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.

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

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

No abstract available.
Fractures, Open*

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

It is difficult to analyze the chemical compositions of aqueous humor because of the impossibility of collection of aqueous humor in normal situation, lack of analyzing technique, limited amount of aqueous humor. We measured the glucose concentration of aqueous humor both in cataractous patients with diabetes mellitus and in cataractous patients without diabetes mellitus, thus we compared and analyzed glucose concentration in aqueous humor and correlation between blood glucose con centration and aqueous humor glucose concentration in cataractous patients with diabetes. The mean concentration of glucose in aqueous houmor in diabetics was 92.81 +/- 35.58mg/dl and that of non-diabetics was 53.62 +/- 34.42mg/dl. Aqueous glucose concentration in diabetics was statistically significantly higher than that in non-diabetics.
Aqueous Humor* ; Blood Glucose ; Cataract* ; Diabetes Mellitus ; Glucose* ; Humans

The aim of this study is to evaluate the effect of viscoelastic substance on corneal thickness, corneal endothelium and intraocular pressure by measuring corneal thickness, corneal endothelial cell density and intraocular pressure in extracapsular cataract extraction with posterior chamber lens implantation. The study comprised of 50 patients, divided into two groups: extracapsular cataract extraction with posterior chamber lens implantation using Healon(R) or 2% methylcellulose(Metrol(R)). The txtracapsular cataract extraction was done by irrigation and aspiration technique and the posterior chamber lens was implanted into posterior chamber. The corneal thickness and endothelial cell density were measured preoperatively and at 6 weeks of postoperative period: intraocular pressure was measured preoperatively and at 24 hours of postoperative preiod. The results were as follows: 1. The preoperative corneal thickness, endothelial cell density and intraocular pressure were 499 +/- 34 micrometer, 2523 +/- 388 cells/mm2 and 15.9 +/- 2.4mmHg in average respectively. 2. The postoperative corneal thickness with Healon(R) was 501 +/- 42 micrometer, and with Metrol(R) was 491 +/- 28 micrometer. There was no significant difference between Healon(R) used and Metrol(R) used group(p<0.05). 3. The loss of endothelial cell with Healon(R) was 10.7%, and with Metrol(R) was 17.1%. The endothelial cell loss was lower in Healon(R) used group than that of Metrol(R) group. 4. The intraocular pressure with Healon(R) was 14.6 +/- 3.9mmHg, and with Metrol(R) was 12.3 +/- 4.4mmHg. There was no significant difference between Healon(R)-used and Metrol(R)-used group(p<0.05).
Cataract Extraction* ; Cataract* ; Cornea* ; Endothelial Cells ; Endothelium, Corneal ; Humans ; Intraocular Pressure* ; Postoperative Period