Leadless pacemaker: State of the art and incoming developments to broaden indications

Theleadless pacemaker (LLPM) therapy has been developed in recent years to overcome the transvenous lead and device pocket-related complications. The LLPMs now available are self-contained right ventricular pacemakers and are limited to single-chamber ventricular pacing modality. This literature review deals with the current status of LLPM technology and current areas of clinical applicability. The safety and efficacy outcomes published from randomized clinical trials and real world registries are analyzed and compared with historical conventional transvenous pacemaker data. Furthermore, new pacing modalities and future perspectives to broaden the clinical use and cover most of pacing indications are discussed. Due to the overall safe and effective profile in the short term and intermediate term, also in fragile patients, the LLPM use is constantly growing in daily clinical practice. Actually, it can be considered a landmark innovation, through which a new era of cardiac pacing has begun.     

Complete genome sequence of a novel hypovirus infecting Phomopsis longicolla

The complete nucleotide sequence and genome organization of a hypovirus from the isolate ME711 of Phomopsis longicolla was determined and compared to sequences of members of the family Hypoviridae. The genome of the hypovirus, tentatively named Phomopsis longicolla hypovirus 1 (PlHV1-ME711), was determined to be 9760 nucleotides long, excluding the 3’ poly (A) tail. The genome contains a single large open reading frame (ORF) encoding a polyprotein designated as P307. Its genomic organization is typical of members of the proposed genus Betahypovirus (Yaegashi et al. in Virus Res 165:143–50, 2012).     

Dendritic fibromyxolipoma: A variant of spindle cell lipoma with extensive myxoid change,with cytogenetic evidence

Dendritic fibromyxolipoma (DFML), a rare, recently described distinct benign soft tissue tumor, has many clinicopathological features reminiscent of spindle cell lipoma and solitary fibrous tumor with myxoid change. It is distinguished histologically from both entities by the presence of spindle and stellate cells with dendritic cytoplasmic prolongations, prominent myxoid stroma with abundant keloidal collagen and occasional small plexiform vascular proliferation. We describe a case of histologically confirmed DFML of the left shoulder in a 67‐year‐old male, in which subsequent cytogenetic analysis revealed deletion involving 13q14.3 region in all the tumor cells, typically detected in spindle cell lipoma. In the presence of many clinicopathological similarities between DFML and spindle cell lipoma including chromosomal abnormalities, we postulate that DFML is merely a rare variant of spindle cell lipoma with extensive myxoid degeneration, and may not be considered as a separate entity. The possible differential diagnosis and their distinguishing features are briefly discussed.     

Long-term effectiveness and safety of interleukin-1 receptor antagonist (anakinra) in Schnitzler's syndrome: A french multicenter study

The aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive this treatment. Forty-two patients were included in the study, 29 of whom received IL1Ra. The mean age at disease onset was 59.9 years. Disease manifestations included urticaria (100%), fever (76%), bone/joint pain (86%), bone lesions (76%), anemia (67%), and weight loss (60%). The monoclonal gammopathy was overwhelmingly IgM kappa (83%). The mean follow-up was 9.5 years (range: 1.6-35). Two patients developed Waldenström's macroglobulinemia and one developed AA amyloidosis. All of the 29 patients who received IL1Ra responded dramatically. After a median follow-up of 36 months (range: 2-79), the effectiveness remained unchanged. All patients remained on anti-IL-1 therapy. Twenty-four patients (83%) went into complete remission and five (17%) into partial remission. Three patients experienced grade 3-4 neutropenia. Six patients developed severe infections. No lymphoproliferative diseases occurred while on IL1Ra. When last seen, all patients without anakinra had an active disease with variable impact on their quality of life. Their median corticosteroids dosage was 6 mg/d (range: 5-25). IL1Ra is effective in SchS, with a sharp corticosteroid-sparing effect. Treatment failures should lead to reconsider the diagnosis. Long-term follow-up revealed no loss of effectiveness and a favorable tolerance profile. The long-term effects on the risk of hemopathy remain unknown.     

Role of p53 Arg72Pro SNP in the pathogenesis of gliomas and its effect on serum level of p53

Gliomas account for almost 80 % of primary malignant brain tumors and are a major cause of morbidity and mortality more than any other tumor. The p53 gene is a tumor suppressor gene that plays a role in genomic stability, cell cycle control, and DNA repair after damage and apoptosis. Malfunction of the p53 pathway is a universal hallmark of human tumors with p53 codon 72 polymorphism reported to affect regulatory networks central to glioma development. The aim of work was to investigate the role of p53 Arg72Pro single nucleotide polymorphism (SNP) in the pathogenesis of gliomas and its effect on the serum level of p53. Forty-five glioma patients and 50 control subjects were included for whom genotyping for p53 Arg72Pro SNP by polymerase chain reaction-restriction fragment length polymorphism as well as measurement of serum p53 level were done. No statistically significant difference was observed in genotype distribution or allele frequency between cases and control and nor was there a significant difference in serum p53 level. In conclusion, in our work, no association was detected between p53 Arg72Pro SNP and the pathogenesis of gliomas, and neither was there an observed effect for the different genotypes on serum p53 levels.     

CD138 mRNA expression from peripheral blood of patients with follicular and diffuse large B cell lymphoma: relation to disease progression and treatment response

Syndecans are among those transmembrane PGs which act via their heparan sulphate chains as receptors for different matrix elements (e.g. collagen I–III, fibronectin, thrombospondin, tenascin) and co-receptors for growth factors (e.g. bFGF, aFGF, GM-CSF, IL-3, IFNg). We hypothesized that there is a positive relationship between the expression of syndecan-1 (CD138) and treatment response in non-Hodgkin’s lymphoma. To identify the expression of syndecan-1 (CD138) in cases of non-Hodgkin’s lymphoma and to correlate its expression with treatment response and disease stage, this study was carried out as a cross-sectional study and included 30 patients with non-Hodgkin’s lymphomas attending Suez Canal University Hospital; diagnosis of patients was made by routine histological and immunohistochemical examination. CD138 mRNA expression was assessed by TaqMan technique using real-time PCR method. There was increased expression of CD138 mRNA in patients with follicular lymphoma than in patients with diffuse large B cell lymphoma (6.25 versus 3.46, respectively) (p?≤?0.05). Syndecan-1 (CD138) mRNA expression from peripheral blood may be a useful marker for follicular lymphoma and can be used as a marker of treatment response in patients with follicular and diffuse large B cell lymphoma.     

Development of a selective and sensitive colour reagent for gold and silver ions and its application to desktop scanner analysis

Desktop scanners can be favorable alternatives to sophisticated spectrophotometers for the assessment of analytes in complex real samples. Distinctively, our method has been thoroughly investigated, optimized, validated and successfully applied to the assessment of silver and gold in complex real samples, applying syringal rhodanine (SR) as a novel specifically tailored chromogenic reagent and using a desktop scanner as a versatile sensor. Maximum colour absorbance was obtained in the presence of cetylpyridinium chloride (CPC) and cetyltrimethylammonium chloride (CTAC) for silver and gold chelates, respectively. For each metal ion, two ternary complexes were formed depending on the SR concentration with stoichiometries of 1 : 1 : 1 and 1 : 2 : 3 (Ag–SR–CPC) and 1 : 2 : 3 and 1 : 3 : 4 (Au–SR–CTAC), respectively. The methods adhered to Beer''s law for 0.15–2.5 and 0.15–2.25 μg mL⁻¹ with detection limits of 0.0089 and 0.0163 μg mL⁻¹ for silver and gold, respectively. The molar absorptivities were 3.63 × 10⁴ and 6.15 × 10⁴ L mol⁻¹ cm⁻¹ at 550 nm and 554 nm, with Sandell''s sensitivity indexes of 0.0029 and 0.0032 μg cm⁻², respectively. The method was successfully applied to the assessment of silver and gold in a wide range of complex environmental samples.

Desktop scanners can be favorable alternatives to sophisticated spectrophotometers for the assessment of analytes in complex real samples.     

PHACES syndrome associated with carcinoid endobronchial tumor

PHACES syndrome consists of the constellation of manifestations including posterior fossa anomalies of the brain (most commonly Dandy-Walker malformations), hemangiomas of the face and scalp, arterial abnormalities, cardiac defects, eye anomalies and sternal defects. We present a case with a possible PHACES syndrome including sternal cleft and supraumbilical raphé, precordial skin tag, persistent left superior vena cava and subtle narrowing of the aorta with an endobronchial carcinoid tumor. All these anomalies were discovered on chest multi-detector CT. This is a unique case of PHACES syndrome associated with carcinoid tumor. Review of the literature revealed 3 cases of PHACES syndrome with glial tumor. The authors tried to find the relationship between PHACES syndrome and carcinoid tumors or gliomas, which all derive from the neural crest cells.