The single enzyme P-450c17 hydroxylase catalyzes the 17a-hydroxylation of both pregnenolone and progesterone and the side-chain cleavage of 17a-hydroxypregnenolone and 17a-hydroxypro- gesterone to dehydroepiandrosterone and androstenedione. This enzyme is located in the endoplasmic reticulum and consists of a P-450c17 and a specific flavoprotein NADPH-cytochrome P-450 reductase. The clinical picture and hormonal pattern in 17a-hydroxylase deficiency have been consistent in both genotypic sexes with hypergonadotropic hypogonadism in whom the virtual absence of gonadal steroids results in a female phenotype with primary amenorrhea and pseudohermaphro- ditism in the male and underdeveloped secondary sex characteristics and hypermineralocorticoidism with hypertension, hypokalemia, suppressed renin-angiotensin system and extremely reduced aldo-sterone production. A 17-year-old girl visited endocrine clinic because of amenorrhea, absence of pubic and axillary hair, and hypertension. she had elevated levels of serum corticosterone, deoxycorticosterone(DOC), 18-hydroxycorticosterone(18-OHB). Stumulation with ACTH effected minimal increase in the elevated steroids and the ACTH-stimulated 18-OHB to aldosterone ratio was more than 280. These hormonal patterns appear to be homozygote in 17a-hydroxylase deficiency.
Adolescent ; Adrenocorticotropic Hormone ; Aldosterone ; Amenorrhea ; Androstenedione ; Corticosterone ; Dehydroepiandrosterone ; Endoplasmic Reticulum ; Female ; Flavoproteins ; Gonads ; Hair ; Homozygote ; Humans ; Hypertension ; Hypogonadism ; Hypokalemia ; Male ; Oxidoreductases ; Phenotype ; Pregnenolone ; Progesterone ; Renin-Angiotensin System ; Sex Characteristics ; Steroids

No abstract available.
Craniocerebral Trauma* ; Head*

OBJECTIVES: This study is to see the transition and pattern of the industrial iureal worker, and to develop the prediction model. METHODS: The data of the study are based on the samples from data-warehouse of Occupational Safety & Health Research Institute and are summed monthly from Jan 1986 to Dec 1999. This study data used data mart and Meta data from DW in KOSHA. The prediction model of the injured worker in Industry is designed by using a winters time series method after data preparing (i. e. sample, explore, modify) from DW. RESULTS: Thls predicted model obtained Winters-method multiplicative in exponential smoothing among applied all models, after the tlme series (total 163 months). It showed that the prediction power was 95.5 %. CONCLUSIONS: In the process of exploring the data, totally the rate of industrial injureal workers reduced, and in the yearly circulation, in February and September the number is the lowest but in June, July, October and November the higher. The number of monthly average injureal workers is 8709 (95 % confidence interval 8277, 9140). From the developed prediction model, since Aug 1999 the industrial injureal worker reduced rapidly in Dec 1999 and first period of 2000. But In second period of 2000 the number of the injured workers is increasing. To conclude, as the total economic situation is becoming better in 2000 than In 1999, its is supposed that the injured workers will increase more than the predictive injured workers because of the increase of production rate and labor force.
Academies and Institutes ; Data Mining* ; Employment ; Humans ; Occupational Health

The authors presented a case of gastroschisis with a pedunculated gallbladder embedded in hepatic tissue. The patient was born after fullterm gestation. Due to extensive abdominal wall defect, she succumbed 3 days after birth. There was a pedunculated mass measuring 2.5x1.5x1.5 cm between the liver and distended stomach without any attachment to the liver. The pedicle of the mass was connected to the common bile duct. Cut surface revealed that the mass was an accessory hepatic lobe encircling the gallbladder. The histologic feature of both accessory liver and gallbladder was markedly altered probably due to vascular obstruction. We could find only a single similar case in literlature. That case was associated with Beckwith syndrome and ours was associated with large atrial spetal defect, large patent ductus arteriosus, accessory spleen as well as gastroschisis. The common feature of abdominal wall defect might be a predisposing factor.

The most important spinal disorders in childhood are malformations and mass lesions. High-resolution, real-time ultrasonography of the spine enables the differentation of intraand paraspinal structures in a significant way. This examination does not require sedation, does not use radiation, can be performed in the nursery or an incubator and is less expensive than computed tomography or magnetic resonance imaging. The sonographic appearence of the various kinds of spinal dysraphism is demonstrated and illustrated. Based on our preliminary experience spinal sonography appears to be useful in neonates and infants as the first imaging device for differentiation of cmplex malformation and as a screeing method for occult spinal dysraphism.
Humans ; Incubators ; Infant* ; Infant, Newborn* ; Magnetic Resonance Imaging ; Neural Tube Defects ; Nurseries ; Spinal Dysraphism* ; Spine ; Ultrasonography*

Persistent trigeminal artery is an embryonic remnant of the anastomotic channel linking the internal carotid artery and the basilar artery. Cases of vertebrobasilar insufficiency caused by the persistent trigeminal artery with internal carotid artery stenosis has been described previously, but vertebrobasilar insufficiency entirely due to in situ stenosis of the persistent trigeminal artery has not been reported. A 71-year-old man presented with frequent dizzy episodes. The brain MRI showed no parenchymal lesions. MR angiography showed poor visualization of vertebrobasilar system. He was diagnosed as having vertebrobasilar insufficiency. Cerebral angiography revealed that there was complete occlusion at the vertebrobasilar junction, and the basilar artery was supplied by the persistent trigeminal artery which had severe stenosis at its origin. There was no stenosis of the internal carotid artery of both sides. We believe that this is the first report of vertebrobasilar insufficiency due to stenosed persistent trigeminal artery, without internal carotid artery stenosis.

No abstract available.
Ileus* ; Lymph Nodes*

The present study was undertaken to investigate serum carboxyhemoglobin levels in smokers according to the amount of cigarrettes. many studies have shown definite and consistent relations between smoking and ischemic heart disease. The purpose of this study is to show that COHb levels can be used to discriminate between persons having certain ischemic heart disease and persons without these disease more accurately than is possible by using smoking history alone, and thus to suggest that this may be a good indicators of risk. The results of this study are as follow : 1) The incidence of ischemic heart disease in smoker is higher than that in non-smoker. 2) For a given COHb level there is no clear increasing trend in the population of affected subjects with increasing cigarette consumption and there is no significant change cigarette consumption under duration of 10 year's smoking history. 3) For a moderate and a heavy smoking categories there is a consistent effect of an increasing proportion of affected subjects as the COHb level rises and there is a significant change with COHb level under duration of 10 year's smoking history. 4) The same results are as over duration of 10 year's history. 5) There were no significant interaction between under duration of 10 year's smoking history and over duration of 10 year's smoking history. In conclusion, it seemed that cigarette smoking may play an important factor in development of ischemic heart disease by increasing serum carboxyhemoglobin level.
Carboxyhemoglobin* ; Humans ; Incidence ; Myocardial Ischemia* ; Smoke* ; Smoking* ; Tobacco Products

Intracranial lipoma is a rare condition, though lipoma in other sites can be found throughout the body. Many authors have reported intracranial lipoma since Rokitansky and Soman who described first in autopsy and living patient respectively. Intracranial lipoma can be diagnosed simply be simple skull and computed tomography. The authors experienced three cases of intracranial lipoma diagnosed by simple skull, tomography, cerebral angiography and computed tomography, and reported with a brief review of the literature.
Autopsy ; Cerebral Angiography ; Humans ; Lipoma* ; Skull ; Soman

Osteogenesis imperfecta is a hereditary condition characterized by fragility of bone, deafness, blue sclera, laxity of joints and a tendency to improvement with age. Authors experienced osteogenesis imperfecta that develped over 3 generations in one pedigree. A 28 years old female exhibited severe anterior bowing deformity of left tibia. She has a past history of many times of bone fractures. She was treated with corrective osteotomy, plate fixation for correction of bowing deformity of left tibia and medullary nailing with fibular graft for paeudarthrosis of Rt. forearm bone.
Congenital Abnormalities ; Deafness ; Family Characteristics ; Female ; Forearm ; Fractures, Bone ; Humans ; Joints ; Osteogenesis Imperfecta ; Osteogenesis ; Osteotomy ; Pedigree ; Sclera ; Tibia ; Transplants