Tibial torsion?

A longitudinal study evaluating the degree of rotation of the bimalleolar axis of tibia and fibula at birth, 6 month, 12 months and 24 months revealed a gradual statistically significant external rotation from a mean of 4 degrees at birth to a mean of 11 degrees at 24 months. There was no correlation with the sex or race of the child or with the age at which the child began independent walking. It is concluded that this external rotation is a part of normal growth and development of the infants' lower extremities.     

Arterial radio-anatomy of the extrahepatic biliary system (author's transl)

The uni-arterial vascularisation of the lateral bilary duct makes it impossible to systematise the arteries which supply the common bile duct. Only the cystic artery is seen at coelio-mesenteric catheterization. The techniques essential for visualisation of the cystic artery are discussed, on the basis of one hundred coelio-mesenteric arteriograms: frequency of demonstration, origin, course, termination, calibre and length. The frequent finding of opacification of the wall of the gall bladder under normal conditions is stressed.     

Isolation, physicochemical properties, and the macromolecular composition of the vitelline and fertilization envelopes from Xenopus laevis eggs

As a step toward defining in molecular terms the sperm-triggered block to polyspermy reaction established by the egg at fertilization, vitelline (VE) and fertilization (FE) envelopes were isolated from eggs of the Sounth African clawed toad Xenopus laevis and some of their physicochemical properties determined. Envelopes were isolated after lysis of the fertilized or unfertilized eggs by sieving techniques; isolated envelopes retained their in situ morphology as determined by electron microscopy. The isolated envelopes had different solubility properties and, in general, VE was more readily dissolved by aqueous solvents than FE, although both could be completely dissolved by detergents or chaotropic agents. Changes in envelope solubility correlated with the progression of the cortical reaction implicating a role for cortical granule material in modifying the solubility properties of the envelope. The VE and FE were composed of protein and carbohydrate with no lipid components detected. As determined by immunodiffusion experiments, the FE contained the same antigens as the VE plus components derived from the cortical granules and the innermost jelly layer, J. The macromolecular composition of the envelopes was determined by sodium dodecyl sulfate gel electrophoresis. The VE contained at least 11 glycoproteins with molecular weights ranging from 125 000 to less than 16 000 with two components (40 000 and 33 000) accounting for almost two-thirds of the total stainable material. The FE contained ten glycoproteins that had the same molecular weights as those in the VE. One glycoprotein component underwent a reduction in molecular weight from 77 000 to 67 500 when the VE was converted to the FE. This molecular weight change was interpreted as the probable result of limited proteolysis. In addition, the FE gel electrophoresis patterns contained macromolecular components derived from the cortical granules and jelly layer, J, consistent with the immunodiffusion experiments. These components were absent when the FE was prepared in the absence of Ca2+, suggesting a role for Ca2+ in binding the VE, cortical granules, and J components together. We concluded that the conversion of the glycoproteinaceous VE to FE at fertilization is caused by interaction of the VE with components from the cortical granules and jelly layer J. These interactions are of both a chemical and physical nature.     

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.     

Molecular modeling of immunoglobulin light chains implicates hydrophobic residues in non-amyloid light chain deposition disease

Light chain deposition disease is a severe complication of certain immunoproliferative disorders, due to the secretion of a monoclonal light chain which precipitates close to basement membranes of several tissues. A kappa isotype restriction and an unusual frequency of a variable region subgroup (VkappaIV) suggest that precise structural features govern the propensity of pathogenic light chains to precipitate in extracellular spaces. We studied primary structures of light chains from six patients with light chain deposition disease in comparison with light chains from other pathological conditions. Sequence alignment revealed the presence of certain amino acids only in light chain deposition disease, in particular non-polar replacing hydrophilic residues. To determine the role of these residues, structures of the variable domain from four kappa chains belonging to VkappaI and VkappaIV subgroups responsible for deposition disease were modeled using known immunoglobulins as templates. The most evident structural features shared by all pathogenic light chains were hydrophobic residues exposed to the solvent in complementarity determining regions 1 or 3. In contrast to immunoglobulin light chain- related amyloidosis, where deposition of organized material might be due to electrostatic interactions between light chain dimers, hydrophobic interactions could enhance amorphous precipitation in non- amyloid light chain deposition disease.