Understanding stakeholder important outcomes and perceptions of equity, acceptability and feasibility of a care model for haemophilia management in the US: a qualitative study.

INTRODUCTION: Care for persons with haemophilia (PWH) is most commonly delivered through the integrated care model used by Hemophilia Treatment Centers (HTCs). Although this model is widely accepted as the gold standard for the management of haemophilia; there is little evidence comparing different care models. AIM: We performed a qualitative study to gain insight into issues related to outcomes, acceptability, equity and feasibility of different care models operating in the US. METHODS: We used a qualitative descriptive approach with semi-structured interviews. Purposive sampling was used to recruit individuals with experience providing or receiving care for haemophilia in the US through either an integrated care centre, a specialty pharmacy or homecare company, or by a specialist in a non-specialized centre. Persons with haemophilia, parents of PWH aged ≤18, healthcare providers, insurance company representatives and policy developers were invited to participate. RESULTS AND CONCLUSIONS: Twenty-nine interviews were conducted with participants representing 18 US states. Participants in the study sample had experience receiving or providing care predominantly within an HTC setting. Integrated care at HTCs was highly acceptable to participants, who appreciated the value of specialized, expert care in a multidisciplinary team setting. Equity and feasibility issues were primarily related to health insurance and funding limitations. Additional research is required to document the impact of care on health and psychosocial outcomes and identify effective ways to facilitate equitable access to haemophilia treatment and care.

Safety of Grass Pollen Sublingual Immunotherapy for Allergic Rhinitis in Concomitant Asthma.

Seasonal allergic rhinitis (AR) occurs predominantly as a result of grass pollen allergy. Grass pollen sublingual immunotherapy (SLIT) has been proven effective in treating AR1. SLIT is currently licensed for use in AR with concomitant stable mild asthma. There is evidence that SLIT improves asthma control when primarily used to treat AR2. The aim was to assess the safety of SLIT in patients with severe seasonal allergic rhinitis who have co-existing stable mild asthma. The secondary aim was to determine whether asthma control improved post SLIT. There was no deterioration in asthma control after 6-36 months of SLIT. 27/30 (90%) patients' asthma control remained stable or indeed improved (p < 0.021). Of this 15 (50%) patients' asthma improved. There was no statistically significant change in their asthma pharmacotherapy after SLIT (p = 0.059). In conclusion, grass pollen SLIT is safe and can potentially treat dual allergic rhinitis- mild asthmatic patients.

Isolation of translactone-containing triterpenes with thrombin inhibitory activities from the leaves of Lantana camara.

Methanolic extracts prepared from the leaves of Lantana camara have been found to inhibit human thrombin. An assay, in which thrombin activity is measured as a function of clot formation from fibrinogen, was used to guide the fractionation and purification of five principal active constituents (1-5), which were all characterized as 5,5-trans-fused cyclic lactone-containing euphane triterpenes.

Lesions associated with mineral deposition in the lymph node and lung of the dog.

This report includes details of the clinical and pathologic features of 31 dogs with a range of systemic illness and granulomatous lymphadenopathy associated with the presence of birefringent crystalline material within lymph nodes. Similar crystalline material was found in the lymph nodes of dogs with lymphoma (n = 9) and as an incidental finding within the canine lung (n = 9). The mineral content of these crystals was determined by electron microprobe analysis and interpreted in light of the composition of known geological or human-made compounds. A wide range of elements was identified including silicon, sulfur, copper, calcium, and aluminium, with lesser proportions of phosphorus, sodium, potassium, iron, magnesium, titanium, nickel, and chromium. Many of these compounds may have originated from exogenous natural and human-made sources, but some compounds (notably phosphates and sulfates) are uncommon or not found in nature and may have been formed within the tissues of the body (biomineralization). The inflammatory response induced by the presence of these minerals within lymphoid tissue may trigger altered immunoregulation, accounting for the spectrum of disease observed.

Startle modulation studies in autism.

We studied acoustic startle response and its modulation by prestimulation and by short-term and long-term habituation in 54 autistic patients and 72 normal age-matched controls. The startle response was measured as the amplitude and onset latency of the integrated orbicularis oculi EMG. There were no consistent significant differences between the autistic and control subjects in startle modulation by inhibitory or facilitatory prestimulation, short-term habituation of startle amplitude, long-term habituation of either startle amplitude or latency, or unmodulated startle amplitude. Differences between autistic and control subjects were limited to prolongation of unmodulated startle onset latencies in the autistics in all of the experimental paradigms (significant p = .005 only in the context of short-term habituation) and a statistically significant (p < .05) slower rate of short-term habituation of startle onset latency in the autistic patients, relative to the controls. Results provide only limited support for hypotheses of brainstem pathophysiology and no support for hypotheses of cerebellar pathophysiology in autism.

Modulatory influence of continuous tone, tone offset, and tone onset on the human acoustic startle response.

Startle modulation in young adult men, by continuous background tone and its offset, a 2-s sustained tone and its offset, and the onset of a 25-ms tone pip were compared. Tone (75dB 1000 Hz) offset and onset occurred either 2000 ms or 100-120 ms before the startle stimuli (104dB (SPL), 50-ms white noise bursts). Blink amplitude and latency were unaffected by continuous background tone. Blink amplitude was reliably inhibited by 100-ms offset of both the continuous background tone and the 2-s sustained tone or 120-ms onset of the tone pip, whereas effects on latency were more variable. Facilitation of blink amplitude and latency was significant but weak and only following the 2-s sustained tone, and only with respect to one of two experimental contexts. These findings support those of others and suggest that startle inhibition results from activation of neurons responding to transient environmental changes. The degree of inhibition appears to be related to stimulus value. Startle amplitude facilitation following long sustained prestimulation intervals is dependent on experimental context. Overall latency and amplitude modulation tend to be concordant, leading to the conclusion that the mechanism(s) underlying both are context dependent and linked in the adult human.

Maturation of startle facilitation by sustained prestimulation.

The startle response to a 104dB (SPL) 50-ms burst of white noise was facilitated by prestimulation with a nonstartling tone sustained for 2 s prior to the startling stimulus in 3, 4, 5, and 8 year old children and young adults. Both startle amplitude and onset latency showed significantly greater facilitation in the preschool children than in the 8-year-olds and adults. The results of this experiment, which used a fixed prestimulation interval, were compared to those of an earlier study in which the prestimulation interval was varied. The maturational changes in startle facilitation in response to the 2-s prestimulation interval were similar in the two experiments. Hence the maturational effect on startle facilitation was independent of the uncertainty (as to when the startling stimulus would be given), which might be associated with variable prestimulation intervals. These findings suggest that the neuronal mechanisms that mediate startle facilitation undergo development during early childhood and mature about 8 years of age, and that this maturational sequence is relatively independent of attentional effects.