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

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

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*

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

No abstract available.

To evaluate the effects of pregnancy-induced hypertension (PIH) to the iron status of fetuses, umbilical cord blood of 35 newborn borne by PIH mothers and of 37 normal term infants delivered at Yeungnam University Hospital from September 1, 1993 to September 30, 1994, were studied. The serum hemoglobin concentration of women with PIH was significantly higher than normal full-term pregnant women. There was no significant difference in serum hemoglobin concetration between women with PIH and normal full-term pregnant women and their newborn infants. There was no significant difference in serum hemoglobin concentration between infants iof women with PIH and normal full-term infants. The serum iron concentration of newborn infants of women with PIH was higher and the serum ferritin concentration of newborn infants of women with PIH was lower than normal full-term infants, but there were no significant difference between the two groups. The serum total iron-binding capactity and unsaturated iron-binding capacity of infants of women with PIH were significantly higher than normal full-term infants. The newborn infants of PIH women seemed that they might have occult depletion of iron store and need meticulous follow up during early neonatal period.
Female ; Ferritins ; Fetal Blood ; Fetus ; Follow-Up Studies ; Humans ; Hypertension* ; Hypertension, Pregnancy-Induced ; Infant ; Infant, Newborn* ; Iron ; Mothers* ; Pregnancy ; Pregnant Women