Invasive lobular carcinoma of the breast; clinicopathologic profile and response to neoadjuvant chemotherapy over a 15-year period.

INTRODUCTION: Invasive lobular carcinoma (ILC) accounts for 5-15% of invasive breast cancers. Typical ILC is oestrogen receptor (ER) positive and human epidermal growth factor receptor 2 (HER2) negative. Atypical biomarker profiles (ER- and HER2+, ER+ and HER2+ or triple negative) appear to differ from typical ILCs. This study compared subtypes of ILC in terms of clinical and pathological parameters, and response to neoadjuvant chemotherapy (NACT) according to biomarker profile. METHODS: All patients with ILC treated in a single centre from January 2005 to December 2020 were identified from a prospectively maintained database. Clinicopathologic and outcome data was collected and analysed according to tumour biomarker profile. RESULTS: A total of 582 patients with ILC were treated. Typical ILC was observed in 89.2% (n = 519) and atypical in 10.8% (n = 63). Atypical ILCs were of a higher grade (35% grade 3 vs 9.6% grade 3, p < 0.001). A larger proportion of atypical ILC received NACT (31.7% vs 6.9% p < 0.001). Atypical ILCs showed a greater response to NACT (mean RCB (Residual Cancer Burden Score) 2.46 vs mean RCB 3.41, p = 0.0365), and higher pathological complete response rates (15% vs 0% p = 0.017). Despite this, overall 5-year disease-free survival (DFS) was higher in patients with typical ILC (91% vs 83%, p = 0.001). CONCLUSIONS: Atypical ILCs have distinct characteristics. They are more frequently of a higher grade and demonstrate a superior response to NACT. Despite the latter, atypical ILCs have a worse 5-year DFS which should be taken into consideration in terms of prognostication and may assist patient selection for NACT.

Factors associated with the reporting of Down syndrome as the underlying cause of death on US death certificates.

BACKGROUND: Efforts aimed at preventing premature mortality for people with Down syndrome are hindered by the practice of reporting disability as the underlying cause of death. Prior research suggests this form of diagnostic overshadowing may be the result of increased uncertainty surrounding the death. METHODS: This study uses bivariable analysis and multivariable logistic regression models to investigate associations between sociodemographic characteristics, comorbidities, and death context and processing characteristics with the reporting of Down syndrome as the underlying cause of death in 2005-2017 US Multiple Cause of Death data files. RESULTS: The reporting of Down syndrome as the underlying cause of death was associated with characteristics indicative of an increased amount of uncertainty surrounding the death. Results also suggest other mechanisms may inform inaccurate reporting, such as racial bias, and the continued conflation of disability and health. CONCLUSIONS: Medical personnel certifying death certificates should strive for accuracy when reporting the causes of death. To ensure this outcome, even in the midst of increased uncertainty, Down syndrome should not be reported as the underlying cause of death unless the decedent was diagnosed with Alzheimer's disease or unspecified dementia. Future research should further explore the possibility that increased death certification errors for adults with Down syndrome, or other developmental disabilities, are associated with racial bias.

Cause of death in adults with intellectual disability in the United States.

BACKGROUND: Prior studies report that adults with intellectual disability (ID) have cause of death patterns distinct from adults in the general population but do not provide comparative analysis by specific causes of death. METHODS: Data are from the National Vital Statistics System 2005-2017 US Multiple Cause-of-Death Mortality files. We utilised adjusted odds ratios to identify causes of death that were more common for adults whose death certificate indicated ID (N = 22 512) than for adults whose death certificate did not indicate ID (N = 32 738 229), controlling for severity level of ID. We then examine the associations between biological sex and race-ethnicity and causes of death solely among adults with ID. RESULTS: The leading cause of death for adults with and without ID indicated on their death certificate was heart disease. Adults with ID, regardless of the severity of the disability, had substantially higher risk of death from pneumonitis, influenza/pneumonia and choking. Adults with mild/moderate ID also had higher risk of death from diabetes mellitus. Differences in cause of death trends were associated with biological sex and race-ethnicity. CONCLUSIONS: Efforts to reduce premature mortality for adults with ID should attend to risk factors for causes of death typical in the general population such as heart disease and cancer, but also should be cognisant of increased risk of death from choking among all adults with ID, and diabetes among adults with mild/moderate ID. Further research is needed to better understand the factors determining comparatively lower rates of death from neoplasms and demographic differences in causes of death among adults with ID.

Heterogeneity in age at death for adults with developmental disability.

BACKGROUND: Although increased attention has been devoted to mortality trends for adults with developmental disability, research has not accounted for possible differences in age at death between disability types. We examine whether heterogeneity is present in age at death between adults with different types of developmental disability. METHODS: Data were from the 2012-2016 U.S. Multiple Cause-of-Death Mortality files. Mean age at death and age at death distributions were analysed for adults, aged 18-126, with and without developmental disability collectively and then stratified by biological sex. RESULTS: There were 33 154 decedents with and 13 026 759 without developmental disability. Compared with adults without developmental disability, age at death was lower for all decedents with developmental disability but varied markedly by disability type and biological sex. Among adults with developmental disability, those with intellectual disability had the highest age at death, and those with cerebral palsy or other rare developmental disabilities, especially if co-morbid for a second developmental disability, had the lowest age at death. CONCLUSION: Research on age at death for adults with developmental disability must account for heterogeneity among disability types in order to ensure reliable estimates. Failure to do so conceals important differences between disability types, which can misguide public health and preventive care efforts to reduce premature mortality and/or provide aging-related supports.

Functional impairment severity is associated with health status among older persons with intellectual disability and cerebral palsy.

BACKGROUND: Studies have noted high rates of specific health disorders in adults with cerebral palsy (CP). However, it remains unclear how growing older with a lifelong neuromotor physical disability confers risk for health outcomes in adults who have both intellectual disability (ID) and CP. AIM: To assess the relationship between health status in older adults with ID either with or without coincident diagnoses of CP. METHOD: Health status data were drawn from 1373 adults aged 33 to 79 years with ID living in small group homes in New York State. Their health status was defined by the presence of common health disorders. Of these, 177 subjects had coincident CP. Prevalence data for nine diseases representing different organ systems were obtained and compared in individuals with and without CP. A Severity of Functional Impairment Index (SFII) was developed based on subjects' capabilities in activities of daily living (ADLs) and mobility. Two logistic regression analyses were conducted to determine if CP diagnosis was an independent predictor of health disorder prevalence, or rather exerted effects similar to those without CP via severity of functional impairment as determined by SFII scores. In addition, older age, gender, and severity of intellectual disability were examined as predictors of health disorder prevalence in all study subjects. RESULTS: Individuals with CP had higher frequencies in four out of the nine health disorders (overweight/obesity, gastroesophageal reflux, urinary tract infections and dysphagia). Analysis revealed a statistically significant association between SFII score and CP diagnosis. CP diagnosis alone was a statistically significant predictor for all of the above four common disorders; however, after adjustment for SFII score was included in health disorder models, only dysphagia showed an independent correlation with a CP diagnosis. CONCLUSION: With the exception of dysphagia, impairment in ADLs and walking capabilities, and not CP diagnosis alone, accounted for disparities in specific diseases. Although the diagnosis of CP may be correlated with functional impairment, it alone may play a minor role in determining health trajectories in older persons with conjoint ID and CP.

The health of women with cerebral palsy.

Over the past 10 years, there has been interest in describing the health and functional status of persons with CP. More recently, information specific to women with CP has become available. From these studies a better appreciation of the affect a lifelong primary disability such as CP has on an individual's life has developed. An understanding of aging with a disability, secondary conditions, associated conditions, comorbidities, and health have promoted a health and wellness agenda for women and men with CP. Women with CP are generally healthy. There appears to be no significant risk for any specific comorbiditv from CP. Women may note a modest change in function over time that may represent issues of aging, but there should be no dramatic loss of function. Any loss of function must be evaluated further for cause, and cannot be attributed to aging alone. Common secondary conditions and health issues include pain and musculoskeletal issues, bladder and bowel problems, poor dental hygiene, and possibly gastroesophageal reflux. Osteoporosis at all ages from limited mobility (secondary osteoporosis) likely is common, but only recently has bone densitometry been used for more routine evaluations; consequently, no data are available in women with CP regarding this. All health issues should be evaluated and appropriate intervention prescribed. There appear to be no significant reproductive health issues for women with CF. Women should engage in all decisions regarding diagnosis and treatment. Women with CP likely participate in some healthy behaviors. However implementing health promotion programs requires some understanding of health behavior theories and models. It may not be enough to modify existing programs for accessibility (e.g., transportation, cost. environment). Engagement in health promotion programs by women with CP must take into account individual perceptions and values, social networks, a sense of personal control, and a readiness to change attitude toward changing lifestyles.

The health status of women with cerebral palsy.

OBJECTIVE: To determine preliminary associations between collected health status variables of women with cerebral palsy (CP) residing in the community. DESIGN: Cross-sectional study using survey research. PARTICIPANTS: Sixty-three women residing in the community were administered the Telephone Questionnaire when contacted to arrange their visit to the study site located within the medical clinic of a local developmental services office. During the course of their visit to the study site, all 63 women completed the CP Study Protocol, in addition to the Mail-in Questionnaire upon completion of study participation. The women ranged in age from 20 to 74 years. MAIN OUTCOME MEASURES: Health status, consisting of four elements: (1) self-reported health status; (2) associated conditions; (3) secondary conditions; and (4) health behaviors (alcohol and tobacco use, physical activity, diet, and health care visit). RESULTS: Women with CP residing in community living arrangements perceived themselves as healthy. The majority of women did not smoke (98%), had not consumed alcohol in the previous month (95%), and ate a balanced diet (52%). Participants also reported engaging in common physical activities (83%) and stretching and doing range-of-motion exercises in the previous week (43%), and participation in aerobic exercise in the previous week (43%). Sixty-eight percent (41 of 60) of the women walked, and more than 50% of the women did not require assistance with activities of daily living. The ability to walk and the use of a wheelchair were associated with participation in the common physical activities. The women reported associated conditions of mental retardation (34%), learning disabilities (26%), and a seizure history (40%). Additionally, the women in the sample reported the occurrence of several secondary conditions common among individuals with CP, including pain (84%), hip and back deformities (59%), bowel problems (56%), bladder problems (49%), poor dental health (43%), and gastroesophageal reflux (28%). Poor dental health was associated with a history of seizures, and associations were also found between pain and mental retardation, and between gastroesophageal reflux and mental retardation. CONCLUSIONS: Women with CP residing in the community perceived themselves as healthy, and the observed health status measures (eg, self-reported health, associated conditions, secondary conditions, and selected health behaviors) support this concept. For the most part, independent relationships were found between several of these measures indicating no significant association among the variables. Where associations were found, however, such as between walking and participation in physical activity, further investigation is warranted for a better understanding of their ramifications in the design of health promotion activities for women with CP.

Photophobia in patients with traumatic brain injury: Uses of light-filtering lenses to enhance contrast sensitivity and reading rate.

Patients who have sustained traumatic brain injury (TBI) often experience a new, intense and chronic photophobia. Photophobia, an intolerance to light, is an incompletely understood, subjective symptom, which has been divided into ocular and central types. Various commercial sources of light-filtering lenses have been developed, which have proven to be successful in diminishing visual symptoms expressed by patients who are photophobic. However, despite the many subjective reports of improved visual performance and comfort with use of these filters, there has been little documentation of actual enhanced visual sensitivity/efficiency. Letter contrast sensitivity (CS) and reading rate were measured in patients with TBI, who, despite good ocular health, experienced significant light intolerance. These patients exhibited up to two fold increases (0.3 log units) in binocular letter contrast sensitivity, as measured with the Pelli-Robson Letter CS Chart, in the presence of selected Corning Photochromic Filters (CPF), as compared to performance in the absence of CPF filters, or to that of similarly treated normal observers. These same patients demonstrated reading rates enhanced up to 39% above that measured in the presence of nearpoint optical correction alone. Reading performance of normal observers was unaffected by similar light filtration. These data provide objective evidence for improvement of visual function provided by light-filtering lenses in patients who become photophobic after TBI. Contrast sensitivity testing and assessment of reading rate add objective criteria for the clinical selection of light-filtering lenses in the treatment of TBI-induced photophobia.

Sequential mesenteric arteriography in pony foals during repeated inoculations of Strongylus vulgaris and treatments with ivermectin.

Semiselective mesenteric arteriography was performed at regular intervals (inoculation weeks [IW] 0, 11, 18, and 24) in 9 of 10 pony foals raised to be free of parasites. Fifty infective larvae (L3) of Strongylus vulgaris were administered weekly for 4 weeks, then every 2 weeks through the 20th week. Three ponies were given ivermectin (oral paste, 0.2 mg/kg of body weight) treatment at IW 8, 16 and 24. Four ponies were inoculated, but did not receive ivermectin, and a third group of 2 ponies acted as uninoculated controls. Control ponies did not have gross or arteriographic lesions, whereas the inoculated untreated ponies had gross and progressive arteriographic lesions typical of verminous arteritis. Arteriographic lesions in the ivermectin-treated inoculated ponies were not as severe those in the untreated inoculated group, and there was either a partial resolution or a lack of progression of arteriographic lesions in all treated ponies. One untreated inoculated pony did not have progressive arterial lesions as did the 3 others in the group, and may develop resistance to the parasite.