Excellent outcome with reduced treatment for infants with disseminated neuroblastoma without MYCN gene amplification.

Purpose: On the assumption that most infants with disseminated neuroblastoma without MYCN amplification (MYCNA) have a favorable prognosis, two concomitant prospective trials were started in which chemotherapy was limited to patients presenting life- or organ-threatening symptoms or overt metastases to skeleton, lung, or CNS. Surgery was to be performed only in the absence of surgical risk factors.

Patients And Methods: One hundred seventy infants with disseminated neuroblastoma without MYCNA, diagnosed between June 1999 and June 2004 in nine European countries were eligible for either of the two studies.

Diagnostic and prognostic markers in infants with disseminated neuroblastoma: a retrospective analysis from the Italian Cooperative Group for Neuroblastoma.

Background: One fourth of infants with disseminated neuroblastoma experience unfavorable outcome. Treatment strategies vary and are based on clinical characteristics at diagnosis which lead to the definition of stage 4 or 4s. To identify the distribution and effect of different prognostic factors, a series of such infants diagnosed in Italy between 1991-1999 was reviewed.

Neurotoxicity during ifosfamide treatment in children.

Background: Neurotoxicity has been reported in about 5% of children treated with ifosfamide for tumors not involving the central nervous system. The entity of ifosfamide neurotoxicity can be of different degree, from very light and transient to fatal.

Case Reports: All cases of ifosfamide neurotoxicity recorded at the Pediatric Hematology and Oncology Unit in the 15-year period between 1989 and 2003 are reported.

Beneficial effects of rutin and L-arginine coadministration in a rat model of liver ischemia-reperfusion injury.

Reperfusion following liver ischemia results in oxidative stress leading to liver injury. The aim of this study was to investigate the combined effects of two antioxidant agents, rutin and L-arginine, in rat liver ischemia-reperfusion (I/R). Male Wistar rats were divided into five groups: 1) sham operated, 2) I/R, 3) I/R+rutin, 4) I/R+L-arginine, and 5) I/R+rutin+L-arginine.

Marked calpastatin (CAST) depletion in Alzheimer's disease accelerates cytoskeleton disruption and neurodegeneration: neuroprotection by CAST overexpression.

Increased activity of calpains is implicated in synaptic dysfunction and neurodegeneration in Alzheimer's disease (AD). The molecular mechanisms responsible for increased calpain activity in AD are not known. Here, we demonstrate that disease progression is propelled by a marked depletion of the endogenous calpain inhibitor, calpastatin (CAST), from AD neurons, which is mediated by caspase-1, caspase-3, and calpains.

Metachronous liver metastases and resectability: Fong's score and laparoscopic evaluation.

Background And Aims: The aim of this retrospective study was to establish whether Fong's risk score can predict rate of resectability and whether laparoscopic exploration with ultrasonography can reduce the number of useless laparotomies to any extent.

Material And Methods: Fong's score was calculated for each of the 43 potential resectable patients. We analysed: the relation between score and resectability; the probability of unnecessary laparotomy with respect to each level of score; and which of the five Fong parameters was the most indicative of non-resectability.

Idiopathic myenteric ganglionitis underlying acute 'dramatic' intestinal pseudoobstruction: report of an exceptional case.

Inflammation of the myenteric plexus of the gastrointestinal tract is a very rare pathological condition, with few reports in the medical literature. This pathological condition causes atonic gut motor dysfunction and is principally secondary to other diseases, being reported nearly solely as a paraneoplastic phenomenon in neuroendocrine lung tumors, including small cell carcinomas or neuroblastomas. In addition it can also be associated with disorders of the central nervous system, although it has rarely been described in Chagas disease.

Involvement of GTA protein NC2beta in neuroblastoma pathogenesis suggests that it physiologically participates in the regulation of cell proliferation.

Background: The General Transcription Apparatus (GTA) comprises more than one hundred proteins, including RNA Polymerases, GTFs, TAFs, Mediator, and cofactors such as heterodimeric NC2. This complexity contrasts with the simple mechanical role that these proteins are believed to perform and suggests a still uncharacterized participation to important biological functions, such as the control of cell proliferation.

Results: To verify our hypothesis, we analyzed the involvement in neuroblastoma (NB) pathogenesis of GTA genes localized at 1p, one of NB critical regions: through RT-PCR of fifty eight NB biopsies, we demonstrated the statistically significant reduction of the mRNA for NC2beta (localized at 1p22.

Down syndrome fibroblast model of Alzheimer-related endosome pathology: accelerated endocytosis promotes late endocytic defects.

Endocytic dysfunction is an early pathological change in Alzheimer's disease (AD) and Down's syndrome (DS). Using primary fibroblasts from DS individuals, we explored the interactions among endocytic compartments that are altered in AD and assessed their functional consequences in AD pathogenesis. We found that, like neurons in both AD and DS brains, DS fibroblasts exhibit increased endocytic uptake, fusion, and recycling, and trafficking of lysosomal hydrolases to rab5-positive early endosomes.

[Carcinoma of the Bellini collecting duct in paediatric patients: a case report and review of the literature].

Collecting duct carcinoma is an uncommon variant of renal cell carcinoma that usually occurs at an earlier age compared to conventional renal cell carcinoma. It is characterised by an aggressive, often fatal, course. Renal cell carcinoma rarely occurs in paediatric patients, and is almost always in association with specific genetic alterations; the most common histotypes are the clear cell and chromophobe cell variants.

In vivo MRI identifies cholinergic circuitry deficits in a Down syndrome model.

In vivo quantitative magnetic resonance imaging (MRI) was employed to detect brain pathology and map its distribution within control, disomic mice (2N) and in Ts65Dn and Ts1Cje trisomy mice with features of human Down syndrome (DS). In Ts65Dn, but not Ts1Cje mice, transverse proton spin-spin (T(2)) relaxation time was selectively reduced in the medial septal nucleus (MSN) and in brain regions that receive cholinergic innervation from the MSN, including the hippocampus, cingulate cortex, and retrosplenial cortex. Basal forebrain cholinergic neurons (BFCNs) in the MSN, identified by choline acetyltransferase (ChAT) and nerve growth factor receptors p75(NTR) and TrkA immunolabeling were reduced in Ts65Dn brains and in situ acetylcholinesterase (AChE) activity was depleted distally along projecting cholinergic fibers, and selectively on pre- and postsynaptic profiles in these target areas.

The surgeon as a prognostic factor in the surgical treatment of rectal cancer.

Over the past 2 decades the surgeon and the hospital where he or she works have been considered to play an important role in the prognosis of the rectal cancer patients. The rate of sphincter-sparing rectal resection, local recurrence and survival are the factors more frequently utilized in the literature to evaluate if surgeons are able to affect the natural history of the rectal cancer. The quantitative aspect, high volume of the surgeon, is not enough but in order to achieve better results in the treatment of rectal cancer a specific interest in colorectal surgery is more important.

Evaluation and art therapy treatment of the burnout syndrome in oncology units.

We undertook a pilot study to evaluate and potentially reduce the level of burnout in the operators of two oncology centers. The study included 65 doctors and nurses of an adult (Group A) and a pediatric oncology unit (Group B). We used the Maslach Burnout Inventory to estimate the level of burnout obtained in three dimensions: emotional exhaustion, distancing (cognitive and emotional) and reduced personal achievement.

Methodologies for the treatment of acute and chronic nononcologic pain in children.

Pain represents the most feared symptom of illness but for many years it has been underestimated in children. Only recently has analgesic treatment become an integral part in the assistance of ill children with acute pain, such as postoperative pain, and with chronic pain. Postoperative pain is a complication of surgery: postoperative pain prevention should replace the current theory and practice of postoperative treatment through the creation of ''pain services'' and adequate pain prevention planning.

Rab5 mediates an amyloid precursor protein signaling pathway that leads to apoptosis.

Alzheimer's disease (AD) involves activation of apoptotic pathways that may be regulated through signaling cascades initiated by the amyloid precursor protein (APP). Enlarged endosomes have been observed in postmortem AD brains at very early stages of the disease. We show here that exogenous expression of a familial AD (FAD) mutant of APP or of the APP binding protein APP-BP1 in neurons causes enlargement of early endosomes, increased receptor-mediated endocytosis via a pathway dependent on APP-BP1 binding to APP, and apoptosis.

Anastomotic dehiscence after gastrectomy for cancer. Personal series.

Background/aims: Nowadays the risk of anastomotic dehiscence after gastrectomy still exists. So the aim of this study was to analyze our experience regarding these anastomoses.

Methodology: In our Surgical Unit, which is located in the Department of Surgical Sciences, Organ Transplantation and Advanced Technologies of the University of Catania, from January 1st 1985 to December 31st 2000, 249 patients underwent surgery for gastric cancer.

Rutin in rat liver ischemia/reperfusion injury: effect on DDAH/NOS pathway.

Nitric oxide (NO) plays a key role in the relationship between microcirculatory disorders and I/R injuries. Our results demonstrated a significant modification in the hepatic function of I/R rats compared with the control group; treatment with rutin reported hepatic damage markers to control value. Levels of plasmatic and hepatic thiol groups decreased in the I/R untreated group, and this decrease was inhibited by rutin treatment.

Skeletal involvement in infants with neuroblastoma a quality control attempt.

Aims And Background: In 1994, French authors hypothesized that positive skeletal mlBG spots in infants with stage 4 neuroblastoma were not prognostically unfavorable unless associated to abnormal standard X-ray or CT findings. In 1999, the European Infant Neuroblastoma Study adopted this definition, indeed reducing the number of patients candidate to chemotherapy. Such an approach requires high quality scans and standardized procedures.

Cancer of the sigmoid colon: left hemicolectomy or sigmoidectomy?

Left hemicolectomy is the ideal treatment of sigmoid cancer, but sometimes sigmoidectomy is a safe treatment. We radically treated 102 patients affected by sigmoid cancer: 83 were gross sigmoid cancer treated by left hemicolectomy, 19 were residual cancer after endoscopic polypectomy; of these, 4 underwent left hemicolectomy and 15 underwent sigmoidectomy. No recurrence was observed in the 15 patients treated by sigmoidectomy.

Mature and immature teratomas: results of the first paediatric Italian study.

Teratoma is the most common germ cell tumour in childhood; mature (MT) and immature teratomas (IT) are benign tumours, but if they recur, they can be in some cases malignant. The aim of this paper is to evaluate Italian patients with MT and IT enrolled from 1991 to 2001, in a prospective multicentric study. One hundred and eighty-three patients, observed in 15 Italian Centers of Paediatric Oncology and three Paediatric Surgical Units were enrolled.

[Myasthenia gravis and intestinal resection: is dehiscence likely to occur?].

Experimental and clinical data have proved that anticholinesterase drugs are responsible for vigorous peristaltic contractions and for an increase of the intraluminal pressure, because they determine muscarinic effects on the smooth muscle of the intestine both in small and large intestine. Therefore, a greater incidence of intestinal anastomotic disruption has been supposed when anticholinesterases are used both in the early postoperative period, to reverse curarization, and in myasthenic patients. The authors report a case of a patient with myasthenia gravis who received maximal doses of pyridostigmine and underwent left hemicolectomy and small intestine resection and afterwards total colectomy in order to treat a sigmoid perforated diverticulitis.