A case of Aicardi syndrome with cleft lip and palate was experienced at the Department of Pediatrics, College of Medicine, Hallym University, and the patient's infantile spasm was treated with ACTH. In previous studies, four cases of Aicardi syndrome accompanied by cleft lip and palate were reported. We present the fifth case of Aicardi syndrome with cleft lip and palate in the world. Thus, facial clefts may be found as an occasional manifestation of Aicardi syndrome.
Adrenocorticotropic Hormone ; Aicardi Syndrome* ; Cleft Lip* ; Infant ; Infant, Newborn ; Palate* ; Pediatrics ; Spasms, Infantile

Shwachman-Diamond syndrome (SDS) is a rare genetic disorder of unknown pathogenesis involving exocrine pancreatic insufficiency and hematological and skeletal abnormalities. About 25% of patients develop hematopoietic malignancies. We report on a case of acute myeloid leukemia (M2) in a 21-year-old woman affected by SDS. She was treated with conventional chemotherapy (idarubicin plus cytarabine) and reached complete remission of leukemia. After induction chemotherapy, she underwent allogeneic bone marrow transplantation (BMT). The BMT preparative regimen consisted of total body irradation (TBI) followed by cyclophosphamide. Cyclosporin A and short term methotrexate were used for graft-versus-host disease prophylaxis. After a follow-up of 12 months, she is alive leukemia free off any immunosuppressive agent. Although experience in this field is scarce, we speculate that bone marrow failure in SDS is an indication for BMT which is the only curative trentment option.
Adult ; *Bone Marrow Transplantation ; Case Report ; *Cell Transformation, Neoplastic ; Female ; Human ; Leukemia, Myelocytic, Acute/*pathology/*therapy ; Myelodysplastic Syndromes/*complications/*therapy ; Pancreatic Insufficiency/complications/therapy ; Syndrome ; Transplantation, Homologous

BACKGROUND: Interleukin 31 (IL-31) is a T helper type 2 effector cytokine that plays an important role in the pathogenesis of atopic and allergic diseases. IL-31 may be involved in promoting allergic inflammation and in inducing airway epithelial responses such as allergic asthma. METHODS: Single-base extension analysis was used to detect the genotypes of IL-31 single nucleotide polymorphisms (SNPs), and we compared the genotype and allele frequencies of the IL-31 SNPs between patients with asthma and healthy controls. RESULTS: There were no significant differences in the genotype and allele frequencies of the IL-31 SNPs between patients with asthma and healthy controls. Furthermore we compared the genotype and allele frequencies of IL-31 SNPs between patients with atopic asthma, those with non-atopic asthma and healthy controls. This showed that the SNPs were not associated with the susceptibility to atopic asthma. There were no significant differences in the haplotype frequencies of IL-31 SNPs between patients with asthma and healthy controls. In patients with asthma, the IL-31 SNPs were significantly correlated with total serum levels of IgE (p=0.035). CONCLUSIONS: Our results indicate that, the IL-31 SNPs may be associated with IgE production in patients with asthma.
Asthma ; Gene Frequency ; Genotype ; Haplotypes ; Humans ; Immunoglobulin E ; Inflammation ; Interleukins ; Polymorphism, Single Nucleotide

BACKGROUND/AIMS: It is still unclear whether Super Paramagnetic Iron Oxide-Magnetic Resonance Imaging (SPIO-MRI) is a clinically useful imaging modality for patients with hepatocellular carcinoma (HCC). This study searched for the clinical usefulness and limitations of SPIO-MRI with respect to tumor detection capacity, false positive and negative rate, and early recurrence rate. METHODS: From December 1999 to February 2001, 218 patients who were surgical candidates by 3-phase dynamic helical CT (3dHCT) were enrolled. We reviewed the medical records and radiologic findings, retrospectively, and postulated the post-operative pathologic findings and the early recurrences within 3 months as the standards for the true positive lesion. RESULTS: The mean number of nodules detected by SPIO-MRI was significantly more numerous than that of 3dHCT (p<0.01). Modifications of treatment strategy due to the discordant findings between SPIO-MRI and 3dHCT for tumor resectability were observed in 22 (10.1%) out of 218 patients. Early recurrences were observed in 10 patients (7.8%). The false positive and negative rates of SPIO-MRI were 6.3% and 13.3%, respectively. CONCLUSIONS: We demonstrated that the tumor detection rate of SPIO-MRI was better than that of 3dHCT. Given the relatively acceptable false positive and negative rates, SPIO-MRI could be an appropriate preoperative imaging modality for patients with HCCs.
Adult ; Aged ; Carcinoma, Hepatocellular/*diagnosis ; *Contrast Media ; False Negative Reactions ; False Positive Reactions ; Female ; Ferrosoferric Oxide ; Humans ; Iron/*diagnostic use ; Liver Neoplasms/*diagnosis ; *Magnetic Resonance Imaging ; Male ; Middle Aged ; Oxides/*diagnostic use ; Tomography, Spiral Computed

PURPOSE: The purpose of this study was to evaluate the effects of hydroxyapatite containing toothpaste for patients who received periodontal therapy and felt hypersensiptivity. MATERIAL AND METHODS: After application of toothpaste, patients were evaluated for VAS(Visual Analog scale) scores to a cold stimulate on baseline, 1 week, and 4 weeks. VAS scores were analyzed by statistical methods. RESULTS: The results of this study were as follows. 1. VAS scores in control group on baseline, 1 week, 4 weeks were 5.39+/-2.05, 4.75+/-2.00, 4.21+/-1.75. 2. VAS scores in experimental group on baseline, 1 week, 4 weeks were 5.61+/-2.37, 4.81+/-2.46, 4.08+/-2.54. 3. Decrease of VAS scores on hypersensitivity after 1week was 0.64+/-0.49(p<.0001) in control group, 0.80+/-1.65 (p<.0001) in experimental group. 4. Decrease of VAS scores on hypersensitivity after 4weeks was 1.18+/-1.60(p<.0001) in control group, 1.53+/-1.88 (p<.0001) in experimental group. 5. When compared, decrease of VAS scores after 1 week between 2 groups were not statistically different(p=0.2622). 6. When compared, decrease of VAS scores after 4 weeks between 2 groups were not statistically different(p=0.1219). CONCLUSION: It was confirmed that hydroxyapatite containing toothpaste have the similar effect with pre-existing proven dentifrice for relieving teeth hypersensitivity.
Cold Temperature ; Dentifrices ; Durapatite ; Humans ; Hypersensitivity ; Tooth ; Toothpastes

The major goals of periodontal therapy are the functional regeneration of periodontal supporting structures already destructed by periodontal disease as well as the reduction of signs and symptoms of progressive periodontal disease. There have been many efforts to develop materials and therapeutic methods to promote periodontal wound healing. There have been increasing interest on the chitosan made by chtin. Chitosan is a derivative of chitin made by deacetylation of side chains. Chitosan has been widely studied as bone substitution and membrane material in periodontology. Many experiments using chitosan in various animal models have proven its beneficial effects. Tetracycline has been considered for use in the treatment of chronic periodontal disease and gingivitis. The aim of this study is to evlauate the osteogenesis of tetracycline blended chitosan membranes on the calvarial critical size defect in Sprague Dawley rats. An 8mm surgical defect was produced with a trephine bur in the area of the midsagittal suture. The rats were divided into five groups: Untreated control group versus four experimental group. Four types of membranes were made and comparative study was been done. Two types of non-woven membranes were made by immersing non-woven chitosan into either the tetracycline solution or chitosan-tetracycline solution. Other two types of sponge membranes were fabricated by immersing chitosan sponge into the tetracycline solution, and subsequent freeze-drying. The animals were sacrificed at 2 and 8 weeks after surgical procedure. The specimens were examined by histologic analyses. The results are as follows: 1. Clinically the use of tetracycline blended chitosan membrane showed great healing capacity. 2. The new bone formations of all the experimental group, non-woven and sponge type membranes were greater than those of control group. But, there was no significant difference between the experimental groups. 3. Resorption of chitosan membranes were not shown in any groups at 2 weeks and 8 weeks. These results suggest that the use of tetracycline blended chitosan membrane on the calvarial defects in rats has significant effect on the regeneration of bone tissue in itself. And it implicate that tetracycline blended chitosan membrane might be useful for guided tissue regeneration.
Animals ; Bone and Bones ; Chitin ; Chitosan* ; Gingivitis ; Guided Tissue Regeneration ; Membranes* ; Models, Animal ; Osteogenesis ; Periodontal Diseases ; Porifera ; Rats ; Rats, Sprague-Dawley* ; Regeneration ; Sutures ; Tetracycline* ; Wound Healing