The preparation of fibrinogen concentrate for use as fibrin glue by four different methods

Fibrinogen concentrates for use as fibrin glue were prepared by modification of a cryoprecipitate method. The goals were the optimization of a method for different centrifuges and anticoagulants and the assay of factors not previously analyzed. Following a -70 degrees C freeze and a 4 degrees C thaw, CPDA-1 and ACD plasma were centrifuged at 6500 × g for 5 minutes or, alternatively, at 5000 × g for 7 minutes. The supernatant plasma was expressed to a final volume of 15.5 +/− 3 mL, and concentrates were stored at -30 degrees C. Preconcentration and postconcentration samples were analyzed for fibrinogen, fibronectin, factor XIII, and plasminogen content. Fibrinogen in CPDA-1 plasma was significantly higher than that in ACD plasma both before and after concentration at both centrifugation speeds. Fibronectin, factor XIII, and plasminogen concentrations were not significantly affected by centrifugation speed or the type of anticoagulant used. Fibronectin and plasminogen concentrations were significantly increased in components that were held for 5 to 6 days, as compared to those held for 0 to 1 day before freezing. Storage for up to 20 days in CPDA-1 and up to 5 months in ACD did not affect analyte concentration. It is concluded that ACD plasma centrifuged at 5000 × g yields a significantly low concentration of fibrinogen, while CPDA-1 plasma centrifuged at 6500 × g yields the highest amount. Acceptable yields were obtained from centrifugation of ACD plasma at 6500 × g and of CPDA-1 plasma at 5000 × g for use as fibrin glue.     

Respiratory disease in Niemann‐Pick type C2 is caused by pulmonary alveolar proteinosis

Griese M, Brasch F, Aldana VR, Cabrera MM, Goelnitz U, Ikonen E, Karam BJ, Liebisch G, Linder MD, Lohse P, Meyer W, Schmitz G, Pamir A, Ripper J, Rolfs A, Schams A, Lezana FJ. Respiratory disease in Niemann‐Pick type C2 is caused by pulmonary alveolar proteinosis. Niemann‐Pick diseases are hereditary neurovisceral lysosomal lipid storage disorders, of which the rare type C2 almost uniformly presents with respiratory distress in early infancy. In the patient presented here, the NPC2 exon 4 frameshift mutation c.408_409delAA caused reduced NPC2 protein levels in serum and lung lavage fluid and the synthesis of an aberrant, larger sized protein of around 28 kDa. Protein expression was strongly reduced also in alveolar macrophages. The infant developed failure to thrive and tachypnea. Lung lavage, computer tomography, and histology showed typical signs of pulmonary alveolar proteinosis with an abnormal intraalveolar accumulation of surfactant as well as macrophages. An NPC2‐hypomorph animal model also showed pulmonary alveolar proteinosis and accumulation of macrophages in the lung, liver, and spleen long before the mice died. Due to the elevation of cholesterol, the surfactant had an abnormal composition and function. Despite the removal of large amounts of surfactant from the lungs by therapeutic lung lavages, this treatment was only temporarily successful and the infant died of respiratory failure. Our data indicate that respiratory distress in NPC2 disease is associated with a loss of normal NPC2 protein expression in alveolar macrophages and the accumulation of functionally inactive surfactant rich in cholesterol.     

Complete atrioventricular septal defect repair: Simplified single patch technique

Background: There has been a rekindling of interest in alternatives to conventional two patch technique for the repair of complete atrioventricular septal defect in infancy in the recent past. We applied the simplified single patch technique to 15 consecutive infants and herein report our intermediate term results. Methods: Between March 1998 and September 2001, fifteen patients underwent repair of complete atrioventricular septal defect with this technique (mean age 6 months, mean weight 5.4 kg). Downs syndrome was present in 11 patients. Repair was done in all patients by direct suturing of the common atrioventricular valve leaflets to the crest of the ventricular septum irrespective of the size of the ventricular septal component. The cleft in the anterior mitral leaflet was closed in all patients. The atrial septal component was closed by a pericardial patch. Results: There was no mortality. There were no pulmonary arterial hypertensive crises or heart block. The mean follow up was 13.2 months. One patient underwent mitral valve replacement after one year due to severe mitral regurgitation. The remaining fourteen patients had no significant mitral regurgitation, residual ventricular septal defect or left ventricular outflow tract obstruction on echocardiography. Conclusion: Simplified single patch technique is an easily reproducible method for surgical repair of complete atrioventricular septal defect. It is less time consuming and minimises ischaemic time. Atrioventricular valve function is preserved and there is no incidence of obstruction to left ventricular outflow tract. The intermediate term results are encouraging. Presented in the poster session of the 37^th Annual Meeting of Association for European Paediatric Cardiology (AEPC) at Porto, Portugal, May 2002     

HLA antigens in South India: I. Major groups of Tamil Nadu

HLA-A, B profile of 385 normal healthy individuals living in Tamil Nadu, India was studied by microlymphocytotoxicity testing. Antigen, gene and haplotype frequencies of this population were calculated and compared to those already available in the literature. The sample was further divided into four major groups and the frequencies calculated. Genetic distances between the various major groups were also calculated: the analyses suggest that these different groups may differ by origin. The study further reveals that in any HLA genetic and disease association studies in India, one should give due consideration to the caste system of the sample studied.     

RANDOMISED,CONTROLLED, BLINDED FIELD TRIAL ON THE EFFICACY OF BIOCIDE FORMULATION (BACILLUS SPP.) IN THE CONTROL OF MOSQUITO VECTORS

A randomised, controlled, blinded field trial was conducted in a large cantonment area, to study the efficacy of the biocide formulation (Bacillus spp.) indigenously produced by Central Drug Research Institute, Lucknow, India, as a mosquito larvicide, in comparison with Fenthion and Temephos. The study revealed that the biocide formulation in dosage of 5 grams per square metre brought about a very high level of control of culicine larvae from third day of application and the effect persisted till at least 28 days (median values of percentage reduction achieved being 93% to 100%). This effect was statistically significant from 7th to 28th day, as compared to Fenthion, Temephos or the biocide in dosage of 2 grams per square metre. Fenthion, on the other hand was statistically more efficacious in bringing about a quick reduction, with a 100% reduction being obtained within a day, but the effect declined to a low level by 7th day.KEY WORDS: Mosquito control, Biological pest control, Randomized control trial     

Prevalence of heterozygotes for hemochromatosis in the white population of the United States

In previous studies, the prevalence of HLA-linked hemochromatosis, thought to be the most common genetic illness in whites, has been estimated by identifying homozygotes in the population. Because not all homozygotes express the disease phenotypically, the accuracy of these estimates is uncertain. We analyzed the distribution of transferrin saturation values in the second National Health and Nutrition Examination Survey to estimate the prevalence of hemochromatosis heterozygotes in the US population. After removing values for possible homozygotes, two populations were present (P < .01 for each gender). When weighted to reflect the US adult white male population as a whole, a proportion of 850 per 1,000 (95% confidence interval, 0.81 to 0.89) were included in a population with a lower mean saturation of 29.7% (29.1% to 30.3%), whereas 150 per 1,000 (0.11 to 0.19) comprised a population with a higher mean saturation of 47.0% (45.1% to 49.0%). Similar results were found for the female population. The gene frequencies were estimated to be 0.081 from the male population and 0.070 from the female population corresponding to prevalences of homozygotes of 6.6 and 4.8 per 1,000, respectively. Our results confirm that the gene for hemochromatosis is common.