Rud's syndrome (RS), basically composed of ichthyosis, mental deficiency and hypogonadism, is a rare hereditary disease. Some varying dermatologic, neurologic, endocrinologic, ophthalmologic and musculoskeletal abnormalities have coincided with RS. No case of RS has been documented from Asian countries except one from Japan. We describe a 16-year-old girl who presented with lamellar ichthyosis, mental retardation, hypogonadism, short stature, alopecia, sparse eyebrows, strabismus, cataracts, and congenital dislocation of the hip. To our knowledge, RS coexisting congenital dislocation of the hip herein is the first case in English literature.
Adolescent ; Alopecia ; Asian Continental Ancestry Group ; Cataract ; Dislocations ; Eyebrows ; Female ; Genetic Diseases, Inborn ; Hip ; Humans ; Hypogonadism ; Ichthyosis ; Ichthyosis, Lamellar ; Intellectual Disability ; Japan ; Musculoskeletal Abnormalities ; Strabismus

Primary biliary cirrhosis is frequently associated with a variety of disorders presumed to be autoimmune in nature, such as Sjogrens syndrome, scleroderma, rheumatoid arthritis, systemic lupus erythematosus, and autoimmune thyroiditis. Scleroderma has been recognized in association with primary biliary cirrhosis. Most cases present as the CREST (calcinosis cutis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. Isolated pulmonary hypertension develops in a small proportion of patients, nearly all of whom have limited cutaneous involvement. We report a case who has diffuse scleroderma associated with primary biliary cirrhosis and isolated pulmonary hypertension.
Arthritis, Rheumatoid ; Esophageal Motility Disorders ; Humans ; Hypertension, Pulmonary* ; Liver Cirrhosis, Biliary* ; Lupus Erythematosus, Systemic ; Scleroderma, Diffuse* ; Sjogren's Syndrome ; Thyroiditis, Autoimmune

Polycyclic aromatic hydrocarbons (PAHs) are ubiquitous environmental contaminants present in air and food. Among PAHs, benzo(a)pyrene(BaP), phenanthrene (PH) and pyrene (PY) are considered to be important for their toxicity or abundance. To investigate the changes of biomarkers after PAH exposure, rats were treated with BaP (150 microgram/kg) alone or with PH (4,300 microgram/kg) and PY (2,700 microgram/kg) (BPP group) by oral gavage once per day for 30 days. 7-ethoxyresorufin-O-deethylase activity in liver microsomal fraction was increased in only BaP groups. The highest concentration (34.5 ng/g) of BaP, was found in muscle of rats treated with BaP alone at 20 days of treatment; it was 23.6 ng/g in BPP treated rats at 30 days of treatment. The highest PH concentration was 47.1 ng/g in muscle and 118.8 ng/g in fat, and for PY it was 29.7 ng/g in muscle and 219.9 ng/g in fat, in BPP groups. In urine, 114-161 ng/ml 3-OH-PH was found, while PH was 41-69 ng/ml during treatment. 201-263 ng/ml 1-OH-PY was found, while PH was 9-17 ng/ml in urine. The level of PY, PH and their metabolites in urine was rapidly decreased after withdrawal of treatment. This study suggest that 1-OH-PY in urine is a sensitive biomarker for PAHs; it was the most highly detected marker among the three PAHs and their metabolites evaluated during the exposure period and for 14 days after withdrawal.
Adipose Tissue/chemistry/drug effects ; Animals ; Benzo(a)pyrene/analysis/metabolism/*toxicity ; Biological Markers/metabolism/urine ; Blood Chemical Analysis ; Body Weight/drug effects ; Cytochrome P-450 CYP1A1/metabolism ; Environmental Pollutants/blood/metabolism/*toxicity/urine ; Female ; Liver/drug effects/enzymology ; Lymphocytes/drug effects/metabolism ; Muscle, Skeletal/drug effects/metabolism ; Organ Size/drug effects ; Phenanthrenes/blood/metabolism/*toxicity/urine ; Pyrenes/analysis/metabolism/*toxicity ; Rats ; Rats, Sprague-Dawley ; Time Factors

We report a case of uncrossed complete ureteral duplication with the orifice of the upper system ureter opening in the cranial and lateral positionin the bladder. There was reflux only to the upper system of the duplex kidney and the orifice of the lower system ureter in caudal position was associated with a ureterocele, and the lower pole system was large and non-functioning. The patient was treated successfully by lower pole heminephrectomy and ureterectomy.
Humans ; Kidney ; Ureter* ; Ureterocele* ; Urinary Bladder

Mediastinal fibrosis is pathologically characterized by chronic inflammation and fibrosis of mediastinal soft tissue. Mediastinal fibrosis is local expression of a family of systemic fibrosing syndroms. This can result in compression of adjacent mediastinal structures. Idiopathic fibrosing syndromes include retroperitoneal fibrosis, sclerosing cholangitis of the orbit and fibrosis of the thyroid gland(Riedel's struma). The cause of these disorders is obscure, in some instance there is an underlying malignancy, infection, history of drug ingestion, or trauma with retoperitoneal bleeding. Treatment of mediastinal fibrosis depends on structures involved by the fibrotic process. The disease is self limited in most case or improved by steroids uses. We experienced a case of idopathic solerosing mediastinitis with orbital fibrous dysplasia of unknown cause, which was confirmed by open lung biopsy, so reported it with a review of literature.
Biopsy ; Cholangitis, Sclerosing ; Eating ; Fibrosis* ; Hemorrhage ; Humans ; Inflammation ; Lung ; Mediastinitis ; Orbit ; Retroperitoneal Fibrosis ; Steroids ; Thyroid Gland

PURPOSE: With the use of personal computers generalized, departmental society leveled computerization isgoing on in some other departments. So we tried to develop a program having a simple user interface, various retrieval functions and, analytic & statistic process system to effectively help patient care suitable for works concerned with department of diagnostic radiology and works of department. MATERIALS AND METHODS: This programdeals with such target works as department of diagnostic radiology and some works to need a lot of book keeping. Itis deviced to operate with windows (Microsoft, America), and central processing unit(486DX-2), memory unit(8Mbyte). As a developmental tool, Foxpro 2.6 for windows R(Microsoft, America). RESULTS: This Program can be easily accessed even by staffs poor at computer and it can make many books recording various check-ups and operations unnecessary, which were difficult to keep. Besides, it can keep data as a unified form, and so it provides patient care and other works with convenience and helps applying those stored data scientific research. CONCLUSION: The above result shows that works of department can be effectively controlled by analyzing or printing various check-up and operation done by department of diagnostic radiology.
Humans ; Memory ; Microcomputers* ; Patient Care

Monoclonal antibody (mAb, NVRQS-DON) against deoxynivalenol (DON) was prepared. DON-Ag coated enzyme linked immunosorbent assay (ELISA) and DON-Ab coated ELISA were prepared by coating the DON-BSA and DON mAb. Quantitative DON calculation ranged from 50 to 4,000 ng/mL for DON-Ab coated ELISA and from 25 to 500 ng/mL for DON-Ag coated ELISA. 50% of inhibitory concentration values of DON, HT-2, 15-acetyl-DON, and nivalenol were 23.44, 22,545, 5,518 and 5,976 ng/mL based on the DON-Ab coated ELISA. Cross-reactivity levels of the mAb to HT-2, 15-acetyl-DON, and nivalenol were 0.1, 0.42, and 0.40%. The intra- and interassay precision coefficient variation (CV) were both <10%. In the mAb-coated ELISA, mean DON recovery rates in animal feed (0 to 1,000 microg/kg) ranged from 68.34 to 95.49% (CV; 4.10 to 13.38%). DON in a buffer solution (250, 500 and 1,000 ng/mL) was isolated using 300 microg of NVRQS-DON and 3 mg of magnetic nanoparticles (MNPs). The mean recovery rates of DON using this mAb-MNP system were 75.2, 96.9, and 88.1% in a buffer solution spiked with DON (250, 500, and 1,000 ng/mL). Conclusively we developed competitive ELISAs for detecting DON in animal feed and created a new tool for DON extraction using mAb-coupled MNPs.
Animal Feed/analysis ; Animals ; Antibodies, Fungal/analysis ; Antibodies, Monoclonal/analysis ; Chemistry Techniques, Analytical/*methods ; Enzyme-Linked Immunosorbent Assay/*methods/veterinary ; Female ; Food Contamination/*analysis ; Fusarium/immunology ; Imidazoles/chemistry ; Magnetics/methods ; Mice ; Mice, Inbred BALB C ; Mycotoxins/*analysis/chemistry ; Nanoparticles/chemistry ; Ovalbumin/chemistry ; Trichothecenes/*analysis/chemistry

Although lipomas are common benign tumors in humans, endobronchial lipomas are quitely rare. Up to date, about 60 cases have been reported in the English literature. But endobronchial lipomas causing middle lobe syndrome were only 4 cases. These benign slow-growing tumors generally occur in the proximal portion of the lobar or segmental bronchi and originate from fatty tissue that is normally present in the bronchial tree. Smoking or chronic inflammation may be important in the pathogenesis of these tumors. Bronchoscopy is the definite diagnostic tool but, in general, open thoracotomy is required for diagnosis and treatment. Here, we present a case of endobronchial lipoma, with a review of the literature.
Adipose Tissue ; Bronchi ; Bronchoscopy ; Diagnosis ; Humans ; Inflammation ; Lipoma* ; Middle Lobe Syndrome* ; Smoke ; Smoking ; Thoracotomy

OBJECTIVES: This multicenter clinical trial involving 13 hospital sites compared the safety of switching to olanzapine between 'direct switching method' and 'start-tapering switching method'. METHOD: This study included both inpatients and outpatients who fulfilled the criteria for schizophrenia as defined in the ICD-10, and were in need to be appropriate for switching antipsychotics. Subjects were randomly assigned to one of the two switching methods. For 'direct switching method' group, previous antipsychotics were abruptly discontinued and 10mg of olanzapine was administered, whereas for 'start-tapering switching method' group, initially 10mg of olanzapine was administered and previous antipsychotics was gradually tapered for 2 weeks. Olanzapine was used for 6 weeks and the dose was adjusted within the range of 5-20mg. The safety of switching to olanzapine was measured with vital signs including body weight, adverse events reported spontaneously, laboratory tests, and various scales such as Simpson-Angus Scale(SAS), Barnes Akathisia Rating Scale(BARS), Abnormal Involuntary Movement Scale(AIMS), and Liverpool University Neuroleptic Side Effect Rating Scale(LUNSERS). RESULTS: 103 patients were switched to olanzapine in this study. The comparison between two switching methods did not show any significant difference in the dosage of olanzapine used, the concomitant use of benzodiazepine, the rate and reasons of drop-out, the adverse events, vital signs, laboratory tests, and most scales for measuring side-effects. However, the decrease in AIMS scores was significantly lower in 'direct switching method' group, and the concomitant use of anticholinergics was comparatively greater in 'start-tapering switching method' group. At baseline, SAS and BARS scores were 3.5 and 1.8 points respectively, and more than 70% of the subjects showed hyperprolactinemia. After switching to olanzapine, SAS, BARS, and AIMS scores were significantly decreased and the proportion of the patients with hyperprolactinemia was also decreased to less than 30%. However significant weight gain after the treatment of olanzapine was observed regardless of switching method. CONCLUSION: This study may suggest that switching to olanzapine can be done with relatively high safety regardless of switching methods and olanzapine can significantly decrease some side-effects induced by other antipsychotics.
Antipsychotic Agents ; Benzodiazepines ; Body Weight ; Cholinergic Antagonists ; Dyskinesias ; Humans ; Hyperprolactinemia ; Inpatients ; International Classification of Diseases ; Outpatients ; Psychomotor Agitation ; Schizophrenia ; Vital Signs ; Weight Gain ; Weights and Measures

OBJECTIVES: This randomized, multicenter, open-label, parallel clinical trial was carried to compare the therapeutic efficacy and the proportion of successful switch between 'direct switching method' and 'start-tapering switching method' when switching an antipsychotic to olanzapine. METHODS: This study included both inpatients and outpatients who fulfilled the criteria for schizophrenia as defined in the ICD-10 from 13 hospitals, and were in need to be appropriate for switching antipsychotics. Subjects were randomly assigned to one of the two switching methods. For 'direct switching method' group, previous antipsychotics were abruptly discontinued and 10mg of olanzapine was administered, whereas for 'start-tapering switching method' group, initially 10mg of olanzapine was administered and previous antipsychotics was gradually tapered for 2 weeks. Olanzapine was used for 6 weeks and the dose was adjusted within the range of 5-20mg. The therapeutic efficacy was measured with PANSS, BPRS, and CGI-Severity. A successful switching was defined as the completion of the 6 week trial without either worsening of the symptom(i.e. CGI-S score becomes worse twice consecutively) or the exacerbation of extrapyramidal symptoms(i.e. Simpson-Angus Scale scores becomes worse). RESULTS: 103 schizophrenic patients were participated in this study. There were no differences in baseline characteristics such as the demographic variables, the severity of symptoms, the history of previous antipsychotics treatments, the dosage of olanzapine used and the compliance between two groups. The proportion of successful switch was 71.1% for "direct switching method" and 82.2% for "start-tapering switching method", and there was no significant difference between the two switching methods. Also response rates to olanzapine based on total PANSS total scores were not different between the two groups(26.9% vs. 31.1%). At the time of completion of the trial, the scores of PANSS total, PANSS subscales, CGI-S and BPRS have significantly decreased after switching to olanzapine. But the changes of all scales measuring therapeutic efficacy in both endpoint and weekly analyses were not significantly different between the two switching methods. CONCLUSION: Although this study trial has many limitations and problems as an open clinical trial, the results may suggest that there were no significant differences between the two switching methods in the therapeutic efficacy. It was also found that the additional therapeutic benefits could be obtained by switching their antipsychotics to olanzapine.
Antipsychotic Agents ; Compliance ; Humans ; Inpatients ; International Classification of Diseases ; Outpatients ; Schizophrenia ; Weights and Measures