Epidemiology, Clinico-Haematological Profile and Management of Aplastic Anaemia: AIIMS Experience.

BACKGROUND: The incidence of aplastic anaemia (AA) is higher in Asia than in the West. The precise incidence of AA in India is not known due to lack of epidemiological study. 20-40% of pancytopenic patients in referral centres are of aplastic anaemia. PATIENTS AND METHODS: This was an analysis of 1501 patients diagnosed with aplastic anaemia over a period of seven and half years (January 2007- June 2014) attending the Aplastic clinic of department of haematology of All India Institute of Medical Sciences, New Delhi. The details regarding medical history, physical examination, complete blood count, bone marrow aspirate and biopsy, treatment received, were retrieved. Inherited bone marrow failure was screened in patients below 35 years. Treatment response was analysed for various treatment modalities. RESULTS: 1501 patients of AA from 20 different states of India were analysed. The bulk of patients were from Uttar Pradesh (28.7%), Bihar (23.6%), Delhi/NCR (20%) and Haryana (7%).The average number of new aplastic anaemia patients enrolled per year 214 (range: 101 -263). The median age at presentation was 25 years (range 2-83),with M;F - 2.3:1. Severity of AA revealed: severe (SAA): 75%, very severe (VSAA): 15%, non-severe (NSAA): 10%. Inherited bone marrow failure syndromes constituted 5% (75 patients) of all aplastic anaemia patients. The most common clinical presentations were pallor (97%), bleeding manifestations (69.6%) and fever (54%). The haematological parameters showed: median level of haemoglobin level: 5.9 gm/dL, WBC: 2700/mm3, ANC: 380/mm3, platelet: 1 0000/mm3. PNH clone was present in 13.5% of patients. 107 patients (7%) were lost to follow up or expired before any treatment was initiated. Only 69 patients (4.5%) received treatment with HLA-matched sibling stem cell transplantation and another 232 (15.5%) patients received ATG plus cyclosporine as immunosuppressive therapy. Seven hundred thirteenpatients (47.5%) received cyclosporine. The overall response to various treatment modalities was: HLA matched sibling haematopoietic stem cell transplant: 75.3%, Anti-thymocyte globulin plus cyclosporine: 58.7%, cyclosporine plus androgen: 45.6%, cyclosporine alone: 32.2%. CONCLUSION: Management of AA is a real challenge in developing countries.This is one of the largest case series from a single centre from India. It is our endeavour to reduce the detrimental outcome by increasing awareness among patients and referring physicians to reduce the delay between diagnosis and treatment.

Assessment of insulin resistance, dyslipidemia and inflammatory response in North Indian male patients with angiographically proven coronary artery disease.

AIM: Coronary artery disease (CAD) is a leading cause of morbidity and mortality in the developed world and is rapidly assuming epidemic proportions in developing countries, including India. Extensive research has proven the role of multiple etiologies such as dyslipidemia, inflammation, endothelial dysfunction and insulin resistance in the pathogenesis of CAD. The following study was undertaken to determine a possible inter-relationship between insulin resistance, inflammation and dyslipidemia, which are important risk factors for CAD in the atherosclerosis-prone north Indian male population. METHODS: The present study was conducted in 100 patients of myocardial infarction diagnosed on electrocardiographic and biochemical criteria, who subsequently underwent coronary angiography and 100 age matched healthy controls. The parameters that were evaluated include lipid profile, hsCRP, apolipoprotein B, insulin levels and HOMA-IR. RESULTS: Significantly higher serum levels of cholesterol, triglycerides, LDL and apolipoprotein B was observed in the patients as compared to the controls. On further classification, the dyslipidemia was marked in the patients with triple vessel disease as compared to single and double vessel disease. Similar pattern was observed for insulin resistance and CRP. Upon plotting the ROC curves, hsCRP emerged as the strongest predictor for CAD followed by apolipoprotein B. A significantly positive correlation was discerned between apolipoprotein B, CRP and HOMA-IR. CONCLUSION: The present study illustrates interplay between insulin resistance, inflammation and dyslipidemia in the CAD prone north Indian population. It also highlights the superiority of hs CRP in risk stratification of patients with angiographically proven CAD.

Heart rate variability assessment to stratify risk of autonomic imbalance during subarachnoid block: A prospective study.

BACKGROUND: Hypotension after subarachnoid block is a common adverse event which can be predicted by simple, safe and indirect measure of autonomic activity. CONTEXT: Heart rate variability has been accepted as an indirect measure of autonomic activity. AIM: It was to evaluate preoperatively risk of hypotension after subarachnoid block. SETTING AND DESIGN: This is controlled, randomized blind prospective study. MATERIALS AND METHODS: One hundred adult patients of either sex in the age group of 25 to 60 years belonging to ASA physical status of I to III, scheduled for elective infra-umbilical surgery, were enrolled for this randomized prospective study. During preanesthetic check their HRV was analyzed for time domain and frequency domain parameters. They were classified into two groups of 50 patients each depending on their low to high frequency ratios (LF/HF). Group I included patients with LF/HF <2.5 and Group II included patients with LF/HF >2.5. Sensitivity of LF/HF for prediction of hypotension greater than 20% of baseline was tested. RESULT: The present study showed significant differences of systolic blood pressure (SBP) after subarachnoid block, depending on baseline LF/HF. Patients with low LF/HF showed lowest SBP of 106.08 ± 3.19 (15.22% fall of base line SBP) as compared to high LF/HF which showed 87.62 ± 8.71 (30.26% fall of base line SBP). Baseline LF/HF parameter correlated significantly with proportionate decrease in SBP after subarachnoid blocks. STATISTICAL ANALYSIS: Hemodynamic parameter was analyzed by using student t test on statgraphic version 5.1. CONCLUSION: Analysis of low and high frequency ratio, reflect autonomic control and may be used as an indirect measure for risk stratification of hypotension after subarachnoid block with high sensitivity.

Smile Train project: a blessing for population of lower socio-economic status.

The Smile Train Project is an international charity dedicated to poor children with cleft lip and cleft palate abnormalities with an aim to restore normal or near normal anatomy, function, satisfactory facial appearance and speech. A review was done among 241 patients of cleft lip and palate anomaly, admitted at Subharti Medical College, Meerut, for cheiloplasty and palatoplasty between May 2006 and December 2008 in collaboration with the department of maxillofacial surgery and its incidence in relation to age and sex distribution. Ideally infants with cleft lip alone should be repaired within the first six months of age and cleft palate should be repaired before development of speech ie, at the age of 2 years. But in this study only 25% of patients who underwent corrective surgery were up to 2 years of age and more than 47% cases were operated between the ages of 2 and 10 years. Sixty-six cases (27%) were operated between ages of 10 and 35 years. It may be due to ignorance, poverty or unawareness about the fact that cleft anomaly can be corrected by surgery. Without repair, these children would have suffered from facial disfigurement, feeding problems, social isolation and abnormal speech. Smile Train Project along with motivation of health workers of this institute has made their smiles more socially acceptable.

An arteriographic assessment of renal vasculature in young patients with essential hypertension.

Twenty-five patients below the age of 40 years with Uncomplicated hypertension underwent selective renal arteriography to assess the involvement of interlobar and arcuate arteries. Renal vasculature was abnormal in all 25 patients. Patients with severe hypertension had markedly abnormal renal vasculature. None of the patients with mild hypertension had severe vascular involvement. Functional significance of these arteriographic abnormalities is discussed.