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5. The clinical course of Duchenne muscular dystrophy in the corticosteroid treatment era: a systematic literature review

Author

Shelagh M. Szabo, Renna M. Salhany, Alison Deighton, Meagan Harwood, Jean Mah, and Katherine L. Gooch

Subjects
Duchenne muscular dystrophy, DMD, Clinical course, Loss of ambulation, Systematic review, Medicine
Abstract

Abstract Background Duchenne muscular dystrophy (DMD) is a severe rare progressive inherited neuromuscular disorder, leading to loss of ambulation (LOA) and premature mortality. The standard of care for patients with DMD has been treatment with corticosteroids for the past decade; however a synthesis of contemporary data describing the clinical course of DMD is lacking. The objective was to summarize age at key clinical milestones (loss of ambulation, scoliosis, ventilation, cardiomyopathy, and mortality) in the corticosteroid-treatment-era. Methods A systematic review was conducted using MEDLINE and EMBASE. The percentage experiencing key clinical milestones, and the mean or median age at those milestones, was synthesized from studies from North American populations, published between 2007 and 2018. Results From 5637 abstracts, 29 studies were included. Estimates of the percentage experiencing key clinical milestones, and age at those milestones, showed heterogeneity. Up to 30% of patients lost ambulation by age 10 years, and up to 90% by 15 years of age. The mean age at scoliosis onset was approximately 14 years. Ventilatory support began from 15 to 18 years, and up to half of patients required ventilation by 20 years of age. Registry-based estimates suggest that 70% had evidence of cardiomyopathy by 15 years and almost all by 20 years of age. Finally, mortality rates up to 16% by age 20 years were reported; among those surviving to adulthood mortality was up to 60% by age 30 years. Conclusions Contemporary natural history studies from North America report that LOA on average occurs in the early teens, need for ventilation and cardiomyopathy in the late teens, and death in the third or fourth decade of life. Variability in rates may be due to differences in study design, treatment with corticosteroids or other disease-modifying agents, variations in clinical practices, and dystrophin mutations. Despite challenges in synthesizing estimates, these findings help characterize disease progression among contemporary North American DMD patients.

Published
2021
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6. L’onere della prova: profili di diritto internazionale privato

Author

Amatucci F., Anelli F., Ansanelli V., Avanzini P.P., Azara A., Bagarotto E.-M., Bizioli G., Bonfanti B., Bresciani I., Buccico C., Buta L., Campione F. Candeloro C., Carinci A., Carpentieri L., Cattaneo C.M., Cecchinato E., Cipolla G.M., Colli Vignarelli A., Coppola P., Corasaniti G., Cordeiro Guerra R., Cordopatri S., Corso S.M., D’Onofrio M., De Michiel F., De Poli M., del Federico L., Della Valle E., Di Noia F., Di Stasi A., Duprè G., Falsone M., Ferluga L., Ferrante V., Ficari V., Filice G., Fransoni G., Franza G., Franzina P., Galluzzo F., Garavaglia M., Giovanardi A., Giovannini A., Gragnoli E., Greco M.G., Greggi M., Greggio M., Ingrao G., Logozzo M., Marinelli M., Marini G., Marino G., Martinoli R., Melis G., Messina S.M., Montanari M., Muleo S., Nocera I.L., Nussi M., Olivelli F., Orlandi M., Ortoleva M.G., Palladini S., Paparella F., Parente S.A., Passanante L., Peruzzi M., Petrocelli F., Pozzaglia P., Prussiani C., Ragucci G., Randazzo F., Renna M., Romano A., Salvini L., Schiavone G., Serra C., Simionato A., Stevanato D., Tassi M., Tommasini D., Tosi L., Travi A., Trivellin M., Tundo F., Ubertis G., Uricchio A.F., Vanzetti M., Vianello R., Viotto A., Widmann P., Zaccarelli L., Zaccarelli V., Zizzo G., Briguglio A., Chizzini A., Franzina, Pietro, Franzina, P. (ORCID:0000-0001-9601-2281), Amatucci F., Anelli F., Ansanelli V., Avanzini P.P., Azara A., Bagarotto E.-M., Bizioli G., Bonfanti B., Bresciani I., Buccico C., Buta L., Campione F. Candeloro C., Carinci A., Carpentieri L., Cattaneo C.M., Cecchinato E., Cipolla G.M., Colli Vignarelli A., Coppola P., Corasaniti G., Cordeiro Guerra R., Cordopatri S., Corso S.M., D’Onofrio M., De Michiel F., De Poli M., del Federico L., Della Valle E., Di Noia F., Di Stasi A., Duprè G., Falsone M., Ferluga L., Ferrante V., Ficari V., Filice G., Fransoni G., Franza G., Franzina P., Galluzzo F., Garavaglia M., Giovanardi A., Giovannini A., Gragnoli E., Greco M.G., Greggi M., Greggio M., Ingrao G., Logozzo M., Marinelli M., Marini G., Marino G., Martinoli R., Melis G., Messina S.M., Montanari M., Muleo S., Nocera I.L., Nussi M., Olivelli F., Orlandi M., Ortoleva M.G., Palladini S., Paparella F., Parente S.A., Passanante L., Peruzzi M., Petrocelli F., Pozzaglia P., Prussiani C., Ragucci G., Randazzo F., Renna M., Romano A., Salvini L., Schiavone G., Serra C., Simionato A., Stevanato D., Tassi M., Tommasini D., Tosi L., Travi A., Trivellin M., Tundo F., Ubertis G., Uricchio A.F., Vanzetti M., Vianello R., Viotto A., Widmann P., Zaccarelli L., Zaccarelli V., Zizzo G., Briguglio A., Chizzini A., Franzina, Pietro, and Franzina, P. (ORCID:0000-0001-9601-2281)

Abstract

1. La diversità delle regole statali in tema di onere probatorio e il problema del conflitto di leggi. - 2. Il quadro delle fonti. - 3. Il diritto internazionale privato delle prove, fra lex processus e lex causae. - 4. La legge regolatrice dell’onere della prova: la tesi processualistica e quella sostanzialistica. - 5. La soluzione accolta nella convenzione di Roma del 1980 e nei regolamenti Roma I e Roma II. - 6. Il generale favore tributato alla tesi sostanzialistica. - 7. Rilievi conclusivi: lo stato dell’arte in Italia.

Published
2024

7. Art. 59 Accordi quadro

Author

Villata, R, Ramajol, M, Benedetti, A., Marra, A., Bruti Liberati, E., Allena, A, Renna, M, Del Signore, M, Avanzini, G, Cafagno, M, Bertonazzi, L, Berti, N., Cassatella, A, Chirulli, P, Cerbo, P, D'Angelo, G, Fracchia, F, Giannelli, A, Gaffuri, F, Goisis, F, Marchetti, B, Micciché, C, Pantalone, P, Police, A, Romeo, A, Saitta, F, Sessa, V.M, Sica, M, Silvestri, M, Spuntarelli, S, Squazzoni, A, Tonoletti, B, Trimarchi, M, Troise Mengoni, W, Tropea, G, Vaccari, S, Valaguzza, S, Vernile, S, Villamena, S., Ramajoli M, Benedetti, A, Villata, R, Ramajol, M, Benedetti, A., Marra, A., Bruti Liberati, E., Allena, A, Renna, M, Del Signore, M, Avanzini, G, Cafagno, M, Bertonazzi, L, Berti, N., Cassatella, A, Chirulli, P, Cerbo, P, D'Angelo, G, Fracchia, F, Giannelli, A, Gaffuri, F, Goisis, F, Marchetti, B, Micciché, C, Pantalone, P, Police, A, Romeo, A, Saitta, F, Sessa, V.M, Sica, M, Silvestri, M, Spuntarelli, S, Squazzoni, A, Tonoletti, B, Trimarchi, M, Troise Mengoni, W, Tropea, G, Vaccari, S, Valaguzza, S, Vernile, S, Villamena, S., Ramajoli M, and Benedetti, A

Published
2024

11. Crisi d’impresa e contratti pubblici. Riflessioni critiche sull’istituto dell’avvalimento obbligatorio e ‘rinforzato’

Author

Renna, M, Vaccari, S, Renna, M, and Vaccari, S

Abstract

The paper deals with the study of the relationship between bankruptcy law and public procurement. Given the complexity of the subject, the analysis proceeds through a brief diachronic analyis of the stratified regulatory framework, with the aim of highlighting the reasons underlying the evolution of the relationship between these two branches of the legal system. A specific attention is devoted to the so-called ‘mandatory pooling’, which in the current Public Procurement Code (Legislative Decree No. 50/2016) was supposed to represent the balancing point with respect to the plurality of public and private interests involved in the cases of participation of economic operators in a state of insolvency or bankruptcy in public tender procedures. As the analysis shows, several critical issues characterise the aforementioned legal institute suggesting, therefore, its elimination in light of the ongoing reform process of Italian public procurement law.

Published
2023

12. Crisi d’impresa e contratti pubblici. Riflessioni critiche sull’istituto dell’avvalimento obbligatorio e ‘rinforzato’

Author

Capo, G, Esposito, GM, Vaccari, S, Renna, M, Capo, G, Esposito, GM, Vaccari, S, and Renna, M

Abstract

Il presente contributo è dedicato allo studio dei rapporti tra il diritto della crisi d’impresa e la contrattualistica pubblica. In ragione della complessità del tema, l’analisi procede attraverso un breve esame diacronico della stratificata disciplina normativa con l’obiettivo di evidenziare le ragioni sottese all’evoluzione delle relazioni tra i due settori dell’ordinamento giuridico. Un approfondimento specifico è dedicato all’istituto dell’avvalimento c.d. obbligatorio e ‘rinforzato’, che nel vigente Codice dei contratti pubblici (d.lgs. n. 50/2016) avrebbe dovuto rappresentare il punto di equilibrio rispetto alla pluralità degli interessi pubblici e privati coinvolti nelle fattispecie di partecipazione di operatori economici in stato di crisi alle procedure a evidenza pubblica. Si evidenziano, tuttavia, le plurime criticità che connotano il suddetto istituto e che, pertanto, ne suggeriscono l’eliminazione in occasione dell’attuale processo di riforma del diritto dei contratti pubblici.

Published
2023

13. Insect lipids as feed: Potential to modulate the fatty acid profile of animal products

Author

Consejo Superior de Investigaciones Científicas (España), Toral, Pablo G. [0000-0002-1913-7707], Hervás, Gonzalo [0000-0002-0013-7459], Frutos, Pilar [0000-0002-4919-5094], Toral, Pablo G., Renna, M., Hervás, Gonzalo, Gasco, L., Frutos, Pilar, Consejo Superior de Investigaciones Científicas (España), Toral, Pablo G. [0000-0002-1913-7707], Hervás, Gonzalo [0000-0002-0013-7459], Frutos, Pilar [0000-0002-4919-5094], Toral, Pablo G., Renna, M., Hervás, Gonzalo, Gasco, L., and Frutos, Pilar

Published
2023

14. The HSPB1-p62/SQSTM1 functional complex regulates the unconventional secretion and transcellular spreading of the HD-associated mutant huntingtin protein

Author

Bonavita, R, primary, Scerra, G, additional, Di Martino, R, additional, Nuzzo, S, additional, Polishchuk, E, additional, Di Gennaro, M, additional, Williams, S V, additional, Caporaso, M G, additional, Caiazza, C, additional, Polishchuk, R, additional, D’Agostino, M, additional, Fleming, A, additional, and Renna, M, additional

Published
2023
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22. The clinical course of Duchenne muscular dystrophy in the corticosteroid treatment era: a systematic literature review

Author

Alison Deighton, Meagan Harwood, Katherine Gooch, Shelagh M. Szabo, Renna M. Salhany, and Jean Mah

Subjects
0301 basic medicine, Adult, Duchenne muscular dystrophy, Pediatrics, medicine.medical_specialty, Adolescent, MEDLINE, Cardiomyopathy, Scoliosis, Review, 030105 genetics & heredity, Dystrophin, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Clinical course, Adrenal Cortex Hormones, DMD, medicine, Humans, Pharmacology (medical), Child, Genetics (clinical), business.industry, Mortality rate, General Medicine, medicine.disease, Natural history, Muscular Dystrophy, Duchenne, Systematic review, Loss of ambulation, Breathing, Disease Progression, Medicine, business, Cardiomyopathies, 030217 neurology & neurosurgery
Abstract

BackgroundDuchenne muscular dystrophy (DMD) is a severe rare progressive inherited neuromuscular disorder, leading to loss of ambulation (LOA) and premature mortality. The standard of care for patients with DMD has been treatment with corticosteroids for the past decade; however a synthesis of contemporary data describing the clinical course of DMD is lacking. The objective was to summarize age at key clinical milestones (loss of ambulation, scoliosis, ventilation, cardiomyopathy, and mortality) in the corticosteroid-treatment-era.MethodsA systematic review was conducted using MEDLINE and EMBASE. The percentage experiencing key clinical milestones, and the mean or median age at those milestones, was synthesized from studies from North American populations, published between 2007 and 2018.ResultsFrom 5637 abstracts, 29 studies were included. Estimates of the percentage experiencing key clinical milestones, and age at those milestones, showed heterogeneity. Up to 30% of patients lost ambulation by age 10 years, and up to 90% by 15 years of age. The mean age at scoliosis onset was approximately 14 years. Ventilatory support began from 15 to 18 years, and up to half of patients required ventilation by 20 years of age. Registry-based estimates suggest that 70% had evidence of cardiomyopathy by 15 years and almost all by 20 years of age. Finally, mortality rates up to 16% by age 20 years were reported; among those surviving to adulthood mortality was up to 60% by age 30 years.ConclusionsContemporary natural history studies from North America report that LOA on average occurs in the early teens, need for ventilation and cardiomyopathy in the late teens, and death in the third or fourth decade of life. Variability in rates may be due to differences in study design, treatment with corticosteroids or other disease-modifying agents, variations in clinical practices, and dystrophin mutations. Despite challenges in synthesizing estimates, these findings help characterize disease progression among contemporary North American DMD patients.

Published
2021

23. Impacts of Great Cormorant and Cattle Egret Nesting on Other Waterbirds in a Shared Breeding Site in Piedmont (NW Italy)

Author

Giammarino, M, Quatto, P, Renna, M, Giammarino M., Quatto P., Renna M., Giammarino, M, Quatto, P, Renna, M, Giammarino M., Quatto P., and Renna M.

Abstract

Some waterbird species are believed to have an impact on other waterbird species as they are able to usurp a limited habitat and destroy vegetation. In this study, we have analysed the nesting dynamics of five waterbird species: Grey Heron Ardea cinerea, Great Cormorant Phalacrocorax carbo, Little Egret Egretta garzetta, Black-crowned Night Heron Nycticorax nycticorax and Cattle Egret Bubulcus ibis, that had settled in a colony in the Royal Castle Park of Racconigi (Cuneo, NW Italy) over a 17-year period (2000 to 2017). The Great Cormorant and the Cattle Egret settled in the colony in 2008 and 2012, respectively. We analysed the relationships between the number of breeding pairs of the five species. In addition, we used the time series forecasting method to study the nesting dynamics and the impact of the new settlements on the pre-existing colony. The number of nests of the Great Cormorant and the Cattle Egret increased annually, and these species significantly affected the nesting trends of the other pre-existing species: Grey Heron, Black-crowned Night Heron and Little Egret. The Grey Heron mainly suffered from the occupation of its old nests by the Great Cormorant from 2008 to 2013. However, since 2014, the Grey Heron has been able to react, by displacing its breeding site to the tops of trees adjacent to the old heronry, and this has resulted in an even greater number of nests than before 2008. The Black-crowned Night Heron and the Little Egret suffered more from the settlement of the new species than the Grey Heron did, and started to suddenly or gradually abandon the breeding site. The colony has been modified profoundly by the settlement of the Great Cormorant and the Cattle Egret, and it is still subject to dynamics that could transform it in the coming years.

Published
2021

24. Digestibility of defatted insect meals for rainbow trout aquafeeds

Author

Gasco, L., primary, Caimi, C., additional, Trocino, A., additional, Lussiana, C., additional, Oddon, S. Bellezza, additional, Malfatto, V., additional, Anedda, R., additional, Serra, G., additional, Biasato, I., additional, Schiavone, A., additional, Gai, F., additional, and Renna, M., additional

Published
2022
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27. Lagrange-Kirchhoff Model for the Deformation of a Coated Taiko Silicon Plate.

Author

Landi, A. and Renna, M.

Subjects
STATIC equilibrium (Physics), SILICON, SILICON carbide
Abstract

Internal stress in a film deposited on a plate-like substrate causes the film and substrate pair to warp until the mechanical equilibrium is reached. This condition is reached when both the net force and the resulting bending momenta reach a null value. In this paper, the analytical Lagrange-Kirchhoff model for the deformation of a coated Taiko plate silicon is proposed. Under the conditions of validity of the linear theory of thin plates, the model is in agreement with the experimental data. These results can be extended to other substrates, such as silicon carbide (SiC). [ABSTRACT FROM AUTHOR]

Published
2023

28. Raggruppamenti temporanei di imprese e avvalimento: relazioni giuridiche e principali criticità

Author

Cogliandro, RD, Renna, M, Vaccari, S, Cogliandro, RD, Renna, M, and Vaccari, S

Abstract

Il presente contributo ha come oggetto lo studio delle relazioni giuridiche tra gli istituti del raggruppamento temporaneo di imprese e dell’avvalimento, entrambi finalizzati a incentivare la massima partecipazione degli operatori economici ai procedimenti a evidenza pubblica. A tale scopo, la classificazione proposta si fonda sugli schemi del ‘cumulo’ e dell’‘alternatività’, con l’obiettivo di evidenziare i differenti rapporti di concorrenza o di convergenza che possono connotare i due strumenti di cooperazione tra imprese. Una volta tracciata la suddetta distinzione, l’analisi si concentrerà sulle principali criticità che interessano la fattispecie del ricorso all’avvalimento da parte di imprese raggruppate. Una particolare attenzione sarà dedicata alle figure dell’avvalimento interno ed esterno al raggruppamento nonché ad alcune questioni di ‘forma’ nell’ambito della più generale distinzione teorica tra ‘avvalimento-contratto’ e ‘avvalimento-risultato’.

Published
2022

44. Digestibility of defatted insect meals in rainbow trout aquafeeds

Author

Gasco, L., Caimi, C., Trocino, A., Lussiana, C., Bellezza Oddon, S., Malfatto, V., Anedda, R., Serra, G., Biasato, I., Schiavone, A., Gai, F., and Renna, M.

Subjects
aquaculture, feeds, insects, rainbow trout, proteins
Abstract

The assessment of the apparent digestibility coefficients (ADCs) of defatted insect meals (IMs) is fundamental for the correct inclusion of these innovative protein sources in diets for carnivorous fish species. This study aimed at assessing the ADCs of main nutrients, amino acids and fatty acids (FA) of four defatted IMs (two from yellow mealworms, one from black soldier fly and one from lesser mealworm) in rainbow trout (Oncorhynchus mykiss). Five experimental diets were prepared, a control diet and four diets containing 30% of each tested IM based on the substitution method. Celite® was used as digestibility marker. The trial was performed on 80 rainbow trout (initial body weight: 140±5.6 g) kept into tanks connected to in flow-through open water system. After 10 days of diet adaptation, fish faeces were collected across four consecutive weeks using an automatic Choubert device and then analysed. Significant differences were observed among the IMs for the ADCs of dry matter, crude protein, gross energy, methionine, cysteine, glycine and tyrosine, with the lesser mealworm meal showing the overall lowest digestibility values. Such differences were imputed to both insect species and meal processing techniques. The ADCs of ether extract (in all cases higher than 96%) and main FA (namely lauric, myristic, palmitic, oleic, linoleic and ?-linolenic acids; in all cases higher than 85%) did not significantly vary among the IMs. Based on the results, the tested IMs resulted highly digestible and could be a valuable protein source for the formulation of sustainable rainbow trout feeds.

Published
2022
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