Cerebral hemodynamics in children with sickle cell disease in India: An observational cohort study.

India has the second highest number of cases of sickle cell disease (SCD) and affects the most socioeconomically disadvantaged communities living in a horizontal belt from Gujarat to Odisha state. Despite high prevalence, information about cerebral hemodynamics among children with SCD in India remains scarcely described. We performed transcranial Doppler (TCD) to assess cerebral hemodynamics among Indian children with SCD and evaluated their association with clinical and hematological parameters. Children aged 3-18years, diagnosed with SCD living in Raipur in Chhattisgarh and Ahmedabad in Gujarat state were recruited. TCD was performed to obtain flow velocities from middle cerebral (MCA), intracranial internal carotid (ICA) and basilar artery. Associations were evaluated between timed-average-mean-maximum velocities (TAMMV) and various clinical and hematological parameters. Our prospective study included 62 consecutive children with known SCD. Mean ± SD age of the study population was 9.8 ± 3.9 years and 31 (50%) were male. Mean ± SD hemoglobin was 8.64 ± 1.34 Gm/dL while the mean HbSS ± SD was 70.25 ± 15.27%. While 6 (9.6%) children had suffered from stroke during previous 2 years, 7 (11%) demonstrated abnormal TAMMV. Higher HbSS level along with history of iron chelation therapy, blood transfusion and/or stroke showed a trend towards having higher TAMMV. Stroke and cerebral hemodynamic alterations are common among Indian children with SCD. Larger studies with detailed neuroimaging and genetic evaluations are needed for better understanding, characterization, risk stratification as well as optimization of the timing of blood transfusion to reduce physical disabilities among Indian children with SCD.

Functionalized Carbon Nanotube and MnO2 Nanoflower Hybrid as an Electrode Material for Supercapacitor Application.

Functionalized carbon nanotube (FCNT) and Manganese Oxide (MnO2) nanoflower hybrid material was synthesized using hydrothermal technique as a promising electrode material for supercapacitor applications. The morphological investigation revealed the formation of 'nanoflower' like structure of MnO2 connected with FCNT, thus paving an easy path for the conduction of electrons during the electrochemical mechanism. A significant improvement in capacitance properties was observed in the hybrid material, in which carbon nanotube acts as a conducting cylindrical path, while the major role of MnO2 was to store the charge, acting as an electrolyte reservoir leading to an overall improved electrochemical performance. The full cell electrochemical analysis of FCNT-MnO2 hybrid using 3 M potassium hydroxide (KOH) electrolyte indicated a specific capacitance of 359.53 F g-1, specific energy of 49.93 Wh kg-1 and maximum specific power of 898.84 W kg-1 at 5 mV s-1. The results show promise for the future of supercapacitor development based on hybrid electrode materials, where high specific energy can be achieved along with high specific power and long cycle life.

Modified Activation Process for Supercapacitor Electrode Materials from African Maize Cob.

In this work, African maize cobs (AMC) were used as a rich biomass precursor to synthesize carbon material through a chemical activation process for application in electrochemical energy storage devices. The carbonization and activation were carried out with concentrated Sulphuric acid at three different temperatures of 600, 700 and 800 °C, respectively. The activated carbon exhibited excellent microporous and mesoporous structure with a specific surface area that ranges between 30 and 254 m2·g-1 as measured by BET analysis. The morphology and structure of the produced materials are analyzed through Field Emission Scanning Electron Microscopy (FESEM), Fourier Transform Infrared Spectroscopy (FTIR), X-Ray Diffraction (XRD), Boehm titration, X-ray Photoelectron Spectroscopy (XPS) and Raman Spectroscopy. X-ray photoelectron spectroscopy indicates that a considerable amount of oxygen is present in the materials. The functional groups in the activated carbon enhanced the electrochemical performance and improved the material's double-layer capacitance. The carbonized composite activated at 700 °C exhibited excellent capacitance of 456 F g-1 at a specific current of 0.25 A g-1 in 6 M KOH electrolyte and showed excellent stability after 10,000 cycles. Besides being a low cost, the produced materials offer good stability and electrochemical properties, making them suitable for supercapacitor applications.

Visual failure and sinus thrombosis following depressed skull fracture: management with single session lumboperitoneal shunt and sinus decompression -case report.

Elevation of a depressed skull fracture (DSF) overlying a venous sinus is generally avoided due to risk of sinus injury. Rarely, the sinus may be compressed by the fractured segment, causing intracranial hypertension (IH) or encephalopathy and can only be diagnosed with cerebral angiography or MR imaging techniques. The posterior third of the superior sagittal sinus (SSS) was found involved in all the reported cases. There is no consensus on its management, but most patients did not have any sinus thrombosis and improved after elevation of the compressing DSF alone. There are isolated reports of improvement with serial lumbar punctures or ventriculoperitoneal shunt.We report for the first time, a single session lumboperitoneal shunt and DSF elevation for a patient with thrombosis of the posterior part of SSS due to an overlying DSF, causing florid papilledema and impending blindness. Elevation of the DSF alone would not have achieved patency of the thrombosed sinus immediately to save his vision. Hence a lumboperitoneal shunt was done for immediate relief of IH. This CSF diversion alone was insufficient to achieve patency of the thrombosed sinus in the presence of significant external compression by the DSF; even if anticoagulants were used. Hence we elevated the DSF after craniotomy using a technical modification; by making a wide gutter circumferentially around the DSF with a high speed drill, to avoid sinus injury; prior to its elevation. The patient's vision improved in 48 hours and anticoagulants were used till 2 months when sinuses were found normal on MRI. Serial CT &MR images showing progression to thrombosis and recovery after decompression are presented. Both CSF diversion and DSF elevation are necessary when vision is threatened by IH due to sinus thrombosis with an overlying DSF.

Mediastinal Pseudocyst in Acute on Chronic Pancreatitis.

Pseudocyst is a common complication of Acute and chronic pancreatitis. However, its extension into the mediastinum is a rare entity. We present a case of 52 years male with acute on chronic pancreatitis (alcohol related) who presented with dysphagia and dyspnoea and was found to have a pancreatic pseudocyst extending upto the neck. Ultrasound fails to pick up mediastinal pseudocysts and requires additional imaging modalities - CT and MRI. Management of Mediastinal pseudocyst depends upon underlying etiology, ductal anatomy, size of the pseudocyst, and availability of expertise. Small pseudocysts in asymptomatic patients may resolve spontaneously, but requires prolonged conservative therapy with somatostatin or its analogue and Total Parenteral Nutrition. Ruptured pseudocyst in a symptomatic unstable patient requires surgical resection. Endoscopic ultrasound guided drainage (transmural or transpapillary) and Main Pancreatic Duct stenting are safe and effective treatment modality.

Comparison of speed of inhalational induction in children with and without congenital heart disease.

BACKGROUND: Conduct of stable inhalational anesthetic induction in children with congenital heart disease (CHD) presents special challenges. It requires in-depth understanding of the effect of congenital shunt lesions on the uptake, delivery, and equilibration of anesthetic drugs. Intracardiac shunts can alter the induction time and if delivery of anesthetic agent is not carefully titrated, can lead to overdosing and undesirable myocardial depression. AIMS: To study the effect of congenital shunt lesions on the speed of inhalational induction and also the impact of inhalational induction on hemodynamics in the presence of congenital shunt lesions. SETTING: Tertiary care hospital. DESIGN: A prospective, single-center clinical study. MATERIALS AND METHODS: Ninety-three pediatric patients undergoing elective surgery were segregated into three equal groups, namely, Group 1: no CHD, Group 2: acyanotic CHD, and Group 3: cyanotic CHD. General anesthesia was induced with 8% sevoflurane in 6 L/min air-oxygen. The time to induction was noted at loss of eyelash reflex and decrease in bispectral index (BIS) value below 60. End-tidal sevoflurane concentration, minimum alveolar concentration, and BIS were recorded at 15 s intervals for the 1 st min followed by 30 s interval for another 1 min during induction. Hemodynamic data were recorded before and after induction. RESULTS: Patients in Group 3 had significantly prolonged induction time (99 ± 12.3 s; P < 0.001), almost twice that of the patients in other two groups (51 ± 11.3 s in Group 1 and 53 ± 12.0 s in Group 2). Hypotension occurred after induction in Group 1. No other adverse hemodynamic perturbations were observed. CONCLUSION: The time to inhalational induction of anesthesia is significantly prolonged in patients with right-to-left shunt, compared to patients without CHD or those with left-to-right shunt, in whom it is similar. Sevoflurane is safe and maintains stable hemodynamics in the presence of CHD.

Infective endocarditis of main pulmonary artery in tetralogy of Fallot: "Transesophageal echocardiography adds lease of life".

Infective endocarditis is a rare occurrence in the main pulmonary artery trunk and even rarer in tetralogy of Fallot.

Lactate, endothelin, and central venous oxygen saturation as predictors of mortality in patients with Tetralogy of Fallot.

BACKGROUND: Lactate and central venous oxygen saturation (ScVO2) are well known biomarkers for adequacy of tissue oxygenation. Endothelin, an inflammatory marker has been associated with patient's nutritional status and degree of cyanosis. The aim of this study was to explore the hypothesis that lactate, ScVO2 and endothelin before induction may be predictive of mortality in pediatric cardiac surgery. METHODS: We conducted a prospective observational study of 150 pediatric (6 months to 12 years) patients who were posted for intracardiac repair for Tetralogy of Fallot and measured lactate, ScVO2 and endothelin before induction (T1), 20 minutes after protamine administration (T2) and 24 hours after admission to ICU (T3). RESULTS: Preinduction lactate and endothelin levels were found to predict mortality in patients of Tetralogy of Fallot with an odds ratio of 6.020 (95% CI 2.111-17.168) and 1.292(95% CI 1.091-1.531) respectively. In the ROC curve analysis for lactate at T1, the AUC was 0.713 (95% CI 0.526-0.899 P = 0.019). At the cutoff value of 1.750 mmol/lt, the sensitivity and specificity for the prediction of mortality was 63.6% and 65.5%, respectively. For endothelin at T1, the AUC was 0.699 (95% CI 0.516-0.883, P = 0.028) and the cutoff value was ≤2.50 (sensitivity, 63.6%; specificity, 58.3 %). ScVO2 (odds ratio 0.85) at all three time intervals, suggested that improving ScVO2 can lead to 15% reduction in mortality. CONCLUSIONS: Lactate, ScVO2 and endothelin all showed association with mortality with lactate having the maximum prediction. Lactate was found to be an independent, reliable and cost-effective measure of prediction of mortality in patients with Tetralogy of Fallot.

Hydrogen Adsorption in Flame Synthesized and Lithium Intercalated Carbon Nanofibers--A Comparative Study.

Carbon nanofibers (CNF) have been synthesized under partial combustion conditions in a flame reactor using different mixtures of hydrocarbon gases in the presence and absence of precursors. The hydrogen (H2) adsorption studies have been carried out using a high pressure Sievert's apparatus maintained at a constant temperature (24 degrees C). The flame synthesized CNFs showed high degree of H2 adsorption capacities at 100 atm pressure. The highest H2 capacities recorded have been 4.1 wt% [for CNF produced by liquefied petroleum gas (LPG)-Air (E-17)], 3.7 wt% [for nano carbons produced by Methane-Acetylene-Air (EMAC-4)] and 5.04 wt% for [Lithium intercalated sample (Li-EMAC-4)] respectively.

Interventricular septal hydatid cyst: Transesophageal echocardiography as a therapeutic tool during bypass.

Cystic echinococcosis (hydatid disease) arising from infestation with a larval or adult form of the Echinococcus granulosus tapeworm is endemic in certain states of India, but affecting interventricular septum (IVS) solitarily is a scarce phenomenon. We present a rare case of transesophageal echocardiography guided management of IVS hydatid cyst even during cardiopulmonary bypass, which presented with a rather unusual complaint of repeated syncope.

Paediatric pulmonary haemorrhage: Independent lung ventilation as effective strategy in management.

Pulmonary haemorrhage is an uncommon symptom in paediatrics with the etiology varying among the series by age, location, and the diagnostic tests employed. Once airway protection and volume resuscitation are ensured, localization of the anatomic site of bleeding, isolation of the involved airway, control of haemorrhage and treatment of the underlying cause of becomes essential. In localized persistent bleeding, airway control may be achieved by lung isolation with double lumen endotracheal tube and synchronous independent lung ventilation.

Flame synthesis of carbon nano onions using liquefied petroleum gas without catalyst.

Densely agglomerated, high specific surface area carbon nano onions with diameter of 30-40 nm have been synthesized. Liquefied petroleum gas and air mixtures produced carbon nano onions in diffusion flames without catalyst. The optimized oxidant to fuel ratio which produces carbon nano onions has been found to be 0.1 slpm/slpm. The experiment yielded 70% pure carbon nano onions with a rate of 5 g/h. X-ray diffraction, high-resolution electron microscopy and Raman spectrum reveal the densely packed sp(2) hybridized carbon with (002) semi-crystalline hexagonal graphite reflection. The carbon nano onions are thermally stable up to 600 °C.

The major mutation in the RMRP gene causing CHH among the Amish is the same as that found in most Finnish cases.

Cartilage-hair hypoplasia (CHH), or McKusick type metaphyseal chondrodysplasia, was originally described in the Old Order Amish in the United States and subsequently found to be unusually frequent among Finns. The major mutation causing CHH in Finns is a 70A --> G nucleotide substitution in the RMRP gene, which encodes the untranslated RNA that is a component of mitochondrial RNA-processing endoribonuclease. Here we report that the same mutation is the most frequent one, perhaps the only one, in the Amish population in which CHH was first characterized. The fact that the mutation segregates with the same major haplotype in these two populations and others suggests that it is very ancient. Unlike some other ordinarily rare recessive disorders that are limited in their high frequency to a single Amish deme (subisolate), e.g., Ellis-van Creveld syndrome, CHH occurs in high frequency in at least three distinct Amish demes, indicating, along with genealogic data, that there were multiple heterozygotes among the founders, as proposed by McKusick et al. [1965: Bull Johns Hopkins Hosp 116:231-272].

Epigenetics: the role of methylation in the mechanism of action of tumor suppressor genes.

Epigenetics is the study of mitotically heritable changes in gene expression without any changes in the primary DNA sequence. The major step in epigenetic gene regulation is gene inactivation by hypermethylation of CpG islands located in the promoter region. Specific enzymes and methylated DNA binding proteins play a major role in causing reduced expression of tumor suppressor genes, resulting in tumor formation and its progression. Prevention approaches are needed to avoid tumor formation. One approach to inhibiting inactivation of tumor suppressor genes is to use chemical agents such as 5-azacytidine to prevent hypermethylation of DNA. Increased understanding of the mechanism of epigenetic silencing and the identification of additional molecular mechanisms (e.g., histone methylases) that may be targeted by pharmaceutical interventions may lead to more preventive strategies. The current status of the epigenetic regulation of tumor suppressor genes is discussed in this review article.

Early detection and risk assessment: proceedings and recommendations from the Workshop on Epigenetics in Cancer Prevention.

Recent advances in molecular biology that have provided a greater understanding of multistage carcinogenesis include the use of biomarkers of early detection and risk assessment. Prominent among such biomarkers are epigenetic changes. The field of epigenetics has seen a recent surge of interest among cancer researchers since alterations in DNA methylation have emerged as one of the most consistent molecular alterations in multiple neoplasms. Chromatin condensation, histone deacetylation, and promoter methylation are major steps in the epigenetic regulation of gene expression. Epigenetic changes may occur due to environmental factors, aging, and genomic imprinting. An important distinction between genetic and epigenetic alterations in cancer prevention is that the latter might be more easily reversed using therapeutic interventions. In the workshop the following areas of research were recognized for emphasis in future work: (1) basic epigenetic mechanisms in cancer need further investigation; (2) technology development in the area of epigenetics, such as high-throughput quantitative assays and increased sensitivity/specificity, is essential for the early detection and risk assessment of cancer; (3) the clinical application of epigenetic changes to cancer prevention and risk assessment needs further investigation. Further research will lead to the identification of new targets for cancer prevention.