Seckel syndrome is a rare, autosomal recessive disorder of severe growth retardation and distinct craniofacial, orodental, and skeletal anomalies. We report hereby the first two Korean cases of typical Seckel syndrome who had characteristic symptoms of intrauterine growth retardation, small head, large eyes, sharp facial features (beaked nose, dysplastic ears and narrow face) with underdeveloped chin, dwarfism, severe mental retardation, and other malformation. We report two cases of Seckel syndrome with a brief review of related literatures.
Chin ; Dwarfism ; Ear ; Fetal Growth Retardation ; Head ; Intellectual Disability ; Nose

No abstract available.
Breast Neoplasms* ; Breast* ; Estrogens*

Management of patients with unstable fracture or fracture-dislocation of thoraco-lumbar spine continues to be controversial. One area of disagreement involves spinal fusion with internal fixation for prevention of later spinal instability. Intemal fixation of the spine without fusion has been advocated in the treatment of the unstable thoraco-lumbar fracture or dislocation. During the period of January 1981 to March 1984, thirty-two patients underwent fixation by Harrington distraction rod without fusion for the treatment of thoraco-lumbar fractures. Eight cases among them had second operation for the removal of the Harrington rods in 13.4months after Harrington rod fixation and were followed up clinically and radiolagically for 1 year at the Department of Orthopaedic Surgery, St. Mary's Hospital, Catholic Medical College and Center. The rationales for this procedure were to minimize the number of permanently stabilized segments and to provide more spinal mobility. We have expected that spontaneous fusion of vertebral body occur by the prolonged immobilization with Hamngton distraction rods. After the Harrington rods, the average conection rate of kyphotic angle was 41.8% and average correction rate of height of collapsed vertebral body or displacement of vertebral body was 42.3% at one year follow up. Instrumentations in eight patient were removed at 13.4 months after the initial surgery and the patients were followed up for one year. At the time of removal of instrumentation, unilateral partial facetectomy was done for histologic examination of the facet joint which is above the lower Harrington hook in four patients. The average correction rate of kyphotic angle was 37.5% and the average correction rate of height of collapsed or displaced vertebral body was 40.6% at one year after the removal of H-rods. The range of spine motions were incresed gradually. But histologic findings of the immobilized facet joint revealed fibrillation, fissures, thinning of the normal cartilagenous surface, decreased cartilagenous cellularity, and vascular tidemark invasion which are characteristics of osteoarthritis. Through this study, we obtained following conclusions: 1. Immediate Harrington distraction rodding was effective for the anatomic reduction of the collapsed vertebral body. And it provided immediate spinal stability, and spontaneous fusion of vertebral body. 2. Prolonged immobilization of the spine by Harrington rods is believed to have deleterious effect on articular cartilage of the immobilized facet joint as the degenerative changes of the cartilage of the facet joint was consistently found in this study. And it was regarded as a predisposing factors in the development of symptomatic arthritis of the spine. 3. If a fractured and collapsed vertebral body can be reduced by Harrington distraction rods, a spontaneous fusion of vertebral body occur. Harrington rodding without fusion could be one of the useful surgeries for management of the fractures and fracture-dislocation of the spine.
Arthritis ; Cartilage ; Cartilage, Articular ; Causality ; Dislocations ; Follow-Up Studies ; Humans ; Immobilization ; Osteoarthritis ; Spinal Fusion ; Spine ; Zygapophyseal Joint

BACKGROUND: Urticaria pigmentosa(UP) is primarily a disease of children. There have been no clinical studies of UP in Korea. OBJECTIVE: This study was performed to find the clinical characteristics of UP in Korean child-hood patients. METHOD: Twenty-nine cases of urticaria pigmentosa confirmed clinically and histopathologically were analyzed. RESULTS: The maculopapular type was the most common with the usual age of onset before the age of 6 months, and the lesions tended to be distributed in the central portion of the body. Darier sign was positive in 92% of the patients(24/26). No systemic involvements were detected in any of the patients with minimal associated symptoms. Seven patients of the maculopapular type and one patient of the multiple nodular type followed up for more than 2 years showed a tendency to improve or clear by the age of 6 years. CONCLUSION: Neonatal or infantile-onset patients of UP in Korean pediatric population were considered to have a benign clinical course and to require no aggressive therapy.
Age of Onset ; Child* ; Clinical Study* ; Humans ; Korea ; Methods ; Urticaria Pigmentosa* ; Urticaria*

No abstract available.
Myocardial Infarction*

PURPOSE: We evaluated the diagnostic usefulness of the renal resistive index in acute unilateral ureteral obstruction. MATERIALS AND METHODS: Our study consisted of 26 patients, with 52 kidneys, who presented with unilateral acute ureteral obstruction documented with excretory urography, and 20 men with both kidneys normal. The contralateral nonobstructed kidneys served as control. All patients underwent Doppler ultrasonography for determination of the resistive index (RI) and the difference between RI of the corresponding and contralateral kidney (deltaRI). RI and deltaRI were considered positive for obstruction at values of 0.70 or greater and 0.04 or greater, respectively. We calculated the sensitivity, specificity, and positive predictive values of RI and deltaRI for the diagnosis of acute ureteral obstruction. RESULTS: Kidneys with ureteral obstruction (n=26) had a mean RI of 0.73+/-0.05, contralateral normal kidneys (n=26) had a mean RI of 0.63+/-0.04, a significant difference (p<0.05). Similarly, the mean deltaRI of the patients was significantly higher than that of men with both normal kidneys (0.08+/-0.006 versus 0.02+/-0.004, respectively). The sensitivity for RI was 70%, the specificity was 82%. The corresponding values for deltaRI were 88% and 93%, respectively. The positive predictive values were 85% and 91%, respectively. CONCLUSIONS: Renal resistive index measurements are not valuable for the definite diagnosis of acute obstructive uropathy. Nevertheless, they are helpful in situations in which invasive radiologic study is undesirable.
Diagnosis ; Humans ; Kidney ; Male ; Sensitivity and Specificity ; Ultrasonography, Doppler ; Ureter* ; Ureteral Obstruction* ; Urography

BACKGROUND: The diastolic transmitral flow velocity pattern has been commonly used to assess left ventricular(LV) diastolic function. The effects of multiple factors(such as, LV preload, afterload and heart rate, etc.) make difficulties in accurate interpretation. METHODS: In order to investigate the diastolic transmitral filling patterns according to the changes of the proload or heart rate, we studied 27 normal subjects with pulsed Doppler echocardiography after the administration of nitroglycerin(0.6mg/tab.) sublingually or atropine(0.5mg/amp.) intravenously. RESULTS: 1) After nitroglycerin administration, the folowings were obtained. ; The systolic blood pressure and LV diastolic filling time(DFT) decreased by 10.1% and 15.3%, respectively(p<0.001), compared with baseline data. The ratio of peak early to late diastolic transmitral flow velocities (E/A) and time-velocity integrals(TVIE/TVIA) decreased by 10.3% and 14.8%, respectively(p<0.01). The early diastolic filling time(Time E) was unchanged. Therefore, we suggest that time E is helpful, compared with the increments of the preload or the diastolic dysfunction. 2) After atropin administration, the heart rate, peak late diastolic transmitral flow velocity(PA) and percent atrial contribution(%AC) significantly increased by 43.6%, 25.1% and 41.4%, respectively(p<0.001). The E/A, TVIE/TVIA and DFT significantly decreased by 42.9%, 38.9% and 43.0%, respectively(p<0.001) compared to the data before drug administration. 3) The heart rate correlated negatively to the E/A, TVIE/TVIA and DFT. It was correlated positively to %AC(r=+0.63; p<0.001). The normalized E/A ratio by DFT(E/A/DFT) didn't correlate. Therefore, E/A/DFT is helpful on the exclusion of the influences of heart rate by the administration of the atropine. CONCLUSION: The decrement of preload or the increment of heart rate changes the diastolic transmitral flow velocity patterns. Therefore, when the diastolic function is assessed by interpretation of the Doppler transmitral flow velocity pattern with pulsed Doppler echocardiogram, the potential influences of preload and heart rate must be taken into account.
Atropine* ; Blood Pressure ; Echocardiography ; Echocardiography, Doppler* ; Echocardiography, Doppler, Pulsed ; Heart Rate ; Humans* ; Nitroglycerin* ; Time

No abstract available.
Microvascular Decompression Surgery*

No abstract available.

No abstract available.
Adenoids* ; Carcinoma, Adenoid Cystic* ; Salivary Glands*