Health-related quality of life and functional changes in DMD:A 12-month longitudinal cohort study

Family caregivers of people with amyotrophic lateral sclerosis (ALS) live stressful lives in which they spend most of their time caring for their loved ones and managing difficult situations, thereby reducing the time spent in taking care of themselves. This situation may last several years. Previous literature has widely highlighted that this situation reduces caregivers' quality of life and increases their psychological distress and risk of health problems, but there is a lack of studies that focus on psychological interventions for these situations. This qualitative study examined a pilot experience of two mutual support groups for family caregivers of people with ALS. The aim was to identify caregivers' needs, the prominent aspects of their experience, and to understand whether and how this intervention strategy might help them. Six partners (four men and two women) and six adult children (five women and one man) participated in the groups, which were conducted in northern Italy. After the support groups finished, participants underwent semi-structured interviews. The authors conducted a content analysis of the transcripts of the interviews and the 20 group sessions. The thematic areas identified were "caregiving," "being the son/daughter of a person with ALS," "being the partner of a person with ALS," "group experience" and "group evaluation." The caregiving experience was profoundly different depending on whether the caregiver was a son/daughter or a partner of a patient with ALS. Moreover, comparison with peers and mutual support helped participants to better cope with ALS and its consequences, to improve their care for their relatives and to overcome typical caregiver isolation. These results suggest the usefulness of involving communities in caregiver support in order to create new networks and activate personal and social resources for well-being

Economic consequences of investing in anti-HCV antiviral treatment from the Italian NHS perspective : a real-world-based analysis of PITER data

OBJECTIVE: We estimated the cost consequence of Italian National Health System (NHS) investment in direct-acting antiviral (DAA) therapy according to hepatitis C virus (HCV) treatment access policies in Italy. METHODS: A multistate, 20-year time horizon Markov model of HCV liver disease progression was developed. Fibrosis stage, age and genotype distributions were derived from the Italian Platform for the Study of Viral Hepatitis Therapies (PITER) cohort. The treatment efficacy, disease progression probabilities and direct costs in each health state were obtained from the literature. The break-even point in time (BPT) was defined as the period of time required for the cumulative costs saved to recover the Italian NHS investment in DAA treatment. Three different PITER enrolment periods, which covered the full DAA access evolution in Italy, were considered. RESULTS: The disease stages of 2657 patients who consecutively underwent DAA therapy from January 2015 to December 2017 at 30 PITER clinical centres were standardized for 1000 patients. The investment in DAAs was considered to equal €25 million, €15 million, and €9 million in 2015, 2016, and 2017, respectively. For patients treated in 2015, the BPT was not achieved, because of the disease severity of the treated patients and high DAA prices. For 2016 and 2017, the estimated BPTs were 6.6 and 6.2 years, respectively. The total cost savings after 20 years were €50.13 and €55.50 million for 1000 patients treated in 2016 and 2017, respectively. CONCLUSIONS: This study may be a useful tool for public decision makers to understand how HCV clinical and epidemiological profiles influence the economic burden of HCV

Friction and lubrication of pleural tissues

The frictional behaviour of rabbit\u2019s visceral pleura sliding against parietal pleura was assessed in vitro while oscillating at physiological velocities and amplitudes under physiological normal forces. For sliding velocities up to 3 cm s 121 and normal compressive loads up to 12 cm H2O, the average value of the coefficient of kinetic friction (\u3bc) was constant at 0.019\ub10.002 (S.E.) with pleural liquid as lubricant. With Ringer-bicarbonate solution, \u3bc was still constant, but significantly increased (\u394\u3bc=0.008\ub10.001; P < 0.001). Under these conditions, no damage of the sliding pleural surfaces was found on light and electron microscopy. Additional measurements, performed also on peritoneum, showed that changes in nominal contact area or strain of the mesothelia, temperature in the range 19\u201339 \ub0C, and prolonged sliding did not affect \u3bc. Gentle application of filter paper increased \u3bc 10-fold and irreversibly, suggesting alteration of the mesothelia. With packed the red blood cells (RBC) between the sliding mesothelia, \u3bc increased appreciably but reversibly on removal of RBC suspension, whilst no ruptures of RBC occurred. In conclusion, the results indicate a low value of sliding friction in pleural tissues, partly related to the characteristics of the pleural liquid, and show that friction is independent of velocity, normal load, and nominal contact area, consistent with boundary lubrication

A chimeric Potato virus X encoding a heterologous peptide affects Nicotiana benthamiana chloroplast structure

The cytopathology of a Potato virus X (PVX) recombinant variant (encoding as fusion of an epitope of immunological interest with the N-terminus of the coat protein, PVX Sma P18DD) has been compared with that induced by the wild-type virus (PVX wt) in Nicotiana benthamiana plants. Both PVX wt and PVX Sma P18DD caused similar ultrastructural alterations, characterized by the presence of laminated inclusion components and bulk virus accumulations in mesophyll cells. However, some striking differences were observed not only in the morphology of these accumulations (typically ordered in PVX wt infection and disordered in PVX Sma P18DD infection) but also because the chimeric virus caused peculiar alterations in chloroplasts structure

Peritalar release according to Simons for treatment of congenital clubfoot: medium-term clinical and X-ray results

Introduction The objective of the present study is to evaluate medium-term results of the Simons procedure for the treatment of congenital clubfoot. Material and methods Fifteen patients affected by III degree congenital talipes equinovarus (TEV) were treated surgically. TEV was bilateral in seven cases, thus the total number of operated feet were 22. The patients underwent peritalar release according to Simons, and were evaluated postoperatively with antero-posterior and lateral view X-rays. Clinical and radiological follow-up was at mean 6.7 years (range 2\u201313 years). Clinical and morpho-functional evaluations were performed in agreement with Manes and Laaveg/Ponseti. Also, all patients were evaluated at standard X-ray two-projection stress views, photopodogram and baropodometric exam. Results Of the 15 operated patients, two were not available for followup. Therefore, a total of 20 feet were evaluated. Two patients underwent another surgical intervention for deformity recurrence. At follow-up no patient presented with pain at rest. According to Manes 13 cases had good results, five cases had satisfactory results, and two cases had bad results. Results at Laaeveg and Ponseti evaluation were excellent in 16 cases, good in two cases, and unsatisfactory in two cases. Anteroposterior radiographic exam revealed an alteration of the astragalo-calcanear divergence in seven feet and a reduction of Kite angle in three patients. At lateral view, X-rays revealed a reduction of the astragalo-calcanear angle, compared to normal values, in 12 cases. The scaphoid was dorsally subdislocated in eight cases. Photopodogram evaluation showed accentuation of the plantar vault in five cases, Static baropodometric examination showed a backward shift of the body baricenter, which determined an overload at the normal hindfoot. Discussion In the present study, we perform a complete peritalar release as described by Simons, which seems to guarantee better chances of restoring correct astragalo-calcanear anatomy. In terms of deformity correction, the clinical and morphological results were satisfactory in 90% of cases. However, a data analysis of long-term follow-up studies reported in the literature over the last years demonstrates that less invasive treatment is better than the surgical approach. In fact, the latter is more likely to determine development of pain, functional limitation, and beginning and progression of foot osteoarthritis. Despite the good results obtained with the peritalar release technique in short-term and mid-term studies, the therapeutic choice for treating TEV is unanimously shifting from extensive releases to less aggressive treatments

Trasfusioni di emazie concentrate (EC) in terapia intensiva (TI) nell\u2019Azienda Sanitaria Universitaria Integrata di Udine (ASUIUD). Una valutazione di appropriatezza

INTRODUZIONE: La limitata disponibilit\ue0 di sacche di emazie concentrate (EC) e i rischi connessi alla pratica trasfusionale impongono l\u2019adozione di Linee Guida (LG) per il buon uso del sangue, e di procedure aziendali che ne regolino l\u2019utilizzo. Lo studio si \ue8 proposto di verificare l\u2019appropriatezza delle richieste di EC in TI in termini di compliance rispetto alle LG adottate. MATERIALI E METODI: \uc8 stata condotta una revisione delle cartelle cliniche di tutti pazienti che avevano avuto accesso in TI tra 1 ottobre 2016 e 31 marzo 2017. Tutte le trasfusioni di EC sono state tracciate e per ciascuna di esse sono state registrate emoglobina (Hb) ed ematocrito (Ht) pre e post-trasfusionali. Sono state considerate appropriate le trasfusioni fatte sulla scorta di valori pre-trasfusionali di Hb <8 g/dL (<10 g/dL per cardiopatici e pneumopatici) o Ht <24%. Sono state ricercate le trasfusioni che a posteriori non esitavano n\ue9 in un aumento dell\u2019Hb di almeno 0,5 g/dL, n\ue9 dell\u2019Ht di almeno 1,5 punti percentuali, al fine di individuare le situazioni di sanguinamento attivo, per le quali sarebbe stato pi\uf9 indicato l\u2019intervento chirurgico. Per ciascun paziente sono stati rilevati et\ue0 ed eventuale intervento chirurgico prima dell\u2019ingresso in TI. RISULTATI: I pazienti nel periodo considerato erano 436. L\u2019et\ue0 media \ue8 risultata 60,8 anni (DS \ub120,4 anni). La quota sottoposta a intervento chirurgico era 60% (260 pazienti). Il 24% (106) dei pazienti \ue8 stato trasfuso durante la degenza, e il 18% (19) di essi risultava cardiopatico o pneumopatico. Il tasso di trasfusioni (214 totali) su tutta la popolazione considerata era di 7,6/100 giorni-paziente. Per 209 trasfusioni \ue8 stato possibile risalire ai parametri pre e post. Il 41% (86) di queste risultava non rientrare nei criteri pre-trasfusionali per Hb ed Ht, mentre il 12% (26) delle trasfusioni non evidenziava l\u2019aumento atteso per nessuno dei parametri post. Il 24% (21 su 86) delle trasfusioni che non rispettavano i criteri pre risultava anche inefficace in base ai parametri post. CONCLUSIONI: Si segnalano margini per ridurre il consumo inappropriato di EC, anche attraverso lo sviluppo di programmi di formazione specifica per gli operatori. La mancata aderenza alle LG esita in inefficacia in circa un quarto dei casi. Appare importante monitorare il grado della stessa, tenendo in considerazione i parametri tanto pre quanto post-trasfusionali