Apathy as Marker of Frail Status

Frailty is a complex and dynamic condition associated with aging. This condition is characterised by the difficult adaptation of an old subject to new challenges occurring during life. Frailty is supposed to be due to the progressive decrease of physiological reserves and multiorgan and multisystem change. It coincides with a reduced or absent resilience. In general comorbidities like hypertension, heart disease, inflammation and infectious diseases are potential risk factors for and psychophysical decline. The aim of this work is to highlight the importance of impaired cognition as factor predisposing to frailty. The authors are convinced and suggest that the presence of neurobehavioral disturbance like apathy associated to impaired executive function could be the major predisposing factor for frailty and unsuccessful aging. Unfortunately available literature largely underestimates the presence of these factors. Thus to better identify markers of frailty, a good neuropsychological assessment and the evaluation of behavioural disturbances are suggested

Preliminary data revealing efficacy of Streptococcus salivarius K12 (SSK12) in Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome: A multicenter study from the AIDA Network PFAPA syndrome registry

Objective: To evaluate the potential role of Streptococcus salivarius K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction. Patients and methods: The medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00 ± 7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00 ± 28.00 months. Results: The number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p < 0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [p < 0.001]. Similarly, the highest temperature in°C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p < 0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p < 0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis (p < 0.001), oral aphthae (p < 0.001) and cervical lymphadenopathy (p < 0.001) significantly decreased following SSK12. Conclusion: SSK12 prophylaxis given for at least 6.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the duration of the single febrile episode, lowered body temperature by 1°C in the febrile flare, provided a steroid-sparing effect, and significantly reduced the accompanying symptoms related to the syndrome

Apathy as Marker of Frail Status

Frailty is a complex and dynamic condition associated with aging. This condition is characterised by the difficult adaptation of an old subject to new challenges occurring during life. Frailty is supposed to be due to the progressive decrease of physiological reserves and multiorgan and multisystem change. It coincides with a reduced or absent resilience. In general comorbidities like hypertension, heart disease, inflammation and infectious diseases are potential risk factors for and psychophysical decline. The aim of this work is to highlight the importance of impaired cognition as factor predisposing to frailty. The authors are convinced and suggest that the presence of neurobehavioral disturbance like apathy associated to impaired executive function could be the major predisposing factor for frailty and unsuccessful aging. Unfortunately available literature largely underestimates the presence of these factors. Thus to better identify markers of frailty, a good neuropsychological assessment and the evaluation of behavioural disturbances are suggested

Emergency management in patients with haemophilia A and inhibitors on prophylaxis with emicizumab: AICE practical guidance in collaboration with SIBioC, SIMEU, SIMEUP, SIPMeL and SISET

Emicizumab has been approved in several countries for regular prophylaxis in patients with congenital haemophilia A and FVIII inhibitors because it substantially reduces their bleeding risk and improves quality of life. However, although significantly less frequent, some breakthrough bleeds may still occur while on emicizumab, requiring treatment with bypassing or other haemostatic agents. Thrombotic complications have been reported with the associated use of activated prothrombin complex concentrates. In addition, when surgery/invasive procedures are needed while on emicizumab, their management requires multidisciplinary competences and direct supervision by experts in the use of this agent. Given this, and in order to expand the current knowledge on the use of emicizumab and concomitant haemostatic agents, and reduce the risk of complications in this setting, the Italian Association of Haemophilia Centres (AICE) here provides guidance on the management of breakthrough bleeds and surgery in emergency situations in patients with haemophilia A and inhibitors on emicizumab prophylaxis. This paper has been shared with other National Scientific Societies involved in the field