Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

Introduction: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations.

Methods: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects.

Clinical history of cancer-associated splanchnic vein thrombosis.

Background: Cancer represents a risk factor for splanchnic vein thrombosis (SVT) and usual site venous thromboembolism (VTE).

Objectives: To compare characteristics and outcomes of patients with cancer-associated SVT and usual site VTE.

Patients/methods: Patients with solid cancer and SVT were enrolled in an international, prospective registry between May 2008 and January 2012.

Clinical history and antithrombotic treatment of incidentally detected splanchnic vein thrombosis: a multicentre, international prospective registry.

Background: Little information is available about the clinical history of patients with incidentally detected splanchnic vein thrombosis and its therapeutic management remains controversial. The aim of this study was to assess the risk factors, therapeutic strategies, and long-term outcomes of incidentally detected splanchnic vein thrombosis.

Methods: We analysed data from patients with incidentally detected splanchnic vein thrombosis who were enrolled in an international, multicentre, prospective cohort study of splanchnic vein thrombosis between 2008 and 2012.

Long-term Clinical Outcomes of Splanchnic Vein Thrombosis: Results of an International Registry.

Importance: Little information is available on the long-term clinical outcome of patients with splanchnic vein thrombosis (SVT).

Objective: To assess the incidence rates of bleeding, thrombotic events, and mortality in a large international cohort of patients with SVT.

Design, Setting, And Participants: A prospective cohort study was conducted beginning May 2, 2008, and completed January 30, 2014, at hospital-based centers specialized in the management of thromboembolic disorders; a 2-year follow-up period was completed January 30, 2014, and data analysis was conducted from July 1, 2014, to February 28, 2015.

Portopulmonary hypertension.

Portopulmonary hypertension (PPHT) is a respiratory complication of portal hypertension, defined as an increase in mean pulmonary artery pressure (PAP) of > 25 mmHg with an increase in pulmonary vascular resistance of > 240 dyn.s/cm(-5) and a normal pulmonary capillary wedge pressure ( < 15 mmHg), which often occurs in subjects with liver cirrhosis. Histopathological features of PPHT are endothelial and smooth-muscle cell proliferation and fibrosis leading to luminal obstruction in the resistance arteries.

Refined cytogenetic-risk categorization for overall and leukemia-free survival in primary myelofibrosis: a single center study of 433 patients.

We have previously identified sole +9, 13q- or 20q-, as 'favorable' and sole +8 or complex karyotype as 'unfavorable' cytogenetic abnormalities in primary myelofibrosis (PMF). In this study of 433 PMF patients, we describe additional sole abnormalities with favorable (chromosome 1 translocations/duplications) or unfavorable (-7/7q-) prognosis and also show that other sole or two abnormalities that do not include i(17q), -5/5q-, 12p-, inv(3) or 11q23 rearrangement are prognostically aligned with normal karyotype, which is prognostically favorable. These findings were incorporated into a refined two-tired cytogenetic-risk stratification: unfavorable and favorable karyotype.

Residual vein thrombosis and D-dimer for optimizing duration of anticoagulation in idiopathic deep vein thrombosis.

Long-term anticoagulant treatment is highly effective in preventing recurrent Venous Thrombo-Embolism (VTE) in patients with idiopathic Deep Vein Thrombosis (DVT) of the lower limbs, though associated with an increased risk for major bleeding that may offset the benefits of anticoagulation. Accordingly to recent guidelines, patients with idiopathic DVT should be treated for at least 3 months and then should be evaluated for the risk-benefit ratio of long-term therapy. However, such 'time for decision' is often unclear and the optimal duration of VKA remains debatable.

Chromosome 1 abnormalities in myeloid malignancies: a literature survey and karyotype-phenotype associations.

Chromosome 1 is the largest human chromosome and contains over 1600 known genes and 1000 novel coding sequences or transcripts. It is, therefore, not surprising that recurrent chromosome 1 abnormalities are regularly encountered in both neoplastic and non-neoplastic medical conditions. The current review is focused on myeloid malignancies where we summarize the relevant published literature and discuss specific karyotype-phenotype associations.

How should we determine length of anticoagulation after proximal deep vein thrombosis of the lower limbs?

The current approach for deciding the duration of vitamin K antagonist (VKA) treatment after an episode of venous thrombo-embolism (VTE) is mainly based on the characteristic of the index event (3 months or longer in case of unknown/persistent risk factors, 3 months or less in case of removable causes). However, the length of anticoagulation should be tailored on the patient's risk for recurrent thrombosis as well as for bleeding, but such 'time for decision' is often unclear and the optimal duration of VKA remains debatable. The presence of persistent residual vein thrombosis and increased D-dimer levels after stopping therapy are predictors for recurrent deep vein thrombosis (DVT).

Vesicoureteral reflux in young patients: comparison of voiding color Doppler US with echo enhancement versus voiding cystourethrography for diagnosis or exclusion.

We assessed the accuracy of voiding color Doppler ultrasonography (US) with echo enhancement for diagnosis or exclusion of vesicoureteral reflux (VUR) versus voiding cystourethrography (VCUG) and evaluated patient tolerance of the echo-enhancing agent. One hundred twenty-two patients (ages range, 1 month to 17 years) with 244 ureterorenal units underwent voiding color Doppler US with echo enhancement, which was followed by VCUG on the same day. After US of the urinary tract, the bladder was filled with saline solution via catheter.

Continuous intravenous infusion of dipyridamole as adjunctive therapy in the treatment of thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is an uncommon hematologic thrombotic disorder characterized by fever, hemorrhagic and neurologic signs. The advent of plasma exchange has dramatically improved the prognosis of this disease, which was once inevitably fatal. However, mortality rates remain significant.

[Otoneurological findings in a case of congenital central hypoventilation syndrome (Ondine's curse)].

The authors report a case of congenital central hypoventilation syndrome (CCHS) studied from the otoneurological point of view. Emphasis is placed on the numerous alterations in the electronystagmograph and auditory potentials. The results obtained confirm the hypothesis that such patients are subject to CNS alterations, suggesting that the disease pathogenesis derives from an alteration in the mechanism of central chemoreceptor stimuli integration.

[Residual hyperinsulinism after glucose oral load in obese subjects with normal glucose tolerance].

Forty-five NGT obese subjects were submitted to OGTT with IRI and CPR determinations and to euglycemic hyperinsulinemic clamp, and divided into two groups: A) those with return of insulinemia toward basal values, and B) those with residual hyperinsulinism, in order to evaluate possible differences in insulin secretion and/or insulin action among them. Our data show the younger age of those with residual hyperinsulinism, that also seems related to insulin secretion, represented by IRI and CPR basal values, but not to insulin resistance parameters.