Repair of cleft lip with nonsurgical correction of nasal deformity in the early neonatal period

Auricular cartilage is soft and plastic at birth, so that congenital auricular deformities can easily be corrected nonsurgically in the early neonatal period. However, as the infant grows older, the flexibility of the auricle decreases. Alar cartilage exhibits the same elasticity as auricular cartilage in the early neonate. When a cleft lip is repaired, typically when the infant is about 3 months of age, it becomes difficult to correct the nasal deformity without surgical intervention. However, based on our experience, there is a fair possibility of correcting the cleft lip nasal deformity with a nonsurgical procedure in the early neonatal period. We performed cleft lip repair accompanied by nonsurgical correction of the nasal deformity in 44 neonates aged 2 to 7 days. A special retainer was placed in the affected nostril for 3 months. Following observation of 31 infants for 12 months or longer, their nasal shapes and symmetry were considered superior to those conventionally operated on at about 3 months of age. Except for one nasal infection, there were no complications.     

Hereditary syndrome of ectrodactyly, congenital heart defect and cleft lip and palate

A case of a family of he proband suffering from ektrodaktyly, together with heart defect, cleft lip and palate is reported. The parents came to the genetic counselling center to get the genetic prognosis for a next child. All together 3 cases demonstrating different types of radial defects were registered in this pedigree. The authors discuss possible causes of clinical polymorphism and accent the complications of giving genetic prognosis in such cases.     

唇瘘、腭裂及唇裂综合征两个家系的遗传分析

我们在此报道北京地区没有亲缘关系的两个家庭中属于van der woude综合征的常染色体显性遗传病,其临床症状包括下唇近中线处唇瘘(下唇有先天性向下的瘘管)、腭裂、唇裂(具有或不具有腭裂)。这三个临床症状可以同时发生在一个受累个体身上,或任何两个症状出现在一个个体身上。这种人携带了这种致病基因。在我们所报道的家庭中,先证者每人还具有舌系带过短的体征。     

Reconstruction of a compound facial deformity involving the columella, nasal base, and upper lip

A compound facial defect including the columella, a wide full-thickness loss of the upper lip, and the nasal floor is a severe disfigurement. This patient was a victim of noma in childhood. This report includes a discussion of the problem and a way of solving it with possible available methods in order to obtain an acceptable result.     

Simultaneous repair of the upper lip and nostril floor after tumor excisions.

A lateral lip flap, with a perialar extension, is described for reconstruction of defects of the upper lip which extend into the columella and/or the nostril floor.     

Effect of insulin and cortisol on oxidation of (1-14C)glucose, (6-14C)glucose, (1-14C)palmitate and (1-14C)leucine in the tissues of swine during the neonatal period

The intensity of [1-14C]glucose, [6-14C]glucose, [1-14C]palmitate and [1-14C]leucine oxidation and the effect of insulin and hydrocortisone on this process were studied in the brain, duodenum mucosa, liver and skeletal muscle of 1- and 5-day old piglets in vitro. Most of the studied substrates are oxidized in the tissues of 5-day piglets more intensively. Insulin stimulates oxidation of [1-14C]glucose, [6-14C]glucose and [1-14C]leucine in the brain and duodenum mucosa in 1- and 5-day old piglets, while in the liver and skeletal muscle--only in 5-day old piglets. Hydrocortisone administration enhances oxidation of [1-14C]leucine in most of the studied tissues in 1-day piglets and oxidation of [1-14C]glucose and [6-14C]glucose--in 5-day piglets. Both hormones produce no essential influence on the intensity of [1-14C]palmitate oxidation in the studied tissues of piglets or somewhat weaken it.