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Ebstein anomaly is the most common form of tricuspid valve congenital anomalies. The tricuspid valve is abnormal with different degrees of displacement of the septal leaflet and abnormal rotation of the valve towards the right ventricular outflow tract. In severe forms, it results in significant tricuspid regurgitation and requires surgical repair. There is an increased interest in understanding the anatomy of the tricuspid valve in this lesion as the surgical repair has evolved with the invention and wide adoption of the cone operation. Multimodality imaging plays an important role in diagnosis, follow-up, surgical planning and post-operative care. This review provides anatomical tips for the cardiac imagers caring for patients with Ebstein anomaly and will help provide image-based personalized medicine.

Microbial population dynamics associated with corn silage, with and without Lactobacillus plantarum treatment, was studied. Whole crop corn was ensiled using laboratory silos and sampled at different times, up to 3 months. The dominant bacteria, before ensiling, were Acinetobacter (38.5%) and Klebsiella (16.3%), while the dominant fungi were Meyerozyma (53.5%) and Candida (27.7%). During ensiling, the microbial population shifted considerably, and Lactobacillus (> 94%) and Candida (> 74%) became the most dominant microbial genera in both treated and untreated silages. Yet, lactic acid content was higher in the treated silage, while the microbial diversity was lower than in the untreated silage. Upon aerobic exposure, spoilage occurred more rapidly in the treated silage, possibly due to the higher abundance of lactic acid-assimilating fungi, such as Candida. Our study is the first to describe microbial population dynamics during whole-crop corn ensiling and the results indicate that microbial diversity may be an indicator of aerobic stability.

Electron transfer from H2 to Th79+ was studied in a cryogenic Penning trap by non-destructively observing the sequential development with time of the charge state of one or a few stored particles. The ratio of the true double capture cross section to the total capture cross section was found to be 0.21 ± 0.11 at a mean center-of-mass energy near 6 eV.

Spontaneous remission of congenital nephrotic syndrome is rare. We present a 5-week-old infant who developed proteinuria, hypoalbuminemia, and edema which spontaneously resolved with supportive care. Clinical and histopathological features of this patient and six previous reported remissions of congenital nephrotic syndrome are discussed.

Background Once-daily (QD) ritonavir 100 mg-boosted fosamprenavir 1400 mg (FPV/r100) or atazanavir 300 mg (ATV/r100), plus tenofovir/emtricitabine (TDF/FTC) 300 mg/200 mg, have not been compared as initial antiretroviral treatment. To address this data gap, we conducted an open-label, multicenter 48-week study (ALERT) in 106 antiretroviral-naïve, HIV-infected patients (median HIV-1 RNA 4.9 log10 copies/mL; CD4+ count 191 cells/mm3) randomly assigned to the FPV/r100 or ATV/r100 regimens. Results At baseline, the FPV/r100 or ATV/r100 arms were well-matched for HIV-1 RNA (median, 4.9 log10 copies/mL [both]), CD4+ count (mean, 176 vs 205 cells/mm3). At week 48, intent-to-treat: missing/discontinuation = failure analysis showed similar responses to FPV/r100 and ATV/r100 (HIV-1 RNA < 50 copies/mL: 75% (40/53) vs 83% (44/53), p = 0.34 [Cochran-Mantel-Haenszel test]); mean CD4+ count change-from-baseline: +170 vs +183 cells/mm3, p = 0.398 [Wilcoxon rank sum test]). Fasting total/LDL/HDL-cholesterol changes-from-baseline were also similar, although week 48 median fasting triglycerides were higher with FPV/r100 (150 vs 131 mg/dL). FPV/r100-treated patients experienced fewer treatment-related grade 2–4 adverse events (15% vs 57%), with differences driven by ATV-related hyperbilirubinemia. Three patients discontinued TDF/FTC because their GFR decreased to Conclusion The all-QD regimens of FPV/r100 and ATV/r100, plus TDF/FTC, provided similar virologic, CD4+ response, and fasting total/LDL/HDL-cholesterol changes through 48 weeks. Fewer FPV/r100-treated patients experienced treatment-related grade 2–4 adverse events.

PLATELET ACTIVATING FACTOR (PAF) AND PAF-ACETYLHYDROLASE (PAF-AH) ACTIVITY IN NECROTIZING ENTEROCOLITIS (NEC). † 1200

A family is described showing deafness in three consecutive generations. Hirshchsprung's disease was present in at least two of the affected patients and a history of bowel dysfunction was present in the third. The assocation of the two disorders in this family may be due to a single autosomal dominant gene and in this regard differs from previously reported isolated patients with Hirschsprung's disease and deafness.

A malignant mesenchymoma exhibiting a varied spectrum of differentiation developed within a congenital pulmonary cyst 6 1/2 years after the cyst was first recognized. Related tumors with a similar gross appearance have been previously described and have included rhabdomyosarcomas and so-called pulmonary blastomas. There is a low but distinct risk for the development of mesenchymal sarcomas within congenital peripheral pulmonary cysts.

A fetus with a 46,XX,r(17) karyotype identified initially by amniocentesis and confirmed by banding studies is described. No significant phenotypic abnormalities were identified despite isolation of the ring chromosome from multiple fetal tissues. This paucity of abnormal features of gross development is consistent with findings in 3 previously reported patients with ring 17 chromosomes.

Severe pseudomembranous colitis was observed in 3 members of a single family: the father and 2 of his children. 2 of the affected family members received the antibiotic lincomycin prior to the onset of their symptoms. The findings in this family raise the possibility that antibiotic-induced pseudomembranous colitis in some patients may be the result of a pharmacogenetic interaction.

Zirconium containing coatings deposited by reactive sputtering on molybdenum surfaces were analysed by means of X-ray photoemission spectroscopy (ESCA) and electron-induced X-ray emission (EDAX), with an emphasis on zirconium carbide creation. For comparison ZrC layers deposited by cataphoresis were investigated. The results show that while EDAX is useful for bulk analysis of heavy atom contaminants, chemical state information vital for the reactive deposition processes is only obtained through ESCA. The spectra indicate that reactively sputtered zirconium leads to appreciable amounts of ZrC in the films only under special reaction conditions; if these are not present, unreacted Zr coatings are found, which transform to ZrO2 upon exposure to air. By monitoring the chemically shifted Zr 3d peaks the reaction conditions in the sputtering process which are favourable for ZrC deposition were determined.

The prognosis of polyarteritis in children is poor, particularly if complicated by early renal failure. Three girls, 9 to 10 years of age, have been treated with a combination of corticosteroids and cyclophosphamide. A complete remission of the disease ensued in all three cases. An early diagnosis has been made by biopsies of the kidney or skin. All three patients initially were severely ill presenting with high fever, skin and joint manifestations, hypertension, and seizures. Additional complications included renal failure in two girls, primary pulmonary infiltrates in one, hallucinations in one. All three patients were started on Prednisone treatment (1.5-2 mg/kg) and after two to four weeks cyclophosphamide treatment (2 mg/kg) was added. After an initially stormy course all clinical symptoms gradually subsided and the renal function improved. Cyclophosphamide treatment was continued for up to 12 months. All three patients are now off medication for periods of 15 to 24 months with no or minimal residual symptoms. The creatinine clearance has returned to normal in two girls, proteinuria is still present in two patients. The additional administration of cyclophosphamide seems to effectively influence the disease process of polyarteritis leading to a resolution of infiltrates and necrosis which have not been accessible to therapy previously.