Length-weight relationships and relative condition factor of Parapenaeopsis sculptilis (Heller, 1862) from the coastal waters of Perak, Peninsular Malaysia

Length-weight relationship (LWR) parameters and relative condition factor (K n) of marine shrimp, Parapenaeopsis sculptilis (Heller, 1862) were estimated using length-weight data collected between February 2012 and January 2013 from the coastal waters of Terong, Perak, Peninsular Malaysia. The estimated length-weight relationship of P. sculptilis for both sexes was W = 0.00027TL2.80. Meanwhile, the estimated relative growth coefficient (b) was 2.80 for both sexes, indicating a negative allometric growth pattern of P. sculptilis in the investigated area. Relative condition factor (Kn) values ranged from 0.99 to 1.064 (1.013±0.005, mean ±SD). Kn value changes in various months: the highest peak was in March-April, indicating the spawning period and the trough and small peaks indicating the cycle gonadal development

Population dynamics of sergestid shrimps Acetes japonicus in the estuary of Tanjung Dawai, Kedah, Malaysia

Population parameters of male and female A. japonicus were studied using the monthly length frequency data to evaluate the mortality rates and its exploitation level. The sex ratio (male: Female) was found at 1: 0.94. Asymptotic length (L∞) was 25.20 mm and 28.88 mm for male and female, respectively. Growth co-efficient (K) for males and females was estimated at 1.80 and 1.30 year-1, respectively. Total mortality (Z) was calculated at 5.98 and 4.44 year-1 for male and female of A. japonicus respectively. Natural mortality (M) was 2.82 and 2.19 year-1 for the male and female shrimps. The fishing mortality (F) was 3.16 year-1 for male and 2.25 year-1 for female. Exploitation level (E) for male and female of A. japonicus was calculated at 0.53 and 0.51. The exploitation level was slightly over (E>0.50) the optimum level of exploitation (p = 0.50). The stock of A. japonicus was found to be slightly over exploited in Tanjung Dawai estuarine waters

Impact of long-term elosulfase alfa treatment on respiratory function in patients with Morquio A syndrome

OBJECTIVE: To present long-term respiratory function outcomes from an open-label, multi-center, phase 3 extension study (MOR-005) of elosulfase alfa enzyme replacement therapy (ERT) in patients with Morquio A syndrome. METHODS: In part 1 of MOR-005, patients initially randomized to ERT in the 24-week pivotal study (MOR-004) remained on their regimen (2.0 mg/kg/week or every other week); placebo patients were re-randomized to one of the two regimens. During part 2, all patients received elosulfase alfa 2.0 mg/kg/week. Respiratory function was one of the efficacy endpoints evaluated in MOR-005. Change from MOR-004 baseline to 120 weeks of treatment for the combined population was determined and compared with results from untreated patients from a Morquio A natural history study (MorCAP). RESULTS: Maximum voluntary ventilation (MVV) improved up to week 72 and then stabilized; forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) increased continuously over 120 weeks. Mean increases in the modified per-protocol population was 9.2 % for FVC, 8.8 % for FEV1, and 6.1 % for MVV after 120 weeks. All patients ≤14 years showed respiratory improvements, presumably in part related to growth; however, these were greater in treated patients. For those >14 years, treated patients showed improvements, while deterioration occurred in untreated. Altogether, the improvements were significantly greater (P < 0.05) in treated patients. CONCLUSIONS: Long-term ERT is associated with sustained improvements in respiratory function in Morquio A. In younger patients (≤14 years), some improvement may be ascribed to growth. In older patients, other mechanisms, e.g., decreased glycosaminoglycan storage, are likely involved

Long-term endurance and safety of elosulfase alfa enzyme replacement therapy in patients with Morquio A syndrome

Long-term efficacy and safety of elosulfase alfa enzyme replacement therapy were evaluated in Morquio A patients over 96weeks (reaching 120weeks in total from pre-treatment baseline) in an open-label, multi-center, phase III extension study. During this extension of a 24-week placebo-controlled phase III study, all patients initially received 2.0mg/kg elosulfase alfa either weekly or every other week, prior to establishment of 2.0mg/kg/week as the recommended dose, at which point all patients received weekly treatment. Efficacy measures were compared to baseline of the initial 24-week study, enabling analyses of changes over 120weeks. In addition to performing analyses for the entire intent-to-treat (ITT) population (N=173), analyses were also performed for a modified per-protocol (MPP) population (N=124), which excluded patients who had orthopedic surgery during the extension study or were non-compliant with the study protocol (as determined by ≥20% missed infusions). Six-minute walk test (6MWT) was the primary efficacy measure; three-minute stair climb test (3MSCT) and normalized urine keratan sulfate (uKS) were secondary efficacy measures. Mean (SE) change from baseline to Week 120 in 6MWT distance was 32.0 (11.3)m and 39.9 (10.1)m for patients receiving elosulfase alfa at 2.0mg/kg/week throughout the study (N=56) and 15.1 (7.1)m and 31.7 (6.8)m in all patients combined, regardless of dosing regimen, for the ITT and MPP populations, respectively. Further analyses revealed that durability of 6MWT improvements was not impacted by baseline 6MWT distance, use of a walking aid, or age. Mean (SE) change at Week 120 in the 3MSCT was 5.5 (1.9) and 6.7 (2.0)stairs/min for patients receiving elosulfase alfa at 2.0mg/kg/week throughout the study and 4.3 (1.2) and 6.8 (1.3)stairs/min in all patients combined, regardless of dosing regimen, for the ITT and MPP populations, respectively Across all patients, mean (SE) change at Week 120 in normalized uKS was -59.4 (1.8)% and -62.3 (1.8)% in the ITT and MPP populations, respectively. In the absence of a placebo group, significance of the sustained improvements could not be evaluated directly. However, to provide context for interpretation of results, comparisons were performed with untreated patients from a Morquio A natural history study. In contrast to the results of the extension study, the untreated patients experienced constant uKS levels and a gradual decline in endurance test results over a similar period of time. Differences from the untreated natural history study patients were significant for 6MWT, 3MSCT, and uKS outcomes for the cohort of patients receiving optimal dosing throughout the study and for all cohorts pooled together, for both ITT and MPP populations (P<0.05). Safety findings were consistent with those of the initial 24-week study, with no new safety signals identified

Genetic and phenotypic characterization of NKX6‐2‐related spastic ataxia and hypomyelination

Background and purpose Hypomyelinating leukodystrophies are a heterogeneous group of genetic disorders with a wide spectrum of phenotypes and a high rate of genetically unsolved cases. Bi‐allelic mutations in NKX6‐2 were recently linked to spastic ataxia 8 with hypomyelinating leukodystrophy. Methods Using a combination of homozygosity mapping, exome sequencing, and detailed clinical and neuroimaging assessment a series of new NKX6‐2 mutations in a multicentre setting is described. Then, all reported NKX6‐2 mutations and those identified in this study were combined and an in‐depth analysis of NKX6‐2‐related disease spectrum was provided. Results Eleven new cases from eight families of different ethnic backgrounds carrying compound heterozygous and homozygous pathogenic variants in NKX6‐2 were identified, evidencing a high NKX6‐2 mutation burden in the hypomyelinating leukodystrophy disease spectrum. Our data reveal a phenotype spectrum with neonatal onset, global psychomotor delay and worse prognosis at the severe end and a childhood onset with mainly motor phenotype at the milder end. The phenotypic and neuroimaging expression in NKX6‐2 is described and it is shown that phenotypes with epilepsy in the absence of overt hypomyelination and diffuse hypomyelination without seizures can occur. Conclusions NKX6‐2 mutations should be considered in patients with autosomal recessive, very early onset of nystagmus, cerebellar ataxia with hypotonia that rapidly progresses to spasticity, particularly when associated with neuroimaging signs of hypomyelination. Therefore, it is recommended that NXK6‐2 should be included in hypomyelinating leukodystrophy and spastic ataxia diagnostic panels

Chemokine CXCL13 is overexpressed in the tumour tissue and in the peripheral blood of breast cancer patients

The abilities of chemokines in orchestrating cellular migration are utilised by different (patho-)biological networks including malignancies. However, except for CXCR4/CXCL12, little is known about the relation between tumour-related chemokine expression and the development and progression of solid tumours like breast cancer. In this study, microarray analyses revealed the overexpression of chemokine CXCL13 in breast cancer specimens. This finding was confirmed by real-time polymerase chain reaction in a larger set of samples (n=34) and cell lines, and was validated on the protein level performing Western blot, ELISA, and immunohistochemistry. Levels of CXCR5, the receptor for CXCL13, were low in malignant and healthy breast tissues, and surface expression was not detected in vitro. However, we observed a strong (P=0.0004) correlation between the expressions of CXCL13 and CXCR5 in breast cancer tissues, indicating a biologically relevant role of CXCR5 in vivo. Finally, we detected significantly elevated serum concentrations of CXCL13 in patients with metastatic disease (n=54) as compared with controls (n=44) and disease-free patients (n=48). In conclusion, CXCL13 is overexpressed within breast cancer tissues, and increased serum levels of this cytokine can be found in breast cancer patients with metastatic disease pointing to a role of CXCL13 in the progression of breast cancer, suggesting that CXCL13 might serve as a useful therapeutic target and/or diagnostic marker in this malignancy

Uses of simulation-based education for anesthesiology training, certification and recertification: A scoping review

الملخص: أهداف البحث: تم إجراء هذه الورقة لإجراء مراجعة نطاق شاملة تركز على التدريب المنشور الخاص بالتخدير بناءً على المحاكاة لتقييم الاستخدامات الحديثة للتعليم القائم على المحاكاة في التدريب على التخدير. هدفنا هو إنشاء موثوقية لبناء مناهج موحدة ومعتمدة للتعليم القائم على المحاكاة في تخصص التخدير في المستقبل القريب. طريقة البحث: تم البحث في المقالات في ” ببميد “ و ”سيناهل“ و ”إمبيز“ في 10 مايو 2021م. لقد اتبعنا منهجية مراجعة النطاق المكونة من خمس مراحل: تحديد سؤال البحث، وتحديد الدراسات ذات الصلة، واختيار الدراسة، ورسم البيانات وتوليف النتائج. النتائج: حدد بحثنا الإلكتروني الأولي 5609 مقالة محتملة. بعد فحص الملخصات وإزالة التكرارات، تم تقييم 636 مقالة في النص الكامل لإدراجها أو استبعادها. وبناء على ذلك، تم تضمين 283 مقالة في هذه المراجعة. قمنا برسم خريطة الممارسة الحالية للمحاكاة في التدريب ومنح الشهادات عبر تخصصات فرعية مختلفة من التخدير. الاستنتاجات: لقد تم بذل جهد كبير في استخدام المحاكاة للتدريب ومنح الشهادات وإعادة التأهيل في مجال التخدير. الجهود المستقبلية لتطوير تدريب قائم على المحاكاة يمكن تعميمه على المتدربين في هذا التخصص، على غرار المجالات الأخرى مثل علوم الطيران والفضاء لتعزيز توحيد التدريب وبالتالي سلامة المرضى. Abstract: Objectives: To conduct a comprehensive scoping review focusing on published anesthesiology-specific training based on simulation to assess up-to-date uses of simulation-based education in anesthesiology training. Our goal was to establish a solid ground for building standardized and accredited curricula for simulation-based education in the specialty of anesthesia in the near future. Methods: We searched the PubMed, CINAHL and EMBASE databases on May 10, 2021 for relevant articles. We followed the five-stage scoping review methodology: identifying a research question, identifying relevant studies, study selection, charting the data, and synthesis of results. Results: Our initial electronic search identified 5609 potential articles. After abstract screening and removing duplicates, 636 articles were evaluated in full text for inclusion or exclusion. Based on this strategy, 283 articles were included in this review. We mapped the current practice of simulation in training and certification across different anesthesiology subspecialties. Conclusions: Significant effort has been placed into the use of simulation for training, certification, and recertification in anesthesiology. Future effort to develop simulation-based training that can be generalized to trainees in this specialty, similar to other fields such as aviation and space sciences, will enhance the standardization of training and hence patient safety