Diagnosis of Paroxysmal Nocturnal Hemoglobinuria: Recent Advances

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder with its protean clinical manifestations. This is due to partial or complete absence of ‘glycophosphatidyl-inositol-anchor proteins’ (GPI-AP). The main aim of this review is to highlight various diagnostic modalities available, basic principle of each test and recent advances in the diagnosis of PNH. Recently among various tests available, the flow cytometry has become ‘the gold standard’ for PNH testing. In order to overcome the difficulties encountered by the testing and research laboratories throughout the world, International Clinical Cytometry Society has come up with guidelines regarding the indications for testing, protocol for sample collection, processing, panel of antibodies as well as gating strategies to be used, how to interpret the test and reporting format to be used. It is essential to test at least two GPI-linked markers on at least two different lineages particularly on red cells and granulocytes/monocytes. The fluorescent aerolysin combined with other monoclonal antibodies in multicolour flow cytometry offered an improved assay not only for diagnosis but also for monitoring of PNH clones. It is equally important to diagnose this rare entity with high index of suspicion.     

Recent progress and prospects in the electrode materials of flexible sodium-ion battery

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Pilot study of salivary butyrylcholinesterase,phosphodiesterase, thiols and cerulopalsmin in auditory neuropathy

ObjectiveTo estimate butyrylcholinesterase (BChE), phosphodiesterase (PDE), thiols and cerulopalsmin by non – invasive means in saliva a of subjects (both cases and controls) and correlated to their hearing sensitivity.MethodsTotal of 13 subjects participated in this study. Among them 7 were having auditory neuropathy and 6 were healthy controls. Unstimulated saliva (10 ml) was collected from each participant. Ceruloplasmin, thiols, phosphodiesterase and pseudocholinesterase were estimated by colorimetric method in the salivary samples.ResultsSalivary BChE and PDE levels were marginally elevated and protein thiols were marginally decreased in cases as compared to that of controls. Salivary ceruloplasmin was significantly decreased (p = 0.022) in cases as compared to that of controls.ConclusionsSaliva can be used as a potential noninvasive tool for evaluation of disorders.     

Cranial melioidosis with extradural extension after a fall in the bathroom

A 32-year-old diabetic male, with a past history of head injury and seizures, presented with a painful swelling over his forehead present for the past three months. Cranial MRI demonstrated the presence of a scalp collection with extradural extension through a bony defect. Biopsy from the area showed caseating necrosis suggestive of tuberculosis. Although the patient failed to return for initiation of anti-tubercular therapy for the next 11 months, the swelling did not progress, and there were no constitutional symptoms. The indolent nature of the swelling prompted re-evaluation and delayed cultures of pus from the collection grew Burkholderia pseudomallei.     

HIV–tuberculosis co–infection in an Indian scenario: The role of associated evidence of immunosuppression

ObjectiveTo determine the relationship between tuberculosis and the degree of immunosuppression as determined by CD4 count. The impact of immunosuppression on the severity of tuberculosis was also studied.MethodsA retrospective analysis was performed in patients newly diagnosed with HIV infection and antiretroviral therapy (ART)-naive patients with known HIV seropositivity. All patients were diagnosed with active tuberculosis between January 2008 and December 2010, based on review of their medical records. Patients on chemoprophylaxis for opportunistic infection were excluded. Pattern and severity of tuberculosis, associated stigmata of immunosuppression, and CD4 counts were noted.ResultsOf 140 patients satisfying the inclusion criteria, 52 had mild tuberculosis with no other evidence of immunosuppression, 52 had tuberculosis of variable severity with associated evidence of immunosuppression, and 36 had severe tuberculosis with no other evidence of immunosuppression. The CD4 count was highest in the first group [(109.2±99.9) cells/μL] and least in the second group [(58.4±39.8) cells/μL], and the difference was statistically significant (P=0.004). No statistical difference was observed in the CD4 count between those with mild tuberculosis and those with severe tuberculosis.ConclusionsIn developing countries with a high prevalence of tuberculosis in the general population, the possibility of incidental tuberculosis in patients with HIV should always be considered. CD4 count does not appear to influence the severity of tuberculosis. The presence of concomitant evidence of immunosuppression in the form of category B and C conditions is indicative of underlying immunosuppression and associated with a significantly lower CD4 count.     

Lipoprotein(a) as an independent risk factor for coronary artery disease in patients below 40 years of age

Coronary artery disease has assumed alarming proportions in Indians and often affects people at younger age. Traditional risk factors fail to explain the high incidence of disease. Although lipoprotein(a) has been shown to be a powerful risk factor for atherosclerosis, there is very limited data with regard to its significance in premature coronary artery disease. The present study was therefore undertaken to assess lipoprotein(a) levels and its role as a marker of coronary artery disease in patients below the age of 40 years. Lipid profile and lipoprotein(a) levels were estimated in 50 patients of angiographically proven coronary artery disease and an equal number of age-matched healthy controls. There was no significant difference in the family history of coronary artery disease, body mass index and waist-hip ratio between the two groups. Total plasma cholesterol, triglyceride and LDL-cholesterol levels were significantly higher and HDL-cholesterol significantly lower in patients as compared to controls. In patients of coronary artery disease, mean lipoprotein(a) levels, measured by ELISA method, were 35.0 +/- 32.4 mg/dL and the median was 26.7 mg/dL. These values were significantly higher than the mean of 20.3 +/- 17.0 mg/dL (p < 0.002) and the median of 13.8 mg/dL (p < 0.015) in controls. Multiple regression analysis, to assess the influence of various risk factors, showed that low HDL-cholesterol (odds ratio 4.62, 95% CI 1.84-11.60; p < 0.015) and elevated lipoprotein(a) levels (odds ratio 3.06, 95% CI 1.24-7.55; p < 0.001) were independent risk factors, whereas high total cholesterol and triglyceride levels did not have any independent influence on premature coronary artery disease. Our data thus suggest that lipoprotein (a) levels are elevated and constitute an independent risk factor in patients with premature coronary artery disease below 40 years of age.