No abstract available.
Bilirubin* ; Brain Stem* ; Humans ; Infant, Newborn* ; Kernicterus*

We report a case of acute intermittent porphyria presenting with variable symptoms and signs such as hypertension, polyneuropathy, syndrome of inappropriate secretion of antidiuretic hormone and cerebral infarction. A 47 year-old female patient entered hospital with abdominal pain followed by generalized seizure. She was diagnosed to have acute intermittent porphyria in consequence of Watson-Schwartz test and δ-ALA in 24 hours urine. She was managed with conservative treatment but died of respiratory failure.
Abdominal Pain ; Cerebral Infarction ; Female ; Humans ; Hypertension ; Polyneuropathies ; Porphyria, Acute Intermittent* ; Respiratory Insufficiency ; Seizures

No abstract available.
Animals ; Cerebral Cortex* ; Imipramine* ; Rats* ; Receptors, Adrenergic*

Congenital ichthyosiform erythroderma is most severe type of ichthyosis. This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration of the mid and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover tirne. This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local trea- tments. The autopsy findings had exhibited fatty necrotic degenerative changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
Autopsy ; Biopsy ; Cause of Death ; Dermatitis, Exfoliative ; Extremities ; Hepatocytes ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant ; Male ; Meninges ; Parturition ; Skin ; Weights and Measures ; Wills

No abstract available.
Guinea*

Intraperitoneal hyperthermic perfusion(IPHP) is gaining popularity in the world as a method of prevention and treatment of peritoneal metastasis following gatrointestinal cancer. The procedure presents significant problems to the anesthegiologist with regard to tempera- ture control, fluid and electrolyte balance, acid-base change and postoperative care. During IPHP, there is a potential for heat gain from the peritoneal cavity. Several workers have reported a significant increase in core temperature. Therefore, it is true that accurate monitoring of temperature is essential. We studied that acid-base balance, electrolyte balance, level of blood suger following core temperature change in Intraperitoneal Hyperthermo-chemotherapeutic Perfusion patients.
Acid-Base Equilibrium ; Anesthesia* ; Hot Temperature ; Humans ; Neoplasm Metastasis ; Perfusion* ; Peritoneal Cavity ; Postoperative Care ; Stomach Neoplasms* ; Stomach* ; Water-Electrolyte Balance

Intraspinal tumors in children are of especially interest to orthopaedic surgeons for two reasons; First, musculoskeletal abnormalities, are often treated for long periods before the correct diagnosls is suspected; Second, important orthopaedic problems are encountered in almost everychild treated for intraspinal tumor, not only during the immediate postoperative management but all through subsequent growth. We report a case presentation of the intraspinai tumor, dermoid cyst, successfully treated with operation.
Child ; Dermoid Cyst ; Humans ; Musculoskeletal Abnormalities ; Surgeons

The Dandy-Walker syndrome is a developmental disorders of the brain characterized by cystic deformity of the 4th ventricle and agensis of the cerebellar vermis. Other systemic anomalies and chromosomal abnormalities are associated with this syndrome. We are experienced a case in a 9 months old male infant who presented initially with frequent vomiting, low birth weight, On the physical examination, a prominent occiput, palpable mass below the right upper quadrant, pulmonary valve stenosis, congenital dislocation of the hips, ventral flexion of fingers, clubfoots and the rocker-bottom deformities of feet were present. On the chromosomal study, there were chromosomal polymorphisms in a thickened C-band of chromosome No. 1 by C-banding method. The brain CT revealed a large, thin-walled, low density mass of CSF without enhancement in the posterior fossa, showing upward displacement of cerebellar hemisphere with absent inferior vermis(or associated with dysplastic cerebellar hemisphere). A brief review of the related literatures were included in this report.
Brain ; Chromosome Aberrations* ; Clubfoot ; Congenital Abnormalities ; Dandy-Walker Syndrome* ; Dislocations ; Fingers ; Foot ; Hip ; Humans ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Male ; Physical Examination ; Pulmonary Valve Stenosis ; Vomiting

Open fractures of the shaft of the tibia occur frequently because of the progression of industrialization, Different methods of treatment have been advocated as regards the care of the open wound and the method of stabilization of the fracture fragments. The reports, published until recently, have differed with respect to rates of morbidity and end results. With this in mind, we reviewed a consecutive series of 30 open fractures of the tibia at the Department of Orthopedic Surgery of the Eulji General Hospital between 1981 and 1983 by a variety of methods. The following results were obtained. 1. The highest incidence of fractures was encountered in 3rd decades(43.3%) and male to female ratio was 4: 1. 2. The most common cause of fractures was traffic accident(66.6%). 3. The most common anatomical level of fractures was middle third of the shaft(46.7%). 4. The severity of injury was classified according to the Ellis classification as follows. Minor group, 8 cases(40%), Moderate group, 8 cases(26.7%,), Major group, 10 cases(33.3%). 5. The most common associated injury was ipsilateral fibular fracture. 6. Of 30 cases, 11 patients(36.6%) had an open wound more than 5cm in length, 10(33.3%.) between 2.5 and Scm and 4(13.4%) below 2.5cm, and primary closure after thorough debridement and copious irrigation was done in 17 cases(56.7%). 7. Pin and plaster method was applied in 3 cases of minor group and 3 of moderate group, intramedullary nailing was applied in 9 of minor group and 5 of moderate group, and Hoffmann device was applied in 10 of major group. 8. The majority of cases(66.7%) were operated on within 1 week after injury, 9(30%) between 1 and 2 weeks and 1(3.3%) between 2 and 3 weeks. 9. The fractures in middle third of the shaft of the tibia treated with intramedullary nailing revea.ed the shortest healing time(average 13 weeks). 10. Complications such as delayed union, infection and disabled joint shiffness were encountered mainly in cases with severe soft tissue injuries.
Classification ; Clinical Study ; Debridement ; Female ; Fracture Fixation, Intramedullary ; Fractures, Open ; Hospitals, General ; Humans ; Incidence ; Joints ; Male ; Methods ; Orthopedics ; Soft Tissue Injuries ; Tibia ; Wounds and Injuries

No abstract available.
Radiosurgery*