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At an early stage, esophageal cancer can be removed surgically or endoscopically. T1a tumors are removed by endoscopic resection, whereas T1b tumors are preferably surgically removed (esophagectomy) due to possible lymph node involvement. However, the diagnostic tools (endoscopic ultrasonography (EUS) and CT/PET-CT) used to distinguish between T1a and T1b tumors and to detect malignant lymph nodes are imprecise. This study aimed to review the accuracy of preoperative locoregional staging in superficial esophageal cancer in a Danish setting. This was a retrospective, multicenter study including patients with a cT1 esophageal tumor, both adenocarcinoma and squamous cell carcinoma. Patients receiving neoadjuvant treatment were excluded. Results from the preoperative diagnostics tools were compared with the pathology report to determine the diagnostic accuracy. Sixty-eight patients were included, 34 patients underwent esophagectomy, and 34 patients underwent endoscopic resection. The positive predictive value (PPV) of PET-CT (or CT) (when staged as a T1 tumor) was 77% (95%CI: 0.59–0.88), and the PPV of EUS (when staged as a T1a or T1b tumor) was 52% (95%CI: 0.32–0.72) and 60% (95%CI: 0.36–0.82), respectively. Six patients had malignant lymph nodes in the surgical specimen; none of them was identified before surgery. In conclusion, neither CT/PET-CT nor EUS is a precise tool to distinguish mucosal from submucosal esophageal tumors. Furthermore, in superficial malignant lesions, lymph node involvement is rare, but when present, it is rarely detected before surgery. To improve staging accuracy, all patients with cT1 tumors could be referred for a diagnostic endoscopic resection prior to possible other treatments. [ABSTRACT FROM AUTHOR]

Objective: To create a language independent version of the Listening in Spatialised Noise – Sentences test (LiSN-S) and evaluate it in an English-speaking population. Design: Test development and normative data collection. LiSN-Universal (LiSN-U) targets consisted of CVCV pseudo-words (e.g. /mupa/). Two looped distracter tracks consisted of CVCVCVCV pseudo-words. The listener's task was to repeat back the target pseudo-words. Stimuli were presented over headphones using an iPad. Speech reception thresholds were measured adaptively. In the co-located condition all stimuli came from directly in front. In the spatially-separated condition the distracters emanated from +90° and −90° azimuth. Perceived location was manipulated using head-related transfer functions. Spatial advantage was calculated as the difference in dB between the co-located and spatially separated conditions. Study samples: Stimulus intelligibility data were collected from 20 adults. Normative data were collected from native English speakers (23 adults and 127 children). Results: Children's spatially separated, co-located, and spatial advantage results improved significantly with age. Spatial advantage was 4–6 dB larger in the LiSN-U than LiSN-S depending on age group. Conclusion: Whereas additional research in non-native English populations is required, the LiSN-U appears to be an effective tool for measuring spatial processing ability. [ABSTRACT FROM AUTHOR]

Calreticulin (CALR) mutations are detected in the majority of JAK2 wild type patients with essential thrombocythemia (ET). Unlike JAK2V617F and MPL point mutations, CALR mutations are highly heterogeneous, with several types of indels being reported so far. CAL2 is a monoclonal antibody specifically recognizing the C-neoterminal peptide derived from all the frameshift mutations of CALR. We retrospectively analysed 172 ET patients diagnosed at our Institution from 1980 to 2015. In JAK2V617F- and MPLW515K/L-wild type patients CALR mutations were searched on peripheral blood and CAL2 immunostaining was performed on bone marrow. In addition, bone marrow biopsies were histologically reviewed for megakaryocytic features. Thirty-one patients (18%) were CALR-mutated. Concordance between molecular and immunohistological detection of CALR mutations was near complete, albeit a single patient was found to be positive by molecular tests only. Two patterns were defined in CAL2-positive bone marrow samples, characterized by staining of almost only megakaryocytes (pattern A: 41%) or staining of megakaryocytes and ≥ 2% small non megakaryocytic elements (pattern B: 59%), at least partially being myeloid precursors. Pattern B biopsies had higher cellularity and number of megakaryocytes compared to pattern A samples. In this series, CAL2 allowed rapid and cost-efficient identification of CALR-mutated ET patients. The biological significance of different staining pattern should be confirmed in wider and independent series. [ABSTRACT FROM AUTHOR]

Abstract: The detection of specific factors of the developmental dyslexia at an early stage, and the identification of the role of those factors responsible for its manifestation, is a fundamental area of study on dyslexia in the recent literature. The objective of the present study is to clarify that dysfunctions in the following specific domains contribute in a causal model to the occurrence of dyslexia at an early stage: phonological awareness, psychomotor ability (body shape, spatio-temporal orientation, grapho-motor ability and laterality), perception, memory, attention, prereading and prewriting skills. The results of three studies, – carried out in Greece – which revealed the above factors as main predictors of the early onset of Specific Developmental Dyslexia (SDD) and confirmed the importance of intervention methods to it, led us to the construction of the proposed, causal model. The findings of these three studies converge on the perspective that the understanding, diagnosis and treatment of dysfunctions in the above domains, from preschool age, enable the early and reliable prevention of future difficulties in the learning process of children. [Copyright &y& Elsevier]

Background: Capsule endoscopy (CE) retention remains a concern in patients with suspected or known Crohn's Disease (CD).Aim: The aim of this study was to evaluate the ability of a patency capsule (PC) to establish functional patency in pediatric patients with suspected or known, symptomatic IBD.Methods: A prospective, single center study evaluating the impact of CE on CD management used PC to qualify patients for CE. Patients excreting an intact PC, usually within 40 h of ingestion, were able to undergo standard video CE. Excretion time, structural integrity and patient safety were evaluated.Results: Eighteen patients (10-16 years of age; 9 male; 5 known CD, 3 indeterminate colitis, 1 ulcerative colitis, 9 suspected CD) ingested the PC. Fifteen patients excreted intact PC (mean 34.5 h), 12 patients within 40 h (range 9-60 h). Sixteen (89%) underwent subsequent CE successfully. CD was eventually diagnosed in all patients having PC transit ≥40 h, whereas CD was the diagnosis in 9/12 (75%) in those patients who passed the PC within 40 h. The mean time of passage for an intact PC was 34.7 h, the longest 60 h. There were no capsule retentions or adverse events.Conclusions: The PC appears to be a useful screening tool for functional patency of the small bowel in suspected or known pediatric CD. Delayed passage of an intact PC requires careful interpretation. [ABSTRACT FROM AUTHOR]

Mucopolysaccharidoses (MPS) are a group of inherited, progressive, metabolic diseases, caused by the deficiency of one of the enzymes involved in the degradation of glycosaminoglycans (GAGs). The disease is usually fatal, with the life span of most untreated MPS patients being between one and two decades. In this report, on the basis of scanning electron microscopy (SEM) studies, we demonstrate that, besides the many other symptoms of MPS, there are characteristic abnormalities in the hair morphology of patients suffering from some types of this disease (MPS I, MPS II, MPS IIIA, MPS IIIB), but not from other types (MPS IVA, MPS IVB, MPS VI), where the changes are minor, if any. Different GAGs accumulate in the tissues of patients suffering from the various MPS types, and analysis of the disease types in which severe hair abnormalities occur or not could suggest that the accumulation of heparan sulfate, rather than dermatan sulfate or keratan sufate, may be responsible for the major changes in hair morphology. Considerable abnormalities in hair morphology occur in patients suffering from MPS I, MPS II, MPS IIIA, and MPS IIIB, but not in patients suffering from MPS IVA, MPS IVB, and MPS VI; this feature might potentially be used as an additional test for the assessment of the efficacy of treatments for MPS patients (types I, II, IIIA, and IIIB). [ABSTRACT FROM AUTHOR]