The phenomenon of Myotonia consist in a failure of voluntary muscles to relax immediately when voluntary innervation ceases. The stiffness is accentuated by cold and relieved by exercise, while generalized muscle weakness and atrophy (or not commonly hypertrophy of muscle) is common. Myotonia is a feature of four principal clinical syndromes which have been classified myotonia congenita (Thomsens disease), myotonia atrophica (Steinerts disease), paramyotonia and myotonia and myotonia acquisita by Walton et al. We report here a forty six years old male of myotonia atrophica (Dystrophia myotonia, Steinerts disease) who presents bilateral cataracts, frontal baldness, gonadal atrophy, facial myopathy, sterno-cleidomastoid muscle atrophy and a progressive generalized myopathy of peripheral distribution in the limbs.
Alopecia ; Atrophy ; Cataract ; Extremities ; Gonads ; Humans ; Hypertrophy ; Male ; Muscle Weakness ; Muscle, Skeletal ; Muscular Atrophy ; Muscular Diseases ; Myotonia Congenita ; Myotonia ; Myotonic Dystrophy

No abstract available.
Gonadal Dysgenesis, 46,XY*

Contact Urticaria is a wheal-and-flare response to chemicals applied to the skin. Contact urticaria due to bee sting therapy has never been reported in Korea. A 45-year-old male was seen for erythematous plsques and patches with the duration of 3 days. Histologicall, the skin biopsy specimen showed necrosis and dyskeratotic cells in the epidermis. Collagen degenerati in and perivascular lymphocytic infiltrates were seen in the upper dermis.
Bees* ; Biopsy ; Bites and Stings* ; Chronic Pain* ; Collagen ; Dermis ; Epidermis ; Humans ; Korea ; Male ; Middle Aged ; Necrosis ; Skin ; Urticaria*

The effects and safety of using oral Nicardipine(NCP, Perdipine(R)) a calcium blocking agent of the dihydropyridine derivative, were assessed in 29 patients(14 males, 15 females, mean age 56.3 years) with essensed hypertension with complications of left ventricular hypertrophy and/or hypertensive retinopathy. The patients with a sitting blood pressure(BP) of more than 160/100mHg at 4th week while off all antihypertensive agents, were entered into the study and recieved NCP, 20mg tid for 2 weeks, BP being measured once every week. NCP significantly reduced. The systolic BP from 179.5+/-18.2mmHg to 143.4 +/-18.2mmHg, the diastolic BP from 112.9+/-16.2mmHg to 93.1+/-10.4mmHg and the mean BP from 135.2+/-11.7mmHg to 110.0+/-9.9mmHg(mean+/-S.D. of weekly BP for 4 weeks)(P<0.001). Fall in systolic, distolic and mean BP was 35.1+/-15.1mmHg, 20.0+/-8.3mmHg and 25.3+/-10.2mmHg, respectively. A fall in systolic BP of more than 20mmHg, in diastolic BP of more than 10mmHg and in mean BP of more than 13mmHg was obtained in 86.2%, 89.6% and 86.2% of the cases, respectively. The heart rate did not change with oral NCP. The side effects were headache, facial flushing, dizziness and palpitation, but they were tolerable. These finding suggest that NCP is an effective and safe drug for the treatment of sustained essential hypertension.
Antihypertensive Agents ; Calcium ; Dizziness ; Female ; Flushing ; Headache ; Heart Rate ; Humans ; Hypertension* ; Hypertensive Retinopathy ; Hypertrophy, Left Ventricular ; Male ; Nicardipine*

BACKGROUND: Extravasation of chemotherapeutic agents during intravenous infusion may cause extensive inflammatory reaction, ulceration, crust formation, and skin necrosis. But the exact mechanism of cutaneous reactions after extravasation of adriamycin remains unknown. OBJECTIVE: Our purpose was to evaluate the clinical and histological features in rabbits after injection of adriamycin and a mixed solution of adriamycin and triamcinolone. METHODS: Three rabbits were divided into three groups and were subcutaneously injected with the following materials on the back:adriamycin, a mixed solution of adriamycin and triamcinolone and 0.9% saline respectively. The injection sites including the skin and subcustaneouskfat were excised and fixed with natural huffered formalin. The biopsied specimens were stained with hematoxylin and eosin. RESULTS: l. In the adriamycin injected group, there were induration, crust formation and ulceration after three weeks. After seven weeks crust and focal ulceration were found. Histologically, after one day mild epidermal necrosis, dermal inflammatory infiltration, destruction of epidermal appendag es, edema and hemorrhage were seen. After one week collagen necrosis was advanced and granulation tissue formation and calcium deposition were found. After seven weeks granulation tissue formation and calcium d position still remained. 2. In the adriamycin and triamcinolone injected group, focal healing ulceration was seen after three weeks. After seven weeks the ulceration healed and normal gross finding was seen. Histologically, destructior of epidermal appendages and edema were found after one day. After three weeks granulation tissue formation and fatty changes in the dermis were seen. After five weeks normal histologic findings were seen in the epidermis. Collagen necrosis became less prominent and hyalinizationand transepidermal elimination were found. After seven weeks nearly normal histological findings in the epidermis and dermis were seen. CONCLUSION: We conclude that there was a marked difference in the gross and histological findings between two groups of adriamycin injected and a mixed solution of adriamycin and triamcinolone injected rabbits. Therefore, we suggest triamcinolone can be used as an adjuvant mode in the treatment of lesions where adriamycin is extravasated.
Calcium ; Collagen ; Dermis ; Doxorubicin* ; Edema ; Eosine Yellowish-(YS) ; Epidermis ; Formaldehyde ; Granulation Tissue ; Hematoxylin ; Hemorrhage ; Infusions, Intravenous ; Injections, Intralesional* ; Necrosis ; Rabbits ; Skin ; Triamcinolone ; Ulcer

PURPOSE: The purpose of this study was to evaluate effect of alpha-lipoic acid on apoptotic cell death in rat hippocampal neuron following transient forebrain ischemia-reperfusion (I/R) injury. METHODS: The four-vessel occlusion method was used to induce transient I/R injury in the forebrain of Sprague-Dawley rats. In the treatment group, alpha-Lipoic acid (LA) was administered subcutaneously at 50 mg/kg/day for 7 days before induction of I/R injury. RESULTS: Pretreatment with LA significantly reduced the number of TUNEL-positive neurons in the pyramidal cells layer of the hipocampal CA1 region 5 days after the ischemia, suggesting a marked reduction of apoptotic cell death. Pretreatment with LA also resulted in marked suppression at the transcript level of mRNA for caspase-3 at 24 hours, and decreased concentration of the active form of caspase-3 protein in the hippocampus at 1, 3, and 5 days after I/R injury. Furthermore, as indicated by western blot analysis, the concentration of apoptosis-inducing factor (AIF) in the hippocampus was reduced at 1 and 3 days after a transient I/R injury by pretreatment with LA. CONCLUSION: The results of this study suggest that LA has the potential to prevent neuronal cell death in the hippocampus by inhibiting intracellular signaling pathways responsible for apoptosis following transient I/R injury.
Animals ; Apoptosis ; Apoptosis Inducing Factor ; Blotting, Western ; Caspase 3 ; Cell Death* ; Hippocampus* ; Ischemia ; Neurons ; Prosencephalon* ; Pyramidal Cells ; Rats* ; Rats, Sprague-Dawley ; Reperfusion Injury* ; RNA, Messenger ; Thioctic Acid*

No abstract available.
Nitric Oxide* ; Paclitaxel* ; Urinary Bladder Neoplasms* ; Urinary Bladder*

PURPOSE: The purpose of this study was to evaluate spatiotemporal evaluation of pERK1/2 protein expression in the forebrain following hypoxic-ischemic (HI) injury in adult Sprague-Dawley rats. METHODS: HI injury was induced by occlusion of the bilateral common carotid artery (CCA) and respiration with 5% O2 hypoxic gas for 8 minutes, followed by unilateral release of CCA. RESULTS: Immunoreactivity for pERK1/2 protein in the bilateral cortex began to increase at 2 hours, reached peak levels at 6 hours, and then decreased by 24 hours after HI injury. In a cortical neuron, the expression of pERK1/2 protein was observed in all cellular components and processes including dendrites, cell body and nuclei at 6 hours, but persisted only in the cell body by 24 hours after HI injury. Temporal changes in the immunoreactivity for pERK1/2 protein in the hippocampus was very similar to that of the cortex following HI injury. In contrast, the temporal changes in the cellular distribution of pERK12 protein in hippocampal neurons was largely different from that of the cortex following HI injury. CONCLUSION: The results of the present study suggest that HI injury causes an early activation of ERK1/2 signaling with a differential cellular distribution of pERK1/2 protein among different forebrain structures. Further study needs to be done in order to elucidate a possible role of ERK1/2 signaling for neural damage in the adult rodent HI model.
Adult ; Brain ; Carotid Artery, Common ; Dendrites ; Extracellular Signal-Regulated MAP Kinases ; Hippocampus ; Humans ; Neurons ; Prosencephalon ; Respiration ; Rodentia

Familial adenomatous polyposis(FAP) is a rare hereditary disorder characterized by the development of hundreds to thounds polyps throughout the colon and rectum. Moreover, several extracolonic manifestations are seen. Recently, this disease is recognized as a adenomatous polyposis syndrome which can involve the entire astrointestinal tract. Several reports have demonstrated a high incidence of gastroduodenal polyps in patients with familial adenomatous polyposis. These colon polyps can be eventually developed as colon cancer, if not be treated. So early diagnosis is needed and prophylactic surgery should be erformed. We experienced a case of familial adenomatous polyposis with a large sentinel polyp on the sigmoid colon, presenting hematochezia and mucoid diarrhea. He was early treated before progression to carcinoma by total colectomy, rectal mucosectomy and J pouch ileoanal anastomosis.
Adenomatous Polyposis Coli* ; Colectomy ; Colon ; Colon, Sigmoid ; Colonic Neoplasms ; Colonic Pouches ; Diarrhea ; Early Diagnosis ; Gastrointestinal Hemorrhage ; Humans ; Incidence ; Polyps* ; Rectum

To estimate the incidence of uterine cervix cancer among Korean women, we have conducted a study using the claim data on the beneficiaries of Korea Medical Insurance Corporation(KMIC). All medical records of the potential cases with diagnosis of ICD-9 180, 181, 182, 199, 219, 233 in the claims sent by medical care institutions in the whole country to the KMIC from January 1988 to December 1989, were abstracted and Gynecology specialist reviewed the records to identify the new cases of uterine cervix cancer among the potential cases during the corresponding period. Using these data, the incidence of uterine cervix cancer among Korean women was estimated as of July 1, 1988 to June 30, 1989. The crude rate was estimated to be 17.34(95% CI: 16.76~17.92) per 100,000 and the cumulative rates for the ages 0~64 and 0~74 were 1.7% and 2.2%, respectively. The age-adjusted rate for the world population was 19.93 per 100,000 which was higher than those of other Asian countries including China and Japan in 1983~1987. The truncated rate for ages 35~64 was 52.05 per 100,000 which was one of the highest in the world. With increasing age, the incidence rate increased to 78.11 per 100,000 in women aged 55~59 years, then it decreased in the older groups. This finding suggests that detecting rate of uterine cervix cancer may decrease in women aged 60 years or older due to detecting rate of uterine cervix cancer may decrease in women aged 60 years or older due to inadequate medical care seeking behavior. In the geographical area, the SIR of Jeju province was significantly low but it might be due to statistical unstability by small case numbers.
Asian Continental Ancestry Group ; Cervix Uteri* ; China ; Diagnosis ; Epidemiology ; Female ; Gynecology ; Humans ; Incidence* ; Insurance ; International Classification of Diseases ; Japan ; Korea ; Medical Records ; Specialization