17alpha-hydroxylase and 17,20-lyase are enzymes encoded by the CYP17A1 gene and are required for the synthesis of sex steroids and cortisol. In 17alpha-hydroxylase deficiency, there are low blood levels of estrogens, androgens, and cortisol, and resultant compensatory increases in adrenocorticotrophic hormone that stimulate the production of 11-deoxycorticosterone and corticosterone. In turn, the excessive levels of mineralocorticoids lead to volume expansion and hypertension. Females with 17alpha-hydroxylase deficiency are characterized by primary amenorrhea and delayed puberty, with accompanying hypertension. Affected males usually have female external genitalia, a blind vagina, and intra-abdominal testes. The treatment of this disorder is centered on glucocorticoid and sex steroid replacement. In patients with 17alpha-hydroxylase deficiency who are being raised as females, estrogen should be supplemented, while genetically female patients with a uterus should also receive progesterone supplementation. Here, we report a case of a 21-year-old female with 17alpha-hydroxylase deficiency who had received inadequate treatment for a prolonged period of time. We also include a brief review of the recent literature on this disorder.
Adrenocorticotropic Hormone ; Amenorrhea ; Androgens ; Corticosterone ; Estrogens ; Female ; Genitalia ; Humans ; Hydrocortisone ; Hypertension ; Male ; Mineralocorticoids ; Progesterone ; Puberty, Delayed ; Steroid 17-alpha-Hydroxylase* ; Steroids ; Testis ; Uterus ; Vagina ; Young Adult

No abstract available.

No abstract available.
Fractures, Open*

Epidural venography is a relatively simple and highly accurate method in the diagnosis of lumbar disc herniation. Angiographic visualization of the epidural vein has been tried and investigated by intraosseus injection of contrast agent into lumbar spinous process and intravascular injection into iliac vein since first report in 1954. Recently, lumbar epidural venography is instituted by selective catheterization of ascending lumbar vein or internal iliac vein. Lumbar epidural venography is valuable for the diagnosis of herniated lumbar disc, not demonstrated by myelography. Especially, lumbar epidural venography is more accurate than myelography in the diagnosis of L5-Sl disc herniation and in the case of extreme lateral herniation. Other advantages of epidural venography are low incidence of complication and ease of performance. Epidural venography may be recommended as an alternative rnethod rather than as adjunctive to myelography. We report 20 cases of epidural venography, compared with myelographic and operative findings.
Catheterization ; Catheters ; Diagnosis ; Iliac Vein ; Incidence ; Methods ; Myelography ; Phlebography ; Veins

Intertrochanteric fractures frequently occur in elderly patients. Early mobilization after rigid internal fixation of unstable intertrochanteric fractures has recently reduced the mortality and morbidity. Between March, 1976 and February, 1980, eighteen patients over 60 years old with unstable intertrochanteric fractures were treated by Jewett nailing after Dimon & Hughstons reduction at the department of Orthopaedic Surgery, Chung Nam University. Among the eighteen patients, twelve patients could be followed, ranging from 6 months to 2.1 years, with an average follow-up of 11 months. The results were obtained as follows: 1. The main causes of fractures were falling down and slip down. 2. In treatment of unstable intertrochanteric fractures by medial displacement and valgus nailing with Jewett nail, early ambulation and early weight bearing were possible with satisfactory results. 3. The average time for fracture union in twelve cases who were followed up were 17.1 weeks, but two cases with severe comminution of posterior and medial fragment of the trochanter revealed delayed union. 4. The medial displacement of distal fragment and valgus nailing in unstable intertrochanteric fracture shortened the operation time and reduced complications by early ambulation, but had disadvantages such as some limitation of motion of affected hip joints, shortening of affected extremities and delayed union.
Accidental Falls ; Aged ; Early Ambulation ; Extremities ; Femur ; Follow-Up Studies ; Hip Fractures ; Hip Joint ; Humans ; Mortality ; Weight-Bearing

The appendiceal tuberculosis is a rare from of skeletal tuberculosis in child and adults, arising from a focus or foci in posterior compartment of spine. This from may be unilateral and may be isolated or present at multiple levels, characterieed by slight change of the disk space and deformity, but large psravertebral abscess are invariable. Extradural extension may lead to the development of paraplegia. We had experienced one case of appendiceal tuberculosis at L1 and treated with antituberculosis medication and radical curettage, followed with body jacket cast.
Abscess ; Adult ; Child ; Congenital Abnormalities ; Curettage ; Humans ; Paraplegia ; Spine ; Tuberculosis

One case of Tachycardia-Bradycardia Syndrome was reported with brief review. A 42 year old woman was admitted to Seoul national University Hospital because of palpitation and dizziness. She had had the intermittent episodes of such attacks for 4 years. The electrocardiogram checked 3 days prior to admission showed atrial fibrillation but the electrocardiogram at admission revealed irregular bradycardia with wandering pacemaker. The patient had complained palpitation and dizziness throughout the hospital days, but only one attack of Adams-Stokes Syndrome was developed. She was improved symptomatically by isoproterenol infusion during the hospital course.
Adams-Stokes Syndrome ; Adult ; Atrial Fibrillation ; Bradycardia ; Dizziness ; Electrocardiography ; Female ; Humans ; Isoproterenol ; Seoul

Since shoulder have a higher proportion of muscle which would have low electrical resistance, there could be more electrical damage to the rotator cuff muscles. We present a patient with acute rotator cuff tear by sudden uncontrolled jerking contractions caused by an electrical shock. A case of 42-year-old man with acute rotator cuff tear due to electrical injury to the shoulder was presented. Magnetic resonance imaging showed a full thickness tear and an undulating appearance of the peripheral end of the torn supraspinatus and infraspinatus muscle, suggesting an acute complete rupture. By arthroscopic surgery, the torn rotator cuff tendons were repaired with a suture bridge technique. At the final follow-up, the patient had a full, pain-free range of motion and had fully recovered shoulder muscle power.
Adult ; Arthroscopy ; Burns, Electric ; Electric Impedance ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Muscle Contraction ; Muscles ; Range of Motion, Articular ; Rotator Cuff ; Rupture ; Shock ; Shoulder ; Sutures ; Tears ; Tendons

The nuclear accumulation of p53 protein is known to correspond with mutation of p53 tumor supressor gene. To investigate the relationsbip between clinico-pathologic features and expression of p53 mutations, 65 cases of primary cervical carcinoma were analysed by an immunohistochemieal method using monoclonal antibody of p53. Immunostaining demonstrated varioua nuclear stinings of cancer cells in 48.4% of squamous cell carcinoma(51.9% in large cell carcinoma and 25.0% in small cell carcinomas) and 23.5% of adenocarcinoma(p<0.05) No differences in clinical stage and p53 positivity were found. There was no significant correlation between p53 positive cases and status of recurrence. This data suggests that p53 protein is not correlated with the disease progression and prognosis.
Carcinoma, Large Cell ; Cervix Uteri* ; Disease Progression ; Female ; Genes, p53* ; Prognosis ; Recurrence

We have clinically evaluated 40 cases of high myopia(47 catarctous eyes) with an axial length of over 26 mm which were treated with ECCE and posterior chamber intraocular lens implantation from January 1987 to June 1990 at the 51. Mary's Hospital in the Department of Ophthalmology of Catholic University Medical College. The results were as follows: 1. Most common axial length was from 26.00 mm to 26.50 mm in 24 eyes(51.5%)and most common anterior chamber depth was from 2.51 mm to 3.00 mm in 22 eyes(48.6%). 2. The most common associated diseaes was vitreous opacity(19 eyes: 40.4%). 3. Posterior chamber lens power ranged from +10.00D to +14.00D, and the most frequently implanted posterior chamber lens power was +10.50D. 4. Postoperative corrected visual acuity was the same as preoperative corrected visual acuity in 46 eyes(97.9%) and improved up to 0.5 or more in 31 eyes(66.0%).
Anterior Chamber ; Lens Implantation, Intraocular ; Myopia* ; Ophthalmology ; Visual Acuity