Outcomes of patients with exomphalos and associated congenital heart diseases.

INTRODUCTION: Exomphalos is an anterior abdominal wall defect resulting in herniation of contents into the umbilical cord. Severe associated chromosomal anomalies and congenital heart disease (CHD) are known to influence mortality, but it is not clear which cardiac anomalies have the greatest impact on survival. METHODS: We performed a retrospective review of the treatment and outcome of patients with exomphalos over a 30-year period (1990-2020), with a focus on those with the combination of exomphalos major and major CHD (EMCHD). RESULTS: There were 123 patients with exomphalos identified, 59 (48%) had exomphalos major (ExoMaj) (defect > 5 cm or containing liver), and 64 (52%) exomphalos minor (ExoMin). In the ExoMaj group; 17% had major CHD (10/59), M:F 28:31, 29% premature (< 37 weeks, 17/59) and 14% had low birth-weight (< 2.5 kg, 8/59). In the ExoMin group; 9% had major CHD (6/64), M:F 42:22, 18% premature and 10% had low birth-weight. The 5-year survival was 20% in the EMCHD group versus 90% in the ExoMaj with minor or no CHD [p < 0.0001]. Deaths in the EMCHD had mainly right heart anomalies and all of them required mechanical ventilation (MV) for pulmonary hypoplasia prior to cardiac intervention. In contrast, survivors did not require mechanical ventilation prior to cardiac intervention. CONCLUSION: EMCHD is associated with high mortality. The most significant finding was high mortality in those with right heart anomalies in combination with pulmonary hypoplasia, especially if pre-intervention mechanical ventilation is required.

Quasi-Chemical Theory with Cluster Sampling from Ab Initio Molecular Dynamics: Fluoride (F-) Anion Hydration.

Accurate predictions of the hydration free energy for anions typically has been more challenging than that for cations. Hydrogen bond donation to the anion in hydrated clusters such as F(H2O) n - can lead to delicate structures. Consequently, the energy landscape contains many local minima, even for small clusters, and these minima present a challenge for computational optimization. Utilization of cluster experimental results for the free energies of gas-phase clusters shows that even though anharmonic effects are interesting they need not be of troublesome magnitudes for careful applications of quasi-chemical theory to ion hydration. Energy-optimized cluster structures for anions can leave the central ion highly exposed, and application of implicit solvation models to these structures can incur more serious errors than those for metal cations. Utilizing cluster structures sampled from ab initio molecular dynamics simulations substantially fixes those issues.

Comparison of single-ion molecular dynamics in common solvents.

Laying a basis for molecularly specific theory for the mobilities of ions in solutions of practical interest, we report a broad survey of velocity autocorrelation functions (VACFs) of Li+ and PF6- ions in water, ethylene carbonate, propylene carbonate, and acetonitrile solutions. We extract the memory function, γ(t), which characterizes the random forces governing the mobilities of ions. We provide comparisons controlling for the effects of electrolyte concentration and ion-pairing, van der Waals attractive interactions, and solvent molecular characteristics. For the heavier ion (PF6-), velocity relaxations are all similar: negative tail relaxations for the VACF and a clear second relaxation for γt, observed previously also for other molecular ions and with n-pentanol as the solvent. For the light Li+ ion, short time-scale oscillatory behavior masks simple, longer time-scale relaxation of γt. But the corresponding analysis of the solventberg Li+H2O4 does conform to the standard picture set by all the PF6- results.

Molecular-Scale Description of SPAN80 Desorption from a Squalane-Water Interface.

Extensive all-atom molecular dynamics calculations on the water-squalane interface for nine different loadings with sorbitan monooleate (SPAN80), at T = 300 K, are analyzed for the surface tension equation of state, desorption free-energy profiles as they depend on loading, and to evaluate escape times for adsorbed SPAN80 into the bulk phases. These results suggest that loading only weakly affects accommodation of a SPAN80 molecule by this squalane-water interface. Specifically, the surface tension equation of state is simple through the range of high tension to high loading studied, and the desorption free-energy profiles are weakly dependent on loading here. The perpendicular motion of the centroid of the SPAN80 headgroup ring is well-described by a diffusional model near the minimum of the desorption free-energy profile. Lateral diffusional motion is weakly dependent on loading. Escape times evaluated on the basis of a diffusional model and the desorption free energies are 7 × 10-2 s (into the squalane) and 3 × 102 h (into the water). The latter value is consistent with desorption times of related lab-scale experimental work.

Molecular characterization and combined genotype association study of bovine cluster of differentiation 14 gene with clinical mastitis in crossbred dairy cattle.

AIM: The present study was undertaken with the objectives to characterize and to analyze combined genotypes of cluster of differentiation 14 (CD14) gene to explore its association with clinical mastitis in Karan Fries (KF) cows maintained in the National Dairy Research Institute herd, Karnal. MATERIALS AND METHODS: Genomic DNA was extracted using blood of randomly selected 94 KF lactating cattle by phenol-chloroform method. After checking its quality and quantity, polymerase chain reaction (PCR) was carried out using six sets of reported gene-specific primers to amplify complete KF CD14 gene. The forward and reverse sequences for each PCR fragments were assembled to form complete sequence for the respective region of KF CD14 gene. The multiple sequence alignments of the edited sequence with the corresponding reference with reported Bos taurus sequence (EU148610.1) were performed with ClustalW software to identify single nucleotide polymorphisms (SNPs). Basic Local Alignment Search Tool analysis was performed to compare the sequence identity of KF CD14 gene with other species. The restriction fragment length polymorphism (RFLP) analysis was carried out in all KF cows using Helicobacter pylori 188I (Hpy188I) (contig 2) and Haemophilus influenzae I (HinfI) (contig 4) restriction enzyme (RE). Cows were assigned genotypes obtained by PCR-RFLP analysis, and association study was done using Chi-square (χ (2)) test. The genotypes of both contigs (loci) number 2 and 4 were combined with respect to each animal to construct combined genotype patterns. RESULTS: Two types of sequences of KF were obtained: One with 2630 bp having one insertion at 616 nucleotide (nt) position and one deletion at 1117 nt position, and the another sequence was of 2629 bp having only one deletion at 615 nt position. ClustalW, multiple alignments of KF CD14 gene sequence with B. taurus cattle sequence (EU148610.1), revealed 24 nt changes (SNPs). Cows were also screened using PCR-RFLP with Hpy188I (contig 2) and HinfI (contig 4) RE, which revealed three genotypes each that differed significantly regarding mastitis incidence. The maximum possible combination of these two loci shown nine combined genotype patterns and it was observed only eight combined genotypes out of nine: AACC, AACD, AADD, ABCD, ABDD, BBCC, BBCD, and BBDD. The combined genotype ABCC was not observed in the studied population of KF cows. Out of 94 animals, AACD combined genotype animals (10.63%) were found to be not affected with mastitis, and ABDD combined genotyped animals was observed having the highest mastitis incidence of 15.96%. CONCLUSION: AACD typed cows were found to be least susceptible to mastitis incidence as compared to other combined genotypes.

Loop-Closure and Gaussian Models of Collective Structural Characteristics of Capped PEO Oligomers in Water.

Parallel-tempering MD results for a CH3(CH2-O-CH2)mCH3 chain in water are exploited as a database for analysis of collective structural characteristics of the PEO globule with a goal of defining models permitting statistical thermodynamic analysis of dispersants of Corexit type. The chain structure factor, relevant to neutron scattering from a deuterated chain in null water, is considered specifically. The traditional continuum-Gaussian structure factor is inconsistent with the simple k → ∞ behavior, but we consider a discrete-Gaussian model that does achieve that consistency. Shifting and scaling the discrete-Gaussian model helps to identify the low-k to high-k transition near k ≈ 2π/0.6 nm when an empirically matched number of Gaussian links is about one-third of the total number of effective atom sites. This short distance-scale boundary of 0.6 nm is directly verified with the r space distributions, and this distance is thus identified with a natural size for coarsened monomers. The probability distribution of Rg(2) is compared with the classic predictions for both the Gaussian model and freely jointed chains. ⟨Rg(2)(j)⟩, the contribution of the jth chain segment to ⟨Rg(2)⟩, depends on the contour index about as expected for Gaussian chains despite significant quantitative discrepancies that express the swelling of these chains in water. Monomers central to the chain contour occupy the center of the chain globule. The density profiles of chain segments relative to their center of mass can show distinctive density structuring for smaller chains due to the close proximity of central elements to the globule center. However, that density structuring washes out for longer chains where many chain elements additively contribute to the density profiles. Gaussian chain models thus become more satisfactory for the density profiles for longer chains.

Concentration dependence of the Flory-Huggins interaction parameter in aqueous solutions of capped PEO chains.

The dependence on volume fraction φ of the Flory-Huggins interaction parameter χwp(φ) describing the free energy of mixing of polymers in water is obtained by exploiting the connection of χwp(φ) to the chemical potential of the water, for which quasi-chemical theory is satisfactory. We test this theoretical approach with simulation data for aqueous solutions of capped PEO (polyethylene oxide) oligomers. For CH3(CH2-O-CH2)mCH3 (m = 11), χwp(φ) depends strongly on φ, consistent with experiment. These results identify coexisting water-rich and water-poor solutions at T = 300 K and p = 1 atm. Direct observation of the coexistence of these two solutions on simulation time scales supports that prediction for the system studied. This approach directly provides the osmotic pressures. The osmotic second virial coefficient for these chains is positive, reflecting repulsive interactions between the chains in the water, a good solvent for these chains.

Demonstration of side coupling to cladding modes through zinc oxide nanorods grown on multimode optical fiber.

A novel concept is introduced that utilizes the scattering properties of zinc oxide nanorods to control light guidance and leakage inside optical fibers coated with nanorods. The effect of the hydrothermal growth conditions of the nanorods on light scattering and coupling to optical fiber are experimentally investigated. At optimum conditions, 5% of the incident light is side coupled to the cladding modes. This coupling scheme could be used in different applications such as distributed sensors and light combing. Implementation of the nanorods on fiber provides low cost and controllable nonlithography-based solutions for free space to fiber coupling. Higher coupling efficiencies can be achieved with further optimization.

Prospective, randomised controlled trial comparing intense endoscopic cleaning versus minimal intervention in the early post-operative period following functional endoscopic sinus surgery.

OBJECTIVE: There is currently no standardised management protocol following functional endoscopic sinus surgery. This study assessed frequent endoscopic cleaning versus minimal intervention in the early post-operative period following such surgery. STUDY DESIGN: Prospective, randomised controlled, single-blinded, within-subject trial involving 24 patients with bilateral chronic rhinosinusitis undergoing bilateral functional endoscopic sinus surgery. MAIN OUTCOME MEASURE: The primary outcome measure was ethmoid cavity healing, based on endoscopic appearance, graded using a modified Lund-MacKay endoscopic score. SECONDARY OUTCOME MEASURE: Lund-MacKay symptom score before and after surgery. RESULTS: There was no overall statistically significant difference between the two groups (p = 0.37). Subgroup analysis revealed a significant effect of regular suction clearance on adhesions at three months (p = 0.048), but not on oedema, polyps, granulation, discharge or crusting. CONCLUSION: There is no evidence from this study to support frequent endoscopic cleaning in the early post-operative period after functional endoscopic sinus surgery. Less intensive post-operative management is recommended, resulting in decreased patient morbidity and fewer post-operative follow-up appointments.

Cardiac transplantation in adults with congenital heart disease.

BACKGROUND: Due to increasing success with repair or palliation in childhood, there is a rapidly growing population of adult patients with complex congenital heart disease who may require transplantation. There remains little data on outcomes of cardiac transplantation in this group. METHODS: 38 orthotopic cardiac transplants were performed in 37 patients (18 men) > or =18 years of age with congenital heart disease (CHD) from 1988 to 2009 in our institution. Outcomes were reviewed using medical records and transplant databases. RESULTS: 15 patients (41%) had univentricular and 22 (59%) biventricular physiology. The biggest group was transposition of the great arteries following atrial switch in eight patients (22%). Six (16%) had no previous surgical intervention. Mean age at transplant was 33.5 years (range 19.1-59.9 years). 11 patients (30%) required additional surgical procedures at transplant. 16 (43%) died, 12 early and 4 late deaths (1.8, 2.4, 2.7 and 7 years). Survival was 70% at 30 days, 68% at 1 year, 58% at 5 years and 53% at 10 and 15 years. Outcome improved in later eras with reduction in 30-day mortality from 50% to 18% and increase in 5-year survival from 50% to 69%. Two patients developed post-transplant lymphoproliferative disease. None required long-term renal replacement therapy. One patient was re-transplanted for cardiac allograft vasculopathy. CONCLUSIONS: While operative mortality following cardiac transplantation for adult congenital heart disease is higher than for other diagnostic groups, long-term survival is good and comparable to patients without CHD. Disappointing early results are improved with increasing experience.

The Oncosurge strategy for the management of colorectal liver metastases - an external validation study.

INTRODUCTION: Because most patients with colorectal liver metastases (CRLM) present to general surgeons and oncologists without a specialist interest in their management, a computer program (OncoSurge) has been created that identifies individual patient resectability and recommends optimal treatment strategies. The aim of this study was to validate the Oncosurge strategy by comparing its recommendations with the decisions made by a multidisciplinary (MDT) meeting, in a supra-regional hepatobiliary referral centre, using real cases with known outcomes. METHODS: We reviewed the records and imaging of 98 consecutive patients with CRLM, who had been referred for decision making to our MDT meeting between January 1, 2004 and December 31, 2004. All patient and tumour characteristics were entered onto the Oncosurge decision model, which was accessed at www.evidis.com/oncosurge/ RESULTS: There was concordance between Oncosurge and MDT decisions in 93/98 cases. The observed kappa (kappa) was 0.850 (95% CI: 0.728-0.972). In descriptive terms, a kappa score greater than 0.8 equates to "almost perfect agreement". CONCLUSIONS: Our results demonstrate the validity of the Oncosurge system when compared to one year of decision making in an established hepatobiliary MDT meeting. This confirms the utility of the Oncosurge system for decision making, audit and educational purposes.

Impaired reproductive fitness in mothers of children with juvenile autoimmune arthropathies.

OBJECTIVE: To assess the reproductive fitness of mothers of children with juvenile idiopathic arthritis (JIA). METHODS: A mail survey assessing pregnancy outcome was carried out among mothers of children with JIA (JIA mothers) treated at a tertiary paediatric rheumatology centre. The best friends of the JIA mothers served as controls. Besides family history, sociodemographics and reproductive outcomes were measured, including the number of pregnancies, pregnancy complications and gestational age at the time of delivery. RESULTS: JIA mothers (n = 227) and controls (n = 235) had similar sociodemographics and racial backgrounds. On average, JIA mothers reported a greater number of conceptions than controls (3.5 vs 3.1; P = 0.01) but had significantly higher rates of pregnancy complications (25% vs 15%; P<0.001). Corrected for differences in the absolute number of pregnancies between groups, the chances of having a miscarriage [mean (s.d.), 0.12 (0.18) vs 0.09 (0.16); P = 0.02] or preterm delivery [0.08 (0.21) vs 0.04 (0.15); P<0.02] were significantly greater among JIA mothers than controls. CONCLUSIONS: Mothers of children with JIA have impaired reproductive fitness. This phenomenon is unlikely to be the result of difficulty with conception but rather to be due to higher rates of pregnancy loss and premature delivery.

Plasminogen activators: a comparison.

Thrombolytic drugs play a crucial role in the management of patients with acute myocardial infarction, pulmonary embolism, deep vein thrombosis, arterial thrombosis, acute thrombosis of retinal vessel, extensive coronary emboli, and peripheral vascular thromboembolism. Recognition of the importance of fibrinolytic system in thrombus resolution has resulted in the development of different fibrinolytic agents. Now a days several newer plasminogen activators with different pharmacokinetic and pharmacodynamic properties have been developed to treat thrombotic disease, which are fibrin specific with prolonged half-life and can be administered as a single bolus.

Impacts of mining activities on water and soil.

Seven coal mines are situated in Wardha River Valley. These mines are located at Wani (Dist. Yavatmal of Maharashtra). Out of these, 5 open cast coal mines are run by Western Coal Field Ltd. India. The present study has been undertaken to assess the impacts of mining activities in the adjacent areas. Total 25 samples of water and 19 samples of soil from Nilapur, Bramhani, Kolera, Gowari, Pimpari and Aheri were analyzed for pH, TDS, hardness, alkalinity, fluoride, chloride, nitrite, nitrate, phosphate, sulfate, cadmium, lead, zinc, copper, nickel, arsenic, manganese, sodium and potassium, and the results were compared with the limits of Indian Standards: 10500.

Sildenafil in neonatal pulmonary hypertension due to impaired alveolarisation and plexiform pulmonary arteriopathy.

We report a case of severe pulmonary hypertension in a neonate associated with impaired alveolarisation and plexiform pulmonary arteriopathy. Treatment with oral sildenafil in addition to inhaled nitric oxide (NO) resulted in recovery from the pulmonary hypertensive crisis. Long term sildenafil therapy was associated with complete resolution of the pulmonary hypertension.

Usefulness of Blalock-Taussig shunt Doppler flow velocity profiles in the assessment of pulmonary artery pressure and flow.

AIM: To assess the utility of continuous wave Doppler evaluation of velocity profiles across a Blalock-Taussig (BT) shunt in the evaluation of pulmonary artery pressure and pulmonary blood flow. METHODS AND RESULTS: Eleven children with complex congenital heart disease with a BT shunt as the sole source of pulmonary blood supply were studied prospectively (median age 5 (0.3-21) months). Doppler evaluations of shunt flow velocity profile and cardiac catheterization were carried out simultaneously. Pulmonary artery pressure and flow were estimated using the modified Bernoulli equation and velocity time integral of shunt flow. There was a positive correlation between (1) the Doppler estimates for mean pulmonary artery pressure, using the diastolic flow velocity, and the mean pulmonary venous wedge pressure ( r = 0.93, SEE = 1.0 mmHG; P<0.001 ) and (2) the Doppler derived and calculated pulmonary blood flow ( r = 0.9, SEE = 0.19 l/min; P<0.001). In contrast, the Doppler estimates for mean pulmonary artery pressure using the peak or mean Doppler gradients were poor. CONCLUSION: The diastolic BT shunt flow velocity can be used reliably to predict mean pulmonary artery pressure when a BT shunt is the sole source of pulmonary artery flow. There was agreement between Doppler predicted pulmonary blood flow and catheter based calculations. These findings may prove a useful tool for perioperative management.

Modified technique of stent fenestration of the atrial septum.

OBJECTIVES: To develop a modified technique for stenting the atrial septum in the treatment of patients with a failing Fontan operation or pulmonary hypertension. SETTING: Two tertiary referral centres. STUDY DESIGN: Prospective collaborative clinical study. PATIENTS AND METHODS: A stent was mounted on a standard valvoplasty balloon catheter which was constricted to a predefined diameter by a loop created from a temporary pacing wire. Full balloon inflation created a diabolo shaped stent configuration. The technique was employed in 12 consecutive patients to relieve symptoms of a failing Fontan circulation (n = 6) or severe pulmonary hypertension (n = 6). RESULTS: Ex vivo studies confirmed that a diabolo shaped stent configuration could be achieved using the above technique. Transcatheter stent implantation was successful in all 12 patients. All six Fontan patients showed significant clinical improvement. Right atrial pressure decreased from (mean (SD)) 16.8 (2.5) to 13.7 (1.9) mm Hg (p < 0.05), and arterial oxygen saturation from 92.8 (1.8)% to 82.7 (3.8)% (p < 0.01). Six patients underwent successful stent fenestration for treatment of pulmonary hypertension. All stents were patent and stable at the most recent follow up (mean 1.75 (1.05) years). No early or late stent related complications were encountered. CONCLUSIONS: This new technique allows placement of a diabolo shaped stent with a predefined diameter across the atrial septum. The diabolo shape increases stent stability, facilitates recrossing of the stent during future catheter interventions, and ensures medium term stent patency.