Dieulafoy lesion is an uncommon cause of gastrointestinal bleeding, reported to be only 2% of acute or chronic upper gastrointestinal bleeding episodes. Bleeding occurs from a small mucosal erosion involving an unusually large submucosal artery in an otherwise normal mucosa. It is associated with massive, life threatening hemorrhage and is difficult to diagnosis. In most cases the lesion is encountered in the proximal stomach, antrum, duodenum, colon and rectum. In particular, extragastric Dieulafoy lesion is an extremely rare source of intestinal bleeding. In Korea, no case of bleeding from a Dieulafoy lesion of the small intestine has been previously reported. We experienced one case of bleeding from a jejunal Dieulafoy lesion, which was confirmed by the pathologic examination of the resected specimen, and report here.
Adult ; Arteries/abnormalities* ; Case Report ; Female ; Gastric Mucosa/blood supply* ; Gastrointestinal Hemorrhage/etiology* ; Human ; Intestinal Mucosa/blood supply*

Pelizaeus-Merzbacher disease (PMD) is an X-linked recessive disorder characterized by dysmyelination of the central nervous system (CNS) caused by mutations in the proteolipid protein (PLP) gene. PLP is located at Xq22 and its mutation result in abnormal expression or production of PLP, the most abundant protein in CNS myelin. We present a case of PMD in the 7-year-old boy with nystagmus, ataxia, spastic quadriplegia and severe psychomotor delay. His brain MRI revealed totally dysmyelinated white matter involving entire supratentorial region, atrophic change, and overaccumulation of the iron in both basal ganglia. He also showed soft-tissue contractures of the hip adductors, associated hip dislocations and equinovarus foot deformities due to severe spasticity of lower extremities. Orthopaedic surgery was performed on both hips. Antispastic medication and physical therapy were maintained for reduction of spasticity. We report this case with the review of literatures.
Ataxia ; Basal Ganglia ; Brain ; Central Nervous System ; Child ; Clubfoot ; Contracture ; Foot Deformities ; Hip ; Hip Dislocation ; Humans ; Iron ; Lower Extremity ; Magnetic Resonance Imaging ; Male ; Muscle Spasticity ; Myelin Sheath ; Pelizaeus-Merzbacher Disease* ; Quadriplegia

A 50 year old female presented unusual electrocardiographic changes including AV block, accelerated idioventricular rhythm, ventricular premature systole with severe fluctuation of blood pressure and clinical features of angina pectoris. Deep ST segment depression was demonstrated in spite of the normal coronary arteriogram and the negative coronary artery spasm study. Urinary excretion of catecholamines and their metabolites were elevated and a huge pheochromocytoma was found in the left adrenal glandd. After removal of the pheochromocytoma, the electrocardiographic abnormalities and the blood pressure were normalized and teh aptient became asymptomatic.
Accelerated Idioventricular Rhythm ; Angina Pectoris* ; Arrhythmias, Cardiac ; Atrioventricular Block ; Blood Pressure ; Catecholamines ; Coronary Vessels ; Depression ; Electrocardiography* ; Female ; Humans ; Middle Aged ; Pheochromocytoma* ; Spasm ; Systole