Overcoming Underpowering in the Outcome Analysis of Repaired-Tetralogy of Fallot: A Multicenter Database from the CMR/CT Working Group of the Italian Pediatric Cardiology Society (SICPed).

Managing repaired tetralogy of Fallot (TOF) patients is still challenging despite the fact that published studies identified prognostic clinical or imaging data with rather good negative predictive accuracy but weak positive predictive accuracy. Heterogeneity of the initial anatomy, the surgical approach, and the complexity of the mechanism leading to dilation and ventricular dysfunction explain the challenge of predicting the adverse event in this population. Therefore, risk stratification and management of this population remain poorly standardized.

Efficacy and safety of N-acetyl-GED-0507-34-LEVO gel in patients with moderate-to severe facial acne vulgaris: a phase IIb randomized double-blind, vehicle-controlled trial.

Background: Preliminary in vitro and in vivo studies have supported the efficacy of the peroxisome proliferator-activated receptor-γ (PPARγ) modulator N-acetyl-GED-0507-34-LEVO (NAC-GED) for the treatment of acne-inducing sebocyte differentiation, improving sebum composition and controlling the inflammatory process.

Objectives: To evaluate the efficacy and safety of NAC-GED (5% and 2%) in patients with moderate-to-severe facial acne vulgaris.

Methods: This double-blind phase II randomized controlled clinical trial was conducted at 36 sites in Germany, Italy and Poland.

Adjunctive IgM-enriched immunoglobulin therapy with a personalised dose based on serum IgM-titres versus standard dose in the treatment of septic shock: a randomised controlled trial (IgM-fat trial).

Introduction: In patients with septic shock, low levels of circulating immunoglobulins are common and their kinetics appear to be related to clinical outcome. The pivotal role of immunoglobulins in the host immune response to infection suggests that additional therapy with polyclonal intravenous immunoglobulins may be a promising option in patients with septic shock. Immunoglobulin preparations enriched with the IgM component have largely been used in sepsis, mostly at standard dosages (250 mg/kg per day), regardless of clinical severity and without any dose adjustment based on immunoglobulin serum titres or other biomarkers.

7q31.32 partial duplication: First report of a child with dysmorphism, autistic spectrum disorder, moderate intellectual disability and, epilepsy. Literature review.

Introduction: Duplication of long arm of chromosome 7(q) is uncommon. It may occur as "pure", isolated anomaly or in association with other mutations involving the same or other chromosomes. "Pure" chromosome 7q duplication has recently been classified by segment involved: the interstitial, proximal, or distal segment of the arm.

Vaccination coverage in healthcare workers: a multicenter cross-sectional study in Italy.

Introduction: In recent years, a phenomenon known as "vaccine hesitancy" has spread throughout the world, even among health workers, determining a reduction in vaccination coverage (VC).A study aimed at evaluating VC among healthcare workers (HCWs) in 10 Italian cities (L'Aquila, Genoa, Milan, Palermo, Sassari, Catanzaro, Ferrara, Catania, Naples, Messina) was performed.

Materials And Methods: Annex 3 of the Presidential Decree n.

Effect of Combined Epigenetic Treatments and Ectopic NIS Expression on Undifferentiated Thyroid Cancer Cells.

Background: Poorly differentiated (PDTC) and anaplastic thyroid (ATC) cancer cells are characterized by the acquisition of epigenetic abnormalities, leading to the silencing of both the sodium iodide co-transporter and the Coxsackie adenovirus receptor. As aberrant histone acetylation and DNA methylation represent epigenetic mechanisms involved in neoplastic transformation, our study investigated the anticancer properties of epigenetic modifiers in thyroid carcinoma.

Materials And Methods: The cytotoxicity and gene expression modulation of histone deacetylase and DNA methyltransferase inhibitors were evaluated in both PDTC and ATC.

Post-operative radiograph assessment of children undergoing closed reduction and spica cast immobilization for developmental dysplasia of the hip: does experience matter?

Purpose: Closed reduction and spica cast immobilization are routinely used for young patients with developmental dysplasia of the hip with reducible hips. Our primary objective was to assess the interpretation quality of immediate post-operative pelvis radiographs after treatment.

Methods: A series of 28 randomly selected patients (30 hips) with pre- and post-operative pelvis radiographs and post-operative magnetic resonance imaging were included.

A severe myopathy case in aged patient treated with high statin dosage.

Statins (S) are widely used drugs for cardiovascular prevention however their utilization may cause a various grade of muscle toxicity. Sometime S discontinuation alone is not sufficient to revert muscle injury and this can evolve in serious inflammatory muscle disease. In this case immunosuppressive medications are required to achieve remission.

Surgical treatment for symptomatic non-ossifying fibromas of the lower extremity with calcium sulfate grafts in skeletally immature patients.

Background: Non-ossifying fibromas (NOFs) are common benign bone lesions found in children and adolescents. They usually involve metaphysis of long bones, tend to gradually disappear with age and usually do not require surgery, while they are not associated with pathological fractures. The aim of this study was to evaluate the outcome and efficacy of a single-stage procedure, comprising curettage of the lesion and calcium sulfate pellet (CaSP) grafting, in skeletally immature patients with large, symptomatic NOF of the lower extremity, and the possible limitations of the procedure.

Propranolol: Effectiveness and Failure in Infantile Cutaneous Hemangiomas.

Infantile hemangiomas (IHs) occur in early infancy and are considered to be one of the most frequent childhood tumors. Poor responsiveness to the treatment and complications are reported in about 10 % of patients. For years, the most common treatments for cutaneous and complicated IHs were corticosteroids, interferon-α, vincristine, laser therapy, or surgical intervention.

Clinical dissection of childhood occipital epilepsy of Gastaut and prognostic implication.

Background And Purpose: Our aim was to describe the clinical and electrical features and the long-term evolution of childhood occipital epilepsy of Gastaut (COE-G) in a cohort of patients and to compare long-term prognosis between patients with and without other epileptic syndromes.

Methods: This was a retrospective analysis of the long-term outcome of epilepsy in 129 patients with COE-G who were referred to 23 Italian epilepsy centres and one in Austria between 1991 and 2004. Patients were evaluated clinically and with electroencephalograms for 10.

Diagnosis and management of fetal ductus arteriosus constriction-closure.

Pathognomonic features of in utero premature restriction/closure of the ductus arteriosus (DA) are increased right ventricular afterload, impaired right ventricular function, and consequently tricuspid regurgitation and right heart dilation. The most common reason for constriction-closure of DA is maternal administration of non-steroidal anti-inflammatory drugs (NSAIDs) during the 3rd trimester of gestation. The idiopathic form is a rare event and, maybe, an underestimated abnormality that, if it is not promptly recognized, may result in severe fetal-neonatal compromise.

Hypomelanosis of Ito: a round on the frequency and type of epileptic complications.

There is an ample evidence that hypopigmentation of the skin along the Blaschko's lines is frequently associated with neurological disorders. Nowadays, the term "Hypomelanosis of Ito" (HI) is applied when, together with the cutaneous lesions, various and multisystem organs are involved. Among these, the most frequent are cerebral manifestations, such as cognitive delay and epileptic seizures.

Treatment of transient neonatal diabetes mellitus: insulin pump or insulin glargine? Our experience.

Neonatal diabetes mellitus (NDM) results from impaired insulin secretion, occurring within the first 6 months of life. NDM is classified as transient NDM (TNDM) or permanent NDM. To date there are no universal guidelines regarding its management.

Unilateral Eye Blinking Arising From the Ictal Ipsilateral Occipital Area.

We report on an 18-month-old boy with unilateral left eye blinking as a single ictal manifestation without facial twitching. The clinical onset of this phenomenon was first recorded (as an occasional event) at age 3 months, and it was overlooked. By age 6 months, the child's blinking increased to almost daily occurrence in clusters: during blinking the infant showed intact awareness and occasional jerks in the upper limbs and right leg.

Rapid spontaneous resolution of fibromatosis colli in a 3-week-old girl.

Fibromatosis colli is an uncommon benign, congenital fibrous tumor or pseudotumor of the sternocleidomastoid muscle that manifests in infancy. In some of these patients tightening of the muscle results in torticollis. We report the case of a 3-week-old child, who presented with a neck mass localized in the left side with reduced mobility of the head.

Ohtahara syndrome with emphasis on recent genetic discovery.

Ohtahara syndrome or Early Infantile Epileptic Encephalopathy (EIEE) with Suppression-Burst, is the most severe and the earliest developing age-related epileptic encephalopathy. Clinically, the syndrome is characterized by early onset tonic spasms associated with a severe and continuous pattern of burst activity. It is a debilitating and early progressive neurological disorder, resulting in intractable seizures and severe mental retardation.

Acute disseminated encephalomyelitis: a long-term prospective study and meta-analysis.

Background: There are only a few series in the literature on acute disseminated encephalomyelitis (ADEM) in children.

Objectives And Methods: the aims of this study were to perform (i) a prospective clinical/imaging study (1992-2009) on ADEM in children consecutively referred to our institution in Catania, Italy, and (ii) to undertake a systematic review and meta-analysis of published ADEM pediatric cohorts (>10 cases).

Results: We identified 17 patients with ADEM (incidence <10 years of age=1.

A boy born with multiple lesions of atrophoderma.

Aims: The Atrophodermas include a large group of disorders appearing as localized or widespread depressed skin areas and characterized by underlying dermal atrophy. The present study aims to report a peculiar form of previously unreported focal dermal atrophy.

Methods: We studied over a period of 5 years a boy who manifested, since birth, multiple hypopigmented cutaneous atrophic lesions of the atrophoderma type in a mosaic distribution over the body and the legs.