No abstract available.

Osteopetrosis or albers-Schonberg disease is extremely rare disease. It is characterized by osteoporosis, stunted growth, deformity, increased likelihood of fractures, also patients suffers anemia, recurrent infections and hepatosplenomegaly. However, we recently came upon a 14-month-old female as the 1st child of osteopetrosis with hydrocephalus and rickets. She has the typical symptoms such as nystagmus, osteosclerosis -especially in skull. Brain Magnetic Resonance Imaging (MRI), MRI shows hydrocephalus and x-ray finding are consistent with rickets. This is the first report of osteopetrosis with hydrocephalus and rickets in Korea by pediatrician.
Anemia ; Brain ; Child ; Congenital Abnormalities ; Female ; Humans ; Hydrocephalus* ; Infant ; Korea ; Magnetic Resonance Imaging ; Osteopetrosis* ; Osteoporosis ; Osteosclerosis ; Rare Diseases ; Rickets* ; Skull

Although liver metastasis is commonly found in cancer patients, fulminant hepatic failure secondary to diffuse cancer infiltration into the liver is rare. Liver metastasis-induced fulminant hepatic failure has been reported in patients with primary cancer of the gastrointestinal tract, breast and uroepithelium, and in patients with melanoma and hematologic malignancy. Small cell lung cancer is so highly invasive that hepatic metastasis is common, but rapid progression to fulminant hepatic failure is extremely rare. We report here on a case of a patient who died because of rapid progression to fulminant hepatic failure as a result of hepatic metastasis of small cell lung carcinoma.
Aged ; Carcinoma, Small Cell/complications/pathology/*secondary ; Female ; Humans ; Liver Failure, Acute/diagnosis/*etiology ; Liver Neoplasms/complications/pathology/*secondary ; Lung Neoplasms/complications/*pathology ; Neoplasm Invasiveness ; Tomography, X-Ray Computed

The ovarian hyperstimulation syndrome is a rare but serious complication of ovulation induction therapy with gonadotropin. The clinical manifestations are generalized edema, ascites with pleural effusion and may become life-threatening in severe cases. The pathophysiology is still unknown therefore, the treatment should be symptomatic and conservative. We report a case of severe OHSS with massive right pleural effusion in excess of ten liters after human menopausal gonadotropin therapy because of secondary infertility. Fluid and electrolyte imbalances were corrected and albumin was administered. A right chest tube was placed for a total of sixteen days, draining eleven liters of pleural effusion totally, resulting a dramatic decrease of pleural effusion and improvement of symptoms.
Ascites ; Chest Tubes ; Edema ; Female ; Gonadotropins ; Humans ; Infertility ; Ovarian Hyperstimulation Syndrome* ; Ovulation Induction ; Pleural Effusion*

BACKGROUND: The administration of adjuncts to local anesthetics in brachial plexus block affects the onset of sensory or motor block and the duration of analgesia. But, the effect of opioid with ropivacaine is controversial, and epinephrine addition does not significantly affect the duration of analgesia. So, we evaluated the effects of fentanyl or epinephrine added to ropivacaine for brachial plexus block. METHODS: Forty-five patients scheduled for upper extremity surgery (ASA 1, 2) were randomly divided into 3 groups: Group 1 received 0.5% ropivacaine 52 ml, Group 2 received 0.5% ropivacaine 52 ml with 1:200,000 epinephrine, and Group 3 received 0.5% ropivacaine 50 ml with fentanyl 100microgram. At 1-min intervals after injecting local anesthetic, sensory block was assessed in the C5-6 dermatome by pinprick testing and motor block was assessed at the shoulder by asking the patient to elevate the affected arm while keeping the elbow straight. Duration of analgesia (the time between injection and the onset of pain) and the duration of motor block (the time between injection and the restoration of shoulder mobility) were recorded. RESULTS: No significant difference was observed in the onset of sensory or motor block, or in the duration of analgesia or motor block between the three groups. CONCLUSIONS: The addition of fentanyl or epinephrine to ropivacaine in brachial plexus block does not affect the onset of sensory or motor block, or the duration of analgesia or motor block.
Analgesia ; Anesthetics, Local ; Arm ; Brachial Plexus* ; Elbow ; Epinephrine* ; Fentanyl* ; Humans ; Shoulder ; Upper Extremity

Ninety two strains of Streptococcus pyogenes were isolated from patients with pharyngitis, scarlet fever, skin infection, and invasive streptococcal infections in Seoul, Korea from January to December, 1998. All isolates were epidemiologically characterized by T protein serotype, and serum opacity factor (OF) detection to phenotypes. To analyze the genetic relationship, fifty two isolates including 32 erythromycin-clindamycin (Em-Cm) resistant strains, 20 antimicrobial susceptible strains were attempted to the pulsed-field gel electrophoresis (PFGE). T protein serotype showed 16 kinds in distribution including T12 and T4. Among the total isolates, 40 strains (43.5%) belonged to the T12 serotype and twenty strains (21.7%) to T4 serotype. On the other hand, when infection aspect of S. pyogenes isolates were analysed by T serotype distribution, T12 type was predominant for pharyngitidis which contributed to 21 strains (53%) and for skin infection isolates which contributed to 11 strains (28%), respectively. In case of T4 type, it was the most predominant pharyngitidis isolates which contributed to 8 strains (40%). In T serotype distribution of Em-Cm resistant strains, 27 strains (84%) of the thirty two showed T12 serotype. In minimum inhibitory concentration (MIC) values of Em-Cm resistance isolates, thirty two isolates showed resistant to erythromycin 27 strains (84%), had high MIC of >128 mug/ml. And also to clindamycin, twenty two strains (69%) had high MIC of >128 mug/ml. When OF detection of Em-Cm resistance of S. pyogenes isolates were analyzed by T serotype distribution, T12 serotype isolates revealed that all of the isolates except one strain were OF negative. In PFGE profile analysis to Em-Cm resistance isolates, of the twenty seven, Em-Cm resistance of T12 serotype isolates, 26 strains showed identical PFGE profile and all of these isolates revealed that OF negative. Eighty four percent of Em-Cm resistance S. pyogenes isolates had identical phenotype and PFGE profile. These results strongly suggested that the Em-Cm resistant S. pyogenes isolates from Seoul area showed close genetic correlation and PFGE could be available tool for molecular epidemiology.
Clindamycin ; Electrophoresis, Gel, Pulsed-Field* ; Erythromycin ; Hand ; Humans ; Korea* ; Microbial Sensitivity Tests ; Molecular Epidemiology ; Pharyngitis ; Phenotype ; Scarlet Fever ; Seoul ; Skin ; Streptococcal Infections ; Streptococcus pyogenes* ; Streptococcus*

Desmoplastic trichoepithelioma is a rare benign adnexal tumor that usually presents as an asymptomatic, firm, annular plaque. It most commonly occurs on sun-exposed areas in middle-aged women, especially on the face. Patients typically give a history of a single, slow-growing lesion that has been present for several years. The diagnosis of desmoplastic trichoepithelioma can be made when a triad of microscopic findings are observed in combination with a compatible clinical picture. We herein report a case of desmoplastic trichoepithelioma associated with syringomas on the face of a young woman.
Diagnosis ; Female ; Humans ; Syringoma*

A twenty six months-old boy developed hemophagocytic syndrome during the course of Kawasaki disease. Despite the appropriate treatment modalities for Kawasaki disease, he developed thrombocytopenia, hepatomegaly, high-grade fever, hypertriglyceridemia, peripheral gangrene, and evidence of hemophagocytosis in bone marrow biopsy. Although the course was stormy, he responded well to a combination therapy of corticosteroid and etoposide.
Biopsy ; Bone Marrow ; Etoposide ; Fever ; Gangrene* ; Hepatomegaly ; Humans ; Hypertriglyceridemia ; Lymphohistiocytosis, Hemophagocytic* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Thrombocytopenia

A biloma is an extrahepatic or intrahepatic bile collection caused by traumatic, iatrogenic, or spontaneous rupture of the biliary tree. Prior reports have documented an association of a biloma with abdominal trauma and surgery, but spontaneous bile leakage associated with other primary causes has rarely been reported. A 72-year-old man was admitted to our hospital with the complaint of epigastric pain and yellowish discoloration of the sclera. Ultrasonography and computed tomography revealed a large fluid collection in the abdominal cavity. Endoscopic retrograde cholangiography demonstrated leakage of contrast medium from a distended segmental biliary branch in the left lobe of the liver. A perihepatic biloma was confirmed by sonographically guided percutaneous aspiration, and the patient underwent a left lateral segmentectomy of the liver, a cholecystectomy and T-tube choledochostomy. Histological examination showed left lateral bile duct hyperplasia, with abscess formation and chronic cholecystitis. Herein, a case of a biloma associated with choledocholithiasis is reported, with a review of the literatures.
Abdominal Cavity ; Abscess ; Aged ; Bile ; Bile Ducts ; Bile Ducts, Intrahepatic* ; Biliary Tract ; Cholangiography ; Cholecystectomy ; Cholecystitis ; Choledocholithiasis* ; Choledochostomy ; Humans ; Hyperplasia ; Liver ; Mastectomy, Segmental ; Rupture* ; Rupture, Spontaneous ; Sclera ; Ultrasonography

Paroxysmal kinesigenic dyskinesia (PKD), previously referred to as movement-provoked seizures, is a rare neurological condition that is characterized by short duration dystonic or choreoathetotic movements precipitated by sudden movement, a change in position or hyperventilation. It can be difficult to distinguish this syndrome from seizures. We reported on three brothers in one family all of whom developed abnormal involuntary dystonic or choreoathetotic movement with a tingling or stiffness sensory aura. Evaluations of the patients included general physical examinations, endoclinologic, metabolic studies, chromosomal analysis, video electroencephalograms and brain MRI imaging. All of these studies were normal except for an arachnoid cyst found in one patient. All symptoms showed excellent response to oxcarbamazepine (Trileptal(R)) or carbamazepine. Use of the video electroencephalogram can help differentiate familial PKD from seizures.
Arachnoid ; Brain ; Carbamazepine ; Dyskinesias* ; Electroencephalography ; Epilepsy ; Humans ; Hyperventilation ; Magnetic Resonance Imaging ; Physical Examination ; Seizures ; Siblings*