Chronic granulomatous disease (CGD) is one of congenital immunodeficient disease and a rare X-linked or autosomal recessive disease characterized by recurrent life- threatening infections and granuloma formation. We observed clinical features, laboratory findings and genetic subgroups of 33 children who were diagnosed with chronic granulomatous disease in the Department of Pediatrics, Seoul National University Children's Hospital. There were 23 males and 10 females. Activated NBT test of all patients revealed 0% positive cell and mothers of 15 patients had 25%- 75% normal neutrophils in the activated NBT test. According to the result of activated NBT test and family history, the ratio of X-linked and autosomal recessive inheritance was 2:3. There was a significant difference for the age at onset of the first infection in the different genetic subgroups. The X-linked group had the mean onset at 1.98 months of age and autosomal recessive group had a mean onset as late as 3.82 months (p<0.05). The most common type of the first infection was lymphadenopathies (41%) and other infections were skin pustules, fever, perianal abscess, pneumonia and chronic diarrhea. However, the age at diagnosis was not significant in the different genetic subgroups. Lymphadenitis (27%) was the most common infection, and pneumonia, gastrointestinal tract infection, skin infection were also common. The most common infectious agent was Candida sp. (5%) and other microorganisms involved were BCG, coagulase-negative staphylococcus, S. aureus, K/ebsiella pneumoniae, Aspergi/lus sp., and Enterococcus faecium. Chronic condition associated with CGD were hepatomegaly (59%), splenomegaly, and anemia of chronic disease, underweight, and lymphadenopathy. The leukocyte count of patients at diagnosis was within normal limit except in three patients and leukopenia was not observed in any of the patients. The humoral and cellular immunity and complement system were normal, but the level of Ig E in four patients was elevated. Early diagnosis of CGD can be made by suspicion if there are lymphadenitis after BCG vaccination and recurrent pyogenic infections under the first year of age. Though progression in the treatment of CGD, like gene therapy, is concerned, genetic counseling and prenatal diagnosis by carrier detection and molecular genetic analysis is thought to be necessary.
Abscess ; Anemia ; Candida ; Child ; Chronic Disease ; Complement System Proteins ; Diagnosis ; Diarrhea ; Early Diagnosis ; Enterococcus faecium ; Female ; Fever ; Gastrointestinal Tract ; Genetic Counseling ; Genetic Therapy ; Granuloma ; Granulomatous Disease, Chronic* ; Hepatomegaly ; Humans ; Immunity, Cellular ; Korea* ; Leukocyte Count ; Leukopenia ; Lymphadenitis ; Lymphatic Diseases ; Male ; Molecular Biology ; Mothers ; Mycobacterium bovis ; Neutrophils ; Pediatrics ; Pneumonia ; Prenatal Diagnosis ; Seoul ; Skin ; Splenomegaly ; Staphylococcus ; Thinness ; Vaccination ; Wills

This study investigated the influence of IRM on marginal microleakage of 5th generation adhesives. Class V cavities with gingival margins in dentin were prepared on both buccal and lingual surfaces of 60 extracted human molar teeth. Prepared teeth were randomly divided into six groups. Group 1 and 4 received no temporary restoration with IRM. Group 2 and 5 were covered with IRM mixed at P/L ratio(10g/1g). Group 3 and 6 were covered with IRM mixed at P/L ratio(10g/2g). The temporary restorations were removed mechanically with an ultrasonic scaler after one-week storage in distilled water. The cavities were restored using one of two adhesives and composites; Single Bond/Filtek Z 250(Group 1, 2 and 3), UniFil Bond/UniFil F(Group 4, 5 and 6). Following one day storage in distilled water, the restored teeth were thermocycled for 500 cycles(between 5degrees C and 55degrees C) and immersed in 2% methylene blue for dye penetration testing. The results were analysed using Kruskal-Wallis Test, Mann-Whitney and Wilcoxon signed ranked test at a significance level of 0.05. The results of this study were as follows: 1. Ranking of mean microleakage scores at the enamel margins was Group 10.05). 4. At the dentin margins, the microleakage of the group not pretreated with IRM was lower than that of the group pretreated with IRM. And the microleakage of UniFil Bond was lower than that of Single Bond. 5. Compared with microleakages between the enamel and dentin margins of each groups, Group 1, 2, 3, 4, 5 and 6 at dentin margin were higher microleakage than those at enamel margin. There were significant difference between enamel and dentin microleakage of Group 2 and 3(p<0.05).
Adhesives* ; Dental Enamel ; Dentin* ; Humans ; Methylene Blue ; Molar ; Tooth ; Ultrasonics ; Water

To differentiate the postoperative changes from the recurrence of tumor and to evaluate MR imaging of early postoperative leptomeningeal seeding in medulloblastoma, We have retrospectively analysed 34 cases of MR images of 17 patients who were confirmed as medulloblastoma by histopathology. Noncontrast and postcontrast T1 weighted MR images were obtained in all patients. In 11 patients follow-up MR was done more than once (average:1.5 times) and average interval of MR imaging was 6 months. The timing of 34 MR images was as follow: 6 case within 2 months, 9 cases between 2 months and 1 year, 19 cases more than 1 year after surgery respectively. MR images within 2 month after surgery revealed contrast enhancement at operation site and adjacent meninges, hemorrhage, residual tumor. In patients who had no evidence of tumor recurrence, these early postoperative changes were markedly decreased within 6 month after sugery. MR images obtained more than 1 year after sugery showed no abnormal contrast enhancement or mild focal dural enhancement at operation site. Diffuse moderate dural enhancement was noted in one patient who had the history of post-surgical subdural hemorrhage. In six patients with tumor recurrences which were detected from as early as 9months to 6 years after surgery, the findings of recurrence included leptomeningeal enhancement of brain stem and cerebellar surface at early stage, variable sized enhancing leptomeningeal nodules, linear or irregular sulcus obliterating enhancing lesions, enhancing mass at primary or metastatic site. We have concluded that leptomeningeal enhancement detected after 6 months of surgery is an important MR finding suggesting the possibility of tumor recurrence. Small nodular and linear enhancement of leptomeninges at brainstem or cerebellar surface is considered as the early manifestation of intracranial tumor seeding.
Brain Stem ; Follow-Up Studies ; Hematoma, Subdural ; Hemorrhage ; Humans ; Magnetic Resonance Imaging* ; Medulloblastoma* ; Meninges ; Neoplasm, Residual ; Recurrence ; Retrospective Studies

One patient underwent transpubic urethroplasty in posterior urethral stricture, Reseciton of both pubic bones was proved excellent exposure for urethroplasty without any orthopedic disabilities. The result was satisfactory in the patient without incontinence or impotence.
Erectile Dysfunction ; Humans ; Male ; Orthopedics ; Pubic Bone ; Urethral Stricture*

PURPOSE: Functional MR imaging is the method of demonstrating changes in regional cerebral blood flow produced by sensory, motor, and any other tasks. Functional MR of visual cortex os performed as a patient stares a photic stimulation, so adaptable photic stimulation is necessary. The purpose of this study to evaluate whether the size of photic stimulation can affect the degree of visual cortex activation. MATERIALS AND METHODS: Functional MR imaging was performed in 5 volunteers with normal visual acuity. Photic stimulator was made by 39 light-emitting diodes on a plate ,operating at 8 Hz. The sizes of photic stimulator were full field, half field and focal central field. The MR imager was Siemens 1.5-T Magenton Vision system, using standard head coil. Functional MRI utilized EPI sequence (TR/TE=1.0/51. 0msec, matrix No.=98x128, slice thickness=8mm) with 3sets of 6 imaging during stimulation and 6 imaging during stimulation and 6 imaging during rest, all 36 scanning were obtained. Activated images were obtained using postprocessing software(statistical analysis by Z-zone, and these images were combined with T-1 weighted anatomical images. The activated signals were quantified by numbering the activated pixels, and activation index was obtained by dividing the pixel number of each stimulator size with the sum of the pixel number of 3 study using 3 kinds of stimulators. The correlation between the activation index and the stimulation size was analysed. RESULTS: Mean increase of signal intensities on the activation area using full field photic stimulator was about 9.6%. The activation index was greatest on full field. second on half field and smallest in focal central field in 4, The index of half field was greater than that of full field in 1. The ranges of activation index were full field 43-73%(mean 55%), half field 22-40%(mean 32%), and focal central field 5-24%(13%). CONCLUSION: The degree of visual cortex activation increases with the size of photic stimulator.
Head ; Humans ; Magnetic Resonance Imaging* ; Photic Stimulation ; Visual Acuity ; Visual Cortex* ; Volunteers

Ureteroneocystostomy may be used in the cases of the vesicoureteral reflux, reteral stricture, ectopic ureteral orifice, ureterovaginal fistula and damage on the lower part of ureter during operation. 0nce ureteroneocystostomy is decided on every effort should be made to perform an antireflux procedure. The results of ureteroneocystostomy have improved consistently during last 20 years, but cases of reflux and postoperative ureterovesical obstruction are still encountered. A clinical study was made on 8 cases who had been performed ureteroneocystostomy at the Department of Urology, Jeonbug National University, Medical School from January 1978 to February 1980. The results were as follows` 1. The age of the patients ranged from 14 to 56 years, most common in fifth decade. The sex ratio was 3:1, with female 6 and male 2. 2. Underlying diseases for ureteroneocystostomy were ureteral stricture, megaloureter, ectopic ureteral orifice and vesicoureteral reflux. 3. 3 Ureters were performed with the method of paquin procedure, 2 ureters with the combination of paquin with Psoas-bladder hitch procedure, one ureter with the combination of Boari flap with Psoas-bladder hitch procedure, one ureter with Politano-Leadbetter procedure and one ureter with Lich procedure 4. Relatively good results of ureteral reimplantation were obtained in distal ureteral stricture, ectopic ureteral orifice and vesicoureteral reflux, except one case of megaloureter in which nephroureterectomy was performed later due to adynamic ureter 5. Complications were followed as persistent pyuria in one case and urine leakage in one case, and nephroureterectomy for adynamic ureter in one case.
Constriction, Pathologic ; Female ; Fistula ; Humans ; Jeollabuk-do ; Male ; Pyuria ; Replantation ; Schools, Medical ; Sex Ratio ; Ureter ; Urology ; Vesico-Ureteral Reflux

Neurilemmomas of the extremities are nerve sheath tumors involving peripheral nerves of the extremities. We analyzed MR images of 16 surgically proved tumors in 12 patients and compared the MR images with gross pathologic specimens in two tumors (2/16). Spin echo T1-weighted images were obtained in all the patients but T2-weighted images were obtained in six patients with a gradient echo technique. Gadolinium dimeglumine enhanced T1-weighted images were obtained in 11 patients (15/16). Signal on T1-weighted images was iso or slightly higher than that of the adjacent muscles. Signal on T2-weighted images ranged from homogeneous high to heterogeneous. After Gadolinium injection, all the 15 tumors showed enhancement. Small sized tumors were enhanced homogeneously but there was a tendency tobe enhanced heterogeneously to the central portion with peripheral rim enhancement as the size of the tumors increased. Pathologically, the enhanced portion was correlated the with solid protion was correlated the with solid portion of the tumors. Also encapsulation of the mass (12/16), suspended nerve strings (9/16) and bony erosion (1/16) were detected. MR characteristics of neurilemmoma include heterogeniety on enhanced T1 and T2 weighted images, encapsulation, and suspended nerve strings. Signal heterogeneity on enhanced T1 and T2 weignted images may be attributed to the variable cellularity, cystic changes, vascularity and focal hemorrhage of the tumors.
Extremities ; Gadolinium ; Hemorrhage ; Humans ; Magnetic Resonance Imaging* ; Muscles ; Nerve Sheath Neoplasms ; Neurilemmoma* ; Peripheral Nerves ; Population Characteristics

Neurilemmomas of the extremities are nerve sheath tumors involving peripheral nerves of the extremities. We analyzed MR images of 16 surgically proved tumors in 12 patients and compared the MR images with gross pathologic specimens in two tumors (2/16). Spin echo T1-weighted images were obtained in all the patients but T2-weighted images were obtained in six patients with a gradient echo technique. Gadolinium dimeglumine enhanced T1-weighted images were obtained in 11 patients (15/16). Signal on T1-weighted images was iso or slightly higher than that of the adjacent muscles. Signal on T2-weighted images ranged from homogeneous high to heterogeneous. After Gadolinium injection, all the 15 tumors showed enhancement. Small sized tumors were enhanced homogeneously but there was a tendency tobe enhanced heterogeneously to the central portion with peripheral rim enhancement as the size of the tumors increased. Pathologically, the enhanced portion was correlated the with solid protion was correlated the with solid portion of the tumors. Also encapsulation of the mass (12/16), suspended nerve strings (9/16) and bony erosion (1/16) were detected. MR characteristics of neurilemmoma include heterogeniety on enhanced T1 and T2 weighted images, encapsulation, and suspended nerve strings. Signal heterogeneity on enhanced T1 and T2 weignted images may be attributed to the variable cellularity, cystic changes, vascularity and focal hemorrhage of the tumors.
Extremities ; Gadolinium ; Hemorrhage ; Humans ; Magnetic Resonance Imaging* ; Muscles ; Nerve Sheath Neoplasms ; Neurilemmoma* ; Peripheral Nerves ; Population Characteristics

The number of post-irradiated surgical specimen appears to have risen in recent years due to the increasing utiliz ation of radiotherapy for cancer patients. Radiation therapy damages cells by its effects on the deoxyribonucleic acid and the spectrum of injury ranges from acute self-limited lesion to irreversible chronic lesion. We reviewed 24 cases of post-irradiated specimen and thought that time interval is the main factor influencing the morphologic change. Within six weeks, the individual cytologic changes such as biz arre nuclei, altered nucleus/cytoplasm ratio, amphophilic and vacuolated cytoplasm are noted. Chronic injury resulting from progressive changes in the fibrovascular tissue of the radiated area occur in six months after the initial course of radiation and the vascular changes of intimal thickening and fibrosis, foamy histiocytes within media, periadventital fibrosis and chronic inflammatory cells infiltration are present. Althouhg above mentioned finding are not pathognomonic, we thought them quite constant nd reproducible characteristics of radiation injury.

Renal angiogram is a safe and excellent procedure for an early detection and accurate diagnosis of benign or malignant diseases. Selective renal angiography has been used mainly in the diagnosis of surgical disease, but now many informations are available regarding the vascular disease, pyelonephritis and renal tuberculosis. A clinical observation was made on 18 cases of the renal angiography from January. 1978 to June, 1981.
Angiography* ; Carcinoma, Renal Cell ; Diagnosis ; Pyelonephritis ; Tuberculosis, Renal ; Vascular Diseases