Nonsurgical correction of nasal deformity in unilateral complete cleft lip: a 6-year follow-up.

Nasal deformity in unilateral cleft lip and palate patients increases with time, tongue malposition being one of the causes. Some authors have emphasized the role of nasal and adjacent facial musculature as active extrinsic agents. Another cause of alar deformity can be the lack of a proper foundation because of a maxillary hypoplasia in the region of the pyriform foramen. If alar collapse occurs, the septum bends convexly toward the cleft side. Tissues are soft and plastic during the neonatal period. Once the infant is about 3 months of age, it becomes difficult to correct the nasal deformity. Therefore, any resource used from the first day, and mainly during the first 15 days of life, will be useful to prevent the increasing deformity and to avoid the surgical correction. A controlled clinical trial was planned to compare the anthropometric measurements of the nasal region in two series of patients with unilateral complete cleft lip. In the first group, we included 44 patients who came to our clinic during the first 2 days of life and the second group consisted of 47 patients who were more than 15 days of age at the time of the first consultation. To provide control data for the evaluation of the results after 6 years of follow-up in both series of cleft patients, we also included a third group of 48 healthy 6-year-old children. A nasal component added to the occlusal prostheses was only used in the first group up to the time of surgery. The same surgeon performed a Millard II procedure with muscular reposition as described by Delaire in all the patients. Nasal measurements taken with a caliper, obtained directly from plaster models by using surface impressions of the babies, were confirmed by a laser three-dimensional measuring device. The statistical comparison between both series showed a significant increase of the columellar length in the first group. A 6-year follow-up to compare growth and cosmetic results of the nose revealed a better and permanent nasal nostril symmetry and no alar cartilage luxation in the patients who had had the nasal component. These results highlight the importance of the early treatment and allow us to suggest the nasal prostheses as a way to prevent the increasing nasal deformity, to help nasal remodeling, to obtain columellar elongation, and to avoid or decrease the need for primary surgery of the cleft nose.

Effect of total food deprivation on the proteoglycans of rat hyaline cartilage and bone.

Existing hypotheses suggest that the effect of food deprivation on bone occurs via alterations in the synthesis of the organic matrix. Thus, this work was carried out to characterize the modifications of the physico-chemical properties of the proteoglycans (PG) of rat hyaline cartilage and femur. Male Wistar rats were assigned at random to a control group which was fed a standard pellet diet or to an experimental group which was given water "ad libitum" and starved over the experimental period. On day 4 or 8 the animals were administered a dose of 35S, weighed and killed. PG and glycosaminoglycans (GAG) were isolated from femurs and xyphoid cartilages. Uptake of 35S, GAG distribution patterns, PG molecular weight, molecular size of the side chains and the PG density gradient were determined. The aforementioned parameters decreased significantly after 4 and 8 days of total starvation. The GAG distribution pattern only exhibited a reduction of the Chondroitin-4-Sulphate fraction. These changes could alter the binding properties of PG to other macromolecules such as collagen which plays an important role in the ossification process.

Effect of total food deprivation on the proteoglycans of rat hyaline cartilage and bone.

Existing hypotheses suggest that the effect of food deprivation on bone occurs via alterations in the synthesis of the organic matrix. Thus, this work was carried out to characterize the modifications of the physico-chemical properties of the proteoglycans (PG) of rat hyaline cartilage and femur. Male Wistar rats were assigned at random to a control group which was fed a standard pellet diet or to an experimental group which was given water [quot ]ad libitum[quot ] and starved over the experimental period. On day 4 or 8 the animals were administered a dose of 35S, weighed and killed. PG and glycosaminoglycans (GAG) were isolated from femurs and xyphoid cartilages. Uptake of 35S, GAG distribution patterns, PG molecular weight, molecular size of the side chains and the PG density gradient were determined. The aforementioned parameters decreased significantly after 4 and 8 days of total starvation. The GAG distribution pattern only exhibited a reduction of the Chondroitin-4-Sulphate fraction. These changes could alter the binding properties of PG to other macromolecules such as collagen which plays an important role in the ossification process.

Characterization of a newly discovered alpha-thalassaemia-1 in two Spanish patients with Hb H disease.

A new deletion of more than 27 kb, removing the psi zeta 1, psi alpha 2, psi alpha 1, alpha 2, alpha 1 and theta 1 globin genes has been found in four members of a Spanish family, including two patients with Hb H disease. The 5' end point of the deletion is located between the zeta and psi zeta genes, and the 3' end of the deletion is downstream of the 3' hypervariable region.

Hb Las Palmas or alpha 2 beta 2(49)(CD8)Ser----Phe, a mildly unstable hemoglobin variant.

A new hemoglobin variant with a Ser----Phe substitution at position beta 49(CD8) was discovered in two members of a family living in the Canary Islands, Spain. Detection was by polyacrylamide gel electrophoresis and by reversed phase high performance liquid chromatography. The variant, which constituted 43% and 45%, respectively, in the two heterozygotes, was slightly unstable. Its presence did not affect hematological values though there was a mild reticulocytosis.