Congenital arhinia: A rare case.

PATIENT: Male, 4 months FINAL DIAGNOSIS: Congenital arhynia Symptoms: Absence of the nose Medication: - Clinical Procedure: - Specialty: Pediatrics and Noenatology • Genetics. OBJECTIVE: Congenital defects. BACKGROUND: Congenital nasal absence (arhinia) is an extremely rare malformation. Arhinia causes severe airway obstruction and poor feeding in the affected neonate. There is an association with other facial anomalies, especially defects of the eyes, ears, palate, and midline defects. CASE REPORT: A full-term boy was born via an uncomplicated vaginal delivery. The mother was 40 years old and had a normal pregnancy. The mother had 4 previous uncomplicated pregnancies. There was no history of drug use during pregnancy. CONCLUSIONS: Congenital arhinia is a rare defect of embryogenesis, often associated with other anomalies that significantly influence the immediate and long-term outcomes of the neonate. It is a potentially life-threatening condition and requires the presence of a highly skilled neonatal resuscitation team at the time of delivery. Parental counseling is vital and a multidisciplinary team approach is required to optimize neonatal outcome.

[Construction of a tissue engineering skin with epidermal stem cells].

OBJECTIVE: To constitute a composite skin substitute that can proliferate well with epidermal stem cells and fibroblasts on collagen sponge. METHODS: Epidermal stem cells were selected by rapid attachment to collagen IV for 10-15 min and cultured on 3T3 feeder layers. Collagen was extracted from rat tail. The matrix lattice was fabricated by freeze-dryer and cross-linked with glutaraldehyde. Fibroblasts were inoculated on collagen sponge and cultured for 4 days prior to inoculation of epidermal stem cells to construct composite skin substitute. The composite skin substitute were examined by means of histology, immunohistochemistry and electron microcopy, the histologic appearance was similar to that of normal epidermis. RESULTS: The epidermal stem cells formed large colonies at 7-8 days, expressed K19 antigen. The percentages of cells at G0/G1 phase of cell cycle and the percentage of alpha6 briCD71dim cells in ESC groups were higher than those in the control group. The skin substitute had epidermis and dermis, the histologic appearance was similar to that of normal skin. The artificial skin expressed keratin antigen by immunocytochemical methods. CONCLUSIONS: Epidermal stem cells proliferated well and differentiated properly on this artificial skin dermis which contained fibroblasts. It seemed that the composite skin to be a good equivalent.