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.

Anal vector volume analysis: an effective tool in the management of pelvic floor disorders.

INTRODUCTION: Different trials have investigated the role of conventional anal manometry in the diagnosis of pelvic floor disorders. The aim of the present study is to define the role and the effectiveness of vector anal manometry and vector asymmetry index scoring in the assessment of pelvic floor disorders i.e. fecal incontinence and obstructed defecation. METHODS: Between 2005 and 2007, 387 patients underwent clinical and manometric evaluation in the Department of Surgery, University Hospital Tor Vergata, Rome, Italy, and were included in the present prospective cohort study. All the patients underwent clinical examination, Wexner incontinence scoring, and anal vector manometry and were classified into three groups. Group I included patients with normal resting anal pressure values (195 patients). Groups II and III consisted of patients with resting anal pressure higher and lower than normal values, respectively (90 and 102 patients, respectively). All patients were classified into asymmetric and non-asymmetric according to the vector asymmetry index using a cut-off of 20%. We investigated the correlation between anal asymmetry and pelvic floor disorders, i.e. fecal incontinence due to sphincter injury, rectal prolapse, and obstructed defecation. RESULTS: In Group III, the number of asymmetric patients was significantly higher than non-asymmetric ones (P < 0.0001). Asymmetry values were significantly higher in group III than in groups I and II considering squeeze (P < 0.0001) or resting pressures (P < 0.0001). Furthermore, there was a statistically significant association between anal asymmetry and both anal incontinence (P < 0.0001) and rectal prolapse (P = 0.0270). No such association was found between anal asymmetry and obstructed defecation. CONCLUSION: Anal vector manometry using vector analysis of resting and squeeze pressures is complementary to endoanal ultrasonography, as it provides information on anal sphincter function and integrity. The vector asymmetry index >20% correlates with fecal incontinence due to anal sphincter lesions. Therefore, anal vector manometry may be useful as an independent method of screening for pregnant women at risk of sphincter injury and for patients undergoing anorectal surgery with risk factors for incontinence, like previous anorectal surgery or a history of two or more previous vaginal deliveries.

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.

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.

Treatment of childhood sarcoma with irinotecan: bilirubin level as a predictor of gastrointestinal toxicity.

Irinotecan is a promising anticancer agent for the treatment of childhood cancer unresponsive to conventional chemotherapy. Its active metabolite, 7-ethyl-10 hydroxycamptothecin (SN-38) is glucuronidated by a uridine-diphosphoglucuronosyltransferase (UGT1A1) to form an inactive metabolite. It was supposed that patients with the UGT1A1*28 polymorphism would have a greater prevalence of elevated pretreatment serum bilirubin levels and higher toxicity. The aim of our study was to investigate the predictive value of pre-treatment bilirubin levels in the development of severe diarrhea in solid tumor patients treated with irinotecan. The survey included 14 pediatric patients with refractory sarcomas treated with irinotecan (CPT-11). Patients were grouped based on the development of mild (G0-2) or severe (G3) gastrointestinal toxicity. The simple linear regression model and the non-parametric paired wilcoxon test were adopted for the analysis. p <0.05 was judged to indicate a significant difference. The results showed a significant increase in severity of diarrhea with increasing total pre-treatment bilirubin. therefore, we propose that pre-treatment bilirubin levels can predict gastrointestinal toxicity in pediatric cancer.

[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.

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.

Chronic idiopathic thrombocytopenic purpura in children: case reports of spontaneous recovery without splenectomy.

Children with chronic idiopathic thrombocytopenic purpura generally show a favorable outcome with a high spontaneous recovery rate even many years after the initial diagnosis. In this retrospective study, 5 out of 12 children with chronic ITP achieved a spontaneous recovery. A careful follow-up appears to be adequate for most of the patients, reserving splenectomy to the rare severely affected patients.

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.

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.

Second case of hepatoblastoma in a young patient with Simpson-Golabi-Behmel syndrome.

Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked overgrowth syndrome associated with multiple congenital anomalies caused by a mutant X-linked recessive trait. The authors report on a 14-month-old male patient affected by hepatoblastoma. As far as is known, this is the second patient reported with SGBS and hepatoblastoma. The observations emphasize that an increased risk of neoplasia in SGBS must be kept in mind, especially in young patients.

[Neutropenia in childhood]. / Neutropenia in età pediatrica.

The discovery of a neutropenia is a common problem in pediatric population. Some children with neutropenia have an uncomplicated clinical course and require no treatment. However, some patients are more severely affected and present a management challenge for the pediatrician and for the hematologist. The clinical approach to these children is directed primarily to understand the etiology of neutropenia in order to define a correct therapeutic behaviour, such as glucocorticoids, intravenous immunoglobulins or, more recently, rhG-CSF. In our article we have reviewed the etiology, the clinical course and the therapeutic options of constitutional and acquired neutropenias.