Apparent total alpha1-antitrypsin deficiency: report of a case

A 29-year-old female, with chronic renal failure and chronic bilateral emphysema, was admitted with severe uremia and septicemia secondary to multiple abscesses in the right kidney. Her condition improved after right nephrectomy. Pulmonary function studies showed marked obstructive and restrictive lung disease consistent witht the diagnosis of primary emphysema. On biochemical and histological examination, the liver was found to be normal. Alpha1-antitrypsin could not be demonstrated in the patient's serum at normal pH by any of the known techniques, but protein molecules with alpha1-antitrypsin antigencity were found at pH 4.8; this suggests a pH-dependent structural difference in alpha1-antitrypsin protein. Starch gel electrophoresis gave a multibanding pattern not previously described. A new form of apparent total alpha-1-antitrypsin deficiency is postulated.     

Long tube implants in the management of glaucoma

The design, surgical insertion and results of a plastic draining implant for severe glaucoma are reported. The need for pharmacological control of bleb inflammation is stressed and the favourable long-term outlook for patients with such implants is discussed.     

Direct evidence for the role of haem doming as the primary event in the cooperative transition of haemoglobin

The study of cooperative ligand binding among the four subunits of haemoglobin has played a central role in the understanding of allosteric transitions in a large number of enzymes. Haem iron out-of-plane motion has been suggested to be the trigger for the cooperative transition of haemoglobin. To function as a trigger in a dynamic sense, haem-iron doming must be the first conformational change to occur following ligand dissociation. Here we present the first direct demonstration that haem-iron doming occurs on the same time scale as the breaking of the iron-ligand bond, thus establishing haem-iron doming as the primary event which lead to the R-->T transition in haemoglobin.     

Food allergy to peanuts in France--evaluation of 142 observations

BACKGROUND: The increase in frequency of peanut allergy and fatal cases have been reported. OBJECTIVES: The objective of this study is to document the severity of food allergy to peanuts by evaluating the reactive dose of peanuts and to search for the role of peanut oil. METHODS: This study is carried out on the basis of 142 observations collected according to the same diagnostic methodology in two allergy centres in France. Skin-prick-tests were performed with peanut powder, peanut oil and peanut oil proteinic extract. Labial provocation tests were performed on 121 patients. The reactive dose of peanuts and the role of peanut oil were determined by standardized oral provocation tests in 50 and 62 patients respectively. The data are computerized and the data bank includes 509 food allergic patients. RESULTS: Allergy to peanuts represents 28% of food allergies and occurs under 1 year of age in 46% of cases, under 15 years of age in 93%. The clinical features were atopic dermatitis (40%), angioedema (37%), asthma (14%), anaphylactic shock (6%) and digestive symptoms (1.4%). The specific IgE were class 3 or higher in 80% of cases. The total reactive dose was less than 100 mg in 25% of cases, from 100 mg to 1 g in 62.5%. All patients reacted to a dose of less than 7.1 g. The threshold of peanut reactivity was lower than the threshold of egg reactivity. An allergy to peanut oil was demonstrated in 14 patients. CONCLUSION: The severity of peanut allergy and the early onset of the occurrence of this allergy is documented. The role of residual allergenic proteins in peanut oil is established by positive skin-prick tests to proteic extracts from peanut oil and by double-blind placebo-controlled challenges to peanut oil. The increased consumption of allergens in the form of peanut oil and fats can contribute to the occurrence or persistence of symptoms and may be suspected to increase the risk of sensitisation.     

Evolving magnetic resonance spinal cord trauma in child: from hemorrhage to intradural arachnoid cyst

Acquired arachnoid cysts of the spinal cord are uncommon causes of spinal cord compression in the pediatric group. Meningitis, trauma and hemorrhage are considered to be causative or contributing factors. Interestingly, no spinal arachnoid cysts have been reported in patients after subarachnoid hemorrhage or meningitis, conditions expected to cause arachnoid scarring. We describe a child of 1 year and 10 months with thoracic spine trauma with crural paraplegia and anesthesia at level T5 submitted to serial magnetic resonance imagery at 5 days and 18 months, after trauma showing evolution from subarachnoid hemorrhage and adhesions of the arachnoid space to a posterior hypertensive thoracic intradural arachnoid cyst.     

DEHYDRATION BY DESORPTION AND BY SPRAY DRYING OF DAIRY PROTEINS: INFLUENCE OF THE MINERAL ENVIRONMENT

A drying method by desorption in a water activity meter was used to simulate the conditions of spray drying and to determine the water transfer inside dairy concentrates towards the surface and from the surface to the drying air. The concentrates were also spray dried and solubility index of powders were determined during reconstitution. Whey protein concentrates (WPC) and native phosphocaseinate suspensions (NPCS) were used to study the effect of NaCl (420 mM), CaCl₂ (222 raM), sodium phosphate (173 mM) and sodium citrate (238 mM) on the water transfers. The decrease in water transfer during drying was explained by the high hygroscopicity of added mineral salts to WPC. NaCl addition to NPCS decreased the water transfer during drying, but increased the solubility index. Citrate and phosphate addition to NPCS increased the water transfer during drying and reconstitution. CaCl₂ increased the water transfer during drying but the solubility index was always low. Results are discussed as a function of the aqueous environment, of the nature of mineral salts, of the structure of dairy proteins and of protein solvation.     

美试射小功率战术激光炮

据外电报道．美国空军首次试验了一种新式激光武器．并成功击中标靶。美军提供的录像显示,一架在高空飞行的C-130H“大力神”运输机利用其装备的“先进战术激光”系统．准确命中了位于白沙导弹靶场的目标——一辆汽车前发动机盖上手帕大小的区域．激光束融化了发动机盖．并产生了大量火花。