Septo-optic dysplasia, as first described by de Morsier, is a rare developmental anomaly of absent or hypoplastic septum pellucidum, a primitive optic vesicle, and dysplasia of the optic nerve, chiasm, and optic tracts. It is a common cause of hypopituitarism in children and has variable hypothalamic-pituitary dysfunction. We experienced a case of septo-optic dysplasia in 5 month old female baby who presented with developmental delay and seizure. A magnetic resonance imaging of the brain showed absence of the septum pellucidum and the fundoscopic examination disclosed the absence of left optic disc and atrophy of right optic disc.
Atrophy ; Brain ; Child ; Female ; Humans ; Hypopituitarism ; Infant ; Magnetic Resonance Imaging ; Optic Nerve ; Seizures ; Septo-Optic Dysplasia* ; Septum Pellucidum ; Visual Pathways

OBJECTIVE: Moyamoya disease (MMD) is an uncommon cerebrovascular disorder, characterized by progressive occlusion at the terminal portion of the internal carotid artery. Incidence of the disease is high in East Asia and familial MMD accounts for about 15% of the disease. Although the pathogenesis is unknown, association of HLA class I or II alleles with MMD has been reported with conflicting results. We investigated whether there is a difference in HLA class II association between familial and non-familial forms of the disease. METHODS: A total of 70 Korean children with MMD, including 16 familial cases (10 probands), and 207 healthy controls were studied. Among familial cases, only 10 probands were used for the HLA frequency analysis. High resolution HLA-DRB1 and DQB1 genotyping was performed using polymerase chain reaction (PCR)-sequence specific oligonucleotide hybridization and PCR-single strand conformation polymorphism methods. RESULTS: The phenotype frequencies of HLA-DRB1*1302 (70.0%) and DQB1*0609 (40.0%) were significantly increased in familial MMD compared to both controls [vs. 15.5%, corrected p (pc) = 0.008, odds ratio (OR) = 12.76; vs. 4.3%, pc = 0.02, OR = 14.67] and non-familial MMD patients (vs. 14.8%, pc = 0.02, OR = 13.42; vs. 1.9%, pc = 0.02, OR = 35.33). The frequencies of DRB1 and DQB1 alleles in non-familial MMD patients were not significantly different from those in controls. CONCLUSION: Our findings suggest that the genetic polymorphism of HLA class II genes or other closely linked disease relevant gene(s) could be a genetic predisposing factor for familial MMD.
Alleles ; Carotid Artery, Internal ; Cerebrovascular Disorders ; Child ; Chimera ; Far East ; Genes, MHC Class II ; HLA-DQ Antigens ; HLA-DR Antigens ; HLA-DRB1 Chains ; Humans ; Incidence ; Moyamoya Disease ; Odds Ratio ; Phenotype ; Polymerase Chain Reaction ; Polymorphism, Genetic

No abstract available.
Antibodies, Neutralizing* ; Feces* ; Rotavirus*

No abstract available.
Amenorrhea* ; Female

No abstract available.
Endometrial Neoplasms* ; Female ; Ovary*

No abstract available.
Bone Density* ; Female ; Humans ; Hypogonadism*

We have expressed GFP in Sf9 and Bm5 cells or Bombyx by larvae by using Ac-Bm hybrid virus capable of replicating in both Bm5 and Sf9 cells. Genomic DNA of Ac-Bm hybrid virus expressing P-galactosidase was cotransfected with baculovirus transfer vector containing GFP gene, pBacPAK-GFP in Sf9 cells. The Ac-Bm hybrid virus harboring GFP was named as Ac-Bm hybrid virus-GFP. The Ac-Bm hybrid virus-GFP-infected insect cells were easily selected by detecting the emission of GFP from each well of cell culture dish on the UV illuminator. GFP produced by Ac-Bm hybrid virus-GFP in Sf9 and Bm5 cells or B. mori larvae was confirmed by SDS-PAGE and Western blot analysis using GFP antibody. In addition, B. mori larvae infected with Ac-Bm hybrid virus-GFP was apparently appeared fluorescence from the whole body at 5 days postinoculation. The fluorescence of GFP from the hemolymph and fat body of B. mori larvae infected with Ac-Bm hybrid virus-GFP was also observed by fluorescence microscope. In conclusion, our results demonstrated that in baculovirus expression vector system, use of Ac-Bm hybrid virus have an additional advantage of expanded host range for producing recombinant proteins.
Animals ; Baculoviridae ; Blotting, Western ; Bombyx* ; Cell Culture Techniques ; DNA ; Electrophoresis, Polyacrylamide Gel ; Fat Body ; Fluorescence ; Hemolymph ; Host Specificity ; Insects ; Larva* ; Recombinant Proteins ; Sf9 Cells ; Spodoptera*

Endoscopic sphincterotomy(EST) is now an established therapeutic procedure for various disorder of the pailla of Vater, the biliary tract, and the pancreas. From November 1992 to September l993, 123 cases of E.S.T were performed in our hospital. The success rate of EST was 97.8%, and choledocholithiasis was the indication for EST in 63. 4% of cases. Among 78 cases of choledocholithiasis, 47 cases were presence of gall bladder with stone (16 cases) or without stone (31 cases), especially 46 cases were assisted with needle type papillotome and 23 cases were assisted with guidewire. EST hae relatively low complications and is the therapy of choice for choledocholithiasis and various diisease of biliary tract. Guidewire assisted stanard papillotome probable reduce the use of needle type papillotome in the difficult cases that EST with pull type papillotome was impossible.
Biliary Tract ; Choledocholithiasis ; Needles ; Pancreas ; Sphincterotomy, Endoscopic* ; Urinary Bladder

No abstract available.
Arteriovenous Malformations* ; Hysterectomy* ; Uterus*

No abstract available.
Diagnosis* ; Pancreas* ; Ultrasonography*