Congenital hypohidrotic ectodermal dysplasia is a rare genodermatosis. It is characterized by hypohidrosis hypotrichosis, dental hypoplasia and characterstic facial features, which reflect a wide constellation of developmental defec of tissue from the ectoderm. We have encountered three cases of congenital hypohidrotic ectodermal dysplasia in a 28-year-old female, her new-born baby, and a 10-month-old boy with a family history. All of the three patients had hypohidrosis, hypotrichosis, defective dentition, and characterstic facial features, which were characterstic features of this disorder. In addition, they showed dry skin, sparse and thin hairs. Histopathologic findings of previous cases revealed no eccrine gland structure in the dermis with routine and immunohistochemical stainning such cytokeratin and filaggrin. We report three typical cases of hypohidrotic ectodermal dysplasia with the review of literature.
Adult ; Dentition ; Dermis ; Eccrine Glands ; Ectoderm ; Ectodermal Dysplasia 1, Anhidrotic* ; Female ; Hair ; Humans ; Hypohidrosis ; Hypotrichosis ; Infant ; Keratins ; Male ; Skin

A collodion baby is born with a tough, inelastic parchment-like membrane covering the whole body surface. As the meinbrane fissures and peels, a more characteristic ichthyosiform change is evident beneath the collodion membrane. Uncommonly, normal skin is found under it. We herein present two cases af collodion baby, which were confirmed as a sporadic case of lamellar ichthyosis and a case of lamellar exfoliation of the newborn, respectively, after long-term clinical observation. It is necessary that detailed genetical and molecular biological studies should be perforrned in order to elucidate the fur:damental, molecular changes that cause these dramatic cutaneous changes.
Collodion* ; Humans ; Ichthyosis, Lamellar ; Infant, Newborn ; Membranes ; Skin

No abstract available.
Knee*

No abstract available.
Femur*

No abstract available.
Dislocations* ; Shoulder*

Bioelectrical impedance analysis (BIA) was used to determine total body water (TBW) and lean body mass (LBM) in patients with renal failure. The body's electrical resistance (R) was measured by the voltage to current ratio, injecting an 800microA alternating current with a frequency of 50KHZ and detecting a voltage drop between the wrist and the ankle. Impedance index (Height2/Resistance) compared favorably with TBW measured by deuterium (D2O) dilution method as the reference, giving the correlation coefficient (r) of 0.966 and standard error estimation (SEE) of 2.71 liter. The index was compared with LBM determined by dual-energy x-ray absorptiometry (DEXA) as the reference, giving r of 0.970 and SEE of 3.00kg. The r of 0.985 and SEE of 2.15kg were found between the reference method. BIA appeared to have a somewhat lower accuracy than those of the reference method. However, it is a useful clinical tool for estimating body composition, because it is easy, rapid and non-invasive. The existing BIA method is based on an extremely simple conductor model of the body. The accuracy may be improved further, based on a more realistic model for the body.
Absorptiometry, Photon ; Ankle ; Body Composition ; Body Water* ; Deuterium ; Electric Impedance* ; Humans ; Renal Insufficiency* ; Wrist

OBJECTIVE: To evaluate the association between transient bradycardia following cordocentesis and adverse pregnancy outcome. METHODS: 117 cordocentesis was performed at Samsung Cheil hospital and women's healthcare center, between January 1, 2000, and December 31, 2002. Clinically significant bradycardia was defined as a drop in the heart rate to less than 120 bpm. The adverse outcome included major anomaly, chromosomal abnormality, intrauterine growth restriction, preterm birth, and intrauterine fetal death. RESULTS: Bradycardia following cordocentesis was observed in 13 cases (11.1 per cent). The fetal heart rate before (149.2 +/- 0.7 bpm) and after (144.7 +/- 2.8 bpm) cordocenteis was significantly different. The adverse outcome rate was 50.0 per cent (6/12) in cases with bradycardia and 27.7 per cent (26/94) in those without bradycardia (RR=2.6, P=NS). CONCLUSION: Our result is that the association between transient bradycardia following cordocentesis and adverse pregnancy isn't statistically significant, even though adverse outcome is more frequent in pateints with bradycaredia. Therefore, patient with bradycardia following cordocentesis need periodic fetal surveillance.
Bradycardia* ; Chromosome Aberrations ; Cordocentesis* ; Delivery of Health Care ; Female ; Fetal Death ; Heart Rate ; Heart Rate, Fetal ; Humans ; Pregnancy ; Pregnancy Outcome* ; Pregnancy* ; Premature Birth

OBJECTIVE: To evaluate the association between transient bradycardia following cordocentesis and adverse pregnancy outcome. METHODS: 117 cordocentesis was performed at Samsung Cheil hospital and women's healthcare center, between January 1, 2000, and December 31, 2002. Clinically significant bradycardia was defined as a drop in the heart rate to less than 120 bpm. The adverse outcome included major anomaly, chromosomal abnormality, intrauterine growth restriction, preterm birth, and intrauterine fetal death. RESULTS: Bradycardia following cordocentesis was observed in 13 cases (11.1 per cent). The fetal heart rate before (149.2 +/- 0.7 bpm) and after (144.7 +/- 2.8 bpm) cordocenteis was significantly different. The adverse outcome rate was 50.0 per cent (6/12) in cases with bradycardia and 27.7 per cent (26/94) in those without bradycardia (RR=2.6, P=NS). CONCLUSION: Our result is that the association between transient bradycardia following cordocentesis and adverse pregnancy isn't statistically significant, even though adverse outcome is more frequent in pateints with bradycaredia. Therefore, patient with bradycardia following cordocentesis need periodic fetal surveillance.
Bradycardia* ; Chromosome Aberrations ; Cordocentesis* ; Delivery of Health Care ; Female ; Fetal Death ; Heart Rate ; Heart Rate, Fetal ; Humans ; Pregnancy ; Pregnancy Outcome* ; Pregnancy* ; Premature Birth

Bone and Bones ; Calcium ; Glycerophosphates ; Mandible ; Osseointegration ; Sulfur ; Swine

PURPOSE: This multi-center, open-label, clinical study was designed to evaluate the safety and immunogenicity of a trivalent, live, attenuated measles-mumps-rubella (MMR) vaccine, Priorix (TM) in Korean children. METHODS: From July 2002 to February 2003, a total of 252 children, aged 12-15 months or 4-6 years, received Priorix (TM) at four centers: Han-il General Hospital, Kyunghee University Hospital, St. Paul's Hospital at the Catholic Medical College in Seoul, and Korea University Hospital in Ansan, Korea. Only subjects who fully met protocol requirements were included in the final analysis. The occurrence of local and systemic adverse events after vaccination was evaluated from diary cards and physical examination for 42 days after vaccination. Serum antibody levels were measured prior to and 42 days post-vaccination using IgG ELISA assays at GlaxoSmithKline Biologicals (GSK) in Belgium. RESULTS: Of the 252 enrolled subjects, a total of 199 were included in the safety analysis, including 103 from the 12-15 month age group and 96 from the 4-6 year age group. The occurrence of local reactions related to the study drug was 10.1 percent, and the occurrence of systemic reactions was 6.5 percent. There were no episodes of aseptic meningitis or febrile convulsions, nor any other serious adverse reaction. In immunogenicity analysis, the seroconversion rate of previously seronegative subjects was 99 percent for measles, 93 percent for mumps and 100 percent for rubella. Both age groups showed similar seroconversion rates. The geometric mean titers achieved, 42 days post- vaccination, were: For measles, in the age group 12-15 months, 3, 838.6 mIU/mL [3, 304.47, 4, 458.91]; in the age group 4-6 years, 1, 886.2 mIU/mL [825.83, 4, 308.26]. For mumps, in the age group 12-15 months, 956.3 U/mL [821.81, 1, 112.71]; in the age group 4-6 years, 2, 473.8 U/mL [1, 518.94, 4, 028.92]. For rubella, in the age group 12-15 months, 94.5 IU/mL [79.56, 112.28]; in the age group 4-6 years, 168.9 IU/mL [108.96, 261.90]. CONCLUSION: When Korean children in the age groups of 12-15 months or 4-6 years were vaccinated with GlaxoSmithKline Biologicals' live attenuated MMR vaccine (Priorix (TM) ), adverse events were limited to those generally expected with any live vaccine. Priorix (TM) demonstrated excellent immunogenicity in this population.
Belgium ; Child ; Enzyme-Linked Immunosorbent Assay ; Gyeonggi-do ; Hospitals, General ; Humans ; Immunoglobulin G ; Korea ; Measles ; Measles-Mumps-Rubella Vaccine* ; Meningitis, Aseptic ; Mumps ; Physical Examination ; Rubella ; Seizures, Febrile ; Seoul ; Vaccination