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

OBJECTIVE: To analyze the tubal patency in the young-aged women underwent diagnostic laparoscopy with Fitz-High-Curtis Syndrome (FHCS). METHODS: Clinicopathologic results of young-aged women who underwent diagnostic laparoscopy with pelvic inflammatory disease from March 2005 through April 2007 were reviewed. Twenty six patients aged 19-29 years old and preserved their both tubes after diagnostic laparoscopy were included in this study. All were underwent chromopertubation test (CPT) during laparoscopy and postoperative hysterosalpingography (HSG) 3 months later. RESULTS: Of 26 patients, eight patients were diagnosed with FHCS and the others were not. There was no significant difference in mean age, mean hospitalization days, and frequency in past-history of parturition, abortion, chlamydia and gonococci infection between patients with FHCS and with non-FHCS (P>0.05). There was no significant difference in frequency of tubal obstruction in laparoscopic CPT (P>0.05) but not in postoperative outpatient HSG (P<0.05). CONCLUSION: The tubal obstruction may be more frequent in FHCS than non-FHCS and the reliability of laparoscopic CPT for diagnosing the tubal obstruction is supposed to be low. Therefore, as for FHCS patients, HSG should be executed to investigate tubal factor which is helpful to forecast the fertility.
Aged ; Chlamydia ; Fallopian Tube Diseases ; Female ; Fertility ; Hospitalization ; Humans ; Hysterosalpingography ; Laparoscopy ; Outpatients ; Parturition ; Pelvic Inflammatory Disease

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

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

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*

"We retrospectively reviewed the results of 1,850 fine needle aspiration cytology (FNAC) of thyroid nodules performed from 1990 to 1991 in the Department of Pathology, Seoul National University Hospital. Among 1,528 cases and 322 cases aspirated by clinicians and a pathologist, 465 cases (30.4%) and 13 cases (4.0%) of the aspirates were inadequate, respectively. In 227 cases, correlation of the FNAC diagnosis and histologic diagnosis was done. Excluding the inadequate cases, the sensitivity for the detection of neoplasm (malignancy together with follicular adenoma) was 86.4% and the specificity was 70.7%. The overall diagnostic accuracy was 79.0%. There were 16 false-positive cases (7.0%), and 19 false-negative cases (8.4%). The predictive value of each cytologic diagnosis was 92% in papillary carcinoma, and 100% in Hashimoto's thyroiditis. The expectancy of malignancy was 52.8% in ""suspicious malignancy"" and 26.7% in ""atypical lesion""."
Biopsy, Fine-Needle ; Carcinoma, Papillary ; Diagnosis ; Pathology ; Retrospective Studies ; Sensitivity and Specificity ; Seoul ; Thyroid Gland ; Thyroid Nodule ; Thyroiditis

No abstract available.
Arthritis* ; Immunoblastic Lymphadenopathy*

Ceftriaxone is widely used in patients for the treatment of serious gram-negative infections. Ceftriaxone can induce some potential side effects, including neurotoxicity, however, nonconvulsive status epilepticus has rarely been reported. We report a case of acute reversible neurotoxicity associated with ceftriaxone. A 65-yr-old woman with chronic kidney disease developed altered consciousness during ceftriaxone treatment for urinary tract infection. The electroencephalogram demonstrated continuous bursts of generalized, high-voltage, 1 to 2 Hz sharp wave activity. Neurologic symptoms disappeared following withdrawal of ceftriaxone. The possibility of ceftriaxone-induced neurotoxicity should be considered in patients developing neurological impairment during ceftriaxone use, and the discontinuation of the drug could lead to complete neurological improvement.
Aged ; Anti-Bacterial Agents/*adverse effects/therapeutic use ; Anticoagulants/therapeutic use ; Ceftriaxone/*adverse effects/therapeutic use ; Electroencephalography ; Female ; Humans ; Nervous System Diseases/*etiology ; Renal Dialysis ; Renal Insufficiency, Chronic/pathology ; Seizures/etiology ; Thrombosis/diagnosis/drug therapy ; Tomography, X-Ray Computed ; Urinalysis ; Urinary Tract Infections/diagnosis/drug therapy

OBJECTIVES: The protective effects of estrogen against the development of schizophrenia have been investigated in various perspectives. Previous studies showed patients with earlier age of menarche had later onset of schizophrenia and less frequent admissions. This study was performed to evaluate the effect of the age of menarche on subsequent courses including hospitalizations, suicidal attempts, physical harm to others, and psychotic symptoms in women with schizophrenia. METHODS: Self-report menarche age, onset of psychotic symptoms, and clinical status were collected in a sample of 108 premenopausal women with the diagnosis of schizophrenia. RESULTS: There was significant correlation between the age of menarche and the frequency of admissions (r=.321, p=.006). Also, patients with history of physical harms to others showed later age of menarche than those without (t=2.025, df= 106, p=.045). CONCLUSION: In women with schizophrenia, the age of onset of menarche affects subsequent courses indicated by frequency of admissions and violent behaviors. It is suggested that identifying the age of menarche might be helpful to predict the courses in women with schizophrenia. Further investigations regarding the relationship between estrogen and the courses of schizophrenia in women are needed.
Age of Onset ; Diagnosis ; Estrogens ; Female ; Hospitalization ; Humans ; Menarche* ; Schizophrenia*

OBJECTIVES: The protective effects of estrogen against the development of schizophrenia have been investigated in various perspectives. Previous studies showed patients with earlier age of menarche had later onset of schizophrenia and less frequent admissions. This study was performed to evaluate the effect of the age of menarche on subsequent courses including hospitalizations, suicidal attempts, physical harm to others, and psychotic symptoms in women with schizophrenia. METHODS: Self-report menarche age, onset of psychotic symptoms, and clinical status were collected in a sample of 108 premenopausal women with the diagnosis of schizophrenia. RESULTS: There was significant correlation between the age of menarche and the frequency of admissions (r=.321, p=.006). Also, patients with history of physical harms to others showed later age of menarche than those without (t=2.025, df= 106, p=.045). CONCLUSION: In women with schizophrenia, the age of onset of menarche affects subsequent courses indicated by frequency of admissions and violent behaviors. It is suggested that identifying the age of menarche might be helpful to predict the courses in women with schizophrenia. Further investigations regarding the relationship between estrogen and the courses of schizophrenia in women are needed.
Age of Onset ; Diagnosis ; Estrogens ; Female ; Hospitalization ; Humans ; Menarche* ; Schizophrenia*