Seckel syndrome is a rare, autosomal recessive disorder of severe growth retardation and distinct craniofacial, orodental, and skeletal anomalies. We report hereby the first two Korean cases of typical Seckel syndrome who had characteristic symptoms of intrauterine growth retardation, small head, large eyes, sharp facial features (beaked nose, dysplastic ears and narrow face) with underdeveloped chin, dwarfism, severe mental retardation, and other malformation. We report two cases of Seckel syndrome with a brief review of related literatures.
Chin ; Dwarfism ; Ear ; Fetal Growth Retardation ; Head ; Intellectual Disability ; Nose

BACKGROUND: Recently, the incidence of acute myocardial infaction has been gradually increasing as prolongation of life spans and improvements of diet and life styles in Korea. The rate of mortality and sudden death is higher than other diseases. The purpose of this study is to evaluate factors which can affect on the mortality of AMI during initial 24 hrs. METHODS: A retrospective clinical study was done on 364 consecutive patients with AMI who had been presented to Keimyung University Dong-sang Medical Center from January 1990 to May 1997(M:F ratio=254:110). The subjects were divided two groups. The Group I was patients who had expired during the initial 24hrs period of AMI(47 patients, 13%), the Group II was patients who had survived(317 patients, 87%). We compared clinical features, EKG, laboratory results in both groups and tried to analyse the vulnarable factors. RESULTS: The results were as follows; 1) The mean age in Group I (64.4 yearly) was older than in Group II(61.3 yearly) and female gender was also higher in Group I. The mean systolic/diastolic blood pressures of the Group I(103/61mmHg) were lower than those of the Group II(123/75mmHg). 2) The chest pain and mental change were noted more frequently in Group I than in Group II and the dyspnea was less frequent in Group I than Group II. 3) The higher grades of Killip classification was significantly more frequent in Group I than in Group II. 4) The mean onset to drug time for thrombolytics in Group I and Group II were 14.1 hrs and 6.6 hrs. 5) The mortality rate of the Group I and the Group II were respectively 13%, 6.6%. The most common causes of death were cardiogenic shock and ventricular arrhythmia. CONCLUSION: The mortality rate of initial 24 hrs of onset as form of sudden death was higher than post-24hrs group(66.2%). The factors for the higher mortality group were old age and female gender, and they were unstable in vital signs, higher Killip classifications. Therefore, these groups demand more rapid and aggressive approach than the other groups.
Arrhythmias, Cardiac ; Cause of Death ; Chest Pain ; Classification ; Death, Sudden ; Diet ; Dyspnea ; Electrocardiography ; Female ; Humans ; Incidence ; Korea ; Life Style ; Life Support Care ; Mortality* ; Myocardial Infarction* ; Retrospective Studies ; Risk Factors* ; Shock, Cardiogenic ; Vital Signs

No abstract available.
Hernia*

No abstract available.
Hydrops Fetalis*

No abstract available in English.
Classification ; Clinical Study

We retrospectively evaluated the 20 patients who underwent the Raz operation for genuine stress urinary incontinence from January, 1993 to January, 1994. The mean periods of follow-up was 11.1+/-4.3 months. Four patients with grade I stress incontinence, twelve patients with grade II stress incontinence and four patients with grade III stress incontinence underwent the Raz bladder neck suspension. All patients had no previous anti-incontinence operation. Seventeen patients(85%) were cured, while two patients(10%) were significantly improved with only rare episodes of incontinence not requiring protection. One(5%) was failure. When the results were stratified by degree of incontinence preoperatively 4 of 4 patients(100%) with mild, 11 of 12(9l.7%) with moderate and 4 of 4(100%) with severe incontinence had a successful outcome. Namely, 95 percent of the patients were either cured or improved. The complications were not significant except temporary urinary retention of 2 to 20 days, there was no permanent urinary retention. The advantages of a needle suspension procedure, as compared with the abdominal retropubic techniques, include simplicity, less operative time, decreased postoperative morbidity, and a shorter hospital stay, because it avoids an extensive abdominal operation. In addition, the vaginal approach enables the surgeon to perform concomitant pelvic floor surgery for other conditions, such as cystocele, rectocele, enterocele, and uterine prolapse. Therefore, in a relatively short-term follow-up and a small group, Raz bladder neck suspension seems to be the ideal procedure for the woman presenting with stress urinary incontinence.
Cystocele ; Female ; Follow-Up Studies ; Hernia ; Humans ; Length of Stay ; Neck ; Needles ; Operative Time ; Pelvic Floor ; Rectocele ; Retrospective Studies ; Urinary Bladder ; Urinary Incontinence* ; Urinary Retention ; Uterine Prolapse

GRP was known as the modulator of pain transmission in central nervous system and local effector to peripheral tissue causing vasodilation, increased blood flow, modulation of immune system, stimulation of endothelial cell proliferation, and stimulation of bone formation. Numerous study, therefore, were done to elucidate involvement of CGRP to tooth movement. To investgate the response of CGRP immunoreactive nerve cells according to cell size in trigemeinal ganglion during tooth movement, immunohistochemical study was performed using rat. Experimental rats(9 weeks old, 210 gm) were divided as six groups(normal(n=6), 3 hours group(n=5), 12 hour group(n=4), 1 day group(n=5), 3 day group(n=5), 7 day group(n=5)), and were applied orthodontic force (approximately 30 gm) to upper right maxillary molar. After frozen sections of trigeminal ganglions were immunostained using rabbit antisera, the changes of CGRP immunoreactive cells in regard to cell size distribution(small cell(up to 20 microgramm), medium cell(20-35 microgramm), large cell(above 35 microgramm)) were observed. The results were as follows 1. The percentage of CGRP immunoreactive cells to all nerve cells in trigeminal ganglion was 33.0% in normal control group, was decreased to 24.5% in 1 day group, and was increased to 41.8% in 7 day group. 2. The percentage of small, medium, and large cells expressing CGRP immunoreactivity in normal trigeminal ganglion to all CGRP immunoreactive cells were 51.3%, 44.0%, 4.7%, respectively. 3. The percentage of small cells with CGRP immunoreactivity to all CGRP immunopositive cells was increased in 3 hour and 12 hour groups. 4. The percentage of medium cells with CGRP immunoreactivity was increased in 3 day and 7 day groups. 5. The percentage of large cells with CGRP immunoreactivity was increased in 7 day group. Conclusively, the small cells with CGRP immunoreactivity in trigeminal ganglion respond to orthodontic force during initial phase of tooth movement, and later the medium and large with CGRP immunoreactivity respond.
Animals ; Cell Size ; Central Nervous System ; Endothelial Cells ; Frozen Sections ; Ganglion Cysts ; Immune Sera ; Immune System ; Molar ; Neurons* ; Osteogenesis ; Rats* ; Tooth Movement* ; Tooth* ; Trigeminal Ganglion* ; Vasodilation

We report a case of disseminated calcific cysticercosis of brain, accompanied with marked hydrocephalus. Histopathologically, the calcific spots consisted of mummified scolex and bladder of cysticercus cellulosae with a diffuse calcification, but no area of cystic change were noted. Surrounding brain tissue showed a dense fibrous reaction and gliosis with little foreign body reaction.
Brain* ; Cysticercosis ; Cysticercus ; Foreign-Body Reaction ; Gliosis ; Hydrocephalus ; Urinary Bladder

BACKGROUND: Urticaria pigmentosa(UP) is primarily a disease of children. There have been no clinical studies of UP in Korea. OBJECTIVE: This study was performed to find the clinical characteristics of UP in Korean child-hood patients. METHOD: Twenty-nine cases of urticaria pigmentosa confirmed clinically and histopathologically were analyzed. RESULTS: The maculopapular type was the most common with the usual age of onset before the age of 6 months, and the lesions tended to be distributed in the central portion of the body. Darier sign was positive in 92% of the patients(24/26). No systemic involvements were detected in any of the patients with minimal associated symptoms. Seven patients of the maculopapular type and one patient of the multiple nodular type followed up for more than 2 years showed a tendency to improve or clear by the age of 6 years. CONCLUSION: Neonatal or infantile-onset patients of UP in Korean pediatric population were considered to have a benign clinical course and to require no aggressive therapy.
Age of Onset ; Child* ; Clinical Study* ; Humans ; Korea ; Methods ; Urticaria Pigmentosa* ; Urticaria*

BACKGROUND: The clinical behavior of vitiligo has not been clearly understood and hypothesis concerning the pathogenesis of the disease has been confusing and contradictory though autoimmune mechanisms have been considered important by many authors. OBJECTIVE: The purpose of this study was to develop a better understanding of the clinical features and pathogenesis of vitiligo. METHODS: We investigated clinical features of vitiligo in 1315 patients, and also compared the clinical course and features of non-segmental type(type A) and segmental type(type B) vitiligo patients to see whether the two types of vitiligo have a different pathogenic mechanism. RESULTS: Previously reported clinical patterns of the disease were reviewed and compared with our data, and the different clinical findings between the two types which supported the hypothesis of Koga et al. that type A and type B vitiligo had a different pathogenesis and autoimmune mechanisms played a role only in type A were shown. CONCLUSION: We investigated the clinical characteristics of vitiligo in Korea and showed that the type A vitiligo might have a different pathogenic mechanism with type B.
Clinical Study* ; Humans ; Korea ; Vitiligo*