Congenital ectodermal dysplasia are a group of familial disorders that affect tissues and organs of ectodermal origin to varying degrees. It devided hidrotic type and anhidrotic type by geneologic origin and distinct clinical manifestationa. The hidrotic type usually trait by autosomal dominant gene and manifested dystrofic nails, defect of hair and eyebrow and palmoplantar hyperkeratosis. This patient is 16 year oId male. He had been dystrophic nails, alopecia since at birth, thereafter palmoplantar hyperkeratosis, clubbing fingers noted. C.R.C. Urinalysis, stool examination, chest x-ray were within normal limit. Biopsy specimen from palm revealed hyperkeratosis, hypergranulosis, acanthosis and, mild inflamrnatory cell infiltrate on upper dermis. Biopsy speeimen from the scalp revealed norrriaI findings except slight hypoplasia of ebaceaus gland.
Alopecia ; Biopsy ; Dermis ; Ectoderm ; Ectodermal Dysplasia* ; Eyebrows ; Fingers ; Genes, Dominant ; Hair ; Humans ; Male ; Parturition ; Scalp ; Thorax ; Urinalysis

No abstract available.
Burns* ; Cerebral Hemorrhage*

BACKGROUND: According to the 1992 version of ACLS guideline, adenosine is recommended as the first line drug far the treatment of paroxysmal supraventricular tachycardia(PSYT). But adenosine is not used frequently in our country, despite currency proven effect and safety. Therefore we tried to compare the efficacy and safety of adenosine with verapamil for the treatment far PSVT. MATERIALS AND METHODS: We prospectively reviewed charles of PSVT patients admitted to YongDong Severance Hospital from Jan. 1995 to Dec. 1996. These patients were randomly divided into two groups. The flat group was given 6mg of adenosine initially, and another 12mg was given within 5 minutes if fast dose failed. The other group was given 5mg of verapamil initially, and if failed, 10mg was given within 10 minutes. The results were analyzed by Chi-square test and student-t method. RESULTS: In converting PSVT to normal rhythm, adenosine and verapamil showed similar results and there was no difference between the two Groups in frequency of side elects, but serious arrhythmia such as ventricular fibrillation and ventricular tachycardia was not seen in the adenosine group. CONCLUSION: Adenosine not only showed comparable effect and safety, but also had shorter action time than verapamil. Therefore we recommand adenosine, as a safe and effective fort line drug for PSVT.
Adenosine* ; Arrhythmias, Cardiac ; Humans ; Prospective Studies ; Tachycardia, Supraventricular* ; Tachycardia, Ventricular ; Ventricular Fibrillation ; Verapamil*

Fractures significantly involving the weight-bearing articular surface and the overlying metaphysis of the distal tibia have deserved notoriety as a severe and challenging subject of ankle injuries. It was not until 1963 that the AO group developed principles for open reduction and internal fixation of pilon fracture. But recently, limited internal fixation of the joint surface combined with external fixation may avoid the soft tissue complications associated with formal open reduction and internal fixation. We have treated 22 cases of tibial pilon fracture with biologic principle. The mean follow-up period was two years ranging from one to three years. According to the Riiedi and Allgower classification, three cases were type I, five cases were type II, and fourteen cases were type III Biologic treatment includes combination of internal and external fixation, external fixation alone, or plating was done with careful soft tissue dissection, limited stripping of fracture fragments, and indirect reduction technique. Radiographic results were assessed by Burwell and Charnley criteria. Three cases of type I, three cases of type II and seven cases of type III pilon fractures obtained good anatomic radiographic results. We also assessed the functional results by Mast and Teipner criteria. Three cases of type I, three cases of type II, six cases of type III pilon fractures obtained good functional outcome. We believed that combination of internal and external fixation provides the patient who has a markedly displaced pilon fracture and diaphyseal comminution with the best chance of good clinical result and minimal risks. As such, we believe biologic treatment is excellent for these injuries.
Ankle Injuries ; Classification ; Follow-Up Studies ; Humans ; Joints ; Tibia ; Weight-Bearing

The author measured the value of serum triglyceride, total cholesterol and phospholipid in normal Korean adults and patients with complicated hypertension. Sera obtained from 295 cases of normal Korean male and female, and also 109 cases of patients with hypertension were analysed for triglyceride, cholesterol and phospholipid. The patterns of serum lipoprotein fraction in 171 normal Korean and 109 patients with complicated hypertension including cerebrovascular diseases and ischemic heart disease were studied by agarose gelelectrophoresis. The results are summarized as follows; 1. The mean value of serum triglyceride, cholesterol and phospholipid in normal Korean were 85.4+/-20.5mg%, 187.9+/-16.7mg%, and 193.4+/-34.6mg% respectively. 2. Sex and age difference showed no statistical significance in the mean values of serum triglyceride, cholesterol and phospholipid in hypertensive patients. 3. The mean values of serum triglyceride in the patients with complicated hypertension such as cerebral thrombosis gave higher value than in the patients with other cerebral complications. 4. The mean value of alpha-lipoprotein, pre-beta-lipoprotein and beta-lipoprotein in normal Korean were 29.6+/-5.0% (142.6+/-30.1mg%), 13.0+/-4.3% (62.7+/-24.8mg%), and 57.4+/-30.1% (276.1+/-56.6mg%) respectively. 5. The alpha-lipoprotein fraction in normal Korean demonstrated the tendency of decreasing as age is progressing in both sex, but pre-beta and beta-lipoprotein fraction gave a tendency of increasing by progressing age in both sex, but was not confirmatory in nature. 6. The alpha-lipoprotein fraction in patients with hypertension gave lower value than in the normal subjects, but the value of pre-beta and beta-poprotein were elevated significantly among patients with complicated hypertension than in normal person. 7. The incidence of hyperlipidemia in normal Korean and patients with hypertension were 31.9% and 66.5% respectively. 8. The relative incidences of hyperlipidemic probable phenotypes in normal Korean were as follows: 7% of normal person had Type II (IIa 3.3%, IIb 3.7%), 3.5% had Type III, and 21.4% had Type IV.
Adult* ; Cholesterol ; Female ; Humans ; Hyperlipidemias ; Hypertension* ; Incidence ; Intracranial Thrombosis ; Lipoproteins* ; Male ; Myocardial Ischemia ; Phenotype ; Sepharose ; Triglycerides

No abstract available.
Fingers* ; Replantation*

Erdheim Chester disease (ECD) is very rare non-Langerhans cell histiocytosis (LCH) which occurs in the skeletal system and multiple organs. As it is progressive, sometimes it causes fatal results. However, it is often misdiagnosed as LCH or multiple bone metastasis and, thus, is very difficult to diagnose. In Korea, only 10 cases were first reported in 1999. In particular, there have been a few orthopedic approaches or reports in English-speaking literatures, and no report has been issued in Korea. The authors performed bone biopsy in patients with knee and lower extremity pain who were referred for the integrated treatment. We attempts to report this diagnosis experience with literature review.
Biopsy ; Erdheim-Chester Disease ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Knee ; Korea ; Lower Extremity ; Neoplasm Metastasis ; Orthopedics

This case was an acquired aplastic anemia patient who required a major operation for excision of a mediastinal mass. The authors previewed that the coagulation abnormalities would be developed due to major operation. Thus we decided to monitoring the coagulation function using the thromboelastography during the perioperative period and checked the complete blood count, concommitantly. The total blood volume lost during operation was 1800 ml, so we gave him a transfusion of 10 U's of platelet concentrate, 10 U's of pheretic platelet rich plasma and 5 U's of whole blood. The thromboelastography was a good guide that helped us to avoid excessive treatment of the coagulation abnormalities. We concluded that the thromboelastograhy was a reliable and effective monitoring system at the intraoperative coagulation management.
Anemia, Aplastic* ; Anesthesia, General ; Blood Cell Count ; Blood Platelets ; Blood Volume ; Humans ; Perioperative Period ; Platelet-Rich Plasma ; Thrombelastography

This study was performed to evaluate the clinical significance of SCC as a tumor marker in patient with squamous cell carcinoma of the cervix. We measured the serum levels of SCC by radioimmunoassay in patient with invasive squamous cell carcinoma of cervix to determine the prognostic value, correlation with the presence of lymph node metastasis, response to treatment, and those value in the early detection of recurrence after treatment. The result was: 1) In 117 of 174 patients with cervical cancer, the pretreatment positive rate of SCC was 47.0%(57/117). In each stage, the stage Ia was 25.0%, Ib 26.3%, IIa 56.0%, 62.6%, III 57.1%, IV 100%, and the recurrent case was 37.5%. 2) In 79 of 174 patients with cervical cancer, the pretreatment positive rate of SCC was 44,0%(30/69) in no evidence of malignancy patients, but 60.0%(6/10) in recurrent or permanent patients(p>0.05), 3) In 133 of 174 patients with cervical cancer, the posttreatment positive rate of SCC was 2.6%(3/117) in no evidence of malignancy patients, but 50.0%(8/16) in recurrent or permanent patients(p<0.05). 4) In 48 patients of 174 with cervical cancer who underwent radical hystrectomy, the positive rate of SCC was 71.4%(5/7) in pelvic lymph node positive patients but 19,5%(8/41) in pelvic lymph node negative patients(p<0.05). We concluded that the pretreatment SCC level was not effective as a prognostic value, but well correlated with pelvic lymph node metastasis, and serial measurements of serum levels of SCC provided a reliable clue for early detection of recurrence or progression of disease, so it may be useful for monitoring cervical carcinoma patient.
Carcinoma, Squamous Cell* ; Cervix Uteri* ; Female ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Radioimmunoassay ; Recurrence ; Uterine Cervical Neoplasms

OBJECTIVES: Subacute thyroiditis is a nonsuppurative inflammation of thyroid gland and is probably caused by a cytopathic virus. Typical clinical symptoms and features of subacute thyroiditis vary widely during the course of illness. It has a clinical course, evolving from hyperthyroidism through a temporary hypothyroidism to recovery. However, the final outcome of this disease remains unpredictive in some patients. Permanet hypothyroidism occurs infrequently. METHODS: Thirty-three patients proven to have subacute thyroiditis at the Ewha Womans University Hospital from September 1993 to November 1995 were studied. We analyzed their clinical features, laboratory findings, and duration of recovery to cha- racterize the course of the disease. RESULTS: 1) Total 33 patients were studied: 31 patients were female and 2 patients were male. Their mean age was 42.6+/-8.3 years old. The peak months were August through October in this study. 2) Initial mean ESR was 73.0+/-35.2mm/hr, mean T3 was 217.3+/-73.9ng/dl, mean T4 was 15.2+/-8.5microgram/ dl, and TSH was 0.06+/-0.09microIU/ml. The positive rates of antithyroglobulin and anitmicrosomal antibodies were 31% and 6% respectively, and TSH receptor antibody was elevated in one patient. 3) In the thyroid scan, 91% showed both lobes nonvisualisation, and 9% showed one lobe nonvisualization. Radioactive iodine uptake(RAIU) at 24 hour was 2.4+/-3.3%. 4) With the predisolone therapy, 90% of patients completely recovered, 57% of these patients had no hypothyroid phase and remaining 33% of them had hypothyroid phase during course of the disease. Three of the patients had permanent hypothyroidim. 5) The average duration of recovery was 3.2+/-1.4 months and it has no correlation with initial thyroid hormone levels, antithyroid antibodies and duration of steroid administration. CONCLUSION: There was no historical, physical, laboratory findings that help us predict those patients likely to have an exacerbation of the disease.
Antibodies ; Female ; Humans ; Hyperthyroidism ; Hypothyroidism ; Inflammation ; Iodine ; Male ; Prednisolone ; Receptors, Thyrotropin ; Thyroid Gland ; Thyroiditis, Subacute