On the Stabilizing Effect of Aspartate and Glutamate and Its Counteraction by Common Denaturants.

By performing differential scanning calorimetry(DSC) measurements on RNase A, we studied the stabilization provided by the addition of potassium aspartate(KAsp) or potassium glutamate (KGlu) and found that it leads to a significant increase in the denaturation temperature of the protein. The stabilization proves to be mainly entropic in origin. A counteraction of the stabilization provided by KAsp or KGlu is obtained by adding common denaturants such as urea, guanidinium chloride, or guanidinium thiocyanate.

Destabilization of the D2 domain of Thermotoga maritima arginine binding protein induced by guanidinium thiocyanate and its counteraction by stabilizing agents.

D2 is a structural and cooperative domain of Thermotoga maritima Arginine Binding Protein, that possesses a remarkable conformational stability, with a denaturation temperature of 102.6°C, at pH 7.4.

Molecular Characterization of the Recombinant Ig1 Axl Receptor Domain: An Intriguing Bait for Screening in Drug Discovery.

Axl receptor tyrosine kinase and its ligand Gas6 regulate several biological processes and are involved in both the onset and progression of tumor malignancies and autoimmune diseases. Based on its key role in these settings, Axl is considered a promising target for the development of molecules with therapeutic and diagnostic purposes. In this paper, we describe the molecular characterization of the recombinant Ig1 domain of Axl (Ig1 Axl) and its biochemical properties.

Noonan syndrome-like disorder with loose anagen hair: a second case with neuroblastoma.

Noonan-like syndrome with loose anagen hair (NSLH), also known as Mazzanti syndrome, is a RASopathy characterized by craniofacial features resembling Noonan syndrome, cardiac defects, cognitive deficits and behavioral issues, reduced growth generally associated with GH deficit, darkly pigmented skin, and an unique combination of ectodermal anomalies. Virtually all cases of NSLH are caused by an invariant and functionally unique mutation in SHOC2 (c.4A>G, p.

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.

Speaking about emotional events in hospital: the role of health-care professionals in children emotional experiences.

This paper presents a qualitative study aimed at exploring the role of health-care professionals in hospitalized children's emotional experiences. A total of 27 children and adolescents from ages 6 to 15 years admitted to the Pediatric Hematology and Oncology ward of an Italian hospital participated in the study. Each participant was asked to talk about an emotional experience of happiness, anger, sadness and fear, felt in the presence of a doctor or nurse on the ward.

Hospitalized children's representations of their relationship with nurses and doctors.

This article reports an explorative study which aims to investigate hospitalized children's views of their relationships with nurses and doctors. Twenty-seven school-aged children and adolescents from 6 to 15 years old in the paediatric haematology and oncology ward of an Italian hospital participated in the study. Each participant was asked to draw him or herself with a doctor or nurse from the ward while they were doing something.

Atipical Kawasaki disease with coronary aneurysm in infant.

Kawasaki disease is an acute febrile disease of unknown etiology, characterized by systemic vascular inflammation involving the small and medium sized arteries, with a predilection for the coronary arteries. It represents the leading cause of acquired heart diseases in children in developed countries. Diagnosis, difficult because of the clinical characteristics of the disease with typical signs and symptoms appearing sequentially and not simultaneously, may be even more complicated in case of unusual presentation, leading to delay in recognition, particularly in infant in whom a higher incidence of coronary arteries aneurysms has been reported.

Clinical outcomes of rhabdomyosarcoma and Ewing's sarcoma of the head and neck in children.

Objective: To review our experience and critically evaluate treatment strategy and results in children with head and neck rhabdomyosarcoma and Ewing's sarcoma.

Methods: Retrospective charts review of children affected by non-orbital rhabdomyosarcoma or Ewing's sarcoma of the head and neck who were treated at our institution from January 1996 to August 2009.

Results: Seven consecutive children with head and neck rhabdomyosarcoma or Ewing's sarcoma were identified.

Improved survival of children with neuroblastoma between 1979 and 2005: a report of the Italian Neuroblastoma Registry.

Purpose: To describe treatment, clinical course, and survival of a cohort of Italian patients with neuroblastoma.

Patients And Methods: The study includes data from 2,216 children (age 0 to 14 years) diagnosed between 1979 and 2005. Overall survival (OS) was analyzed by clinical and biologic features at presentation and periods of diagnosis: 1979 to 1984, 1985 to 1991, 1992 to 1998, and 1999 to 2005.

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.

Clinical relevance of contrast-enhanced ultrasound in monitoring anti-angiogenic therapy of cancer: current status and perspectives.

Angiogenesis regulation is one of the newest fronts in the fight against cancer. Anti-angiogenic therapy is based on inhibiting factors required to solicit vessel formation thus cutting-off the tumor's supply of nutriments and oxygen. Initial vascular response is followed by formation of necrosis.

A case of colloid milium in patient with beta thalassaemia major.

Colloid milium (CM) is a rare cutaneous condition characterized by translucent papules occurring on sun-exposed regions including the face, neck and dorsal aspects of the hands and back. Clinically, there are two variants of CM: an adult-onset type and a juvenile form. The juvenile form is inherited and presents before puberty.

Molecular epidemiology of norovirus infections in sporadic cases of viral gastroenteritis among children in Northern Italy.

Surveillance of norovirus infections in sporadic cases of pediatric gastroenteritis admitted to a main hospital in Northern Italy during a full-year period (2002) showed that noroviruses (10.4%) were the second most common causative viral agent, following rotaviruses (21.1%), and noroviruses (81%) were mostly implicated in mixed infections.

Rotavirus and not age determines gastroenteritis severity in children: a hospital-based study.

Background: The severity of childhood gastroenteritis is generally believed to be age-related rather than aetiology-related. Rotavirus-induced gastroenteritis is more severe than gastroenteritis caused by other enteric pathogens and is also age-related. We thus addressed the question of whether the increased severity of rotavirus-induced gastroenteritis is related to age or to features intrinsic to the agent.

A monosymptomatic Melkersson-Rosenthal syndrome in an 8-year old boy.

Melkersson-Rosenthal Syndrome (MRS) is a systemic neuro-mucocutaneous granulomatous disease, characterized in its classical form by a triad of recurrent facial nerve paralysis, swelling of the lips and lingua plicata. However, this classical triad is rarely present, while the monosymptomatic or oligosymptomatic forms are more frequent. The presence of two or one of the manifestations mentioned above, with granulomatous cheilitis in the biopsy, is sufficient to make the diagnosis of monosymptomatic or oligosymptomatic form of MRS.

Topotecan plus ifosfamide in patients with platinum refractory advanced/metastatic non-small cell lung cancer: a phase II trial.

A number of second line treatments have been proposed in patients with advanced pretreated non-small cell lung cancer (NSCLC). However, either single agents or two or three drug combinations achieved very poor results with no superiority of any combination over monotherapy. We have treated 42 patients (30 males) affected by advanced/metastatic NSCLC progressing during front line cisplatin-based chemotherapy with a combination of topotecan (1.

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.

Epidemiological aspects of human rotavirus infection in children hospitalized with acute gastroenteritis in an area of northern Italy.

Human rotavirus (HRV) is recognized as the most common cause of severe gastroenteritis in children under 5 years of age. Due to the lack of recent reports about the surveillance of HRV infection in Italy, in this study we assessed the prevalence rate of HRV infection on 1,340 stool samples belonging to 1,264 pediatric patients hospitalized with acute gastroenteritis in the period January 2000--December 2002. The stool samples were submitted to virological investigations by electron microscopy (EM) and conventional cell culture, as well as from January 2002 by RT-PCR for norovirus detection.

Platinum Compound-Related Ototoxicity in Children: Long-Term Follow-Up Reveals Continuous Worsening of Hearing Loss.

Objectives: The purpose of this study was to evaluate the severity of hearing loss after cisplatin and/or carboplatin treatment in young children and to analyze its evolution and its relation to different therapy schedules.

Methods: One hundred twenty patients treated in the Pediatrics Department at the Institut Gustave-Roussy from 1987 to 1997 for neuroblastoma, osteosarcoma, hepatoblastoma, or germ cell tumors were analyzed. Median age at diagnosis was 2.

Platinum compound-related ototoxicity in children: long-term follow-up reveals continuous worsening of hearing loss.

Objectives: The purpose of this study was to evaluate the severity of hearing loss after cisplatin and/or carboplatin treatment in young children and to analyze its evolution and its relation to different therapy schedules.

Methods: One hundred twenty patients treated in the Pediatrics Department at the Institut Gustave-Roussy from 1987 to 1997 for neuroblastoma, osteosarcoma, hepatoblastoma, or germ cell tumors were analyzed. Median age at diagnosis was 2.

[Early discharge project appropriate for the healthy newborn].

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.

[Vertical transmission of the hepatitis C virus].

The incidence of hepatitis C in childhood is approximately 0.4%. The mode of transmission can be parenteral, sexual, occupational and also vertical.