Oesophageal atresia: The growth gap

Poor growth is an under-recognised yet significant long-term sequelae of oesophageal atresia(OA) repair. Few studies have specifically explored the reasons for growth impairment in this complex cohort. The association between poor growth with younger age and fundoplication appears to have the strongest supportive evidence, highlighting the need for early involvement of a dietitian and speech pathologist, and consideration of optimal medical reflux management prior to referring for anti-reflux surgery. However, it remains difficult to reach conclusions regarding other factors which may negatively influence growth, due to conflicting findings, inconsistent definitions and lack of validated tool utilisation. While swallowing and feeding difficulties are particularly frequent in younger children, their relationship with growth remains unclear. It is possible that these morbidities impact on the diet of children with OA, but detailed analysis of dietary composition and quality, and its relationship with these complications and growth, has not yet been conducted. Another potential area of research in OA is the role of the microbiota in growth and nutrition. While the microbiota has been linked to growth impairment in other paediatric conditions,it is yet to be investigated in OA. Further research is needed to identify the most,important contributory factors to poor growth, the role of the intestinal microbiota, and effective interventions to maximise growth and nutritional outcomes in this cohort.     

Imaging-based algorithmic approach to gallbladder wall thickening

Gallbladder(GB) wall thickening is a frequent finding caused by a spectrum of conditions. It is observed in many extracholecystic as well as intrinsic GB conditions. GB wall thickening can either be diffuse or focal. Diffuse wall thickening is a secondary occurrence in both extrinsic and intrinsic pathologies of GB, whereas, focal wall thickening is mostly associated with intrinsic GB pathologies. In the absence of specific clinical features, accurate etiological diagnosis can be challenging. The survival rate in GB carcinoma(GBC) can be improved if it is diagnosed at an early stage, especially when the tumor is confined to the wall. The pattern of wall thickening in GBC is often confused with benign diseases, especially chronic cholecystitis, xanthogranulomatous cholecystitis, and adenomyomatosis. Early recognition and differentiation of these conditions can improve the prognosis. In this minireview, the authors describe the patterns of abnormalities on various imaging modalities(conventional as well as advanced) for the diagnosis of GB wall thickening. This paper also illustrates an algorithmic approach for the etiological diagnosis of GB wall thickening and suggests a formatted reporting for GB wall abnormalities.     

Correlation between peripheral arterial disease and coronary artery disease using ankle brachial index-a study in Indian population

ObjectiveTo study the prevalence of peripheral arterial disease (PAD) of the lower limbs in a high-risk population and its correlation with coronary artery disease (CAD), using the ankle brachial index (ABI).MethodsThe present study was conducted in randomly selected indoor patients >45 years of age with one or more risk factors for PAD admitted in the cardiology and medicine wards in a tertiary care institute.ResultsBased on ABI <0.9, PAD was diagnosed in 32 of the 182 (18%) patients. Coronary artery disease was present in 15 cases of PAD which was statistically significant.ConclusionThere is a definite and strong correlation between PAD and CAD. Correct diagnosis and supervision of patients with PAD is important for preventing the local progression of the disease and effective secondary prevention of future coronary and cerebrovascular events.     

Outcome after repair of cor triatriatum

Cor triatriatum represents <0.1% of all congenital cardiac malformations. Symptoms in patients with cor triatriatum are related to pulmonary venous obstruction and pressure loading of the right side of the heart. The aim of this study was to describe our institutional experience with repair of cor triatriatum. From June 1963 to June 2010, 65 patients underwent repair at a median age of 7.2 months (range 2 days to 47.6 years). Among these patients, 49 (75%) had associated congenital heart defects. Atrial septal defect (n = 29), ventricular septal defect (n = 15), partially or totally anomalous pulmonary venous return (n = 14), mitral valve abnormalities (n = 11), and supravalvar mitral ring (n = 5) were the most common associated defects. Surgical treatment consisted of excision of the membrane, along with additional procedures in 47 patients (72%). Five patients had new postoperative supraventricular arrhythmias. During a median follow-up period of 5.4 years, no patients underwent reintervention for recurrent left atrial obstruction, 7 patients were noted to have minor residual cor triatriatum without obstruction, and 8 patients (including 4 diagnosed before cor triatriatum repair) had pulmonary vein stenosis, 6 of whom underwent intervention for that reason. In conclusion, in this large surgical series of patients who underwent repair of cor triatriatum, there were no cases of significant residual or recurrent cor triatriatum. Although the association between cor triatriatum and pulmonary vein stenosis has been described previously, the relative frequency of this condition in our cohort (>10%, including patients diagnosed before and after cor triatriatum repair) is noteworthy. Abnormalities of the mitral valve and a supravalvar mitral ring were also seen more often than the existing research would suggest, which may be another important consideration in evaluating and following these patients.     

Role of minimal residual disease monitoring in acute promyelocytic leukemia treated with arsenic trioxide in frontline therapy

Data on minimal residual disease (MRD) monitoring in acute promyelocytic leukemia (APL) are available only in the context of conventional all-trans retinoic acid plus chemotherapy regimens. It is recognized that the kinetics of leukemia clearance is different with the use of arsenic trioxide (ATO) in the treatment of APL. We undertook a prospective peripheral blood RT-PCR-based MRD monitoring study on patients with APL treated with a single agent ATO regimen. A total of 151 patients were enrolled in this study. A positive RT-PCR reading at the end of induction therapy was significantly associated on a multivariate analysis with an increased risk of relapse (relative risk = 4.9; P = .034). None of the good risk patients who were RT-PCR negative at the end of induction relapsed. The majority of the relapses (91%) happened within 3 years of completion of treatment. After achievement of molecular remission, the current MRD monitoring strategy was able to predict relapse in 60% of cases with an overall sensitivity and specificity of 60% and 93.2%, respectively. High-risk group patients and those that remain RT-PCR positive at the end of induction are likely to benefit from serial MRD monitoring by RT-PCR for a period of 3 years from completion of therapy.     

Association of antibiotics with status epilepticus

The objective of this study is to evaluate clinical picture, radiological findings and response to treatment in patients with antibiotic associated status epilepticus (SE). In a retrospective review, 12 out of 117 (10%) patients with SE had temporal association with antibiotic administration. Their medical history, clinical findings, and duration and type of SE were recorded. Serum chemistry, blood counts, cranial MRI, EEG and CSF examination were carried out. The offending antibiotics were withdrawn and the patients were treated with phenytoin, lorazepam, sodium valproate or midazolam. The response to treatment was recorded and death during hospital stay noted. The median age of the patients was 36 (18 and 74) years and 5 were females. Eight patients had convulsive and four nonconvulsive SE. The median duration of status was 12 h. The antibiotics related to SE included intravenous cephalosporin (ceftazidime 5, amoxyclavulenic acid 2, piperacillin 2, cefepime 1) and quinolones (levofloxacin 3, ofloxacin 1, ciprofloxacin 2) in isolation or in combinations. Five patients had hepatic (41.7%) and 6 (50%) renal failure; the later received higher than the recommended dose of antibiotics. Cranial MRI was abnormal in 7 out of 9 (77.8%) patients that include cortical lesion in one, corticosubcortical in three and subcortical in three. SE responded to first antiepileptic drug in four and to second in five patients. Three patients (25%) had refractory SE. Eight (66.7%) patients died and death was related to SE in 2 patients. 10% SE patients may be related to antibiotics. Hence the antibiotic should be carefully chosen in patients with hepatic and renal failure, and the dose should be modified.     

Utility of Sonoclot analysis and tranexamic acid in tetralogy of Fallot patients undergoing intracardiac repair

Sonoclot analysis is a point of care test to monitor the coagulation process, presenting a comprehensive evaluation of the clot formation and retraction as well as platelet function. This randomized double-blinded study was designed to investigate the utility of Sonoclot analysis in monitoring the coagulation profile as also the antifibrinolytic effects of tranexamic acid administered in patients with tetralogy of Fallot undergoing intracardiac repair. Eighty of a total 94 patients were randomly divided into two groups of 40 each. In the study group, TA was administered thrice at a dosage of 10 mg/kg, i.e. before CPB, on CPB and after CPB, whereas in the control group, placebo was administered at the same time intervals. Sonoclot analysis and D-dimer measurement were performed at baseline and following heparin neutralisation. An additional variable, DR?? (diminishing rate of clot strength at 15 min postmaximal clot strength), was calculated from the Sonoclot graph and was compared with d-dimer levels as a measure of fibrinolysis. The three Sonoclot variables, i.e. activated clotting time, clot rate and platelet function, were deranged at baseline in all the patients. Post-CPB, the change in these variables was not significant. ACT, clot rate and platelet function showed no significant (P > 0.05) difference in both the groups at both the time intervals. DR?? and d-dimer values were comparable at baseline in both the groups. However, a significant (P < 0.05) difference was seen in these variables in the control group as compared with the TA group following heparin neutralisation. To conclude, Sonoclot analysis is a useful, point of care method for the monitoring of coagulation and fibrinolysis in patients with tetralogy of Fallot undergoing intracardiac repair.     

QRS characteristics fail to reliably identify ventricular tachycardias that require epicardial ablation in ischemic heart disease

Criteria for Epicardial Origin in Ischemic VT. Objectives: We tested proposed algorithms for idiopathic and nonischemic tachycardias for their ability to identify epicardial LV‐VT origins. Backgroud: Several ECG features have been reported to identify epicardial origins for left ventricular tachycardias (LV‐VTs) in the absence of myocardial infarction. Only limited data exist in postinfarction patients. Methods: The QRS features of 24 VTs that were ablated from the epicardium and 39 left ventricular VTs ablated from the endocardium were retrospectively analyzed for various 12‐lead ECG features previously reported. Results: No ECG feature consistently predicted an epicardial LV‐VT origin in infarct‐related tachycardias, with epicardial VTs showing slightly longer QRS durations (189 ± 32 ms in epicardial vs 179 ± 37 ms in endocardial, P = 0.28). Pseudo‐delta duration was 38 ± 27 versus 47 ± 27 ms (P = 0.2), intrinsicoid deflection time 93 ± 35 versus 86 ± 32 ms (P = 0.4), shortest RS 97 ± 38 versus 99 ± 32 ms (P = 0.77), and median deflection index 0.82 ± 0.25 versus 0.87 ± 0.22 (P = 0.43). The finding of a Q wave in lead I and the absence of a Q wave in the inferior leads failed to predict an epicardial origin in superior LV‐VT sites. Q waves in any inferior lead and aVR/aVL‐ratio<1 were not specific for an epicardial origin in inferior sites (all P = ns). Furthermore, all inferior LV‐VTs showed a Q wave in the inferior leads which correlated with pre‐existing Q‐waves in sinus rhythm (P = 0.045). Conclusion : Proposed 12‐lead ECG features for differentiation of epicardial versus endocardial sites for nonischemic LV‐VTs do not reliably identify VTs that require ablation from the epicardium. Endocardial mapping should be the first approach to catheter ablation for VTs in patients with ischemic heart disease. (J Cardiovasc Electrophysiol, Vol. 23, pp. 188‐193, February 2012)     

Association of serum sex steroid receptor bioactivity and sex steroid hormones with breast cancer risk in postmenopausal women

Postmenopausal women with elevated serum sex steroids have an increased risk of breast cancer. Most of this risk is believed to be exerted through binding of the sex steroids to their receptors. For the first time, we investigate the association of estrogen receptor (ER) and androgen receptor (AR) serum bioactivity (SB) in addition to hormone levels in samples from women with breast cancer collected before diagnosis. Two hundred postmenopausal women participating in the UK Collaborative Trial of Ovarian Cancer Screening who developed ER-positive breast cancer 0.6-5 years after sample donation were identified and matched to 400 controls. ER and AR bioassays were used to measure ERα, ERβ, and AR SB. Androgen and estrogen levels were measured with immunoassays. Subjects were classified according to quintiles of the respective marker among controls and the associations between SB and hormones with breast cancer risk were determined by logistic regression analysis. ERα and ERβ SB were significantly higher before diagnosis compared with controls, while estrogens showed no difference. Women had a twofold increased breast cancer risk if ERα SB (odds ratio (OR), 2.114; 95% confidence interval (CI), 1.050-4.425; P=0.040) was in the top quintile >2 years before diagnosis or estrone (OR, 2.205; 95% CI, 1.104-4.586; P=0.029) was in the top quintile <2 years before diagnosis. AR showed no significant association with breast cancer while androstenedione (OR, 3.187; 95% CI, 1.738-6.044; P=0.0003) and testosterone (OR, 2.145; 95% CI, 1.256-3.712; P=0.006) were significantly higher compared with controls and showed a strong association with an almost threefold increased breast cancer risk independent of time to diagnosis. This study provides further evidence on the association of androgens and estrogens with breast cancer. In addition, it reports that high ER but not AR SB is associated with increased breast risk >2 years before diagnosis.