Background: Early Infantile Krabbe disease (EIKD) is the most common phenotype in the US. Despite... more Background: Early Infantile Krabbe disease (EIKD) is the most common phenotype in the US. Despite this, the only large series in the literature is the Hunter's Hope Krabbe Family Database which relied solely on parent questionnaires. The WorldWide Registry, which includes not only parent questionnaires but medical records, was developed to better understand the natural history of the EIKD phenotype. Objectives: To analyze and report age at symptom onset, symptoms, initial neurodiagnostic testing, and survivals in a large cohort of EIKD patients. Methods: 67 symptomatic children with EIKD were identified in the WWR. Parent questionnaires and medical records were reviewed and analyzed. Transplanted patients were excluded from analysis of survivals. Results: 39 patients were male and 28 were female. Symptom onset was most common in the 3-5 month interval. The most common initial symptoms (>20%) were crying/ irritability, cortical fisting, poor head control and poor feeding. Initial neurodiagnostic testing revealed 92% (n= 25) had elevated CSF protein, 76 %(n = 42) had abnormal MRIs; 67% (n= 15) had abnormal CT scans; 43% (n = 28) had abnormal EEGS; 100% (n= 5) had abnormal NCVs; 83% (n = 6) had abnormal BAERs; and 50% (n= 6) had abnormal VERs. One, 2 and 3 year survivals following onset of symptoms were 47%, 20% and 13%. Overall survivals at 1, 2 and 3 years were 60%, 25% and 3%. Conclusions: Information from the WWR will provide invaluable natural history data for future therapeutic trials in EIKD and other Krabbe phenotypes.
International Journal of Radiation Oncology*Biology*Physics, 1989
Thirty patients with Stages I-III Hodgkin's disease receiving mantle irradiation were prospective... more Thirty patients with Stages I-III Hodgkin's disease receiving mantle irradiation were prospectively evaluated prior to therapy with spirometry, lung volumes, and tests of diffusing capacity (D&O). Follow-up examinations were performed at 3,6, and 12 months and then yearly. Sixteen patients had Hodgkin's disease involving the mediastinum at presentation, 10 were smokers, and 16 received either preirradiation or postirradiation chemotherapy. Mantle doses ranged between 2300 cGy and 4000 cGy (mode of 3750 cGy) given at 150 cGy to 170 cGy tumor dose per day with split-course technique. Pulmonary function test results were translated to percent change from predicted values obtained from normal standards for each age, sex, race, and height. These percent changes were then analyzed as a linear function of time. Twenty patients have been tested 24 years after treatment with a median time from treatment to last pulmonary function test of 8 years. Changes over time in spirometry included an early, mild decrease in both forced vital capacity (FVC) and forced expiratory volume at 1 second (FEVJ, which returned to baseline by 2 years and then gradually decreased to a lo-15% deficit as compared with predicted values at 6-10 years. Additionally, there was a very slight decrease in FEVJFVC beginning at 1 year and gradually increasing to an 8% deficit at 6-10 years. Changes over time in lung volumes included a mild nadir of total lung capacity (TLC) and functional residual capacity (FRC) at 6 months to a year, which returned to baseline at 2-4 years and then gradually dropped to a 5-10% deficit at 6-10 years. Mean DLCO for the study group was 20% below predicted values prior to treatment and dropped to a low of 30% below predicted at 6 months following treatment, then gradually returned to baseline by 4 years and showed continued improvement to an overall deficit of approximately 10% at 6-10 years. With the exception of FEVJFVC, the changes noted in spirometry and lung volumes were of insufficient degree to be classified as abnormal. The decrease in FEVJFVC is indicative of a significant and progressive obstructive ventilatory defect. The effects on pulmonary function tests of smoking, the presence of mediastinal involvement by Hodgkin's disease, and exposure to chemotherapy were assessed by statistical analysis. No subset of patients demonstrated consistent evidence of a restrictive ventilatory defect expected after irradiation. All subsets of patients demonstrated baseline D&O values significantly below predicted values, but significant improvements over time in hC0 were observed in patients presenting with Hodgkin's disease involving the mediastinum. The significant decrease with time in FEVJFVC consistent with a progressive obstructive ventilatory defect was confined to the subset of patients who smoked. Chemotherapy had no clinically significant effect on the pulmonary function test results. In summary, no significant late effects on pulmonary function attributable to mantle irradiation have been observed.
Fifty-six children with chronic renal failure who either received a kidney transplant, were hemod... more Fifty-six children with chronic renal failure who either received a kidney transplant, were hemodialyzed or peritoneally dialyzed, or who were being medically managed were given a series of neuropsychological tests every 6 months for a total of four testing sessions. Each child was matched by age, sex, and race to healthy children who received the same sequence of tests. The performance of each treatment group was compared with their controls longitudinally, using a repeated measure analysis of variance. In general, renal subjects performed at lower levels than their controls on tasks of verbal ability, visual perception, memory and visual motor skills. Visual motor performance was the most clinically affected. When compared with controls, the renal subjects did not improve as much or actually deteriorated on some measures of memory and learning skills.
Background: Mortality in patients with ischemic cardiomyopathy is usually due to cardiac causes; ... more Background: Mortality in patients with ischemic cardiomyopathy is usually due to cardiac causes; however, the mechanism of death is almost equally split between sudden, primarily arrhythmic events ...
1. J Am Diet Assoc. 1983 Jun;82(6):657-9. Assessment of the diet of patients with rheumatoid arth... more 1. J Am Diet Assoc. 1983 Jun;82(6):657-9. Assessment of the diet of patients with rheumatoid arthritis and osteoarthritis. Kowsari B, Finnie SK, Carter RL, Love J, Katz P, Longley S, Panush RS. There have been many and divergent ...
From October 1962 through April 1982, 21 patients with the diagnosis of primary lymphoma of bone ... more From October 1962 through April 1982, 21 patients with the diagnosis of primary lymphoma of bone (18 monostotic, stages IE and IIE; 3 polyostotic) were treated with curative intent. A combination of chemotherapy and radiation therapy was used in 11 patients, local treatment alone in 9 patients, and chemotherapy alone in one patient. Overall 5-year survival for the patients treated with curative intent was 56%. Standard work-up has changed over the 20-year study period. Five-year survival for the subset of eight stage I and II patients with full pretherapy staging was 83%. Prognosis was significantly correlated with extent of pretherapy staging. Treatment parameters that also seemed to predict outcome were the aggressiveness of chemotherapy and the use of irradiation or surgery for local-regional disease; the only local failure occurred in the patient who received chemotherapy alone. Complications of radiation therapy alone and in combination with chemotherapy are discussed and correlated with irradiation dose. Radiation therapy techniques are described, and a management approach is recommended.
The purpose of this study was to assess the effect of flipping the classroom on final exam scores... more The purpose of this study was to assess the effect of flipping the classroom on final exam scores in a terminal general education college mathematics course for a diverse student population. We employed a quasiexperimental design. Seven instructors collectively taught 13 sections of each pedagogy (flipped/traditional). Six hundred thirty-two students participated. Common final exams were graded concurrently. Mixed-model analyses were performed. Students in flipped sections scored 5.1 percentage points higher on average than those in traditional sections (p = .02) when controlling for math SAT and financial aid status, an improvement of 7.8 points among Black students (p < .01) and 1.0 points among Whites (p = .67). The estimated average difference between White and Black students, conditional on covariates, was 5.2 percentage points in traditional sections (p < .01) and –1.6 in flipped sections (p = .39). The 6.8-point difference in achievement gap between pedagogies was stati...
Menu does not appear -- SiteMap. Technical Report No. 15-91. Combining diagnostic categories to i... more Menu does not appear -- SiteMap. Technical Report No. 15-91. Combining diagnostic categories to improve agreement between death certificate and autopsy classifications of cause of death for atomic bomb survivors, 1950-87. Carter RL, Ron E, Mabuchi K. ...
Targeting of ICD therapy to patients at highest risk could be significantly advanced by identifyi... more Targeting of ICD therapy to patients at highest risk could be significantly advanced by identifying factors that predict Background-Previous studies have identified multiple risk factors that are associated with total cardiac mortality. Nevertheless, identifying specific factors that distinguish patients at risk of arrhythmic death versus heart failure could better target patients likely to benefit from implantable cardiac defibrillators, which have no impact on nonsudden cardiac death. Methods and Results-We performed a pilot competing risks analysis of the National Institutes of Health-sponsored PAREPET trial (Prediction of Arrhythmic Events with Positron Emission Tomography). Death from cardiac causes was ascertained in subjects with ischemic cardiomyopathy (n=204) eligible for an implantable cardiac defibrillator for the primary prevention of sudden cardiac arrest after baseline clinical evaluation and imaging at enrollment (positron emission tomography and 2-dimensional echo). Mean age was 67±11 years with an ejection fraction of 27±9%, and 90% were men. During 4.1 years of follow-up, there were 33 sudden cardiac arrests (arrhythmic death or implantable cardiac defibrillator discharge for ventricular fibrillation or ventricular tachycardia >240 bpm) and 36 nonsudden cardiac deaths. Sudden cardiac arrest was correlated with a greater volume of denervated myocardium (defect of the positron emission tomography norepinephrine analog 11 C-hydroxyephedrine), lack of angiotensin inhibition therapy, elevated B-type natriuretic peptide, and larger left ventricular end-diastolic volume index. In contrast, nonsudden cardiac death was associated with a higher resting heart rate, older age, elevated creatinine, larger left atrial volume index, and larger left ventricular end-diastolic volume index. Conclusions-Distinct clinical, laboratory, and imaging variables are associated with cause-specific cardiac mortality in primary-prevention candidates with ischemic cardiomyopathy. If prospectively validated, these multivariable associations may help target specific therapies to those at the greatest risk of sudden and nonsudden cardiac death. Clinical Trial Registration-URL: https://clinicaltrials.gov. Unique identifier: NCT01400334.
International urology and nephrology, Jan 24, 2017
The prevalence of chronic kidney disease (CKD) has been rising steadily in the elderly population... more The prevalence of chronic kidney disease (CKD) has been rising steadily in the elderly population. We studied the rate of progression of CKD in this population and the factors associated with progression of CKD to better identify patients who are likely to progress to ESRD. This was an observational study including 4562 patients older than 65 years with two outpatient estimated glomerular filtration rates (eGFRs) of <60 ml/min/1.73 m(2), at least 90 days apart with no intervening eGFR >60 ml/min/1.73 m(2) (March 1, 2001, and March 31, 2008) at VA healthcare facilities. Patients with eGFR <15 ml/min/1.73 m(2) were excluded. Annual rate of decline of eGFR was studied and categorized as <1 ml/min/1.73 m(2), 1-4 ml/min/1.73 m(2), and >4 ml/min/1.73 m(2). Mean age of the study participants was 77.2 years. 24.3% were diabetics. 4.3% had proteinuria. In univariate comparison of different rates of progression, 54.2% patients had an annual rate of progression of <1 ml/min/1...
Krabbe's disease (KD) is a fatal neurodegenerative disorder, with the early-infantile form (E... more Krabbe's disease (KD) is a fatal neurodegenerative disorder, with the early-infantile form (EIKD) defined by onset of symptoms before age 6 months. Early and highly accurate identification of EIKD is required to maximize benefits of hematopoietic stem cell transplantation treatment. This study investigates the potential for accurate prediction of EIKD based on a novel newborn screening (NBS) tool developed from two biomarkers, galactocerebrosidase (GALC) enzyme activity and galactosylsphingosine concentration (psychosine [PSY]). Normative information about PSY and GALC, derived from distinct samples of normal newborns, was used to develop the novel diagnostic tool. Bivariate normal limits (BVNL) were constructed, assuming a multivariate normal distribution of natural logarithms of GALC and PSY of normal newborns. The (lnGALC, lnPSY) points for newborns in various "abnormal groups," including one group of infants who subsequently suffered EIKD, were plotted on a graph o...
While Apgar scores provide a valid prediction of mortality in term infants (primarily reflecting ... more While Apgar scores provide a valid prediction of mortality in term infants (primarily reflecting asphyxia), intervening variables in premature newborns complicate interpretation. Physiologic states normal to preterm infants (such as, decreased muscle tone) can depress scores but may not influence survival significantly. Therefore the relationship between Apgar scores and survival in term and preterm infants differs. Because of the paucity of studies on preterm infants, we tested Apgar scores, as well as birthweight and gestational age, as outcome predictors in 748 low-birthweight infants (500-1800 gm). Our purpose was to assess the relationship between 1- and 5-minute Apgar scores and survival, and to evaluate all combinations of the four variables as outcome predictors. Univariate analysis showed a significant relationship between each of the four variables and survival; however, no single variable accounted for more than 32% of the variance in outcome, thus no single factor could be invoked as the major determinant of survival. Logistic regression analyses demonstrated the interrelationships of the four variables to survival. While both Apgar scores were related to survival, independent of the effects of birthweight and gestational age, they were slightly less predictive than either of these variables alone. However, when 1- and 5-minute Apgar scores were combined with gestational age, the predictive value was slightly better than any of the four variables alone or in other possible combinations.
To describe trends in mortality rates, in New York State, due to cervical, endometrial and ovaria... more To describe trends in mortality rates, in New York State, due to cervical, endometrial and ovarian cancer and to assess how these rates varied with proximity to a comprehensive cancer treatment center or population density (rural/urban). Data were obtained from the Centers for Disease Control and Prevention (CDC)&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s Compressed Mortality Files, Census Bureau records, and online maps. Poisson regression models were fitted to estimate death rates (mean number of deaths per 100,000 women per year) due to gynecologic cancer type. Trends in death rates were compared with respect to driving time to the nearest comprehensive cancer treatment center and population density, controlling for race, county income level, and age at death. Cervical and endometrial but not ovarian death rates declined over time. For both cervical and endometrial cancers, death rates varied significantly with driving time and between rural and urban counties. In the case of cervical cancer, the decline over time was steeper in rural than in urban counties. For endometrial cancer, the decline steepened with increasing distance from a treatment center. Improvements in cervical and endometrial cancer mortality from 1979 to 2001 followed increases in gynecologic cancer treatment research efforts, number of specialists trained to treat such cases, and in the emphasis on gynecologic cancer in the training of physicians in general. Our results are consistent with an interpretation that the progressive actions by leaders in the gynecologic oncology profession during the late 1960&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s and early 1970&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s contributed to improvements in mortality rates in subsequent decades.
Background: Early Infantile Krabbe disease (EIKD) is the most common phenotype in the US. Despite... more Background: Early Infantile Krabbe disease (EIKD) is the most common phenotype in the US. Despite this, the only large series in the literature is the Hunter's Hope Krabbe Family Database which relied solely on parent questionnaires. The WorldWide Registry, which includes not only parent questionnaires but medical records, was developed to better understand the natural history of the EIKD phenotype. Objectives: To analyze and report age at symptom onset, symptoms, initial neurodiagnostic testing, and survivals in a large cohort of EIKD patients. Methods: 67 symptomatic children with EIKD were identified in the WWR. Parent questionnaires and medical records were reviewed and analyzed. Transplanted patients were excluded from analysis of survivals. Results: 39 patients were male and 28 were female. Symptom onset was most common in the 3-5 month interval. The most common initial symptoms (>20%) were crying/ irritability, cortical fisting, poor head control and poor feeding. Initial neurodiagnostic testing revealed 92% (n= 25) had elevated CSF protein, 76 %(n = 42) had abnormal MRIs; 67% (n= 15) had abnormal CT scans; 43% (n = 28) had abnormal EEGS; 100% (n= 5) had abnormal NCVs; 83% (n = 6) had abnormal BAERs; and 50% (n= 6) had abnormal VERs. One, 2 and 3 year survivals following onset of symptoms were 47%, 20% and 13%. Overall survivals at 1, 2 and 3 years were 60%, 25% and 3%. Conclusions: Information from the WWR will provide invaluable natural history data for future therapeutic trials in EIKD and other Krabbe phenotypes.
International Journal of Radiation Oncology*Biology*Physics, 1989
Thirty patients with Stages I-III Hodgkin's disease receiving mantle irradiation were prospective... more Thirty patients with Stages I-III Hodgkin's disease receiving mantle irradiation were prospectively evaluated prior to therapy with spirometry, lung volumes, and tests of diffusing capacity (D&O). Follow-up examinations were performed at 3,6, and 12 months and then yearly. Sixteen patients had Hodgkin's disease involving the mediastinum at presentation, 10 were smokers, and 16 received either preirradiation or postirradiation chemotherapy. Mantle doses ranged between 2300 cGy and 4000 cGy (mode of 3750 cGy) given at 150 cGy to 170 cGy tumor dose per day with split-course technique. Pulmonary function test results were translated to percent change from predicted values obtained from normal standards for each age, sex, race, and height. These percent changes were then analyzed as a linear function of time. Twenty patients have been tested 24 years after treatment with a median time from treatment to last pulmonary function test of 8 years. Changes over time in spirometry included an early, mild decrease in both forced vital capacity (FVC) and forced expiratory volume at 1 second (FEVJ, which returned to baseline by 2 years and then gradually decreased to a lo-15% deficit as compared with predicted values at 6-10 years. Additionally, there was a very slight decrease in FEVJFVC beginning at 1 year and gradually increasing to an 8% deficit at 6-10 years. Changes over time in lung volumes included a mild nadir of total lung capacity (TLC) and functional residual capacity (FRC) at 6 months to a year, which returned to baseline at 2-4 years and then gradually dropped to a 5-10% deficit at 6-10 years. Mean DLCO for the study group was 20% below predicted values prior to treatment and dropped to a low of 30% below predicted at 6 months following treatment, then gradually returned to baseline by 4 years and showed continued improvement to an overall deficit of approximately 10% at 6-10 years. With the exception of FEVJFVC, the changes noted in spirometry and lung volumes were of insufficient degree to be classified as abnormal. The decrease in FEVJFVC is indicative of a significant and progressive obstructive ventilatory defect. The effects on pulmonary function tests of smoking, the presence of mediastinal involvement by Hodgkin's disease, and exposure to chemotherapy were assessed by statistical analysis. No subset of patients demonstrated consistent evidence of a restrictive ventilatory defect expected after irradiation. All subsets of patients demonstrated baseline D&O values significantly below predicted values, but significant improvements over time in hC0 were observed in patients presenting with Hodgkin's disease involving the mediastinum. The significant decrease with time in FEVJFVC consistent with a progressive obstructive ventilatory defect was confined to the subset of patients who smoked. Chemotherapy had no clinically significant effect on the pulmonary function test results. In summary, no significant late effects on pulmonary function attributable to mantle irradiation have been observed.
Fifty-six children with chronic renal failure who either received a kidney transplant, were hemod... more Fifty-six children with chronic renal failure who either received a kidney transplant, were hemodialyzed or peritoneally dialyzed, or who were being medically managed were given a series of neuropsychological tests every 6 months for a total of four testing sessions. Each child was matched by age, sex, and race to healthy children who received the same sequence of tests. The performance of each treatment group was compared with their controls longitudinally, using a repeated measure analysis of variance. In general, renal subjects performed at lower levels than their controls on tasks of verbal ability, visual perception, memory and visual motor skills. Visual motor performance was the most clinically affected. When compared with controls, the renal subjects did not improve as much or actually deteriorated on some measures of memory and learning skills.
Background: Mortality in patients with ischemic cardiomyopathy is usually due to cardiac causes; ... more Background: Mortality in patients with ischemic cardiomyopathy is usually due to cardiac causes; however, the mechanism of death is almost equally split between sudden, primarily arrhythmic events ...
1. J Am Diet Assoc. 1983 Jun;82(6):657-9. Assessment of the diet of patients with rheumatoid arth... more 1. J Am Diet Assoc. 1983 Jun;82(6):657-9. Assessment of the diet of patients with rheumatoid arthritis and osteoarthritis. Kowsari B, Finnie SK, Carter RL, Love J, Katz P, Longley S, Panush RS. There have been many and divergent ...
From October 1962 through April 1982, 21 patients with the diagnosis of primary lymphoma of bone ... more From October 1962 through April 1982, 21 patients with the diagnosis of primary lymphoma of bone (18 monostotic, stages IE and IIE; 3 polyostotic) were treated with curative intent. A combination of chemotherapy and radiation therapy was used in 11 patients, local treatment alone in 9 patients, and chemotherapy alone in one patient. Overall 5-year survival for the patients treated with curative intent was 56%. Standard work-up has changed over the 20-year study period. Five-year survival for the subset of eight stage I and II patients with full pretherapy staging was 83%. Prognosis was significantly correlated with extent of pretherapy staging. Treatment parameters that also seemed to predict outcome were the aggressiveness of chemotherapy and the use of irradiation or surgery for local-regional disease; the only local failure occurred in the patient who received chemotherapy alone. Complications of radiation therapy alone and in combination with chemotherapy are discussed and correlated with irradiation dose. Radiation therapy techniques are described, and a management approach is recommended.
The purpose of this study was to assess the effect of flipping the classroom on final exam scores... more The purpose of this study was to assess the effect of flipping the classroom on final exam scores in a terminal general education college mathematics course for a diverse student population. We employed a quasiexperimental design. Seven instructors collectively taught 13 sections of each pedagogy (flipped/traditional). Six hundred thirty-two students participated. Common final exams were graded concurrently. Mixed-model analyses were performed. Students in flipped sections scored 5.1 percentage points higher on average than those in traditional sections (p = .02) when controlling for math SAT and financial aid status, an improvement of 7.8 points among Black students (p < .01) and 1.0 points among Whites (p = .67). The estimated average difference between White and Black students, conditional on covariates, was 5.2 percentage points in traditional sections (p < .01) and –1.6 in flipped sections (p = .39). The 6.8-point difference in achievement gap between pedagogies was stati...
Menu does not appear -- SiteMap. Technical Report No. 15-91. Combining diagnostic categories to i... more Menu does not appear -- SiteMap. Technical Report No. 15-91. Combining diagnostic categories to improve agreement between death certificate and autopsy classifications of cause of death for atomic bomb survivors, 1950-87. Carter RL, Ron E, Mabuchi K. ...
Targeting of ICD therapy to patients at highest risk could be significantly advanced by identifyi... more Targeting of ICD therapy to patients at highest risk could be significantly advanced by identifying factors that predict Background-Previous studies have identified multiple risk factors that are associated with total cardiac mortality. Nevertheless, identifying specific factors that distinguish patients at risk of arrhythmic death versus heart failure could better target patients likely to benefit from implantable cardiac defibrillators, which have no impact on nonsudden cardiac death. Methods and Results-We performed a pilot competing risks analysis of the National Institutes of Health-sponsored PAREPET trial (Prediction of Arrhythmic Events with Positron Emission Tomography). Death from cardiac causes was ascertained in subjects with ischemic cardiomyopathy (n=204) eligible for an implantable cardiac defibrillator for the primary prevention of sudden cardiac arrest after baseline clinical evaluation and imaging at enrollment (positron emission tomography and 2-dimensional echo). Mean age was 67±11 years with an ejection fraction of 27±9%, and 90% were men. During 4.1 years of follow-up, there were 33 sudden cardiac arrests (arrhythmic death or implantable cardiac defibrillator discharge for ventricular fibrillation or ventricular tachycardia >240 bpm) and 36 nonsudden cardiac deaths. Sudden cardiac arrest was correlated with a greater volume of denervated myocardium (defect of the positron emission tomography norepinephrine analog 11 C-hydroxyephedrine), lack of angiotensin inhibition therapy, elevated B-type natriuretic peptide, and larger left ventricular end-diastolic volume index. In contrast, nonsudden cardiac death was associated with a higher resting heart rate, older age, elevated creatinine, larger left atrial volume index, and larger left ventricular end-diastolic volume index. Conclusions-Distinct clinical, laboratory, and imaging variables are associated with cause-specific cardiac mortality in primary-prevention candidates with ischemic cardiomyopathy. If prospectively validated, these multivariable associations may help target specific therapies to those at the greatest risk of sudden and nonsudden cardiac death. Clinical Trial Registration-URL: https://clinicaltrials.gov. Unique identifier: NCT01400334.
International urology and nephrology, Jan 24, 2017
The prevalence of chronic kidney disease (CKD) has been rising steadily in the elderly population... more The prevalence of chronic kidney disease (CKD) has been rising steadily in the elderly population. We studied the rate of progression of CKD in this population and the factors associated with progression of CKD to better identify patients who are likely to progress to ESRD. This was an observational study including 4562 patients older than 65 years with two outpatient estimated glomerular filtration rates (eGFRs) of <60 ml/min/1.73 m(2), at least 90 days apart with no intervening eGFR >60 ml/min/1.73 m(2) (March 1, 2001, and March 31, 2008) at VA healthcare facilities. Patients with eGFR <15 ml/min/1.73 m(2) were excluded. Annual rate of decline of eGFR was studied and categorized as <1 ml/min/1.73 m(2), 1-4 ml/min/1.73 m(2), and >4 ml/min/1.73 m(2). Mean age of the study participants was 77.2 years. 24.3% were diabetics. 4.3% had proteinuria. In univariate comparison of different rates of progression, 54.2% patients had an annual rate of progression of <1 ml/min/1...
Krabbe's disease (KD) is a fatal neurodegenerative disorder, with the early-infantile form (E... more Krabbe's disease (KD) is a fatal neurodegenerative disorder, with the early-infantile form (EIKD) defined by onset of symptoms before age 6 months. Early and highly accurate identification of EIKD is required to maximize benefits of hematopoietic stem cell transplantation treatment. This study investigates the potential for accurate prediction of EIKD based on a novel newborn screening (NBS) tool developed from two biomarkers, galactocerebrosidase (GALC) enzyme activity and galactosylsphingosine concentration (psychosine [PSY]). Normative information about PSY and GALC, derived from distinct samples of normal newborns, was used to develop the novel diagnostic tool. Bivariate normal limits (BVNL) were constructed, assuming a multivariate normal distribution of natural logarithms of GALC and PSY of normal newborns. The (lnGALC, lnPSY) points for newborns in various "abnormal groups," including one group of infants who subsequently suffered EIKD, were plotted on a graph o...
While Apgar scores provide a valid prediction of mortality in term infants (primarily reflecting ... more While Apgar scores provide a valid prediction of mortality in term infants (primarily reflecting asphyxia), intervening variables in premature newborns complicate interpretation. Physiologic states normal to preterm infants (such as, decreased muscle tone) can depress scores but may not influence survival significantly. Therefore the relationship between Apgar scores and survival in term and preterm infants differs. Because of the paucity of studies on preterm infants, we tested Apgar scores, as well as birthweight and gestational age, as outcome predictors in 748 low-birthweight infants (500-1800 gm). Our purpose was to assess the relationship between 1- and 5-minute Apgar scores and survival, and to evaluate all combinations of the four variables as outcome predictors. Univariate analysis showed a significant relationship between each of the four variables and survival; however, no single variable accounted for more than 32% of the variance in outcome, thus no single factor could be invoked as the major determinant of survival. Logistic regression analyses demonstrated the interrelationships of the four variables to survival. While both Apgar scores were related to survival, independent of the effects of birthweight and gestational age, they were slightly less predictive than either of these variables alone. However, when 1- and 5-minute Apgar scores were combined with gestational age, the predictive value was slightly better than any of the four variables alone or in other possible combinations.
To describe trends in mortality rates, in New York State, due to cervical, endometrial and ovaria... more To describe trends in mortality rates, in New York State, due to cervical, endometrial and ovarian cancer and to assess how these rates varied with proximity to a comprehensive cancer treatment center or population density (rural/urban). Data were obtained from the Centers for Disease Control and Prevention (CDC)&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s Compressed Mortality Files, Census Bureau records, and online maps. Poisson regression models were fitted to estimate death rates (mean number of deaths per 100,000 women per year) due to gynecologic cancer type. Trends in death rates were compared with respect to driving time to the nearest comprehensive cancer treatment center and population density, controlling for race, county income level, and age at death. Cervical and endometrial but not ovarian death rates declined over time. For both cervical and endometrial cancers, death rates varied significantly with driving time and between rural and urban counties. In the case of cervical cancer, the decline over time was steeper in rural than in urban counties. For endometrial cancer, the decline steepened with increasing distance from a treatment center. Improvements in cervical and endometrial cancer mortality from 1979 to 2001 followed increases in gynecologic cancer treatment research efforts, number of specialists trained to treat such cases, and in the emphasis on gynecologic cancer in the training of physicians in general. Our results are consistent with an interpretation that the progressive actions by leaders in the gynecologic oncology profession during the late 1960&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s and early 1970&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s contributed to improvements in mortality rates in subsequent decades.
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