Congenital chylothorax: from foetal life to adolescence

Aim: To analyse the main prenatal and postnatal features of congenital chylothorax (CC), and the outcome including mid‐term follow‐up. Methods: We searched our databases for CC diagnosed between 1990 and 2006. Data of 29 cases were retrieved and analysed. Follow‐up until 3 years of age was available for all patients. Results: Most patients were diagnosed prenatally (94%) and most cases were complicated by foetal hydrops (66.7%). The overall survival rate at 3 years was 56%. A significantly poorer outcome was observed when foetal hydrops, preterm birth < 34 weeks, large effusions and/or early‐onset pneumothorax were present. An important but not significant improvement in the survival rate was observed through the study period; while in 1990–1998, the survival rate was 41.7%, from 1999 to 2006 it was 66.7% (p = 0.19). In the mid‐term follow‐up, we did not observe any recurrence of CC and most infants remain asymptomatic. However, 27% of survivors were diagnosed as having asthma in early infancy. Conclusion: CC still carries a significant risk of perinatal mortality. However, continuous advances in foetal and neonatal medicine are improving the prognosis of these patients, and nowadays most of them are likely to survive. Beyond the neonatal period, most survivors have an uneventful outcome.     

The role of free radicals in endogenous adaptation and intracellular signals

Oxidative stress can generate a mass of oxygen free radicals (OFR) in the cells, and these OFRs can induce several acute and chronic symptoms and diseases. If the amount of the generated OFRs overwhelms the antioxidant capacity of the cells, the pathophysiological changes may lead to the death of the cell or the development of chronic degenerative diseases.     

The final destiny of acantholytic cells in pemphigus is Fas mediated

Background  Pemphigus is an autoimmune disease characterized by the formation of intra-epidermal blisters. Patients develop auto-antibodies against desmoglein 1 and 3 proteins and induce acantholysis.
Objective  This work addresses the issue of whether the Fas pathway mediates acantholysis. Furthermore, the possible suppliers of the Fas pathway were investigated.
Methods  Seventeen biopsies of pemphigus patients were studied by haematoxylin and eosin staining, and apoptosis was defined by TUNEL. The expression of Fas, FasL and caspase 3 was studied by in situ hybridization and immunohistochemistry. Cell infiltrates were studied by immunofluorescence with monoclonal anti-CD3, CD4, CD8, CD19 and CD69.
Results  All of the biopsies showed intra-epidermal blisters, acantholytic cells and inflammatory infiltrates. The blisters expressed Fas, FasL and caspase 3. Cell infiltrates were composed of CD8 and a few CD4⁺CD69⁺ cells. Additionally, CD19⁺ cells were detected. Interestingly, the Fas expression was increased in acantholytic cells and perilesional keratinocytes. Incidentally, these cells exhibited apoptotic features. Interestingly, the CD8 cells expressed FasL.
Conclusion  This paper presents the morphological evidence that apoptosis and acantholysis are linked. Therefore, the Fas pathway is associated with CD8 cells in pemphigus lesions.

Conflicts of interest

None declared.     

Central and peripheral residual vision in humans with bilateral deprivation amblyopia

Summary Residual visual capacities were investigated in the central and peripheral visual fields of 13 patients with bilateral deprivation amblyopia secondary to congenital cataracts removed at an early age. In comparison with a control group of normal subjects, spatial modulation sensitivity function of the amblyopes was markedly impaired in each experimental condition, i.e. both when the stimuli were stationary or drifting (8 Hz) gratings and both in central or peripheral visual field, at 10 and 20° eccentricity. The sensitivity drop was observed over the whole spatial frequency range, although it was much more severe at high frequencies. Threshold elevation, with respect to controls, was very similar in conditions using stationary or drifting gratings, suggesting that both sustained and transient mechanisms are affected by stimulus deprivation amblyopia. Temporal modulation sensitivity function was uniformly impaired over the whole temporal frequency range. When compared with other types of amblyopia, this pattern of spatiotemporal sensitivity loss appeared characteristic of deprivation amblyopia. The peripheral deficit was particularly striking by its severity and extent, as ascertained by static perimetry and visual acuity measurements up to 50° eccentricity. This finding emphasizes the susceptibility of peripheral as well as central human vision to early deprivation and suggests that peripheral visual functions are still immature in newborns. Finally, the overall deficit varied with the severity of the deprivation, in that patients with complete neonatal cataract performed much worse than those with incomplete cataract.     

Factors Affecting the Risk of Blood Bank Specimen Hemolysis

OBJECTIVES: To evaluate simultaneously several possible risk factors for blood bank specimen hemolysis. METHODS: This was a prospective cohort study of emergency department and labor and delivery patients to estimate the effect of various factors on the risk of blood bank specimen hemolysis. Study variables included patient demographics, type and gauge of needle or catheter, anatomic location of venipuncture, and patient care area. Hemolysis was determined by blood bank laboratory technicians. Cox proportional hazards multivariate regression modeling was performed to estimate the adjusted relative risks for hemolysis. RESULTS: Of the 605 subjects with complete data, 194 (32.1%) subjects had blood specimens drawn directly with a steel needle, and 411 (69.1%) had specimens drawn through a Vialon (BD Medical Systems, Inc., Sandy, UT) intravenous (IV) angiocatheter. The overall risk of hemolysis for all was 7%, 10% for Vialon IV angiocatheters and 1.5% for steel needles. In the multivariate analysis, the factors most closely associated with hemolysis were the use of Vialon IV catheters and sampling from an anatomic site other than the antecubital area. CONCLUSIONS: Blood bank specimens drawn from Vialon IV catheters (particularly smaller gauge catheters) and from veins outside the antecubital area are at significantly increased risk to hemolyze.