Spectrum of Secondary Glomerular Diseases in Adult Nephrotic Syndrome.

Background: In developing countries like India, thecauses for end stage renal failure has been changingwith glomerular diseases occupying an important place.The trend of various primary and secondary causes ofNephrotic Syndrome (NS) over the past few decadeshas been debatable especially with reference togeographical locations. Aim and Objectives: This studywas intended to find out the histologic patterns ofglomerular disease associated with NS in South Indiaand analyze the corresponding clinical and biochemicalabnormalities associated with these conditions.Material and Methods: One hundred eighty eightpatients in the age group of 18-80 years with NS whounderwent renal biopsy between the periodst thfrom1 January 2018 to 30 September 2019 wereincluded in this study. Baseline investigations and otherinvestigations related to renal profile evaluationincluding complement levels and HIV antibody weredone. Results: Focal Segmental Glomerulosclerosis(FSGS) accounted for the majority (25.5%) amongprimary glomerular diseases while among thesecondary glomerular diseases Lupus Nephritis (LN)(51.19%) constituted the majority and a substantialcontribution from Human Immunodeficiency VirusAssociated Nephropathy (HIVAN). LN was seen onlyin female patients with a mean age of 33.36 ± 10.74years. Among LN, Class III was the most predominantwhile Class IIIC was less frequent. C3 and C4complement levels were less than normal in Class IVand Class variants of LN. Conclusion: This studyhighlights the spectrum of Lupus Nephritis and HIVassociated nephropathy as a cause for adult NS in atertiary care center in South India

Cardiac magnetic resonance in tropical endomyocardial fibrosis

Background: Endomyocardial fibrosis has varied presentatation and difficult to diagnose. Aim to elucidate the role of Cardiac Magnetic Resonance (CMR) imaging in the evaluation of Endomyocardial Fibrosis (EMF) and to devise diagnostic criteria for the disease.Methods: Retrospective analysis of cases of restrictive cardiomyopathy referred for Magnetic resonance imaging over a period of 5 years. All patients underwent 1.5 T CMR imaging (Magnetom Avanto, Siemens, Germany) with standard cardiomyopathy protocol. Criteria for diagnosis of RCM included normal sized ventricles, normal/reduced systolic function, uni-/bi-atrial enlargement, normal pericardium and absent septal bounce. Cases diagnosed as EMF on CMR were included in this study. Statistical analysis performed using SPSS software.Results: EMF was diagnosed in 20 patients (31%) [12 males; age 39±18 years]. Ten patients had Right Centricular (RV) EMF, 3 had Left Ventricular (LV) EMF, while 7 had bi-ventricular EMF. Oedema indicating ongoing inflammation was seen in 4 (20%) cases. Apical thrombus was seen in 8(40%) cases and was present in 35% cases of RV and 20% cases of LV involvement. Subendocardial delayed enhancement was always present in the involved ventricle. The RV apex was obliterated in 100% of patients with RV EMF, while LV apex was similarly obliterated in 66% cases with LV disease. Mild-moderate pericardial effusion was observed in 8 patients. On the basis of CMR findings, the disease was classified as early necrotic phase in 1, thrombotic necrotic in 4 and late fibrotic phase in 13 and of different stages in ventricles in 2 cases.Conclusions: EMF was the commonest cause of RCM in this series. Major diagnostic criteria of EMF on CMR include subendocardial delayed enhancement and apical obliteration. Oedema and thrombus are variable findings, depending on disease severity.

Clinical and angiographic profiles and six months outcomes of smokers with acute ST segment elevation myocardial infarction undergoing primary percutaneous coronary angioplasty

Background Outcomes of primary percutaneous coronary intervention (PCI) for acute STEMI (ST-segment elevation myocardial infarction) in smokers are expected to be better than non-smokers as for patients of acute STEMI with or without fibrinolytic therapy. Objectives This comparative study was designed to evaluate the outcomes of primary PCI in patients with acute STEMI in smokers and non-smokers. Clinical and angiographic profile of the two groups was also compared. Methods Over duration of two year, a total of 150 consecutive patients of acute STEMI eligible for primary PCI were enrolled and constituted the two groups [Smokers (n = 90), Non-smokers (n = 60)] of the study population. There was no difference in procedure in two groups. Results In the present study of acute STEMI, current smokers were about a decade younger than non-smokers (p value = 0.0002), majority were male (98.9% vs 56.6%) were male with a higher prevalence of hypertension and diabetes mellitus (61.67% vs 32.28% and 46.67% vs 14.44%, p = 0.001) respectively. Smokers tended to have higher thrombus burden (p = 0.06) but less multi vessel disease (p = 0.028). Thirty day and six month mortality was non-significantly higher in smokers 4.66% vs 1.33% (p = 0.261) and 5.33% vs 2.66% (p = NS) respectively. Rate of quitting smoking among smokers was 80.90% at 6 months. Conclusion The study documents that smokers with acute STEMI have similar outcomes as compared to non smokers with higher thrombus burden and lesser non culprit artery involvement. Smokers present at much younger age emphasizing the role of smoking cessation for prevention of myocardial infarction.

Management protocols for chronic heart failure in India

Heart failure is a common clinical syndrome and a global health priority. The burden of heart failure is increasing at an alarming rate worldwide as well as in India. Heart failure not only increases the risk of mortality, morbidity and worsens the patient’s quality of life, but also puts a huge burden on the overall healthcare system. The management of heart failure has evolved over the years with the advent of new drugs and devices. This document has been developed with an objective to provide standard management guidance and simple heart failure algorithms to aid Indian clinicians in their daily practice. It would also inform the clinicians on the latest evidence in heart failure and provide guidance to recognize and diagnose chronic heart failure early and optimize management.

Ocular infections caused by Scedosporium apiospermum: A case series

The aim of our retrospective study is to report a case series of ocular infections caused by a rare fungus, Scedosporium apiospermum, in a South Indian population. Thirteen cases of culture-positive S. apiospermum infections diagnosed between January 2011 and March 2016 were included in this study. The parameters evaluated were predisposing factors, treatment and final clinical outcome. The most common mode of presentation was keratitis (84.6%) followed by sclerokeratitis (15.3%). The predisposing factors involved were unspecified foreign body injury (30.7%), organic matter injury (15.3%), uncontrolled diabetes (7.6%), and recent manual small-incision cataract surgery (7.6%). Five cases (38.46%) had no predisposing factor. Of the 11 keratitis cases, nine (69.2%) responded well to combination medical therapy while one case (7.6%) required therapeutic keratoplasty. One case was lost to follow-up. Both cases which presented with sclerokeratitis showed no response to medico-surgical treatment progressing to panophthalmitis and evisceration.

Maculopapular rash presentation of febrile illness in an adult with Varicella zoster virus infection.

Varicella zoster usually manifests as maculopapular rash (MPR), which later progresses to vesicle. It can also manifest as MPR without progression to the vesicle stage. This atypical manifestation is more common in adults and immunocompromised patients. A 30‑year‑old female presented with high‑grade fever and rash over face and body for 5 days. She was diagnosed to have Varicella zoster virus (VZV) infection by positive VZV immunoglobulin M enzyme‑linked immunosorbent assay and polymerase chain reaction. We present this case to increase awareness among clinicians on the atypical manifestations of VZV and prevent complications by early diagnosis.

Oscillometric Blood Pressure in Indian School Children: Simplified Percentile Tables and Charts.

Background: Data on blood pressure recorded by oscillometric method is limited. Objective: To develop simplified tables and charts of blood pressure recorded by oscillometric method in children. Design: Cross-sectional. Setting:Ballabhgarh, Haryana. Participants: Healthy school-children. Main outcome measures: Blood pressure measured by oscillometric method. Results: The study group included 7,761 children (58.4% males) with mean (SD) age of 10.5 (2.8) years. Age and gender were used to create simplified percentile tables and charts, as height was seen to explain very little variability of either systolic or diastolic blood pressure. Formulae for SBP and DBP thresholds for hypertension were derived as [110 + 1.6 x age] and [79 + 0.7 x age], respectively, with 1 mm Hg to be added for females. 95th percentile values suggest simple levels indicating hypertension to be 120/80, 125/85 and 135/90 at ages of 5, 10 and 15 years, respectively. Conclusions: Simplified reference tables and charts, formulae for SBP and DBP, and simple convenient thresholds may be useful for rapid screening of hypertension using oscillometric method.

Molecular & phenotypic characterization of Staphylococcus epidermidis in implant related infections.

Background & objectives: The discrimination between the Staphylococcus epidermidis colonizing the deep seated indwelling devices and those which are mere commensals has always been a challenge for the clinical microbiologist. This study was aimed to characterize the S. epidermidis isolates obtained from device related infection for their phenotypic and molecular markers of virulence and to see whether these markers can be used to differentiate the pathogenic S. epidermidis from the commensals. Methods: Fifty five S. epidermidis isolates from various device related infections such as endophthalmitis following intra-ocular lens (IOL) implantation, intravascular (IV) catheter related sepsis and orthopaedic implant infections, were studied for slime production, biotyping, antibiotic sensitivity; and mec A and ica positivity by the recommended procedures. Results: Twenty three (41.8%) isolates were multi-drug resistant, 26 (65.2%) were slime producers, 30 (54.5%) were adherent, 23 (41.8%) possessed the intercellular adhesin (ica) gene, and 28 (50.9%) harboured the mec A gene. Biotypes I and III were the commonest, most members of which were multi- drug resistant. Twenty two (73.3%) of the 30 adherent bacteria were slime producers as opposed to only 4 (16%) of the 25 non-adherent bacteria (P<0.001). A vast majority i.e. 21 (91.3%) of the 23 ica positive organisms were adherent to artificial surfaces in contrast to only 9 (28.1%) of the 32 non-ica positive organisms (P<0.001). Twenty (86.9%) of the 23 ica positive bacteria were slime producers, as opposed to only 6 (18.7%) of the 32 ica negative bacteria (P<0.001). Of the 23 multi-drug resistant isolates, 19 (82.6%) carried the mec A gene. Interpretation & conclusions: The present findings showed that ica AB and mec A were the two important virulence markers of S. epidermidis in implant infections and slime was responsible for the sessile mode of attachment on the devices.

Drug therapy of cardiac diseases in children.

Justification: The indications and doses of most drugs used for heart ailments in children are extrapolated from data in adult patients. Separate guidelines are needed for neonates, infants and children because of the differences in underlying heart diseases and metabolic clearance of some of these drugs. Process: Consensus emerged following expert deliberations at the National Meeting on Management of Congenital Heart Diseases in India, held on 13th September 2008, at the All India Institute of Medical Sciences, New Delhi, India, supported by Pediatric Cardiac Society of India. Objectives: To review the literature and frame evidence based guidelines for (i) indications, doses, adverse effects and safety profile of commonly used drugs in pediatric cardiology practice; and (ii) to provide an algorithm for treatment in various clinical settings. Recommendations: Consensus review and recommendations are given for drugs used in children for heart failure, hypertension, thrombosis, supraventricular tachycardia and intensive care. Guidelines are also given for use of intravenous immunoglobulins and sildenafil in children.

Prevalence of rheumatic heart disease: Has it declined in India.

Rheumatic heart disease continues to be a major health problem in many parts of the world. The epidemiology of rheumatic heart disease in India is of special interest as it may help to understand the effects of economic transition on this enigmatic disease. Critical appraisal of the published literature suggests the possibility of a real decline in the occurrence of the disease in some parts of the country, but a continuing onslaught in several other regions. The rate of decline seems to correlate more with improved public health facilities than with economic development alone. However, the cumulative burden of the disease remains high, and sustained efforts for the prevention of rheumatic heart disease are warranted.

Syndrome of tubulointerstitial nephritis and uveitis.

We report a case of acute tubulointerstitial nephritis and uveitis syndrome (TINU syndrome) in an elderly Indian woman. TINU is one of the rare causes of acute tubulo interstitial nephritis and is likely to be missed as there is often temporal gap between the appearance of the renal and ocular manifestations. ©

Wiring of tortuous coronary arteries-let the heart do the hard work!

Tortuous coronary arteries pose a special problem for the cardiac interventionists. They are associated with difficulty in advancement of guidewire, balloons and stents across the coronaries with higher complications. We describe an interesting phenomenon of "self advancement of guidewire" in tortuous coronary arteries with an illustrative case.

Acute hemodynamic effects of sildenafil in patients with idiopathic dilated cardiomyopathy.

Sildenafil, a phosphodiestrase-5 inhibitor, decreases pulmonary artery pressures (PAP) in patients with idiopathic pulmonary hypertension. There is little data pertaining to its use in unselected patients with idiopathic dilated cardiomyopathy (IDCM). A single oral dose of sildenafil (50 mg) was administered to 11 patients (mean age 44.9 +/- 7 years, 7 males) with IDCM with left ventricular ejection fraction < or = 40% in New York Heart Association class II/III at the time of right heart catheterization. There was a significant decrease in pulmonary artery systolic pressure (from 31.5 +/- 9.7 to 19.0 +/- 5.2 mmHg, p < 0.001) and pulmonary vascular resistance (PVR) (from 3.0 +/- 2.1 to 1.6 +/- 0.8 dyne/s/m(2)/cm(5), p = 0.01) following sildenafil administration. The systemic vascular resistance (SVR) and pulmonary wedge capillary pressure also significantly decreased. No significant differences in heart rate, cardiac index and PVR/SVR ratio were observed. There were no side effects documented. Sildenafil produces favorable vasodilation in both pulmonary and systemic vascular beds with decrease in left ventricular filling pressures, in stable patients with IDCM.

Estimation of hydrogen sulphide in the human lymphocytes.

Hydrogen sulphide (H2S), a signaling gasotransmitter and a potent vasorelaxant is endogenously produced by the enzymes cystathionine-beta-synthase (CBS) and cystathionine-gamma-lyase (CSE). CBS is a predominant source of H2S in the central nervous system, while CSE is the major H2S producing enzyme in the brain and other nervous tissues. Though the expression of these enzymes in the blood lymphocytes is known, H2S formation in the lymphocytes has not been reported so far. In the present study, H2S levels in the lymphocytes of healthy control subjects were estimated, after suitable modifications in a routine method [Stipanuk M H & Beck P W (1982) Biochem J 206, 267-277] used for detecting tissue levels of H2S. In this method, homocysteine (Hcys) due to its higher solubility was used as the substrate in place of L-cysteine and NaOH was used in place of zinc acetate to increase the entrapment of H2S in the central well. A mean H2S level of 11.64 +/- 6.36 microM/min/mg protein was detected in the lymphocytes of 8 subjects (mean age, 24 +/- 2; 2 male, 6 female). The modified method was found to be more sensitive for H2S estimation in human lymphocytes. As endogenous H2S is reported to be involved in the pathogenesis of various cardiovascular and pulmonary diseases, the levels of H2S in lymphocytes can be a marker of the endogenous tissue levels.

Eales' disease: oxidant stress and weak antioxidant defence.

Eales' disease (ED) is an idiopathic retinal periphlebitis characterized by capillary non-perfusion and neovascularization. In addition to the existing system, a new staging system has been proposed by Saxena et al. Immunological, molecular biological and biochemical studies have indicated the role of human leucocyte antigen, retinal S antigen autoimmunity, Mycobacterium tuberculosis genome, free radical damage and possibly hyperhomocysteinemia in its etiopathogenesis, which appears multifactorial. Oxidant stress has been shown by increase in the levels of thiobarbituric acid reactive substances (lipid oxidation) in the vitreous, erythrocytes, platelets and monocytes. A decrease in vitamins E and C both in active and healed vasculitis, superoxide dismutase, glutathione and glutathione peroxidase showed a weakened antioxidant defence. Epiretinal membrane from patients of ED who underwent surgery showed, by immunolocalization, presence of carboxy methyl lysine, an advanced glycation end product formed by glycoxidation and is involved in angiogenesis. OH. free radical accumulation in monocytes has been directly shown by electron spin resonance spectrometry. Free radical damage to DNA and of protein was shown by the accumulation of 8 hydroxy 2 deoxyguanosine (in leucocytes) and nitrotyrosine (in monocytes), respectively. Nitrosative stress was shown by increased expression of inducible nitric oxide synthase in monocytes in which levels of iron and copper were increased while those of zinc decreased. A novel 88 kDa protein was found in serum and vitreous in inflammatory condition and had antioxidant function. Platelet fluidity was also affected. Oral, methotrexate in low dosage (12.5 mg/week for 12 weeks) as well as oral vitamin E (400 IU) and C (500 mg) daily for 8 weeks are reported to have beneficial effects.

Biochemistry of homocysteine in health and diseases.

The amino acid homocysteine (Hcy), formed from methionine has profound importance in health and diseases. In normal circumstances, it is converted to cysteine and partly remethylated to methionine with the help of vit B12 and folate. However, when normal metabolism is disturbed, due to deficiency of cystathionine-beta-synthase, which requires vit B6 for activation, Hcy is accumulated in the blood with an increase of methionine, resulting into mental retardation (homocystinuria type I). A decrease of cysteine may cause eye diseases, due to decrease in the synthesis of glutathione (antioxidant). In homocystinurias type II, III and IV, there is accumulation of Hcy, but a decrease of methionine, thus, there is no mental retardation. Homocysteinemia is found in Marfan syndrome, some cases of type I diabetes and is also linked to smoking and has genetic basis too. In hyperhomocysteinemias (HHcys), clinical manifestations are mental retardation and seizures (type I only), ectopia lentis, secondary glaucoma, optic atrophy, retinal detachment, skeletal abnormalities, osteoporosis, vascular changes, neurological dysfunction and psychiatric symptoms. Thrombotic and cardiovascular diseases may also be encountered. The harmful effects of homocysteinemias are due to (i) production of oxidants (reactive oxygen species) generated during oxidation of Hcy to homocystine and disulphides in the blood. These could oxidize membrane lipids and proteins. (ii) Hcy can react with proteins with their thiols and form disulphides (thiolation), (iii) it can also be converted to highly reactive thiolactone which could react with the proteins forming -NH-CO- adducts, thus affecting the body proteins and enzymes. Homocystinuria type I is very rare (1 in 12 lakhs only) and is treated with supplementation of vit B6 and cystine. Others are more common and are treated with folate, vit B12 and in selected cases as in methionine synthase deficiency, methionine, avoiding excess. In this review, the role of elevated Hcy levels in cardiovascular, ocular, neurologial and other diseases and the possible therapeutic measures, in addition to the molecular mechanisms involved in deleterious manifestations of homocysteinemia, have been discussed.