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Background The aim of this study was to describe the patterns of marriage and parenthood in a cohort of childhood cancer survivors included in the Off-Therapy Registry maintained by the Italian Association of Pediatric Hematology and Oncology. Design and Methods We analyzed a cohort of 6,044 patients diagnosed with cancer between 1960 and 1998, while aged 0 to 14 years and who were 18 years old or older by December 2003. They were followed up through the regional vital statistics registers until death or the end of follow up (October 30, 2006), whichever occurred first, and their marital status and date of birth of their children were recorded. The cumulative probabilities of being married and having a first child were computed by gender and compared by tumor type within the cohort. Marriage and fertility rates (the latter defined as the number of live births per woman-year) were compared with those of the Italian population of the same age, gender, area of residence and calendar period by means of the observed to expected (O/E) ratios. Results During the follow-up period, 4,633 (77%) subjects had not married. The marriage O/E ratios were 0.56 (95% CI: 0.51-0.61) and 0.70 (95% CI: 0.65-0.76) among men and women, respectively. Overall, 263 men had 367 liveborn children, and 473 women had 697 liveborn children. The female fertility O/E ratio was 0.57 (95% CI: 0.53-0.62) overall, and 1.08 (95% CI: 0.99-1.17) when analyses were restricted to married/cohabiting women Conclusions Childhood cancer survivors are less likely to marry and to have children than the general population, confirming the life-long impact of their previous disease on their social behavior and choices. The inclusion of counseling in the strategies of management and long-term surveillance of childhood cancer patients could be beneficial to survivors as they approach adulthood. ©2011 Ferrata Storti Foundation.

Tertiary care centres, grouped in the Italian Association of Paediatric Haematology and Oncology (AIEOP) are unevenly distributed across the country. In an attempt to describe their perceived efficacy, we matched the residence and the location of the treatment centre in 18,441 patients aged ⩽15 years treated in the AIEOP network between 1989 and 2005. Overall, centres located in the central and southern regions were less appealing than those located in the North, although this trend decreased over the study period. Patients with solid tumours migrated more frequently than those with leukaemia or lymphoma. Information resulting from better knowledge of the non-random migrations for treatment of children with cancer will be useful to refine planning of the national paediatric haematology-oncology network with social and economic implications.

We describe the survival patterns of 10,791 Italian children (age 0-14) diagnosed with cancer during 1989-1998 and who were included in the hospital-based registry of the Italian Association of Paediatric Haematology and Oncology. Five-year cumulative survival percentages were 76% for lymphoproliferative disorders and 68% for solid tumours. Survival rates in 1994-1998 significantly improved for acute lymphocytic leukaemia (ALL), acute non-lymphocytic leukaemia, Hodgkin's lymphoma and Wilms' tumour. Gender and age were determinants of survival for some specific types of cancer. Girls with ALL and neuroblastoma exhibited a significant advantage (hazard ratio HR 0.72, 0.62-0.83) and disadvantage (HR 0.73, 0.59-0.90) over boys, respectively. Children with a Wilms' tumour diagnosed above age 3 had a worse prognosis than younger children (HR 2.3, 1.4-4.1). The persisting gender-related difference in survival rate for ALL requires understanding as to whether it is attributable to delays in the adoption of more recent therapeutic protocols, while the corresponding findings for Wilms' tumour and neuroblastoma deserve further biological interpretation.

Hepatitis A virus infection is usually asymptomatic in children. Classic symptomatic forms and atypical clinical manifestations are known. We report a paediatric case of hepatitis A with marked cholestasis, treated with steroids, and with an unusual prolonged course.

IP is an uncommon X-linked dominant disorder (incidence: 1/40.000 newborn). It is caused by mutations in NEMO. It is characterized by cutaneous lesions and dental, ocular, neurologic, nails, hair disorders. The ocular and neurologic sequelae represent the major morbidity in IP. We present a case-report with classical cutaneous features diagnostic for IP. The clinical, ophtalomologic and neurologic examinations revealed no other pathological manifestations.

The aim of this study was to evaluate the reliability of measuring body temperature with an infrared non contact thermometer versus conventional devices in infants and children.A total of 90 pediatric patients, comprising inpatients and ambulatory patients, examined at the Second Children's Clinic of the University of Milan were recruited and divided by age into 3 groups (1 year, 1-5 years,5 years of age). The body temperature of each patient was measured using the study device at three sites (forehead, navel, axilla) and three other commercially available thermometers. Correlation between the measurements was tested with linear regression analysis.The correlation between the measurements was statistically significant; the temperature measured at the three body sites (forehead, navel, axilla) were comparable with those taken with the other thermometers in a statistically significant way.The noncontact Thermofocus infrared thermometer can measure body temperature accurately and reproducibly at various body sites. Thanks to its technology, the device can measure body temperature quickly and noninvasively, an essential feature in pediatric practice.

We report a case of Castleman's disease in 3-month old infant with cervical swelling, treated with surgical excision. This case is interesting because of the rarity of this disease in childhood, of the cervical localization and of the complexity of long- term follow-up.

Incontinentia Pigmenti (IP) is an X-linked dominant disorder of skin with neurologic and ophthalmologic involvement. IP predominantly affects females because the mutations are usually lethal in males in utero. IP is characterized by abnormalities of neuroectodermal tissues. IP is caused by mutations in a gene called NEMO, which is required to activate the NF-kB pathway. We present a diagnostic protocol for IP and a meta-analysis of the clinical spectrum of IP in 82 patients cited by MEDLINE in the European literature from 2000 to 2006.

Aims: To evaluate how often children seen in paediatric accident & emergency (A&E) departments were suspected of abuse or neglect, and to explore some of the correlates of suspected child maltreatment. Methods: Multicentre, cross-sectional study of 15 randomised census days during a six month period. Trained research assistants working with local paediatric staff completed a purpose made anonymised checklist covering sociodemographic and medical information. A six point suspicion index was used to rate compatibility with child maltreatment based on the occurrence of observable harm. Statistical analysis was carried out on the basis that a score of 4 or more was suspicious of child maltreatment. Nineteen hospitals provided standardised paediatric A&E consultation data on 0–14 year olds presenting between 10 am and 10 pm. Results: Of 10 175 assessed children, 204 aroused suspicion of child maltreatment (95% CI 163 to 214 per 10 000). In a logistic regression model of suspected maltreatment statistically significant associations were found with socioeconomic disadvantage, children living in single parent families, and developmental delay. There was no correlation with pre-school age, male gender, foreign origin, or living in urban areas. Conclusions: Child maltreatment based on immediate scoring of suspicion, focused on observable harm, occurred in 2% of a representative sample of paediatric emergency consultations in Italy. This was more common if there were associated social and developmental vulnerabilities. True prevalence of child maltreatment in emergency departments remains elusive because of changing definitions and forensic validation problems.

Following various articles in literature which have appeared in the last 3 years regarding the neurotoxicity of desferrioxamine B we studied, from the auditory functionality point of view, 95 patients affected with transfusion-dependent thalassemic syndromes, under regular chelation treatment with desferrioxamine B. Our results lead us to conclude that at standard doses, between 40 and 60 mg/Kg/day, desferrioxamine B does not demonstrate a neurotoxicity enough to cause an organic deficit in the auditory sphere, while maintaining therapeutic efficacy.

We investigated 63 household contacts of 29 children positive for serologic markers of hepatitis B virus, in order to assess the intrafamiliar spread of HBV infection. 40 relatives of 15 children affected by chronic hepatitis B (Group I) and 23 relatives of 14 patients affected by hemophilia (Group II) were studied. HBV markers (HBsAg, anti-HBs, anti-HBc, HBeAg, anti-HBe) were tested in all the subjects by RIA. Positive markers were found in 39 (62%) of the subjects; the combined prevalence for all HBV markers was 75% in the Group I, and 39% in the Group II. The higher prevalence rate in Group I can be explained by the possible previous infection of another member of the family, whereas in Group II the child is almost surely the first infected subject. Besides, subjects of Group I have probably been in contact with HBV infected children a longer time, compared to subjects in Group II. Our data suggest that household contacts of children with HBV infection are at high risk and should therefore be immunized against HBV.

A 13-years old boy who presented a severe degree of iron-deficiency anemia and diffuse parenchimal infiltrates on the chest roentgenogram is reported. The clinical picture and the presence of hemosiderin laden macrophages in bronchial washing suggest Idiopathic Pulmonary Hemosiderosis (I.P.H.): open lung biopsy confirmed the diagnosis. Immunofluorescence studies showed no deposition of IgG, IgA, IgM and B1C in the lung. The most striking abnormality observed at electron microscopy was hemosiderin deposition in the alveolar-capillary basement membrane. One year after cyclophosphamide therapy was both in complete hematologic and pulmonary remission.

Acute acalculous cholecystitis (CAA) is very rare in children and it is usually related to infectious agents. We report 2 paediatric cases of CAA complicating hepatitis type A, with a favourable evolution with conservative treatment.