No abstract available.
Intussusception*

We would like to correct the affiliation for the first author.

No abstract available.
Osteogenesis Imperfecta* ; Osteogenesis*

Pseudoxanthoma elasticum is a rare, heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the loose and wrinkled flexural skin. An 18-year-old man presented cutis laxa-like marked wrinkling on the axillae, trunk and thighs for about 10 years. There was no similar skin lesion on his family members and no other systemic involvement was found. Histopathological examination of the abdomen revealed fragmentation and calcification of elastic fibers in the middle and lower dermis. Von Kossa staining showed calcium deposition along the altered elastic fibers. Herein, we report a rare case of autosomal recessive pseudoxanthoma elasticum presenting generalized cutaneous laxity.
Abdomen ; Adolescent ; Axilla ; Calcium ; Dermis ; Elastic Tissue ; Humans ; Pseudoxanthoma Elasticum* ; Skin ; Thigh

A 28-year-old woman presented with multiple, asymptomatic, erythematous to bluish papules located on the chest. Histopathologically, three round, well defined cystic structures were seen on the upper and lower dermis. The first cyst was milia, the second was apocrine hidrocystoma and the other, largest cyst was an eruptive vellus hair cyst (EVHC). A diagnosis of multiple pilosebaceous cysts combined with apocrine hidrocystoma was made. Since the milia and EVHC originate from the pilosebaceous unit, and the apocrine duct opens to the pilosebaceous orifice, we suggest that they can occur simultaneously in the same unit.
Adult ; Biopsy ; Dermis ; Female ; Hair ; Hidrocystoma ; Humans ; Thorax

No abstract available.
Leukemia, Eosinophilic, Acute* ; Trisomy*

BACKGROUND: Purified major allergens of house dust mite are essential for evaluation of the allergic mechanism in molecular basis and development of new modalities of immunemodulation. OBJECTIVE: In this study, we aimed to purif group 1 and group 2 allergens from Dermatophagoides pteronyssinus (Dp). In addition, cDNAs corresponding to Der pI and II in Korean Dp were isolated and recombinant Der p1 and Der pII were synthesized. MATERIALS AND and METHOD: Der pI allergen was purified by ammonium sulfate precipitation, anion -exchange column chromatography, and gel filtrat,ion chromatography. Der pII allergen was purified by anion exchange chromatography, gel filtration chromatography, and a preparative isoelectric focusing method. RESULTS: Eight hundred ug of Der pI and 50 ug of Der pII were obtained from 100 g of culture medium and 1 g of mite bodies, respectively. The purities of these allergens were confirmed by SDS PAGE and the strong reactivity to the patient sera was identified. In order to produce a recombinant allergens, poly(A) RNA from house dust mites were isolated and used for cDNA synthesis by RT PCR. The cDNA was inserted into prokaryotic expression vector and the vectors were transformed into E. coli. A little amount of recombinant Der pI protein was produced due to the low solubility, and 1.2 mg of recombinant Der pII was produced from 1 L of E. coli culture medium. The antigenicity of Der pI was relatively weak, however, Der pII showed a strong antigenicity. Amino acid sequence of the amplified cDNA deduced from DNA sequences of Der pII showed 6 different variants. The variation of amino acid sequences suggests the possibility of high incidence of mutation of Der pII protein. CONCLUSION: A simplified method for the purification of Der pI and Der pII was developed. Recombinant allergens will be useful for the diagnosis and treatment of allergy with lower costs.
Allergens* ; Amino Acid Sequence ; Ammonium Sulfate ; Base Sequence ; Chromatography ; Chromatography, Gel ; Dermatophagoides pteronyssinus* ; Diagnosis ; DNA, Complementary ; Electrophoresis, Polyacrylamide Gel ; Humans ; Hypersensitivity ; Incidence ; Isoelectric Focusing ; Mites ; Polymerase Chain Reaction ; Pyroglyphidae* ; RNA, Messenger ; Solubility

OBJECTIVE: We analyzed the clinical features of amniotic fluid embolism confirmed by autopsy or clinical dicision for making a plan of rapid diagnosis and intensive emergency care. STUDY DESIGN: We experienced 2 clincal cases in Ewha Mokdong Hospital and Severance Hospital at 1995, and reviewed the medical record & autopsy report of 15 cases in National Institute of Scientific Investigation from Jan, 1991 to Dec, 1995. RESULTS: The mean maternal age was 31+/-3 years. The mean gestational age was 40+/-1 weeks. The number of delivery were 1.4+/-0.8. The number of abortion history. were 2.6+/-2. The sexuality of fetus were six males (35 %), five females (29%), and unknown six cases (35 %) and the fetal mortality rate was 29 % (5 cases). The initial clinical symptoms and signs were hypotension (12 patients, 71%), vaginal bleeding(ll patients, 65%), cardiac arrest (6 patients, 35 %), dyspnea (5 patients, 29 %), and seizure (2 patients, 12%). In the case of normal spontaneous vaginal delivary (NSVD), the symptoms occurred during induction in 3 (18%), during delivery in 1 (6%), and after delivery in 9 (53%). In the case of Cesarean section, the symptoms occurred during section in 2 (12%), and after section in 1 (6%). There are one case whose symptoms occurred during 3rd gestational period. CONCLUSIONS: We should alert for the amniotic fluid embolism in the clinical findings of acute collapse and vaginal bleeding, respiratory symptoms, and seizure at any peripartum. With eary suspicion of this disease and aggressive intensive care we can reduce amniotic fluid embolism mortality rate and it's legal problem.
Abortion, Induced ; Amniotic Fluid* ; Autopsy ; Cesarean Section ; Diagnosis ; Dyspnea ; Embolism, Amniotic Fluid* ; Emergency Medical Services ; Female ; Fetal Mortality ; Fetus ; Gestational Age ; Heart Arrest ; Humans ; Hypotension ; Critical Care ; Male ; Maternal Age ; Medical Records ; Mortality ; Peripartum Period ; Pregnancy ; Seizures ; Sexuality ; Uterine Hemorrhage

To determine the perinatal mortality and morbidity of fetuses with absent end-diastolic velocities (AEDV) of the umbilical artery in severe pre-eclampsia and eclampsia, the outcome of 5 fetuses with AEDV was compared with that of 35 fetuses with positive end-diastolic velocities (PEDV). The study population comprised 38 cases of severe pre-eclampsia and 2 cases of eclampsia with structurally normal singletons, who had had umbilical artery Doppler velocimetry weekly from admission to delivery. The Doppler velocimetry result was not used for the clinical management. Perinatal death and neonatal morbidity from both groups were further examined in gestational age category to control the influence of preterm births. The incidence of AEDV of the umbilical artery Doppler velocimetry in severe pre-eclampsia and eclampsia was 12.5% (5/40). The AEDV group had a significantly higher incidence than the PEDV group in terms of ceasarean section due to fetal distress (60% : 17%), Apgar score < 7 at 5 minutes (60% : 14%), perinatal death (25% : 0%) and assisted mechanical ventilation (67% : 9%) both at 32-36 weeks. Time intervals from the detection of AEDV to delivery of live neonates varied from the day to 15 days. In conclusion, AEDV in the umbilical artery might be of clinical value in routine surveillance of pregnancies complicated by severe pre-eclampsia and eclampsia, and predict hypoxic fetal condition which needs operative interventions before or during labor and mechanical ventilation after birth.
Apgar Score ; Cesarean Section ; Dystocia ; Eclampsia* ; Female ; Fetal Distress ; Fetus ; Gestational Age ; Humans ; Incidence ; Infant, Newborn ; Maternal Death ; Mortality ; Parturition ; Parturition* ; Perinatal Mortality ; Pre-Eclampsia* ; Pregnancy ; Premature Birth ; Respiration, Artificial ; Rheology ; Trial of Labor ; Umbilical Arteries* ; Uterine Rupture

No abstract available.
Hemophilia A* ; Humans ; Intussusception*