Mauriac syndrome consists of a triad of poorly controlled diabetes, profound growth retardation and hepatomegaly. We experienced a case of Mauriac syndrome in an 18-year-old girl who had poorly controlled diabetes mellitus, short stature, hepatomegaly and central obesity. Also at the time of examination, she had complications of diabetic cataract and nephropathy. Fourteen years prior to admission, she was diagnosed as diabetes mellitus at a hospital. Thereafter, she had been managed with irregular insulin injection. On physical examination at admission, her height was 135cm(<3 percentile) and her weight was 39kg(<3 percetile). She was short and obese. The liver was 5 cm palpable below the right subcostal margin. Her sexual maturation was Tanner stage I. On ophthalmologic examination, the cataracts were observed on both eyes and diabetic retinopathy was absent. Diabetic nephropathy could not be confirrned by kidney biopsy due to her mother's refusal. We studied the hormonal, radiographic and histological abnormalities. The hormonal study was normal and the bone age was by delayed as much as 10 years. The liver biopsy revealed glycogen accumulation in hepatocyte. She was consistent with Mauriac syndrome. She was managed by strict diabetic control with insulin therapy, diabetic diet and intensive education. She was discharged with well controlled blood glucose. Five months later, growth acceleration and sexual maturation have not been observed, but hepatomegaly subsided. (J Korean Pediatr Soc 2000;43-837-841)
Acceleration ; Adolescent ; Biopsy ; Blood Glucose ; Cataract ; Diabetes Mellitus* ; Diet, Diabetic ; Diabetic Nephropathies ; Diabetic Retinopathy ; Disulfiram ; Education ; Female ; Glycogen ; Hepatocytes ; Hepatomegaly ; Humans ; Insulin ; Kidney ; Liver ; Obesity, Abdominal ; Physical Examination ; Sexual Maturation

Sir James Paget described the first case of Paget's disease of the breast in 1874, and Du-breuilh, in 1901, reported the first case of extramammary Paget's disease of the vulva. Paget's disease of vulva is a rare intraepithelial neoplasm of uncertain histogenesis. Recently we expe-rienced a case of Paget's disease of the vulva that we present a brief review of literature.
Carcinoma in Situ ; Paget Disease, Extramammary ; Paget's Disease, Mammary ; Vulva*

No abstract available.
Neuroblastoma*

No abstract available.
Radiation Oncology*

A 13-month-old boy had irregularly shaped, erythematous to dusky red, slightly raised patches and plaques on the left antecubital fossa and arm for 7 months. The lesions were firm and tender. Histopathologically the lesion consisted of small, circumscribed, angiomatous tufts and lobules with a cannonball appearance scattered throughout dermis, a characteristic feature of tufted angioma. For 15 weeks, interferon-a intralesional injections have been performed and the lesions showed an improvement.
Arm ; Dermis ; Hemangioma* ; Humans ; Infant ; Injections, Intralesional ; Interferon-alpha* ; Male

Between June 1990 and May 1994, 350 laser conization and 200 LEEP were performed. Indications of conization were that directed biopsy specimen was proved CIN II, III or suggests possible microinvasion. In all the cases the procedures were carried out with the patients under local anesthesia. Excisional cone sections(6,600) were evaluated for lesion length,depth and margin status. Invasive cancer was found in 5(1.4%) women of laser group. Operative time was shorter LEEP group than laser group significantly. In laser group, 25(8.3%) women had bleeding that required treatment. One case(0.3%) of pelvic infection and 7 cases(2.3%) of cervical stenosis were observed. In LEEP group, 10(5.3%) women had bleeding, 3(1.6%) patients became cervical stenosis. The diameter of bumed tissue was 0.28mm in laser group and 0.25mm in LEEP group. Success rate were 97.4% in former and 96.3% in later. This study demontrated that CO2 laser conization and LEEP were effective methods for treating high grade cervical intraepithelial neoplasia and added benefit of preserving reproductive function and rule out invasive carcinoma.
Anesthesia, Local ; Biopsy ; Cervical Intraepithelial Neoplasia* ; Conization* ; Constriction, Pathologic ; Female ; Hemorrhage ; Humans ; Lasers, Gas* ; Operative Time ; Pelvic Infection

OBJECTIVES: This study was conducted to examine health inequality for gender and region in Korea. Especially it focused on health status such as disease prevalence and medical utilization of rural women. METHODS: Data from the Korea national health and nutrition survey in 2001 were used. The final sample size was 37,108 individuals with age 20 and over. This study applied the logistic regression for nominal variables such as disease prevalence and unmet care needs and with the regression for continuos variables such as the length and costs of medical services. RESULTS: Rates of disease prevalence and unmet care needs for chronic disease in rural area are higher than those in middle cities and big cities, and regional differences of those for women are more than those for mens with controlling ages. There could be interaction effect with region and sex. CONCLUSIONS: This study suggests that health policy maker should take consider of special status of rural women who are in health inequality.
Chronic Disease ; Female ; Health Policy ; Humans ; Korea ; Logistic Models ; Male ; Nutrition Surveys ; Prevalence ; Rural Health ; Sample Size ; Socioeconomic Factors

A 3-year-old girl presented with polydipsia, polyuria, hyponatremia, hypertension and congestive heart failure. Her polyuria was unresponsive to water restriction and vasopressin challenge tests, and her blood pressure was not effectively controlled by antihypertensive drugs. Radiologic examinations revealed a Wilms' tumor in the right kidney. Her plasma renin activity and aldosterone concentration were greatly increased. After surgical removal of the tumor, the congestive heart failure disappeared. Congestive heart failure due to Wilms' tumor is very rare and we report here on such a case, with a brief review of the literature.
Aldosterone ; Antihypertensive Agents ; Blood Pressure ; Child, Preschool ; Estrogens, Conjugated (USP)* ; Female ; Heart Failure* ; Humans ; Hypertension* ; Hyponatremia ; Kidney ; Plasma ; Polydipsia* ; Polyuria* ; Renin ; Vasopressins ; Water ; Wilms Tumor*

BACKGROUND: The autonomic nervous system plays a critical role in triggering ventricular arrhyhmia and sudden death early after acute myocardial infarction. This study was designed to determine whether or not vagal and sympathetic responses are impaired after myocardial infarction and to evaluate the utility of physiologic stress tests for assessing autonomic dysfunction after myocardial infarction. METHODS: 8 male patients with acute myocardial infarction(Group A) and old myocardial infarction(Group B) were studied with 8 control subjects of coinciding age and sex. 5 physiologic stress tests(deep breathing, sudden standing, Valsalva maneuver, ice bag application on the face, 70degrees headd-up tilt test) were performed. RESULTS: 1) Variation in heart rate during deep breathing, Standing-up, Valsalva maneuver, and ice bag application on the face was less in Group A than in Group B or Group C. 2) There was no significant difference in variation of heart rate between Group B and Group C. 3) Variation in heart rate caused by 70degrees tilt was not significantly different among the three groups. CONCLUSION: Early after myocardial infarction, parasympathetic responses were significantly impaired, whereas sympathetic responses remained intact. Heart rate variability using physiologic stress test may be provide a means of detecting autonomic dysfunction after acute myocardial infarction.
Autonomic Nervous System* ; Death, Sudden ; Exercise Test ; Heart Rate ; Humans ; Ice ; Male ; Myocardial Infarction* ; Respiration ; Valsalva Maneuver

BACKGROUND: Spinal stenosis and herniated intervertebral discs are the principal causes of lumbosacral radiculopathy. This study was conducted to compare the therapeutic value and duration of pain relief of fluoroscopic guided transforaminal epidural steroid injections (TFESIs) in patients with refractory radicular leg pain. METHODS: Between August 2006 and March 2007, 87 patients (H group: patients with herniated intervertebral disc, S group: patients with spinal stenosis) who met the inclusion criteria were treated with fluoroscopic guided TFESIs. Prior to treatment, the VAS and ODI scores were determine to evaluate the degree of pain and level of disability. The degree of pain relief was then assessed 1 month after treatment with the TFESIs and graded as excellent (no residual pain), good (improvement of pain symptoms by more than 50%), fair (improvement of pain symptoms by less than 50%) and Poor (no improvement of pain). In addition, the duration of pain relief was evaluated by regular outpatient visits for 6 months, and by telephone interviews after 6 months. RESULTS: The H and S group both had excellet results at 1 month after treatment with TFESIs showing improvements of 44.1% and 20.8% respectively. However this difference was not significant between groups. In addition, a duration of pain relief greater than 6 months was achieved in 32.4% of the patients in the H group and 37.7% of those in the S group. CONCLUSIONS: TFESIs had a similar degree of therapeutic effectiveness and duration of pain relief in patients with spinal stenosis and herniated intervertebral discs.
Humans ; Intervertebral Disc ; Interviews as Topic ; Leg ; Outpatients ; Radiculopathy ; Spinal Stenosis