultaneously considering height, age, race, sex, ascertainment, and smoking status was used on pulmonary function data on 1-second forced expiratory volume (FEV1) and the natural logarithm of the ratio of FEV1 to forced vital capacity (lnFEV%) from 402 adults in 152 white sibships and 172 adults in 59 black sibships. Crude correlations of .271 +/- .048 (FEV1) and .342 +/- .047 (lnFEV%) decreased significantly to .206 +/- .048 and .231 +/- .048, respectively, after adjustment. For black and white sibs, adjusted intraclass correlations, although not statistically different, were .153 +/- .089 and .225 +/- .055 (FEV1), respectively, and were .103 +/- .088 and .275 +/- .054 (lnFEV%), respectively, suggesting that pulmonary function may aggregate more strongly among whites. This analysis illustrates how risk factor adjustment can be readily incorporated into familial correlation studies.

Adult↗
PubMed1992

The suture tension adjustment reel. A new device for the management of skin closure.

The initial clinical experience with a new device, called the Suture Tension Adjustment Reel (STAR), is described. The historical precedence and rationale underlying linear tissue expansion is presented. Case reports illustrate the use of the STAR for preoperative tissue stretching, for intraoperative tissue stretching, and for tissue mobilization as applied to scalp reduction surgery. The multipurpose device is a safe, simple, and reliable adjunct to sutured surgery. It is of value in the general management of tensioned and nontensioned skin closures.

Adult↗
PubMed1991

Applications of the concept of attributable fraction in medical genetics.

Attributable fraction, the fraction of cases of a disease in a population attributed to a particular risk factor, is a useful measure in the design and interpretation of epidemiologic studies of disease etiology. We review here the applications of the concept of attributable fraction in medical genetics. Specifically, attributable fraction can be used 1) in studies of the association between genetic traits and specific diseases to quantitate the contribution of specific alleles to disease occurrence in a population; 2) in population studies of mutations and birth defects to estimate the impact of mutagens and teratogens; and 3) in genetic analyses of family data, to evaluate the contribution of putative single gene loci to disease etiology. In the latter context, the concept of attributable fraction can be contrasted with the more commonly used concept of heritability. Examples from the literature provide illustrations of the usefulness of attributable fraction in medical genetic studies.

Alleles↗
PubMed1991

Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma.

Between 1975 and 1989, 108 children with newly diagnosed medulloblastoma/primitive neuroectodermal tumor (MB/PNET) of the posterior fossa were treated at the authors' institution. The patients were managed uniformly, and treatment included aggressive surgical resections, postoperative staging evaluations for extent of disease, and craniospinal radiation therapy with a local boost. Beginning in 1983, children with MB/PNET were prospectively assigned to risk groups; those with "standard-risk" MB/PNET were treated with radiation therapy alone, while those in the "poor-risk" group received similar radiation therapy plus adjuvant chemotherapy with 1-(2-chloroethyl)-3-cyclohexyl-1-nitrosourea (CCNU), vincristine, and cisplatin. The 5-year actuarial disease-free survival rate for all patients treated between 1975 and 1982 was 68%, and 73% when patients who died within 2 weeks after operation were excluded. This survival rate was statistically better for patients treated after 1982 (82%) compared to those treated between 1975 and 1982 (49%) (p less than 0.004). There was no difference in disease-free survival rates over time for children with standard-risk factors; however, there was a significant difference in the 5-year survival rate for poor-risk patients treated prior to 1982 (35%) compared to those treated later (87%) (p less than 0.001). For the group as a whole, a younger age at diagnosis correlated with a poorer survival rate; however, this relationship between age and outcome was significant only for children treated before 1983 (p less than 0.001). These results demonstrated an encouraging survival rate for children with MB/PNET, especially those treated with aggressive surgical resection followed by both radiation therapy and chemotherapy. The results strongly suggest that chemotherapy has a role for some, and possibly all, children with MB/PNET.

Adolescent↗
PubMed1990

Hyperfractionated radiotherapy for children with brainstem gliomas: a pilot study using 7,200 cGy.

Brainstem gliomas, constituting approximately 10% of all childhood central nervous system tumors, remain the most resistant of all brain tumors to therapy. A subgroup of high-risk patients with tumors that diffusely involve the brainstem or that microscopically demonstrate foci of anaplasia on biopsy specimens rarely survive after treatment. Conventional doses of radiotherapy result in temporary clinical improvement in the majority of these high-risk patients; however, few if any remain alive 18 months after treatment. Hyperfractionated radiotherapy, with delivery of larger numbers of smaller fractions of radiotherapy, is a possible way to increase tumor control without increasing neurological toxicity. In 1985, a multiinstitutional phase I/phase II trial, using 100 cGy of radiation therapy twice daily to a total dose of 7,200 cGy, was undertaken for patients with high-risk brainstem gliomas. At the time of writing, 24 (69%) had developed progressive disease and 11 remained in continuous progression-free remission. Actuarial progression-free survival at 20 months is approximately 30%. Twenty-three of 31 evaluable patients had an objective radiographic response to therapy. In comparison to both historical control patients and patients treated in a previous trial using 6,480 cGy of hyperfractionated radiation therapy, there was a statistically significant improvement in progression-free survival rate for patients treated with 7,200 cGy of hyperfractionated radiation therapy (p less than 0.01). To date no patient has died as a result of treatment. Six patients developed transient neurological deterioration or cystic intralesional changes, as demonstrated on magnetic resonance imaging, within 6 weeks of the completion of radiotherapy. Postmortem examination performed in 7 patients did not disclose significant radiation necrosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1990

Major genetic mechanisms in pulmonary function.

Regressive models were used to search for possible major gene effects on pulmonary function in two groups of families: one ascertained through patients with chronic obstructive pulmonary disease [COPD defined as forced expiratory volume in one second (FEV1) less than 70% forced vital capacity (FVC)] and the other ascertained through patients with non-pulmonary disorders. There were 85 COPD families with data on 270 individuals and 56 non-pulmonary families with data on 199 individuals. The analysis was done on residuals obtained from a regression of FEV1 on age, sex, race, height, and ascertainment group. Smoking status was incorporated directly as a covariate in the regressive models. Data on probands were excluded in this analysis as a partial correction for ascertainment bias. The best fitting model for the 85 COPD families included a major gene effect with sex specific variances, but no residual familial correlation. The best fitting model for the non-pulmonary families was one with no major gene effect and no residual familial correlation. Cigarette smoking was a significant covariate in both groups of families. Testing for heterogeneity showed a significant difference in the control of pulmonary function among these COPD and non-pulmonary families (X2 = 20.12 on 6 df; p = 0.0026). Major gene effects appear to be limited to these COPD families, while there was no evidence for major gene effects in the non-pulmonary families.

Adult↗
PubMed1990

Path analysis of familial resemblance of pulmonary function and cigarette smoking.

The techniques of path analysis were utilized to assess the relative importance of genetic factors, personal smoking behavior, and shared environment in the resemblance of pulmonary function among relatives using both cross-sectional and longitudinal data from nuclear families. Data on 1-s forced expiratory volume, FEV1 (adjusted for age, sex, race, height, and ascertainment group) and the number of cigarettes smoked per day were available on 978 individuals in 384 nuclear families residing in the Baltimore metropolitan area. All these individuals were seen twice between 1971 and 1981, with an average of 5 yr between visits. The direct effect of an individual's own smoking explained 10 and 3% of variation in adjusted FEV1 among parents and offspring, respectively. Shared environmental factors influencing personal smoking behavior accounted for 5% of the parent-offspring correlation in adjusted FEV1 and 3% of the sibling correlation in adjusted FEV1 in this sample. Undefined environmental factors that influenced an individual's smoking habits and could be shared among relatives were found to explain 19% of the familial correlations in smoking. Genetic heritability estimates ranged between 36 and 40%, with no evidence of intergenerational differences in the expression of apparent genetic control of pulmonary function.

Adult↗
PubMed1990

Localized 31P magnetic resonance spectroscopy of large pediatric brain tumors.

Fourteen children aged 1 week to 16 years, with a variety of large or superficial brain tumors, underwent localized in vivo 31P magnetic resonance spectroscopy of their tumor. Quantitative spectral analysis was performed by measuring the area under individual peaks using a computer algorithm. In eight patients with histologically benign tumors the spectra were considered to be qualitatively indistinguishable from normal brain. The phosphocreatine/inorganic phosphate ratio (PCr/Pi) averaged 2.0. Five patients had histologically malignant tumors; qualitatively, four of these were considered to have abnormal spectra, showing a decrease in the PCr peak. The PCr/Pi ratio for this group averaged 0.85, which was significantly lower than that seen in the benign tumor group (p less than 0.05). No difference between the two groups was seen in adenosine triphosphate or phosphomonoesters. It is concluded that a specific metabolic "fingerprint" for childhood brain tumors may not exist, but that some malignant tumors show a pattern suggestive of ischemia.

Adenosine Triphosphate↗
PubMed1989

Vitamin A, cigarette smoking, and airway obstruction.

The hypothesis that vitamin A, or foods rich in vitamin A such as milk, protects against the development of airway obstruction was tested in a subsample of data from the 1971-1975 National Health and Nutrition Examination Survey. The relative risk (estimated by relative odds) of having airway obstruction, defined as a FEV1/FVC less than or equal to 65%, was measured for different levels of nutrient and food group intakes after adjustment for age, sex, Quetelet Index, caloric intake, and cigarette smoking. Among the white respondents (never smokers or current smokers) whose recalled diet could be considered as typical of their usual diet, the relative odds of having airway obstruction was inversely related to vitamin A intake (x2 for trend = 7.2, p less than 0.01). In the analysis by food groups, the adjusted relative odds of airway obstruction for meat and poultry were 2.4 (95% confidence limits: 1.0 to 5.8) and for milk 1.6 (95% confidence limits: 1.0 to 2.5). The association of airway obstruction with lack of vitamin A or milk intake was clearer among smokers. These findings suggest that a diet poor in vitamin A increases the risk of airway obstruction, and are consistent with the previously reported association of chronic bronchitis, milk intake, and smoking.

Adult↗
PubMed1989

Gadolinium-DTPA-enhanced magnetic resonance imaging in childhood brain tumors.

Gadolinium DTPA (Gd-DTPA) is a paramagnetic blood-brain barrier contrast agent for MRI that has been used primarily in adults. During May through October 1987, 17 children between the ages of 3 and 18 years with brain tumors underwent MRI examinations, before and after Gd-DTPA (11 gliomas, 4 medulloblastomas, 1 craniopharyngioma, and 1 child with neurofibromatosis and no pathologic diagnosis). We compared T1 and T2 Gd-DTPA-enhanced MRI with concurrent unenhanced MRI and enhanced CT, and then correlated this with the clinical and pathologic findings. Gd-DTPA enhanced tumors in all 7 patients with newly diagnosed tumors and enhanced tumors in 7 of 10 patients without clinical evidence of progressive disease at the time of the study. In the 7 new patients, Gd-DTPA defined tumor margins in all, and demonstrated internal tumor architecture (vessels, necrosis, and cysts) in 5. Areas believed to represent surgical scars showed varying degrees of enhancement. Leptomeningeal tumor spread, including spinal, not seen on pre-Gd-DTPA MRI or on contrast CT, was evident in 2 patients. Gd-DTPA enhancement obscured hemorrhage within the tumor (methemoglobin) in 2 patients. There were no significant side effects. These results suggest that Gd-DTPA-enhanced MRI (1) is safe in children, (2) demonstrates the extent and character of tumors better than unenhanced MRI and enhanced CT, and (3) may allow for noninvasive imaging of leptomeningeal disease, including the spine, not previously demonstrated by any other noninvasive neuroimaging technique.

Adolescent↗
PubMed1989

Incidence, types, and management of cancer in patients with neurofibromatosis.

Neurofibromatosis Type 1 (NF1) is a common genetic disease with an incidence of 1 in 3,000 to 4,000. Clinical manifestations may include cafe-au-lait macules, axillary freckling, neurofibromas, Lisch nodules, skeletal dysplasias, learning disabilities, and a variety of neoplasms. In persons with NF1, some malignancies occur at an increased incidence compared to the general population. These include central nervous system astrocytomas, especially involving the visual pathways; ependymomas; meningiomas; neurofibrosarcomas; rhabdomyosarcomas; nonlymphocytic leukemias; and pheochromocytomas. The authors discuss the dysplastic and malignant features associated with NF and the concept of NF as a "neurocristopathy."

Adult↗
PubMed1989

Cancer in relatives of leukemic patients with chromosomal rearrangements at rare (heritable) fragile-site locations in their malignant cells.

The cancer occurrence in relatives (N = 407) of 40 case probands (who had leukemia and rearrangements at the same chromosomal location as at least one of 23 recognized rare [heritable] autosomal fragile sites [Sutherland and Mattei 1987]) was compared both to cancer occurrence in relatives (N = 390) of 40 control probands (who had leukemia or other hematologic illness but no recognized chromosomal rearrangements) and to cancer incidence in the general population of the United States. Fragile-site carrier status was not determined in case or control probands. No significant excess of cancer in case relatives, compared with either control relatives or to general (SEER) population expectancies, was found. Furthermore, there was neither evidence of cancer at younger ages, when cases were compared with control relatives, nor an excess of cancer at multiple sites. Male relatives of cases did, however, show a small excess of cancer, especially in older age groups. There was a slight, but not statistically significant, excess of lung cancer in case relatives, with this deviation occurring almost exclusively in relatives of probands having rearrangements at 11q23 and having lymphoid leukemia. It is possible that heritable tendency to chromosomal rearrangement--and thus to cancer--is expressed in such a small proportion of family members that cancer excess in these families could not be detected with the numbers of relatives analyzed in this study, although there was no significant evidence for a hereditary predisposition to cancer in the families of probands with leukemia and with chromosomal rearrangements at the same apparent chromosomal location as rare fragile sites.

Adolescent↗
PubMed1988

Perceiver bias in the processing of human faces: neuropsychological mechanisms.

Previous research has suggested that in face-to-face contexts perceivers are biased to judge the side of the poser's face to their left as more similar to the full face than the side to their right. Traditional explanations of the perceiver bias have presumed that it is a visual field effect, with the side of the poser's face falling within the perceiver's left visual field dominating impressions of the full face. In this study, five experiments are reported. In the first experiment, the validity of the perceiver bias phenomenon was supported. The remaining experiments examined three alternative accounts of the neuropsychological processes that underlie the perceiver bias. No support was obtained for the visual field explanation, nor for an account of the bias as due to asymmetry in gaze patterns. Support was obtained for an account emphasizing a hemispatial bias in central processing. Despite equivalent intake of information from both sides of space, the brain may differentially weight information as a function of hemispatial origin. Practical and theoretical implications are discussed.

Adult↗
PubMed1988

Microcystic adnexal carcinoma: a case showing eccrine duct differentiation.

Microcystic adnexal carcinoma (MAC) is a cutaneous neoplasm comprised of pilar and sweat duct structures. Characteristically, the ductal structures manifest immunoreactivity for carcinoembryonic antigen (CEA) on immunostaining. The case of MAC discussed here shows strictly sweat duct differentiation. Using a monoclonal antibody to salivary mucin (CF-1) that is specific for eccrine duct, we identify the neoplastic structures to be of eccrine sweat duct histogenesis.

Aged↗