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

Cryptococcosis is an acute, subacute or chronic infectian caused by the encap sulated yeast Cryptococcus neoformans. The case of Cryptococcosis is a 2 5/12 year-old boy with involvement of the skin, lungs, liver, spleen, lymph node and central nervous system is reported. He has suffered from fever, nausea., vomiting and multiple skin lesions for a month. Thcre were various cutaneous lesions consisting of papules, pustules, nodules, crusts and ulcers on the scalp, face, back, buttocks and both extremities. Diagnosis was confirmed by the clinical characteristics, histopathologic findings and culture of the C. neoformans from cerebrospinal fluid and marcerated skin tissue. He was successfully treated with combination of Amphotericin B and 5-fluorocytosine.
Amphotericin B ; Buttocks ; Central Nervous System ; Cerebrospinal Fluid ; Cryptococcosis* ; Cryptococcus neoformans ; Diagnosis ; Extremities ; Fever ; Flucytosine ; Humans ; Liver ; Lung ; Lymph Nodes ; Male ; Nausea ; Scalp ; Skin ; Spleen ; Ulcer ; Vomiting ; Yeasts

We report CT and MR findings in tow cases of primary malignant melanoma of the vagina, one arising from cervicovaginal junction mimicking squamous cell carcinoma of the cervix and the other one recurring at vagina after resection. Two cases of malignant melanoma had high-attenuation on CT and high signal intensity on T1-weighted MR images and enhanced well after gadopentetate dimeglumine administration.
Carcinoma, Squamous Cell ; Cervix Uteri ; Female ; Gadolinium DTPA ; Melanoma* ; Vagina*

We report CT and MR findings in tow cases of primary malignant melanoma of the vagina, one arising from cervicovaginal junction mimicking squamous cell carcinoma of the cervix and the other one recurring at vagina after resection. Two cases of malignant melanoma had high-attenuation on CT and high signal intensity on T1-weighted MR images and enhanced well after gadopentetate dimeglumine administration.
Carcinoma, Squamous Cell ; Cervix Uteri ; Female ; Gadolinium DTPA ; Melanoma* ; Vagina*

Histiocytic medullary reticulosis is a rare, uniformly fatal, acute disease of the reticuloendothelial system. The disease is associated with fever, malaise, weakness, weight loss, lymphadenopathy, hepatosplenomegaly, jaundice, and purpura. Common laboratory findings are severe anemia, leukopenia, and thrombocytopenia. A 49-year-old male patient is described who had multiple purple nodules and tumors in the skin. A biopsy taken from a skin lesion showed a diffuse proliferation of atypical histiocytes in the dermis and subcutaneous tissue.However, erythrophagocytosis of these atypical histiocytes was not seen.
Acute Disease ; Anemia ; Biopsy ; Dermis ; Fever ; Histiocytes ; Humans ; Jaundice ; Leukopenia ; Lymphatic Diseases ; Male ; Middle Aged ; Mononuclear Phagocyte System ; Purpura ; Skin ; Thrombocytopenia ; Weight Loss

Fentanyl-O2 anesthesia has gained wide popularity as an anesthetic technique for patients undergoing cardiac surgery because of its minimal cardiovascular effects and total amnesia for intraoperative events. But, some authors recently reported intraoperative awareness and the excessive cardiovascular response to surgical stimulation during high dose fentanyl-oxygen anesthesia far cardiac operation and suggested the necessity of supplementary anesthetic agent in addition to fentanyl to prevent the intraoperative awareness and maintain hemodynamic stability during the surgical procedure. A variety of supplementary drugs have been used in combination with the opioids in an effort to reduce the incidence of awareness, to control hypertension, and to attenuate the extent of postoperative respiratory depression. One of supplementary drugs, diazepam has little cardiovascular effects by itself, but causes significant depression of arterial blood pressure and cardiac output when given to patients who have received fentanyl or morphine. We measured the heart rate (HR), systolic blood pressure (SBP), diastolic blood pressure (DBP), mean arterial pressure (MAP), and rate pressure product (RPP) at induction, tracheal intubation, skin incision, and sternotomy time to evaluate the effects of the diazepam on reduction of the excessive cardiovascular response to anesthetic and surgical stimulation and observed the presence of the intraoperative awareness, under fentantyl-diazepam-O2 anesthesia for open heart surgery on 12 patients with cardiac disease. Anesthesia was induced with fentanyl 20 ug/kg + diazepam 0.1 mg/kg and maintained with continuous infusion of fentanyl (1.5 ug/kg/min. prior to sternotomy and 0.3 ug/kg/min. until the end of cardiopulmonary bypass) and diazepam 0.1 mg/kg was injected just before sternotomy and at the end of cardiopulmonary bypass. The patients required the total dose of fentanyl 76+13.1 ug/kg and diazepam 0.3 mg/kg for the entire operation. These measurements were compared with control data (before induction). The results were as followings: 1)During induction period (infusion of fentanyl 20 ug/kg with diazepam 0.1 mg/kg); HR, SBP, DBP, and MAP slightly decreased compared with control data, but there were not statistically significant. RPP decreased significantly from 15898+/-5099 torr. beatsmin. to 12371+/-2407 torr. beatsmin. and there was statistical significance (p<0.05). 2) During intubation and skin incision; HR, SBP, DBP, MAP and RPP revealed no significant change compared with control data. 3) During sternotomy; HR, SBP, DBP, MAP, and RPP slightly increased, but there were not statistically significant. 4) There was no patient who had the recall or awareness for intraoperative events. 5) Duration of controlled or assisted ventilatory support. postoperatively, was 216+/-36 min. These results suggest that fentanyl-diazepam-O2 anesthesia might be more useful than fentanyl-O2 anesthesia for prevention of intraoperative awareness and attenuation of excessive cardiovascular response during open heart surgery. But, continuous and careful monitoring for hemodynamic changes of patients will be needed necessarily to prevent the significant depression of arterial blood pressure and cardiac output throughout the entire operative procedures.
Amnesia ; Analgesics, Opioid ; Anesthesia* ; Anesthetics ; Arterial Pressure ; Benzodiazepines ; Blood Pressure ; Cardiac Output ; Cardiopulmonary Bypass ; Depression ; Diazepam* ; Fentanyl* ; Heart Diseases ; Heart Rate ; Heart* ; Hemodynamics ; Humans ; Hypertension ; Hypnotics and Sedatives ; Incidence ; Intraoperative Awareness ; Intubation ; Morphine ; Respiratory Insufficiency ; Skin ; Sternotomy ; Surgical Procedures, Operative ; Thoracic Surgery*

Purpose: To report a case of subacute methicillin-resistant coagulase-negative Staphylococcus hominis endophthalmitis after re-operation for penetrating keratoplasty in a patient who had a penetrating keratoplasty rejection.Case summary: A 74-year-old man with underlying diabetes and hypertension who underwent bilateral penetrating keratoplasty at another hospital underwent penetrating keratoplasty reoperation due to corneal transplant rejection in the right eye. He had a history of end-stage glaucoma in the right eye and the visual acuity was finger counting at 30 cm. About 25 days postoperatively, the corneal epithelial defect was first found. Treatment based on suspicion of herpes simplex virus infection did not result in improvement and endophthalmitis was suspected in the right eye about 35 days after surgery. Anterior chamber irrigation, pars planar vitrectomy, and intravitreal antibiotic injection were performed. Culture revealed methicillin-resistant coagulase negative Staphylococcus hominis. After 2 months of treatment, the anterior chamber and vitreous inflammation decreased and best corrected visual acuity in the right eye recovered to 0.06. Conclusions Subacute endophthalmitis caused by methicillin-resistant coagulase-negative Staphylococcus may occur after penetrating keratoplasty. Transplant failure due to infection can be avoided with vitrectomy and antibiotic treatment at the right time.

Supraaortic stenosis is a localized or diffuse congenital narrowing of the ascending aorta at the levels of superior margin of the sinus of Valsalva, just above coronary arteries. Hemodynamically, it is a usually manifestated as aortic stenosis especially when it combined with subaortic membrane. And there is no case report of supraaortic stenosis combined with subaortic membrane which has aortic valvular reurgitation due to secondary change of dilated sinus of Valsalva by supraaortic ridge. We have experienced a case of aortic regurgitation associated with supraaortic ridge combined with subvalvular aortic membrane.
Aorta ; Aortic Valve Insufficiency* ; Aortic Valve Stenosis ; Constriction, Pathologic ; Coronary Vessels ; Membranes* ; Sinus of Valsalva

PURPOSE: The reliability and validity of a Korean version of the Obsessive-Compulsive-Inventory-Revised (OCI-R) was examined in non-clinical student samples. MATERIALS AND METHODS: The Korean version of OCI-R was administered to a total of 228 Korean college students. The Maudsley Obsessive Compulsive Inventory (MOCI), Beck's Depression Inventory (BDI), and Beck's Anxiety Inventory (BAI) were administered to 228 students. RESULTS: The total and each of subscale of the Korean OCI-R demonstrated excellent internal consistency, good test-retest reliability, moderate convergent validity and good divergent validity. CONCLUSION: It was concluded that the Korean version of the OCI-R has strong psychometric properties as the original version.
Female ; Humans ; Korea ; Male ; Obsessive-Compulsive Disorder/*diagnosis ; *Psychological Tests/statistics & numerical data ; Psychometrics ; Questionnaires ; Reproducibility of Results ; Young Adult

PURPOSE: During the childhood, Mantoux test is very popular for detecting tuberculous infection and also useful for evaluating the immunity against tuberculosis after BCG vaccination. But the response and conversion rate is affected by the age of vaccination, the quality of vaccine, the duration after vaccination and the frequency of the test. Recently percutaneous multipuncture technique of BCG vaccination was introduced and used because of local side effect and technical problem of intradermal BCG vaccination, however there is only a study of conversion rate and side effect at 3month after multipuncture BCG vaccination. We performed this study to evaluate of the difference of the Mantoux reaction and conversion rate according to duration (3month, 9month and 15month) after vaccination, gestational age, sex, and feeding type. METHODS: 174 neonates (155 of full term infants, 19 of preterm infants) who were born Kangnam St. Mary's Hospital were given multipuncture BCG vaccination within 4weeks after birth between July, 1995 and June 1997. All subjects was performed Mantoux test using 5TU PPD (NIH, Korea) at 3month, 9month and 15month. Injecting BCG and interpretating the result of the Mantoux test was done by the same person. RESULTS: 1) The positive rates of Mantoux test which was done 3month , 9month and 15month after vaccination were 38.7%, 58.1%, 71.6% in the full term infant group and 31.6%, 31.6%, 47.4% in the preterm infant group. 2) The size of induration in Mantoux test, which was done 3month, 9month and 15month after vaccination were 7.3 mm, 9.4 mm, and 10.6 mm in the full term infant group and 5.6 mm, 7.7 mm, 9.2 mm in the preterm infant group. The size of induration was significantly lower in the preterm infant group as compared with that of the full term infant group 9month after vaccination. 3) The positive conversion rate, less than 5 mm to more than 10 mm, was 6.3% (3 infants of 48 infants who were negative Mantoux test 3month became positive 9month) and 26.3% (5 infants of 19 infants who were negative 9month became positive 15month) in the full term infant group, and 33.3% (one infant of 5 infants who were negative 9month became positive 15month). 4) There is no difference in conversion rate of Mantoux test according to the sex, feeding type in both groups5) Complication rate for multipuncture is 3.2%, which is leaded by local ulceration and fever in the full term infant group, and 5.3%, which is only fever in the preterm infant group. CONCLUSION: In those result the size of induration and conversion rate is increased as the duration after vaccination, then their smaller size in the preterm group 9month after vaccination maybe due to immunological immaturity of preterm group.
Fever ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Mycobacterium bovis* ; Parturition ; Tuberculosis ; Ulcer ; Vaccination*