We experienced a case of Rotor syndrome in an 8 year 6 month old boy who presented with icteric sclera and icteric skin on whole body. His clinical and laboratory characteristics were as follows; 1) Jaundice appeared from several months ago before admission without any clinical disturbance. 2) Direct bilirubin was more increased than indirect bilirubin. 3) Plasma indocyanine green (ICG) kinetics test revealed hepatic excretory in this patient that after intravenous injection of a standard dose of ICG (0.5mg/kg), its initial plasma disappearance is decreased, resultin in markedly increased retention of the dye 45 to 50 minutes after administration. 4) Oral cholangiography, ultrasonogram, and computed tomogram of abdomen were normal. 5) Liver biopsy specimen showed no dark brown pigmentations in the hepatic cells and any other pathologic abnormalities. 6) Nearly similar clinical and laboratory findings were observed 26 months later.
Abdomen ; Bilirubin ; Biopsy ; Cholangiography ; Hepatocytes ; Humans ; Hyperbilirubinemia, Hereditary* ; Indocyanine Green ; Infant ; Injections, Intravenous ; Jaundice ; Kinetics ; Liver ; Male ; Pigmentation ; Plasma ; Sclera ; Skin ; Ultrasonography

To elucidate the clinical characteristics-especially its clinical pattern and outcome-of children with encephalopathy, we carried out a retrospective review on medical records of 45 patients who were initially diagnosed as encephalopathy except mumps meningitis, Reye's syndome and neonatal hypoxic ischemic encephalopathy at the Department of pediatrics, Hanyang University children's hospital from Jaunary 1986 to February 1994. The results were summarized as follows: 1) Twenty five were male and twenty were female with male to female ratio 1.3:l. 2) There were no association with death rate and conscious level, abnormal liver function test, and sugar level in CSF on admission. 3) Death rate (33.3%) of group with seizure was higher than that (13.3%) of group without seizure on admission, but there was no statistical significance (p=0.28). 4) Death rate (7.5%) of group (l) which initial therapy started before 12hours was lower than that (43.4%) of group (ll) which initial therapy started after 12hours, but there was no statistical significance (p=0.09). However complete recovery rate (66.7%) of the former group was higher than that (26.7%) of the latter group with statistical significance (p<0.05). Therefore, early diagnosis and treatment before 12hours was essential to recovery and good prognosis in childern with acute encephalopathy.
Child* ; Early Diagnosis ; Female ; Humans ; Hypoxia-Ischemia, Brain ; Liver Function Tests ; Male ; Medical Records ; Meningitis ; Mortality ; Mumps ; Pediatrics ; Prognosis ; Retrospective Studies ; Seizures

No abstract available.
Child* ; Humans ; Prognosis*

Most neonatal cutaneous hemangiomatosis are complicated by visceral involvement and. are associated with a high mortality rate in the first month of life. Some neonates with multiple cutaneous hemangiomas, however, may follow a benign course of spontaneous resolution without visceral involvement. Such cases are called benign neonatal hemangiomatosis (BNH). BNH is characterized by a lack of mucosal or symptomatic visceral involvement. rapid spontaneous regression of cutaneous hemangimas, and an excellent prognosis. We report herein a case of BNH which is atypical compared to previously reported BNFI cases. Our patient had a more prolonged course than those of usual cases with BNH and showed a conjunctival involvement.
Hemangioma* ; Humans ; Infant, Newborn ; Mortality ; Prognosis

Dariers disease is a domirantly inherited skin disease that is characterized by keratotic papules predominantly on the sebornieic areas, palmar pits, and a nail dystrophy. The diagnosis is difficult in the small group with flexural involvement because the features of flexural Dariers disease overlap clinically and histolopica.lly with those of Hailey-Hailey disease. The diagnosis of Dariers disease may be confirmed if the patient has the pathognomonic nail dystrophy. We presents a case of flexured. Dariers disease in a 65 year old woman. She has suffered from skin lesions which have some resemblance to those of Hailey-Hailey disease. We could diagnose her skin lesions as Dariers disease because she had the eharacteristic nail dystrophy.
Aged ; Darier Disease ; Diagnosis ; Female ; Humans ; Pemphigus, Benign Familial ; Skin ; Skin Diseases

BACKGROUND: Osteoporosis is an important health concern among the postmenopausal women. Therefore, it is necessary to find out acceptable screening tools for osteoporotic patients. The purpose of this study was to evaluate the Risk Index by OSTA as a screening test for osteoporosis. METHODS: The study population was 322 naturally caused menopausal women in Korea. Their was of femoral neck and lumbar spine BMD measured by DEXA. Risk Index by OSTA, based on weight and age, was calculated and the sensitivity and specificity for osteoporosis and osteopenia were evaluated. To find out the proper cut-off point for osteoporosis and osteopenia among the postmenopausal women in Korea, we also compared the sensitivity and specificity of each Risk Index value. RESULTS: The mean age and ages at menopause were 59.1(+/-6.2) and 50.7 (+/-2.7) years, respectively. The prevalence of osteoporosis was 9.6% with femoral neck BMD. For oeteoporosis, using a cut-off point of -1 yielded a sensitivity of 74.3% and a specificity of 52.2% with femoral neck BMD. Using a cut-off of -2 yielded a sensitivity of 87.3% and a specificity of 70.9%. The ROC curve showed an AUROC 0.88 for Risk Index in identifying osteoporosis. CONCLUSION: The Risk Index is an acceptable, simple and useful method in the diagnosis of osteoporosis with a Risk Index of -2 in Korean postmenopausal women.
Bone Diseases, Metabolic* ; Diagnosis ; Female ; Femur Neck ; Humans ; Korea ; Mass Screening* ; Menopause ; Osteoporosis* ; Prevalence ; ROC Curve ; Sensitivity and Specificity ; Spine

Melanoma is relatvely uncommon malignant tumor arising in the skin from epidermal melanocytes, from the junctional component of a cellular nevus or, rarely within a blue nevus and frequently metastasizes to regional lymph nodes first, followed by hematogenous spread to lungs, liver, gastrointestinal tract, pancreas, adrenal, heart and brain. All melanoma patients are carefully evaluated according to several parameters. A complete history and physical examination are carried out, with particular attention being devoted to other pigmented tumors that the patient may have. The patient should be further evaluated to determine the presence or absence of metastases by chest X-ray examination, metastatic series or bone scan, liver function studies, liver scan, EKG, etc. Special factors such as age, sex, and race, as well as site, size, and growth rate of lesions are also evaluated. We had a case of 59 year-old male patient with disseminated malignant melanoma. Upon his first visit, we did not find any abnormality in his physical examination and routine laboratory findings except skin tumors on left heel and left leg.
Brain ; Continental Population Groups ; Electrocardiography ; Gastrointestinal Tract ; Heart ; Heel ; Humans ; Leg ; Liver ; Lung ; Lymph Nodes ; Male ; Melanocytes ; Melanoma* ; Middle Aged ; Neoplasm Metastasis ; Nevus ; Nevus, Blue ; Pancreas ; Physical Examination ; Skin ; Thorax

The provision of adequate soft tissue cover for a complex hand and forearm wound-dirty, infected and exposed bone can be a challenging problem. Conventionally, a distant flap or free flap have been used, but these have been associated with several problems such as joint stiffness, risk of microsurgical procedures, negligible donor site morbidity, prolonged operation time and economic restriction. As well, fasciocutaneous flap has been the limitation of infection control. In these situations, the ipsilateral muscle flap provides the ideal cover with its rich blood supply and single operative field. Previously described by Lendrum(1980), by Lae et al.(1981), and later by McGeorge(1991), the brachioradilis muscle is the most superficial muscle on the radial side of the forearm. Its flap is based on the radial artery and venae comitantes. From August 1996 to October 1997, we performed branchioradialis muscle flap in seven patients. Despite the sacrifice of the major hand artery, brachioradialis muscle flap has many advantages including the reliability of flap survival, easy dissection, early mobilization and absence of functional and aesthetic problems at the donor site. It is a relatively simple and rapid method for resurfacing soft tissue defect in the hand and forearm. We obtained satisfactory and reliable results.
Arteries ; Early Ambulation ; Forearm* ; Free Tissue Flaps ; Hand* ; Humans ; Infection Control ; Joints ; Radial Artery ; Tissue Donors

Measurement of the normal pituitary gland height was performed on magnetic resonance (MR) images of 144 subjects with no known or suspected pituitary or hypothalamic diseases. Midsagittal T1 weighted images (T1WI) were used for measurement, and mean vertical height according to age and sex group was obtained. In all age groups, the pituitary height was greater in females than in meles, The group of 0-9 years in both genders showed the minimum mean pituitary height, The maximum mean height was observed in the 10-19 years age group in both genders. The height gradually decreased with increasing age after age 20 years. There was no subject with a height more than 9.0 mm in females or 8.0 mm in males. In conclusion the measurement of the normal pituitary pland height using mid sagittal MR imaging can be used for the evaluation of the pituitary gland lesions.
Female ; Humans ; Hypothalamic Diseases ; Magnetic Resonance Imaging ; Male ; Pituitary Gland* ; Sex Differentiation*

The serum level of immunoglobulin E (IgE) in 28 atopic patients and 41 healthy, normal, non-allergic subjects were measured by standard radial immunodiffusion method (RIDT). The correlation between IgE level and clinical manifestations are studied. The results are as follows. 1) IgE value was higher in atopic dermatitis than normal subjects. 2) IgE value in atopie dermatitis revealed no sigaificant difference between in male and in female. 3) IgE value was increased parallel with the severity of the disease. 4) IgE value was not correlated with the extent of disease. 5) IgE value was not correlated with the duration of disease. 6) IgE value was not correlated with the age of onset. 7) IgE value was not correlated with the eosinophil count of peripheral blood.
Age of Onset ; Dermatitis ; Dermatitis, Atopic* ; Eosinophils ; Female ; Humans ; Immunodiffusion ; Immunoglobulin E* ; Immunoglobulins ; Male