Refractory deformity in ankylosing spondylitis is caused by loss of normal lordotic curvature in lumbar spine. For the correction of deformity, monosegmental osteotomy, intracorporal decancellation and multisegmental osteotomy are used. Among them, multisegmental osteotomy is reported to be safe because of its small correction amount at each level. Since 1989, authors treated 5 cases of ankylosing spondylitis with severe kyphotic deformity by multisegmental osteotomy and transpedicular instrumentation. All were young males between 22 and 35 years of age. Preoperative kyphotic deformity was 80°, 105°, 72°, 35°, 55° (Av. 70°) and amount of correction was 55°, 105°, 72°, 20°, 40° (Av. 58°) respectively. Levels for osteotony were 4 to 8 segments and correction at a level was 5° to 13° (Av. 8.3°). Normal standing upright posture and vision for straight forward were obtained in all. Instrumentation was Zielke in three Cotrel-Dubousset in two. One case of Zielke instrumentation showed rod failure. However, all showed solid bony union without any loss of correction or pseudoarthrosis. From the above experience, multisegmental osteotomy for the treatment of kyphotic deformity in ankylosing spondylitis was believed to be a safe and effective method of treatment.
Congenital Abnormalities ; Humans ; Male ; Methods ; Osteotomy ; Posture ; Pseudarthrosis ; Spine ; Spondylitis, Ankylosing

No abstract available.
Tonsillectomy*

The closed reduction has made an important contribution to the treatment of children under the age of 24 months. The authors report a clinical experience of 20 patients(21 hips) with congenital dislocation of hip who were treated by closed reduction at the department of Orthopaedic Surgery of Chonnam University Hospital. There were total 230 patients(237 hips) disgnosed as having congenital dislocated hip for 10 years from 1978 to 1987 and 20 patients of them were included in this study. The follow-up period ranged from 1 year to 10 years with an average 3 years. The results are as follows: l. All but one were girls, comprising 19 (20 hips) of 20 patients. 2. The age at closed reduction were under 6 months in 2, 7 to 12 months in 4, 13 to 18 months in 10 and 19 to 24 months in 4. 3. Of the 21 hips treated by closed reduction, 17 hips achieved a satisfactory result(81%). The other four hips had redislocation(2 hips) and subluxation(2 hips). 4. Four additional treatments were open reduction in 3 and Salter innominate osteotomy in l. 5. The acetabular and center-edge angle improved from the averge 36.1 and
Acetabulum ; Child ; Dislocations ; Female ; Follow-Up Studies ; Hip ; Humans ; Jeollanam-do ; Joints ; Methods ; Necrosis ; Osteotomy

BACKGROUND: Ropivacaine is an amino amide local anesthetic that has an advantage of a low-toxicity profile. Clonidine, an alpha2 adrenergic agonist, is known to prolong and intensify anesthesia from epidural local anesthetics. The aim of this study was to evaluate the dose-response effects of added clonidine to ropivacaine-fentanyl epidural anesthesia for lower extremity surgery. METHODS: Forty-five patients undergoing lower extremity surgery were randomly allocated to three groups to be given the following agents by an epidural route: 0.75% ropivacaine 15 ml+fentanyl 50microgram with clonidine 50microgram (group I), 100microgram (group II), or 150microgram (group III). Onset and maximal height of sensory block, and duration of sensory and motor block were assessed. Also, blood pressure, heart rate and sedation score were measured. RESULTS: Duration of sensory and motor block of groups II and III was significantly longer than that of group I, but there was no difference between group II and group III. Two segment regression times were significantly different among the three groups. Onset of sensory block, blood pressure, heart rate, and sedation scores were not significantly different among the three groups. CONCLUSIONS: The addition of Clonidine 100 and 150microgram prolonged duration of anesthesia more than 50microgram with ropivacaine-fentanyl epidural anesthesia for lower extremity surgery.
Adrenergic Agonists ; Anesthesia ; Anesthesia, Epidural* ; Anesthetics, Local ; Blood Pressure ; Clonidine* ; Heart Rate ; Humans ; Lower Extremity*

Morning glory syndrome (MGS) is a rare congenital optic disc anomaly with a characteristic fundal finding with severe visual impairment. It may occur in association with various systemic manifestations, even though most of the reported cases were isolated. A 6-year-old male visited the nephrology clinic with a history of microscopic hematuria and at the age of 12 years, he was diagnosed thin glomerular basement membrane nephropathy by kidney biopsy. After the following years, the patient had progressive deterioration of visual acuity, and diagnosed as MGS. Whole Exome Sequencing of this patient and his mother revealed heterozygous COL4A4 mutations [c.81_86del (p.Ile29_Leu30del)]. It is more reasonable to consider MGS seen in this patient as a coincidental finding of autosomal dominant Alport syndrome. To our knowledge, this case represents the first case report of autosomal dominant Alport syndrome associated with MGS.

Nonautoimmune hyperthyroidism is a very rare cause of congenital hyperthyroidism that is usually caused by an activating mutation in the thyroid-stimulating hormone receptor (TSHR) gene. In this report, we describe a case of nonautoimmune hyperthyroidism in a patient with TSHR mutation. Our patient was the younger of a set of twins born at 36 weeks and 6 days of gestation. The patient was noted to be more irritable than the older twin at 80 days of age, and the mother was taking methimazole for Graves’ disease that had been diagnosed 12 years prior. Therefore, a thyroid function test was conducted for the patient. The results revealed subclinical hyperthyroidism, and tests of antithyroglobulin antibody, antithyroid peroxidase antibody, and anti-thyroid-stimulating hormone (TSH) receptor antibody were all negative. During follow-up, at around 4 months of age, free T4 increased to 2.89 ng/dL, and TSH was still low at 0.01 μIU/mL; therefore, 3 mg/day of methimazole was initiated. Whole-exome sequencing showed a heterozygous variant of c.1800C>T (p.Ala627Val) in the TSHR gene. Testing in the family confirmed an identical variant in the patient's mother, leading to diagnosis of familial nonautoimmune hyperthyroidism inherited in an autosomal dominant pattern. This is the second report of A627V confirmed as a germline variant.