A perplexing developmental error is 46,XX congenital absence of vagina ( Mayer-Rokitan-sky-Kster-Hauser syndrome, Mullerian dysgenesis, vaginal aplasia ). The Mayer-Rokitansky-Kster-Hauser syndrome refers to the climical entity consisting of primary amenorrhea associated with congenital absence of the vagina, 46,XX karyototype, a rudimentary uterus or complete absense, normal overian function and normal ovulation, normal female breast development, body proprotion and body hair, frequent association of renal, skeletal and other cpngenital anomalies. This syndrome results from agenesis of both Mllerian ducts or from a failure of the Mllerian ducts to estavlish a proper communication with that part of the vagina from the urogenital sinus. A case of mayer-Rokitansky-Kster-Hauser syndrom was reviewed briefly.
Amenorrhea ; Breast ; Female ; Hair ; Humans ; Ovulation ; Uterus ; Vagina

BACKGROUND: Glucose intolerance and diabetes mellitus are frequently observed in chronic liver disease. However, the causal relationships between these two are difficult to prove. Chronic hepatitis B infection, which is prevalent in Korea, is thought to be a good model to study the natural history of abnormal glucose metabolism in chronic liver disease because many patients with chronic hepatitis B infection eventually progress to liver cirrhosis. METHODS: In order to evaluate glucose metabolism in chronic hepatitis B infection, we did intravenous glucose tolerance test in patients with chronic hepatitis B and age, sex and body mass index matched controls and compared the first phase insulin response and glucose disappearance rates between 2 groups. RESULTS: Patients with chronic hepatitis B showed lower glucose disappearance rate and higher plasma insulin and C-peptide area (0-10min after iv glucose) than controls. Patients with decreased glucose disappearance rate had higher AST level and decreased plasma C-peptide area (0-10min). CONCLUSION: Most of the patients with chronic hepatits B infection is associated with insulin resistance and compensatory increase in the first phase insulin secretion. Inadequate insulin secretion may contribute to decreased glucose disappearance rate in these patients.
Body Mass Index ; C-Peptide ; Diabetes Mellitus ; Glucose Intolerance ; Glucose Tolerance Test ; Glucose* ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Humans ; Insulin Resistance ; Insulin* ; Korea ; Liver Cirrhosis ; Liver Diseases ; Metabolism* ; Natural History ; Plasma

BACKGROUND: Many countries have been legally prescribing health warning messages on cigarette packages as a part of their national policy of smoking cessation. This study was designed to evaluate the effectiveness for smoking cessation of graphic health warning labels, and compare them with the existing health warning messages. METHODS: From February to December 2002, we surveyed 103 smokers who were college students of fine arts. After letfing them see the graphic health warning labels, the self recorded questionnaires were collected. RESULTS: The effectiveness for the smoking cessation of the existing health warning messages was evaluated low, despite its wide recognition. But there was no difference between the visual effect and the effectiveness for the smoking cessation of the graphic health warning labels. The graphic health warning labels were more effective for the smoking cessation than the existing health warning messages, and it was the same in the subgroup analysis such as sex, degree of recognition of disease, and non- smoking planning. CONCLUSION: To quit smoking, it is reasonably concluded that the graphic health warning labels on cigarette packages was very effective. Hereafter, it is recommended more useful designs of graphic health warning labels be developed.
Humans ; Smoke* ; Smoking Cessation ; Smoking* ; Tobacco Products*

PURPOSE: Antiepileptic drugs may alter serum lipid status in epileptic patients. We conducted this study to assess the effect of valproate on serum levels of total cholesterol(TC), triglycerides(TG), low-density lipoprotein(LDL), high-density lipoprotein(HDL), and TC/HDL ratio, and to investigate the factors affecting serum lipid status in children with epilepsy who had been receiving valproate therapy. METHODS: Thirty epileptic children(16 males, 14 females, mean age 7.4+/-3.3 years) were evaluated for serum lipid status at the onset and the 6, 12 and 24 months of valproate therapy, and were analysed changes and potential factors of affecting changes such as sex, body mass index, valproate concentration, in serum lipid levels during valproate therapy. RESULTS: TC were significantly lowered during first 12 months of valproate theraphy (P<0.05). LDL were lowered during first 12 months. HDL and TC/HDL ratio were not changed and TG were increased during valproate theraphy but not reach to statistical significance. TC, TG, LDL, and HDL return to pretreatment levels after 24 months of valproate theraphy. TC, LDL, HDL, and TC/HDL ratio changes were not significantly different by sex and initial body mass index, but TG were significantly increased in group of BMI below 20(P<0.05). LDL levels were significantly decreased correlation to serum valproate concentration(r=-0.2915. P<0.05). CONCLUSION: Our results suggest that valproate therapy would not increase a risk for atherosclerotic disorders in adulthood, but weight gain with a metabolic consequence of obesity would increase risk for atherosclerotic disorders in adulthood.
Anticonvulsants ; Body Mass Index ; Child* ; Epilepsy* ; Female ; Humans ; Male ; Obesity ; Valproic Acid* ; Weight Gain

BACKGROUND: Reports on serum thyroglobulin(Tg) levels being used to predict recurrence or distant metastasis during the follow-up of patients with differentiated thyroid cancer(DTC) has been inconsistent. In addition, there have been few reports that attempt to define the cut-off value of Tg for recurrence or distant metastasis obtained by a receiver operating characteristic(ROC) curve. As well, there are differences in opinions on what the value should be on the first serum Tg level measured just before radioactive iodine(RAI) ablation(Tg-RAI), during thyroxine administration (Tg-on), and after thyroxine withdrawal(Tg-off) during the follow-up. Reports on the positive predictive values(PPVs) and negative predictive values(NPVs) of these Tg values are rare. METHODS: A total of 205 patients(42 males, 163 females) with DTC were studied. All patients had undergone total or near-total thyroidectomy. After surgery and RAI ablation, annual thyroxine withdrawal 131I-whole body scan(WBS) with Tg measurements was performed. The mean duration of follow-up was 5.0 (1.4~7.4) years. The most sensitive and specific Tg values(cut-off values) for tumor recurrence and/or distant metastasis were selected by using ROC curves. We also calculated the PPVs and NPVs for recurrence and/or distant metastasis using two-by-two tables. RESULTS: Cut-off values of Tg-RAI, Tg-on, and Tg-off for recurrence were 11.8, 1.4, and 3.3ng/mL, respectively. For these values, the sensitivities were 85.4, 82.2, and 93.3%, with the specificitiesat 89.2, 92.4, and 88.0%. PPVs were 71.9, 77.1, and 77.0% while NPVs were 95.0, 94,4, and 97.8%. The cut-off values for distant metastasis were 27.4, 2.5, and 7.9ng/mL, respectively. For these cut-off values, the sensitivities were 86.7, 87.5, and 92.3%, with the specificities being 86.2, 90.8, and 80.2%. PPVs were 34.2, 46.7, and 25.0% and NPVs were 98.7, 98.8, and 99.3%. CONCLUSION: All three serum Tg levels were sensitive and specific markers for recurrence and distant metastasis. Their PPVs were low in contrast to the high NPVs. In comparison with Tg-on, Tg-off showed higher sensitivity and NPV as well as lower specificity and PPV. Therefore, in the case of higher Tg-on during the follow-up period, efforts to find recurrence and distant metastasis,such as 131I-WBS, should be done. In addition, regular measurement of Tg-off or Tg after stimulation with recombinant human TSH is recommended as a screening test.
Follow-Up Studies ; Humans ; Male ; Mass Screening ; Neoplasm Metastasis* ; Recurrence* ; ROC Curve ; Sensitivity and Specificity ; Thyroglobulin* ; Thyroid Gland* ; Thyroid Neoplasms* ; Thyroidectomy ; Thyrotropin Alfa ; Thyroxine

Primary Pulmonary leiomyosarcomas are rare tumors. These tumors may arise at any level of the lung in which smooth muscle fibers are found. The highest incidence of sarcoma is during the fourth decade whereas for bronchogenic carcinoma the maximum incidence is during the sixth and seventh decades. Leiomyosarcomas are aggressive and progressive. Treatment is mainly surgical. The chemotherapy is ineffective and the effectiveness of radiotherapy depends on the total dose of irradiation. Prognosis and significant survival rate are related to the size of the lesion. We report one patient with primary pulmonary leiomyosarcoma involving the right lower lung.
Carcinoma, Bronchogenic ; Drug Therapy ; Humans ; Incidence ; Leiomyosarcoma* ; Lung ; Muscle, Smooth ; Prognosis ; Radiotherapy ; Sarcoma ; Survival Rate

We report a patient with autosomal dominant polycystic kidney disease(ADPKD) undergoing maintenance hemodialysis who underwent transcatheter arterial embolization(TAE) of the renal arteries to control the persistent renal hemorrhage. In 1991, the patient had been diagnosed as having ADPKD by computed tomography(CT) of abdomen. In 1997, the patient started hemodialysis. In October 2000, she was admitted to our hospital because of gross hematuria and both flank pain. Abdominal CT showed marked enlargement of the bilateral kidneys with numerous cysts. Renal angiography using the Seldinger technique showed stretched and deformed segmental renal arteries without active bleeding. Renal hemorrhage was continued despite of medical treatment. TAE with stainless steel coils was performed on both renal arteries. Renal bleeding was stopped immediately after TAE and anemia was improved and stabilized thereafter. Follow-up CT after five months later TAE showed the marked decrease in size of both kidneys, and patient's abdominal symptoms were disappeared too. This report shows that TAE is a safe and effective therapy for ADPKD patients with persistent renal bleeding refractory to medical therapy.
Cysts

Malignant tumor originated from external auditory canal (EAC) is very rare with an annual incidence of around 1 per million. Pathologically, squamous cell carcinoma is incidentally most common, and adenoid cystic carcinoma, basal cell carcinoma, and melanoma follow in decreasing order. Due to the rarity of malignant tumor of EAC, there is no widely accepted treatment modality yet. But basal cell carcinoma, known to be less aggressive tumor, can be removed with a minimal safety margin and have better treatment results. Recently we experienced a case of basal cell carcinoma in the EAC, confined in the cartilaginous portion of EAC, presenting with intermittent otorrhea for several years. The patient was treated with a sleeve resection of the EAC with a safety margin reconstructed with a split-thickness skin graft. No tumor recurrence or complication was noted in the first postoperative year.
Aminocaproic Acids ; Carcinoma, Adenoid Cystic ; Carcinoma, Basal Cell ; Carcinoma, Squamous Cell ; Ear Canal ; Humans ; Incidence ; Melanoma ; Recurrence ; Skin ; Transplants

PURPOSE: We have done this retrospective study to know the relative incidences and clinical manifestations of organic acidopathies in Korea. METHODS: The results of quantitative organic acid analysis of 1,125 samples of 712 patients, referred from Jul. 1997 to Jun. 2000, were analyzed retrospectively according to four age groups (-2 mon, 3 mon-2 year, 3 years-12 years, over 12 years) and major clinical manifestations. Quantification of 83 organic acids was done with gas chromatography and mass spectrometry(GC/MS). RESULTS: We diagnosed 214 patients with 27 diseases of organic acid metabolism during this study period. Diseases found more than 10 cases are cytosolic 3-ketothiolase deficiency, mitochondrial repsiratory chain disorders, PDHC deficiency, glutaric aciduria type II and propionic aciduria. Other diseases were diagnosed in less than 10 cases, mostly one or two cases during this study period. Most of the patients had some symptoms of neurological dysfunction such as seizure activity(195 patients), developmental delay(122), mental retardation(99), hypotonia(84), movement disorders(81) and vomiting(68). CONCLUSION: Though the incidence of individual organic acidemia is low, the overall incidence of organic acidemia as a whole seems to be relatively high in Korea. Most of the patients showed some signs of neurological dysfunction.
Acetyl-CoA C-Acyltransferase ; Chromatography, Gas ; Cytosol ; Humans ; Incidence ; Korea* ; Metabolism ; Multiple Acyl Coenzyme A Dehydrogenase Deficiency ; Propionic Acidemia ; Pyruvate Dehydrogenase Complex Deficiency Disease ; Retrospective Studies ; Seizures

This study is designed to evaluate the effect of low-dose whole body irradiation (WBI) for treatment of myasthenia gravis(MG) and changes of immunological parameters. According to MG protocol of low dose WBI, eleven patients were selected and followed up for at least one year. Clinical status and immunological parameters were assessed at the time of pretreatment (baseline) and 1, 2, 3, 6, 12 months after initial dose irradiation. The improvement began five weeks after WBI and lasted for 12-month follow-up in 7 patients (good responders) Clinical improvement and immunological changes were more pronounced in good responders. So, it is suggested that low dose WBI may have the role in the treatment of the MG as an another therapeutic modality.
Follow-Up Studies ; Humans ; Myasthenia Gravis* ; Whole-Body Irradiation*