A genetic and clinical study was done on 20 index cases and their families who had lived in Chonnam province, visited Chonnam University Hospital from 1968 through 1983, and were diagnosed as peudoxanthoma elasticum based on clinical and histopathological findings. In Chonnam province, pseudoxanthoma elsticum occurred in a incidence of more thar, one per 125,000. The male to female ratio was 1: 1.25 (8: 10) with only the cases of familial occurrence although the sex ratio was 1: l. 85 (7: 13) among 20 index cases. Among 8 index cases showing familial occurrence, 6 showed autosomal recessive inheritance with 5 Type 1 and 1 Type 2 whereas 2 showed the autosomal dominant inheritance with both of thern Type 2 (Pope), pointing out the genetic heterogeneity in pseudoxanthorna elsticum. The age of patients ranged from 12 to 42 years old at the time of their first visit to our hospital, but the age that the patients first recognized the disease onset ranged from 9 to 22 years old. The duration of t he disease was also variable ranging from 11/2 to 30 years. The angioid streaks were observed in 38g of the cases whose disease duration ranged from 11/2 to 20 years. The sites of skin lesions were the neck, axillae and groin in the order of frequency.
Adult ; Angioid Streaks ; Axilla ; Classification ; Female ; Genetic Heterogeneity ; Groin ; Humans ; Incidence ; Jeollanam-do ; Male ; Neck ; Pseudoxanthoma Elasticum* ; Sex Ratio ; Skin ; Wills ; Young Adult

No abstract available.
Bone Transplantation ; Maxilla ; Osteotomy

No abstract available.
Brain* ; Central Nervous System Diseases* ; Central Nervous System* ; Child* ; Humans

No abstract available.
Brain* ; Central Nervous System Diseases* ; Central Nervous System* ; Child* ; Humans

No abstract available.

No abstract available.
Occupations

Panner's disease, osteonecrosis of the capitellum of the elbow, was first reported by Panner in 1927. The disease occurs mainly in boys between 6 and 15 years old and shows unilateral distribution. Pain, stiffness, localized tenderness over the lateral condyle of the elbow, and decreased range of motion are the typical clinical symptoms. Conservative treatment is generally recommended for patients in the early stage of this disease. A few cases of Panner's disease have been reported and few are related to long-term follow-up results. To the best of our knowledge, all reported cases were over six years. Therefore, we report on a five-year-old boy diagnosed as Panner's disease that showed resorption and regeneration of the humeral capitellum with no limitation of motion over three years. The current study was exempted from review by the institutional review board because it was a single retrospective case report. Informed consent was obtained from the patient's guardian.
Child* ; Elbow ; Ethics Committees, Research ; Follow-Up Studies ; Humans ; Informed Consent ; Male ; Osteochondrosis ; Osteonecrosis ; Range of Motion, Articular ; Regeneration ; Retrospective Studies

PURPOSE: This study was performed to evaluate the level of I-TAC in tears of patients with dry eye syndrome and its correlation with the tear surface parameters. METHODS: I-TAC levels were measured by enzyme-linked immunosorbent assay (ELISA) in tear samples collected from 33 dry eye patients (16 patients with Sjogren syndrome and 17 patients with non-Sjogren syndrome) and 15 control subjects. The correlations between I-TAC levels and tear surface parameters including tear film break-up time (BUT), basal tear secretion, tear clearance rate (TCR), corneal sensation, keratoepitheliopathy and conjunctival goblet cell density were analyzed. RESULTS: The mean levels of I-TAC were 717+/-377 pg/ml in patients with dry eye and 444+/-119 pg/ml in control subjects (P=0.003). The mean levels in Sjogren syndrome patients (1261+/-324 pg/ml) were significantly higher than those of non-Sjogren syndrome patients (614+/-131 pg/ml, P=0.003). I-TAC levels correlated significantly with basal tear secretion (P=0.006), TCR (P=0.007), keratoepitheliopathy (P=0.022) and goblet cell density (P<0.001), but did not correlate with BUT (P=0.110) and corneal sensation (P=0.090). CONCLUSIONS: The level of I-TAC is elevated in tears of patients with dry eye syndrome including Sjogren syndrome. The level correlates with disease severity and tear surface parameters.
Dry Eye Syndromes ; Enzyme-Linked Immunosorbent Assay ; Eye ; Goblet Cells ; Humans ; Sensation ; Sjogren's Syndrome ; Tears

After surgical operation in patients with arteriovenous malformation (AVM), normal pressure perfusion breakthrough (NPPB) is one of the major complications. Brain perfusion SPECT with acetazolamide stress was known to be useful to evaluate the vascular reserve in several neurological and neurosurgical conditions. The authors performed acetazolamide brain perfusion SPECT in patients with AVM and compared the brain perfusion in the post-operative clinical courses. The acetazolamide brain perfusion SPECT was helpful in defining the prognosis of the patients with AVM. We describe 4 patients with AVM who had acetazolamide brain perfusion SPECT to examine the prognosis.
Acetazolamide* ; Arteriovenous Malformations* ; Brain* ; Humans ; Perfusion* ; Prognosis* ; Technetium Tc 99m Exametazime ; Tomography, Emission-Computed, Single-Photon*

PURPOSE: We tried to find out the urodynamic features of the male patients suffering with lower urinary tract symptoms (LUTS) and we wanted to know the effects of the preoperative parameters on the outcome of transurethral resection of the prostate (TURP). MATERIALS AND METHODS: One hundred ninety four patients who had LUTS and who had undergone urodynamic study were available for analysis. The history taking and physical examination, urinalysis, International Prostate Symptom Score (IPSS), transrectal ultrasonography, serum prostate-specific antigen (PSA) level check and urodynamic study were performed for all the patients. Also, the postoperative IPSS and satisfaction level were accessed for the patients who had undergone TURP for bladder outlet obstruction (BOO), and the preoperative factors such as age, prostate volume, PSA and urodynamic findings were analyzed. RESULTS: For the total 194 patients, there were 123 BOO patients (63.4%), 62 detrusor overactivity patients (13.4%) and 19 detrusor underactivity patients (9.8%). For the patients who had undergone TURP, the IPSS was decreased from a preoperative value of 20.5 to a postoperative value of 9.0. We divided the patients into two groups in accordance with the level of satisfaction after the operation. Within the two groups, the dissatisfied group showed a preponderance of preoperative detrusor overactivity. When performing IPSS, both groups showed a decreased score with each having a different range: from 20.8 to 6.1 in the satisfied group, and from 23.2 to 14.8 in the dissatisfied group. CONCLUSIONS: BOO is most common cause among the male patients who have LUTS and who are more than 50 years old, but other causes should be considered. Moreover, if BOO accompanies detrusor overactivity, the importance of the preoperative urodynamic findings deserves emphasis, and it will help to predict the results of performing TURP.
Humans ; Lower Urinary Tract Symptoms ; Male* ; Middle Aged ; Physical Examination ; Prostate* ; Prostate-Specific Antigen ; Transurethral Resection of Prostate ; Ultrasonography ; Urinalysis ; Urinary Bladder Neck Obstruction ; Urinary Tract ; Urodynamics*