Rett syndrome is a newly characterized developmental disorder that affect girls exclusively. These girls are born clinically normal, but their psychomotor development stagnates and deteriorates between the age of 6 months and 2 1/2 years. The full syndrome comprises; aquired microcephaly, severe dementia, autism, loss of purposeful use of the hands, characteristic hand-wringing stereotypies, jerky ataxia of the trunk, epilepsy with various types of fits, and in later years spastic and trophic changes of the lower limbs. There is no known cytogenetic, biochemical or molecular marker for the disorder; the diagnosis is based on clinical criteria. We report a case of Rett syndrome with a brief review of related literatues.
Ataxia ; Autistic Disorder ; Cytogenetics ; Dementia ; Diagnosis ; Epilepsy ; Female ; Hand ; Humans ; Lower Extremity ; Microcephaly ; Muscle Spasticity ; Rett Syndrome*

No abstract available.
Biopsy* ; Child* ; Humans

BACKGROUND: The shelf life should be determined by health care facility's infection control program. The current standards for preserving the sterility can be changing. So, to measure and compare the recontamination time of sterile packs among wrappers and storage conditions, and develop a practical standard for the shelf life and storage conditions of sterile products. The purpose of this study was placed on measurement of the recontamination time in packs wrapped in all kinds of wrapping materials used in Il-San hospital and extra-costs. METHODS: The first phase study was from June to November 2002 and tile second phase study was planned to extend further the shelf life of the pouch bag from December 2002 to June 2003. Total 504 sterile sample packs containing cotton ball (5 cm-length) prepared and wrapped in four wrapping materials (randomized reprocessed two folds of lines, a fold of new craft paper, a fold of new nonwoven fabric, a fold of new pouch bag) and stored in three location (open shell of one of typical wards, open shelf of one of ICUs, open shelf of CSSD). Each type of packs were opened weekly and the cotton balls inoculated into thioglycollate broth at 35degrees C for seven days by infection control nurse. The temperature and relative humidity was monitored monthly. RESULTS: There was on growth found for any types of the pack and storage conditions studied. The temperature and relative humidity of storage locations were 24.8degrees C and 35.5 %. The extra-cost of reprocessing in Ethylene-Oxide gas sterilization was \32,800,000 and Steam sterilization was \36,900,000 by year. CONCLUSION: This study was conducted in attempt to identify the possibility of extending the current shelf life for sterile goods we use in a sense of cost effectiveness. It was possible to extend shelf life from four weeks to six months in the study hospital. We are going to continue this study and take all steps need to apply this new concept to our practice, and then trying to extend to other hospitals.
Cost-Benefit Analysis ; Costs and Cost Analysis* ; Delivery of Health Care ; Humidity ; Infection Control ; Infertility ; Steam ; Sterilization

No abstract available.
Diarrhea, Infantile* ; Infant ; Soybean Proteins*

No abstract available.
Apnea*

Twenty-five neonates were observed for the relationship between phenobrbital loading dose, plasma concentrations and control of seizures. Nineteen neonates (76%) had seizures controlled by phenobarbital alone. Effective plasma concentration were 10.0~31.1microgram/ml. Although 14 neonates had seizures controlled by phenobarbital concentration below 20microgram/ml, neonate required level above 30microgram/ml. We found that continued benefit was obtained until phenobarbital plasma concentrations reached 40microgram/ml and there was no adverse effects on cardiorespiratory function. We recommend that plasma phenobarbital concentrations should equal or surpass 40microgram/ml before additional anticonvulsants are used for neonates with seizure.
Anticonvulsants ; Humans ; Infant, Newborn ; Phenobarbital* ; Plasma ; Seizures*

No abstract available.

No abstract available.
Down Syndrome* ; Humans ; Infant* ; Leukemia, Megakaryoblastic, Acute*

Percutaneous cannulatien has become an accepted technique for monitoring central venous pressure and pulmonary wedge pressure via a Swan Gans catheter. The technique is not without hazard. Complications include thrombophlebitis, infection and hydrothorax. These are case reports demonstrating the complications of hydrothorax and broken catehters from central venous catheterization.
Catheterization, Central Venous* ; Catheters* ; Central Venous Catheters* ; Central Venous Pressure ; Hydrothorax* ; Pulmonary Wedge Pressure ; Thrombophlebitis

This study included fifty two premature babies in whom EEG was performed at National Medical Center, from Jan. 1990 to Jun. 1991. clinical and EEG records of all subjects were analyzed and the following results were obtained. 1) The day of first EEG performed were;11 cases within 10 days, 20 cases from 11 days to days, 13 cases from 21 days to 30 days and 8 cases beyond 31 days. 2) The results of first EEG were; within normal limit in 34 cases, mild abnormality in 3 cases, moderate abnormality in 11 cases and marked abnormality in 4 cases. 3) The abnormal EEG ratio according to the sex was higher in female than in male (43.5% vs 27.6%). 4) The abnormal EEG ratio according to the gestational age was higher in shorter gestational age group than in longer gestational age group (40.9% under 32 weeks of G.A. vs 30.0% beyond 33 weeks of G.A.). 5) The abnormal EEG ratio according to the birth weight was not significant. 6) The abnormal EEG ratio according to the ventilator use was higher in used group than in not used group (38.5% vs 30.8%). 7) Combined diseases in 18 cases of abnormal EEG group were jaundice (14 cases), hyaline membrane disease (7 cases), asphyxia (4 cases), anemia (4 cases), hydrocephalus, sepsis, PDA, hypocalcemia, UTI and IVH. 8) The mean apgar score in abnormal EEG group was lower than normal EEG group both in one minute and five minute. 9) Two cases among 18 cases of abnormal EEG group showed abnormal brain sonographic findings, but one case among 34 cases of normal EEG group showed abnormal brain sonographic finding. 10) Ten cases were followed up with serial EEG, six cases of which were treated with anticonvulsant. We observed normalization of the EEG in 5 cases of anticonvulsant treated group.
Anemia ; Apgar Score ; Asphyxia ; Birth Weight ; Brain ; Electroencephalography* ; Female ; Gestational Age ; Humans ; Hyaline Membrane Disease ; Hydrocephalus ; Hypocalcemia ; Infant, Newborn ; Jaundice ; Male ; Sepsis ; Ultrasonography ; Ventilators, Mechanical