No abstract available.
Neurofibroma*

Kawasaki disease is an acute febrile illness frequently developed in infants and children. This disease may involve coronary arteries in 15- 25% of the patients and may progress to coronary aneurysms, ischemic cardiac diseases, and sudden cardiac death. Recently we experienced successful balloon angioplasty followed by coronary stenting in a 15-year old boy with unstable angina and severe coronary arterial occlusive disease secondary to Kawasaki disease. He was diagnosed as unstable angina by 24 hours Holter monitoring, treadmill exercise stress test, echocardiography, and Dipyridamole 99mTc-sestamibi scan. And coronary angiogram revealed severe multiple stenosis and aneurysmal changes due to Kawasaki disease. We successfully performed a percutaneous transluminal coronary angioplasty with stent implantation at left circumflex arterial occlusive lesion.
Adolescent ; Aneurysm ; Angina, Unstable ; Angioplasty, Balloon ; Angioplasty, Balloon, Coronary ; Arterial Occlusive Diseases ; Child ; Constriction, Pathologic ; Coronary Aneurysm ; Coronary Stenosis* ; Coronary Vessels* ; Death, Sudden, Cardiac ; Dipyridamole ; Echocardiography ; Electrocardiography, Ambulatory ; Exercise Test ; Heart Diseases ; Humans ; Infant ; Male* ; Mucocutaneous Lymph Node Syndrome* ; Stents* ; Technetium Tc 99m Sestamibi

No abstract available.
Progeria*

A 12-year-old boy developed cholestatic hepatitis with Steven-Johnson syndrome following the use of amoxicillin. The skin lesion and general condition were improved over 2 weeks, but jaundice was gradually aggrevated. We performed liver biopsy, on 30th hospital day, which showed cholestatic hepatitis. The patient improved gradually and liver function was normalized 5 months later.
Amoxicillin ; Biopsy ; Child ; Hepatitis* ; Humans ; Jaundice ; Liver ; Male ; Skin

No abstract available.
Friction* ; Hyperpigmentation* ; Skin Diseases*

Lichen myxedematosus (synonym, papular mucinosis) is a disorder characterized by lichenoid papules, nodules and plaques due to dermal mucin deposition, and a variable degree of fibrosis without thyroid dysfunction. Discrete lichen myxedematosus is a subtype of the localized lichen myxedematosus. Clinically, firm, smooth, waxy or flesh-colored papules measuring 2 to 5 mm in diameter, numbering just a few to hundreds, and involving limbs and trunk. The lesions progress slowly without systemic symptoms and rarely resolve spontaneously. Histologically, the upper and mid dermis shows edema and diffuse or focal mucinous deposit under normal epidermis. Fibroblast proliferation is variable, but there is neither collagen deposition nor sclerosis. On experiencing a case of discrete grouped papules on the arm and thigh of a sixty three year-old women, we present it as discrete lichen myxedematosus.
Arm ; Collagen ; Dermis ; Edema ; Epidermis ; Extremities ; Female ; Fibroblasts ; Fibrosis ; Humans ; Lichens* ; Mucins ; Scleromyxedema* ; Sclerosis ; Thigh ; Thyroid Gland

Hereditary motor and sensory neuropathy is an unusual disease which is characterized by deformity of phe foot, acral sensory loss, decreare of deep tendon reflexes, enlargement of peripheral nerve and diminished motor conduction velocity. We report a case of hereditary motor and sensory neuropathy type I.
Congenital Abnormalities ; Foot ; Hereditary Sensory and Motor Neuropathy ; Peripheral Nerves ; Reflex, Stretch

The hypokalemic periodic paralysis is characterized by intermittent falccid paralysis of extremities with spontaneous recovery. It is rarely accompanied by cardiac arrhythmia, especially fatal ventricular tachycardia or torsades de pointes. We observed a 29 year old man, who had suffered from intermittent periodic paralysis and fatal ventricular tachyarrhythmia. He had the first episode of muscle weakness in his low grade of elementary school, which lasted for 20 -30 hours. Similar episodes of muscle weakness occurred 1 -7 times per year, especially after carbohydrate rich food. On admission to emergency room, his chief complaints were generalized weakness and chest tightness, serum potassium level was 1.6mEq/l, and four extremities showed Grade 0 motor weakness. His electrocardiography(ECG) showed Atrioventricular dissociation due to sinus tachycardia and accelerated junctional rhythm, intraventricular conduction distrubance. During intravenous potassium administration, ECG showed sustained ventricular tachycardia and cardiovascular collapse occurred. So we carried out resuscitation and cardioversion. After resuscitation, he recovered from cardovascular collapse and ECG showed sinus tachycardia. But during continuous monitoring ECG showed torsades de pointes with cardiovascular collapse. We carried out resuscitation and defibrillation repeatedly. Serum potassium level was 1.7 - 1.8mEq/L at that time. After successful resuscitation, ECG showed sinus rhythm, and his mental status was fully recovered. After he admitted to intensive care unit, paralytic attack and cardiac arrhythmia did not occurred any more. Serum potassium level was maintained between 3.9 -6.1lmEq/L during his hospital days. He was fully recovered but could not take any medications(e.g. acetazolamide, potassium supplying agent and antiarrhythmic drugs) due to severe gastrointestinal disturbances. During the 30 months of postdischarge period, he experienced three mild paralysis attacks, but they were not accompanied by chest tightness, palpitation or syncope.
Acetazolamide ; Adult ; Arrhythmias, Cardiac* ; Electric Countershock ; Electrocardiography ; Emergency Service, Hospital ; Extremities ; Heart Block ; Humans ; Hypokalemia ; Hypokalemic Periodic Paralysis* ; Intensive Care Units ; Muscle Weakness ; Paralysis ; Potassium ; Resuscitation ; Syncope ; Tachycardia ; Tachycardia, Sinus ; Tachycardia, Ventricular ; Thorax ; Torsades de Pointes

Intramural hematoma(IMH) and penetrating aortic ulcer have been increasingly recognized as causes of acute aortic pathology in addition to aortic dissection. The presence of the intimal tear and a flap traversing the aortic lumen is considered to be a most reliable differential point of aortic dissection and IMH. Transesophageal echocardiography(TEE) has become a valuable modality for the diagnosis, prognosis and management of acute aortic syndrome with the unique advantages of portability and the ability to obtain high-resolution real time images. Endovascular Stent-graft placement over the primary entry tear may be an alternative to open surgery because it can close the intimal tear, which leads to thrombosis of the false lumen, excluding flow through the intimal tear and redirecting aortic flow exclusively into the true lumen. We report 88 year-old male with aortic dissection in descending thoracic aorta, successfully treated with endovascular Stent-graft implantation, which was mimicking intramural hematoma by its appearance and subclinical intimal tear diagnosed exclusively by TEE against other imaging studies.
Aged, 80 and over ; Aorta, Thoracic* ; Diagnosis ; Hematoma* ; Humans ; Male ; Pathology ; Prognosis ; Thrombosis ; Ulcer

No abstract available.
Bacteriology* ; Biliary Tract*