Homozygous cystinuria and the oculo-cerebro-renal dystrophy of Lowe in same family

The mother and daughter in a family had homozygous cystinuria and were also heterozygous carriers of the oculo-cerebro-renal dystrophy of Lowe. The daughter was also epileptic. The son had Lowe's syndrome and the father an increased urinary excretion of cystine and lysine. This evidence together with other case reports suggests that the defect in cystinuria and that of Lowe's syndrome may be connected.     

Post-traumatic abnormalities of the pubic bone simulating malignancy

Three cases are presented in which unusual healing patterns of pubic features simulated malignant processes. Consideration of trauma as an etiologic factor in such cases may obviate the need for extensive clinical and histologic evaluation.     

PATENT FORAMEN OVALE IN ELDERLY STROKE PATIENTS

The aim of this study was to determine the incidence of echocardiographically detectable patent foramen ovale (PFO) in elderly patients who have strokes from cerebral infarction, as well as to assess the association between PFOs and other risk factors for stroke disease. Forty-three patients aged ≥65 years admitted consecutively with cerebral infarction shown on computerised tomography of the brain were assessed using transoesophageal echocardiography. A PFO was present in 8 (19%) of the 43 patients. Four patients (50%) in this PFO group had stroke risk factors. Two were in atrial fibrillation and, of the six patients in sinus rhythm, a risk factor was present in two, both of whom had diabetes mellitus. A PFO was not detected in 35 patients. Twenty-nine (83%) of the patients in this group had risk factors, with 18 having two or more. Seven patients were in atrial fibrillation. Of the 28 patients in sinus rhythm, risk factors were present in 22 (78%).     

Plasmacytoid myoepithelioma of minor salivary glands: report of case with emphasis in the immunohistochemical findings

Extracorporeal formation of mineralized bone-like tissue is still an unsolved challenge in tissue engineering. Embryonic stem cells may open up new therapeutic options for the future and should be an interesting model for the analysis of fetal organogenesis. Here we describe a technique for culturing embryonic stem cells (ESCs) in the absence of artificial scaffolds which generated mineralized miromasses. Embryonic stem cells were harvested and osteogenic differentiation was stimulated by the addition of dexamethasone, ascorbic acid, and ß-glycerolphosphate (DAG). After three days of cultivation microspheres were formed. These spherical three-dimensional cell units showed a peripheral zone consisting of densely packed cell layers surrounded by minerals that were embedded in the extracellular matrix. Alizarine red staining confirmed evidence of mineralization after 10 days of DAG stimulation in the stimulated but not in the control group. Transmission electron microscopy demonstrated scorching crystallites and collagenous fibrils as early indication of bone formation. These extracellular structures resembled hydroxyl apatite-like crystals as demonstrated by distinct diffraction patterns using electron diffraction analysis. The micromass culture technique is an appropriate model to form three-dimensional bone-like micro-units without the need for an underlying scaffold. Further studies will have to show whether the technique is applicable also to pluripotent stem cells of different origin.     

Platelets interact with fibrin only after activation

Interactions between platelets and fibrin have been visualized by phase contrast, epifluorescence, and scanning electron microscope examination of clots formed with dansylcadaverine-labeled fibrin and gel-filtered platelets. After thrombin activation, the platelets appeared as fluorescent aggregates with bridging strands of fibrin; formaldehyde- fixed platelets were not fluorescent under the same experimental conditions. Scanning electron micrographs demonstrated that thrombin- activated cells had numerous pseudopods to which the fibrin strands adhered; fixed platelets exhibited a smooth discoid appearance and did not interact with the clot. Platelets trapped in clots formed with Batroxobin (Pentapharm) (platelets are not activated by Batroxobin as confirmed by light-scattering aggregometry measurements) remained as nonfluorescent, discoid cells, whereas platelets first activated by adenosine diphosphate formed brightly fluorescent aggregates. Light- scattering data of thrombin activation (0.2 U/mL) indicated that preincubation of platelets with 0.1 mmol/L prostaglandin E1 (PGE1) prior to addition of thrombin decreased the extent and rate of platelet shape change and resulted in 100-fold slower aggregation. Clots formed in the presence of PGE1 revealed decreased fluorescence intensity and fewer platelet-fibrin contacts. Gly-Pro-Arg-Pro, which blocks fibrinogen binding and fibrin assembly, was also effective in blocking platelet-fibrin interactions. These results indicate that platelet activation is a prerequisite for attachment of platelets to fibrin.     

Validation of a model to predict all-cause in-hospital mortality in vascular surgical patients

Objective

To develop and validate a pre- and postoperative model of all-cause in-hospital mortality in South African vascular surgical patients.

Methods

We carried out a retrospective cohort study. A multivariate analysis using binary logistic regression was conducted on a derivation cohort using clinical, physiological and surgical data. Interaction and colinearity between covariates were investigated. The models were validated using the Homer-Lemeshow goodness-of-fit test.

Results

Independent predictors of in-hospital mortality in the pre-operative model were: (1) age (per one-year increase) [odds ratio (OR) 1.03, 95% confidence interval (CI) 1.0–1.06), (2) creatinine > 180 μmol.l^-1 (OR 6.43, 95% CI: 3.482–11.86), (3) chronic beta-blocker therapy (OR 2.48, 95% CI: 1.38–4.48), and (4) absence of chronic statin therapy (OR 2.81, 95% CI: 1.15–6.83). Independent predictors of mortality in the postoperative model were: (1) age (per oneyear increase) (OR 1.05, 95% CI: 1.02–1.09), (2) creatinine > 180 μmol.l^-1 (OR 5.08, 95% CI: 2.50–10.31), (3) surgery out of hours without statin therapy (OR 8.27, 95% CI: 3.36–20.38), (4) mean daily postoperative heart rate (HR) (OR 1.02, 95% CI: 1.0–1.04), (5) mean daily postoperative HR in the presence of a mean daily systolic blood pressure of less than 100 beats per minute or above 179 mmHg (OR 1.02, 95% CI: 1.01–1.03) and (6) mean daily postoperative HR associated with withdrawal of chronic beta-blockade (OR 1.02, 95% CI: 1.01–1.03). Both models were validated.

Conclusion

The pre-operative model may predict the risk of in-hospital mortality associated with vascular surgery. The postoperative model may identify patients whose risk increases as a result of surgical or physiological factors.