Challenges in the Management of Mucopolysaccharidosis Type II (Hunter’s Syndrome) in a Developing Country: a Case Report

BACKGROUND: Mucopolysaccharidosis type II (Hunter's syndrome) is an X-linked chromosomal storage disorder due to deficiency of the lysosomal enzyme iduronate-2-sulfatase with patients rarely living till adulthood. Failure to identify patients early could contribute to an increased morbidity as identified in this case report.CASE DETAILS: An eight year old patient with Hunter's syndrome identified five years after disease onset with severe cardiovascular complications exemplifies the challenges faced in resource-limited countries towards making diagnosis and treatment of rare conditions. Elevated urinary glycosaminoglycans levels or a strong clinical suspicion of Hunter's syndrome, as identified in the index case, is a prerequisite for enzyme activity testing. Urinary mucopolysaccharide(MPS) level was 69.6mg/mmol(normal range is 0.0 - 11.6mg/mmol), and the confirming MPS electrophoresis analysis showed elevated heparan sulphate in the urine sample. Enzyme activity testing, with absent or very low iduronate-2-sulfatase activity, is diagnostic. However, the scarce availability and high cost of these tests is another constraint in making a diagnosis.CONCLUSION: Identification and management of mucopolysaccharidosis type II pose a problem in resource-constrained countries due to late presentation, lack of facility for diagnosis and treatment, cost and expertise required for the management.KEYWORDS: Mucopolysaccharidosis, Hunter's syndrome, Mucopolysaccharid

Sucrose assimilation and the role of sucrose transporters in plant wound response

Plant cells are commonly exposed to a variety of injuries such as mechanical and herbivore wounding. Wounding is a continual threat to the survival of all organisms and an open wound caused bymechanical or herbivore wounding is a potential infection site for pathogens, thus expression of defense genes at the wound site is a barrier against opportunistic pathogens. Wounding in multicellulareukaryote cells result in marked changes in gene repression that contribute to cell defense and repairs. The sudden changes in cellular metabolism and additional metabolism requirements for these woundedtissues or cells can only be met by an increased utilization of exogenously supplied carbohydrate in the form of sucrose. Sucrose transporters’ involvement in the import of sucrose from the apoplastic cells will be of great significance for the cellular metabolic needs, and also for energy and carbon requirements for the activation of defense responses of the adjacent injured tissues or cells

A Novel Algorithm to Forecast Enrollment Based on Fuzzy Time Series

In this paper we propose a new method to forecast enrollments based on fuzzy time series. The proposed method belongs to the first order and time-variant methods. Historical enrollments of the University of Alabama from year 1948 to 2009 are used in this study to illustrate the forecasting process. By comparing the proposed method with other methods we will show that the proposed method has a higher accuracy rate for forecasting enrollments than the existing methods

Nutritional Status of Primary School Children in Ilorin-West LGA, Kwara State, Nigeria

Background: Malnutrition in children remains a global health problem in developing countries including Nigeria. Nearly half of all under-five deaths in Africa and South-East Asia can be traceable to malnutrition. The nutritional status of primary school children in Ilorin-West Local Government Area of Kwara State is not known, therefore the study set out to determine this and its associated factors.Methods: This study was a descriptive cross-sectional study carried out between February and May 2016 across 16 primary schools in Ilorin-West LGA. Four hundred and eighty school children aged 6-12 years were recruited into the study through a multi-stage sampling technique. Anthropometry was taken following standard procedures; weight was measured with a digital bathroom weighing scale (Camry, Model: EB9323H, China), while height was measured with a stadiometer (Seca Model: 213, USA). Anthropometric indices were determined. Data collected was analysed using the IBM Statistical Package for Social Sciences (SPSS) Tm version 20.0 for windows.Results: The prevalence of underweight, stunting, wasting and obesity were 3.0%, 5.0%, 4.4% and 1.0%, respectively. Stunting was more prevalent among female pupils, pupils attending the public primary schools and children of mothers with no formal education, while obesity was more prevalent in the private primary schools.Conclusion: The prevalence of obesity recorded in this study confirms the emerging concept of ‘the double burden of malnutrition,’ and poses a public health challenge to policy makers. It is recommended that community nutrition enlightenment campaigns be strengthened and possibly school meals be re-introduced to both private and public schools. Keywords: Nutritional status; School-aged; Stunting; Wasting; Obesit

Histomorphology, ultrastructure and fatty acid composition of the adipose tissue in pansteatitis, the potentials in understanding the underlying mechanism and diagnosis of pansteatitis in the Nile crocodile

BACKGROUND : In an effort to characterize the fat body and other adipose tissue in the Nile crocodile and the effects of pansteatitis on the structure and composition of the adipose tissue, we evaluated the regional variation in structure and fatty acid composition of healthy farmed crocodiles and those affected by pansteatitis. METHODS : Adipose tissue samples were collected from the subcutaneous, visceral and intramuscular fat and the abdominal fat body of ten 4-year old juvenile crocodiles from Izinthaba Crocodile Farm, Pretoria, South Africa while pansteatitis samples were collected from visceral and intramuscular fat of crocodiles that had died of pansteatitis at the Olifant River, Mpumalanga, also in South Africa. Histomorphology, ultrastrustucture and fatty acid composition by fatty acid methyl ester (FAME) analysis were conducted. RESULTS : Histological examination showed regional variations in the adipose tissue especially in the collagen content of the ECM, tissue perfusion and division into lobes and lobules by fibrous capsule. Considerable fibrosis, mononuclear cell infiltration especially by macrophages and lymphocytes and toxic changes in the nucleus were observed in the pansteatitis samples. Regional variation in lipid composition especially in Myristoleic (C14:1), Erucic acid (C22:1n9), and Docosadienoic acid (C22:2n6) was observed. Most of the saturated and trans fatty acids were found in significant quantities in the pansteatitis samples, but had very low levels of the cis fatty acid and the essential fatty acids with C18 backbone. CONCLUSION : This study demonstrates that there exists some regional variation in histomorphology and fatty acid composition in the healthy adipose tissue of the Nile crocodile. It also showed that pansteatitis in the Nile crocodile might have been triggered by sudden change in energy balance from consumption of dead fish; and probable exposure to toxic environmental conditions with the evidence of up scaled monounsaturated long chain fatty acids composition and toxic changes in the leucocytes observed in pansteatitis in the present study.http://www.sherpa.ac.uk/romeo/issn/1476-511X/am2017Anatomy and PhysiologyParaclinical SciencesVeterinary Tropical Disease

The impact of electronic versus paper-based data capture on data collection logistics and on missing scores in thyroid cancer patients.

The purpose of this study was to investigate the impact of the type of data capture on the time and help needed for collecting patient-reported outcomes as well as on the proportion of missing scores. In a multinational prospective study, thyroid cancer patients from 17 countries completed a validated questionnaire measuring quality of life. Electronic data capture was compared to the paper-based approach using multivariate logistic regression. A total of 437 patients were included, of whom 13% used electronic data capture. The relation between data capture and time needed was modified by the emotional functioning of the patients. Those with clinical impairments in that respect needed more time to complete the questionnaire when they used electronic data capture compared to paper and pencil (OR <sub>adj</sub> 24.0; p = 0.006). This was not the case when patients had sub-threshold emotional problems (OR <sub>adj</sub> 1.9; p = 0.48). The odds of having the researcher reading the questions out (instead of the patient doing this themselves) (OR <sub>adj</sub> 0.1; p = 0.01) and of needing any help (OR <sub>adj</sub> 0.1; p = 0.01) were lower when electronic data capture was used. The proportion of missing scores was equivalent in both groups (OR <sub>adj</sub> 0.4, p = 0.42). The advantages of electronic data capture, such as real-time assessment and fewer data entry errors, may come at the price of more time required for data collection when the patients have mental health problems. As this is not uncommon in thyroid cancer, researchers need to choose the type of data capture wisely for their particular research question

Evaluation of andrological indices and testicular histology following administration of varied doses of nicotine

This study is aimed at determining the effect of Nicotine on male fertility by evaluating some andrological parameters of male Wistar rat such as sperm parameters (spermatozoa count and motility), serum concentration of testosterone and testicular weight. Histopathology of the testis was also carried out on the effect of nicotine on testicular microstructure. 20 adult male rats were randomly divided into four groups, the test groups were administered with 0.2mg/100g, 0.4/100g and 0.6/100g body weight daily for 30 days while the control were administered with 2mls 0.9% physiological saline. Nicotine caused a significant reduction (P < 0.05) and (P< 0.01) in the mean values of sperm count, serum testosterone concentration and testicular weight in the test when compared with the control. Also, in the test group, the deleterious effect of nicotine on the sperm parameters and testosterone concentration was corroborated by histopathology which revealed a marked degeneration of germ cell layers in the seminiferous tubule and disruption of interstitial cells of the testis thereby interfering with spermatogenesis and testosterone secretion while there was no visible change in the control group. It was concluded that nicotine exerted toxic effect on the germ cell layers in seminiferous tubule with concomitant reduction in reproductive potentials of the male rat whilst showing no significant change in sperm motility. Nicotine and nicotine based products should therefore be taken with caution in cases of infertility. Key words: germ cells, testes, testosterone, fertility, spermatogenesis

Efficient PAPR reduction of OFDM signal using PTS technique with hybrid partitioning method

The high peak-to-average power ratio (PAPR) is one of the major problems of orthogonal frequency division multiplexing (OFDM) systems in wireless transmission.Therefore, partial transmit sequence (PTS), a promising scheme that can provide good PAPR reduction performance, has been proposed for OFDM transmission to eliminate distortion. The PTS method divides the input data block into disjoint sub-blocks, computes Inverse Fourier Transform of the subblocks, rotates the sub-blocks with appropriate phase factors and combines them to form the transmitted signal.This paper presents an enhanced PTS approach that combines two PTS partitioning schemes (adjacent and interleaved) to effectively reduce the PAPR of the OFDM systems. The influence of the proposed approach on performance is investigated by varying the size of the disjoint sub-blocks.The PAPR reduction performance of the proposed PTS method is compared with two well known sub-blocks partitioning schemes, namely, Adjacent Partitioning (AP), Interleaved Partitioning (IP).The various computer simulation results for the various sub-blocks confirmed that the proposed method provides better PAPR reduction performance compared with AP and IP partitioning based PTS scheme. In addition, these PTS schemes largely depend on the chosen size of the partitions

EFFICIENT PAPR REDUCTION OF OFDM SIGNAL USING PTS TECHNIQUE WITH HYBRID PARTITIONING METHOD

ABSTRACT The high peak-to-average power ratio (PAPR) is one of the major problems of orthogonal frequency division multiplexing (OFDM) systems in wireless transmission. Therefore, partial transmit sequence (PTS), a promising scheme that can provide good PAPR reduction performance, has been proposed for OFDM transmission to eliminate distortion. The PTS method divides the input data block into disjoint sub-blocks, computes Inverse Fourier Transform of the subblocks, rotates the sub-blocks with appropriate phase factors and combines them to form the transmitted signal. This paper presents an enhanced PTS approach that combines two PTS partitioning schemes (adjacent and interleaved) to effectively reduce the PAPR of the OFDM systems. The influence of the proposed approach on performance is investigated by varying the size of the disjoint sub-blocks. The PAPR reduction performance of the proposed PTS method is compared with two well known sub-blocks partitioning schemes, namely, Adjacent Partitioning (AP), Interleaved Partitioning (IP). The various computer simulation results for the various sub-blocks confirmed that the proposed method provides better PAPR reduction performance compared with AP and IP partitioning based PTS scheme. In addition, these PTS schemes largely depend on the chosen size of the partitions