No abstract available.

No abstract available.
Cholangitis, Sclerosing*

No abstract available.
Hemarthrosis* ; Knee*

Ganglioneruroblastoma and neuroblastoma are among commonest types of childhood malignancy and a number of unique paraneoplastic syndromes have associated with both localized and disseminated neuroblastoma. The coincidence of neuroblastoma and myoclonic encephalopathy or other paraneoplastic syndromes occurs relatively rare, and therefore, failure to recognize this association could result in delays in both diagnosis and treatment, and the result could prove to be unfortunately fatal. The mechanism which underlies the remote damaging effect of neural crest tumor, especially neuroblastoma, on the nervous system resulting in myoclonic encephalopathy is by no means clear. In addition the nature and the extent of the pathologic lesion are inconsistent. We experienced a case of myoclonic encephalopathy associated with an occult mediastinal ganglioneuroblastoma in a 22-month-old girl who was hospitalized for inability to walk without support and tilting of the head to the left side. She became increasingly ataxic, and during the hospitalization myoclonic jerks of upper extremities and head along with chaotic, rapidly flickering, multidirectional spontaneous eye movements, were noted. Laboratory data included normal complete blood count, urinalysis, BUN and creatinine, electrolytes and bone marrow. Chest X-ray and chest CT revealed a relatively well marginated right posterior mediastinal mass. In a 24 hours urine excretion test, VMA and catecholamines were increased. Over the next 2 weeks, a surgical exploration revealed a right posterior mediastinal mass. Microscopically the mass proved to be a ganglioneuroblastoma, extending to right innominate artery and right axillary lymph nodes. Within 2 weeks after the surgery, radiotherapy (2,400 rads) and chemotherapy (CTX, DTIC, VCR) were started, but corticosteroid was not used. She has been free of tumor and abnormal neurological systemic symptoms and signs for 1 1/2 year since the completion of chemotherapy. In the 3 1/2 years follow-up period, her neurologic symptoms has completely resolved by the completion of 2 years chemotherapy. We report a case of mycoclonic encephalopathy associated with hidden ganglioneuroblastoma in 22-month-old girl.
Blood Cell Count ; Bone Marrow ; Brachiocephalic Trunk ; Catecholamines ; Creatinine ; Dacarbazine ; Diagnosis ; Drug Therapy ; Electrolytes ; Epilepsies, Myoclonic* ; Eye Movements ; Female ; Follow-Up Studies ; Ganglioneuroblastoma* ; Head ; Hospitalization ; Humans ; Infant ; Lymph Nodes ; Myoclonus ; Nervous System ; Neural Crest ; Neuroblastoma ; Neurologic Manifestations ; Paraneoplastic Syndromes* ; Radiotherapy ; Thorax ; Tomography, X-Ray Computed ; Upper Extremity ; Urinalysis

OBJECTIVE: Adult onset Still s disease is an acute systemic inflammatory disorder. There are no pathognomonic symptoms or specific laboratory abnormalities. In recent reports, serum ferritin concentration is increased in active disease phase and decreased after defervescence. Our purpose was to determine the clinical significance of serum ferritin as an indicator for disease activity. METHODS: Seven patients who were diagnosed as adult onset Still s disease at Samsung Medical Center between October 1994 and March 1997, were reviewed. In these patients we checked leukocyte count, ESR, CRP and serum ferritin concentrations at the time of diagnosis and during follow-up periods and recorded febrile events during follow-up periods. RESULTS: At the time of diagnosis and during febrile periods, the concentrations of ferritin were extremely high(927ng/ml to 96,650ng/ml normal 10-290.8 ng/ml). The values were unrelated to other manifestations of the disease or laboratory findings. The ferritin concentrations decreased rapidly after adequate treatment. Eleven febrile reattacks happened in 7 patients. Serum ferritin concentrations were increased in 8 febrile attacks, while leukocyte count, ESR, and CRP were increased in 5, 5, 6 febrile attacks respectively, There were 10 events of increased serum ferritin concentrations in 7 patients during follow-up periods and 8 events were related with fever. The increases of other laboratory tests were similar. CONCLUSIONS: In all patients, serum ferritin concentrations were increased at the time of diagnosis and closely related to fever. During follow-up periods, serum ferritin concentrations are helpful in monitoring disease activity and guiding decisions about treatment.
Adult* ; Diagnosis ; Ferritins* ; Fever ; Follow-Up Studies ; Humans ; Leukocyte Count ; Still's Disease, Adult-Onset*

Vulva syringomas are rare in reviewing literature. In our case, a 29-year-old had multiple papular lesions involving both sides of the vulva. Microscopically the lesion showed dilated cystic sweat ducts some of which have small comma like tails resembling tadpoles. It is the purpose of this report to present a rare case of syringoma which was limited to the vulva without involvement of other sites.
Adult ; Humans ; Larva ; Sweat ; Syringoma* ; Vulva*

Lovastatin(Mevacor) belongs to a new generation of potent drugs for the treatment of hypercho- lesterolemia. It acts by competitive inhibition of 3-hydroxyl-3-methylglutaryl coenzyme A(HMG-CoA) reductase, diminishing in that way the cholesterol synthesis in the liver. HMG-CoA reductase inhibitors also affect cutaneous lipid content. This can induce cutaneous side effects such as eczernatous rashes or xerotic, ichthyosiform lesions due to abnormality in the barrier function in some patients. We report a 54-year-old male patient who developed ichthyosiform skin lesions under treatment with lovastatin(Mevacor) for hypercholesterolemia.
Cholesterol ; Exanthema ; Humans ; Hydroxymethylglutaryl-CoA Reductase Inhibitors ; Hypercholesterolemia ; Liver ; Lovastatin ; Male ; Middle Aged ; Oxidoreductases ; Skin*

No abstract available.
Arthrogryposis*

No abstract available.
Humans

PURPOSE: To define the natural history and treatment of osteofibrous dysplasia, we compared the clinical courses of two groups: one group with a surgical treatment and the other group with a conservative treatment. MATERIALS AND METHODS: Seven cases (4 male, 3 female) were followed for more than two years (average f/u: 7 years 2 months). Four cases conservatively and three cases operatively by curettage and bone graft. Were treated the clinical and radiological findings of the two groups were compared. RESULTS: Two of three cases that received surgical treatment had been diagnosed with lesion recurrence by radiography, which showed expansion of the cortical shell, resorption of grafted bone, and reactive bone formation. One other case exhibited shrinkage and confinement of the lesion to the anterior cortex. Two cases, which were treated conservatively, showed shrinkage of the lesion and an other two cases showed no change in the size in radiogram. CONCLUSION: Asymmetrical intra-cortical osteolytic lesion of the tibia in children should be suspected as osteofibrous dysplasia and carefully observed until skeletal maturity, unless the structural integrity is seriously endangered.
Child* ; Curettage ; Fibroma, Ossifying ; Humans ; Male ; Natural History ; Osteogenesis ; Radiography ; Recurrence ; Tibia* ; Transplants