Hallervorden spatz disease – a rare clinicoradiological diagnosis

Hallervorden Spatz disease, also known as pantothenate kinase associated neuro-degeneration, is a rare, progressive neurological disorder usually seen in first decade of life. It is associated with extrapyramidal effects, dysarthria and dementia. Hallervorden Spatz Disease is also associated with psychiatric symptoms, depression and behavioral changes. Affected patients are disabled predominantly by dystonia. MRI, in later stage of the disease, shows “eye of the tiger’ appearance which is fairly diagnostic of Hallervorden Spatz Disease. Response to drugs is often poor and of limited value to these patients. This report highlights a classical case of Hallervorden Spatz disease that presented as an outpatient with dystonia and psychotic symptoms and was diagnosed on the basis of clinical and radiological evidence

Antibacterial Potentials of Human Urine at Acidic pH 5

To identify factors determining susceptibility of individuals to urinary tract infections (UTIs).Methods: In this descriptive study , 55 hospitalized patients' urine samples were analyzed. Presence of red blood cells , pus cells, epithelial cells, casts and crystals were observed and counted under per high power field (HPF) by microscopy. While pH, specific gravity, protein, leukocytes, nitrites, glucose, ketones, urobilinogen, blood and bilirubin, were analyzed using dipstick method. All the samples were streaked on CLED agar for isolation of bacteria; and SDA for yeast.Results: Twenty urine samples were found culture positive, of which 15 were from females and 5 from males. Cultures were isolated and identified as E. coli (11), Enterococcus (4), Klebsiella (3), Pseudomonas (1) and yeast (1). Interestingly, organisms were mainly isolated from urine samples having pH >5.5. In all the culture positive samples, pus cells were >20-40 /HPF. No patient with culture negative had urine pH 6.5 or above in the present study.Conclusion: The probability of bacteriuria (UTI) and pyuria (increase pus cells in urine) increases with rise in urine pH. Persons with urine pH5 are generally protected from UTIs. Thus mechanism/s needs to be elucidated

N,N′-[1,3-Phenylenebis(methyl­ene)]dibenzene­sulfonamide

The complete mol­ecule of the title compound, C20H20N2O4S2, is generated by crystallographic twofold symmetry, with two C atoms lying on the rotation axis. The dihedral angle between the central benzene ring and the pendant ring is 68.42 (6)° and the dihedral angle between the pendant rings is 45.11 (5)°. The torsion angles for the C—S—N—C and S—N—C—C fragments are −73.22 (15) and −150.45 (13)°, respectively. In the crystal, mol­ecules are linked by N—H⋯O hydrogen bonds, generating corrugated (001) sheets. Aromatic π–π stacking [centroid–centroid separation = 3.8925 (12) and 3.9777 (12) Å] and weak C—H⋯O inter­actions also occur

N,N′-Diethyl-N,N′-[1,3-phenylene­bis(methyl­ene)]dibenzene­sulfonamide

In the title compound, C24H28N2O4S2, the dihedral angles between the central benzene ring and the pendant rings are 77.44 (11) and 79.23 (10)°, and the dihedral angle between the pendant rings is 23.31 (12)°. Both sulfonamide groups project to the same side of the central benzene ring and the mol­ecule has approximate non-crystallographic mirror symmetry. One of the ethyl side chains is disordered over two sets of sites in a 0.526 (14):0.474 (14) ratio. In the crystal, inversion dimers linked by pairs of weak C—H⋯O inter­actions occur, generating R 2 2(28) loops

Pollutant-Induced Modulation in Conformation and β-Lactamase Activity of Human Serum Albumin

Structural changes in human serum albumin (HSA) induced by the pollutants 1-naphthol, 2-naphthol and 8-quinolinol were analyzed by circular dichroism, fluorescence spectroscopy and dynamic light scattering. The alteration in protein conformational stability was determined by helical content induction (from 55 to 75%) upon protein-pollutant interactions. Domain plasticity is responsible for the temperature-mediated unfolding of HSA. These findings were compared to HSA-hydrolase activity. We found that though HSA is a monomeric protein, it shows heterotropic allostericity for β-lactamase activity in the presence of pollutants, which act as K- and V-type non-essential activators. Pollutants cause conformational changes and catalytic modifications of the protein (increase in β-lactamase activity from 100 to 200%). HSA-pollutant interactions mediate other protein-ligand interactions, such as HSA-nitrocefin. Therefore, this protein can exist in different conformations with different catalytic properties depending on activator binding. This is the first report to demonstrate the catalytic allostericity of HSA through a mechanistic approach. We also show a correlation with non-microbial drug resistance as HSA is capable of self-hydrolysis of β-lactam drugs, which is further potentiated by pollutants due to conformational changes in HSA

Cobalt availability in the soil plant and animal food chain: a study under a peri-urban environment

Abstract Cobalt metal is considered as an essential trace element for the animals. Present investigation was undertaken in the peri-urban area to analyze the cobalt availability in animal food chain by using different indices. Cow, buffalo and sheep samples along with forage and soil samples were collected from the three different sites of District Jhang and analyzed through atomic absorption spectrophotometer. Cobalt values differed in soil samples as 0.315-0.535 mg/kg, forages as 0.127-0.333 mg/kg and animal samples as 0.364-0.504 mg/kg. Analyzed cobalt concentration in soil, forage and animal samples was found to be deficient in concentration with respect to standard limits. Soil showed the minimum cobalt level in Z. mays while maximum concentration was examined in the forage C. decidua samples. All indices examined in this study has values lesser than 1, representing the safer limits of the cobalt concentration in these samples. Enrichment factor (0.071-0.161 mg/kg) showed the highly deficient amount of cobalt enrichment in this area. Bio-concentration factor (0.392-0.883) and pollution load index (0.035-0.059 mg/kg) values were also lesser than 1 explains that plant and soil samples are not contaminated with cobalt metal. The daily intake and health risk index ranged from 0.00019-0.00064 mg/kg/day and 0.0044-0.0150 mg/kg/day respectively. Among the animals, cobalt availability was maximum (0.0150 mg/kg/day) in the buffaloes that grazed on the C. decidua fodder. Results of this study concluded that cobalt containing fertilizers must be applied on the soil and forages. Animal feed derived from the cobalt containing supplements are supplied to the animals, to fulfill the nutritional requirements of livestock

In Silico Prediction and Analysis of Caenorhabditis EF-hand Containing Proteins

Calcium (Ca+2) is a ubiquitous messenger in eukaryotes including Caenorhabditis. Ca+2-mediated signalling processes are usually carried out through well characterized proteins like calmodulin (CaM) and other Ca+2 binding proteins (CaBP). These proteins interact with different targets and activate it by bringing conformational changes. Majority of the EF-hand proteins in Caenorhabditis contain Ca+2 binding motifs. Here, we have performed homology modelling of CaM-like proteins using the crystal structure of Drosophila melanogaster CaM as a template. Molecular docking was applied to explore the binding mechanism of CaM-like proteins and IQ1 motif which is a ∼25 residues and conform to the consensus sequence (I, L, V)QXXXRXXXX(R,K) to serve as a binding site for different EF hand proteins. We made an attempt to identify all the EF-hand (a helix-loop-helix structure characterized by a 12 residues loop sequence involved in metal coordination) containing proteins and their Ca+2 binding affinity in Caenorhabditis by analysing the complete genome sequence. Docking studies revealed that F165, F169, L29, E33, F44, L57, M61, M96, M97, M108, G65, V115, F93, N104, E144 of CaM-like protein is involved in the interaction with IQ1 motif. A maximum of 170 EF-hand proteins and 39 non-EF-hand proteins with Ca+2/metal binding motif were identified. Diverse proteins including enzyme, transcription, translation and large number of unknown proteins have one or more putative EF-hands. Phylogenetic analysis revealed seven major classes/groups that contain some families of proteins. Various domains that we identified in the EF-hand proteins (uncharacterized) would help in elucidating their functions. It is the first report of its kind where calcium binding loop sequences of EF-hand proteins were analyzed to decipher their calcium affinities. Variation in Ca+2-binding affinity of EF-hand CaBP could be further used to study the behaviour of these proteins. Our analyses postulated that Ca+2 is likely to be key player in Caenorhabditis cell signalling

Global, regional, and national burden of chronic kidney disease, 1990–2017 : a systematic analysis for the Global Burden of Disease Study 2017

Background Health system planning requires careful assessment of chronic kidney disease (CKD) epidemiology, but data for morbidity and mortality of this disease are scarce or non-existent in many countries. We estimated the global, regional, and national burden of CKD, as well as the burden of cardiovascular disease and gout attributable to impaired kidney function, for the Global Burden of Diseases, Injuries, and Risk Factors Study 2017. We use the term CKD to refer to the morbidity and mortality that can be directly attributed to all stages of CKD, and we use the term impaired kidney function to refer to the additional risk of CKD from cardiovascular disease and gout. Methods The main data sources we used were published literature, vital registration systems, end-stage kidney disease registries, and household surveys. Estimates of CKD burden were produced using a Cause of Death Ensemble model and a Bayesian meta-regression analytical tool, and included incidence, prevalence, years lived with disability, mortality, years of life lost, and disability-adjusted life-years (DALYs). A comparative risk assessment approach was used to estimate the proportion of cardiovascular diseases and gout burden attributable to impaired kidney function. Findings Globally, in 2017, 1·2 million (95% uncertainty interval [UI] 1·2 to 1·3) people died from CKD. The global all-age mortality rate from CKD increased 41·5% (95% UI 35·2 to 46·5) between 1990 and 2017, although there was no significant change in the age-standardised mortality rate (2·8%, −1·5 to 6·3). In 2017, 697·5 million (95% UI 649·2 to 752·0) cases of all-stage CKD were recorded, for a global prevalence of 9·1% (8·5 to 9·8). The global all-age prevalence of CKD increased 29·3% (95% UI 26·4 to 32·6) since 1990, whereas the age-standardised prevalence remained stable (1·2%, −1·1 to 3·5). CKD resulted in 35·8 million (95% UI 33·7 to 38·0) DALYs in 2017, with diabetic nephropathy accounting for almost a third of DALYs. Most of the burden of CKD was concentrated in the three lowest quintiles of Socio-demographic Index (SDI). In several regions, particularly Oceania, sub-Saharan Africa, and Latin America, the burden of CKD was much higher than expected for the level of development, whereas the disease burden in western, eastern, and central sub-Saharan Africa, east Asia, south Asia, central and eastern Europe, Australasia, and western Europe was lower than expected. 1·4 million (95% UI 1·2 to 1·6) cardiovascular disease-related deaths and 25·3 million (22·2 to 28·9) cardiovascular disease DALYs were attributable to impaired kidney function. Interpretation Kidney disease has a major effect on global health, both as a direct cause of global morbidity and mortality and as an important risk factor for cardiovascular disease. CKD is largely preventable and treatable and deserves greater attention in global health policy decision making, particularly in locations with low and middle SDI

Global, regional, and national burden of epilepsy, 1990 - 2016 : a systematic analysis for the Global Burden of Disease Study 2016

Background: Seizures and their consequences contribute to the burden of epilepsy because they can cause health loss (premature mortality and residual disability). Data on the burden of epilepsy are needed for health-care planning and resource allocation. The aim of this study was to quantify health loss due to epilepsy by age, sex, year, and location using data from the Global Burden of Diseases, Injuries, and Risk Factors Study. Methods: We assessed the burden of epilepsy in 195 countries and territories from 1990 to 2016. Burden was measured as deaths, prevalence, and disability-adjusted life-years (DALYs; a summary measure of health loss defined by the sum of years of life lost [YLLs] for premature mortality and years lived with disability), by age, sex, year, location, and Socio-demographic Index (SDI; a compound measure of income per capita, education, and fertility). Vital registrations and verbal autopsies provided information about deaths, and data on the prevalence and severity of epilepsy largely came from population representative surveys. All estimates were calculated with 95% uncertainty intervals (UIs). Interpretation: Despite the decrease in the disease burden from 1990 to 2016, epilepsy is still an important cause of disability and mortality. Standardised collection of data on epilepsy in population representative surveys will strengthen the estimates, particularly in countries for which we currently have no or sparse data and if additional data is collected on severity, causes, and treatment. Sizeable gains in reducing the burden of epilepsy might be expected from improved access to existing treatments in low-income countries and from the development of new effective drugs worldwide

Burden of musculoskeletal disorders in the Eastern Mediterranean Region, 1990–2013: findings from the Global Burden of Disease Study 2013

Moradi-Lakeh M, Forouzanfar MH, Vollset SE, et al. Burden of musculoskeletal disorders in the Eastern Mediterranean Region, 1990–2013: findings from the Global Burden of Disease Study 2013. Annals of the Rheumatic Diseases. 2017;76(8):annrheumdis-2016-210146