The waiting room as a relational space: young patients and their families' experience in a day hospital.

BACKGROUND: Waiting in hospital is a condition of high stress for patients and their families, especially in childhood. The literature has investigated the emotional experiences of patients and their families, recognizing the need for a comfortable environment, attention from the staff, information and sharing emotions with others. Waiting time in day hospitals is a topic that has not yet been investigated in the literature, but is nevertheless interesting for researchers. This exploratory study investigates the experience of waiting young patients and their families go through during treatments in day hospitals. METHOD: Fifty children and adolescents from ages 7 to 15 years, admitted to the day hospital of a paediatric haematology and oncology ward of an Italian hospital, completed the Emotional Reaction Instrument (ERI) and the Child Drawing: Hospital. Their parents or relatives completed a semi-structured interview on waiting. RESULTS: The data showed that the young patients displayed a low level of anxiety and negative emotion. In contrast, the adults' experience of waiting in the day hospital entailed boredom, anxiety and concern for the emotional state of their children. These conditions can be alleviated by relationships and sharing emotions with other adults. CONCLUSIONS: This study has shown that day hospital waiting rooms should be organized and should be experienced by adults and children as relational spaces. This could provide useful suggestions in order to improve the organization of day hospital waiting rooms.

The use of central venous catheters in haemophilia patients.

Summary. Central venous access devices (CVADs) play an important role in the management of haemophilia patients requiring repeated and/or urgent administration of coagulation factor concentrates. In this article, we summarize current knowledge regarding the use of central venous catheters in these patients, indicating advantages and disadvantages of both fully implantable and external tunnelled CVADs. Finally, we describe our personal experience on the use of the external tunnelled catheter Broviac.

Cerebral venous sinus thrombosis in childhood: clinical aspects and neurological and cognitive long-term outcome in three cases.

We report clinical findings, risk factors and neurological and cognitive long-term outcome in three Italian children aged 7, 8 and 5, respectively, who experienced cerebral venous sinus thrombosis (CVST). All children presented with headache, associated to nausea, vomiting and papilloedema. None suffered from epileptic seizures. In two of them a paresis of the sixth cranial nerve with diplopia was found. Diagnosis was confirmed by magnetic resonance imaging angiography (angio MRI) in all cases. In all patients plasma levels of protein C, protein S, antithrombin III (AT III), antiphospholipid antibodies (ApA) and homocysteine were detected. Furthermore, factor V Leiden mutation, prothrombin mutation G20210A and MTHFR mutation were searched for. A Protein C reduction was detected in all patients at onset; this finding, however, was not confirmed at follow-up in all of them. At one-year follow-up, neurological examination was normal in all children and neuropsychological assessment, aimed at excluding linguistic and non-linguistic cognitive deficits, revealed normal performances in two of them. In the third child, cognitive assessment confirmed a previously diagnosed developmental dyslexia.

Diamond-Blackfan anemia: report of seven further mutations in the RPS19 gene and evidence of mutation heterogeneity in the Italian population.

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.

Trichosporon beigelii: a life-threatening pathogen in immunocompromised hosts.

Patients undergoing bone marrow transplantation are profoundly immunosuppressed as a result of their intensive myeloablative chemotherapy and are at high risk for opportunistic fungal infections mainly caused by Candida spp and Aspergillus spp. Trichosporon beigelii (T beigelii) has emerged as a life-threatening opportunistic pathogen in granulocytopenic and immunocompromised hosts and there is a marked increase in cases reported in the literature. Response to antifungal agents is poor, mortality is high and immunological recovery is the most important factor for a favorable outcome in patients with trichosporonosis. We present three cases of T. beigelii infection in patients undergoing allogeneic bone marrow transplantation in our center and we review cases described in the literature.

[Vertical transmission of the hepatitis C virus]. / La trasmissione verticale del virus dell'epatite C.

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.

[Early discharge project appropriate for the healthy newborn]. / Progetto di dimissione precoce appropriata del neonato sano.

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.

Bloodstream infections in children with cancer: a multicentre surveillance study of the Italian Association of Paediatric Haematology and Oncology. Supportive Therapy Group-Infectious Diseases Section.

A one-year prospective, multicentre surveillance study on aetiology, main clinical features and outcome of bloodstream infections in children with cancer was conducted in 18 paediatric haematology centres belonging to the Italian Association for Paediatric Haematology and Oncology. A total of 191 bloodstream infections were reported during the study period. Of them, 123 (64%) occurred in neutropenic and 68 (36%) in non-neutropenic patients. Gram-positive cocci caused 45% (85/191) of the episodes, gram-negative rods 41% (78/191), and fungi 9% (18/191). The remaining 5% (10/191) of the episodes were poly-microbial infections. A total of 204 pathogens were isolated (46% gram-positive cocci; 44% gram-negative rods; and 10% fungi). The aetiologic distribution was similar among neutropenic and non-neutropenic patients. A correlation between the infection and the presence of an indwelling central venous catheter was found in 20% (23/114) of the episodes among neutropenic patients and in 55% (23/62) among non-neutropenic patients. Gram-negative micro-organisms were isolated in an unusually high proportion of catheter-related infections (48%). The overall mortality rate from any cause within 30 days from the first positive blood culture was 11%, and was higher among patients who were neutropenic at the onset of the infection than among those who were not neutropenic (15 versus 4%, P = 0.03). In addition, the mortality was significantly higher in recipients of bone marrow transplantation than in patients with acute leukaemia or solid tumour (21, 11 and 6%, respectively) and was also higher in fungaemias and poly-microbial infections (22 and 30%) than in single gram-positive and gram-negative bacteraemias (11 and 6%).

Central nervous system atypical teratoid/rhabdoid tumour of infancy. CT and mr findings.

In 1995, as a result of the observation of Rhabdoid elements among the other components of a Teratoid/Rhabdoid tumor (TRT), a new nomenclature was introduced, Atypical Teratoid/Rhabdoid Tumor (ATRT) of infancy and childhood. We report the clinical history and radiological findings in a child affected by central nervous system (CNS) ATRT.

Italian guidelines for the management of infectious complications in pediatric oncology: empirical antimicrobial therapy of febrile neutropenia.

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.

Preliminary report: the short-term effects of direct p53 DNA injection in primary hepatocellular carcinomas.

A pilot study, to assess the therapeutic potential of percutaneous injection of wild-type p53 (wt-p53) in five patients with primary hepatocellular carcinoma is reported. Three of the five patients showed objective tumor response with reduction of the tumor volume on computed tomographic (CT) scan measurements as well as a significant fall of serum alphafetoprotein. Much further work will be needed to elucidate the mechanism of action.

Pharmacologic treatment of acute congestive heart failure resulting from left ventricular systolic or diastolic dysfunction.

Patients presenting in acute congestive heart failure demonstrate severe dyspnea, rales, hypoxia, and chest radiograph findings of acute pulmonary vascular congestion. Not all patients, however, have systolic left ventricular dysfunction. While initial stabilization of an acute episode in decompensated heart failure may follow a common pathway, understanding the nature of the dysfunction is important in selecting both short-term and long-term pharmacologic interventions required in the patient's management.

Normal values of total reticulocytes and reticulocyte subsets in children and young adults.

This work provides the values for percentage and total reticulocyte count, as well as the values of reticulocyte fluorescence intensity ratios as determined by an automated fluorescent technique in a normal pediatric population. The peripheral reticulocyte subsets, as assessed on the basis of their degree of maturation, allow a more accurate evaluation of bone marrow activity than the percentage or total count do. This would result in a more appropriate management of erythropoietic disorders in children.

Prognostic factors in epithelial ovarian cancer.

In the present study the Authors have statistically evaluated the influence of the main prognostic factors in 160 women affected by epithelial ovarian cancer. They were sequentially observed at Oncologic Institute of Bari, Italy from december 1981 to december 1989. The prognostic factors (age, stage, grading, histological type, performance status and residual disease), have been evaluated through an actuarial analysis of survival and compared with those reported by many authors in literature. Our data confirm the relevance, among all, of the grading, the clinical stage and residual disease as traditional parameters. Furthermore it is proposable the use of new prognostic factors such as nuclear ploidy and proliferative activity (S-phase, labeling index).

Serum levels of TAG 72 and CA 15.3 in patients with gynaecological neoplasms.

In the present report we have evaluated two new serum tumour markers identified by monoclonal antibodies: TAG 72 and CA 15.3 in 62 patients with gynaecological carcinomas, in prevalence ovarian and 36 women with benign gynaecological diseases. Serum levels of both markers were determined using two immunoradiometric assays and the cut-off values were set at 5 U/mL for TAG 72 and at 40 U/mL for CA 15.3. In our study the sensitivity was 57% for TAG 72 and 43% for CA 15.3 with a specificity of 97% and 89% respectively. Even if these two markers show a sensitivity lower than CA 125, these preliminary results seem to suggest the possible role of TAG 72 and CA 15.3 both as confirmatory tests and as adjunctive tests, especially the first for its excellent specificity.

Tumor necrosis factor and soluble interleukin-2 receptor: two immunological biomarkers in female neoplasms.

In this study we have evaluated two new immunological parameters, soluble IL-2 receptor (s IL-2 R) and TNF, in 119 patients with female solid neoplasms (47 ovarian and 72 breast cancer). Our data demonstrate that both these markers have mean serum levels in cancer patients higher than in normal population, particularly in ovarian cases. Also the overall positivities were higher in ovarian (68%) than in breast cancer (51%). Finally we observed no relevant differences according to the status of disease in both groups of cancer patients. These preliminary results could suggest the possible usefulness of an immunological monitoring in cancer patients, above all when an immunotherapy with biological responder modifiers is proposed.