Μεταβολές στη δομή και τη λειτουργία του εγκεφάλου εφήβων και νεαρών ενηλίκων με ψυχογενή ανορεξία, όπως αυτές αναδεικνύονται απεικονιστικά: συστηματική ανασκόπηση Η αναστρεψιμότητα των μεταβολών μετά τη θεραπεία: συστηματική ανασκόπηση

Η νευρική ανορεξία αναγνωρίζεται ως ψυχιατρική νόσος με σημαντική νοσηρότητα που επιφέρει μεταβολές σε σώμα και εγκέφαλο νεαρών ασθενών. Ωστόσο, οι μεταβολές στον εγκέφαλο των ασθενών παραμένουν αβέβαιες. Αυτή η συστηματική ανασκόπηση αποσκοπεί στο να διαπιστώσει πώς η θεραπεία και η ανάκτηση βάρους επιδρά στις εγκεφαλικές δομές ατόμων με νευρική ανορεξία. Διεξήχθη στις βάσεις δεδομένων PubMed, ScienceDirect και Cochrane από την έναρξη τους έως τον Ιανουάριο του 2020. Συμπεριελήφθησαν προοπτικές μελέτες που εξετάζουν νευροαπεικονιστικες μεταβολές σε εφήβους και νεαρούς ενήλικες έως 24 ετών με νευρική ανορεξία πριν και μετά την αύξηση ή την αποκατάσταση του σωματικού βάρους. Ο έλεγχος, η εξαγωγή δεδομένων και η μεθοδολογική αξιολόγηση της ποιότητας πραγματοποιήθηκαν από τουλάχιστον 2 ερευνητές ανεξάρτητα, ακολουθώντας τις οδηγίες PRISMA. Συμπεριλήφθηκαν δώδεκα μελέτες σε δεκατρία άρθρα (238 ασθενείς). Από τις μελέτες με MRI αναδεικνύεται η αναστρεψιμότητα των μετρήσεων επιφανειών και όγκων δομών του εγκεφάλου σύντομα μετά τη θεραπεία, αν και ελλείμματα όγκου της φαιάς ουσίας εξακολουθούν να υφίστανται και μετά την ανάκτηση βάρους, γεγονός που υποδηλώνει την ύπαρξη ενός μη αναστρέψιμου στοιχείου. Οι μελέτες που χρησιμοποίησαν DTI δείχνουν ότι η αναδόμηση συμβαίνει και σε επίπεδο οργάνωσης νευρικών ινών. Το κύριο εύρημα των ερευνών SPECT είναι η αύξηση στην περιφερειακή ροή του εγκεφαλικού αίματος (rCBF) σε ορισμένες περιοχές του εγκεφάλου μετά τη θεραπεία. Συμπερασματικά η θεραπεία της νευρικής ανορεξίας, αποκαθιστά τις περισσότερες εγκεφαλικές αλλοιώσεις, αν και δεν μπορούν να εξαχθούν οριστικά συμπεράσματα λόγω λίγων δεδομένων. Η κατανόηση των μηχανισμών στις οποίες βασίζονται αυτά τα ευρήματα μπορεί να παρέχει σημαντικές προληπτικές δυνατότητες.Importance Anorexia nervosa is recognized as an important cause of morbidity a psychiatric disease with a severe impact on the body and brain of young patients. However, changes in patients' brains remain uncertain.  Objective To evaluate how treatment and weight recovery affect the brain structures of young patients with anorexia nervosa. Data Sources PubMed, ScienceDirect, and Cochrane from database inception to January 2020. Study Selection Prospective studies examining the neuroimaging changes in adolescents and young adults up to 24 years of age with anorexia nervosa before and after weight gain or weight normalisation.  Data Extraction and Synthesis Screening, data extraction, and methodological quality assessment were performed by at least 2 researchers independently, following the PRISMA guidelines. Main Outcomes  Most findings appear to quickly reverse after weight gain.  Results Twelve studies published in thirteen articles (238 participants with anorexia nervosa) were included. MRI studies indicate reversibility of volumetric lesions soon after treatment, although gray matter volume deficits persist after weight gain, suggesting the existence of an irreversible element. Studies using DTI show that remodeling also occurs at the level of nerve fiber organization. The main finding of SPECT studies is the increase in regional cerebral blood flow (rCBF) in certain areas of the brain after treatment.   Conclusions and Relevance Treatment of anorexia nervosa, restores most of the patient's brain lesions, although definite conclusions could not be drawn due to scarce data from longitudinal studies. Understanding the mechanisms underlying these findings could have important preventive potential.

Clinical and laboratory features associated with macrophage activation syndrome in Still's disease: data from the international AIDA Network Still's Disease Registry

: To characterize clinical and laboratory signs of patients with still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. patients with still's disease classified according to internationally accepted criteria were enrolled in the autoInflammatory disease alliance (AIDA) still's disease registry. clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). at multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data

Still's disease continuum from childhood to elderly: data from the international AIDA Network Still's disease registry

Objective: Still's disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still's disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still's disease. Methods: Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still's disease. Results: A total of 411 patients suffering from Still's disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still's disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p<0.0001) were significantly more frequent among elderly-onset patients compared with paediatric-onset subjects. Regarding laboratory data, thrombocytosis was significantly more frequent among paediatric patients onset compared with adult-onset subjects (p<0.0001), while thrombocytopenia was more frequent among elderly-onset patients although statistical significance was only bordered. No substantial differences were observed in the response to treatments. Conclusions: Despite some minor difference between groups, overall, demographic, clinical, laboratory and treatments aspects of Still's disease were similarly observed in patients at all ages. This supports that pediatric-onset, adult-onset and elderly-onset Still's disease is the same clinical condition arising in different ages

Still's disease continuum from childhood to elderly : data from the international AIDA Network Still's disease registry

Abstract: Objective Still's disease is more frequently observed in the paediatric context, but a delayed onset is not exceptional both in the adulthood and in the elderly. However, whether paediatric-onset, adult-onset and elderly-onset Still's disease represent expressions of the same disease continuum or different clinical entities is still a matter of controversy. The aim of this study is to search for any differences in demographic, clinical features and response to treatment between pediatric-onset, adult-onset and elderly-onset Still's disease. Methods Subjects included in this study were drawn from the International AutoInflammatory Disease Alliance Network registry for patients with Still's disease. Results A total of 411 patients suffering from Still's disease were enrolled; the disease occurred in the childhood in 65 (15.8%) patients, in the adult 314 (76.4%) patients and in the elderly in 32 (7.8%) patients. No statistically significant differences at post-hoc analysis were observed in demographic features of the disease between pediatric-onset, adult-onset and elderly-onset Still's disease. The salmon-coloured skin rash (p=0.004), arthritis (p=0.009) and abdominal pain (p=0.007) resulted significantly more frequent among paediatric patients than in adult cases, while pleuritis (p=0.015) and arthralgia (p<0.0001) were significantly more frequent among elderly-onset patients compared with paediatric-onset subjects. Regarding laboratory data, thrombocytosis was significantly more frequent among paediatric patients onset compared with adult-onset subjects (p<0.0001), while thrombocytopenia was more frequent among elderly-onset patients although statistical significance was only bordered. No substantial differences were observed in the response to treatments. Conclusions Despite some minor difference between groups, overall, demographic, clinical, laboratory and treatments aspects of Still's disease were similarly observed in patients at all ages. This supports that pediatric-onset, adult-onset and elderly-onset Still's disease is the same clinical condition arising in different ages

Clinical and laboratory features associated with macrophage activation syndrome in Still's disease : data from the international AIDA Network Still's Disease Registry

Abstract: To characterize clinical and laboratory signs of patients with Still's disease experiencing macrophage activation syndrome (MAS) and identify factors associated with MAS development. Patients with Still's disease classified according to internationally accepted criteria were enrolled in the AutoInflammatory Disease Alliance (AIDA) Still's Disease Registry. Clinical and laboratory features observed during the inflammatory attack complicated by MAS were included in univariate and multivariate logistic regression analysis to identify factors associated to MAS development. A total of 414 patients with Still's disease were included; 39 (9.4%) of them developed MAS during clinical history. At univariate analyses, the following variables were significantly associated with MAS: classification of arthritis based on the number of joints involved (p = 0.003), liver involvement (p = 0.04), hepatomegaly (p = 0.02), hepatic failure (p = 0.01), axillary lymphadenopathy (p = 0.04), pneumonia (p = 0.03), acute respiratory distress syndrome (p < 0.001), platelet abnormalities (p < 0.001), high serum ferritin levels (p = 0.009), abnormal liver function tests (p = 0.009), hypoalbuminemia (p = 0.002), increased LDH (p = 0.001), and LDH serum levels (p < 0.001). At multivariate analysis, hepatomegaly (OR 8.7, 95% CI 1.9-52.6, p = 0.007) and monoarthritis (OR 15.8, 95% CI 2.9-97.1, p = 0.001), were directly associated with MAS, while the decade of life at Still's disease onset (OR 0.6, 95% CI 0.4-0.9, p = 0.045), a normal platelet count (OR 0.1, 95% CI 0.01-0.8, p = 0.034) or thrombocytosis (OR 0.01, 95% CI 0.0-0.2, p = 0.008) resulted to be protective. Clinical and laboratory factors associated with MAS development have been identified in a large cohort of patients based on real-life data