PURPOSE: To obtain basic data on cyclic vomiting syndrome(CVS) and clinical findings of CVS. METHODS: Fifteen patients diagnosed as CVS from April 1995 to July 1999 were enrolled in this study. RESULTS: Among 15 children with CVS(11 boys, 4 girls), 4 patients were put in the migraine group and 11 patients in non-migraine group. The onset age ranged from 7 months to 11.4 years(mean: 5.5 years). The prodromal symptoms of the migraine group were headache, flank pain and unpleasant feeling, and those of the non-migraine group were abdominal pain and dizziness. Vomiting began at characteristic times specific for each individual in 73% of patients; in the migraine group, 75% had syrnptoms on arising in the morning. In the non-rnigraine group, 36% were wakened during the night and 27% had symptoms on arising in the morning and 9% had episodes beginning at other characteristic tirnes and the rernaining 27% at no characteristic times. Durations of episodes were of fairly uniform length and mostly between 2 days and 10 days. In regards to precipitating factors, stress was the most frequently mentioned and other reported precipitants included infections, and sensitivity to food and hot or cold weather. Continuous vomiting causes ketosis, electrolyte irnbalance, poor growth, peptic esophagitis and other cornplications, including learning problems and psychosocial dysfunction. Both groups had positive response to prokinetic agents. CONCLUSION: There was no clinical differences between the migraine group and non-migraine group. The response to therapy was poor, but the response to prokinetic agents was better than that to anti-migraine agents.
Abdominal Pain ; Age of Onset ; Child ; Dizziness ; Esophagitis, Peptic ; Flank Pain ; Headache ; Humans ; Ketosis ; Learning ; Migraine Disorders ; Precipitating Factors ; Prodromal Symptoms ; Vomiting* ; Weather

Wilson s disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson s disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilson s disease 3 weeks after initiation of D-penicillamine.
Brain ; Child, Preschool ; Cornea ; Female ; Glomerulonephritis, Membranous ; Humans ; Liver ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Penicillamine* ; Trientine ; Zinc Sulfate

BACKGROUND: Bronchoscopy is an essential procedure for identifying the bleeding site and evaluating cause of hemoptysis. However, it is controversial regarding to the timing of bronchoscopy in patients with hemoptysis. Early bronchoscopy, which was performed during hemoptysis or with 48hour after cessation of bleeding, was better for identifying the site of bleeding compared with delayed bronchoscopy, which was performed 48 hours after cessation of bleeding. The diagnostic yield of identifying the bleeding site by bronchoscopy was variable in reported literature and the safety of early bronchoscopy was not mentioned in previous literature. Therefore, we evaluated the efficacy and safety of early bronchoscopy in patients with hemoptysis. METHOD: From October 1994 to August 1996 in Samsung Medical Center, bronchoscopy was performed in patients with hemoptysis. Early bronchoscopy was performed prospectively during hemoptysis or within 48 hours after cessation of bleeding from May 1995 to August 1996. Delayed bronchoscopy group included patients who did not recieved early bronchoscopy at the same period or in whom bronchoscopy was performed 48 hour after cessation of bleeding from October 1994 to May 1995. RESULTS: Early bronchoscopy group was performed 73 times in 71 patients. Delayed bronchoscopy was performed in 57 times in 55 patients. There was no difference as to amount and underlying cause of hemoptysis between both groups. Indentification of bleeding site by visualizing active bleeding was significantly higher in early bronchoscopy(38.3%) than delayed bronchoscopy group (8.7%) (p<0.05). Indentification of bleeding site by bleeding after clot removal was 8 in early and 10 in delayed bronchoscopy. Indentification of bleeding site by visualizing active bleeding and bleeding after clot removal was 36 in early and 15 patients in delayed bronchoscopy(p>0.05). Causes of hemoptysis was found in 18 patients in early and 16 patients in delayed bronchoscopy group. patients who had early bronchoscopy underwent surgery. We diagnosed the site of bleeding in 4 patients preoperatively. In 3 patients we made a treatment plan promptly right after bronchoscopy. Among early bronchoscopy group, bleeding over 100cc during bronchoscopy occurred in 2 patients. In early bronchoscopy group there was no other major complication during bronchoscopy. CONCLUSION: In patients with hemoptysis, early bronchoscopy which performed within 48 hours after cessation of bleeding was more effective procedure for indentifying the bleeding site than delayed bronchoscopy which was performed after 48 hour cessation of bleeding.
Bronchoscopy* ; Hemoptysis* ; Hemorrhage ; Humans ; Prospective Studies

Enterovirus 71(EV71), like polioviruses, invades the central nervous system to give rise to aseptic meningitis, encephalitis or myelitis. EV71 was first isolated in California in 1969 from a 9-month- old infant with encephalitis. Since then it has been isolated from the brain of children who died of encephalitis and from feces of patients with meningitis, encephalitis or paralysis. Related strains have been isolated from outbreaks of similar diseases in Australia, Sweden, Bulgaria and Hungary. We have experienced polio-like encephalomyelitis in a 3-month-old girl. Initial brain MR imaging showed tissue destruction in the bilateral posterior portions of the medulla oblongata and the bilateral anterior horns of cervical spinal cord from C3 to C6 level. Follow-up MR imaging was performed 3 months later, which showed minimal residual change on the anterior horn of the cervical spinal cord at C4 level only. This report deals with rare polio-like encephalomyelitis associated with EV71 and discusses its diagnosis and management. Brain stem and cervical spinal cord involvement are characteristic findings of EV encephalomyelitis.
Animals ; Australia ; Brain ; Brain Stem ; Bulgaria ; California ; Central Nervous System ; Child ; Diagnosis ; Disease Outbreaks ; Encephalitis ; Encephalomyelitis* ; Enterovirus* ; Feces ; Female ; Follow-Up Studies ; Horns ; Humans ; Hungary ; Infant ; Magnetic Resonance Imaging ; Medulla Oblongata ; Meningitis ; Meningitis, Aseptic ; Myelitis ; Paralysis ; Poliovirus ; Spinal Cord ; Sweden

OBJECTIVES: Although bronchoscopy is an important diagnostic tool for lung disease, patients compliance is low due to discomfort. Recently, midazolam which has a favorable anterograde amnesia effect and short action duration, has been used to relieve patients discomfort during bronchoscopy. Midazolam was investigated in order to see the beneficial effect and safety during bronchoscopy. METHODS: The study design was single blind, randomized, prospective. 102 patients were included, in whom bronchoscopy was performed between June, 19% and October, 1995 at Samsung Medical Center. They were categorized into midazolam group and control group. Patients were asked about the amnesic effect, discomfort of procedure and the willingness to repeat procedure. The consciousness level of patients during procedure, patient cooperation during procedure and ease of procedure were also reported by bronchoscopists. RESULTS: 1) The difference of oxygen saturation between two groups: There was no significant difference in oxygen saturation between midazolam group and control group before and after bronchoscopy. During procedure, however, mean oxygen saturations in midazolam group (90+/-6.4%) was significantly lower than in control group (93+/-4.7%)(p<0.05). 2) Evaluations by patients (1) Effect of amnesia: 41 patients (82%) in midazolam group could not recall the procedure but 52 patients (100%) recalled the entire procedure in control group. A favorable amnesic effects could be found in midazolam group(p<0.05). {2) The discomfort during the procedure: 43 patents(86%) did not experience discomfort from procedure in midazolam group but 25 patients(48%) complained of discomfort in control group (p<0.05). (3) Most patients except two(96%) were willing to repeat fiberoptic bronchoscopy in midazolam group but 13 patients (25%) answered that they would never repeat bronchoscapy. There was a statistically significant difference between two groups in the willingness to repeat bronchocopy (p<0.05). 3) The evaluations by bronchoscopists Cooperations of the patients and ease of procedure were not different between two groups. The patients in midazolam group except eight could not respond to verbal stimuli but most patients were awakened during procedure in control group(p<0.05). CONCLUSION: Midazolam is a good sedative agent for a patient to give a favorable amnesia, reduction of discomfort during bronchoscopy. We concluded that midazolam is a safe and useful sedative agent and midazolam may be used routinely during bronchoscopy. Monitoring of oxygen saturation, however, is essential to prevent severe hypoxia during procedure.
Amnesia ; Amnesia, Anterograde ; Anoxia ; Bronchoscopy* ; Compliance ; Consciousness ; Humans ; Lung Diseases ; Midazolam* ; Oxygen ; Patient Compliance ; Prospective Studies*

Smith-Lemli-Opitz syndrome (SLOS) is a rare, autosomal recessive disease caused by an inborn error in cholesterol synthesis. Patients with this disease suffer from multiple malformations due to reduced activity of 7-dehydrocholesterol reductase (DHCR7), which increases 7-dehydrocholesterol (7DHC) and 8-dehydrocholesterol (8DHC) concentrations and decreases cholesterol concentration in body fluids and tissue. The SLOS phenotypic spectrum ranges from a mild disorder with behavioral and learning problems to a lethal disease characterized by multiple malformations. Here, we describe a newborn male with ambiguous genitalia who was diagnosed to have type II SLOS during the neonatal period. A clinical examination revealed low levels of unconjugated estriol in the maternal serum, and a variety of fetal ultrasound anomalies, including prenatal growth retardation. After birth, the infant was diagnosed to have congenital heart disease (Tetralogy of Fallot with severe pulmonary artery stenosis), cleft lip and palate, micrognathia, postaxial polydactyly, ambiguous genitalia, and cataracts. Clinical investigation revealed extremely low plasma cholesterol levels and the presence of mutation (homozygote of p.Arg352Gln) in the DHCR7 gene. The patient underwent palliative heart surgery (to widen the pulmonary artery) and received intravenous lipid supplementation. Cholesterol levels increased slightly, but not to normal values. The patient died from cardiopulmonary failure and sepsis 72 days after birth. This report provides the first description of a Korean patient with SLOS confirmed by verification of DHCR7 gene mutation and illustrates the need for early recognition and appropriate diagnosis of this disease.
Body Fluids ; Cataract ; Cholestadienols ; Cholesterol ; Cleft Lip ; Dehydrocholesterols ; Disorders of Sex Development ; Estriol ; Heart Diseases ; Humans ; Infant ; Infant, Newborn ; Learning ; Male ; Oxidoreductases ; Oxidoreductases Acting on CH-CH Group Donors ; Palate ; Parturition ; Plasma ; Polydactyly ; Pulmonary Artery ; Reference Values ; Sepsis ; Smith-Lemli-Opitz Syndrome ; Thoracic Surgery

Cartilaginous choristoma of bony external auditory canal is very rare. Appearing as a horny sha-ped hard mass, cartilaginous choristoma can be found in the medial portion of anterior wall of external auditory canal. Histologically, it consists of adult-type hyaline cartilage without neoplastic features. If cartilaginous choristoma shows growing tendency or ear symptoms, surgical excision would be the treatment of choice. Here, we report a case of cartilaginous choristoma that occurred in the external auditory canal in an 8-year-old boy.
Cartilage ; Choristoma ; Ear ; Ear Canal ; Hyaline Cartilage

Background: The goal of this study was to confirm the relationship between subjective body shape perception and the depression screening tool Patient Health Questionnaire-9 (PHQ-9) in the geriatric population. Methods: A total of 1,251 subjects were analyzed among adults aged 65 or older who performed the 2020 Korean National Health and Nutrition Examination Survey. The subjective body shape perception was divided into three groups (thin, normal, and obese). Depression screening tool PHQ-9 10 points were employed as a cut-off value. The association between subjective body shape perception and the risk of depression based on the PHQ-9 was first verified by achi-square test, and then logistic regression analysis was performed along with items expected to influence the relationship. Results: There was a considerable difference between the depression risk and the normal groups in subjective body shape perception, which was classified as thin, normal, and obese. In the final model, the group who thought they were thin had an increased risk of depression compared with the normal group. Dyslipidemia, being female, and not owning a home have all been demonstrated to greatly raise the risk of depression. Conclusion We verified that subjective body shape was related to the risk of depression through the PHQ-9.

BACKGROUND: Bronchioloalveolar carcinoma (BAC) has been reported to diveres spectrum of chinical presentations and radiologic patterns. The three representative radiologic patterns are followings; 1) a solitary nodule or mass, 2) a localized consolidation, and 3) multicentric or diffuse disease. While, the localized consolidation and solitary nodular patterns has favorable prognosis, the multicentric of diffuse pattern has worse prognosis regardless of treatment BAC presenting as a solitary pulmonary nodule is often misdiagnosed as other benign disease such as tuberculoma. Therefore it is very important to make proper diagnosis of BAC with solitary nodular pattern, since this pattern of BAC is usually curable with a surgical resection. METHODS: We reviewed the clinical and radiologic features of patients with pathologically-proven BAC with solitary nodular pattern from January 1995 to September 1996 at Samsung Medical Center. RESULTS: Total 11 patients were identified. 6 were men and 5 were women. Age ranged from 37 to 69. Median age was 60. Most patients with BAC with solitary nodular pattern were asymptomatic and were detected by incidental radiologic abnormality. The chest radiograph showed poorly defined opacity or nodule and computed tomography showed consolidation, ground glass appearance, internal bubble-like lucencies, air bronchogram, open bronchus sign, spiculated margin or pleural tag in most patients. The initial diagnosis on chest X-ray were pulmonary tuberculosis in 4 patients, benign nodule in 2 patients and malignant nodule in 5 patients. The FDG-positron emission tomogram was performed in eight patients. The FDG-PET revealed suggestive findings of malignancy in only 3 patients. The pathologic diagnosis was obtained by transbronchial lung biopsy in 1 patient, by CT guided percutaneous needle aspiration in 2 patients, and by lung biopsy via video-assited thoracocopy in 8 patients. Lobectomy was performed in all patients and postoperative pathologic staging were T1N0M0 in 8 patients and T2N0M0 in 3 patients. CONCLUSION: Patients of BAC presenting with solitary nodular pattern were most often asymptomatic and incidentally detected by radiologic abnormality. The chest X-ray showed poorly defined nodule or opacity and these findings were often regarded as benign lesion. If poorly nodule or opacity does not disappear on follow up chest X-ray, computed tomography should be performed. If consolidation, ground glass appearance, open bronchus sign, air bronchogram, internal bubble like lucency, pleural tag or spiculated margin are found on computed tomography, further diagnostic procedures, including open thoracotomy, should be performed to exclude the possiblity of BAC with solitary nodular pattern.
Adenocarcinoma, Bronchiolo-Alveolar* ; Biopsy ; Bronchi ; Diagnosis ; Female ; Follow-Up Studies ; Glass ; Humans ; Lung ; Male ; Needles ; Prognosis ; Radiography, Thoracic ; Solitary Pulmonary Nodule* ; Thoracotomy ; Thorax ; Tuberculoma ; Tuberculosis, Pulmonary

OBJECTIVES: The study was designed to evaluate the role of the 5-HT2 and dopanmine D2 antagonist on spontaneous alternation behaviour which is an animal model of obsessive compulsive disorder in rat. On the basis of serotonin-dopamine interaction hypothesis, the effect of clozapine was evaluated by applying the suppressed spontaneous alternation behaviour model. METHODS: The apparatus for spontaneous alternation behaviour was a black plexiglas T-maze with distinctive black and white goal boxes. Black guillotine doors separated the start box and the goal boxes from the main body of the T-maze. Small cups of chocolate milk were placed in the corners of both goal boxes. At 24 hours prior to experiment, rats(spraque-Dawley) were food-deprived. The food-deprived rate were put into T-maze, in which both goal during which it was placed in the start box and allowed to choose one of the goal boxes for each time. The mean number of choices until the occurrence of spontaneous altemation behaviour were checked. After baseline of the number of choices of spontaneous altemation behaviour was stabilized, the spontaneous altemation was disrupted by nonselective 5-HT agonist, 5-MeODMT(1.25mg/kg/IP). The experimental animals were stratified nito 5 groups : clomipramine(5mg/kg/IP), clozapine(10mg/kg/IP), clozapine(20mg/kg/IP), haloperidol(0.1mg/kg/IP), and saline(0.2cc/IP) control groups. They all went through 21 days fo treatment(intraperitoneal). The protective effects against the 5-McODMT-induced disruption of spontaneous alternation behaviour were evaluated on the next day of drug treatment in each group. RESULTS: 1) SAB was supressed by 5-McODMT injection. 2) After 21 days of the drug treatment, the clozapine and the clomipramine groups showed significant difference from the haloperidol and the saline control groups in the reversal of 5-McODMT-induced from the haloperidol and the saline control groups in the reversal of 5-MeODMT-induced suppression of spontaneous altermation behaviour. 3) The clozapine(20mg/kg/IP) group was superior to the clomipramine group in the protective effect of 5-MeODMT-induced suppression of spontaneous alternation behaviour. CONCLUSION: In clinical situation, the we think that atypical antipsychotic drugs those acting as serotonin and dopamine receptor antagonist with no extrapyramidal side effect can be beneficial to improve the symptoms of obsessive-compulsive disorder.
Animals ; Antipsychotic Agents ; Cacao ; Clomipramine ; Clozapine* ; Haloperidol ; Milk ; Models, Animal ; Obsessive-Compulsive Disorder ; Polymethyl Methacrylate ; Rats* ; Receptors, Dopamine ; Serotonin ; Serotonin Receptor Agonists