Serum biomarkers for sepsis in children with febrile neutropenia and cancer.

The National Institute for Health and Care Excellence (NICE) defines febrile neutropenia or "neutropenic sepsis" as a patient with an absolute neutrophil count (ANC) less than 0.5 x 109/L and temperature >38°C or signs and symptoms of sepsis.

Clinical tools to assess nutritional risk and malnutrition in hospitalized children and adolescents.

Malnutrition in children and adolescents may be underestimated during hospital stay. In western countries, children were often hospitalized for acute or chronic diseases that are not necessarily related to malnutrition. However, acute or chronic injuries may hamper nutritional status, prolonging recovery after admission and consequently length of hospital stay. Several methods and techniques are known to investigate malnutrition in children, even if their use is not widespread in clinical practice. Many of these are simple and easy to perform and could be useful to a better management of every kind of illness. In this review, we will focus on clinical tools necessary to reveal a nutritional risk at admission and to assess nutritional status in hospitalized children and adolescents.

Human parvovirus B 19 and Epstein-Barr virus co-infection in a child with hereditary spherocytosis.

BACKGROUND: In patients with chronic congenital haemolytic disorders, human Parvovirus B19 (HPV B19) is frequently involved in pure red-cell aplastic crises. Furthermore, it may inhibit three-lineage haematopoiesis in the bone marrow, causing severe pancytopenia. In such patients, Epstein Barr virus (EBV) infection also seems to share the same mechanism as HPV B19 in inducing bone marrow aplasia, but at present the clinical effect of an infection sustained by both viruses is unknown. CLINICAL REPORT: We present a 7-year-old boy affected by hereditary spherocytosis (HS) who suffered from transient aplastic crisis, in whom laboratory findings revealed a double HPV B19 and EBV infection. CONCLUSIONS: To our knowledge, this is the first report of a case of HPV B19 and EBV co-infection diagnosis in a paediatric patient. Despite underlying HS, no signs of haemolytic anaemia were detected, but the infection only produced transient pancytopenia. Nevertheless, the reason why there was no additive effect of the two viruses on the aplastic crisis is still unclear.

Massive venous thrombosis of inferior vena cava as primary manifestation of renal Ewing's sarcoma.

We report an extraordinarily rare case of a 17-year-old male with an extraskeletal Ewing's sarcoma (ESS) of the kidney and a massive thrombosis involving the inferior vena cava (IVC), from the iliac axis to the right atrium. This onset resembled renal cell carcinoma (RCC), although histological examination revealed it was an extraskeletal Ewing's sarcoma/peripheral neuro-ectodermal tumor (EES/PNET). EES/PNET should benefit from neoadjuvant chemotherapy to reduce the risk of metastasis and of recurrent disease due to delay in suitable treatment. Therefore, in the presence of a renal mass with tumor extension of IVC, it is reasonable to bear in mind that other tumors, apart from RCC, could occur. In such cases, a US or CT-scan guided biopsy could be useful.

Errors involving patients receiving intrathecal chemotherapy.

Errors involving patients receiving intrathecal chemotherapy are a significant problem in oncology. Despite the improvement in the management of antineoplastic agents, unintentional intrathecal administration of chemotherapic drugs that are indicated only for systemic administration or intrathecal overdose of drugs regularly used for intrathecal chemotherapy, continue to occur. These events can result in severe neurotoxicity, usually fatal in outcome. We review reported cases of medication errors in intrathecal administration of chemotherapy described in the literature. Diverse rescue therapies have been proposed but the most effective means of managing these errors remains prevention.

Propofol/alfentanil and propofol/ketamine procedural sedation in children with acute lymphoblastic leukaemia: safety, efficacy and their correlation with pain neuromediator expression.

Invasive procedures, such as the lumbar puncture, can cause anxiety and pain in children undergoing treatment for acute lymphoblastic leukaemia (ALL). We investigated the safety and efficacy of two different protocols for analgo-sedation in 20 children with ALL undergoing lumbar puncture. We have conducted a prospective, cross-over study. Protocol A was composed of an association between propofol and alfentanil. Protocol B consisted in the combination of propofol and ketamine. We also evaluated the levels of nerve growth factor, substance P and enkephalins in the cerebrospinal fluid of these patients. All patients showed a satisfactory sedation and analgesia. We found a statistically significant difference of vital parameters between protocol A and protocol B, while there were no significant differences between sedation scores and the other parameters evaluated. Patients in protocol A showed a higher incidence of major side effects, such as respiratory depression. Pain neuromediator levels did not show any statistical difference between the two groups. This study shows that both protocols are effective to obtain a good sedation and analgesia in children with ALL undergoing lumbar puncture, but the association between propofol and ketamine appears to be safer due to the lower incidence of side effects.

Central pontine and extrapontine myelinolysis in a pediatric patient following rapid correction of hypernatremia.

Central pontine and extrapontine myelinolysis are uncommon disorders characterized by distinctive clinical features and typical findings on neuroimaging. Only a few cases are reported in the pediatric age group. We describe the case of a leukemic, malnourished 14-year-old boy with a high serum sodium concentration that gradually increased to 170 mmol/L. During a septic shock episode, hydration with a low sodium concentration at the rate of 104 mL/h for 24 h was administered. A rapid correction of the high serum sodium occurred, exceeding 0.5 mmol/L/h. The following day the patient developed rapid and progressive neurological impairment with clinical features characteristic of central pontine and extrapontine myelinolysis. Magnetic resonance imaging confirmed the diagnosis 11 days later. The patient was treated with steroids and intravenous immunoglobulins. He achieved an almost full neurological recovery and radiological improvement. The reported case demonstrates that central pontine and extrapontine myelinolysis can occur after excessively rapid correction of hypernatremia.

Severe hyperleukocytosis and multifocal intracranial haemorrhage: not always a fatal outcome.

Severe hyperleukocytosis caused by acute lymphoblastic leukaemia (ALL) is associated with an increased risk of early death due to the intracranial haemorrhage. We report on a boy who presented with ALL with an extremely high leukocyte count, who developed neurological deterioration due to multiple intracerebral haemorrhages. Adequate measures for managing this medical emergency include appropriate supportive measures and initiation of therapy to prevent symptoms of leukostasis. Aggressive measures as a decompressive craniectomy should be considered to improve the poor outcome observed in this subset of patients.

Primary Sjögren's syndrome mimicking lymphoma in a pediatric patient. A rare and possibly misdiagnosed condition.

A 12-year-old girl presented at the Oncoemato-logic Department with an acute onset of generalized lymphadenopathy. Lymphoproliferative disorders were highly suspected. Biopsied cervical and inguinal lymph node disclosed neither malignant cells nor monoclonal proliferation of lymphocytes. Revaluating the diagnosis, anamnestic data revealed multiple episodes of bilateral parotid swelling since age one, without systemic symptoms. Laboratory investigations, parotid ecography, Schirmer test revealed Sjogren's syndrome without associated disease. Sjogren syndrome (SS) in childhood is a rare and possibly underdiagnosed condition.

Pharmacological management of chemotherapy-induced nausea and vomiting in children with cancer.

Nausea and vomiting are respectively the first and third most relevant chemotherapy-associated side effects in pediatric oncology, despite the introduction of new antiemetic agents. Symptoms change according to the antineoplastic agents used, and are influenced by age since emotional and psychological aspects play an important role. Studies conducted on new antiemetic therapies usually focus on adult populations affected by tumors, whereas there are very few series including pediatric patients, who are even more severely affected by this specific side effect.

[The prognostic role of Ki-67 in childhood low-grade glioma].

Gliomas currently represent a group of uncommon diseases originating from glial elements. According to their biologic features they can be distinguished in low-grade gliomas--not very aggressive and with a poor tendency to progression--and high-grade gliomas--with a greater proliferative drive and aggressiveness. Such definitions outline two distinct disease types, which profoundly differ as for epidemiological, clinical, diagnostic and molecular features. The introduction of biomolecular techniques has provided a deeper knowledge of low-grade gliomas: the use of new molecular markers, such as Ki-67, makes it possible to study peculiar features of the neoplasm, with strong prognostic implications. Nonetheless, in the literature there is still no agreement on their role, nor on their prognostic validity in pediatric age, also because the criteria that are currently used for adult patients haven't still been codified for pediatric age.

Successful treatment with oral valganciclovir in immunocompetent infant with gastrointestinal manifestations of cytomegalovirus infection.

A 3-month-old male infant was admitted to hospital with anemia. Follow-up controls revealed the presence of specific cytomegalovirus (CMV) antibodies. Virus was isolated from urine, blood, and saliva. At 7 months of age, he presented with melena. Polymerase chain reaction (PCR) of biopsy samples from the duodenum was positive for CMV. Anemia resolved after starting antiviral therapy with oral valganciclovir.

A newborn with multiple fractures as first presentation of infantile myofibromatosis.

Pathological fractures occur in infancy from a variety of causes, but are a rare condition during neonatal period. We describe the case of a male newborn with a metaphyseal fracture of femur and multiple lytic lesions, diagnosed as infantile myofibromatosis.

Phase II trial of temozolomide in children with recurrent high-grade glioma.

PURPOSE: The objective of the study was to evaluate the efficacy and toxicity of Temozolomide (TMZ) administered for 5 consecutive days in three daily dosing in children with recurrent or refractory high-grade glioma. PATIENTS AND METHODS: Twenty-four patients with a median age of 10.5 years were enrolled onto this open-label, multicenter, phase II study. The patients were previously treated with surgical resection (17 of 24), radiotherapy (19 of 24) and chemotherapy (18 of 24). Therapy was administered orally three times a day for 5 consecutive days at the dose of 200 mg/m(2)/dx5 for chemotherapy naive patients. In patients heavily pretreated with chemotherapy the starting dose was of 150 mg/m(2)/dx5. RESULTS: A total of 95 cycles were administered. The median progression free-survival (PFS) was 3 months for the entire group while disease stabilization was obtained in 7 patients (29.1%), all with supratentorial tumors. No CR or PR was observed. TMZ treatment showed a limited toxicity. Thrombocytopenia was the most common hematological adverse effect. Our data suggest a marginal activity of TMZ in children with recurrent high-grade glioma.

The lumbar puncture in pediatric oncology.

In pediatric oncology, LPs are frequently performed for diagnostic and therapeutic purposes. A LP procedure may be helpful in diagnosing many diseases and disorders. In addition, a LP may be performed therapeutically, to inject medications directly into the spinal canal. Intrathecal administration of antineoplastic drugs allows to bypass the selective filter of BBB and to achieve significant concentrations of the antineoplastic agents in CSF reducing the likelihood of systemic toxicity. Lumbar puncture is generally well tolerated but might be characterized by several disadvantages and risks.

[Nutritional status disorders prevalence rates in a sample of pediatric oncology Day Hospital patients]. / Prevalenza delle alterazioni dello stato nutrizionale in un campione di pazienti afferenti a un day hospital oncologico pediatrico.

BACKGROUND: Nutritional status is more important in children than in adults because it is necessary to support normal growth and development. In industrialized countries the prevalence of nutritional status disorders in the pediatric population are as follows: 35-40% of children are overweight/obese, while the underweight status disappeared in some reports or, when it is present, it is associated with other diseases. The aim of this study was to investigate the prevalence rates of nutritional status disorders among an unselected sample of Pediatric Oncology Day Hospital patients. METHODS: Anthropometric parameters of weight and height have been measured in patients affected with solid tumor either on-therapy or off-therapy (0-24 mo). Then Real Body Weight (% RBW) was estimated referring to National Center for Health Statistic percentiles. The sample was then divided into 4 weight classes (under-weight, normal-weight, over-weight and obese) according to % RBW. RESULTS: Overweight patients (overweight + obese) were 44.4%, and 13.9% underweight. Dividing the patients according to whether they are on-therapy or off-therapy, the prevalence of overweight was 36.9% in the former group and 52.9% in the latter, and the underweight prevalence was 26.3 vs 0%. CONCLUSIONS: Our preliminary data show that in this sample of patients on treatment obesity and overweight are present in a similar percentage of the healthy population, but underweight status prevalence is 26.3%. In the sample of patients off-therapy the underweight status disappeared while the overweight status increased. These data suggest that nutritional assessment in oncologic patients is required in order to provide nutritional strategies.

Ten-day schedule oral etoposide therapy in advanced childhood malignancies.

PURPOSE: The activity of etoposide (VP-16) has been demonstrated to be schedule-dependent. Several studies have been conducted on the efficacy and safety of different schedules of VP-16 both in adults and in children, but the optimal schedule has not been determined. METHODS: In the current study, the feasibility and effectiveness of prolonged oral VP-16 in children with high-risk malignancies were evaluated. Between April 1995 and February 1999, 15 pretreated patients with high-risk tumors received oral VP-16. The schedule of therapy was oral VP-16 50 mg/m2/day for 10 consecutive days and 1-week interval between cycles. Therapy was stopped after 1 year of treatment or at time of progressive disease or possible surgery. All patients had received parenteral VP-16 in their earlier chemotherapy. RESULTS: Twelve patients were evaluable for tumor response. After 2 to 4 months of treatment, one patient had complete remission (CR), two had partial response (PR), two had minor response (MR), two had mixed response (MxR), three had stable disease (SD), and two had progressive disease (PD). A useful palliative effect was noted in patients with stable disease. In three patients, oral VP-16 was administered for maintenance therapy. After an average follow-up of 27.5 months (range, 7-41 months), five patients are alive without disease (in three, total surgery was performed after VP-16 therapy) and three patients are alive with disease. Six patients died of progressive disease, and one died of promyelocytic leukemia. One patient had Grade 34 thrombocytopenia; in the remaining patients, no acute toxicity was observed during treatment. CONCLUSIONS: This schedule of oral VP-16 produced CRs, PRs, and MRs in medulloblastoma, neuroblastoma, teratocarcinoma, and ependymoma. Stable disease was observed in three patients, one with an Askin tumor, one with medulloblastoma, and one with hepatoblastoma. Given the possible leukemogenic risk, this schedule should be used as a palliative form of therapy or in patients with poor prognosis..

Hypersensitivity to carboplatin in children.

BACKGROUND: Hypersensitivity reactions are rare but at times severe complications to cytostatic drugs. PROCEDURE: The percentage of allergic reactions to carboplatin and their clinical features were evaluated in 185 children affected by different solid tumors and treated with etoposide-carboplatin chemotherapy. Allergic reactions that occurred during or immediately following etoposide infusion (5 cases, 2.8%) were excluded from the study. RESULTS: Seventeen out of 185 patients (9.2%) suffered from allergic responses to carboplatin. The first of these occurred after an average of 10.1 courses (range, 1-23; median, 9). The risk calculated according to the number of courses is 2% at 6 courses, 11.3% at 12 courses, and 47% at more than 12 courses. CONCLUSIONS: The high risk of allergic reactions to multiple courses of carboplatin should be kept in mind when developing treatment regimens that include the drug.