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Background Rare diseases are chronic and life-threatening disorders affecting Results To the purpose of our analysis, we considered heart, lung, liver and kidney transplants included in the Transplant Registry (TR) of the Italian National Transplantation Center in the 2002–2019 timeframe. Overall, 49,404 recipients were enrolled in the cohort, 5.1% of whom in the pediatric age. For 40,909 (82.8%) transplant recipients, a disease diagnosis was available, of which 38,615 in the adult cohort, while 8,495 patients (17.2%) were undiagnosed. There were 128 disease categories, and of these, 117 were listed in the main rare disease databases. In the pediatric cohort, 2,294 (5.6%) patients had a disease diagnosis: of the 2,126 (92.7%) patients affected by a rare disease, 1,402 (61.1%) presented with a monogenic condition. As expected, the frequencies of pathologies leading to organ failure were different between the pediatric and the adult cohort. Moreover, the pediatric group was characterized, compared to the adult one, by an overall better survival of the graft at ten years after transplant, with the only exception of lung transplants. When comparing survival considering rare vs non-rare diseases or rare and monogenic vs rare non-monogenic conditions, no differences were highlighted for kidney and lung transplants, while rare diseases had a better survival in liver as opposed to heart transplants. Conclusions This work represents the first national survey analyzing the main genetic causes and frequencies of rare and/or monogenic diseases leading to organ failure and requiring transplantation both in adults and children.

New localities and/or confirmations concerning 85 specific and subspecific plant taxa of Tuscan vascular flora, belonging to 68 genera and 36 families are presented: Amaranthus (Amaranthaceae); Allium (Amaryllidaceae); Bifora, Bupleurum (Apiaceae); Arisarum (Araceae); Maianthemum, Ruscus (Asparagaceae); Bidens, Crepis, Galinsoga, Senecio, Taraxacum, Tolpis (Asteraceae); Anchusa, Cerinthe (Boraginaceae); Cakile, Cardamine (Brassicaceae); Butomus (Butomaceae); Campanula (Campanulaceae); Polycarpon, Silene, Stellaria (Caryophyllaceae); Convolvulus (Convolvulaceae); Phedimus (Crassulaceae); Carex, Cyperus, Schoenus (Cyperaceae); Chrozophora, Euphorbia (Euphorbiaceae); Lotus, Medicago, Ononis, Trifolium, Vicia (Fabaceae); Erodium (Geraniaceae); Myriophyllum (Haloragaceae); Philadelphus (Hydrangeaceae); Vallisneria (Hydrocharitaceae); Gladiolus (Iridaceae); Nepeta, Phlomis (Lamiaceae); Tulipa (Liliaceae); Malva (Malvaceae); Epilobium, Oenothera (Onagraceae); Orchis (Orchidaceae); Orobanche (Orobanchaceae); Passiflora (Passifloraceae); Callitriche (Plantaginaceae) Ampelodesmos, Catapodium, Cenchrus, Ceratochloa, Eragrostis, Hordeum, Melica, Nassella, Polypogon, Sclerochloa, Sporobolus (Poaceae); Aquilegia (Ranunculaceae); Rosa (Rosaceae); Asperula, Galium (Rubiaceae); Atropa (Solanaceae); Typha (Typhaceae); Parthenocissus, Vitis (Vitaceae). In the end, the conservation status of the units and eventual protection of the cited biotopes are discussed.

Italy was the first Western country to face the COVID-19 pandemic. Here we report the results of a national survey on kidney transplantation activity in February and March 2020, and the results of a three-round Delphi consensus promoted by four scientific societies: the Italian Society of Organ Transplantation, the Italian Society of Nephrology, the Italian Society of Anesthesia and Intensive Care, and the Italian Group on Antimicrobial Stewardship. All 41 Italian transplant centers were invited to express their opinion in the Delphi rounds along with a group of seven experts. The survey revealed that, starting from March 2020, there was a decline in kidney transplantation activity in Italy, especially for living-related transplants. Overall, 60 recipients tested positive for SARS-CoV2 infection, 57 required hospitalization, 17 were admitted to the ICU, and 11 died. The online consensus had high response rates at each round (95.8%, 95.8%, and 89.5%, respectively). Eventually, 27 of 31 proposed statements were approved (87.1%), 12 at the first or second round (38.7%), and 3 at the third (9.7%). Based on the Italian experience, we discuss the reasons for the changes in kidney transplantation activity during the COVID-19 pandemic in Western countries. We also provide working recommendations for the organization and management of kidney transplantation under these conditions. Electronic supplementary material The online version of this article (10.1007/s40620-020-00755-8) contains supplementary material, which is available to authorized users.

Background It is unknown whether renal pathology lesions in immunoglobulin A nephropathy (IgAN) correlate with renal outcomes over decades of follow-up. Methods In 1130 patients of the original Validation Study of the Oxford Classification for IgA Nephropathy (VALIGA) cohort, we studied the relationship between the MEST score (mesangial hypercellularity, M; endocapillary hypercellularity, E; segmental glomerulosclerosis, S; tubular atrophy/interstitial fibrosis, T), crescents (C) and other histological lesions with both a combined renal endpoint [50% estimated glomerular filtration rate (eGFR) loss or kidney failure] and the rate of eGFR decline over a follow-up period extending to 35 years [median 7 years (interquartile range 4.1–10.8)]. Results In this extended analysis, M1, S1 and T1–T2 lesions as well as the whole MEST score were independently related with the combined endpoint (P Conclusion Long-term follow-up analyses of the VALIGA cohort showed that the independent relationship between kidney biopsy findings and the risk of progression towards kidney failure in IgAN remains unchanged across all age groups and decades after the renal biopsy.

New localities and/or confirmations concerning 61 specific and subspecific plant taxa of Tuscan vascular flora, belonging to 56 genera and 25 families are presented: Delosperma (Aizoaceae), Ridolfia (Apiaceae), Dracunculus (Araceae), Gamochaeta, Hieracium, Matricaria, Rhagadiolus, Senecio, Tolpis (Asteraceae), Atriplex (Amaranthaceae), Iberis, Thlaspi (Brassicaceae), Legousia (Campanulaceae), Canna (Cannaceae), Lonicera, Symphoricarpos, Valerianella (Caprifoliaceae), Sabulina, Scleranthus, Silene (Caryophyllaceae), Sedum (Crassulaceae), Carex, Cyperus, Eleocharis (Cyperaceae), Orthilia (Ericaceae), Acacia, Albizia, Astragalus, Lathyrus, Medicago, Trifolium, Trigonella (Fabaceae), Streptopus (Liliaceae), Abutilon, Malva (Malvaceae), Epilobium (Onagraceae), Orobanche, Phelipanche (Orobanchaceae), Plantago (Plantaginaceae), Ampelodesmos, Bromopsis, Cenchrus, Ceratochloa, Phyllostachys, Pseudosasa, Sesleria, Sporobolus, Trisetaria (Poaceae), Fallopia (Polygonaceae), Alchemilla, Potentilla (Rosaceae), Galium (Rubiaceae), Dictamnus, Ruta (Rutaceae), Atropa, Solanum (Solanaceae). In the end, the conservation status of the units and eventual protection of the cited biotopes are discussed.

The authors briefly discuss about the list of the taxa endemic to Campania (southern Italy) and the typification of their names. The names Santolina neapolitana and Seseli polyphyllum (and other linked names) are lectotypified on herbarium specimens kept at LY and BOLO. A new combination at subspecific rank is proposed for Globularia neapolitana, validating both a previous invalid proposal and an unpublished lectotypification.

L’Italia è localizzata al centro del bacino Mediterraneo, uno tra i centri di biodiversità a livello globale più minacciati. La sua eterogeneità ambientale e climatica ha permesso la differenziazione di un ampio numero di specie vegetali (oltre 8,200 taxa), che tuttavia sono sottoposte ad un crescente numero di minacce principalmente legate ai cambiamenti di dinamiche socio-economiche. Pertanto, urgenti misure di conservazione sono necessarie per fermare la perdita di diversità vegetale e conservare il patrimonio naturale del nostro paese. Questo volume è il prodotto finale di un progetto iniziato nel 2012, finanziato dal Ministero dell’Ambiente e della Tutela del Territorio e del Mare, portato avanti dalla Società Botanica Italiana. Complessivamente, i criteri per la redazione delle liste rosse elaborati dalla IUCN sono stati applicati a 2.488 taxa della flora vascolare autoctona del nostro paese, al fine di valutare il loro attuale stato di conservazione e mettere in evidenza le principali minacce che incombono su di esse. Dopo la pubblicazione del primo volume (2013), che include la valutazione di tutte le specie italiane incluse nelle normative ratificate a livello nazionale (policy species), quali le specie elencate negli allegati della Direttiva 92/43/UE “Habitat” e negli allegati della Convenzione di Berna, in questo contributo è stato valutato un ulteriore gruppo di 2.191 taxa, incluse tutte le specie endemiche italiane non ancora sottoposte ad assessment. I risultati pubblicati nel presente volume hanno rilevato che 37 taxa sono estinti, estinti in natura o verosimilmente estinti a livello regionale (inclusi 11 taxa endemici), mentre 420 taxa (inclusi 228 taxa endemici) sono stati assegnati ad una categoria di rischio. Inoltre, 338 taxa (inclusi 248 taxa endemici) sono stati inseriti nella categoria DD, ovvero specie per cui i dati a disposizione sono insufficienti per una valutazione. La flora vascolare italiana è minacciata soprattutto dalla modificazione degli habitat naturali e semi-naturali, dovuta al disturbo antropico e, specialmente, ad agricoltura, turismo e sviluppo residenziale. Il numero più alto di taxa estinti o in declino è infatti localizzato nelle zone costiere e di pianura, dove gli impatti antropici e la distruzione degli ecosistemi sono più evidenti. La valutazione di circa un terzo dei taxa vegetali spontanei del nostro paese costituisce un importante passo verso la conservazione della flora italiana. Tuttavia, per mettere in atto una strategia di conservazione efficace del paesaggio naturale italiano e in particolare della flora vascolare, ulteriori ricerche ed approfondimenti sono necessari. Sarebbe infatti urgente risolvere alcune criticità tassonomiche, effettuare monitoraggi sulle specie minacciate e sviluppare strategie specifiche di conservazione in situ ed ex situ e piani di azione specie specifici.

Contains fulltext : 232930.pdf (Publisher’s version ) (Closed access) BACKGROUND: On the basis of findings of observational studies and a meta-analysis, proteinuria reduction has been proposed as a surrogate outcome in IgA nephropathy. How long a reduction in proteinuria needs to be maintained to mitigate the long-term risk of disease progression is unknown. METHODS: In this retrospective multiethnic cohort of adult patients with IgA nephropathy, we defined proteinuria remission as a ≥25% reduction in proteinuria from the peak value after biopsy, and an absolute reduction in proteinuria to

Primary hyperoxaluria type 1 is a rare genetic disease; the onset of symptoms ranges from childhood to the sixth decade of life and the disease may go unrecognized for several years. There is an urgent need for drugs able to inhibit the liver production of oxalate and to prevent the disease progression; lumasiran, an innovative molecule based on RNAi interference, is one of the most promising drugs. A group of leading Italian experts on this disease met to respond to some unmet medical needs (early diagnosis, availability of genetic tests and dosage of plasma oxalate, timing of liver transplantation, need for etiologic treatment), based on the analysis of the main scientific evidence and their personal experience. Children showing the characteristic symptoms of the disease usually undergo a metabolic screening and obtain an early diagnosis, while the experience is very limited in adults and the diagnosis difficult. It is therefore essential to increase the knowledge around this disease and the importance of metabolic and genetic screening to define a checklist of shared clinical and laboratory criteria and to establish a multidisciplinary management of potential patients. Oxalate is the cause of the disease: it is crucial to reduce both oxaluria and oxalemia through appropriate therapeutic strategies, able to prevent and/or reduce renal and systemic complications of primary type 1 hyperoxaluria. Lumasiran allows to significantly reduce the levels of oxalate both in blood and in urine, halting the course of the disease and preventing serious renal and systemic complications, if the therapy is started at an early stage of the disease.

Background On the basis of findings of observational studies and a meta-analysis, proteinuria reduction has been proposed as a surrogate outcome in IgA nephropathy. How long a reduction in proteinuria needs to be maintained to mitigate the long-term risk of disease progression is unknown. Methods In this retrospective multiethnic cohort of adult patients with IgA nephropathy, we defined proteinuria remission as a $25% reduction in proteinuria from the peak value after biopsy, and an absolute reduction in proteinuria to,1 g/d. The exposure of interest was the total duration of first remission, treated as a time-varying covariate using longitudinal proteinuria measurements. We used time-dependent Cox proportional hazards regression models to quantify the association between the duration of remission and the primary outcome (ESKD or a 50% reduction in eGFR). Results During a median follow-up of 3.9 years, 274 of 1864 patients (14.7%) experienced the primary outcome. The relationship between duration of proteinuria remission and outcome was nonlinear. Each 3 months in sustained remission up to approximately 4 years was associated with an additional 9% reduction in the risk of disease progression (hazard ratio [HR], 0.91; 95% confidence interval [95% CI], 0.89 to 0.93). Thereafter, each additional 3 months in remission was associated with a smaller, nonsignificant risk reduction (HR, 0.99; 95% CI, 0.96 to 1.03). These findings were robust to multivariable adjustment and consistent across clinical and histologic subgroups. Conclusions Our findings support the use of proteinuria as a surrogate outcome in IgA nephropathy, but additionally demonstrate the value of quantifying the duration of proteinuria remission when estimating the risk of hard clinical endpoints. Copyright © 2021 by the American Society of Nephrology.

DYNAMAP (Dynamic Acoustic Mapping) is a EU LIFE project which aims at developing a dynamic noise mapping system in urban areas (Milan city) and along the motorway A90 surrounding the city of Rome. The two pilot areas present very different characteristics such as the presence of multiple noise sources, roads junctions, traffic and weather conditions that require different approaches and solutions. This work summarizes the main progresses achieved in noise mapping operations in the city of Rome, its criticalities and ongoing proposed solutions regarding the treatment of Anomalous Noise Events detection unrelated to vehicle traffic sources.

Contributions for a vascular flora of Tuscany. X (606-663). New localities and/or confirmations concerning 57 specific and subspecific plant taxa of Tuscan vascular flora, belonging to 42 genera and 25 families are presented: Chenopodium, Polycnemum, Salsola (Amaranthaceae), Torilis (Apiaceae), Hieracium, Tragopogon (Asteraceae), Mahonia (Berberidaceae), Lepidium (Brassicaceae), Campanula (Campanulaceae), Herniaria (Caryophyllaceae), Dichondra (Convolvulaceae), Sedum (Crassulaceae), Cyperus, Schoenoplectus (Cyperaceae), Euphorbia (Euphorbiaceae), Ervilia, Medicago, Trifolium, Trigonella, Vicia (Fabaceae), Geranium (Geraniaceae), Juncus (Juncaceae), Tulipa (Liliaceae), Malva (Malvaceae), Broussonetia (Moraceae), Olea (Oleaceae), Oenothera (Onagraceae), Oxalis (Oxalidaceae), Callitriche (Plantaginaceae), Aira, Anthoxanthum, Catapodium, Hordeum, Imperata, Lolium, Poa, Polypogon, Setaria, Triticum (Poaceae), Fagopyrum (Polygonaceae), Aphanes (Rosaceae), Rubia (Rubiaceae). In the end, the conservation status of the units and eventual protection of the cited biotopes are discussed.