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Objective: To review our experience and critically evaluate treatment strategy and results in children with head and neck rhabdomyosarcoma and Ewing's sarcoma., Methods: Retrospective charts review of children affected by non-orbital rhabdomyosarcoma or Ewing's sarcoma of the head and neck who were treated at our institution from January 1996 to August 2009., Results: Seven consecutive children with head and neck rhabdomyosarcoma or Ewing's sarcoma were identified. Four children had rhabdomyosarcoma, 3 children had Ewing's sarcoma. Regions involved were: cheek, ethmoid and maxillary sinuses, nasopharynx, middle ear/mastoid and frontal bone. In one case, surgery was performed as primary treatment modality; the other children were treated firstly with chemotherapy. Three patients underwent surgical resection after chemotherapy, while 4 patients received radiotherapy. Five children are disease free after a median of 7.7 years from initial diagnosis. Two patients relapsed after 10 and 29 months from initial diagnosis respectively; despite the administration of additional therapy both children died of disease., Conclusion: Treatment for rhabdomyosarcoma and Ewing's sarcoma consists in a multimodal therapy involving chemotherapy, radiotherapy and surgery. The optimum use, timing and intensity of these three treatments are still matters of international debate. Chemotherapy in association with radiotherapy has proven capable to obtain local and distant control of disease. But when surgery is unfeasible or fails in radicality, local control is difficult without radiotherapy. Despite additional therapeutic efforts, prognosis of relapsing disease remains poor., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Kawasaki disease is an acute febrile disease of unknown etiology, characterized by systemic vascular inflammation involving the small and medium sized arteries, with a predilection for the coronary arteries. It represents the leading cause of acquired heart diseases in children in developed countries. Diagnosis, difficult because of the clinical characteristics of the disease with typical signs and symptoms appearing sequentially and not simultaneously, may be even more complicated in case of unusual presentation, leading to delay in recognition, particularly in infant in whom a higher incidence of coronary arteries aneurysms has been reported. A high index of suspicion of Kawasaki disease must be maintained in case of prolonged fever in these patients. Timely appropriate treatment is essential to avoid severe sequels. We report the case of a 2 months old male infant with persistent febrile episode, transferred to us from another institution, who presented on echocardiography giant aneurysms on both coronary arteries.

Purpose: To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma., Patients and Methods: The study includes data from 2,216 children (age 0 to 14 years) diagnosed between 1979 and 2005. Overall survival (OS) was analyzed by clinical and biologic features at presentation and periods of diagnosis: 1979 to 1984, 1985 to 1991, 1992 to 1998, and 1999 to 2005. The relative risk of second malignant neoplasm (SMN) was assessed by the standardized incidence ratio (SIR), with the Italian population selected as referent., Results: Yearly patient accrual increased over time from 58 to 102. Patients age 0 to 17 months represented 45.6% of the total population, and their incidence increased over time from 36.5% to 48.5%. The incidence of stage 1 patients increased over time from 5.8% to 23.2%. A total of 898 patients (40.5%) developed disease progression or relapse, 19 patients developed SMN, and two patients developed myelodysplasia. The cumulative risk of SMN at 20 years was 7.1%, for an SIR of 8.4 (95% CI, 5.1 to 13.2). A total of 858 patients (39%) died (779 of disease, 71 of toxicity, six of SMN, and two of tumor-unrelated surgical complications). Ten-year OS was 55.3% (95% CI, 53.0% to 57.6%) and increased over time from 34.9% to 65.0%; it was significantly better for females and patients age 0 to 17 months at diagnosis, with extra-abdominal primary, and stage 1 and 2 disease. OS improved significantly over time in stage 1 and 3 patients. In patients with stage 4 disease, the improvement occurred between the first and second time cohorts (6.7% v 23.5%), but not afterward., Conclusion: The outcome of children with neuroblastoma has progressively improved. Long-term survivors bear a significant risk of SMN.

Melkersson-Rosenthal Syndrome (MRS) is a systemic neuro-mucocutaneous granulomatous disease, characterized in its classical form by a triad of recurrent facial nerve paralysis, swelling of the lips and lingua plicata. However, this classical triad is rarely present, while the monosymptomatic or oligosymptomatic forms are more frequent. The presence of two or one of the manifestations mentioned above, with granulomatous cheilitis in the biopsy, is sufficient to make the diagnosis of monosymptomatic or oligosymptomatic form of MRS. This syndrome is very rare in childhood, instead, it is more frequent in young adults between the second and third decades of life. We present the case of an 8 years old boy who was brought to us because of a non painful swelling of the upper lip, associated with gingival hypertrophy, that had persisted for more than two months. Given the negative results of the hemato-chemical and instrumental assessments, we performed an upper lip biopsy whose histological study showed granulomatous cheilitis. We diagnosed this case as a monosymptomatic MRS and administered an intralesional steroid therapy using triamcinolone, with complete recovery.

Human rotavirus (HRV) is recognized as the most common cause of severe gastroenteritis in children under 5 years of age. Due to the lack of recent reports about the surveillance of HRV infection in Italy, in this study we assessed the prevalence rate of HRV infection on 1,340 stool samples belonging to 1,264 pediatric patients hospitalized with acute gastroenteritis in the period January 2000--December 2002. The stool samples were submitted to virological investigations by electron microscopy (EM) and conventional cell culture, as well as from January 2002 by RT-PCR for norovirus detection. Reovirus-like particles observed by EM were identified by electropherotyping. Single HRV infections were detected in 302 cases (23.9%, ranging from 19.1% in 2000 to 30.2% in 2001). Mixed infections were observed in 28 cases in which HRV was found to be associated with adenovirus in 16 cases (1.3%), with picornavirus in 4 (0.3%), and with norovirus in 8 (2.1% of the 388 cases examined in 2002). The 3 major epidemic periods of HRV infections were March--May 2000 (66 cases), December 2000--May 2001 (128 cases) and September 2001--April 2002 (105 cases) with peaks in March, January and March, and January, respectively. In the periods of major incidence, single HRV infection accounted even for 52.5% of the gastroenteritis cases monthly examined. According to age distribution, 68.9% (208 cases) of HRV infected children was under 4 years (69.6%: 230/330 cases, including mixed infections) and 16.9% (51 cases) was in the 5-12-year age-group. The epidemiological aspects of HRV infection, also compared to other enteric virus infections, will contribute to assess the magnitude of the problem of HRV in different settings and to devise strategies for intervention.

Diamond-Blackfan anemia (DBA) is a congenital disease characterized by defective erythroid progenitor maturation and physical malformations. Most cases are sporadic, but dominant or, more rarely, recessive inheritance is observed in 10% of patients. Mutations in the gene encoding ribosomal protein (RP) S19 have recently been found in 25% of patients with either the dominant or the sporadic form. DBA is the first human disease due to mutations in a ribosomal structural protein. Families unlinked to this locus have also been reported. In an investigation of 23 individuals, we identified eight different mutations in 9 patients. These include five missense, one frameshift, one splice site defect, and one 4-bp insertion in the regulatory sequence. Seven mutations are new; one has so far been found in 8 patients and is a relatively common de novo event. Two mutations are predicted to generate a truncated protein. We also report the prevalence of RPS 19 mutations in the Italian DBA population, as shown by an analysis of 56 patients. No genotype-phenotype correlation was found between patients with the same mutation. The main clinical applications for molecular analysis are clinical diagnosis of patients with an incomplete form of DBA and testing of siblings of a patient with a severe form so as to avoid using those who carry a mutation and a silent phenotype as allogeneic stem cell donors., (Copyright 2000 Academic Press.)

The incidence of hepatitis C in childhood is approximately 0.4%. The mode of transmission can be parenteral, sexual, occupational and also vertical. The latter has an incidence that varies widely and it increases in the case of human immuno-deficiency virus (HIV) coinfection and high titers of HCV in the mother. The vertical transmission is not influenced by breast feeding, however, data are discrepant with regard to child delivery (cesarean section vs vaginal delivery). Ninety-seven babies born from mothers with hepatitis C from 1996 to 1999, were evaluated prospectively in the Day Hospital of the Pediatric Department of Parma. The protocol of observation established a blood sampling for titers of antibodies anti-HCV and HCV-RNA at the 3rd trimester of pregnancy and subsequent clinical and biochemical controls at 3-6-9-12-15 and 18 months. Thirty (31.2%) out of the 96 mothers evaluated were positive for antibodies anti-HCV and 66 (68.8%) were positive for antibodies anti-HCV and HCV-RNA. Five (5.15%) out of the 97 babies evaluated were infected by HCV. Of these 4 were delivered vaginally and 1 by cesarean section. Of the 3 babies born to mothers with HIV coinfection, none was infected by HIV, but 1 was infected by HCV. Vertical transmission is increased by HCV viral load or HIV coinfection in the mother. The vaginal delivery and breastfeeding do not represent an additional risk factor.

Recently the stay in the hospital of the pueperae and of their newborns has progressively been shortened in several Countries. A lot of reports evaluating the risks of early discharges have not shown any significant differences in the hospital readmission rates between newborn early discharged and case-controls. This phenomenon is occurring also in Italy and the early discharge after the delivery is one of the aims of the "Percorso-Nascita" in the National Health Program of 1998-2000. Since three years ago, in the Nursery of the Department of Neonatal Medicine of University of Parma we have been discharging the newborns at term in the second day of life, choosing the healthy newborns in agreement with the obstetricians. The increment of the early discharges has happened in our Department after realizing that those did not create an increment of the hospital admissions in the neonatal period. Since January 2000 in Parma the project of appropriate discharge named "around the cradle" derived from the cooperation between hospitals and territory is working. This project consists of a continuous essistential support for mothers and children throughout an organic integration between hospital and territory. The women that take part to this project have the opportunity to be discharged from the hospital between the 44 and the 56 hours after the physiological delivery, being followed at home by the local obstetrician and paediatrician. The project is divided into three parts. The first part, pre-partum, is based on the information that is delivered to all the pregnant women that are eligible; the second part, the hospital phase, implies the usual and routinary care to the pueperae and their newborns as well as the clinical neonatal and obstetrical evaluation that allows to select our cohort. In the latter phase, extra-hospital, the local paediatrician take care of the newborn and start examining him/her within 72 hours after the discharge, while the home assistance of the local obstetrician will be within 48 hours. After initial difficulties, the organization of this project is quite efficient with a constant increment of attending requests to our project by the patients. Of course this is an organizative model quite complex that needs to verify process, efficiency and results, but anyway it sees the cooperation among many professionists of different specialities: neonatologists, paediatricians, obstetricians, practitioners, all working together for a common programme.

The Italian Association for Paediatric Haematology and Oncology prepared a guideline document aimed at unifying and rationalising as much as possible the management of febrile neutropenia in children with cancer, because of the potential impact of these procedures on hospital costs and on the development of antibiotic resistance. Before starting anti-infective therapy, at least 2 blood cultures, a throat swab, urine-culture, and cultures from any suspected infected site, should be performed. Routine chest X-rays at onset of febrile neutropenia are probably not necessary, in absence of respiratory signs. At the present time, the safer option probably remains the combination of a beta-lactam and an aminoglycoside, and treating febrile neutropenia outside of hospital should be considered an investigational approach. The choice of the most appropriated regimen for each institution should be based also on the local bacteriological statistics and patterns of bacterial resistance. Antibiotic toxicity and cost should be other important factors. Every subsequent addition or substitution of antibiotics should be based on objective signs of clinical deterioration. The only accepted empirical modification is empirical antifungal therapy, while the empirical addition of a glycopeptide antibiotic cannot be recommended.

A case of cholestasis induced by ulcerative papillitis in an 11 month-old boy with AIDS is described. Resolution of cholestasis has been achieved with medical therapy. The authors underline the necessity of repeated endoscopic follow-up to evaluate the risk of secondary papillary stenosis, a complication usually observed in adults.

The AA describe 82 cases of complications occurred during an outbreak of mumps in 1980-81. Forty-two cases of meningitis, 4 of pancreatitis, 2 of orchitis and 2 cases of encefalitis occurred. All patients had eventual, complete recovery. The other cases are not specific complications. The treatment was almost entirely symptomatic. The headache of meningitis was relieved by a lumbar poncture. In the epicrisis of the AA these cases of complications have some importance in relation to the opportunity of getting herd vaccination against mumps.