Successful pregnancies in the presence of spinal muscular atrophy: two case reports

We report two cases of successful pregnancy in women with chronic, infantile onset, or type II spinal muscular atrophy, both of whom delivered healthy, unaffected babies. The patients required concurrent management by a physiatrist, pulmonologist, and perinatologist throughout the pregnancy. Complications included recurrent urinary tract infections, dyspnea and worsening of pulmonary function, wheelchair seating and positioning problems, and musculoskeletal and low back pain. These problems resolved postpartum. One woman had vaginal delivery, the other had caesarean section, both of which were well-tolerated. Because of severe musculoskeletal deformity, pelvic assessment is necessary to determine the mode of delivery. The uterus has normal contractility and effective labor patterns can be established. Spinal/epidural anesthesia may be contraindicated because of spine deformity. The pregnancies had no deleterious effect on the progression of the disease in our patients, both of whom reported a positive experience with great personal fulfillment.     

Hepatobiliary cystadenoma. A study of five cases with reference to histogenesis

Hepatobiliary cystadenoma is a rare hepatic lesion characterized by a multiloculated cyst lined by cuboidal or columnar epithelial cells. Four cases of hepatobiliary cystadenoma with mesenchymal stroma (HCMS) and one case of hepatobiliary cystadenoma with intracystic epithelial component were studied by light microscopy, immunohistochemical methods, and electron microscopy. Similar studies were conducted on six fetal gallbladder tissues, representing the biliary tree, and two adult ovarian tissues. By light microscopy, the columnar epithelium of the five cases of hepatobiliary cystadenoma was similar to the epithelium of the developing gallbladder. The spindle cell stroma of the HCMS and the subepithelial spindle cells of the developing gallbladders showed similar reactivity to smooth-muscle actin. Vimentin reactivity was strongly positive in the stroma of the HCMS, and in the fetal gallbladders it was only noted in the subepithelial spindle cells of the 15-week gestation fetal gallbladder tissues. By electron microscopy, the epithelium lining the hepatic lesions showed characteristic gastrointestinal features and was identical to the epithelia lining the embryonic gallbladders. Furthermore, the mesenchymal stroma of the HCMS recapitulated the features found in subepithelial tissues in developing gallbladders. Although the ovarian stroma resembled the stroma of the HCMS by light microscopy, the immunohistochemical reactions and the electron microscopic studies showed dissimilarities. This study supports the hypothesis that the hepatobiliary cystadenomas arise from ectopic embryonic tissues destined to form the adult gallbladder.     

ACAT inhibitors derived from hetero-Diels-Alder cycloadducts of thioaldehydes

Acyl-CoA:cholesterol acyltransferase (ACAT) is the enzyme largely responsible for intracellular cholesterol esterification. A systemic inhibitor of ACAT is believed to be able to slow or even reverse the atherosclerotic process. Towards that goal, a series of cyclic sulfides, derived from the hetero-Diels-Alder reaction of thioaldehydes with 1,3-dienes, and bearing carboxamide substituents, were prepared and evaluated for in vitro (in several tissues and species) and ex vivo ACAT inhibition. Minor changes in subsequent structure were found to have a significant effect in optimization of the biological activity of this series of compounds.     

Oxygenation in the rabbit myocardium: assessment with susceptibility-dependent MR imaging

PURPOSE: To determine the feasibility of using hemoglobin (Hb) desaturation as an indicator of myocardial oxygenation. MATERIALS AND METHODS: High-resolution gradient-echo nuclear magnetic resonance (MR) images of isolated, blood-perfused rabbit hearts were obtained at various blood oxygenation levels. The hearts were perfused at 37 degrees C with a Langendorff apparatus modified for nuclear MR imaging. The perfusate contained bovine red blood cells in a cardioplegic solution that eliminated motion artifacts and minimized arteriovenous oxygenation differences. Hb saturation was varied (7%-100%) randomly. Perfusion pressure was continuously monitored, and blood samples were obtained. RESULTS: There was a substantial correlation between image signal intensity in the myocardium and Hb saturation in the blood, believed to be due to susceptibility effects of the paramagnetic species deoxyhemoglobin. CONCLUSION: Direct and noninvasive determination of regional Hb saturation with susceptibility-dependent MR imaging may provide information regarding regional myocardial O2 content.     

Housing Tenure: Pacific Families in New Zealand

Home ownership has been associated with health, social and economic benefits. However, a decline in ownership has been observed over the past decade in New Zealand. Minority groups, including Pacific people, have been disadvantaged in the housing sector. This study investigated housing tenure and the relationship between tenure and health among mothers of a birth cohort of Pacific children in New Zealand. Findings showed that most families lived in state or private rental accommodation with few (15.4 per cent) owning their own homes. Homeowners were more likely to be older, partnered and have higher incomes. Better mental health was observed for homeowners compared to renters. Findings can inform housing and public health policy for Pacific families.     

A Composite Index of Compliance for Chronic In‐Center Hemodialysis Patients

We developed a composite compliance index as the sum of the compliance scores for interdialytic weight gain (IDWG), pre‐dialysis serum potassium and phosphorus concentrations (each scored from zero to 3, with 3 indicating the poorest compliance), and skipping hemodialysis sessions (scored from zero to 9, with 9 indicating the poorest compliance). We used this composite score to prospectively evaluate compliance in 25 prevalent hemodialysis patients over a period of 1 year. We then followed these patients for another 3.5 years. The patients studied were divided into two groups: group A (poor compliance) consisted of 9 subjects with composite score ≥ 9 (13.2 ± 3.2); group B (better compliance) consisted of 16 subjects with composite score < 9 (4.7 ± 1.8). Age, duration of hemodialysis, and frequency of diabetes mellitus did not differ between the groups. Group A contained higher fractions of subjects with history of alcoholism (66.7% vs 12.5%, p = 0.010), other substance addiction (44.4% vs 0%, p = 0.010), and severe psychosocial problems (88.9% vs 18.8%, p = 0.002). Mean survival from the beginning of observation, estimated by actuarial life‐table survival analysis, was 1.19 years in group A and 2.60 years in group B (p = 0.0265). A composite compliance index incorporating domains indicating adherence to diet, medications, and dialysis schedule identified other behavioral problems in poorly compliant patients. Hemodialysis patients characterized by this composite index as poorly compliant had shortened survival.     

Describing metal surfaces and nanostructures with orbital-free density functional theory

Orbital-free density functional theory (OF-DFT) can be made to scale linearly with sample size, allowing thousands of atoms to be treated explicitly with quantum mechanics. State-of-the-art kinetic energy density functionals and ion–electron pseudopotentials are used to obtain accurate structural property predictions for nanoparticles, nanowires, extended surfaces, and nanoindentation of simple metals.