Infantile myofibromatosis with systemic involvement is a very rare disease and is characterized by numerous nodules composed of spindle cells of a myofibroblastic nature. There are often disseminated throughout the subcutis, muscle, skeleton and viscera. We report an autopsy case of infantile myofibromatosis in a stillborn female fetus of 32 weeks of gestation. The nodules, Imm to 2 cm, were found over the whole body and viscera. The involved viscera were the heart, tongue, esophagus, gastrointestinal tract, portal areas of the liver, spleen anc pancreas. There were also associated malformations, viz., frontal meningoencephalocele, flexion defer-mities, syndactyly, cleft palate, agenesis of corpus callosum, pachygyria, diaphragmatic hemia, renal hypoplasia, etc. Multiple basaloid follicular hamartomas of the skin were noted on the face and extremeties. There are no previous reports in the literature of infantile myofibromatosis in conjunction with the above skin lesion and congenital malformations.
Infant ; Male ; Female ; Humans ; Hamartoma

BACKGROUND: We performed electrical cardioversion for the patients with chormic nonvalvular atrial fibrillation under the transesophageal echocardiographic guidance after anticoagulation to evaluate the safety of this procedure and the effects of electrical cardioversion on the atrial function. METHODS: After anticoagulation therapy with coumadine for three weeks, we tried chemical cardioversion with amiodarone first. Failed cases were included in this study. Pre-cardioversion transesophageal echocardiographic parameters were measured after exclusion of thrombi. After sedation with intravenous midazolam, direct-current cardioversion was done with the transesophageal echocardiographic probe in situ. Immediately after sinus conversion, we measured echocardiographic parameters again. Spontaneous echo contrast(SEC), left atrial appendage flow velocity, pulmonary vein flow velocity and time-velocity-integral(TVI), transmitral flow velocity, TVI and deceleration time were measured. All patients were anticoagulated for at least 4 weeks after cardiovesion. RESULTS: The total number of patients was forty one(24 males, 17 females) with the mean age of 58 years(range : 39-70). Mean duration of atrial fibrillation was 65 months(range : 1-360). Hypertension(12), dilated cardiomyopathy(10), cerebrovascular accidents(6), ischemic heart disease(2) and chronic lung disease(1) were associated. There were no complications. SEC increased or newly appeared in 18(43.9%) patients after sinus conversion. The left atrial appendage emptying velocity decreased(32.8+/-17.4 vs. 22.1+/-11.4cm/sec, p=0.020) and systolic TVI of both upper pulmonic vein increased significantly after sinus conversion. In two cases, early systolic forward flow(S1) of pulmonic vein appeared after sinus conversion. Transmitral E velocity decreased(86.9+/-28.8 vs. 76.3+/-30.6cm/sec, p=0.006) and the deceleration time increased(164+/-49 vs. 206+/-53msec, p=0.000) after sinus conversion. Transmitral A velocity was still low(34.9+/-19.5cm/sec) and E/A ratio was high(2.6+/-1.4) immediately after sinus conversion. CONCLUSION: After appropriate anticoagulation therapy and exclusion of left atrium and left atrial appindage thrombi with TEE we could perform electrical cardioversion safety without complications. The changes in transesophageal echocardiographic parameters after sinus conversion revealed the appearance of atrial mechanical activity in concordance with electrical activity. But these findings suggested atrial stunning or electromechanical dissociation which necessitates extended anticoagulation therapy until the full recovery of atrial mechanical function.
Amiodarone ; Atrial Appendage ; Atrial Fibrillation* ; Atrial Function ; Deceleration ; Echocardiography* ; Echocardiography, Transesophageal ; Electric Countershock* ; Heart ; Heart Atria ; Humans ; Lung ; Male ; Midazolam ; Pulmonary Veins ; Veins ; Warfarin

Biofeedback is the treatment of choice for functional defecation disorders such as idiopathic chronic constipation and neurogenic fecal incontinence. The pre-existing biofeedback systems have many disadvantages. The aims of current project are, first, to develop the biofeedback system into the application software in the Windows environment, and, second, to assess the possibility of clinical usage for patients with functional defecation disorders. The hardware and software of the BASCO (Biofeedback Anal Sphincter Control) system were based on the signal measurement and signal processing of anal sphincter EMG (Electromyography). BASCO system was applied to 5 normal healthy controls and 20 patients with functional defecation disorders. Patients group was categorized as constipation group (N1=15) and incontinence group (N2=5). With use of current system, EMG-based biofeedback therapy was performed, and the outcome was analysed. Anal EMG signal data was processed by the software, and displayed in the monitor of personal computer. The software of EMG-display and database management were adequately operated. In N1 group, a paradoxical elevation or equalized activity of anal EMG pattern was shown in the simulated defecation. In N2 group, low electrical activity was shown. These findings were used for the EMG-based biofeedback therapy as a pilot study. The clinical symptoms were improved in 12 of N1 group and 3 of N2 group in the period of 3.7 (range, 1~12) months follow-up. In Conclusion, newly-developed BASCO system was adequately operated in the volunteer and patients groups. The multi-tasking and multi-processing functions were adequately shown in the real time. Current results could be used for clinical appraisal. Specifically, this system could be used for the practical application of biofeedback therapy in the patients with chronic constipation or fecal incontinence.
Anal Canal* ; Biofeedback, Psychology* ; Constipation ; Defecation* ; Fecal Incontinence ; Follow-Up Studies ; Humans ; Microcomputers ; Pilot Projects ; Volunteers

BACKGROUND: Video assisted thoracic sympathectomy or sympathicotomy is a safe and effective therapy for the treatment of palmar hyperhidrosis with immediate symptomatic imporvement. However the degree of satisfaction may diminish with time due to cmpensatory sweating or excessive hand dryness. Therefore by comparing and assessing the degree of symptomatic improvement or compensatory sweating following sympathectomy or sympathicotomy at various levels we aim to determine the optimal level of sympathetic nerve block which will result in minimal side effects and maximal benefit. MATERIAL AND METHOD: Among 194 patients having undergone video assisted thoracic sympathectomy or sympathicotomy between January 1996 and June 1999, 137 patients who responded to either telephone interview or questionnaire were included in the current study. The patients were divided into three groups. Group I(n=61) ; patients having undergone T2,3,4 sympathectomy group II(35) ; patients having undergone T2 sympathicotomy and group III(41) ; patients having undergone limited T2 sympathicotomy which consist of block of interganglionic neuronal fiber on the third rib. The parameters studied comprised of pre- and post-operative palmar temperature change treatment satisfaction the degree of compensatory sweating or discomfort from palmar dryness postoperative complication and changes in plantar sweating. RESULT: There was no difference in age and sex among the groups and the mean postoperative elevation in palmar temperature was 21.59degrees C without any differences among the groups. Patients expressing satisfaction were 65.6%, 62.9% and 90.24% in groups I, II and III, respectively(p<0.05) Moderate to severe compensatory sweating was present in 65.6% 51.4%, and 24.39%, in group I, II, and III, respectively (p<0.05) Slight but comfortable amount of palmar humidness was expressed in decreasing order group III(41.6%) group I(24.6%) and group II(5.7%) (p<0.05) Ineffectiveness or recurrence was present in 5patients in group I(8.2%) 1 patient in group II(2.9%) and none in group III. With regards to plantar sweating decrease in sweating was expressed in 43 patients(31.4%) while similar degree of sweating in 61 patients(44.5%) and increase in sweating in another 33 patinets(24.1%). CONCLUSION: Limited T2 sympathicotomy resection of the lower interganglionic neuronal fiber of the second sympathetic ganglion on the third rib showed immediate effect in palmar hyperhidrosis and caused lesser compensatory sweating and hand dryness.
Autonomic Nerve Block ; Ganglia, Sympathetic ; Hand ; Humans ; Hyperhidrosis* ; Interviews as Topic ; Neurons ; Postoperative Complications ; Surveys and Questionnaires ; Recurrence ; Ribs ; Sweat ; Sweating ; Sympathectomy

PURPOSE: A case of frosted branch angiitis in Kikuchi-Fujimoto disease is reported. CASE SUMMARY: A 33-year-old male complained of a sudden decrease in visual acuity that developed in both eyes 5 days prior. He suffered from a headache, chills, myalgia, and flank pain 1 week before. The initial best-corrected visual acuity (BCVA) was 0.1 in the right eye and 0.2 in the left eye. On slit lamp examination, no inflammatory finding was observed in the anterior chamber and vitreous body of both eyes. On fundus examination, a diffuse vascular sheathing-like frosted branch was found in the retinal vessels, and retinal hemorrhage was observed. Fluorescein angiography showed staining and leakage of dye along the vascular sheathing. Serological findings were negative, showing no evidence of an autoimmune disease or viral infection. Neck ultrasonography revealed non-tender left cervical lymph node enlargement >1 cm in diameter. Ultrasound-guided fine needle aspiration cytology showed findings compatible with Kikuchi-Fujimoto disease, including necrotic changes and pronounced karyorrhexis, plus histiocyte and lymphocyte infiltration without neutrophils. We started systemic steroid therapy. One month after treatment, the BCVA of both eyes improved to 1.0. CONCLUSIONS: In patients with frosted branch angiitis, systemic disease such as Kikuchi-Fujimoto disease should be considered.
Adult ; Anterior Chamber ; Autoimmune Diseases ; Biopsy, Fine-Needle ; Chills ; Flank Pain ; Fluorescein Angiography ; Headache ; Histiocytes ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Lymph Nodes ; Lymphocytes ; Male ; Myalgia ; Neck ; Neutrophils ; Retinal Hemorrhage ; Retinal Vessels ; Slit Lamp ; Ultrasonography ; Vasculitis ; Visual Acuity ; Vitreous Body

Statistical observation was performed on 2,856 in-patients in the Department of Urology, Korea University Hospital, during the period from January 1, 1963 to December 31, 1979.
Korea ; Tuberculosis ; Urolithiasis ; Urology

We followed up the 100 patients (104 eyes) who had undergone cataract extraction and PCL implantation into the bag with 3M diffractive multifocal intraocular lensee for at least 2 month, in order to evaluate the function of this lens. 92 eyes (88.5%) had the far corrected vision more than 0.5 and 94 eyes (90.4%) had the near vision with far correction more than J3. We added additional adds (-0.25, +0.25, +0.50, +0.75D) in order to ascertain the adequacy of +3.50D add in this lens and it was found that the additional add of +0.75D was most preferred. This means that more add is necessary for near vision. There was, also, no more remarkable side effects or difficulties associated with this lenses. So, multifocal Intraocular lenses enable good near vision without any severe disadvatages but the amount of near add is cuntroversial.
Cataract Extraction ; Humans ; Lenses, Intraocular*

Neurilemmoma is the most frequent solitary tumor of the peripheral nerves. It is a tumor from Schwann cells, and it is usually solitary and benign. Treatment of this benign lesion is usually by excisional biopsy. This report concerns a rare case of neurilemmoma developed in the penis in a 51 years old male and the relevant literature has been reviewed.
Biopsy ; Humans ; Male ; Middle Aged ; Neurilemmoma* ; Penis* ; Peripheral Nerves ; Schwann Cells

Endometriosis is defined as the presence of endometrial tissue(gland and stroma) outside the uterus. The incidence of scar endometriosis is quite rare, and must differentiate with cellulitis and abscess. We have experienced one case of perineal endometriosis and one case of abdominal wall endometriosis at the site of postoperative wound scar. The possible pathogenesis of endometriosis and treatment were discussed.
Abdominal Wall ; Abscess ; Cellulitis ; Cicatrix* ; Endometriosis* ; Female ; Incidence ; Uterus ; Wounds and Injuries

BACKGROUND: Carbapenem-resistant Enterobacteriaceae (CRE) with acquired metallo β-lactamase (MBL) resistance have been increasingly reported worldwide and associated with significant mortality and morbidity. Here, an outbreak of genetically related strains of Klebsiella pneumoniae producing the imipenemase (IMP)-1 MBL in a medical intensive care unit (MICU) in Korea is reported. METHODS: Since isolating carbapenem-resistant K. pneumoniae (CRKP) at the MICU of the hospital on August 10, 2011, surveillance cultures for CRE in 31 hospitalized patients were performed from August to September 2011. Carbapenem resistance was determined based on the disk diffusion method outlined in the Clinical and Laboratory Standards Institute guidelines. Polymerase chain reaction (PCR) was performed for genes coding for β-lactamase. Associations among isolates were assessed via pulsed-field gel electrophoresis (PFGE). In addition, a surveillance study of environmental cultures and health-care workers (HCWs) was conducted in the MICU during the same time frame. RESULTS: During the study period, non-duplicated CRKP specimens were discovered in four patients in the MICU, suggestive of an outbreak. On August 10, 2011, CRKP was isolated from the sputum of a 79-year-old male patient who was admitted to the MICU. A surveillance study to detect additional CRE carriers by rectal swab revealed an additional three CRKP isolates. PCR and sequencing of the four isolates identified the presence of the IMP-1 gene. In addition, PFGE showed that the four isolated strains were genetically related. CRE was not identified in specimens taken from the hands of HCWs or other environmental sources during surveillance following the outbreak. Transmission of the carbapenemase-producing Enterobacteriaceae strain was controlled by isolation of the patients and strict contact precautions. CONCLUSIONS: This study shows that rapid and systemic detection of CRE and strict infection controls are important steps in preventing nosocomial transmission.
Aged ; Clinical Coding ; Critical Care* ; Diffusion ; Disease Outbreaks ; Drug Resistance, Bacterial ; Electrophoresis, Gel, Pulsed-Field ; Enterobacteriaceae ; Hand ; Humans ; Infection Control ; Intensive Care Units* ; Klebsiella pneumoniae* ; Klebsiella* ; Korea ; Male ; Methods ; Mortality ; Pneumonia ; Polymerase Chain Reaction ; Sputum