A Rare Case of X-Linked Bulbo-Spinal Muscular Atrophy with Sensory Neuropathy and Tremors

Kennedy disease (KD) is also known as spino bulbar muscular atrophy caused by a tandem C-A-G tri-nucleotide repeat. It is an adult-onset X-linked recessive inherited neurodegenerative disease involving lower motor neuron damage with predominance of facial muscles. It is often accompanied with androgen sensitivity, sensory nerve damage and endocrinal involvement. It has similar confusing symptoms and is often mis-diagnosed with most of the neuromuscular diseases like POEMS syndrome, myasthenia gravis, mitochondrial myopathy and amyotrophic lateral sclerosis. Hence clinical differentiation is important to prevent adverse outcomes and un-necessary treatment. Here we describe a rare case of a 46 year old Pakistani male who presented to us with progressive weakness and tingling of the limbs, bulbar symptoms, postural tremors and painful recurrent ulceration of the feet. Based on family history, clinical and electro diagnostic study he was diagnosed to have Kennedy disease. To the best of our knowledge, it is the first case report of Kennedy disease from Pakistan

Internal mammary artery flow in different racial groups of Pakistan

Objective: To find out any differences in free flow of internal mammary artery among different ethnic groups.Methods: This observational, cross-sectional study was conducted at the Civil Hospital, Karachi, from January 2013 to December 2015, and comprised patients who underwent coronary artery bypass grafting. The participants were divided into 5 groups, i.e. Sindhi, Muslim migrants from India, Punjabi, Pathan and Balochi patients. Free flow of internal mammary artery was measured immediately after harvesting within a syringe, and its flow was measured in 30 seconds. SPSS 18 was used for data analysis.Results: Of the 158 patients, 44(27.8%) were Sindhi, 33(20.9%) Punjabi, 8(5%) Baloch, 21(13.3%) Pathan and 52(32.9%) were migrants. The overall mean age was 52±8 years and the mean flow was 11.6±9.6ml per 30 seconds. The flow was 9.3±6 ml, 10±8ml, 13±11ml, 17±14ml and 15±13 ml in 30 seconds among migrants, Sindhi, Punjabi, Pathan and Baloch patients, respectively, with significant higher flow in Pathan patients compared to Sindhi and migrant patients (pConclusions: Internal mammary artery flow was higher in Pathan and Baloch patients and low in migrants and Sindhis

An Efficient Adaptive Noise Cancellation Scheme Using ALE and NLMS Filters

The basic theme of our paper is to implement a new idea of noise reduction in the real time applications using the concepts of adaptive filters.  Our model which is presented as one of the solutions is based on two stages of operation with the first stage based on the ALE (Adaptive Line Enhancer) filters and the second stage on NLMS (Normalized Least Mean Square) filter. The first stage reduces the sinusoidal noise from the input signal and the second stage reduces the wideband noise. Two input sources of voice are used; one for the normal speech and the other for the noise input, using separate microphones for both signals. The first signal is of the corrupted speech signal and the second signal is of only the noise containing both wideband and narrowband noise. In the first stage the narrowband noise is reduced by using the ALE technique. The second stage gets a signal with ideally only the wideband noise which is reduced using the NLMS technique.  In both the stages the concerned algorithms are used to update the filter coefficients in such a way that the noise is cancelled out from the signal and a clean speech signal is heard at the output.DOI:http://dx.doi.org/10.11591/ijece.v2i3.24

PHYSICAL AND INDEX PROPERTIES OF LATE PRECAMBRIAN AND CAMBRIAN SALT RANGE ROCKS OF PUNJAB

Thirteen rock types including Sandstone, Siltstone, Dolomite, Shale, Gypsum and Rock Salt collected from five different formations of Salt Range area of Pakistan were tested to assess their physical and index properties. Specific gravity, dry and wet unit weights, moisture contents, porosity, water absorption and slake durability index were determined. Results indicate that most of the rocks have their specific gravity and unit weight values in the standard range. Higher durability indices and low water absorption values of sandstones and dolomite make them suitable for construction materials. While higher porosity of Baghanwala sandstone, lower and upper Khewra sandstone indicates their potential as hydrocarbon bearing reservoir and at the same time make them an ideal candidate for greenhouse gases sequestration in the subsurface.&nbsp

A Rare Case of Thiamine Responsive Megaloblastic Anemia Presenting with Seizures

Thiamine responsive megaloblastic anemia (TRMA) also known as Rogers syndrome is a rare entity inherited as an autosomal recessive disorder. It consists of a pentad of diabetes-mellitus, megaloblastic anemia, thrombocytopenia, leukopenia and sensory-neural hearing loss. The defect occurs due to mutations in SLC19A2 gene resulting in the deficiency of a thiamine transporter proteins which prevents the transport of thiamine in to the cells and tissues. Here we report a 13 year old boy with megaloblastic anemia, sensory-neural deafness and young onset diabetes mellitus who presented with generalized tonic-clonic seizures. Diagnosis was based on clinical features and a rapid response to thiamine replacement with adequate control of seizures. This clinical entity and its association with epilepsy is extremely rare and must be thought of amongst the differentials of megaloblastic anemia and epilepsy. This rare case emphasizes the unique association of TRMA with epilepsy. Early diagnosis and management with thiamine drastically improves anemia, seizure control and blood glucose levels. To the best of our knowledge, it is the first case report of TRMA with epilepsy from Pakistan

Does e-HRM improve labour productivity? A study of commercial bank workplaces in Pakistan

Purpose: Drawing on data from a unique, large-scale survey, we examine the links between e-HRM and perceived labour productivity both directly and through the mediating role of HR service quality amongst commercial-bank workplaces in Pakistan, many of which have introduced e-HRM. Design: We use partial least squares structural equation modelling to examine the direct links between e-HRM and productivity as well as the mediated links between e-HRM, perceived HR service quality and productivity. Findings: We show that e-HRM practices have a statistically significant, positive effect on managers’ perceptions of labour productivity. We also reveal that e-HRM practices influence the quality of HR service, and that the quality of HR services fully mediates the relationship between e-HRM practices and managers’ perceptions of labour productivity. Practical implications: Our results highlight the importance of designing and implementing e-HRM systems so that they support organization workflow and enable workers to carry out a range of HR and non-HR activities more efficiently. In particular, this study suggests that managers should focus on how e-HRM impacts on HR service quality in a holistic way, as this is the ‘route’ via which e-HRM can improve labour productivity. Originality: Existing research has demonstrated a link between e-HRM and the quality of HR services; however, these studies downplay the potential impact of e-HRM on labour productivity, a key organizational outcome and one that e-HRM aims to improve. The study contributes to the HRM literature by identifying how e-HRM can improve labour productivity by enhancing the perceived HR service quality. The study, therefore, provides the basis for future theory developments in this area

Comparative Efficacy of Different Strategies for Management of Spotted Bollworms, Earias spp. on Okra, Abelmoschus esculentus (L). Moench

Abstract. -Integrated control measures were tested for efficacy against Earias spp. on okra in Pakistan with the over-riding goal of finding the most cost-effective control. Five control methods: (i) biological control with Trichogramma chilonis Ishii., (ii) cultural control of alternate host plants by hand weeding and hoeing, (iii) botanical control by spraying the plants with 5% neem seed kernel extract, (iv) mechanical control by hand-clipping injured plant parts and (v) chemical control with by spraying the plants with a commercial insecticide (Tracer®, Dow AgroSciences). These treatments were tested alone and as seven combinations of two methods and three combinations of three methods. All treatments were applied each week for six weeks and compared by measuring damaged fruits and shoots, yield and cost-benefit ratio in a controlled, replicated field experiment on Diksha cultivar of okra. Fruit and shoot damage was significantly lowest at 7% and 14%, respectively, in the plots treated with only chemical control and fruit yield was highest, at 63 kg/plot, in plots treated with the combination of mechanical and chemical control. Plots treated with only mechanical control had the highest cost: benefit ratio at 1:2.61, followed by plots treated with mechanical control and chemical control, at 1:2.39 and plots treated with chemcial plus botanical plus mechanical control at 1:2.38