Congenital Myotonic Dystrophy with Combined Heterozygous ATP8B1/ABCB4 Mutation Leading to Progressive Cholestasis and Liver Failure.

Myotonic dystrophy (MyoD) is an inherited genetic disorder caused by the expansion of a CTG trinucleotide repeat in the dystrophia myotonica protein kinase gene. It manifests as a multisystem disease affecting not only skeletal muscles, but also heart, lung, eye, gastrointestinal tract, central nervous system, and endocrine system. However, MyoD is rarely associated with a progressive liver disorder. We report a case of congenital MyoD with combined heterozygous ATP8B1/ABCB4 mutation who developed chronic, progressive low gamma-glutamyltransferase cholestatic liver disease at early infancy, and eventually underwent successful liver transplantation.

An uncommon cause of early infantile liver disease and raised chitotriosidase.

Our subject presented at 11 months of age, following a varicella zoster infection, with acute on chronic liver disease and was found to have raised serum chitotriosidase. White cell enzyme analysis for Gaucher, Niemann Pick A, B and lysosomal acid lipase deficiency were normal. Niemann Pick type C (NPC) disease was considered as a provisional diagnosis and liver transplantation assessment deferred until recovery from varicella and results of mutational analysis of NPC gene were available. Liver biopsy at a later date showed findings suggestive of glycogen storage disease (GSD) type IV but he was too unstable for an urgent liver transplantation and sadly passed away at the age of 13 months. The classic hepatic subtype of glycogen storage disorder type IV (GSD IV) is a rare metabolic cause of early-onset liver disease and raised chitotriosidase. There are very few reports of raised chito in GSD IV. Liver transplantation has a favourable outcome for the hepatic subtype of GSD IV and early diagnosis in our subject could have potentially altered the outcome.

Insecticidal and Genotoxic effects of some indigenous plant extracts in Culex quinquefasciatus Say Mosquitoes.

Five different weed plants viz. Convulvulus arvensis, Chenopodium murale, Tribulus terrestris, Trianthema portulacastrum, and Achyranthes aspera were investigated for their entomocidal and genotoxic effects against Culex quinquefasciatus mosquitoes. High mortality was observed at 72 hours in a dose dependent manner. Among all the tested plants, A. aspera was found highly significant which showed 100% mortality at 250 ppm after 72 hours with LC50 of 87.46, 39.08 and 9.22 ppm at 24, 48, respectively. In combination with Bacillus thuringiensis israelensis (Bti); A. aspera also caused 100% mortality at 250 ppm concentration after 72 hours (LC50 8.29 ppm). Phytochemical analysis of all the tested weed plants showed the presence of flavonoids, saponins, tannins, steroids, cardiac glycosides, alkaloids, anthrequinones and terpenoids. Random Amplification of Polymorphic DNA-Polymerase chain reaction (RAPD-PCR) and comet assay were performed to assess the genotoxic effect of A. aspera but no change in DNA profile was observed. Furthermore, FTIR showed the presence of phenolic compounds in A. aspera extract. It is suggested that certain phenolic compounds such as flavonoids modulate the enzymatic activity and, hence, cause the death of larvae of Cx. quinquefasciatus. Altogether, current study would serve as an initial step towards replacement of synthetic insecticides to plant-microbe based biopesticide against Culex mosquitoes in future.

Irrigation of Zea mays with UASB-treated textile wastewater; effect on early irrigation of Zea mays with UASB-treated textile wastewater; effect on early growth and physiology.

In this study, mature seeds of Zea mays (Malka 16) were irrigated with untreated and UASB-treated wastewater with combination of 50% textile and 50% sewage at hydraulic retention times (HRTs) of 0, 5, 10, and 15 h. Four other treatments diluted with distilled water (DW) were also evaluated. Eight-week analysis of irrigation revealed very small differences in the results of plant biomass and growth parameters of control and those irrigated with 15 h (HRT) treatments. The values of both types of water were observed as chlorophyll a and b contents, 5.9, 3.4, vs 5.5, 3.1 mg g-1, total chlorophyll 9.4 vs 8.8 mg g-1, carotenoids 9.5 vs 8.7 mg g-1, spad values 61.4 vs 56.3, net photosynthetic rate (A) 15.6 vs 14.5 µmol m-2 S-1, transpiration rate (E) 3.98 vs 3.8 µmol m-2 S-1, stomatal conductance 5.9 vs 5.8 µmol m-2 S-1, water use efficiency 10.3 vs 9.7 mmol Cmm-1 H2O, electrolyte leakage 115 vs 98% and total soluble proteins 385 vs 354 in leaves and 260 vs 231 g-1 FW in roots. While this stress enhanced H2O2 92 vs 115 and 195 vs 224 Units g-1, MDA 6.8 vs 9.1 and 5.9 vs 8.3 Units g-1, activities of enzymatic antioxidants SOD 25 vs 63 and 54 vs 63 Units g-1, POD 1170 vs 1310 and 570 vs 650 Units g-1, CAT 570 vs 820 and 880 vs 1040 Units g-1, and APX 235 vs 278 and 134 vs 187 Units g-1 in leaves and roots, respectively. Heavy metals (Cd, Cr, Cu, and Zn) in such plants were mostly within or about permissible limits of NEQS. The results obtained were more close to that of control. This practice may lead to clean environment and its reuse shall also reduce the stress on fresh water. Early researches transpire a little work done on the reuse of UASB-treated textile wastewater with co substrate, for irrigation purpose.

Insecticidal, biological and biochemical response of Musca domestica (Diptera: Muscidae) to some indigenous weed plant extracts.

In the current study; insecticidal, growth regulation, oviposition deterrence and repellency of petroleum ether extracts of Azadirachta indica, Penganum harmala, Datura stramonium, Tribulus terrestris and Chenopodium murale against 2nd instar larvae of housefly was investigated. Five different concentrations (5%, 10%, 15%, 20% and 25%) were used through larval feeding and the mortality data was recorded after 24, 48 and 72 hrs. Highest mortality was induced by P. harmala (63.87%) followed by D. stramonium (62.78%), A. indica (53.84%), T. terrestris (41.86%) and C. murale (4.09%) after 72 h at 25% concentration, respectively. Increased mortality was observed with increased time duration and concentration. Longest larval duration (9.33 ±â€¯0.33 days) and pupal duration (7.33 ±â€¯0.33 days) days) was recorded in larvae treated with 25% concentration of P. harmala which also caused a decrease in the activity of AChE, ACP, AKP, α-Carboxyl, and ß-Carboxyl enzymes. However, at 25% concentration, C. murale showed highest oviposition deterrence activity (81.88%) followed by D. stramonium (79.58%). In comet assay test, at highest concentration (25%) the mean comet tail lengths represented by Penganum harmala, Datura stramonium and Azadirachta indica (Reference plant) were 10.20 ±â€¯0.49, 9.20 ±â€¯0.37 and 7.80 ±â€¯0.49 µm while percent DNA damage was 10.56 ±â€¯0.77, 10.67 ±â€¯1.62 and 8.11 ±â€¯0.85% respectively compared to controls cells. Phytochemical analysis indicated the presence of flavonoids, steroids, saponins, cardiac glycosides, tannins, alkaloids, terpenoids and anthraquinones. Fourier Transform Infrared spectroscopy (FTIR) analysis revealed the presence of phenolic flavonoids, saponins, tannins as major functional groups. Further studies are needed to explore and thus, to incorporate weed plant extracts for the management of house flies.

Comprehensive facts on dynamic antimicrobial properties of polysaccharides and biomolecules-silver nanoparticle conjugate.

Based on progress for the green synthesis of nanoparticle (NPs), the mushrooms have also been utilized extensively for the biogenic synthesis of NPs. In recent years, silver NPs have been fabricated using mushrooms. The antimicrobial drugs are efficient to control the infectious diseases, but due to widespread of drugs, microbes became resistant to drugs, which demands develop of new bioactive agents. The silver NPs have been recognized as efficient broad spectrum antimicrobial agents, which have been fabricated using polysaccharides from mushrooms as reducing and capping agent. This review focused on the comprehensive study that deals silver NPs polysaccharides from Pleurotus mushroom, their synthesis mechanism, action mechanism of silver NPs and their characterization using advanced techniques i.e., ultraviolet-visible (UV-Vis), dynamic light scattering, Fourier transformation infrared spectroscopy (FT-IR), scanning electron microscopy (SEM), energy dispersive spectroscopy (EDS), transmission electron microscopy (TEM) and XRD. The Pleurotus mushroom showed promising efficiency for the biogenic synthesis of polysaccharides­silver NPS and as-prepared NPs showed excellent antimicrobial activity.

Maternal protein restriction induces gastrointestinal dysfunction and enteric nervous system remodeling in rat offspring.

Early-life adversity is a major risk factor for the development of diseases later in life. Maternal protein restriction (MPR) is associated with morbidities in offspring affecting multiple organs, but its impact on the gastrointestinal (GI) tract remains poorly studied. Using a rat model, we examined the consequences of MPR on GI function and on the enteric nervous system (ENS) in the offspring at postnatal d 35 under basal state and following a water avoidance stress (WAS). Compared with control rats, MPR rats exhibited greater colonic motility, permeability, and corticosteronemia. In contrast to controls, MPR rats presented a blunted functional and corticosteronemic response to WAS. Furthermore, MPR rats showed an increased proportion of choline acetyltransferase-immunoreactive (ChAT-IR) neurons and a reduced level of autophagy in colonic myenteric neurons. In ENS cultures, corticosterone treatment increased the proportion of ChAT-IR neurons and reduced autophagy level in enteric neurons. Inhibition of autophagy in ENS cultures resulted in a higher vulnerability of enteric neurons to a cellular stress. Altogether, this study suggests that MPR induced GI dysfunction and ENS alterations in offspring rats and that MPR-induced increased corticosteronemia might be involved in ENS remodeling and altered responsiveness of the gut to stressors later in life.-Aubert, P., Oleynikova, E., Rizvi, H., Ndjim, M., Le Berre-Scoul, C., Grohard, P. A., Chevalier, J., Segain, J.-P., Le Drean, G., Neunlist, M., Boudin, H. Maternal protein restriction induces gastrointestinal dysfunction and enteric nervous system remodeling in rat offspring.

Cadmium stress in rice: toxic effects, tolerance mechanisms, and management: a critical review.

Cadmium (Cd) is one of the main pollutants in paddy fields, and its accumulation in rice (Oryza sativa L.) and subsequent transfer to food chain is a global environmental issue. This paper reviews the toxic effects, tolerance mechanisms, and management of Cd in a rice paddy. Cadmium toxicity decreases seed germination, growth, mineral nutrients, photosynthesis, and grain yield. It also causes oxidative stress and genotoxicity in rice. Plant response to Cd toxicity varies with cultivars, growth condition, and duration of Cd exposure. Under Cd stress, stimulation of antioxidant defense system, osmoregulation, ion homeostasis, and over production of signaling molecules are important tolerance mechanisms in rice. Several strategies have been proposed for the management of Cd-contaminated paddy soils. One such approach is the exogenous application of hormones, osmolytes, and signaling molecules. Moreover, Cd uptake and toxicity in rice can be decreased by proper application of essential nutrients such as nitrogen, zinc, iron, and selenium in Cd-contaminated soils. In addition, several inorganic (liming and silicon) and organic (compost and biochar) amendments have been applied in the soils to reduce Cd stress in rice. Selection of low Cd-accumulating rice cultivars, crop rotation, water management, and exogenous application of microbes could be a reasonable approach to alleviate Cd toxicity in rice. To draw a sound conclusion, long-term field trials are still required, including risks and benefit analysis for various management strategies.

Influence of Pseudomonas aeruginosa as PGPR on oxidative stress tolerance in wheat under Zn stress.

Plant Growth Promoting Rhizobacteria (PGPR), whose role is still underestimated, plays an important (or perhaps essential) role in improving plant growth. The comprehensive understanding of bacterial plant growth promoting mechanism helps to get sustainable agriculture production under biotic and abiotic stresses. In the present study, plant growth promoting (PGP) bacterial strain Pseudomonas aeruginosa having maximum inhibitory concentration of 1500mg kg(-1) against Zn was isolated from arable land, irrigated with industrial effluent and evaluated to determine it bioremediation potential. The study was mainly focused on plant biomass production, nutrient uptake and oxidative stress tolerance in relation to the activities of antioxidative enzymes and the content of non-enzymatic antioxidants. The oxidative stress tolerance was measured by estimating the MDA accumulation as well as H2O2 production in wheat plants under Zn (1000mg kg(-1)) stress and inoculation of soil with Zn resistant Pseudomonas aeruginosa. Zn in rooting medium reduced the plant growth, leaf photosynthetic pigments as well as uptake of N and P. However, content of MDA and H2O2 increased at higher concentration of Zn. Inoculation of P. aeruginosa improved the uptake of P and N in wheat plants with an increase in leaf chlorophyll, total soluble protein and plant biomass production. Analysis of plant root and shoot disclosed that Zn concentration was significantly lowered in P. aeruginosa inoculated zinc stressed plants as compare to the plants grown under Zn stress only. The amelioration of adverse effects of Zn stress on biomass production due to P. aeruginosa inoculation was related with enhanced antioxidative enzyme activities (SOD, POD and CAT), and the contents of non-enzymatic components such as ascorbic acid and total phenolics (TPC) as compare to Zn-treated plants. The up-gradation in antioxidative defense mechanism, resulted a reduction in H2O2 and MDA content due to the scavenging of ROS by antioxidants. It was concluded that P. aeruginosa is an ideal candidate for bioremediation and wheat growth promotion against Zn-induced oxidative stress by improving the availability of necessary nutrient, eliciting antioxidant defense system as well as by lowering the Zn metal uptake.

Awareness and prevention of osteoporosis among South Asian women.

We examined awareness of osteoporosis prevention among peri- and post-menopausal South Asian women attending two community centers in the Dallas/Fort-Worth Metroplex. We conducted a quasi-experimental study (final N = 61) assessing knowledge about osteoporosis among South Asian women (>or=40 years). The mean age was 52.3 years (SD = 8.72). Over 50% were college educated and 64% had no health insurance. We administered a baseline knowledge test, followed by a health education intervention and, 2 weeks later, by a post-test. Participants received one point for each correct answer and scores were added (