Corticobasal degeneration and frontotemporal dementia presentations in a kindred with nonspecific histopathology

We report the clinical, neuropsychological, electroencephalographic and radiologic findings in a kindred with varying clinical presentations of a neurodegenerative disorder. Postmortem examination of one member with clinically suspected corticobasal degeneration (CBD) revealed nonspecific histopathology maximally involving the frontoparietal cortex with negligible degenerative changes in the basal ganglia and substantia nigra. The findings in this and other kindreds demonstrate that (1) similar findings on ancillary testing can occur in relatives presumably suffering from the same pathophysiologic process despite dissimilar clinical presentations, (2) the 'CBD syndrome' is not specific for CBD, (3) extrapyramidal dysfunction can exist in the absence of appreciable basal ganglia and nigral degeneration, (4) nonspecific histopathology can underlie familial focal/asymmetric cortical degeneration syndromes and (5) many of the findings in CBD are comparable to those reported in frontotemporal dementia.     

Feeding behavior problems in children with cystic fibrosis in the UK: prevalence and comparison with healthy controls

BACKGROUND: Feeding behavior problems contribute to inadequate dietary intake for many patients with cystic fibrosis (CF). However, to establish effective intervention programs, more needs to be known about the occurrence and distribution of these difficulties. The aims of this study were to establish the prevalence and range of disruptive child behaviors (DCB) in patients with CF and the inappropriate parental responses (IPR) during mealtimes and to compare the results with those of healthy children. METHODS: In study A, parents of 108 patients (aged 1-7 years) completed a Behavioural Paediatric Feeding Assessment Scale comprising two domains: DCBs and IPRs during mealtimes. Parents rated the frequency of the behaviors and responses and identified those they considered problematic. In study B, data from the CF group (n = 69, aged 1-12 years) were compared with 69 age- and sex-matched control subjects. RESULTS: Parents of children with CF aged 5 to 8 years recorded significantly more DCBs than those in all other age ranges. These parents also reported significantly more IPRs than did parents of children aged 9 to 12 years and 13 to 17 years. Parents of children with CF reported significantly more DCBs and IPRs than did those of the control subjects. There were significantly more problematic DCBs and IPRs in the CF group than in the control group for children aged 5 to 8 years and 9 to 12 years but not for those aged 1 to 4 years. CONCLUSIONS: Parents of children with CF report more feeding behavior problems than do those of healthy control subjects. The high prevalence of feeding behavior problems in older children suggests that preventative and reactive interventions must continue throughout childhood and vary according to the child's developmental abilities.     

Tissue microarray analysis of hepatocyte growth factor/Met pathway components reveals a role for Met,matriptase, and hepatocyte growth factor activator inhibitor 1 in the progression of node-negative breast cancer

Numerous studies have demonstrated that overexpression of Met, the hepatocyte growth factor(HGF) receptor, plays an important role in tumorigenesis. Met activation can either occur through ligand-independent or -dependent mechanisms, both of which are mediated by a series of proteases and modulators. We studied the protein expression of several components of the HGF/Met pathway on a cohort of 330 node-negative breast carcinomas using a tissue microarray annotated with 30-year, disease-specific patient follow-up data. We examined HGF, matriptase (an activator of HGF expressed on mammary epithelial cell surfaces), HAI-I (the cognate inhibitor of matriptase), and the Met receptor itself. Our studies demonstrate tight correlation between the expression of HGF, matriptase, and Met in breast carcinoma. High-level expression of Met, matriptase, and HAI-I were associated with poor patient outcome. Met and HAI-I showed independent prognostic value when compared with traditional breast markers in a multivariate analysis. Intriguingly, antibodies against the intracellular but not the extracellular domain of Met were prognostic, suggesting that overexpression of the cytoplasmic-tail of Met, perhaps through cleavage or truncating mutation, may play an important role in breast cancer progression.     

Enhanced mortality from perfluorocarbon administration in a rat model of kerosene aspiration

BACKGROUND: Aspiration of low-viscosity hydrocarbons may lead to fulminant pneumonitis and acute respiratory distress syndrome. Animal and human studies suggest that partial liquid ventilation with perfluorocarbon improves gas exchange and pulmonary function in acute respiratory failure. The objective of this investigation was to determine the effect of intratracheal perfluorocarbon administration and a brief period of partial liquid ventilation on survival in a rat model of severe hydrocarbon aspiration. METHODS: Two randomized, non-blinded, controlled experiments were performed. First, male Wistar rats (n = 12) were given 0.3 mL/kg kerosene via direct intratracheal instillation and after 5 minutes were randomized to partial liquid ventilation or standard gas ventilation (control) groups. Partial liquid ventilation rats (n = 6) received 20 mL/kg of pre-oxygenated FC-77 intratracheally and positive-pressure gas ventilation (FiO2 = 1.0), and control rats (n = 6) received positive-pressure gas ventilation alone. Animals were observed for survival and 7-day mortality was compared using the Fisher Exact test. The study was then repeated in 12 additional animals using a 15-minute interval between kerosene instillation and treatment (PLV vs control). RESULTS: Mortality was significantly greater in the partial liquid ventilation group (5 of 6; 83%) as compared to the control group (1 of 6; 17% [p = 0.039]). Results were identical in the repeat study. All animals that died succumbed from acute respiratory failure within 24 hours of kerosene instillation. CONCLUSION: In this rat model of severe kerosene aspiration, intratracheal perfluorocarbon administration and a brief period of partial liquid ventilation proved detrimental and significantly increased mortality.     

Effect of oral and inhaled salbutamol in infants with bronchiolitis

The response of bronchiolitis to bronchodilator drugs is controversial. The present study was designed to evaluate the efficacy of oral or metered dose inhaler (MDI) salbutamol using a coffee cup as a spacer device in bronchiolitis. In the trial, 31 hospitalized patients between 6 and 24 months of age, who exhibited the first episode of acute bronchiolitis without any other predisposing illness such as cystic fibrosis, congenital heart disease etc., were randomly assigned to receive oral salbutamol (n=11, 0.1 mg/kg per dose, four times a day), or MDI salbutamol (n = 12, 200 μg per dose, every 3 h) or formed the control group without any bronchodilator therapy (n = 8). All of the patients were given supplemental oxygen as needed and adequate hydration was maintained. The patients were evaluated with clinical symptom scores. There were no differences in the beneficial or side effects of salbutamol, or the number of days in hospital between the treatment groups and the control group. It was concluded that there is no beneficial effect in using bronchodilators in infants with bronchiolitis. Supplemental oxygen and maintenance of normal hydration may be adequate.