Spondylometaphyseal dysplasia (SMD) is an extremely rare, which affects the spine and metaphy-ses of the tubular bones on terms of enchondrogenesis. Children who had Kozlowski dwarfism, type of SMD are not recognized until they reach school age since they have normal clinical feature, weight and size in early childhood. Authors experienced a typical case of Kozlowski type of SMD in a 10 years old male who had i) generalized platyspondyly with anterior tapering of vertebrae ii) generalized metaphyseal dysplasia iii) minimal changes in the carpal and tarsal bones. This case is to be reported with review of references.
Child ; Dwarfism ; Humans ; Male ; Spine ; Tarsal Bones

The physeal injuries in children occur result of trauma, infection, metabolic or ischemic disease, and these conditions cause significant problems, such as growth disturbance, deformity and premature closure of physis. The authors experienced 7 cases of children who had angular deformity of knee and limb shortenings, but no history of obvious causes of physeal injury. All of these cases had severe osteoporosis and underlying disease, which were osteogenesis imperfecta, fibrous dyplasia and long term steroid therapy. The purpose of this study are evaluation of pathomechanism of physeal injuries in severe osteoporotic children. Patients were range of age from 4 to 9 years and had common clinical features, which were angular deformities of knee, limb shortening. Also they had common radiographic findings, that were severe porosis, metaphyseal sclerosis and in some cases, pop corn like calcification around physis. At this, we supposed that chronic mechanical stress in osteoporotic bone make physis be damaged due to loss of capillary circulation and this event appear to result from collapse of bone plate, through which epiphyseal vessel penetrate. In conclusion, the children with severe osteoporosis should be observed carefully about physeal injuries, which pathomechanism are perhaps deprivation of capillary circulation through bone plate. Therefore, We should try to protect bone plate by being conscious of its existence.
Bone Plates ; Capillaries ; Child ; Congenital Abnormalities ; Extremities ; Humans ; Knee ; Osteogenesis Imperfecta ; Osteoporosis* ; Sclerosis ; Stress, Mechanical ; Zea mays

No abstract available.

No abstract available.

The plica is remaining synovial septa in adult life which developed in early fetal life. The suprapatellar plica separates the suprapatellar pouch from the knee joint which sometimes has chnical significance according to its shape, but it has been occasionally overlooked and also pathophysiology of symptomatic plicae may be hard to explain. The authors experienced 7 cases of suprapatellar plica syndrome which mimic soft tissue tumor in 7 patients who had complained of vague pain and ill defined mass around the knee and by arthroscopy found the imperforated suprapatellar plica in which increased hydraulic pressure cavity evokes the clinical symptoms and signs exarnination from 1992 to 1997. We suggest that the suprapatellar plica with complete septum might be clinically significant in patients who are in active life.
Adult ; Arthroscopy ; Humans ; Knee ; Knee Joint

Recently, xenograft is being increasingly used in spinal fusion to reduce complications such as pain or bleeding following autograft bone grafting. The purpose of this study was to compare the fusion rate and clinical results between autograft and xenograft in 69 patients who had posterolateral lumbar fusion with instrumentation from March 1989 to April 1997. The xenograft was mixed with autogenous bone chips obtained from decompresion. Fusion and clinical results were evaluated with Furguson's method and Modified Smiley-Webster' scale, respectively. The mean follow-up time was 26 months with a minimum of 12 months and a maximum of 41 months. The results are as follows: fusion rates of autograft were 64.6% in Furguson grade A, 25.8% in grade B, 6.4% in grade C and 3.2% in grade D. Xenograft was 15.7% in grade A, 47.6% in grade B, 23.6% in grade C and 13.1% in grade D. Clinical results of autograft were excellent in 29.0%, good in 61.3%, fair in 6.5% and poor in 3,2%. Xenograft was excellent in 23.8%, good in 57,8%, fair in 15.8% and poor in 2.6%. Autograft showed a higher fusion rate of 90.4% than xenograft of 63.3% in grade A and B (P=0.012). Clinical results were not different between autograft and xenograft groups (P=0.494). However, the xenograft group showed lower fusion rate than the autograft group. The results indicated that xenograft is less useful for posteolateral fusion of the lumbar spine.
Autografts* ; Bone Transplantation ; Follow-Up Studies ; Hemorrhage ; Heterografts* ; Humans ; Spinal Fusion ; Spine

PURPOSE: Renal tumors consist of heterogeneous groups that frequently show complex and overlapping morphology, thus making it difficult to make a correct diagnosis. One of the most problematic differential diagnoses is to distinguish chromophobe renal cell carcinoma (RCC) from oncocytoma. These should be distinguished by differences in their behavior and clinical outcome. Our study was performed to identify whether caveolin-1 and MOC-31 are useful immunohistochemical markers for differentiating chromophobe RCC from oncocytoma. MATERIALS AND METHODS: We selected 23 chromophobe RCCs, 8 oncocytomas, and 25 clear cell RCCs and performed immunohistochemical staining for caveolin-1 and MOC-31. RESULTS: Caveolin-1 was positive in 20 (87%) of 23 chromophobe RCCs, 0 of 8 oncocytomas, and 21 (84%) of 25 clear cell RCCs. MOC-31 was positive in 22 (96%) of 23 chromophobe RCCs, 2 (25%) of 8 oncocytomas, and 14 (56%) of 25 clear cell RCCs. There was a statistically significant difference in the expression of caveolin-1 and MOC-31 between chromophobe RCC and oncocytoma (p<0.001). In addition, clear cell RCC was also significantly different from oncocytoma in the expression of caveolin-1 (p<0.001) and was significantly different from chromophobe RCC in the expression of MOC-31 (p<0.001). CONCLUSIONS: Caveolin-1 and MOC-31 can be useful markers in the differential diagnosis of chromophobe RCC, oncocytoma, and clear cell RCC.
Adenoma, Oxyphilic ; Antibodies, Monoclonal ; Carcinoma, Renal Cell ; Caveolin 1 ; Diagnosis, Differential ; Kidney Neoplasms

The term tracheal bronchus refers to an abnormal bronchus that comes directly off of the lateral wall of the trachea (above the carina) and supplies ventilation to the upper lobe. Tracheal bronchi occur almost exclusively on the right trachea and are associated with other congenital anomalies. In addition, tracheai bronchus may be related to other inflammatory conditions with persistent wheezing, such as recurrent pneumonia, chronic bronchitis and bronchiectasis, which is a result of the relatively poor local drainage of the involved bronchi. An infant with recurrent wheezing is likely to be a challenge for a clinician in the evaluation of the etiology of airway obstruction and in the differential diagnosis of wheezy breathing. The authors report a case of an 8-month-old female infant with a ventricular septal defect, who presented with stridor and recurrent respiratory infection and finally was finally diagnosed with a tracheal bronchus using computed tomography and a bronchoscopy. Therefore, tracheal bronchus should be included in the differential diagnosis of any child who presents with chronic or recurrent respiratory tract symptoms such as coughing, wheezing, stridor and recurrent respiratory infection, particularly in children with other congenital deformities.
Airway Obstruction ; Bronchi ; Bronchiectasis ; Bronchitis, Chronic ; Bronchoscopy ; Child ; Congenital Abnormalities ; Cough ; Diagnosis, Differential ; Drainage ; Equipment and Supplies ; Female ; Heart Septal Defects, Ventricular ; Humans ; Infant ; Pneumonia ; Respiration ; Respiratory Sounds ; Respiratory System ; Trachea ; Ventilation

This purpose of this study was to establish a new standard for the surgical management of female genital fistula in Korea. From January 1992 to October 2001, 117 patients with female genital fistula who were admitted to the departments of obstetrics and gynecology, urology and general surgery were analyzed. Nine patients with congenital etiologies and 48 patients who were treated conservatively were excluded. The relationships between surgical outcome and the cause of fistula, the location of fistula, and the various surgical methods were analyzed. In spite of appropriate surgical treatment, fistulas due to cervix cancer management had the worst prognosis. In terms of location, fistula recurrence after surgical repair was most common in the bladder fundus and base. The transvaginal and transrectal approaches are suitable for fistulas located in the lower vagina. The transabdominal approach is appropriate for fistulas located in the functional portions such as the bladder and ureter, for fistulas which are difficult to expose surgically by either the vaginal or rectal approach, or in cases with severe adhesions. In cases of cervix cancer, extra care should be taken during surgical expiration or definitive radiotherapy, especially when the areas involved are the bladder fundus and base. The nature of the surgical approach should be decided by the location of the fistula, the functional importance of the area, and the degree of surgical exposure during the corrective procedures.
Adolescent ; Adult ; Child ; Female ; Human ; Korea ; Middle Age ; Rectovaginal Fistula/surgery ; Treatment Outcome ; Urinary Fistula/surgery ; Vaginal Fistula/*surgery ; Vesicovaginal Fistula/surgery

OBJECTIVE: Prenatal cytogenetic diagnosis is limited to metaphase karyotype analysis of cultured cells obtained by amniocentesis or chorionic villus sampling. Moreover, genome wide analysis cannot be performed by FISH analysis using specific probe. Array comparative genomic hybridization (CGH) offers a number of advantages over conventional cytogenetic analysis and FISH. Microarray CGH can be highly comprehensive, amenable to very high resolution, sensitive and fast. The objective of this study was to determine the clinical use of cDNA microarray CGH for detection of fetal aneuploidy. METHODS: 21 amniotic fluid samples and 6 chorionic villi samples were obtained from 27 pregnant women in 9-19 gestational weeks. Genomic DNA was extracted from each sample and amplified. For cDNA microarray CGH analysis, test DNA sample and reference DNA sample were labeled with Cy3-dUTP and Cy5-dUTP, respectively. Each sample of labeled test and reference DNA was hybridized to microarray. The result was analysed with axon scanner and compared with cytogenetic analysis and FISH. RESULTS: In 27 cases, 3 cases with trisomy 21 and 1 case with trisomy 18 had increased hybridization signals on chromosome 21 and chromosome 18. One case with 45,X had decreased signals on chromosome X. One case with 46,X,i(Xq) had decreased signal on short arm of chromosome X and increased signal on long arm. And one case with 47,XYY had two fold increased signal on Y chromosome. cDNA microarray based CGH correctly identified fetal aneuploidy in all of the 7 cases with aneuploid fetuses. CONCLUSION: Prenatal genetic diagnosis by cDNA microarray-based CGH is an useful, innovative, rapid and accurate method. It is promising technique allowing rapid screening for whole chromosomal changes including aneuploidy, and may augment standard karyotyping techniques for prenatal genetic diagnosis by providing additional molecular information. This method may aid the discovery and description of minor genetic aberration, potentially enhancing future prenatal genetic diagnostic application.
Amniocentesis ; Amniotic Fluid ; Aneuploidy* ; Arm ; Axons ; Cells, Cultured ; Chorionic Villi ; Chorionic Villi Sampling ; Chromosomes, Human, Pair 18 ; Chromosomes, Human, Pair 21 ; Comparative Genomic Hybridization* ; Cytogenetic Analysis ; Cytogenetics ; Diagnosis ; DNA ; DNA, Complementary* ; Down Syndrome ; Female ; Fetus ; Genome ; Humans ; Karyotype ; Karyotyping ; Mass Screening ; Metaphase ; Oligonucleotide Array Sequence Analysis ; Pregnancy ; Pregnant Women ; Prenatal Diagnosis* ; Trisomy ; Y Chromosome