Wilson\u27s disease: experience at a tertiary care hospital.

Wilson\u27s disease (WD) is a rare autosomal recessive disorder of copper metabolism. Data regarding WD is not available from Pakistan. A cross-sectional study was conducted at The Aga Khan University Hospital, Karachi, and all patients admitted with primary and secondary diagnosis of Wilson\u27s disease were added. A total of 47 patients were seen; 68% (n = 32) were male. The mean age was 26.6 ± 9.97 years. Most of the patients presented with hepatic, (n = 22, 46.8%), neurological, (n = 17, 36.2%) and psychiatric (n = 8, 17%) symptoms. Mean ceruloplasmin level was 0.17 ± 0.13 g/dl; it was \u3c 0.25 g/dl in 39 (86.6%) patients. Serum copper (Cu) was reduced in 32 (68.1%) patients and 24-hr-urinary Cu was raised in 22 (47.6%) patients. Slit lamp examination for Kayser-Fleischer (KF) rings was done on 15 (31.9%) patients and 9 (60%) of them had KF rings. Mean serum aspartate transaminase (AST) / alanine transaminases (ALT) ratio was 1.92 and median alkaline phosphatase / total bilirubin ratio was 79.30 (IQR 35.05; 166.50)

Relationships between HI Gas Mass, Stellar Mass and Star Formation Rate of HICAT+WISE (HI-WISE) Galaxies

We have measured the relationships between HI mass, stellar mass and star formation rate using the HI Parkes All Sky-Survey Catalogue (HICAT) and the Wide-field Infrared Survey Explorer (WISE). Of the 3,513 HICAT sources, we find 3.4 micron counterparts for 2,896 sources (80%) and provide new WISE matched aperture photometry for these galaxies. For our principal sample of spiral galaxies with W1 $\le$ 10 mag and z $\le$ 0.01, we identify HI detections for 93% of the sample. We measure lower HI-stellar mass relationships that HI selected samples that do not include spiral galaxies with little HI gas. Our observations of the spiral sample show that HI mass increases with stellar mass with a power-law index 0.35; however, this value is dependent on T-type, which affects both the median and the dispersion of HI mass. We also observe an upper limit on the HI gas fraction, which is consistent with a halo spin parameter model. We measure the star formation efficiency of spiral galaxies to be constant 10$^{-9.57}$ yr$^{-1}$ $\pm$ 0.4 dex for 2.5 orders of magnitude in stellar mass, despite the higher stellar mass spiral showing evidence of quenched star formation.Comment: 20 pages, 13 figures; accepted for publication by Ap

Severity of acute hepatitis and its outcome in patients with dengue fever in a tertiary care hospital Karachi, Pakistan (South Asia)

Background: Liver injury due to dengue viral infection is not uncommon. Acute liver injury is a severe complicating factor in dengue, predisposing to life-threatening hemorrhage, Disseminated Intravascular Coagulation (DIC) and encephalopathy. Therefore we sought to determine the frequency of hepatitis in dengue infection and to compare the outcome (length of stay, in hospital mortality, complications) between patients of Dengue who have mild/moderate (ALT 23-300 IU/L) v/s severe acute hepatitis (ALT \u3e 300 IU/L). Methods: A Cohort study of inpatients with dengue viral infection done at Aga Khan University Hospital Karachi. All patients (≥ 14 yrs age) admitted with diagnosis of Dengue Fever (DF), Dengue Hemorrhagic Fever (DHF) or Dengue Shock Syndrome (DSS) were included. Chi square test was used to compare categorical variables and fischer exact test where applicable. Survival analysis (Cox regression and log rank) for primary outcome was done. Student t test was used to compare continuous variables. A p value of less than or equal to 0.05 was taken as significant. Results: Six hundred and ninety nine patients were enrolled, including 87% (605) patients with DF and 13% (94) patients with DHF or DSS. Liver functions tests showed median ALT of 88.50 IU/L; IQR 43.25-188 IU/L, median AST of 174 IU/L; IQR 87-371.5 IU/L and median T.Bil of 0.8 mg/dl; IQR 0.6-1.3 mg/dl. Seventy one percent (496) had mild to moderate hepatitis and 15% (103) had severe hepatitis. Mean length of stay (LOS) in patients with mild/moderate hepatitis was 3.63 days v.s 4.3 days in those with severe hepatitis (P value 0.002). Overall mortality was 33.3% (n = 6) in mild/moderate hepatitis vs 66.7% (n = 12) in severe hepatitis group (p value \u3c 0.001). Cox regression analysis also showed significantly higher mortality in severe hepatitis group (H.R (4.91; 95% CI 1.74-13.87 and P value 0.003) and in DHF/DSS (5.43; CI 1.86-15.84 and P value 0.002). There was a significant difference for the complications like Bleeding (P value \u3c 0.001), Acute Renal failure (ARF) (P value 0.002), Acalculus cholecystitis (P value 0.04) and encephalopathy (P value 0.02) in mild/moderate and Severe hepatitis groups respectively. Conclusion: Severe hepatitis (SGPT\u3e300IU) in Dengue is associated with prolonged LOS, mortality, bleeding and RF

HI galaxies with little star formation: an abundance of LIERs

We present a sample of 91 HI galaxies with little or no star formation and discuss the analysis of the integral field unit (IFU) spectra of 28 of these galaxies. We identified HI galaxies from the HI Parkes All-Sky Survey Catalog (HICAT) with Wide-field Infrared Survey Explorer (WISE) colours consistent with low specific star formation (< 10$^{-10.4}$ yr$^{-1}$), and obtained optical IFU spectra with the Wide-Field Spectrograph (WiFeS). Visual inspection of the PanSTARRS, Dark Energy Survey, and Carnegie-Irvine imaging of 62 galaxies reveals that at least 32 galaxies in the sample have low levels of star formation, primarily in arms/rings. New IFU spectra of 28 of these galaxies reveal 3 galaxies with central star formation, 1 galaxy with low-ionisation nuclear emission-line regions (LINERs), 20 with extended low-ionisation emission-line regions (LIERs) and 4 with high excitation Seyfert (Sy) emission. From the spectroscopic analysis of HI-selected galaxies with little star formation, we conclude that 75% of this population are LINERs/LIERs

Temperatures and Non-ideal Expansion in Ultrarelativistic Nucleus-Nucleus Collisions

The hadronic phase space distributions calculated with the transport model RQMD for central S(200 AGeV) on S and Pb(160AGeV) on Pb collisions are analyzed to study the deviations from ideal hydrodynamical evolution. After the preequilibrium stage, which lasts for approximately 4 (2) fm/c in Pb+Pb (S+S) the source stays in approximate kinetic equilibrium for about 2 fm/c at a temperature close to 140 MeV. The interactions of mesons last until around 14 (5) fm/c during which time strong transverse flow is generated. The interactions in the hadronic resonance gas are not sufficiently strong to maintain ideal fluid expansion. While pions acquire average transverse fluid velocities around 0.47-0.58 c, heavier particles like protons and kaons cannot keep up with the pionic fluid, since their average velocities are smaller by about 20 to 30 \%. Although kinetic equilibrium breaks down in the final dilute stage of $AA$ collisions, the system resembles a thermal system at a temperature of 130 MeV, if the free streaming of hadrons after freeze-out is suppressed. This freeze-out temperature is consistent with estimates based on mean free paths and expansion rates in a thermal fireball but lower than values derived from fits to measured particle ratios and transverse momentum spectra. The processes in RQMD to which the differences can be attributed to are the non-ideal expansion of the hadronic matter and the absence of chemical equilibrium at freeze-out.Comment: 12 pages + 3 postscript figures (uuencoded and included

Bilateral Ocular Myositis Associated with Whipple's Disease

Purpose: To describe the clinical features of a Caucasian female patient with a history of treated gastrointestinal Whipple's disease (WD) who developed new-onset diplopia, with a description of the histopathological features of the extraocular muscle biopsies. Methods: A previously fit 38-year-old Caucasian female presented with acute-onset diplopia after being on a sustained medication regime for biopsy-proven gastrointestinal WD. A magnetic resonance imaging scan of her orbits with gadolinium revealed diffuse enhancement of the bellies of the extraocular muscles bilaterally, particularly the medial rectus, superior rectus, and superior oblique muscles, consistent with an infiltrative myositis. She underwent unilateral extraocular muscle biopsies. Results: The extraocular muscle biopsies contained macrophages between the muscle fibres. These contained periodic acid-Schiff-positive cytoplasmic granules. Immunohistochemistry with an antibody raised to Tropheryma whipplei showed positive staining of the same macrophages. Transmission electron microscopy confirmed the presence of effete T. whipplei cell membranes in lysosomes. Conclusion: This case describes bilateral WD-associated extraocular muscle myositis. The exact mechanism for this unusual presentation is unclear, but both a WD-associated immune reconstitution inflammatory syndrome and treatment failure are possibilities, with a good response observed to antibiotic therapy and adjunctive corticosteroids

SCREENING OF VARIOUS BIVOLTINE HYBRIDS OF SILKWORM BOMBYX MORI L. FOR DISEASE SUSCEPTIBILITY DURING MONSOON SEASON IN UTTAR PRADESH

ABSTRACT Bombyx mori has long been reared as a beneficial insect in the sericulture industry and as an experimental laboratory insect. In tropical countries like India, grasserie, (BmNPV) spreads by the occultation bodies (OBs) in the blood cell of infected silkworm, usually high temperature and humidity prevalent in tropical regions is conductive to proliferation of polyhedrosis disease. It is known to occur in all larval instars during all seasons causing 20-50% cocoon crop losses in India. In the present study, four promising bivoltine hybrids will be screened for their susceptibility to BmNPV and BmIFV, Muscardine and other diseases and the susceptibility status has been compared across monsoon season of Uttar Pradesh. This will help in selecting most promising race for monsoon season of which will be least susceptibility to different Silkworm diseases Viz., Grasserie, Bacterial flacherie, Muscardine and % other diseases (DNV, CPV, IFV, Sotto disease) and farmer will face less difficulty during silkworm rearing and will have no fear of attack of diseases. It will lead to further adoption and spread of Sericulture in other districts of Uttar Pradesh