Epstein's syndrome is a rare disease whish is characterized by the association of thrombocytopenia, macrothrombocytopathia, nephritis and deafness. We experienced a case of Epstein's syndrome in a 12 years old male patient who was presented with a life long history of bleeding, usually as epistaxis, bilateral sensorineural deafness and hematuria with proteinuria starting in late childhood. Hematologic studies showed thrombocytopenia with giant platelets and anemia. A bone marrow aspirate revealed the megakaryocytes to be adequate in number and many giant size platelets. Platelet do not respond to addition of A and epinephrine; collagen and ristocetin induced agglutination response is decreased. It is difficult to be certain the association of thrombocytopenia with giant platelets, nephritis and deafness constitutes a new hereditary disease with a distinct pathogenesis or if it is an expansion of the well recognized Alport's syndrome of hereditary nephritis deafness. We report a case of Epstein's syndrome syndrome with brief review of related literatures.
Agglutination ; Anemia ; Blood Platelets ; Bone Marrow ; Child ; Collagen ; Deafness ; Epinephrine ; Epistaxis ; Genetic Diseases, Inborn ; Hematuria ; Hemorrhage ; Humans ; Male ; Megakaryocytes ; Nephritis ; Nephritis, Hereditary ; Proteinuria ; Rare Diseases ; Ristocetin ; Thrombocytopenia

The purpose of this study was to compare the microleakage of low and high viscosity flowable resins in class V cavities applied with 1-step adhesives. Forty class V cavities were prepared on the cervices of buccal and lingual surfaces of extracted molar teeth and divided into four groups (n=8). Cavities were restored with AQ Bond Plus/Metafil Flo alpha, G-Bond/UniFil LoFlo Plus (Low flow groups), AQ Bond Plus/Metafil Flo and G-Bond/UniFil Flow (High flow group), respectively. Specimens were immersed in a 2% methylene blue solution for 24 hours, and bisected longitudinally. They were observed microleakages at the enamel and dentinal margins. In conclusion, the low viscosity flowable resins showed lower marginal microleakage than do the high viscosity flowable resins in class V cavities.
Dental Enamel ; Dentin ; Dentin-Bonding Agents ; Methylene Blue ; Molar ; Tooth ; Viscosity

Chronic sclerosing sialadenitis, Mikulicz disease or Kuttner tumor has been recently recognized as a spectrum of IgG4-related sclerosing disease. IgG4-related disease is characterized by a high serum IgG4 level and tissue infiltration of IgG4-positive plasmacytes. We report three cases of chronic sclerosing sialadenitis with variably associated systemic involvement. All patients presented with a submandibular mass or swelling, and all the resected submandibular glands showed diffuse lymphocytic infiltration, lymphoid follicles, and septal fibrosis. Two of the specimens revealed numerous IgG-positive plasma cells, most of which were IgG4-positive on immunohistochemical staining. One of them was associated with dacryoadenitis and hypophysitis. The other patient had ureterorenal lesions. Immunohistochemical study was unavailable in remaining one case, but the histologic features along with elevated IgG level and associated pancreatitis supported the diagnosis. All patients received steroid therapy postoperatively and are doing well. Salivary gland involvement in IgG4-related fibrosclerosis should be recognized in systemic medical pathology.
Dacryocystitis ; Fibrosis ; Humans ; Immunoglobulin G ; Mikulicz' Disease ; Pancreatitis ; Plasma Cells ; Salivary Glands ; Sialadenitis ; Submandibular Gland

No abstract available.
Animals* ; Escherichia coli* ; Escherichia* ; Humans*

PURPOSE: To determine the MR findings of cervical lymphadenopathy which distinguish tuberculouslymphadenitis(TL) from metastatic lymphadenopathy(ML). MATERIALS AND METHODS: We retrospectively analyzed the MRfindings of 14 patients with TL and 19 with ML. Thirty-nine lesions in 14 patients with TL and 80 lesions in 19with ML were classified into three categories: conglomerate lesion (TL, 7; ML, 8); discrete lesion withoutnecrosis (TL, 3; ML, 15); and discrete lesion with necrosis (TL, 29; ML, 57). Between TL and ML, we compared age,sex, bilaterality, multiplicity, associated pulmonary tuberculosis, infiltration of adjacent fat, muscle, skin andsubcutaneous tissue, margin, and signal intensities on T1- and T2-weighted images. In discrete lesion, we alsocompared the minimal to maximal dimension ratio, and in discrete necrotic lesion, the thickness and evenness ofrim enhancement, and the location and extent of necrosis. RESULTS: There were statistically significantdifferences between TL and ML in age(34.7+/-13.1 years; 56.1+/-14.3 years), male to female ratio(3:11; 13:6),bilaterality(1/14; 10/19), multiplicity(5/14; 14/19), and associated active pulmonary tuberculosis(7/14; 1/19). OnT2-weighted images, TL showed predominantly homogeneous (30/39) or high (20/39) signal intensity, similar to thatof cerebrospinal fluid, but the signal inteusity of ML was predominantly heterogeneous(58/80), or lower than orsimilar to that of fat(41/80). Between TL and ML, however, there were no statistically significant differences ininfiltration of adjacent tissue, margin, signal intensity and its heterogeneity on T1-weighted image, minimal tomaximal dimension ratio, thickness and evenness of rim enhancement, or the location and extent of necrosis. CONCLUSION: In cervical lymphadenopathy, the existence of TL rather than ML is suggested by single or unilaterallesion in a young woman, by associated active pulmonary tuberculosis, or by homogeneous high signal intensity onT2-weighted MR images.
Cerebrospinal Fluid ; Female ; Humans ; Lymphatic Diseases* ; Male ; Necrosis ; Neoplasm Metastasis* ; Population Characteristics ; Retrospective Studies ; Skin ; Tuberculosis* ; Tuberculosis, Pulmonary

OBJECTIVES: This study compared attitudes toward antipsychotic treatment according to awareness of the name of their illness in patients with schizophrenia. METHODS: Information on sociodemographic and clinical characteristics, including awareness of the importance of antipsychotic treatment, was obtained through a self-report questionnaire. The Drug Attitude Inventory (DAI) was administered. The data were compared according to awareness of the name of their illness. RESULTS: The study analyzed data for 199 patients with schizophrenia. Of these, 115 patients (57.8%) were aware that their illness was called schizophrenia, while 84 patients (42.2%) knew it by their psychotic symptoms or as another mental illness, such as depression. The patients aware of the name of their illness had significantly longer durations of illness and higher scores on the DAI. They were significantly more likely to have stopped taking medication on their own accord and to agree with the importance of antipsychotic treatment. Statistical significance was sustained in a logistic regression analysis after adjusting for the duration of illness and study site, except for the DAI score, which had borderline significance (p=0.055). In subjects with duration of illness > or =5 years, patients aware of the name of their illness had significantly higher scores on the DAI. CONCLUSION: Awareness of the name of their illness was associated with awareness of the importance of, and a positive attitude toward, antipsychotic treatments in patients. Psycho-education, including telling the patient the correct name of his or her illness, might be needed for maintaining antipsychotic treatment in patients with chronic schizophrenia.
Antipsychotic Agents ; Depression ; Humans ; Logistic Models ; Surveys and Questionnaires ; Schizophrenia

OBJECTIVES: This study was performed to determine the levels of quality of life (QOL) according to the grade of long-term care service for the elderly people who were admitted from long-term care insurance, and to reveal its association with the physical and mental functioning such as the Activity of Daily Living (ADL), the Instrumental Activity of Daily Living (IADL), the Center for Epidemiologic Studies Depression Scale (CES-D), and the Mini-Mental State Examination-Korean version (MMSE-K). METHODS: The interviews were performed during the period from March 1 to May 31, 2009, for 958 elderly people in urban and rural areas. The questionnaire items included various indices such as the ADL, IADL, CES-D, and MMSE-K, as independent variables and the index of QOL, as the dependent ones. For statistical analysis, t-tests were used for the mean scores of QOL according to gender and the grade of long-term care services, and Spearman's correlation was used for each variable. The effects of physical and mental functioning for QOL were assessed by covariance structure analysis. The statistical significance was set at p<0.05. RESULTS: The mean scores of QOL among all the subjects was 55.4 +/- 15.62 (Grade I: 49.7 +/- 14.17, Grade II: 56.8 +/- 14.62, Grade III: 59.4+/-16.36), and it was lower according to the higher grade of long-term care insurance. In terms of the correlation matrix of the QOL and the physical and mental function factors, the QOL showed positive correlation with the ADL, IADL and MMSE-K, while it had negative correlation with depression. On the analysis of covariance, mental functioning (depression and the MMSE-K) had a greater influence on the level of QOL than the physical functioning (ADL and IADL). CONCLUSIONS: The level of the QOL in the elderly people who were admitted from long-term care insurance was lower according to higher the grade of long-term care insurance. Also, the mental functioning (depression and MMSE-K) was more influential on the level of the QOL than the physical functioning (ADL and IADL).
Activities of Daily Living/psychology ; Aged ; Depression/psychology ; Female ; *Health Status ; Humans ; Insurance, Long-Term Care/*statistics & numerical data ; Long-Term Care/*psychology/*statistics & numerical data ; Male ; *Mental Health ; Quality of Life/*psychology ; Residence Characteristics ; Socioeconomic Factors

The coronary artery fistula from right coronary artery to left ventricle is a rare disease among coronary artery anomaly. We experienced three cases of rare coronary fistula and report with literature review. Although symptoms of coronary artery fistula are associated with arteriovenous shunt and coronary steal phenomenon, many cases are asymptomatic. In this report, all patients had no symptom. but incidental murmur was noted (two are continuous, one is diastolic rumbling). The diagnosis was made by transthoracic or transesophageal echocardiogram and selective coronary angiography. Because patients were relatively young and the diameter of dilated coronary artery were huge, we perfomed operation on three patients.
Coronary Angiography ; Coronary Vessels* ; Diagnosis ; Fistula* ; Heart Ventricles* ; Humans ; Rare Diseases

The incidence of pulmonary tuberculosis decreased from 5.1% in 1965 to 1.0% in 1995 in Korea but no statistics about the incidence of urinary tuberculosis have been reported yet. The urinary tuberculosis in childhood is known to be a rare disease because of its long latent period. We report a case of urinary tuberculosis in a 9 year old girl.
Child ; Female ; Humans ; Incidence ; Korea ; Rare Diseases ; Tuberculosis* ; Tuberculosis, Pulmonary ; Urinary Tract*

Condylomata acuminata are benign tumors which are mostly venereally transmitted. Common sites were coronal sulcus, perisnal area and prepuce. Among 28 patients, 21 acuminate lesions and 10 papular lesions were found. Twenty eight human genital warts in Korean were analysed by Southern blot hybridization. Sequences related to HPV6/11 are found in 89.3%(25/28) of the condylomata. HPV16 DNA was not found at sll. Subtype of HPV was determined by the restriction pattern of DNA cleaved with PstI restriction enzyme in 7 cases. Six cases of HPV6a and one case of HPV6c are found. The above results suggest that most of condylomata acuminata are caused by HPV6 and HPV11 in Korea.
Blotting, Southern ; Condylomata Acuminata* ; DNA* ; Humans* ; Korea