Gamma glutamyl transferase as an atherogenic predictive marker in acute coronary syndrome

Background: Acute coronary syndrome (ACS) remains a leading cause of mortality and morbidity world-wide. Atherosclerosis is the predominant cause of ACS and biomarkers that could detect vulnerable atherosclerotic plaque could potentially be of great value in identifying patients at risk of developing coronary events. The aim was to assess the role of gamma-glutamyl transferase (GGT) in atherosclerosis process. The objective was to compare serum levels of GGT in patients with ACS and control subjects, and also to find out the association between GGT and atherogenic risk factors such as diabetes mellitus, hypertension, dyslipidemia and smoking.Methods: The design was a prospective case control study where a total of 151 patients, 100 ACS patients and 51 control subjects with the age group of 30-80 years were enrolled for the study. GGT was estimated by kinetic colour test using Beckman Coulter AU2700 analyser.Results: The mean GGT levels of ST-segment elevation myocardial infarction (STEMI), non-ST-segment elevation myocardial infarction (NSTEMI) and unstable angina (UA) subgroups were 93.86, 87.87 and 29.27 U/L respectively, which showed statistical significant difference (p<0.001) when compared with control subjects 21.99 U/L. The higher GGT levels in ACS patients also correlated with angiographic diagnosis of atherosclerosis. No significant difference was noted in GGT levels among ACS subgroups having risk factors and without having risk factors. Conclusions: Significantly higher GGT levels found in ACS patients reflects the burden of atherosclerotic changes and this association implies that GGT estimation can be used as an adjuvant biomarker that may help in identifying patients who are potentially at risk of coronary atherosclerosis

A REVIEW ON POLYCORIA

The presence of multiple iris holes is most commonly called&nbsp;pseudopolycoria, because only the central pupil has a sphincter muscle and can constrict. Iris holes without muscle tissue arise as congenital defects or develop from ocular trauma, inflammation, or degeneration and are thus not considered to be true accessory pupils. Multiple pupils, also known as&nbsp;true polycoria, are distinguished by the presence of a sphincter muscle around each iris hole, permitting synchronous constriction and dilation of the 2 pupils.&nbsp;One presumed mechanism for polycoria is a snaring or pinching off from the margin of another pupil. This patient had a small, oval-shaped accessory pupil, which may have separated from the temporal margin of the central pupil, leaving a thin bridge of no sphincter connecting tissue. The outlook for polycoria is generally good. You may not require any treatment if your visual impairment is minimal and doesn’t interfere with your daily life. However, if treatment is needed, pupilloplasty has so far shown positive results. If you have polycoria, it’s important to have regular check-ups with an eye doctor to monitor your vision and any changes your eyes may have. Having your eye checked regularly is also beneficial for your eyesight as a whole

Prognostic importance of the 6 min walk test in light chain (AL) amyloidosis

OBJECTIVES: In AL amyloidosis, organ response assessment is based on surrogates (eg, cardiac biomarkers). An objective functional test, such as the 6 min walk test (6MWT), capturing overall clinical improvement, is required. We aimed to evaluate the prognostic impact of the 6MWT at baseline and change following chemotherapy. METHODS: This study evaluated the outcomes of patients who enrolled in a prospective observational study at the UK National Amyloidosis Centre (2012-2017). Patients underwent comprehensive assessments inclusive of blood testing, echocardiogram and 6MWT at baseline and annually thereafter. RESULTS: In total, 799 patients were included within the study. Median baseline 6 min walk distance (6MWD) was 362 m (IQR: 231 m). 6MWD progressively decreased with worsening cardiac disease stage (458 m, 404 m, 331 m and 168 m for cardiac Mayo stages I, II, IIIa and IIIb, respectively (p<0.0001)). In patients with a baseline 6MWT of ≥350 m, the median overall survival was not reached (vs 30.0 (95% CI 23.2 to 36.8) months if <350 m and 5.0 (95% CI 2.8 to 7.2) months if unable to attempt 6MWT (p<0.0001). Following chemotherapy, only patients in a complete haematological response improved their 6MWD by 12 months (p=0.001). Improvement in 6MWD prolonged survival in patients with cardiac amyloidosis (p=0.005). CONCLUSION: The 6MWT is prognostic in AL amyloidosis. A baseline distance of ≥350 m independently predicts better survival. These data suggest that 6MWT has utility in AL amyloidosis for baseline prognosis and assessing response

The experience of hereditary apolipoprotein A-I amyloidosis at the UK National Amyloidosis Centre

INTRODUCTION: Hereditary apolipoprotein A-I (AApoAI) amyloidosis is a rare heterogeneous disease with variable age of onset and organ involvement. There are few series detailing the natural history and outcomes of solid organ transplantation across a range of causative APOA1 gene mutations. METHODS: We identified all patients with AApoAI amyloidosis who presented to the National Amyloidosis Centre (NAC) between 1986 and 2019. RESULTS: In total, 57 patients with 14 different APOA1 mutations were identified including 18 patients who underwent renal transplantation (5 combined liver-kidney (LKT) and 2 combined heart-kidney (HKT) transplants). Median age of presentation was 43 years and median time from presentation to referral was 3 (0-31 years). Involvement of the kidneys, liver and heart by amyloid was detected in 81%, 67% and 28% of patients, respectively. Renal amyloidosis was universal in association with the most commonly identified variant (Gly26Arg, n = 28). Across all variants, patients with renal amyloidosis had a median creatinine of 159 µmol/L and median urinary protein of 0.3 g/24 h at the time of diagnosis of AApoAI amyloidosis and median time from diagnosis to end-stage renal disease was 15.0 (95% CI: 10.0-20.0) years. Post-renal transplantation, median allograft survival was 22.0 (13.0-31.0) years. There was one early death following transplantation (infection-related at 2 months post-renal transplant) and no episodes of early rejection leading to graft failure. Liver transplantation led to regression of amyloid in all four cases in whom serial 123I-SAP scintigraphy was performed. CONCLUSIONS: AApoAI amyloidosis is a slowly progressive disease that is challenging to diagnose. The outcomes of transplantation are encouraging and graft survival is excellent

Longitudinal strain is an independent predictor of survival and response to therapy in patients with systemic AL amyloidosis

AIMS: Cardiac involvement, a major determinant of prognosis in AL (light-chain immunoglobulin) amyloidosis, is characterized by an impairment of longitudinal strain (LS%). We sought to evaluate the utility of LS% in a prospectively observed series of patients. METHODS AND RESULTS: A total of 915 serial newly diagnosed AL patients with comprehensive baseline assessments, inclusive of echocardiography, were included. A total of 628/915 (68.6%) patients had cardiac involvement. The LS% worsened with advancing cardiac stage with mean −21.1%, −17.1%, −12.9%, and −12.1% for stages I, II, IIIa, and IIIb, respectively (P < 0.0001). There was a highly significant worsening of overall survival (OS) with worsening LS% quartile: LS% ≤−16.2%: 80 months, −16.1% to −12.2%: 36 [95% confidence interval (CI) 20.9–51.1] months, −12.1% to −9.1%: 22 (95% CI 9.1–34.9) months, and ≥−9.0%: 5 (95% CI 3.2–6.8) months (P < 0.0001). Improvement in LS% was seen at 12 months in patients achieving a haematological complete response (CR) (median improvement from −13.8% to −14.9% in those with CR and difference between involved and uninvolved light chain <10 mg/L). Strain improvement was associated with improved OS (median not reached at 53 months vs. 72 months in patients without strain improvement, P = 0.007). Patients achieving an LS% improvement and a standard N-terminal pro-brain natriuretic peptide-based cardiac response survived longer than those achieving a biomarker-based cardiac response alone (P < 0.0001). CONCLUSION: Baseline LS% is a functional marker that correlates with worsening cardiac involvement and is predictive of survival. Baseline LS% and an absolute improvement in LS% are useful additional measures of prognosis and response to therapy in cardiac AL amyloidosis, respectively

5,5&#8242;-Bis(benzyloxy)-2,2&#8242;-[hydrazinediylidenebis(methanylylidene)]diphenol

The title azine molecule, C28H24N2O4, lies about a center of inversion. The dihedral angle between the phenyl ring and the hydroxy-substituted ring is 70.3&#8197;(5)&#176;. The phenolic O&#8211;H group forms an intramolecular hydrogen bond to the azine N atom

Crystal structure of 2-[(E)-4-benzyloxy-2-hydroxybenzylidene]-N-cyclohexylhydrazinecarbothioamide acetonitrile hemisolvate

The asymmetric unit of the title compound, C21H25N3O2S·0.5C2H3N, contains two independent molecules with almost similar structural properties along with a solvent molecule of acetonitrile. The compound exists in the E conformation with respect to the azomethine C=N double bond. The hydrazinecarbothioamide moieties in both independent molecules are almost planar [maximum deviations of 0.013 (2) and 0.007 (2) Å]. The molecular conformation is stabilized in each case by an intramolecular N—H...N hydrogen bond. In the crystal, pairs of N—H...S hydrogen bonds link each of the independent molecules into inversion dimers. The dimers are interconnected by means of three C—H...π interactions

(E)-2-(4-Benzyloxy-2-hydroxybenzylidene)-N-methylhydrazinecarbothioamide

The molecule of the title compound, C16H17N3O2S, adopts an E conformation with respect to the azomethine C=N bond. The hydrazinecarbothioamide fragment is close to planar, with a largest deviation from the least-squares plane of 0.079 (2) Å for the hydrazide N atom. This fragment forms a dihedral angle of 9.43 (9)° with the central benzene ring. The benzene rings are inclined to one another by 67.55 (12)°. The molecular conformation is stabilized by an intramolecular O—H...N hydrogen bond involving the azomethine N atom. In the crystal, molecules are linked through weak N—H...S and N—H...O hydrogen bonds into double ribbons along [010]. The crystal packing also features C—H...π interactions