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Cerebellar hemorrhage is rare in term newborns and is most often seen after traumatic birth. Lifelong sequelae include motor and cognitive impairment. We report the uncommon case of a late preterm infant born by spontaneous delivery who showed right peripheral facial palsy at 24 hours of life. Cranial ultrasound showed lateral ventricles dilatation and a diffuse hyperechoic round lesion in the right cerebellar hemisphere. The computed tomography scan confirmed a hemorrhagic lesion in the right cerebellar hemisphere and in the vermis with midline shift and intraventricular bleeding. Ommaya reservoir was inserted and used for a few days. The facial palsy gradually recovered to a complete remission after 6 weeks. Follow-up examinations at 12 and 18 months evidenced infant's delayed motor function, hyperreflexia, tremors, and speech delay.

The treatment of brain tumors and neurodegenerative diseases, represents an ongoing challenge. In Central Nervous System (CNS) the achievement of therapeutic concentration of chemical agents is complicated by the presence of distinct set of efflux proteins, such as ATP-Binding Cassette (ABC) transporters localized on the Blood-Brain Barrier (BBB). The activity of ABC transporters seems to be a common mechanism that underlies the poor response of CNS diseases to therapies. The molecular characterization of Breast Cancer Resistance Protein (BCRP/ABCG2), as an ABC transporter conferring multidrug resistance (MDR), has stimulated many studies to investigate its activity on the BBB, its involvement in physiology and CNS diseases and its role in limiting the delivery of drugs in CNS. In this review, we highlight the activity and localization of BCRP on the BBB and the action that this efflux pump has on many conventional drugs or latest generation molecules used for the treatment of CNS tumors and other neurodegenerative diseases.

Background There is no standardized method for total serum bilirubin (TSB) monitoring during phototherapy for neonatal hyperbilirubinemia and national guidelines give heterogeneous indications. Aim To assess the hypothesis that TSB values do not exceed exsanguino-transfusion (EXT) threshold during phototherapy and that it is possible to decrease its monitoring frequency in jaundiced infants. Study design We carried out a prospective observational study in which changes in TSB during phototherapy for non-haemolytic hyperbilirubinemia were recorded in a cohort of late preterm and term infants. TSB values after 6, 12, 18, and 24 h of phototherapy were compared to the EXT threshold matched to infants' gestational and postnatal age according to the specific nomogram of the Italian Society of Neonatology guidelines. Results We studied 105 infants who started phototherapy at a mean age of 89 ± 37 h when mean TSB was 17.1 ± 2.5 mg/dL. We found that TSB decreased during phototherapy and the difference between mean TSB and EXT threshold progressively increased during phototherapy; TSB exceeded EXT threshold in none of our patients (0%). Conclusions Our study demonstrates that differences between mean TSB and EXT threshold increased during phototherapy in late preterm and term infants with non-haemolytic hyperbilirubinemia; in none of our patients TSB exceeded EXT threshold. Our findings support the possibility of safely decreasing TSB monitoring during phototherapy, thus limiting noxious painful stimuli in neonates.

Background Acute diplopia (AD) is an uncommon and distressing symptom of numerous ocular and neurological conditions, with potentially serious sequelaes. No data are present in pediatrics on the presentation and management of AD. Aim This study investigated characteristics, etiology and health care utilization of the pediatric population with AD accessed to pediatric Emergency Departments (ED), trying to identify “red flags” associated with potentially life -threatening (LT) conditions. Methods We conducted a cohort multicenter study on children with AD in ten Italian hospitals. Patients were classified into diagnostic categories, comparing children with and without LT disease. Results 621 children presented AD at a rate of 3.6 per 10.000. The most frequent diagnosis among no-LT conditions (81.2%) were headache, ocular disorders and minor post-traumatic disease, while LT conditions (18.8%) were represented by brain tumors, demyelinating conditions, idiopathic intracranial hypertension and major post-traumatic diseases. The LT group showed a significantly higher age, with the odds increased by 1% for each month of age. Monocular diplopia occurred in 16.1%, but unlike adult one-fifth presented LT conditions. Binocular diplopia, associated ocular manifestations or extraocular neurological signs were significantly more common in the LT group. At regression logistic analysis strabismus and ptosis were associated with LT conditions. Conclusion The majority of children presented no-LT conditions and more than one-fourth of patients had headache. Monocular diplopia in the LT group was never isolated but associated with other signs or symptoms. Our study was able to identify some specific ocular disturbances or neurologic signs potentially useful for ED physician to recognize patients with serious pathologies.

Background The central-line associated bloodstream infections (CLABSI) are the most common healthcare-associated infections in childhood. Despite the international data available on healthcare-associated infections in selected groups of patients, there is a lack of large and good quality studies. The present survey is the first prospective study monitoring for 6 months the occurrence of central-line associated bloodstream infections in all departments of an Italian tertiary care children’s university hospital. Methods The study involved all children aged less than 18 years admitted to Meyer Children’s University Hospital, Florence, Italy who had a central line access between the October 15th, 2014 and the April 14th, 2015. CLABSI were defined according to the Center for Disease Control and Prevention criteria. CLABSI incidence rates with 95% confidence limits were calculated and stratified for the study variables. For each factor the relative risk and 95% confidence intervals were evaluated. Statistical analysis was performed using the statistical software SPSS for Windows, version 22.0 (SPSS Inc., Chicago, IL), p

Positivity for bla(OXA-48)-like carbapenemase genes was revealed by molecular testing of a surveillance rectal swab from a patient who had previously been colonized and infected by an OXA-48-producing Klebsiella pneumoniae. Positivity was due to a coincidental carriage of Shewanella xiamenensis harboring a new bla(OXA-48)-like gene, while the K. pneumoniae was no longer present.

Tuberculosis (TB) is still one of the most difficult infectious diseases to treat, and the second most frequent cause of death due to infectious disease throughout the world. The number of cases of multidrug-resistant (MDR-TB) and extensively drug-resistant TB (XDR-TB), which are characterised by high mortality rates, is increasing. The therapeutic management of children with MDR- and XDR-TB is complicated by a lack of knowledge, and the fact that many potentially useful drugs are not registered for pediatric use and there are no formulations suitable for children in the first years of life. Furthermore, most of the available drugs are burdened by major adverse events that need to be taken into account, particularly in the case of prolonged therapy. This document describes the recommendations of a group of scientific societies on the therapeutic approach to pediatric MDR- and XDR-TB. On the basis of a systematic literature review and their personal clinical experience, the experts recommend that children with active TB caused by a drug-resistant strain of Mycobacterium tuberculosis should always be referred to a specialised centre because of the complexity of patient management, the paucity of pediatric data, and the high incidence of adverse events due to second-line anti-TB treatment.

Objective To evaluate the hypothalamus-hypophysis-gonad axis in a cohort of children and adolescents with nonmosaic triple X syndrome. Design Cross-sectional study with retrospective analysis. Setting University pediatric hospital. Patient(s) Fifteen prepubertal subjects (median age 9.0 years, range 6.9–11.9 years) with nonmosaic triple X syndrome and age- and pubertal-matched control group (30 girls, median age 9.1 y, range 6.9–11.6 years). Intervention(s) None. Main Outcome Measure(s) We evaluated FSH, LH, and E 2 levels and performed an autoimmunity screening as well as a pelvic ultrasonography and an LH-releasing hormone stimulation test. Result(s) All triple X patients (with and without pubertal signs) showed a pubertal LH peak level that was significantly different from controls. Triple X patients showed increased basal and peak FSH and LH values compared with control subjects. However, the mean E 2 level was significantly lower than control subjects. However, triple X patients showed reduced DHEAS levels and reduced inhibin levels compared with control subjects. Finally, triple X patients had a significantly reduced ovarian volume compared with control subjects, in both prepubertal and pubertal patients. Conclusion(s) Triple X patients showed premature activation of the GnRH pulse generator, even without puberty signs. Both basal and peak LH and FSH levels were higher than in control subjects, and E 2 and inhibin levels and ovarian volume were reduced, which led to a reduced gonadal function. Other studies and a longitudinal evaluation is necessary to better understand the endocrinologic features of these subjects.

A key condition for the efficacy of chemotherapy is that it reaches the tumor mass at a therapeutic concentration. In CNS tumors this process is hampered by the blood brain barrier (BBB). Lately emerged that Multi Drug Resistance (MDR) phenomenon is explained through the cooperation of P-glycoprotein (P-gp) and Breast Cancer Resistance Protein (BCRP), two “gatekeeper” efflux pumps working in tandem on the BBB, also present on plasma-membrane of certain brain tumors. Recently, we have demonstrated that morphine, ondansetron and dexamethasone can interfere with the permeability of BBB allowing doxorubicine accumulation within the rat brain, detected by LC-MS/MS mass spectrometry. All these drugs are substrates of P-gp and BCRP transporters. The aim of the current proposal is to understand the action mechanism of BBB “permeabilization” induced by morphine or other drugs and to exploit this method for the “treatment” of brain tumor in an animal model; i) Quantifying the level drugs that do not usually cross the BBB (mitoxantrone or melphalan) after morphine pre-treatment in a preclinical model. Verifying the cytotoxic effect of morphine plus doxorubicin (and other chemotherapeutic agents) treatment by using MTT and TUNEL analysis in glioblastoma cell lines; ii) Quantifying the level of drugs that do not usually cross the BBB after morphine pre-treatment in an artificial BBB through a monolayer of MDCKII cells over-expressing the human P-gp or BCRP; iii) Investigating the regulatory role of certain microRNA in MDR mechanism by RT-PCR and western blot analysis of P-gp, BCRP, miR-21, miR-27a and miR-451. Our preliminary results suggest that blocking efflux transporters by pretreatment with morphine, ondansetron or desamethasone is able to allow doxorubicin penetration inside the brain. These data will enable us to novel therapeutic approaches to refractory or recurrent CNS tumors in which molecules usually stopped by the BBB may have a therapeutic impact.

A 2 year-old child with motor deficits and headache was referred to our Hospital with an history of speech difficulties and walking difficulties. A brain MRI scan evidenced a giant tumor of the right hemisphere with diffuse contrast enhancement, hydrocephalus and mass effect. After diagnosis, he immediately underwent neurosurgery and a massive debulking of the lesion was performed. The histological diagnosis was anaplastic meningioma (WHO-grade III). Despite radical surgery, after one-month evidence of disease progression was documented by MRI (relapse on surgical margins) and lumbar puncture (neoplastic cells in liquor sample). Rapid progression, young age and absence of successful therapeutic alternatives determined the decision to start chemotherapy with a protocol for soft tissue sarcomas (EpSSG NRSSTS 2005 protocol). The treatment was started in December 2011 and ended in April 2012, and consisted in 3 cycles of Ifosfamide (3 g/m2/day for 3 days) plus Doxorubucin (37.5 mg/m2/day for 2 days) every 21 days. The cycles were followed by Ifosfamide (3 g/m2/day for 3 days ) concomitant with radiotherapy (54 Gy on tumor primary site). A last cycle of Ifosfamide plus Doxorubicin was after performed. In the last 2 cycles, Doxorubicin dose was reduced of 50% to avoid toxicity. End therapy evaluation showed stable disease at brain MRI and absence of tumor cells in liquor sample. Nonetheless, considering risk of disease progression and of late chemotherapy side effects, a strict follow-up was started. After 3 years from diagnosis the patient showed good clinical conditions, with no evidence of disease progression. Anaplastic meningioma is the most undifferentiated, invasive and aggressive type of meningioma (approximately 1-4%). Surgery and radiotherapy are the most effective therapeutic options, while chemotherapy is usually useless. This case represents a successful management of an anaplastic meningioma with chemotherapy in a pediatric patient.

We report the first human case of meningitis and sepsis caused in a child by Actinobacillus suis or A. equuli , a common opportunistic pathogen of swine or horses, respectively. Identification was performed by matrix-assisted laser desorption ionization–time of flight mass spectrometry and real-time PCR assay. A previous visit to a farm was suspected as the source of infection.

Systemic Lupus Erythematosus (SLE) is the prototype of systemic autoimmune disorders. Several infectious diseases can mimic autoimmune disorders , eg. Mycobacterium tubercolosis), Parvovirus B19), and Leishmania. Brucella infection has been rarely reported in such differential.

Tuberculosis (TB) is still the world’s second most frequent cause of death due to infectious diseases after HIV infection, and this has aroused greater interest in identifying and managing exposed subjects, whether they are simply infected or have developed one of the clinical variants of the disease. Unfortunately, not even the latest laboratory techniques are always successful in identifying affected children because they are more likely to have negative cultures and tuberculin skin test results, equivocal chest X-ray findings, and atypical clinical manifestations than adults. Furthermore, they are at greater risk of progressing from infection to active disease, particularly if they are very young. Consequently, pediatricians have to use different diagnostic strategies that specifically address the needs of children. This document describes the recommendations of a group of scientific societies concerning the signs and symptoms suggesting pediatric TB, and the diagnostic approach towards children with suspected disease.