Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital vascular disorder. The skin lesions associated with CMTC include persistent, reticulated vascular patches and telangiectasia, and they are sometimes associated with underlying atrophy and ulceration. The condition is present at birth and tends to improve with age, although some skin lesions remain unchanged throughout life. We encountered two patients with CMTC: a 12-day-old infant and a 21-year-old woman with a history of CMTC since birth. Both patients had localized reticulated purpuric patches with atrophy. During follow-up, although the purpuric patches improved in both patients, there were no changes in the skin atrophy in either patient. Herein, we present these cases showing the changes in the cutaneous features of CMTC over time and demonstrate that CMTC at birth may persist throughout life.
Atrophy ; Female ; Follow-Up Studies ; Humans ; Infant ; Parturition ; Skin ; Telangiectasis ; Ulcer ; Young Adult

We report the CT findings of four cases of squamous cell carcinoma, paying special attention to the epicenter of the lesion and the pattern of bony destruction. All four patients had a past history of chronic otitis media. Squamous cell carcinoma affected mainly the hypotympanum and inferior wall of the external auditary canal, and in all cases revealed an irregular pattern of bony destruction. Irregular destruction of the tegmen tympani occurred in two cases. In cases of squamous cell carcinoma, CT findings suggesting involvement of the promontary are usually noted.
Carcinoma, Squamous Cell* ; Humans ; Otitis Media ; Temporal Bone*

PURPOSE: A hospital-based case-control study was conducted to evaluate the role of glutathione-S-transferase(GST)M1 and GSTT1 genetic polymorphism for developing benign breast disorders(fibrocystic diseases and fibroadenoma) in Korea. MATERIALS AND METHODS: Histologically confirmed incident cases of benign breast disorder(n=56) were selected from inpatients at the Department of General Surgery, Seoul National University Hospital since 1994. Women with free of self-reporting past history of any malignancies were regarded as controls who were selected from the inpatients at the same department at three hospitals during 1994 to 1998(n=180). Information on life-styles including reproductive factors were obtained by direct interview using questionnaire. Age and menopausal status were matched and 51 cases and 102 controls were finally selected. Odds ratio and 95% confidence interval were estimated by multiple logistic regression after adjusting for age, education, body mass index, smoking history, drinking history, menstrual regularity, age at menarch, age at first pregnancy, frequency of fullterm pregnancy, breast feeding history, duration of breast feeding, and family history of breast cancer. RESULTS: GSTM1-null type showed no significance related to the risk of benign breast disorder(adjusted OR=0.8, 95% CI=0.38-1.83) and GSTT1-null type was also not significant(adjusted OR=1.4, 95% CI=0.63-3.29). Increasing tendency of disease risk by the number of GSTs null type was not observed (ptrend>0.1) after adjusting for all other variables. DISCUSSIONS: Further investigation with larger sample size should be needed to provide more concrete information on the role of GST genetic polymorphism in benign breast cancer and a lots of studies relation in normal level of GST genetic polymorphism in general population should be performed.
Body Mass Index ; Breast Feeding ; Breast Neoplasms ; Breast* ; Case-Control Studies ; Drinking ; Education ; Female ; Glutathione* ; Humans ; Inpatients ; Korea ; Logistic Models ; Odds Ratio ; Polymorphism, Genetic* ; Pregnancy ; Sample Size ; Seoul ; Smoke ; Smoking ; Surveys and Questionnaires

The causes of sudden death after medullary infarction involve arrhythmia, central respiratory failure, and dysautonomia. Sudden cardiac arrest in a medullary infarction is uncommon. Most of these cases experienced sudden cardiopulmonary arrest within 2 weeks from stroke onset as the extent of lesion increased. Here, we report two cases of medullary infarction presenting as sudden cardiac arrest. These cases indicate that medullary infarction could be one of the causes of sudden cardiac arrest.
Arrhythmias, Cardiac ; Death, Sudden ; Death, Sudden, Cardiac ; Heart Arrest ; Infarction ; Medulla Oblongata ; Primary Dysautonomias ; Respiratory Insufficiency ; Stroke

A 21-year-old female patient complaining of hemiparesis was diagnosed with right middle cerebral artery infarction. No risk factor was found, despite an extensive young-age stroke work-up, except her history of marijuana use. The patient had smoked marijuana for treating depression for more than five years. Magnetic resonance angiography showed multifocal intra- and extracranial stenoses, suggesting cannabinoid-induced vasculopathy. Since the use of illicit drugs has increased nationwide, physicians should consider it as a possible cause of a stroke due to an unknown etiology.

OBJECTIVE: We examined the difference between IQ(Intelligence Quotient) estimated from computerized Standard Progressive Matrices(SPM) score(SPM IQ) and Korean-Wechsler Adult Intelligence Scale(KWAIS) IQ(KWAIS IQ) in Korean patients with mental disorders in order to test the validity of SPM as a brief nonverbal intelligence test, and to find the groups that need standardization. And, we studied the method to predict KWAIS IQ more precisely from SPM test. METHODS: SPM test in Vienna test system was administered to 166 Korean patients with mental disorders. The degree of consistency and Pearson's correlation coefficient between SPM IQ and KWAIS IQ in total subjects and groups by sex, age, education, and clinical diagnosis were investigated. And, we obtained a regression equation to predict KWAIS IQ from SPM score. RESULTS: In total subjects, Pearson's correlation coefficient between SPM IQ and KWAIS IQ was as high as 0.704, and the percent of patients whose absolute value of difference between SPM IQ and KWAIS IQ is equal to or less than 10 was 54.8%. The degree of consistency between SPM IQ and KWAIS IQ was influenced by age and educational level. SPM IQ were higher than KWAIS IQ in younger age and lower education group. SPM IQ were lower than KWAIS IQ in older age and higher education group. The regression equation from SPM score, age, and education years predicted KWAIS IQ more correctly. CONCLUSION: The Computerized Standard Progressive Matrices test is validated and can be used usefully as a brief nonverbal intelligence test in patients with mental disorders. In order to provide more precise evaluation, standardized data should be prepared for age group 16 to 25 and age group 26 to 35 with lower educational level.
Adult ; Diagnosis ; Education ; Humans ; Intelligence ; Intelligence Tests ; Mental Disorders*

Breast cancer ranks second to stomach cancer as a primary cancer site. An increasing trend in mortality and morbidity of breast cancet has been shown since 1980s. Studies on migrant populations, in which higher incidences were shown than population in their motherlands, indicated the importance of environmental factors on breast cancer development. Older age, family history of breast cancer, early menarche, late menopause, late full-term pregnancy, never-having had a breast-fed child, and postmenopausal obesity were idenrified as risk factors in Korea. These are not different from risk factors detected in western countries. Nevertheless, a ^-shape age-specific incidences surve is shown in Korea shose incidence is relatively low. In western countries, where incidence rates are high, the incidences increase with age in a straight line. This difference may be explained by the "Estrogen-Augmented-:rogesterone Hypothesis" that is relevant to sex hormones. In fact, a nuch lower estradiol levels was reported in Korean women compared to those in American. On the other hand, genetic polymorphism is expected for future research on breast cancer etiology. Only preventive strategies based on epidemiology of breast cancer in Korea may effective to counter this malignancy in Korean women.
Breast Neoplasms* ; Breast* ; Child ; Epidemiology ; Estradiol ; Female ; Gonadal Steroid Hormones ; Hand ; Humans ; Incidence* ; Korea* ; Menarche ; Menopause ; Mortality ; Obesity ; Polymorphism, Genetic ; Pregnancy ; Risk Factors ; Stomach Neoplasms ; Transients and Migrants

PURPOSE: Previous reports demonstrated that nitric oxide (NO) plays immuno-regulatory role in immune responses including allograft rejection response. However, its possible role in xenograft rejection has not been examined. The purpose of this study is to elucidate possible immunoregulatory role of NO in skin xenograft rejection by determining the expressions of chemokines and cytokines in the presence or absence of iNOS inhibitors. METHODS: C57BL/6J mice were grafted with Lewis rat tail skin. The mice were injected intraperitoneally with potent inhibitor of iNOS, aminoguanidine (AMG, 200 mg/kg). Graft survival was monitored and cytokine and chemokine mRNA expressions were measured by real-time RT-PCR in context with iNOS expression on day 3, 5, 7 and 9. These data were compared with those of control mice (saline injected). RESULTS: Compared with the control mice, the AMG treated mice showed delayed xenograft rejection by approximately 3 days (8.9+/-0.7 days vs 11.7+/-1.2 days). Infiltrations of CD11b+, MOMA-2+ cells and neutrophils were significantly reduced but not CD4+ and CD8+ cells in AMG treated graft. The expression of cytokines such as IL-1beta, IL-2, IL-6, IL-12, IFN-gamma in AMG treated graft significantly decreased (P<0.01) whereas IL- 10, TNF-alpha and TGF-beta1 were not changed or enhanced. Additionally, the expression of CC-chemokines such as RANTES and MIP-1alpha significantly reduced (P<0.01) whereas CXC-chemokines such as IP-10 and MIG did not change. CONCLUSION: These data imply that NO suppression by iNOS inhibitor may prolong rat to mouse skin xenograft survival through a selective inhibition of pro-inflammatory cytokines and chemokines. The possible role of NO in transplant rejection can be, therefore, extended to regulation of cytokine and chemokine expressions.
Allografts ; Animals ; Chemokine CCL3 ; Chemokine CCL5 ; Chemokines ; Cytokines ; Graft Rejection ; Graft Survival ; Heterografts* ; Interleukin-12 ; Interleukin-2 ; Interleukin-6 ; Mice ; Neutrophils ; Nitric Oxide ; Rats ; RNA, Messenger ; Skin* ; Tail ; Transforming Growth Factor beta1 ; Transplantation, Heterologous ; Transplants ; Tumor Necrosis Factor-alpha

OBJECTIVES: Vulnerability marker in biological relatives of schizophrenia may be very useful in identifying the propensity for the development of schizophrenia. This study suggests that the relatives at risk for genetically developing schizophrenia have impairments in several domains of cognition, social function and affective function as a vulnerability marker. METHODS: A neurocognitve function test, a social function test and an affective function test were administered to fifteen healthy relatives from families with two or more patients with schizophrenia (geneticallly high risk subjects). Fifteen healthy controls matched for age and gender were recruited in this study. RESULTS: Compared to the control subject, the genetic high risk subjects performed significantly more poorly in the selective attention and recall memory of neurocognitive function. The high risk subjects also had lower scores in independence-performance and independence-competence of the social function test and emotion control of the affective function test. CONCLUSION: Selective attention, recall memory, independence-performance, independence-competence and emotion control ability may be a valuable marker for genetic study of schizophrenia.
Cognition* ; Humans ; Memory ; Schizophrenia*

We report three cases of cystinuria, presenting with urinary stones. A 2-year-old girl presented with urinary difficulty, hematuria, dysuria of sudden onset, and her 7-month-old younger brother also was presented with urinary difficulty, irritability on urination & stone passage. Other 6-month-old boy was admitted due to sudden onset anuria. They had radioopague renal & ureter stones and stone analysis revealed mixed cystine stones. The diagnosis of cystinuria was confirmed metabolic studies and stone analysis. Lrinary amino acid analysis showed excessive excretion of dibasic amino acids(cystine, ornithine, lysine, arginine). And they all had hypercalciuria and hyperuricosuria. They were treated with combination of percutaneous lithotripsy for large obstructing senes a nd an oral drug therapy with sodium bicarbonate for rendering the urine more alkaline, and alpha-mercaptopropionylglycine(ThiolaR). This form of treatment was sucessful in our three cases with elimination of recurrent nephrolithiasis, but in one patient, nephrotic syndrome possibly caused by ThiolaR was developed. The nephrotic syndrome was recovered spontaneously after cessation of Thiola. A review of literatures was also attempted briefly.
Anuria ; Child, Preschool ; Cystine ; Cystinuria* ; Diagnosis ; Drug Therapy ; Dysuria ; Female ; Hematuria ; Humans ; Hypercalciuria ; Infant ; Lithotripsy ; Lysine ; Male ; Nephrolithiasis ; Nephrotic Syndrome ; Ornithine ; Siblings ; Sodium Bicarbonate ; Tiopronin ; Ureter ; Urinary Calculi ; Urination