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Abstract Background Lymphatic malformations are vascular developmental anomalies varying from local superficial masses to diffuse infiltrating lesions, resulting in disfigurement. Patients’ outcomes range from spontaneous regression to severe sequelae notwithstanding appropriate treatment. The current classification guides, in part, clinicians through the decision-making process, prognosis prediction and choice of therapeutic strategies. Even though the understanding of molecular basis of the disease has been recently improved, a standardized management algorithm has not been reached yet. Results Here, we report our experience on five children with different lymphatic anomalies of the head and neck region treated by applying a multidisciplinary approach reaching a consensus among specialists on problem-solving and setting priorities. Conclusions Although restitutio ad integrum was rarely achieved and the burden of care is challenging for patients, caregivers and healthcare providers, this study demonstrates how the referral to expert centres can significantly improve outcomes by alleviating parental stress and ameliorating patients’ quality of life. A flow-chart is proposed to guide the multidisciplinary care of children with LMs and to encourage multidisciplinary collaborative initiatives to implement dedicated patients’ pathways.

Abstract Objective Cardio‐facio‐cutaneous syndrome (CFC) is a genetic disorder due to variants affecting genes coding key proteins of the Ras/MAPK signaling pathway. Among the different features of CFC, neurological involvement, including cerebral malformations and epilepsy, represents a common and clinically relevant aspect. Status epilepticus (SE) is a recurrent feature, especially in a specific subgroup of CFC patients with developmental and epileptic encephalopathy (DEE) and history of severe pharmacoresistant epilepsy. Here we dissect the features of SE in CFC patients with a particular focus on longitudinal magnetic resonance imaging (MRI) findings to identify clinical‐radiological patterns and discuss the underlying physiopathology. Methods We retrospectively analyzed clinical, electroencephalogram (EEG), and MRI data collected in a single center from a cohort of 23 patients with CFC carrying pathogenic BRAF variants who experienced SE during a 5‐year period. Results Seven episodes of SE were documented in 5 CFC patients who underwent EEG and MRI at baseline. MRI was performed during SE/within 72 hours from SE termination in 5/7 events. Acute/early post‐ictal MRI findings showed heterogenous abnormalities: restricted diffusion in 2/7, focal area of pcASL perfusion change in 2/7, focal cortical T2/FLAIR hyperintensity in 2/7. Follow‐up images were available for 4/7 SE. No acute changes were detected in 2/7 (MRI performed 4 days after SE termination). Significance Acute focal neuroimaging changes concomitant with ictal EEG focus were present in 5/7 episodes, though with different findings. The heterogeneous patterns suggest different contributing factors, possibly including the presence of focal cortical malformations and autoinflammation. When cytotoxic edema is revealed by MRI, it can be followed by permanent structural damage, as already observed in other genetic conditions. A better understanding of the physiopathology will provide access to targeted treatments allowing to prevent long‐term adverse neurological outcome. Plain Language Summary Cardio‐facio‐cutaneous syndrome is a genetic disorder that often causes prolonged seizures known as status epilepticus. This study has a focus on electroclinical and neuroimaging patterns in patients with cardio‐facio‐cutaneous syndrome. During these status epilepticus episodes, we found different abnormal brain imaging patterns in patients, indicating various causes like brain malformations and inflammation. Understanding these patterns could help doctors find specific treatments, protecting cardio‐facio‐cutaneous syndrome patients from long‐term brain damage.

Collaboration between humans and machines is the core of the Industry 5.0 paradigm, and collaborative robotics is one the most impactful enabling technologies for small and medium Enterprises (SMEs). In fact, small batch production and high levels of product customization make parts assembly one of the most challenging operations to be automated, and it often still depends on the versatility of human labor. Collaborative robots, for their part, can be easily integrated in this productive paradigm, as they have been specifically developed for coexistence with human beings. This work investigates the performance of collaborative robots in machine parts assembly. Design and research activities were carried out as a case study of industrial relevance at the i-Labs industry laboratory, a pole of innovation that is briefly introduced at the beginning of the paper. A fully-functional prototype of the cobotized station was realized at the end of the project, and several experimental tests were performed to validate the robustness of the assembly process as well as the collaborative nature of the application.

Abstract Background Down syndrome is a genetic disorder caused by trisomy of chromosome 21 and characterized by an increased risk of multiorgan involvement. In Down syndrome children, functional constipation and lower urinary tract infections have been described, together with higher risk for incontinence and delayed sphincter control. At present, to our knowledge, no clear association between Down syndrome, Bladder Bowel Dysfunction and neural tube defects has been previously described. Case presentation We describe two female patients with Down syndrome presenting Bladder Bowel Dysfunction in association with neural tube defects, who both underwent personalized multidisciplinary intervention and pelvic floor rehabilitation, with good clinical outcomes. Conclusion At present, no screening program has been established in order to rule out neural tube defects or neurogenic urinary anomalies in Down syndrome patients presenting bowel and/or bladder dysfunction. In our opinion, presence of spinal abnormalities, despite rare, may be contribute to urinary symptoms and should be ruled out in patients presenting progressive or persistent Bladder Bowel Dysfunction. Early diagnosis and management of spinal cord defects associated with neurogenic urinary dysfunction may allow to prevent possible complications.

Abstract Background Cornelia de Lange syndrome is a rare genetic disease with otolaryngological involvement. The classic phenotype is characterized by distinctive facial features, intellectual disability, growth delay, hirsutism, and upper-limb reduction. Nasal polyposis was previously reported in association with chronic rhinosinusitis, however data about prevalence, diagnosis, treatment and prognosis are lacking for this cohort of patients, affected by rare disease. Case presentation We describe the whole diagnostic and therapeutic workflow of nasal polyps in two pediatric patients with Cornelia de Lange, successfully diagnosed and treated by nasal endoscopy. Conclusion Our report confirm that nasal endoscopy is a safe and useful tool in the diagnosis, treatment and follow-up of nasal polyps, even in Cornelia de Lange syndrome pediatric patients. We want to increase the alert for the detection of nasal polyps in patients with Cornelia de Lange syndrome since pediatric age. We recommend endoscopy in all patients with Cornelia de Lange syndrome and symptoms of chronic nasal obstruction and/or OSAS. Multidisciplinary team and sedation service could be useful in the management of Cornelia de Lange syndrome patients with airway obstruction symptoms and sleep disturbance when severe intellectual disability, autism or psychiatric findings are present.

Abstract Smith-Magenis syndrome (SMS) is a neurodevelopmental disorder characterized by cognitive and behavioral symptoms, obesity, and sleep disturbance, and no therapy has been developed to alleviate its symptoms or delay disease onset. SMS occurs due to haploinsufficiency of the retinoic acid-induced-1 (RAI1) gene caused by either chromosomal deletion (SMS-del) or RAI1 missense/nonsense mutation. The molecular mechanisms underlying SMS are unknown. Here, we generated and characterized primary cells derived from four SMS patients (two with SMS-del and two carrying RAI1 point mutations) and four control subjects to investigate the pathogenetic processes underlying SMS. By combining transcriptomic and lipidomic analyses, we found altered expression of lipid and lysosomal genes, deregulation of lipid metabolism, accumulation of lipid droplets, and blocked autophagic flux. We also found that SMS cells exhibited increased cell death associated with the mitochondrial pathology and the production of reactive oxygen species. Treatment with N-acetylcysteine reduced cell death and lipid accumulation, which suggests a causative link between metabolic dyshomeostasis and cell viability. Our results highlight the pathological processes in human SMS cells involving lipid metabolism, autophagy defects and mitochondrial dysfunction and suggest new potential therapeutic targets for patient treatment.

COVID-19 is an infectious disease that has greatly affected worldwide healthcare systems, due to the high number of cases and deaths. As COVID-19 patients may develop cardiac comorbidities that can be potentially fatal, electrocardiographic monitoring can be crucial. This work aims to identify electrocardiographic and vectorcardiographic patterns that may be related to mortality in COVID-19, with the application of the Advanced Repeated Structuring and Learning Procedure (AdvRS&LP). The procedure was applied to data from the “automatic computation of cardiovascular arrhythmic risk from electrocardiographic data of COVID-19 patients” (COVIDSQUARED) project to obtain neural networks (NNs) that, through 254 electrocardiographic and vectorcardiographic features, could discriminate between COVID-19 survivors and deaths. The NNs were validated by a five-fold cross-validation procedure and assessed in terms of the area under the curve (AUC) of the receiver operating characteristic. The features’ contribution to the classification was evaluated through the Local-Interpretable Model-Agnostic Explanations (LIME) algorithm. The obtained NNs properly discriminated between COVID-19 survivors and deaths (AUC = 84.31 ± 2.58% on hold-out testing datasets); the classification was mainly affected by the electrocardiographic-interval-related features, thus suggesting that changes in the duration of cardiac electrical activity might be related to mortality in COVID-19 cases.

Un’analisi della situazione attuale nella scuola italiana evidenzia un aumento degli alunni che presentano bisogni educativi speciali, e in particolare disturbi specifici dell’apprendimento e allievi con disabilità, e al tempo stesso mostra una crescita significativa della dispersione scolastica. Il quadro che emerge sottolinea la necessità di modificare le dinamiche educative, adeguandole ai bisogni specifici di ciascun allievo. Il presente articolo descrive come costrutti quali le Zone di Sviluppo Attuale e Prossimale di Vygotskij e le funzioni cognitive di Feuerstein possano fornire una prospettiva estremamente utile per l’analisi dei processi cognitivi di ciascun allievo, favorendo un approccio personalizzato nel lavoro in classe. Nel 2006, il Parlamento Europeo ha introdotto per la prima volta il concetto di competenze chiave. La quinta competenza, “imparare a imparare”, pone il graduale consolidamento delle funzioni cognitive fragili o carenti degli studenti come uno degli obiettivi fondamentali del percorso scolastico. Tesi del presente articolo è che l’uso degli strumenti del PAS in classe possa fornire un approccio efficace per il consolidamento delle funzioni cognitive degli allievi, che potrà in seguito essere generalizzato agli apprendimenti disciplinari.

Background: Smith-Magenis syndrome (SMS) is caused by either interstitial deletions in the 17p11.2 region or pathogenic variants in the RAI1 gene and is marked by a distinct set of physical, developmental, neurological, and behavioral features. Hypercholesterolemia has been described in SMS, and obesity is also commonly found. Aim: To describe and characterize the metabolic phenotype of a cohort of SMS patients with an age range of 2.9–32.4 years and to evaluate any correlations between their body mass index and serum lipids, glycated hemoglobin (HbA1c), and basal insulin levels. Results: Seven/thirty-five patients had high values of both total cholesterol and low-density lipoprotein cholesterol; 3/35 had high values of triglycerides; none of the patients with RAI1 variants presented dyslipidemia. No patients had abnormal fasting glucose levels. Three/thirty-five patients had HbA1c in the prediabetes range. Ten/twenty-two patients with 17p11.2 deletion and 2/3 with RAI1 variants had increased insulin basal levels. Three/twenty-three patients with the 17p11.2 deletion had prediabetes. Conclusion: Our investigation suggests that SMS ‘deleted’ patients may show a dyslipidemic pattern, while SMS ‘mutated’ patients are more likely to develop early-onset obesity along with hyperinsulinism.

Background: This review systematically examined the scientific literature about electroencephalogram-derived ratio indexes used to assess human mental involvement, in order to deduce what they are, how they are defined and used, and what their best fields of application are. (2) Methods: The review was carried out according to the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) guidelines. (3) Results: From the search query, 82 documents resulted. The majority (82%) were classified as related to mental strain, while 12% were classified as related to sensory and emotion aspects, and 6% to movement. The electroencephalographic electrode montage used was low-density in 13%, high-density in 6% and very-low-density in 81% of documents. The most used electrode positions for computation of involvement indexes were in the frontal and prefrontal cortex. Overall, 37 different formulations of involvement indexes were found. None of them could be directly related to a specific field of application. (4) Conclusions: Standardization in the definition of these indexes is missing, both in the considered frequency bands and in the exploited electrodes. Future research may focus on the development of indexes with a unique definition to monitor and characterize mental involvement.

Abstract Background Costello syndrome (CS) and cardio-facio-cutaneous syndrome (CFCS) belong to the RASopathies, a group of neurodevelopmental disorders with skeletal anomalies. Due to their rarity, the characterization of the musculo-skeletal phenotype in both disorders has been poorly characterized. Patients and methods Herein we reported data on orthopedic findings and functional status of a large sample of CS and CFCS patients. Thirty-four patients (CS = 17 and CFCS = 17) were recruited. Functional and disability evaluations were performed by assessing the 6-min walking test (6MWT) and Pediatric Outcomes Data Collection Instrument (PODCI). Genotype/phenotype correlation was also provided. Results Orthopedic manifestations are highly prevalent in CS and CFCS and overlap in the two disorders. Overall, patients with CS harboring the recurrent HRAS Gly12Ser substitution show a more severe skeletal phenotype compared to patients carrying the Gly12Ala and Gly13Cys variants. Among CFCS patients, those with the MAP2K1/2 variant show different skeletal characteristics compared to BRAF variants, with a higher prevalence of orthopedic abnormalities. Functional assessment showed that patients with CS and CFCS reached lower values compared to the general population, with CFCS patients displaying the lowest scores. Conclusions Orthopedic manifestations appear universal features of CS and CFCS and they can evolve across patients’ life. Longitudinal assessment of disability status by using 6MWT and PODCI could be useful to evaluate the functional impact of orthopedic manifestations on patients’ outcome and help planning a tailored treatment of these comorbidities.

Abstract Background Stickler syndrome is a connective tissue disorder with predominantly autosomal dominant inheritance, with ocular, auditory and joint involvement. Thyroid dysfunction was not described as part of alterations in Stickler syndrome and in particular, the association between Stickler’s syndrome and Graves’ disease has never been previously reported in literature. Moreover, the presence of Graves’ disease is uncommon in the pediatric age (especially in children younger than 6 years old). Case presentation We report the case of a 5-years old child affected by Stickler syndrome who received the diagnosis of Graves’s disease, in absence of suggestive symptoms, during health supervision. Conclusions This is the first evidence of thyroid dysfunction and autoimmune pattern for Sticker syndrome. Further clinical reports are expected before suggesting the implementation of new clinical skills for Stickler syndrome, but this paper may contribute to improve personalized management of this rare disorder.

Severe gastrointestinal symptoms are one of the main reasons for switching from conventional artificial tube feeding to blenderized tube feeding (BTF). This study aimed to describe and quantify the impact of BTF on gastrointestinal symptoms in children and adults. We analyzed four databases (PubMed, Scopus, Cochrane Library, and Google Scholar). The review was performed following the PRISMA extension for Scoping Reviews checklist. The methodological quality of articles was assessed following the NIH quality assessment tools. The initial search yielded 535 articles and, after removing duplicates and off-topic articles, 12 met the inclusion criteria. All included papers unanimously converged in defining an improvement of gastrointestinal symptoms during blenderized feeding: the eight studies involving pediatric cohorts report a decrease from 30 to over 50% in gagging and retching after commencing BTF. Similar rates are reported for constipation and diarrhea improvement in most critically ill adults. Experimental studies and particularly randomized controlled trials are needed to develop robust evidence on the effectiveness of BTF in gastrointestinal symptom improvement with prolonged follow-up and adequate medical monitoring.

Functional gastrointestinal disorders (FGIDs) are very common and life-impacting in children and young adults, covering 50% of pediatric gastroenterologist consultations. As it is known, FGIDs may be due to alterations in the gut–brain axis, dysbiosis and dysregulation of intestinal barrier, causing leaky gut. This may enhance increased antigen and bacterial passage through a damaged mucosa, worsening the impact of different medical conditions such as FGIDs. Little is known about the role of nutrients in modifying this “barrier disruption”. This narrative review aims to analyze the clinical evidence concerning diet and Intestinal Permeability (IP) in FGIDs in children. We searched the PubMed/Medline library for articles published between January 2000 and November 2021 including children aged 0–18 years old, using keywords related to the topic. Since diet induces changes in the intestinal barrier and microbiota, we aimed at clarifying how it is possible to modify IP in FGIDs by diet modulation, and how this can impact on gastrointestinal symptoms. We found that) is that small changes in eating habits, such as a low-FODMAP diet, an adequate intake of fiber and intestinal microbiota modulation by prebiotics and probiotics, seem to lead to big improvements in quality of life.

Background: The Pediatric Eating Assessment Tool (PEDI-EAT-10) is a reliable and valid tool for rapid identification of dysphagia in patients aged 18 months to 18 years. Aims: To translate and adapt the PEDI-EAT-10 into the Italian language and evaluate its validity and reliability. Methods & Procedures: The translation and cross-cultural adaptation of the tool consisted of five stages: initial translation, synthesis of the translations, back translation, expert committee evaluation and test of the prefinal version. The internal consistency of the translated tool was analysed in a clinical group composed of 200 patients with special healthcare needs aged between 18 months and 18 years. They were consecutively enrolled at the Rare Disease Unit, Paediatrics Department, Fondazione Policlinico Agostino Gemelli-IRCCS, Rome. For test-retest reliability, 50 caregivers filled in the PEDI-EAT-10 questionnaire for a second time after a 2-week period. Construct validity was established by comparing data obtained from patients with data from healthy participants (n = 200). The study was approved by the local ethics committee. Outcomes & Results: Psychometric data obtained from patients (104 M; mean age = 8.08 ± 4.85 years; median age = 7 years) showed satisfactory internal consistency (Cronbach's a = 0.89) and test-retest reliability (Pearson r = 0.99; Spearman r = 0.96). A total of 30% of children were classified as having a high risk of penetration/aspiration. The Italian PEDI-EAT-10 mean total score of the clinical group was significantly different from that resulting from healthy participants. Conclusions & Implications: The PEDI-EAT-10 was successfully translated into Italian, validated and found to be a reliable one-page rapid screening tool to identify dysphagia in children and adolescents with special needs.

Abstract Background Activating pathogenic variants in PIK3CA gene usually occur at a mosaic status and underlie a variety of segmental overgrowth phenotypes. Germline variants in PIK3CA have been rarely reported, described in a total of 12 patients with macrocephaly to date. Clinical and prognostic features of these germline variants have not been described in detail yet. Methods Targeted deep sequencing by custom panel of the 21 genes involved in the PI3K/AKT/mTOR pathway was performed in a 13‐year‐old boy with macrocephaly and physical overgrowth. PI3K/AKT/mTOR pathway analysis was performed in fibroblasts by Western blot. The effects of miransertib (AKT inhibitor) and rapamycin (mTOR inhibitor) were assessed. Results A de novo pathogenic variant (c.1090G>C; p.Gly364Arg) in PIK3CA gene was detected in a non‐mosaic status in peripheral blood cells, buccal smears, and skin fibroblasts. Increased levels of phosphorylated AKT residues were observed in fibroblasts, rescued by miransertib. Conclusion Germline variants in PIK3CA are associated to a mild phenotype characterized by overgrowth, severe macrocephaly, mild intellectual disability, and few dysmorphic features. Investigations of PI3K/AKT/mTOR pathway should be performed in patients with severe macrocephaly and unspecific physical overgrowth. Longitudinal studies to assess prognosis and cancer predisposition are recommended.

Noonan syndrome (NS) and the clinically related NS with multiple lentiginous (NMLS) are genetic conditions characterized by upregulated RAS mitogen activated protein kinase (RAS–MAPK) signaling, which is known to impact hippocampus-dependent memory formation and consolidation. The aim of the present study was to provide a detailed characterization of the recognition memory of children and adolescents with NS/NMLS. We compared 18 children and adolescents affected by NS and NMLS with 22 typically developing (TD) children, matched for chronological age and non-verbal Intelligence Quotient (IQ), in two different experimental paradigms, to assess familiarity and recollection: a Process Dissociation Procedure (PDP) and a Task Dissociation Procedure (TDP). Differences in verbal skills between groups, as well as chronological age, were considered in the analysis. Participants with NS and NSML showed reduced recollection in the PDP and impaired associative recognition in the TDP, compared to controls. These results indicate poor recollection in the recognition memory of participants with NS and NSML, which cannot be explained by intellectual disability or language deficits. These results provide evidence of the role of mutations impacting RAS–MAPK signaling in the disruption of hippocampal memory formation and consolidation.

Costello syndrome (CS), a rare syndrome with multisystemic involvement inherited as a dominant trait, is characterized by developmental delay, coarse facial appearance, cardiac defects including hypertrophic cardiomyopathy, skin abnormalities, brain complications, and a predisposition to certain malignancies. The musculoskeletal system is particularly affected in CS, with peculiar orthopedic anomalies that impact posture and gait. Dystonia has been recently documented to contribute to abnormal postures and musculoskeletal anomalies characterizing CS, suggesting the possible use of pharmacological treatments to treat these complications. We report the case of a child affected by CS displaying a particularly severe musculoskeletal involvement with dystonic posture especially in the arms and legs. The Movement Disorder-Childhood Rating Scale (MD-CRS) and a gait analysis were used to assess clinical patterns of hyperkinetic movement disorder and dystonia. The child was further treated with trihexyphenidyl for six months with a final dosage of 14 mg. MD-CRS and gait analysis assessments provided evidence for a significant improvement of posture and the related musculoskeletal problems with no side effects. Our preliminary study report provides first evidence that pharmacological anti-dystonia treatment significantly improves movement and posture disorders in patients with CS. Further studies enrolling larger cohorts of patients should be performed to validate these preliminary observations.

Se sono ampiamente riconosciuti i benefici dell’attività motoria sulla salute, recenti evidenze ne hanno messo in luce una specifica valenza terapeutica. Una particolare forma di tale approccio, sviluppata in ambito italiano con il nome di Montagnaterapia, pone l’accento sugli effetti positivi di una serie di attività motorie svolte nell’ambiente naturale e culturale della montagna. In tale prospettiva si inserisce il Laboratorio di Potenziamento Cognitivo e Montagnaterapia, che si rivolge a ragazzi con disabilità intellettiva dagli 11 ai 15 anni. L’ambiente naturale della montagna fa da sfondo ad attività, quali l’orientamento, lo studio della flora o dei cicli produttivi agroalimentari, in cui i ragazzi possono confrontarsi con situazioni autenticamente complesse, sotto la supervisione di mediatori esperti. Obiettivi cognitivi del Laboratorio sono da un lato il trasferimento di strategie cognitive precedentemente apprese in un ambiente reale, uscendo dal contesto predeterminato che caratterizza il lavoro educativo, e dall’altro lo sviluppo di rappresentazioni mentali fondate su una solida base esperienziale, adeguata per supportare processi di pensiero astratto e di riflessione metacognitiva. Obiettivi coessenziali sono il consolidamento nei ragazzi di più mature competenze relazionali e lo sviluppo di un senso di sé, legati all’esperienza di autoefficacia ed al confronto con il gruppo dei pari.

KBG syndrome is a rare multisystem developmental disorder caused by ankyrin repeat domain-containing protein 11 (ANKRD11) gene haploinsufficiency, resulting from either intragenic loss-of-function mutations or microdeletions encompassing the gene. Concerning the behavioral phenotype, a limited amount of research has been focused on attention deficit and hyperactivity disorder, autistic-like features, anxiety and impairments in emotion regulation, and no study has provided a systematic assessment. The aim of the present work is to investigate the psychopathological profile in children, adolescents, and young adults with KBG syndrome. Seventeen subjects with molecularly confirmed diagnoses were evaluated to investigate cognitive abilities and psychopathological features. Parametric and nonparametric indexes were used to describe the patient cohort according to type and distribution of specific measures. The KBG subjects were characterized by a low mean IQ score, with a distribution characterized by a variability similar to that occurring in the general population. Prevalence of neuropsychiatric disorders were computed as well as the corresponding confidence intervals to compare their prevalence to that reported for the general population. The KBG subjects were characterized by higher prevalence of obsessive-compulsive, tic, depressive and attention deficit and hyperactivity disorders. Obsessive-compulsive disorder is a peculiar aspect characterizing the psychopathological profile of KBG patients, which does not seem to be related to the cognitive level. The present study provides new relevant information towards the definition of a psychopathological phenotype of KBG syndromes useful to plan a better treatment for patients.

Una crescente mole di evidenze scientifiche, in ambito neurobiologico, clinico e pedagogico, conferma da un lato la grande plasticità del cervello umano e dall’altra l’importanza dell’intervento precoce. Tuttavia, mentre alcuni approcci terapeutici e riabilitativi sono disponibili in relazione a specifici quadri patologici, mancano ancora interventi strutturati precoci nell’ambito della disabilità intellettiva. Il Programma di Arricchimento Pre-Strumentale (PAPS) di Leoni e Pavan rappresenta un percorso educativo ed abilitativo strutturato, disegnato per essere applicabile con bambini con disabilità cognitiva e disturbi dell’apprendimento a partire dai 18 mesi di età. Prende le mosse dalla pedagogia della mediazione e dalle teorie e dal metodo Feuerstein, da cui mutua l’idea della centralità dell’Esperienza di Apprendimento Mediato nei processi di apprendimento. Reinventa però in modo totalmente innovativo e originale costrutti teorici, concettuali e metodologici, allo scopo di poter intervenire fin dalla primissima infanzia. Le attività, proposte con un approccio ludiforme, sono graduate per complessità ed astrazione. Cuore del percorso è la strutturazione delle Attività Cognitive Primarie, che rappresentano le prime abilità cognitive che emergono durante lo sviluppo, e costituiscono prerequisiti trasversali per qualsiasi forma di apprendimento successivo. Target coessenziale dell’intervento è rappresentato da un gruppo di concetti di base, scelti da un lato perché contribuiscono all’organizzazione della percezione e dall’altro perché consentono di esporre sistematicamente i bambini ad esperienze-modello di apprendimento.