Savita Kumari

Current status of Takayasu arteritis in India.

The clinical features of 106 patients of Takayasu arteritis (TA) seen over a period of 16 years are documented (65 females and 41 males). The mean age was 27.3 +/- 9.2 years. Hypertension was the commonest mode of presentation (51.3%) and was detected in 82 patients (77.4%) at the time of presentation. Vascular bruits were heard in 72 patients (67.9%) and 13 patients (12.3%) were in congestive heart failure. Aortography was performed in 95 patients. Based on the extent of involvement, Type I (branches of aortic arch) was seen in 7 (6.6%) patients, Type II (aortic arch, its branches and descending thoracic aorta) in 7 (6.6%) patients, Type III (descending thoracic aorta and abdominal aorta) in 4 (3.8%) patients, Type IV (abdominal aorta only) in 29 (27.3%) patients and Type V (aortic arch, descending thoracic aorta and abdominal aorta) in 59 (55.7%) patients. Therapeutic modalities included antihypertensive drug therapy in 81 patients, antitubercular drugs in 8 patients, steroids in 16 patients and cyclophosphamide in one patient. Response to steroids was satisfactory in 5 of these 16 patients while the lesions of vasculitis healed in the patient who was treated with cyclophosphamide. Surgical interventions included nephrectomy and autotransplantation of kidney in 3 patients each and revascularization in 4 patients and angioplasty in 4 patients. In the area of pathogenesis of this disease, a high activity of protein kinase C(PKC), an increased intracellular calcium and inositol 1,4,5 triphosphate in both unstimulated and stimulated T cells of TA was observed. These findings suggest an activation of PKC-calcium pathway in TA.

A follow-up study of balloon angioplasty and de-novo stenting in Takayasu arteritis

Percutaneous balloon angioplasty (PTBA) is a universally accepted mode of therapy for stenotic coronary and peripheral arterial lesions. To establish the role of PTBA and stent placement in patients with Takayasus arteritis (TA), these procedures were performed in 20 patients with TA. All patients received steroids, aspirin and ticlodipine (for stent placement) prior to procedure. Angioplasty was carried in patients with symptomatic stenotic vessel of more than 70% of normal diameter or a peak systolic gradient of more than 50 mm across stenotic aortic lesion. Stenting was performed for ostial lesion, long segment lesion or incomplete relief of stenosis and dissection following angioplasty. Carotid angioplasty and stenting was performed in five patients, aortic angioplasty in nine patients, aortic angioplasty and stenting in four patients, renal angioplasty in three patients, renal angioplasty and stenting in two patients and subclavian angioplasty in two patients, subclavian, angioplasty and stenting in three patients and coronary angioplasty and stent placement in one patient. The procedure was successful in all but one patient. On following up, two patients with carotid stent placement had restenosis. A saccular aneurysm developed at the lower end of stent in one patient with aortic stent placement. The PTBA with or without stent placement is a safe and effective method for relief of stenotic lesion in patients with TA.

Pheochromocytoma associated with pregnancy: case report and review of the literature.

UNLABELLED We report on a young woman with pheochromocytoma associated with pregnancy and review 41 other cases reported in the literature from 1988 to 1997. This review reveals that the overall maternal mortality was 4 percent and the fetal loss 11 percent; antenatal diagnosis of pheochromocytoma reduced maternal mortality to 2 percent; however, fetal loss was 14 percent. Diagnosis of pheochromocytoma was made antepartum in 83 percent of the cases. Although pheochromocytoma associated with pregnancy is rare, a high index of clinical suspicion must be kept and all those at risk must be investigated to achieve an early diagnosis and improved outcome. Once the diagnosis is confirmed, alpha-adrenergic blockade is essential and beta-blockade may be required. Magnetic resonance imaging and computerized tomography scan may be used to localize the tumor during the antenatal period. In early pregnancy, i.e., before 24 weeks, both tumor resection and medical treatment are associated with good fetal outcome; in later pregnancy, elective cesarean delivery followed by tumor resection results in favorable maternal and fetal outcome. TARGET AUDIENCE Obstetricians & Gynecologists, Family Physicians. LEARNING OBJECTIVES After completion of this article, the reader will be able to understand the clinical manifestations of a pheochromocytoma during pregnancy, how to make the diagnosis of a pheochromocytoma during pregnancy, and to know the medical and surgical management of a pheochromocytoma during pregnancy.

Different antioxidants status, total antioxidant power and free radicals in essential hypertension

Hypertension is a multi-factorial process, prevalent in developed as well as in developing countries. Different antioxidants and free radicals play an important role in cardiovascular system. In present study, total antioxidant power in terms of FRAP (ferric reducing activity of plasma), free radicals and different antioxidants have been studied in essential hypertensives (n = 50) and normal subjects (n = 50). Levels of total cholesterol, low-density lipids-cholesterol, malonialdehyde, very low-density lipids (VLDL), uric acid, plasma homocysteine and low-density lipids (LDL), were significantly higher in hypertensives as compared to normotensive. HDL-cholesterol, SOD, GPx, reduced glutahione, total glutathione, oxidized glutathione, total thiols, protein thiols, non protein thiols, RNI, total antioxidant power, vitamin A, ascorbic acid and glutahione-S-transferase (GST) were decreased significantly in normotensive. We observed significantly low nitric oxide levels in hypertensive patients. No correlation was observed between severity of disease and plasma nitric oxide levels. There was a significant decrease in plasma FRAP value in essential hypertensives as compared to normotensive controls, which showed a negative correlation with diastolic blood pressure. In conclusion, our study revealed that there was a consistent significant difference between essential hypertensives versus controls with respect to most of the parameters. These complex changes are consistent in the view that essential hypertension is associated with an abnormal level of antioxidant status compared to normal response to oxidative stress or both.

Lupus myocarditis: marked improvement in cardiac function after intravenous immunoglobulin therapy

Intravenous immunoglobulin (IVIg) is emerging as the mainstay in the treatment of many autoimmune diseases, including systemic lupus erythematosus. IVIg has been found to be beneficial in myocarditis due to dermatomyositis/polymyositis, Kawasaki disease, and viral myocarditis in children. We report an 18-year-old man of active lupus with worsening cardiac systolic function who did not respond to pulse methylprednisolone and cyclophosphamide, but subsequently showed an improvement in his cardiac function after IVIg administration.

Hypertension and insulin resistance in a native unindustrialised rural population of India

A cross-sectional survey of 2964 individuals aged 16-70 years from a rural area of North India was carried out to test the hypothesis that a rural unindustrialised hypertensive population of North India has central obesity and hyperinsulinemia. Oral glucose tolerance test was carried out on 68 newly detected hypertensives, 82 age- and sex-matched community controls and 58 genetically related members of hypertensives (family controls). Fasting blood samples were examined for plasma glucose, serum insulin and lipids. Postglucose-load blood samples were examined for plasma glucose and serum insulin. Three blood pressure readings were recorded using Random Zero Sphygmomanometer. Anthropometric measurements (waist-hip ratio, subscapular and triceps skinfold thickness) of all three groups of individuals (hypertensives, community controls and family controls) were also recorded. The hypertensives had significantly higher fasting and postglucose-load serum insulin levels (P<0.05), body mass index (21.3 vs. 19 kg/m2: P<0.05), waist-hip ratio (0.89 vs. 0.85: P<0.001), subscapular (18.5 mm vs. 12.7 mm: P<0.001) and triceps fold thickness (17.6 mm vs. 12.9 mm: P<0.05) than community controls. The family controls had significantly higher systolic blood pressure than community controls (P<0.05). The lipids were not significantly different in all the three groups. Multiple logistic regression showed that both fasting and postglucose-load serum insulin were significantly associated with hypertension independent of waist-hip ratio and body mass index. The results of the present study suggest that hyperinsulinemia is related to hypertension in a rural unindustrialised population of North India.

Plasma Homocysteine Levels in Indian Patients with Essential Hypertension and Their Siblings

Background. An association between plasma homocysteine and essential hypertension and their non-hypertensive siblings is controversial. Methods and Results. Plasma homocysteine levels were measured in subjects with essential hypertension (n = 50), their normotensive siblings (n = 50) and normotensive controls (n = 50). All the subjects were non-diabetic, had no past history of myocardial infarction, stroke or peripheral vascular disease and had normal renal functions. The mean homocysteine values were 18.96 ± 8.08 µmol/L in patients, 14.84 ± 5.55 µmol/L in siblings and 10.50 ± 4.92 µmol/L in controls. Plasma homocysteine level were found to be significantly higher in patients with hypertension (p < 0.0001) and their normotensive siblings (p<0.0001) when compared to controls. Also patients with hypertension had their higher plasma homocysteine levels compared to their siblings (p<0.0036). Conclusion. Plasma homocysteine levels are significantly elevated in Indian patients with essential hypertension and their normotensive siblings. Thus, plasma homocysteine may serve as a marker for the development of essential hypertension.

Outcome of adult acute lymphoblastic leukemia with BFM protocol in a resource-constrained setting

Cure rates for adult acute lymphoblastic leukemia (ALL) in developing countries are significantly lower because of problems unique to these countries. We assessed some of the problems in adult ALL patients (>12 years of age) in a tertiary care hospital of northwest India with modified BFM regimen. The diagnosis of ALL was made according to FAB criteria. The protocol consisted of Phase I & II induction, consolidation, reinduction and maintenance phases. CNS prophylaxis was administered with 24 Gy radiation and intrathecal methotrexate. One hundred and eighteen patients (72.9% males), aged 12 – 68 years (median 23 years) were treated from January 1997 till December 2003. Follow-up of patients was done till December 2005. Complete remission (CR) was achieved in 85.6% patients after induction therapy and 40% patient relapsed. Most patients (23.7%) relapsed during the maintenance phase or after completion of chemotherapy. At least 15% of patients (15/101) after successful induction abandoned the treatment because of financial constraints, prolonged travel time to treatment facility and switching over to alternative medicines. Fatal infectious complications occurred in 19.5% of patients. The 3-year and 5-year event free survival rates were 29.8% and 21.6% respectively. In conclusion, modified BFM regimen resulted in high induction rates but relatively poor 5-year event free survival. Infections related death and post induction abandonment of treatment were the main reasons for poor overall results.

Thrombotic complications of polycythemia vera

Abstract Background: Polycythemia vera (PV) is an uncommon clonal disorder of stem cells. The literature regarding the thrombotic complications of this disorder in the developing countries is scarce. The present study was undertaken retrospectively to look at the association of thrombotic complications of PV with various patient characteristics. Materials and methods: All the patients diagnosed to have PV from January 1986 to December 2005 according to Polycythemia Vera Study Group criteria were included. Their clinical characteristics, laboratory parameters, clinical complications such as thrombosis and myelofibrosis, treatment modalities, malignancies and deaths, if any were noted. The various characteristics of patients who had developed thrombosis were compared statistically with those of patients without thrombosis. Results: Out of the 9550 patients seen during this period, 32 patients fulfilled the inclusion criteria. The median age at the time of diagnosis was 56·5 years. Eight patients developed thrombotic complications; out of which four had arterial and four had venous thrombosis. There was no statistically significant difference in the mean hemoglobin, white cell count (WBC), platelet count and RBC mass in patients with thrombosis as compared to patients without thrombosis. These parameters were also not statistically significant when patients with arterial thrombosis were compared with patients with venous thrombosis. Conclusions: PV is an uncommon disorder when compared with other hematological disorders in northern India. There was no relationship between the development of thrombotic complications and hemoglobin, RBC mass or platelet count. Twenty patients received hydroxyurea with a median follow-up of 57 months, none developed acute leukemia.

Factors Affecting Early Molecular Response in Chronic Myeloid Leukemia

OBJECTIVES There is controversy about whether 3- or 6-month molecular assessment predicts progression-free and overall survival in those with chronic myeloid leukemia (CML). The factors predicting molecular response at 3, 6, and 12 months have not been studied extensively. The study objective was to study the factors affecting molecular response at 3 and 6 months in patients with CML who are receiving imatinib mesylate. METHODS We prospectively enrolled patients with newly diagnosed CML who were receiving imatinib mesylate as the initial therapy for CML. The diagnosis of CML was based on clinical examination, bone marrow, and demonstration of BCR ABL(IS) transcripts by polymerase chain reaction. The molecular response(IS) was assessed at 3, 6, and 12 months by GeneXpert (Cepheid, Sunnyvale, CA) and co-related with various baseline characteristics of patients. We also looked at whether early achievement of a complete hematologic response within 6 weeks predicts molecular response at 3 or 6 months. The study took place at a tertiary care hospital in Northwest India catering to patients belonging to low-middle socioeconomic status. RESULTS We enrolled 131 patients with CML in the chronic phase from July 1, 2013, to August 31, 2014. The median age of the patients was 40 years (range, 13-67) with a male preponderance (61% were male). Most patients presented with symptoms of low-grade fever (52.7%) and abdominal fullness (26.7%). Spleen was palpable in 84.7% of patients. The median hemoglobin at presentation was 10.8 g/dL (range, 4.8-18.4 g/dL), white cell count was 138.3 × 10(9)/L (4.1-697 × 10(9)/L), and platelet count was 326 × 10(9)/L (85-1819 × 10(9)/L). The median number of peripheral blood basophils was 3% (range, 0%-20%), and blasts were 3% (range, 0%-10%). Myelofibrosis of more than grade 1 was present in 30% of patients. Most patients belonged to intermediate Sokal (45.8%) and Hasford (55%) scores and low EUropean Treatment Outcome Study (78.6%) score. Of 128 evaluable patients at 3 months, 96.9% achieved complete hematologic remission (CHR) and 82.3% achieved BCR ABL(IS) of less than 10%. None of the patients who had BCR ABL(IS) > 10% at 3 months achieved BCR ABL(IS) < 1% at 6 months or < 0.1% at 12 months. Early achievement of CHR (< 6 weeks), peripheral blood blast count of < 5%, and lactate dehydrogenase < 851 U/L were significantly associated with achievement of BCR ABL(IS) < 10% at 3 months and BCR ABL(IS) < 1% at 6 months. CONCLUSIONS We found that BCR ABL(IS) assessment at 3 months is superior to assessment at 6 months. Patients with CML in the chronic phase who achieve CHR within 6 weeks are more likely to achieve BCR ABL(IS) < 10% at 3 months and < 1% at 6 months than patients who achieve CHR between 7 and 12 weeks.