Extracorporeal membrane oxygenation as a bridge to cardiac transplantation in a patient with cardiomyopathy and hemophilia A

OBJECTIVE: To report the use of extracorporeal membrane oxygenation (ECMO) as a bridge to cardiac transplantation in a patient with hemophilia A and dilated cardiomyopathy. DESIGN: Case report. INTERVENTIONS AND RESULTS: During ECMO factor VIII concentrate was administered to maintain a factor VIII level close to 50% of normal control both to decrease the risk of bleeding and to allow standard anticoagulation with heparin. The patient developed an inhibitor to exogenous factor VIII during hospitalization, which was successfully managed with a high-dose continuous infusion of factor VIII during ECMO, the transplant operation, and the postoperative period. CONCLUSIONS: We report the successful use of ECMO as a bridge to cardiac transplantation in a patient with hemophilia A and low-level factor VIII inhibitors.     

Sociodemographic and health characteristics of older Chinese on admission to a nursing home: a cross-racial/ethnic study

OBJECTIVES: To investigate sociodemographic characteristics (SDCs) and health status of older Chinese newly admitted to a nursing home (NH) and to compare them with the characteristics of residents from other racial/ethnic groups. DESIGN: Secondary analysis of the admission Minimum Data Set Plus (MDS+). SETTING: A New York City municipal NH near Chinatown. PARTICIPANTS: Two hundred fifty-eight (125 Chinese, 57 white, 53 Hispanic, and 23 black) of 292 residents consecutively admitted from November 1992 to May 1997 were selected after excluding those younger than 60 or transferred from another NH. MEASUREMENTS: SDCs, health status parameters (cognitive performance, physical functioning, mood/behavior patterns, and psychosocial well-being), and morbidity information (most-frequent diagnoses/conditions and medication use) documented in or generated from the MDS+. RESULTS: The majority of these Chinese were first-generation immigrants and spoke primarily Cantonese or Mandarin Chinese. Compared with whites, they were more likely to be married, less likely to have lived alone, more likely to be using Medicaid, less likely to make medical decision alone, and more likely to depend on family members for decision-making. Nearly three-quarters of Chinese had cognitive impairment. There was an underdiagnosis of dementia in the Chinese subjects on admission. Severe dependence in activity of daily living was identified in more than one-third of Chinese. Fewer Chinese were using psychotropic medications on admission than the whites. Similar to other groups, many of the Chinese subjects were incontinent of bowel and bladder and had chewing or swallowing problems, hypertension, anemia, and stroke. CONCLUSION: This is the first systematic report of the SDCs and health status of a group of newly admitted older Chinese to an urban NH in the United States using the Minimum Data Set database. These findings suggest that Chinese residents are as frail as other racial/ethnic residents on admission. NHs caring for older Chinese need to be sensitive to the presence of dementia, and require a staff that can speak Cantonese and Mandarin Chinese and is comfortable negotiating with families who are more likely to be the designated decision makers.     

Semaphorin-3F is an inhibitor of tumor angiogenesis

The neuropilin-1 (np1) and neuropilin-2 (np2) receptors form complexes with type-A plexins. These complexes serve as signaling receptors for specific class-3 semaphorins. Np1 and np2 function in addition as receptors for heparin-binding forms of vascular endothelial growth factor (VEGF), such as VEGF(165). Human umbilical vein endothelial cells (HUVEC) express tyrosine-kinase receptors for VEGF and basic fibroblast growth factor (bFGF), as well as np1, np2, and several type-A plexins. We have found that semaphorin-3F (s3f), a semaphorin which signals through the np2 receptor, was able to inhibit VEGF(165), as well as bFGF-induced proliferation of HUVECs. Furthermore, s3f inhibited VEGF as well as bFGF-induced phosphorylation of extracellular signal-regulated kinase-1/2. Our experiments indicate that bFGF does not bind to neuropilins, nor does s3f inhibit the binding of bFGF to FGF receptors. It is therefore possible that s3f inhibits the activity of bFGF by a mechanism that requires active s3f signal transduction rather than by inhibition of bFGF binding to FGF receptors. s3f also inhibited VEGF(165), as well as bFGF-induced in vivo angiogenesis as determined by the alginate micro-encapsulation and Matrigel plug assays. Overexpression of s3f in tumorigenic human HEK293 cells inhibited their tumor-forming ability but not their proliferation in cell culture. The tumors that did develop from s3f-expressing HEK293 cells developed at a much slower rate and had a significantly lower concentration of tumor-associated blood vessels, indicating that s3f is an inhibitor of tumor angiogenesis.     

Deciding on surgery: supporting parents of infants with craniosynostosis

Families face a difficult decision when choosing whether or not to have surgery for their infant with isolated craniosynostosis. While the skull deformity may not affect the child's physical health, growth, or development, it may have psychosocial impacts on a child's social-emotional development, self-esteem, and interpersonal interactions. Parents are challenged to balance surgical risks with potentially positive effects on their child's psychosocial health. The purpose of this research study was to explore parental decision-making related to surgery for isolated craniosynostosis, and to identify strategies that facilitate their decision-making. A thematic content analysis of focus group interview data revealed four themes that encapsulated the process of parental decision-making. The decision for some parents was agonizing. However, others found the decision relatively straightforward. In all cases, parents spent time thinking and gathering information. Certain critical events helped parents make their decision. Parents identified a number of strategies that would be helpful to the decision-making process.     

Activation of epidermal growth factor receptor signals induction of nitric oxide synthase-2 in human optic nerve head astrocytes in glaucomatous optic neuropathy

Glaucoma is an optic neuropathy that is associated with elevated intraocular pressure in most patients. We have previously demonstrated that the mechanism by which pressure damages optic nerve axons involves excessive nitric oxide generated by inducible nitric oxide synthase (NOS-2). We have now found that activation of the epidermal growth factor receptor (EGFR) induces NOS-2 in astrocytes of the human optic nerve head (ONH) in vitro and EGFR is significantly upregulated and tyrosine phosphorylated in reactive astrocytes in human glaucomatous ONHs in vivo. Furthermore, in response to elevated hydrostatic pressure, EGFR rapidly becomes phosphorylated in the nucleus. This pressure-dependent activation of EGFR is necessary for NOS-2 induction. Our results suggest that activation and nuclear localization of EGFR may be needed for induction of NOS-2 in response to elevated intraocular pressure in glaucomatous optic neuropathy. Identification of this key signaling pathway provides new therapeutic approaches to pharmacological neuroprotection for glaucoma.     

Treatment of periodontitis associated with cyclosporin-induced severe gingival hyperplasia with regular mechanical plaque removal in a patient with renal transplantation (Case report)

A clinical case of a middle-aged kidney transplanted woman is presented. The woman has been taking Cyclosporin-A for over 13 years and has had advanced periodontitis and severe gingival swellings and gingival inflammation. The kidney transplant patient was treated and followed up for approx. four years. The treatment protocol included very thorough mechanical scaling and root planing, oral hygienic instructions and the regular professional maintenance program resulted in complete remission of the gingival overgrowth and stabilization of the periodontal condition. The gingival and periodontal conditions showed a continuing improvement over the time despite of the continuous CSA administration.     

Effects of diets containing olive oil, sunflower oil, or rapeseed oil on the hemostatic system

Various studies have already shown that the fatty acid composition of dietary fat has different effects on hemostasis and platelet function. However, knowledge on this topic is incomplete. In the present study, fifty-eight healthy students received either a 4-week rapeseed oil [high content of monounsaturated fatty acids (MUFA) and high n-3/n-6 PUFA ratio], an olive oil (high content of MUFA, low n-3/n-6 PUFA ratio) or a sunflower oil (low content of MUFA, low n-3/n-6 PUFA ratio) diet. In each group, effects on hemostatic parameters were compared with a wash-in diet rich in saturated fatty acids with respect to intermediate-time effects on the hemostatic system and platelet function. With the olive oil diet, a reduction of coagulation factors VIIc, XIIc, XIIa, and Xc was found, whereas sunflower oil led to lower values of coagulation factors XIIc, XIIa, and IXc. In all study groups levels of plasmin-alpha2-antiplasmin were lower in week 4 than at baseline. Lower fibrinogen binding on platelets was found after the sunflower oil diet, whereas expression of CD62 and spontaneous platelet aggregation were slightly higher after the olive oil diet. However, given the major differences in the fatty acid compositions of the diets, the differences between the groups with respect to hemostasis tended to be small. Therefore, the clinical significance of the present findings remains to be evaluated.     

Attenuated plasticity in neurons and astrocytes in the mouse model of Sanfilippo syndrome type B

Sanfilippo syndrome type B (MPS III B) is a neurodegenerative disorder characterized by profound mental retardation and early death. It is caused by deficiency of a lysosomal enzyme involved in heparan sulfate (HS) degradation. Because HS accumulation can be a major feature of this disease, we have examined crucial molecular systems associated with HS function. Using a knockout mouse with disruption of the gene responsible for HS degradation, we evaluated the effects of possible HS accumulation on neuroplasticity that are within the spectrum of action of fibroblast growth factors (FGFs) and their receptor (FGFR). We found that levels of mRNA for the FGFR-1 were attenuated in the mutant mice by the age of 6 months, whereas the mRNAs for FGF-1 and FGF-2 were reduced or unchanged in the brain regions tested. Neurogenesis, in which FGF-2 is involved, was inhibited in the MPS III B mouse brain at both young and adult ages. We also examined the expression of the glial fibrillary acidic protein (GFAP) gene and GFAP-positive cell density in both normal and injured conditions to study the functional response of astrocytes to insult. We found that, although the mutation alone caused drastic induction of reactive astrocytes, acute injury to the mutant brains failed to induce additional reactive astrocytes. Our results showed important alterations in the expression of several genes involved in the maintenance of neuroplasticity in the MPS III B. This in turn may result in reduction of neuronal health and brain function.     

Does complete revascularization by the conventional method truly provide the best possible results? Analysis of results and comparison with revascularization of infarct-prone segments (systematic segmental myocardial revascularization): the Sheba Study

Myocardial revascularization is usually considered "complete" if all stenosed major coronaries are bypassed. Attempts were made to compare the results of this method with an approach by which each of the following five left ventricular infarct-prone segments is revascularized if ischemic: anteroseptal, anterolateral, posterosuperior, posteroinferior, and diaphragmatic. Two subsets of patients were studied. A total of 366 patients (Group A) who underwent aortacoronary bypass operations from 1980 to 1982 were followed up for a mean of 16.3 (6 to 43) months and were retrospectively divided into two groups: Group A1 (120 patients) had incomplete segmental revascularization (mean of 3.4 grafts per patient) and Group A2 (246 patients) had complete segmental revascularization (4.0 grafts per patient) (p less than 0.0001). Groups A1 and A2 were identical in all clinical and angiographic parameters: unstable angina, 60%; previous myocardial infarction, 70%; left main stenosis, 10%; and ejection fraction less than 30%, 2%. Overall operative mortality was 2.3%. Results in Groups A1 and A2, respectively, were as follows: operative mortality, 5.8% versus 0.8% (p less than 0.005); perioperative myocardial infarction, 6.9% versus 0.8% (p less than 0.0005); 35 month survival rate, 93.3% versus 97.9% (p less than 0.02); total freedom from symptoms, 54.1% versus 68.3% (p less than 0.025). In addition, 151 patients operated on in 1984 (Group B) were studied prospectively with regard to operative mortality and perioperative myocardial infarction, and the results were identical to those in Group A. Compared to conventional complete revascularization, complete segmental revascularization provides better results.