Rivista di formazione e aggiornamento di pediatri e medici operanti sul territorio e in ospedale. Fondata nel 1982, in collaborazione con l'Associazione Culturale Pediatri
Gli articoli più visti nel mese di Giugno 2022
1) La pagina gialla - Maggio 2022
a cura di Alessandro Ventura
50 kB2) Aggiornamento - Maggio 2022
Malattia di Behçet: una sfida per il pediatra
- Levantino L, Pastore S, Corona F, Lepore L, Tommasini L.
- Behçet’s disease (BD) is a rare chronic inflammatory condition associated with variable-vessel vasculitis, systemic involvement and relapsing course. It is characterized by a wide clinical heterogeneity, presenting with mucocutaneous, ocular, articular, gastrointestinal, neurological and vascular manifestations in the long term, with a high risk of delay in diagnosis: therefore, its morbidity and mortality are significant. The definition of BD probably encompasses a range of disorders with unclear pathogenesis, overlapping autoinflammatory and autoimmune pathological mechanisms. Diagnosis is clinical and multiple classification criteria have been developed. The progressive knowledge about its molecular basis, especially in the so-called monogenic mimics of BD, resulted in an improvement in the disease therapy, with an optimization of the use of conventional immunosuppressive agents and the introduction of new biologic drugs. BD occurs in childhood in up to 20% of cases: paediatric BD differs from adult BD in frequency and severity of clinical findings and, above all, for a frequent incomplete clinical picture, making the recognition and management of the disease a challenge. The new paediatric classification criteria are aimed to allow an early diagnosis and a proper therapy.
3) Ricerca - Maggio 2022
Due anni di MIS-C: peculiarità cliniche e orizzonti terapeutici
- Mambelli L, Uva A, Iacono A, Del Vecchio L, Bianchedi I, Nuzzo A, Malta B, Marchetti F.
- Introduction - MIS-C is the most relevant complication of SARS-CoV-2 infection and has an incidence of 1 case / 2,200 children and adolescents with previous infection. The clinical picture is characterized by a multiorgan involvement and an insufficiently known response to therapy.
Objectives - Retrospective analysis carried out in the Paediatric Department of the Ravenna Hospital from April 2020 to March 2022 of the cases admitted with MIS-C, with description of the presentation characteristics, the differences with respect to Kawasaki disease and the therapeutic strategies adopted also with regard to the clinical response.
Results - Thirteen cases diagnosed with MIS-C with an average age of 6 years and four months were hospitalized, 4 cases were of Italian origin, 6 of Balkan and 3 of African. All the cases presented with fever> 39 ° C with severe malaise / irritability, associated with skin rash in 9 cases, conjunctivitis in 7 and cheilitis in 5. The most prominent manifestations of organ involvement were abdominal pain / diarrhoea (N = 9), myocardial dysfunction in the absence of coronary involvement (N = 5), presence of pulmonary thickening (N = 5), painful laterocervical lymphadenitis with reduced neck motility (N = 5), subnephrotic proteinuria (N = 7) and hypertransaminasaemia (N = 8). Two cases with “encephalitic-like” psychomotor slowing, one with important ascites and one with oedema of the para/retropharyngeal tissues were clinically relevant for the severity of the onset. Twelve cases were treated with IVIG and methylprednisolone (2 mg /kg), 4 of them were found to be non-responders and required the use of steroid boluses (4 cases) and anakinra (4 cases), in three of the latter in association with steroid boluses, with rapid and decisive clinical response.
Conclusions - The serious clinical manifestations that characterize MIS-C do not concern only cardiac involvement. Therapy with an IL-1 inhibitor, anakinra, must also be envisaged as a possible first-line treatment in case of a particularly severe clinical picture at the onset.
5) L'intervista - Maggio 2022
a cura di Alessandro Ventura6) Linee guida - Ottobre 2020
La prima infezione urinaria febbrile in bambini di età compresa tra 2 mesi e 3 anni
- Gruppo di lavoro sulle infezioni delle vie urinarie della Società Italiana di Nefrologia Pediatrica (SINePe)
- The aim was to update the recommendations for the diagnosis, treatment and follow-up of the first febrile urinary tract infection in young children, which were first published in 2012 and endorsed by the Italian Society of Paediatric Nephrology. The Italian recommendations were revised on the basis of a review of the literature published from 2012 to October 2018. An ad hoc evaluation of the risk factors, which were published in the previous recommendations, was carried out to identify children with high-grade vesicoureteral reflux or renal scarring. When evidence was not available, the working group held extensive discussions during various meetings and through email exchanges. Four major modifications have been introduced. The method for collecting urine for culture and its interpretation has been re-evaluated. The algorithm that guides clinical decisions to proceed with voiding cystourethrography has been reformulated. The suggested antibiotics have been revised and further restrictions of the use of antibiotic prophylaxis have been recommended. These updated recommendations have now been endorsed by the Italian Society of Paediatric Nephrology and the Italian Society for Paediatric Infectivology. They can also be used to compare other available recommendations, as a worldwide consensus in this area is still lacking.
7) Percorsi clinici - Maggio 2022
Sincope ricorrente: attenti alla coronaria
- Tessitore A, Chicco D, Trevisiol C, et al.
- Anomalous aortic origin of coronary artery (AAOCA) is a rare condition and the second cause of sudden cardiac death among young athletic patients. Suspicion should arise in the presence of exertional syncope without prodrome, chest pain and/or dyspnea. An accurate transthoracic echocardiogram with Doppler colour flow mapping is the best and first method to identify AAOCA, followed by cardiac CT or MRI to define the coronary anatomy that could benefit from corrective cardiac surgery.
9) L'angolo dell'immunologo - Ottobre 2001
Quando sospettare un’immunodeficienza
- A. Tommasini, A. Insalaco, C. Perez, A.R. Soresina, A. Plebani