By describing 10 new patients recruited in centres for Human Genetics, we further delineate the clinical spectrum of a Crouzon-like craniosynostosis disorder, officially termed craniosynostosis and dental anomalies (MIM614188). Singularly, it is inherited according to an autosomal recessive mode of inheritance. We identified six missense mutations in IL11RA, a gene encoding the alpha subunit of interleukin 11 receptor, 4 of them being novel, including 2 in the Ig-like C2-type domain. A subset of patients had an associated connective tissue disorder with joint hypermobility and intervertebral discs fragility. A smaller number of teeth anomalies than that previously reported in the two large series of patients evaluated in dental institutes points toward an ascertainment bias.
Iraq−Iran war resulted in more than 400,000 people requiring prolonged medical care in Iran.An international team of prominent reconstructive surgeons led by Paul Tessier, the founder of craniofacial surgery, was invited to Iran during the war by official organizations entitled to support war victims. This team provided up-to-date oral and maxillofacial rehabilitation to patients with severe trauma defects in the lower third of the face. We collected the medical notes of 43 patients operated on by the Tessier team in Iran in the 1980s (files property of AFCF). The parameters we collected were: age of the patient, nature of the trauma (when available), previous procedures, number of implants placed (mandibular and maxillary), associated procedures (bone grafts, soft-tissue procedures, orthognathic surgery). A protocol based on soft-tissue rehabilitation using local flaps, parietal or iliac bone grafts and implant placement 6 months later was used in all patients. Paul Tessier's approach emphasizes the importance of keeping high standards of care in difficult situations and maintain standard protocols.
Anatomical variations of the stylohyoid apparatus are frequent. Two types can occur: an elongation of the stylohyoid ligament, from a long styloid process to a complete ossified structure connecting the skull base to the lesser horn of the hyoid bone, or the existence of supernumerary bones in the stylohyoid fibrous matrix, which sometimes resembles phalanges. These variations are in the majority of cases bilateral and symmetrical. The authors report the case of a 43-year-old male patient who presented with an unusual unilateral complete ossification of the stylohyoid apparatus, associated with vertebral and laryngeal calcifications. Original latest generation CT scan and three-dimensional MRI imagery are provided to illustrate this rare case.
This study was to verify the impact of the nature of the fat introduced into the feed pellets on growth performance and health of rabbits before and after weaning. Three hundred and sixty rabbits of mean weight 5091 g and from 40 litters were divided into two groups of 60 rabbits. The experiment was repeated three times. The two groups were fed with two feeds that differ in the nature of the fat introduced into the feed. In the first group, the feed contains 3% of palm oil and the second batch, 3% of soya bean oil. Both oils were characterised and the average weight, the growth rate and the health risk of young rabbits were evaluated before and after weaning. The results show a better performance of rabbits fed soya bean oil compared with those fed palm oil. Before weaning, the rabbits of the group containing soya bean oil showed an average growth of 136.75 g/week and a health risk of 20% against 135.75 g/week and 23% for the batch containing palm oil. After weaning, the average growth and the health risks are respectively 181.25 g/week and 7.5% for the group containing soybean oil against 223.75 g/ week and 17.5% for the lot containing palm oil.
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