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Takayasu's Arteritis (TA) is an uncommon chronic inflammatory and stenotic disease of medium and large-sized arteries characterized by a strong predilection for the aortic arch and its major branches. We report a rare manifestation of TA in a 16-year-old female with no previous history of heart disease who presented with heart failure. She was found to have hypertension and discrepancies of pulses in upper and lower limbs. She developed ST elevated anterior wall myocardial infarction (MI) during hospitalization. Her aortography revealed narrowing of descending thoracic, upper abdominal and infrarenal aorta with significant stenosis of both renal arteries. She was diagnosed as a case of TA on the basis of her clinical profile and arteriography. Her coronary angiography did not reveal any stenosis or occlusion or aneurysm in coronary arteries or at coronary ostia which is in contrast to patients of TA with MI reported in the literature who had focal or diffuse stenosis or aneurysm in the coronaries.

Background: Cerebrovascular stroke is a global health problem. Prolonged length of hospital stay following stroke inflates health care cost, increases risk for hospital-acquired complications, in-hospital death and has been associated with worse outcome. Methods: A hospital based prospective study was performed in acute stroke patients admitted in the department of Medicine of a rural teaching tertiary care hospital located in a town in central India. Data of all consecutive stroke inpatients related to demographic variables (age, gender, social class, education-status), co-morbid conditions, risk factors, imaging variables and various complications during hospital course was collected from patients’ medical records. The type, location, and side of the stroke lesion were noted from the CT/MRI head reports of stroke patients. Stroke assessment scales (GCS, NIHSS, mRS) were also assessed by another trained study person who was blind of the diagnosis and investigations of the patient. Results: A total of 55 stroke patients were studied. The mean age was 58.4 + 12 years; ranging from 23-86 years. Of the total 55 stroke patients, 32 (58.2%) were males and 23 (41.8%) were females. Twenty three (41.8%) of 55 stroke patients had length of stay (LOS) in hospital ≥7 days. The association of location of stroke lesion (LAC/TAC/PAC/POC) and GCS score with LOS in hospital was found to be statistically significant (P 7days). Conclusion: Prolonged length of stay (>7 days) in hospitalized stroke patients was related to location of lesions and low Glasgow coma scale scores. The complications during hospitalization esp. pressure-sores and sepsis were associated with prolonged length of stay in hospitalized stroke patients.

Thoracic endometriosis syndrome is a rare disorder characterized by the presence of functioning endometrial tissue in the pleura, the lung parenchyma, or the airways. The diagnosis of thoracic endometriosis syndrome is usually based on the temporal relationship of the patient’s symptoms with menses

BACKGROUND Cardiovascular risk might be reduced by targeted changes in both low density and high density lipoprotein cholesterol (LDL-C and HDL-C). This dual strategy will require a well tolerated, effective regimen, as well as a better understanding of how HDL-C may be targeted. DESIGN An open-label, uncontrolled, retrospective cohort study of combined statin-niacin therapy. METHODS We reviewed all patients ( n= 132) started on this combination in a referral lipid clinic over a 6.5-year period for tolerability, safety and effectiveness. RESULTS Combined therapy was tolerated by 77% of patients. No serious adverse events attributable to medication were encountered. In drug-naive patients (n = 37), moderate doses of statin and niacin (mean 1180 mg/day) reduced LDL-C 31% and increased HDL-C 29% ( P or= 1000 mg/day (mean 1480) added to a constant statin regimen (n=29), HDL-C increased 20% ( P< 0.001). Even at niacin doses < 1000 mg/day (mean 580, n= 23), HDL-C increased 13% ( P< 0.05). Although mean HDL-C increased, the initial and final HDL-C distributions were broad and largely overlapping. Any chosen cutpoint for HDL-C goal would apply to only a minority of patients. The total/HDL cholesterol ratio had narrower distributions, as the percentage of patients with ratio < 5.0 increased from 17% to 67%. CONCLUSION Combined statin-niacin therapy lowers LDL-C and raises HDL-C with acceptable tolerance and safety. If treating LDL-C is the primary goal, consistent with current guidelines, then a strategy of targeting the total/HDL cholesterol ratio as a secondary goal is applicable to more patients than targeting HDL-C itself.

Background: Difficulties in diagnosis, treatment, and chronic management of Crohn's Disease (CD) may explain the variation in diagnostic evaluation and treatment therapies seen among providers. Our systematic literature review shows that published guidelines do not fully address management of pediatric CD. Therefore, using the Quality Improvement Model, we can systematically gather data from experts on the best way to change current CD management. Objective: To determine if conjoint analysis can summarize current beliefs among pediatric gastroenterologists on the relative importance of implementing evidence-based changes to improve outcomes of children with CD. Methods: We administered an online survey, Physicians Beliefs on Crohn's Management, to a convenience sample of 12 members of the Pediatric Inflammatory Bowel Disease (PIBD) Network. We asked participants to rank 16 different clinic scenarios based on which ones were most likely to improve patient outcomes and analyzed the ranking through conjoint analysis. Results: One physician completed the survey for a response rate of 8.3%. Results suggest that decreasing steroid use is most likely to improve outcomes while planned care and optimizing nutrition are least likely to improve outcomes. We found the largest effect of interaction to be between standardizing diagnostic evaluation and planned care. Conclusion: Conjoint analysis has the potential to summarize current beliefs among pediatric gastroenterologists regarding CD management, but before it can be used reliably, it needs to be tested on a larger sample.

Inheritance patterns in twins and multiplex families led us to hypothesize that two loci were segregating in subjects with juvenile myoclonic epilepsy (JME), one predisposing to generalized tonic-clonic seizures (GTCS) and a second to myoclonic seizures. We tested this hypothesis by performing genome-wide scan of a large family (Family 01) and used the results to guide analyses of additional families. A locus was identified in Family 01 that was linked to GTCS (10q25-q26). Model-based multipoint analysis of the 10q25-q26 locus showed a logarithm of odds (LOD) score of 2.85; similar results were obtained with model-free analyses (maximum nonparametric linkage [NPL] of 2.71; p = 0.0019). Analyses of the 10q25-q26 locus in 10 additional families assuming heterogeneity revealed evidence for linkage in four families; model-based and model-free analyses showed a heterogeneity LOD (HLOD) of 2.01 (alpha = 0.41) and maximum NPL of 2.56 (p = 0.0027), respectively, when all subjects with GTCS were designated to be affected. Combined analyses of all 11 families showed an HLOD of 4.04 (alpha = 0.51) and maximum NPL score of 4.20 (p = 0.000065). Fine mapping of the locus defined an interval of 4.45Mb. These findings identify a novel locus for GTCS on 10q25-q26 and support the idea that distinct loci underlie distinct seizure types within an epilepsy syndrome such as JME.

Cardiovascular risk might be reduced by targeted changes in both low density and high density lipoprotein cholesterol (LDL-C and HDL-C). This dual strategy will require a well tolerated, effective regimen, as well as a better understanding of how HDL-C may be targeted.An open-label, uncontrolled, retrospective cohort study of combined statin-niacin therapy.We reviewed all patients ( n= 132) started on this combination in a referral lipid clinic over a 6.5-year period for tolerability, safety and effectiveness.Combined therapy was tolerated by 77% of patients. No serious adverse events attributable to medication were encountered. In drug-naive patients (n = 37), moderate doses of statin and niacin (mean 1180 mg/day) reduced LDL-C 31% and increased HDL-C 29% ( P0.002, both comparisons). At niacin dosesor= 1000 mg/day (mean 1480) added to a constant statin regimen (n=29), HDL-C increased 20% ( P0.001). Even at niacin doses1000 mg/day (mean 580, n= 23), HDL-C increased 13% ( P0.05). Although mean HDL-C increased, the initial and final HDL-C distributions were broad and largely overlapping. Any chosen cutpoint for HDL-C goal would apply to only a minority of patients. The total/HDL cholesterol ratio had narrower distributions, as the percentage of patients with ratio5.0 increased from 17% to 67%.Combined statin-niacin therapy lowers LDL-C and raises HDL-C with acceptable tolerance and safety. If treating LDL-C is the primary goal, consistent with current guidelines, then a strategy of targeting the total/HDL cholesterol ratio as a secondary goal is applicable to more patients than targeting HDL-C itself.