Uterine tumor resembling ovarian sex cord tumor: A series of six cases displaying varied histopathological patterns and clinical profiles

Introduction: Uterine tumors resembling ovarian sex cord tumor (UTROSCT) are a unique group of neoplasms with diverse morphology and immunophenotypic characteristics, coexpressing sex cord, epithelial, and smooth-muscle markers. To date, less than 100 cases have been reported and there is paucity of data concerning their clinical behavior. Materials and Methods: All cases of uterine body tumors diagnosed over a period of two and a half years (2016-2018) were retrieved. Histopathological features were reviewed and extended panel of immunohistochemistry was performed to identify cases of UTROSCTs. Results: Six cases of UTROSCTs were identified with a median age of 46.5 years. Four of them presented with menorrhagia, while two with postmenopausal bleeding including one with a history of carcinoma breast. Three of these cases were initially misdiagnosed as endometrial stromal sarcoma and adenocarcinomas. They all underwent hysterectomy with bilateral salpingo-oophorectomy. Conclusion: It is considered a tumor with low malignant potential; however, one out of six cases (16.7%) in our study showed metastasis, within 1 year of diagnosis. It is important to recognize this entity as it mimics a wide range of both benign and malignant tumors. Molecular pathogenesis and exact management protocols remain elusive due to rarity,hence, multi-institutional studies are warranted.

Prevalence of familial hypercholesterolemia in premature coronary artery disease patients admitted to a tertiary care hospital in North India

Aims The prevalence of premature coronary artery disease (CAD) in India is two to three times more than other ethnic groups. Untreated heterozygous familial hypercholesterolemia (FH) is one of the important causes for premature CAD. As the age advances, these patients without treatment have 100 times increased risk of cardiovascular (CV) mortality resulting from myocardial infarction (MI). Recent evidence suggests that one in 250 individuals may be affected by FH (nearly 40 million people globally). It is indicated that the true global prevalence of FH is underestimated. The true prevalence of FH in India remains unknown. Methods A total of 635 patients with premature CAD were assessed for FH using the Dutch Lipid Clinical Network (DLCN) criteria. Based on scores, patients were diagnosed as definite, probable, possible, or no FH. Other CV risk factors known to cause CAD such as smoking, diabetes mellitus, and hypertension were also recorded. Results Of total 635 patients, 25 (4%) were diagnosed as definite, 70 (11%) as probable, 238 (37%) as possible, and 302 (48%) without FH, suggesting the prevalence of potential (definite + probable) FH of about 15% in the North Indian population. FH is more common in younger patients, and they have lesser incidence of common CV risk factors such as diabetes, hypertension, and smoking than the younger MI patients without FH (26.32% vs.42.59%; 17.89% vs.29.44%; 22.11% vs.40.74%). Conclusion FH prevalence is high among patients with premature CAD admitted to a cardiac unit. To detect patients with FH, routine screening with simple criteria such as family history of premature CAD combined with hypercholesterolemia, and a DLCN criteria score >5 may be effectively used.

Multi detector computed tomography (MDCT) evaluation of small bowel obstruction: pictorial review.

Small bowel obstruction (SBO) is one of the most perplexing clinical situations encountered by a surgeon in the emergency room. The decision to operate or not depends upon many factors including the probable cause and chances of bowel strangulation / ischemia. The clinical, biochemical and radiological features help the surgeon in making this decision. Plain X-rays have been the mainstay in the radiological diagnosis of SBO and its complications. In the last 20 years, CT scan has ushered in a revolution in establishing the diagnosis of SBO, its causes and complications earlier than the traditional methods and has helped in reducing morbidity and mortality. Here, we have summarised the role of multidetector CT scan in diagnosing various aspects of SBO.

Reducing the burden of coronary artery disease in India: challenges and opportunities.

Asian Indians--living both in India and abroad--have one of the highest rates of coronary artery disease (CAD) in the world, three times higher than the rates among Caucasians in the United States. The CAD among Indians is usually more aggressive at the time of presentation compared with whites or East Asians. The overall impact is much greater because the CAD in Asian Indians affects the "younger" working population. This kind of disproportionate epidemic among the young Indians is causing tremendous number of work days lost at a time when India is experiencing a dizzying economic boom and needs a healthy populace to sustain this boom. While the mortality and morbidity from CAD has been falling in the western world, it has been climbing to epidemic proportions among the Indian population. Various factors that are thought to contribute to this rising epidemic include urbanization of rural areas, large-scale migration of rural population to urban areas, increase in sedentary lifestyle, abdominal obesity, metabolic syndrome, diabetes, inadequate consumption of fruits and vegetables, increased use of fried, processed and fast foods, tobacco abuse, poor awareness and control of CAD risk factors, unique dyslipidemia (high triglycerides, low HDL-cholesterol levels), and possible genetic predisposition due to lipoprotein (a) [Lp(a)] excess. The effect of established, as well as novel, risk factors is multiplicative, not just additive (total effect>sum of parts). The management would require aggressive individual, societal, and governmental (policy and regulatory) interventions. Indians will require specific lower cut-offs and stricter goals for treatment of various risk factors than is currently recommended for western populations. To this end, the First Indo-US Healthcare Summit was held in New Delhi, India on December 14 and 15, 2007. The participants included representatives from several professional entities including the American Association of Physicians of Indian origin (AAPI), Indian Medical Association (IMA), Medical Council of India (MCI), and Government of India (GOI) with their main objective to address specific issues and provide precise recommendations to implement the prevention of CAD among Indians. The summary of the deliberations by the committee on "CAD among Asian Indians" and the recommendations are presented in this document. OBJECTIVES: Discussion of demographics of CAD in Indians-both in India and abroad, current treatment strategies, primordial, primary, and secondary prevention. Development of specific recommendations for screening, evaluation and management for the prevention of CAD disease epidemic among Asian Indians. Recommendations for improving quality of care through professional, public and private initiatives.

Serum lipid response to introducing ghee as a partial replacement for mustard oil in the diet of healthy young Indians.

Ghee (clarified butter) has generally been assumed to be hypercholesterolaemic on the basis of its composition but there is hardly any study to support or refute the assumption. The present study was conducted on sixty-three healthy, young, physically active adult volunteers (52 male, 11 female). The study design was that of a randomized controlled trial with a parallel design. After a lead-in period of 2 wk, the subjects were randomly divided into two groups, Group A (n = 30; 25 male, 5 female) and Group B (n = 33; 27 male, 6 female). Group A (experimental) consumed for 8 wk a diet in which ghee provided 10% of the energy intake. The only other visible fat in the diet was mustard oil, and total energy from fats was 25% of the energy intake. Group B (control) consumed for 8 wk a similar diet except that all visible fat came from mustard oil. The serum total cholesterol level showed a significant rise in the experimental group at 4 wk; the rise persisted at 8 wk. A similar rise was also seen in HDL cholesterol. Hence the total cholesterol/HDL cholesterol ratio did not show any significant change. In the control group, there was a trend towards a fall in LDL cholesterol but the change was not significant. The study does not indicate any adverse effect of ghee on lipoprotein profile. However, more studies are needed on older subjects, hyperlipidaemic subjects, and on subjects following less healthy lifestyles before the results of this study can be extrapolated to the general population.

The effect of ingestion of egg on the serum lipid profile of healthy young Indians.

Thirty four healthy young volunteers (22 men, 12 women; age 25.7 +/- 5.8 years; BMI 20.8 +/- 2.3 kg/m2) participated in a randomized controlled cross-over trial on the effect of consuming one boiled egg every day for 8 wk on the serum lipid profile. The only significant change after 8 wk of egg consumption was an elevation of the total cholesterol/HDL cholesterol ratio. However, scrutiny of individual responses revealed that twelve of the subjects (10 men, 2 women) had a greater than 15% rise in the LDL cholesterol level after 8 wk of egg consumption. These subjects, considered hyperresponders, showed significant increases (P < 0.025) at both 4 wk and 8 wk after egg consumption in total cholesterol and LDL cholesterol levels, and at 8 wk in total cholesterol/HDL cholesterol ratio. The remaining 22 hyporesponders showed no change in any of the variables measured at 4 wk or 8 wk after egg consumption. In view of the high nutritional value of eggs, a blanket ban on eggs is not justified. However, since up to one-third of the population may be hyperresponders, knowing the response of an individual is important before making the egg a regular item of the diet.

The effect of ingestion of egg on serum lipid profile in healthy young free-living subjects.

Egg is a major source of dietary cholesterol. Previous studies on the effect of egg on serum lipid profile have given conflicting results. Further, the serum lipid response to egg shows marked individual variation. Since the variation is at least partly genetically determined, and the response depends partly on the overall diet, studies on different ethnic groups are important. There is hardly any study on the subject available on Indians. In the present investigation, eighteen healthy young volunteers (7 male, 11 female) on a lacto-vegetarian diet were given one boiled egg per day for 8 wk in a randomized controlled cross-over study. Compared to the values obtained after 8 wk of egg-free period, the mean serum total cholesterol, LDL cholesterol, HDL cholesterol, total cholesterol/HDL ratio, VLDL cholesterol and triglycerides were not significantly different after 8 wk of egg consumption. However, the serum total cholesterol after 4 wk of egg consumption was significantly higher than the control values. Further, seven subjects out of 18 had an appreciable elevation of serum total cholesterol or LDL cholesterol, or both, after 8 wk of egg consumption. The study suggests that in young healthy Indian subjects on a vegetarian diet, consuming one egg per day raises serum cholesterol levels at 4 wk but in the majority baseline values are restored by 8 wk. However, some hyper-responders continue to have elevated serum cholesterol even at 8 wk. Knowing the response of an individual may be important before making egg consumption a regular habit.

Effect of partial replacement of visible fat by ghee (clarified butter) on serum lipid profile.

A randomised controlled trial with a parallel design was conducted on 24 healthy young volunteers who were divided into two groups. After a lead-in period of 2 wk, the experimental group (n = 11; 9 male, 2 female) had for 8 wk a lactovegetarian diet providing about 25% of the energy intake in the form of fat, of which ghee provided 10 en% and the remaining fat energy came from mustard oil and invisible fat. The control group (n = 13; 8 male, 5 female) had a similar diet except that all visible fat was in the form of mustard oil. In neither group was there any significant change in the serum lipid profile at any point in time. At 8 wk, 2 volunteers in the experimental group, and 1 volunteer in the control group had more than 20% rise in serum total cholesterol as compared to their 0 wk values. There was also an appreciable increase in HDL cholesterol at 8 wk in the experimental group, but it was not statistically significant. Consuming ghee at the level of 10 en% in a vegetarian diet generally has no effect on the serum lipid profile of young, healthy, physically active individuals, but a few individuals may respond differently.

Coronary angiography using 4 French catheters with power injection: a randomized comparison with 6 French catheters.

BACKGROUND: Coronary angiography using 4 F catheters may reduce access-site complications and enable early ambulation, although earlier studies suggested that the quality of images may be an issue of concern. METHODS AND RESULTS: To ascertain the quality of angiographic images and safety of early ambulation, 500 patients were randomized to coronary angiography with either 4 F or 6 F catheters. Procedural characteristics, angiographic quality scores and results of ambulation were analyzed in the two groups. Patients in the 4 F group were mobilized at 2 hours post-procedure while those in the 6 F group were ambulated at 6 hours. There was no procedure-related complication in either group. The procedure was successfully completed in 250 of 252 patients randomized to the 4 F group. In two patients in the 4 F group, sheaths were upgraded to 6 F to complete the procedure, as difficulty was encountered in hooking the coronary ostium with a 4 F Judkin's catheter. Coronary angiographic quality scores in these two groups were comparable. Angiographic scores for the 4 F and 6 F groups for the left coronary artery averaged 4.45+/-0.5 and 4.58+/-0.3 (p>0.1), respectively. The right coronary artery scores averaged 4.30+/-0.4 and 4.35+/-0.2 (p>0.1) in the 4 F and 6 F groups. Angiographic scores for the left ventricular angiogram averaged 4.22+/-0.1 and 4.44+/-0.3 (p>0.1) in the 4 F and 6 F groups, respectively. None of the angiograms were assigned a score of <3.0 (not diagnostic). The total contrast volume consumed in the two groups was also equivalent. There were no groin-related complications in the 4 F group although these patients were ambulated 2 hours after the procedure. CONCLUSIONS: Coronary angiography performed with a 4 F catheter is a safe and reliable procedure. The quality of image obtained with a 4 F catheter is equivalent to that obtained with a 6 F catheter. Early ambulation at 2 hours is feasible without compromising safety.

Percutaneous transseptal mitral commissurotomy in pregnant women with critical mitral stenosis.

BACKGROUND: Percutaneous transseptal mitral commissurotomy has been successfully performed in selected pregnant patients with severe symptomatic mitral stenosis. Its safety and efficacy needs to be evaluated in a large number of cases. METHODS AND RESULTS: Percutaneous transseptal mitral commissurotomy was performed in 85 severely symptomatic (New York Heart Association functional class III or IV) pregnant women aged 22.7+/-4.1 years (range 18-39 years) with critical mitral stenosis at 24.8+/-4.7 weeks (range 20-34 weeks) of gestation. Percutaneous valvotomy was performed using a flow-guided Inoue balloon in all the patients. The procedure was considered successful in 80 (94%) patients. The hemodynamic mean end-diastolic gradient decreased from 26.7+/-6.8 mm Hg (range 16-35 mmHg) to 4.5+/-3.8 mmHg (range 0-14 mmHg) (p<0.001). The mean diastolic gradient decreased from 29.1+/-9.1 mmHg (range 18-38 mmHg) to 7.2+/-4.1 mmHg (range 4.1-18 mmHg) (p<0.001). The mean mitral valve area assessed by echocardiography increased from 0.75+/-0.5 cm2 (range 0.4-1.0 cm2) to 2.0+/-0.5 (range 1.0-2.7 cm2) (p<0.001). The mean fluoroscopy time was 3.6+/-3.2 minutes. The results of the mitral valvotomy were considered suboptimal in 4 patients. Mitral regurgitation increased by 1 grade in 16 patients and more than 2 grades in 2 patients. One patient developed pericardial tamponade during the procedure and was managed by catheter drainage. Percutaneous mitral valve dilatation was then successfully performed in this patient. No fetal abortion occurred after the procedure. CONCLUSIONS: The results of this study indicate that percutaneous transseptal mitral commissurotomy is a safe and effective procedure for severe symptomatic mitral stenosis in pregnancy.

Isolated congenital coronary arteriovenous fistula.

During the last eight years a total of nine cases of isolated congenital coronary arteriovenous fistula (CAVF) have been diagnosed at our centre. Age of these patients ranged from 11 days to 22 years (mean 6.4 +/- 4.2 years). There were seven male and two female patients. Right and left coronary arteries were the sites of origin in seven and three cases respectively. One patient had two fistulae, one each originating from right coronary artery and obtuse marginal artery. The drainage sites were right ventricle in seven cases and right atrium in two. The pulmonary artery pressure was normal in eight cases and elevated in one case. The pulmonary artery to systemic flow ratio ranged from 1.1:1-2.4:1 in all except the 11 day-old-baby in whom it was 4:1. Pulmonary vascular resistance (PVRI) was below 3.5 in all cases (range 1.0-3.5 units). Surgical ligation of fistula was done in four cases and two patients underwent coil embolisation of fistula. Follow-up is available in five out of eight patients including three cases in whom CAVF was closed. Two other young patients are being followed-up conservatively and all the patients are asymptomatic. CAVF can present at any age; majority of the cases have small left to right shunts and more commonly CAVF arise from right coronary artery. Surgical ligation of fistula is safe in older patients, non-surgical embolisation using coils may be an alternative to surgery in selected cases.

Retardation of coronary atherosclerosis with yoga lifestyle intervention.

BACKGROUND: Yoga has potential for benefit for patients with coronary artery disease though objective, angiographic studies are lacking. MATERIAL AND METHODS: We evaluated possible role of lifestyle modification incorporating yoga, on retardation of coronary atherosclerotic disease. In this prospective, randomized, controlled trial, 42 men with angiographically proven coronary artery disease (CAD) were randomized to control (n = 21) and yoga intervention group (n = 21) and were followed for one year. The active group was treated with a user-friendly program consisting of yoga, control of risk factors, diet control and moderate aerobic exercise. The control group was managed by conventional methods i.e. risk factor control and American Heart Association step I diet. RESULTS: At one year, the yoga groups showed significant reduction in number of anginal episodes per week, improved exercise capacity and decrease in body weight. Serum total cholesterol, LDL cholesterol and triglyceride levels also showed greater reductions as compared with control group. Revascularisation procedures (coronary angioplasty or bypass surgery) were less frequently required in the yoga group (one versus eight patients; relative risk = 5.45; P = 0.01). Coronary angiography repeated at one year showed that significantly more lesions regressed (20% versus 2%) and less lesions progressed (5% versus 37%) in the yoga group (chi-square = 24.9; P < 0.0001). The compliance to the total program was excellent and no side effects were observed. CONCLUSION: Yoga lifestyle intervention retards progression and increases regression of coronary atherosclerosis in patients with severe coronary artery disease. It also improves symptomatic status, functional class and risk factor profile.

Serum levels of lipoprotein (a) and other lipids in angiographically defined coronary artery disease patients and healthy blood bank donors.

Lipoprotein (a) (Lp(a)) and other lipid values have been correlated with angiographically defined [table: see text] coronary artery disease. To study this relationship in Indian patients, plasma levels of Lipoprotein (a) and other lipids were assessed in 74 patients undergoing Coronary arteriography and also in 53 age and sex matched healthy male blood bank donors who served as controls. Total cholesterol (mg/dl) (211 +/- 56 vs 186 +/- 43; p < 0.001), low density lipoprotein Cholesterol (mg/dl) (117 +/- 40 vs 88 +/- 29; p > 0.001) and low density lipoprotein/high density lipoprotein cholesterol ratio (2.6 +/- 0.8 vs 2.2 +/- 0.9; p < .001) were significantly higher in patients than controls. High density lipoprotein-cholesterol (mg/dl) (43.5 +/- 6 vs 42.1 +/- 7; p-ns) very low density lipoprotein-cholesterol (mg/dl) (49.7 +/- 17 vs 56.1 +/- 25; p-ns) and triglycerides (mg/dl) (155 +/- 101 vs 167 +/- 88; p-ns) were not statistically different in two groups. Lipoprotein (a) levels showed highly skewed distribution. Patients (n = 74) showed almost five fold higher lipoprotein (a) levels (mg/dl) as compared to controls (n = 53) [105 +/- 565 vs 23 +/- 76]. Patients with very high lipoprotein (a) levels [values of more than 40 mg/dl] (n = 18) had high density lipoprotein cholesterol and total cholesterol significantly lower than rest of the patient group. [high density lipoprotein cholesterol (mg/dl) 41.00 +/- 3.7 vs 44 +/- 6.4; p < 0.01 and total cholesterol (mg/dl) 192 +/- 34 vs 217 +/- 53; p < 0.05].

Two-catheter approach for ablation of accessory pathways in different locations.

Radiofrequency ablation is the treatment of choice for patients with Wolff-Parkinson-White syndrome and symptomatic tachyarrhythmias. The technique involves localising the pathway with multiple catheters at various sites followed by radiofrequency energy application at that site. Single catheter approach has been described for ablation of manifest left-sided accessory pathways. In this article, we report the ablation of accessory pathways in different locations in patients with Wolff-Parkinson-White syndrome by using a two-catheter approach. Twenty-three consecutive patients with symptomatic Wolff-Parkinson-White syndrome were taken up for radiofrequency ablation with this approach. Pathways could be successfully ablated in 11 out of 13 patients with left free wall, 5 out of 7 with right posteroseptal, one patient of left posteroseptal and each of the 2 patients of right mid septal locations giving an overall success in 19/23 (82.6%) patients. Hence, two-catheter approach can be used safely to ablate accessory pathways in different locations with high success rate, thus minimising the procedure time associated with conventional approach.

Apolipoprotein(a) polymorphism and its association with plasma lipoprotein(a) levels: a north Indian study.

Elevated levels of lipoprotein(a) has been regarded as an independent risk factor for coronary, peripheral and cerebral atherosclerosis. The enormous intra-personal variation in the plasma concentration of lipoprotein(a) is almost entirely controlled by the apolipoprotein(a) i.e. gene locus on the chromosome 6q 26-27. The apolipoprotein(a) molecule is highly polymorphic and is known to exist in multiple, genetically determined isoforms. These polymorphisms may be responsible for difference in promoter activity, variable size of apolipoprotein(a) and thereby variation in plasma lipoprotein(a) concentration. We studied the effect of two types of polymorphisms, (i) variation in length of the pentanucleotide repeat in the 5' flanking region starting -1373 bp upstream of AUG codon, and (ii) the Kringle-4 type 2 size polymorphism, on plasma lipoprotein(a) levels in North Indian population. The study group consisted of 88 angiographically assessed male coronary artery disease patients (age range 30-70 years) and 83 age- and sex-matched healthy controls. The pentanucleotide repeat polymorphism was analysed using polymerase chain reaction. In all, 8/11 pentanucleotide repeat isoforms were observed. Using SDS-agarose gel electrophoresis and immunoblotting isoforms having 12-50 Kringle-4 type 2 repeats were detected. Our study indicates a strong association of elevated plasma lipoprotein(a) concentration with coronary artery disease. An inverse correlation was seen between lipoprotein concentration and isoform size for both the pentanucleotide repeat polymorphism and the Kringle-4 type 2 polymorphisms; statistically significant difference (p = 0.001) was, however, observed only for the later.

Interrelationship between angiographically defined coronary artery disease, plasma insulin levels and lipids in Indian patients.

We investigated the interrelationship between plasma insulin levels, various lipoproteins and coronary artery disease. No significant differences were observed on comparing patients with controls for plasma insulin, high density lipoprotein cholesterol, fasting blood sugar levels and triglyceride levels. However patients showed significantly higher levels of total cholesterol and low density lipoprotein cholesterol compared with controls. No significant differences were observed on comparing patients of multivessel disease with single vessel disease in serum insulin or various lipid subfractions. In addition, there was no correlation between serum insulin quartiles and various biochemical parameters. In conclusion, in this cross-sectional study plasma insulin levels failed to show any significant association with severity and extent of coronary artery disease. Further there was no correlation of various lipid parameters with insulin quartiles.