Noninvasive and invasive ventilation and enteral nutrition for ALS in Italy

: We performed a population‐based study to assess amyotrophic lateral sclerosis (ALS) survival after noninvasive ventilation (NIV), invasive ventilation (IV), and enteral nutrition (EN). Methods : We included patients diagnosed from 2000 to 2009 in Modena, where a prospective registry and a Motor Neuron Diseases Centre have been active since 2000. Results : Of the 193 incident cases, 47.7% received NIV, 24.3% received tracheostomy, and 49.2% received EN. A total of 10.4% of the patients refused NIV, 31.6% refused IV, and 8.7% refused EN. The median survival times after NIV, IV, and EN were 15, 19, and 9 months, respectively. Of the tracheostomized patients, 79.7% were discharged from the hospital; 73.0% were discharged to home. The median survival times for tracheostomized patients who were cared for at home and in nursing homes were 43 and 2 months, respectively. The multivariate analysis demonstrated that the place of discharge was the only independent prognostic factor after IV ( P  < 0.01). Conclusions : Service organizations may promote adherence to NIV, IV, EN, and influence postprocedure survival. These data may be useful in defining health plans regarding advanced ALS care and in patient counseling. Muscle Nerve 50: 508–516, 2014

Restrictive lung involvement in facioscapulohumeral muscular dystrophy

: Few studies have evaluated the frequency or predisposing factors for respiratory involvement in facioscapulohumeral muscular dystrophy type 1 (FSHD1) and type 2 (FSHD2). Methods : We performed a prospective cross‐sectional observational study of 61 genetically confirmed FSHD participants (53 FSHD1 and 8 FSHD2). Participants underwent bedside pulmonary function testing in sitting and supine positions, a standard clinical history and physical assessment, and manual muscle testing. Results : Restrictive respiratory involvement was suggested in 9.8% (95% confidence interval 2.4–17.3): 7.5% FSHD1 and 25.0% FSHD2 ( P  = 0.17). Participants with testing suggestive of restrictive lung involvement ( n  = 6) were more severely affected ( P  = 0.005), had weaker hip flexion ( P  = 0.0007), and were more likely to use a wheelchair ( P  = 0.01). Conclusions : Restrictive respiratory involvement should be considered in all moderate to severely affected FSHD patients with proximal lower extremity weakness. The higher frequency of restrictive lung disease in FSHD2 seen here requires confirmation in a larger cohort of FSHD2 patients. Muscle Nerve 50 : 739–743, 2014

Electrically induced torque decrease reflects more than muscle fatigue

The aim of the study was to compare the fatigue induced by different electrical stimulation (ES) protocols. The triceps surae muscle of 8 healthy subjects was fatigued with 4 protocols (30 H z −500 μs, 30 H z −1 ms, 100 H z −1 ms, and 100 H z −500 μs), composed of 60 trains (4 s on–6 s off), delivered at an intensity evoking 30% of maximal voluntary contraction (MVC). Fatigue was quantified by ES and MVC torque decreases. The amplitude of the twitch delivered at the intensity and pulse width used in each fatiguing protocol (twitch at I stim ) was analyzed. All parameters decreased significantly after all protocols. The ES torque decrease correlated positively with the twitch decrease elicited at I stim only for the 30‐H z protocols. Results show that, during the 100‐H z protocols, phenomena not related to the fatigue of the solicited motor units may occur, including changes in the excitability threshold of the axonal terminal branches. Muscle Nerve 50: 604–606, 2014

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DNS‐Class: immediate classification of IP flows using DNS

SUMMARY Nowadays we see a tremendous growth of the Internet, especially in terms of the amont of data being transmitted and new network protocols being introduced. This poses a challenge for network administrators, who need adequate tools for network management. Recent findings show that DNS can contribute valuable information on IP flows and improve traffic visibility in a computer network. In this paper, we apply these findings on DNS to propose a novel traffic classification algorithm with interesting features. We experimentally show that the information carried in domain names and port numbers is sufficient for immediate classification of a highly significant portion of the traffic. We present DNS‐Class: an innovative, fast and reliable flow‐based traffic classification algorithm, which on average yields 99.8 % of true positives and < 0.1% of false positives on real traffic traces. The algorithm can work as a major element of a modular system in a cascade architecture. Additionally, we provide an analysis on how various network protocols depend on DNS in terms of flows, packets and bytes. We release the complete source code implementing the presented system as open source. Copyright © 2014 John Wiley & Sons, Ltd.

Dynamic finite element formulation for Cosserat elastic plates

Summary A displacement and rotation‐based dynamic finite element formulation for Cosserat plates is discussed in detail in this paper. Special attention is given to the validation of the element through adequate benchmarks and patch tests. The choice of the interpolation functions and the order of integration of the stiffness and the mass matrices are extensively argued. The possibility of local system deficiencies is explored, and a shear locking investigation specifically elaborated for Cosserat plates is carried out. It is shown how the present formulation has interesting computational properties as compared to a classical continuum‐based formulation and how it can provide suitable results despite the use of reduced integration. An example of application of the finite element is given, in which the natural frequencies of a masonry panel modelled by means of discrete elements are computed and compared with the finite elements solution. Copyright © 2014 John Wiley & Sons, Ltd.

Towards the introduction of the ‘Immunoscore’ in the classification of malignant tumours

The American Joint Committee on Cancer/Union Internationale Contre le Cancer ( AJCC / UICC ) TNM staging system provides the most reliable guidelines for the routine prognostication and treatment of colorectal carcinoma. This traditional tumour staging summarizes data on tumour burden (T), the presence of cancer cells in draining and regional lymph nodes (N) and evidence for distant metastases (M). However, it is now recognized that the clinical outcome can vary significantly among patients within the same stage. The current classification provides limited prognostic information and does not predict response to therapy. Multiple ways to classify cancer and to distinguish different subtypes of colorectal cancer have been proposed, including morphology, cell origin, molecular pathways, mutation status and gene expression‐based stratification. These parameters rely on tumour‐cell characteristics. Extensive literature has investigated the host immune response against cancer and demonstrated the prognostic impact of the in situ immune cell infiltrate in tumours. A methodology named ‘Immunoscore’ has been defined to quantify the in situ immune infiltrate. In colorectal cancer, the Immunoscore may add to the significance of the current AJCC / UICC TNM classification, since it has been demonstrated to be a prognostic factor superior to the AJCC / UICC TNM classification. An international consortium has been initiated to validate and promote the Immunoscore in routine clinical settings. The results of this international consortium may result in the implementation of the Immunoscore as a new component for the classification of cancer, designated TNM ‐I ( TNM ‐Immune). © 2013 The Authors. Journal of Pathology published by John Wiley & Sons Ltd on behalf of Pathological Society of Great Britain and Ireland.

Resilience and psychosocial outcomes in parents of children with cancer

Background The psychosocial function of parents of children with cancer can impact the well‐being of the entire family. Resilience resources are likely related to psychosocial outcomes and may be amenable to intervention. We hypothesized that parents with lower resources would report worse outcomes. Methods In the “Understanding Resilience in Parents of Children with Cancer” study, comprehensive surveys were mailed to consecutive, English‐speaking parents of children with cancer who were treated at Seattle Children's Hospital and completed therapy between January 1, 2009 and December 31, 2010. Resilience resources were measured by the Connor‐Davidson Resilience Scale; outcome measures included psychological distress, health‐related behaviors, social and family function, and perceived communication with the medical team. Results Ninety‐six parents (86% of contactable) completed the survey. Compared to population norms, enrolled parents had lower resilience resources, higher psychological distress, and more commonly reported binge drinking. Conversely, they reported higher social support and family adaptability ( P  < 0.001–0.006). Lower resilience resources were associated with higher distress, lower social support, and lower family function ( P  < 0.001–0.007). Parents in the lowest quartile of resilience resources had higher odds of frequent sleep difficulties (OR 5.19, 95% CI 1.74,15.45), lower health satisfaction (OR 5.71, 95% CI 2.05,15.92), and decreased ability to express worries to the medical team (OR 4.00, 95% CI 1.43,11.18). Conclusions Parents of children with cancer are at risk for poor psychosocial outcomes and those with low resilience resources may be at greater risk. Interventions directed at promoting resilience resources may provide a novel and complimentary approach toward improving outcomes for families facing pediatric cancer. Pediatr Blood Cancer 2014;61:552–557. © 2013 Wiley Periodicals, Inc.

CLL/SLL diagnosed in an adolescent

Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is a disease of older adults. Pediatric CLL/SLL is vanishingly rare in the literature. We present a case of CLL/SLL diagnosed in a 17‐year‐old male. The pathologic findings of this case were those of classic CLL/SLL with an ATM deletion, a characteristic genetic abnormality in CLL/SLL. Management guidelines for CLL/SLL are tailored to older adults making determination of the optimal therapy for this patient a unique challenge. Pediatr Blood Cancer 2014;61:1107–1110. © 2013 Wiley Periodicals, Inc.

Effect of time from diagnosis to start of radiotherapy on children with diffuse intrinsic pontine glioma

Background Children with diffuse intrinsic pontine glioma (DIPG) continue to have poor outcomes, and radiotherapy (RT) is the only temporarily effective treatment. In this retrospective analysis, we studied the effect of time from diagnosis to start of RT on event‐free survival (EFS) and overall survival (OS) in children with DIPG. Methods Records of children ( n  = 95) with DIPG treated with RT at a single institution between April 1999 and September 2009 were analyzed. RT was delivered at doses of 54.0–55.8 Gy at 1.8 Gy per fraction, and children were followed prospectively. The effect of gender, race, interruption during treatment course, age at diagnosis, duration of symptoms prior to diagnosis, use of protocol‐based chemotherapy, and time from diagnosis to initiation of RT on EFS and OS was assessed by the Cox proportional hazards model. Results Time as a continuous variable from diagnosis to start of RT did not affect outcome. Time dichotomized to ≤14 days significantly affected OS (hazard ratio [HR] = 1.70, P  = 0.014) and race other than white or black affected EFS (HR = 2.32, P  = 0.017). The 95 patients had a 6‐month EFS and OS of 60 ± 5% and 94.7 ± 2.3%, respectively, and a 12‐month EFS and OS of 11.6 ± 3.1% and 49.5 ± 5%, respectively. Conclusions Time as a continuous variable did not affect OS or EFS in our cohort; however, children treated within 2 weeks of diagnosis had poor outcomes. Although rapid initiation of RT is desirable, our findings do not support intensive efforts aimed at shortening delays from diagnosis to start of RT. Pediatr Blood Cancer 2014;61:1180–1183. © 2014 Wiley Periodicals, Inc.