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

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*

No abstract available.
Gonadal Dysgenesis, 46,XY*

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: Minimally invasive gastrectomy (MIG), including laparoscopic distal subtotal gastrectomy (LDG) and robotic distal subtotal gastrectomy (RDG), is performed for gastric cancer, and requires a learning period. However, there are few reports regarding MIG by a beginner surgeon trained in MIG for gastric cancer during surgical residency and fellowship. The aim of this study was to report our initial experience with MIG, LDG, and RDG by a trained beginner surgeon. MATERIALS AND METHODS: Between January 2014 and February 2015, a total of 36 patients (20 LDGs and 16 RDGs) underwent MIG by a beginner surgeon during the learning period, and 13 underwent open distal subtotal gastrectomy (ODG) by an experienced surgeon in Bundang CHA Medical Center. Demographic characteristics, operative findings, and short-term outcomes were evaluated for the groups. RESULTS: MIG was safely performed without open conversion in all patients and there was no mortality in either group. There was no significant difference between the groups in demographic factors except for body mass index. There were significant differences in extent of lymph node dissection (LND) (D2 LND: ODG 8.3% vs. MIG 55.6%, P=0.004) and mean operative time (ODG 178.8 minutes vs. MIG 254.7 minutes, P<0.001). The serial changes in postoperative hemoglobin level (P=0.464) and white blood cell count (P=0.644) did not show significant differences between the groups. There were no significant differences in morbidity. CONCLUSIONS: This study showed that the operative and short-term outcomes of MIG for gastric cancer by a trained beginner surgeon were comparable with those of ODG performed by an experienced surgeon.
Body Mass Index ; Demography ; Fellowships and Scholarships ; Gastrectomy* ; Humans ; Internship and Residency ; Laparoscopy ; Learning ; Leukocyte Count ; Lymph Node Excision ; Mortality ; Operative Time ; Robotics ; Stomach Neoplasms* ; Surgical Procedures, Minimally Invasive

BACKGROUND: The stratum corneum(SC) has a permeability barrier function which regulates percutaneous absorption by the inhibition of transepidermal water loss(TEWL). Acute mechanical or chemical disruption of the SC induces the impairment of the permeability barrier and so increases the TEWL. The calciumtion has been recognized as an important ion in the recovery of the skin barrier. Recently the main delivery pathway of iontophoretic drugs have been suggested by SC interstices. However the morphologic changes in the SC interstices and calcium after iontophoresis have not been reported. OBJECTIVE: The aim of our study is to confirm that iontophoresis may induce changes in the SC interstices and delay the recovery of the barrier after disruption. MATERIALS AND METHODS: After tape stripping the hairless mouse flank skin, the iontophoresis power supply (6V, 0.6mA) was connected to the patch atiached for 2.5 hours to the stripped site. We checked the THWL 0, 2.5, 6, 12, 18, 24 hours after the tape stripping and obtained specimens and performed osmium tetroxide, ruthenium tetroxide postfixation and calcium ion-capture cytochemical stains for electron microscopic study. RESULTS: The recovery rate of the TEWL in iontophoresis was lower than in the control. This was especially so in the anouse which had received anode iontophoresis for 6 hours. It showed statistically lower TEWL than in the control(p<0.05). Anode iontophoresis induced low calcium in stratum granulosum (SG), but cathode iontophoresis induced high calcium in SC. After iontophoresis there were changes in the SC interstices structures. CONCLUSION: Iontophoresis can induce changes in the SC interstices and calcium distribution and so may help the topical drug delivery system.
Animals ; Calcium ; Coloring Agents ; Drug Delivery Systems ; Electric Power Supplies ; Electrodes ; Iontophoresis* ; Mice ; Mice, Hairless ; Osmium Tetroxide ; Permeability ; Ruthenium ; Skin ; Skin Absorption

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*

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

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