Dieulafoy's vascular malformation is a rare cause of massive gastrointestinal bleeding. Most often it occurs in stomach within 6 cm from the gastroesophageal junction. Only a few cases have been reported to occur in the small intestine and colon. Occasionally, Dieulafoy's lesion of small intestine is difficult to recognize because of rarity, a paucity of symptoms and negative findings on barium studies. Therefore, this lesion needs to be considered in a patient with massive lower gastrointestinal bleeding. We report a case of Dieulafoy's vascular malformation in ileum 2 m proximal to ileocecal value in a 41-year-old woman who visited emergency clinic because of hematemesis, dizziness and vomiting. Small intestine revealed a wide-caliber artery within the submucosa showing intimal thickening, medial muscular hypertrophy and thrombosis.
Adult ; Arteries ; Barium ; Colon ; Dizziness ; Emergencies ; Esophagogastric Junction ; Female ; Hematemesis ; Hemorrhage* ; Humans ; Hypertrophy ; Ileum* ; Intestine, Small ; Stomach ; Thrombosis ; Vascular Malformations* ; Vomiting

PURPOSE: To evaluate the MR imaging findings of patients with mesial temporal sclerosis. MATERIALS AND METHODS:We retrospectively reviewed the MR imaging findings of 116 patients diagnosed by MRI as suffering from mesial temporal sclerosis. In 18 of these, the condition was also histologically proven. RESULTS: Among the 116 patients, volume loss of the hippocampus was found in 95 (81.9%) and signal changes of the hippocampus in 53 (45.7%). Decreased signal intensity in the hippocampus on T1-weighted images was found in 13 (11.2%) and increased signal on T2-weighted images in 50 (43.1%). Signal abnormality in the hip-pocampus on both T1- and T2-weighted images was found in ten, and associated extrahippocampal abnormali-ties, as follows, in 20 (17.2%): atrophy of the fornix (n=10), atrophy of the mammillary body (n=8), atrophy of the amygdala (n=10), atrophy or increased T2 signal intensity of the anterior thalamic nuclei (n=2), atrophy of the cingulate gyrus (n=2), atrophy or increased signal intensity of the anterior temporal lobe (n=8), and cere-bral hemiatrophy (n=4). CONCLUSION: A high T2 signal and atrophy of the hippocampus are the most common and important MRI findings of mesial temporal sclorosis. Other abnormal findings, if any, which may be found in extrahippocampal structures such as the fornix, mammillary body and temporal lobe, should, however, also be carefully observed
Amygdala ; Anterior Thalamic Nuclei ; Atrophy ; Epilepsy ; Gyrus Cinguli ; Hippocampus ; Humans ; Magnetic Resonance Imaging* ; Mamillary Bodies ; Retrospective Studies ; Sclerosis* ; Temporal Lobe

PURPOSE: The need for continuing Growth Hormone(GH) replacement after adolescence in patients with childhood-onset GH deficiency has been recognized. The purpose of this study was to evaluate the abnormalities of lipid profiles in young adults with childhood-onset hypopituitarism who discontinued GH therapy after the completion of height growth. METHODS: Nine male patients(mean age:22.4+/-3.3 years) with childhood-onset hypopituiatarism in whom GH treatment had been discontinued after final height was achieved were included. Their body mass index(BMI) and serum levels of total cholesterol, triglyceride(TG), high-density lipoprotein(HDL) cholesterol, and low-density lipoprotein(LDL) cholesterol were measured. The relationships of duration after GH discontinuation, age, and BMI to lipid profiles were anaylzed. RESULTS: BMI increased significantly from 21.8+/-1.9 kg/m2 before GH discontinuation to 23.0+/-3.0 kg/m2 after GH discontinuation(P<0.05). Serum levels of total cholesterol, TG, HDL cholesterol, and LDL cholesterol were 217.3+/-33.4, 237.8+/-128.2, 42.1+/-7.1, and 127.4+/-27.2 mg/dL, respectively, 3.1+/-2.0 years after GH discontinuation. Percentage of patients who had total cholesterol > or = 200 mg/dL, TG > or = 150 mg/ dL, LDL cholesterol > or = 140 mg/dL, and HDL cholesterol < or = 40 mg/dL were 77.8%, 88.9%, 44.4%, and 33.3%, respectively. All subjects had some abnormalities of lipid profiles. A significant positive correlation was found between duration after GH discontinuation and serum levels of total cholesterol and TG(r=0.84, P<0.01; r=0.83, P<0.01). A significant positive correlation was also found between age and serum levels of total cholesterol and TG(r=0.86, P<0.01; r=0.81, P<0.01). There were no correlations between BMI and serum lipid levels. CONCLUSION: Most of young adult patients with childhood-onset hypopituitarism had abnormal lipid profiles by 1-5 years after discontinuation of GH treatment. These data suggest that continuous GH treatment after completion of height growth is necessary.
Adolescent ; Adult* ; Cholesterol ; Cholesterol, HDL ; Cholesterol, LDL ; Growth Hormone* ; Humans ; Hypopituitarism* ; Male ; Young Adult

Purpose : The main genetic contribution to type 1 diabetes susceptibility is the human leukocyte antigen (HLA) class II gene. Several non-HLA chromosomal regions are also known to be involved. We studied the association of HLA class II and non-HLA candidate genes, which are cytotoxic T lymphocyte antigen-4 (CTLA4), tumor necrosis factor (TNF), lymphotoxin-alpha(LT-alpha, and vitamin D receptor (VDR) gene, polymorphisms with disease susceptibility in Korean children with type 1 diabetes.Methods : Fifty Korean children with type 1 diabetes (29 girls and 21 boys) and 166 healthy Koreans were investigated in this study. HLA class II alleles were determined by PCR-SSP (sequence-specific primer) and PCR-SSOP (sequence specific oliogonucleotide probe) method. CTLA4 exon 1 polymor phism was analyzed by PCR-SSCP (single strand conformation polymorphism), and TNF promotor and LT-alphagene polymorphism by PCR-RFLP (restriction fragment length polymorphism), respectively. VDR gene polymorphisms were analyzed by PCR-RFLP using restriction enzyme FokI, ApaI, TaqI, and BsmI. Results : The frequencies of HLA-DRB1*04, DRB1*09, and DQB1*04 were significantly increased and those of HLA-DRB1*14, DRB1*15, DQB1*05 and DQB1*06 were significantly decreased in the patients with type 1 diabetes compared with the control subjects. No significant differences in the distribution of CTLA4 exon 1, TNF promotor, LT-alpha and VDR gene polymorphisms were observed between the patients with type 1 diabetes and the control subjects. Conclusion : These data suggest that HLA-DRB1*04, DRB1*09, and DQB1*04 are susceptible genes for type 1 diabetes, whereas HLA-DRB1*14, DRB1*15, DQB1*05, and DQB1*06 are protective genes in Korean children. CTLA4 exon 1, TNF promotor, LT-alpha and VDR gene polymorphisms are not associated with susceptibility to type 1 diabetes in Korean children.
Alleles ; Child* ; Diabetes Mellitus, Type 1* ; Disease Susceptibility* ; Exons ; Female ; Genes, MHC Class II ; Humans ; Leukocytes ; Lymphocytes ; Receptors, Calcitriol ; Tumor Necrosis Factor-alpha

No abstract available.
Orbit*

This study was undertaken to evaluate the clinical usefulness of magnetic motor evoked potential (MEP) in the diagnosis of stroke and predicting the motor improvement following stroke. The cortical, cervical and lumbar stimulations were performed in the case of 24 healthy controls and 24 to a target muscle between after transcranial stimulation and after cervical or lumbar stimulation. There was no case showing no response in controls. But in 11 out of 24 ischemic patients, we could not get cortical MEP. Mean CMCT of abductor pollicis brevis muscle was not significantly different in controls and stroke patients in whom MEPs were recorded. There were significant differences between mean CMCT of normal controls and that of stroke patients showing MEPs in AH Muscle. MEP Results from testing the stroke patients were correlated with site of lesion, degree of motor weakness and motor improvement after 1 to 2 months. These results suggest that magnetic MEP is easy and useful in electrophysiological test of central motor pathway and is useful indicator for representing the motor weakness and predicting the motor outcome in acute ischemic stroke patients.
Diagnosis ; Evoked Potentials, Motor* ; Humans ; Stroke*

No abstract available.
Breast* ; Humans*

46,XX male is a rare abnormality of sex determination with an incidence of 1 in 20,000 male neonates. The clinical manifestations of 46,XX males are usually hypogonadism, gynecomastia, azoospermia, and hyalinations of seminiferous tubules, with altered hormonal levels at puberty. Less frequently, some sexual ambiguities are found, always with sterility owing to reduced testicular development. The origin of male phenotype in 46,XX male could be the results of at least three different mechanisms:translocations of Y sequence, including the SRY gene, to an X chromosome or to an autosome(about 90% of cases); a mutation in a yet unknown X-linked or autosomal gene in the testis-determinating pathway, and cryptic Y chromosome mosacism. We experienced a case of SRY-positive 46,XX male in a 21-year-old man with small testes. (J Korean Soc Pediatr Endocrinol 2003;8:184-188)
Adolescent ; Azoospermia ; Genes, sry ; Gynecomastia ; Humans ; Hyalin ; Hypogonadism ; Incidence ; Infant, Newborn ; Infertility ; Male* ; Phenotype ; Puberty ; Seminiferous Tubules ; Testis ; X Chromosome ; Y Chromosome ; Young Adult

46,XX male is a rare abnormality of sex determination with an incidence of 1 in 20,000 male neonates. The clinical manifestations of 46,XX males are usually hypogonadism, gynecomastia, azoospermia, and hyalinations of seminiferous tubules, with altered hormonal levels at puberty. Less frequently, some sexual ambiguities are found, always with sterility owing to reduced testicular development. The origin of male phenotype in 46,XX male could be the results of at least three different mechanisms:translocations of Y sequence, including the SRY gene, to an X chromosome or to an autosome(about 90% of cases); a mutation in a yet unknown X-linked or autosomal gene in the testis-determinating pathway, and cryptic Y chromosome mosacism. We experienced a case of SRY-positive 46,XX male in a 21-year-old man with small testes. (J Korean Soc Pediatr Endocrinol 2003;8:184-188)
Adolescent ; Azoospermia ; Genes, sry ; Gynecomastia ; Humans ; Hyalin ; Hypogonadism ; Incidence ; Infant, Newborn ; Infertility ; Male* ; Phenotype ; Puberty ; Seminiferous Tubules ; Testis ; X Chromosome ; Y Chromosome ; Young Adult

Hereditary angioedema is a rare genetic disease transmitted with a autosomal dominant trait result of a quantitative or functional defect of Cl inhibitor. We report three cases of heriditary angioedema from the same famly respectively the 35-year-old sister, the younger bn)ther of 32, and the younger sister of 20 The older sister died due to her airway being obstructel by acute laryngeal edema, and her you igirbrother and sister experienced recurrent episodes oswelling on the hand and forearm. The ore we examined the two patients serum complement levils to confirm diagnosis. In both, the r;ults showed decreased levels of Cl inhibitor and C4 which are the evidence of hereditary a giedema. The younger sister has been administered danazol (600mg/day) to prevent angioedem or 8 weeks, and we have not yet found a recurrenc of he symptoms. However, well cont ol the dosage of danazol because amenorchea has developec.
Adult ; Angioedema ; Angioedemas, Hereditary* ; Complement System Proteins ; Danazol ; Diagnosis ; Forearm ; Hand ; Humans ; Laryngeal Edema ; Siblings