We have experienced a case of plexiform neurofibrorna of the right upper eyelid and orbit in a 12-year-old girl who had typical skin features of neurofibromatosis and no family history. The non-pulsating proptosis of the right eye and diffuse thickening with hypertrophy of the upper lid, had increased insiduciusly since the birth on. Biopsy taken from eyelirl lesion showed the features of plexiform neurofibroma. Skull X-ray and brain computerized tomogram showed that the right orbit was wider, with the enlarged mass and defects in orbital roof and lesser and greater wings of the sphenoid bone. The surgical excision of the right eyelid lesion was performed.
Biopsy ; Brain ; Child ; Exophthalmos ; Eyelids* ; Female ; Humans ; Hypertrophy ; Neurofibroma, Plexiform* ; Neurofibromatoses* ; Orbit* ; Parturition ; Skin ; Skull ; Sphenoid Bone

Central nervous system impairment is common in diabetic patients, even in the early stages of the disease, and could be associated with peripheral neuropathy. The aims of this study were to prospectively investigate central nerve conduction in young adults with type 1 diabetes using pattern-reversal visual evoked potentials (PRVEP) and to determine how those results were related to clinical risk factors and the parameters of the peripheral nerve conduction study (NCS). A total of 36 type 1 diabetic patients (15 males) 5-24 years of age (mean 14.5 ± 4.7) underwent PRVEP and NCS annually for five years. For comparison, 39 healthy age and sex matched individuals (mean 14.8 ± 5.0) were evaluated as the control group. The P100 latencies of the PRVEP were prolonged at the study entry in the patients compared with the controls (p< 0.001). Significant correlations were not found between any of the parameters of PRVEP and the glycosylated hemoglobin levels; however, the changes in the parameters of the peripheral NCS were well correlated with metabolic control. The latencies and amplitudes of the P100 were not related to the majority of the parameters of the NCS. A prolonged PRVEP latency may be a sign of optic pathway dysfunction, which begins before apparent diabetic retinopathy. Poor glycemic control proved to be an important risk factor over the 5 years in terms of its relation to the development of peripheral neural pathway abnormalities. However, once central conduction was delayed, its changes were poorly related to diabetic control and the attributes of the peripheral nerve conduction study over the 5-year follow-up.
Diabetes Mellitus

Pathologic fracture occurs in a bone with preexisting stuctual weakness. The author reviewed 30 cases of pathologic fractures in which admission record and X-ray finding was well preserved among 53 cases admitted patients in the department of Orthopaedic Surgery, Seoul National Universty Hospital for 10 years from 1967. The results were as follows: 1.Age & Sex incidence showed even distribution. 2. The most frequent involved site was femur (40%) 3. The underlying etiology was as follows: 2 chronic osteomyelitis 1 homophilia 7 benign tumor 20 malignant tumor 4. metastatic tumor was the most frequent cause in 30 cases. 5. Treatment was as follows: Conservative treatment 4 cases Surgery 21 cases.
Clinical Study ; Femur ; Fractures, Spontaneous ; Humans ; Incidence ; Osteomyelitis ; Seoul

29 isoniazid (INH) resistant isolated strains and INH sensitive reference strain (H37Rv) of Mycobacterium tuberculosis were analysed by polymerase chain reaction-single strand conformational polymorphism (PCR-SSCP) and NciI restriction mapping for the detection of mutations in katG gene and inhA gene. The katG gene was divided into 3 parts (Akat, Bkat, Ckat; each part is about 800 bp) and amplified, inhA gene was amplified as a whole. Each of the amplified 800 bp DNA was digested into small fragments of less than 400 bp with restriction enzymes for the direct PCR-SSCP analysis. Firstly, 10 strains were analysed. All the 10 isolates showed clearly distinct SSCP patterns in Bkat from that of the reference strain, but only two isolates showed distinct SSCP patterns in Akat, and no isolated strain showed any distinct SSCP patterns in Ckat. 10 isolates also showed distinct SSCP patterns in inhA. NciI restriction mapping of Bkat showed mutation in codon 463 in 7 strains among 10 isolated strains. With these results an early detection strategy for the INH resistant M. tuberculosis was applied to the rest of 19 isolated INH resistant strains. Firstly, isolates were screened by Ncsl mapping in Bkat, and 13 strains showed mutations in codon 463. Secondly, the rest of 6 INH resistant isolates were analysed by PCR-SSCP with restriction enzyme digestion (PCR-SSCP-RE) in Bkat, and all the strains showed distinct SSCP patterns from that of the INH sensitive reference strain. This proved our strategy as effective and economic and time saving method in early detection of INH resistant M. tuberculosis.
Codon ; Diagnosis* ; Digestion ; DNA ; Isoniazid* ; Korea* ; Mycobacterium tuberculosis* ; Mycobacterium* ; Polymorphism, Single-Stranded Conformational ; Restriction Mapping ; Tuberculosis

No abstract available.
Blood Group Antigens*

Melorheostosis is a rare disease entity of bone with an etiology and pathogenesis. It cause pain and stiffness in an affected limb, and dense bone formation along the side of bone resembles the flow of candle dripping. Two cases of the disease involving left hand and wrist, and right lower leg are reported with the review of literature.
Extremities ; Hand ; Hyperostosis ; Leg ; Melorheostosis ; Osteogenesis ; Rare Diseases ; Wrist

Choroidal metastatic carcinoma is very rare. We recently experienced a case of lung adenocarcinoma which presented to the clinic with ocular symptoms. This 57-year-old Korean male patient visited the department of ophthalmology due to decreased visual acuity and pain of the left eye. On MRI scan, a nodule was attached to the retina of the left eyeball. On simple chest radiograph, a large amount of pleural effusion was noted in the left pleural cavity. Emergency enucleation of the left eyeball was done with an impression of malignant melanoma causing an intractable ocular pain. Grossly, the lesion in the eyeball was rising from the choroid. On histologic examination, tumor cells formed many irregular, small gland-like structures. The tumor cells showed alcian blue-positive mucin in the cytoplasm and glandular lumens and were positive for CEA. Chest CT scan was performed postoperatively and showed a huge mass in the left lower lobe and multiple nodular opacities in both lung fields. Bronchoscopic biopsy revealed moderately differentiated adenocarcinoma similar to that of the eyeball.
Adenocarcinoma* ; Biopsy ; Choroid* ; Cytoplasm ; Emergencies ; Humans ; Lung Neoplasms ; Lung* ; Magnetic Resonance Imaging ; Male ; Melanoma ; Middle Aged ; Mucins ; Neoplasm Metastasis* ; Ophthalmology ; Pleural Cavity ; Pleural Effusion ; Radiography, Thoracic ; Retina ; Tomography, X-Ray Computed ; Visual Acuity

Primary malignant parovarian epithelial tumors are extremely rare, with only 56 cases previously reported in the world literature. Most parovarian epithelial tumors are of paramesonephric (Mullerian) origin. In this article, we report the first case in the Korean literature of papillary serous cystadenoma of borderline malignancy from paramesonephric parovarian cyst. This case presented here is of particular interest since this neoplasm is rare. A 48-year-old female underwent a hysterectomy with bilateral adnexectomy and pelvic lymph node dissection for a cystic tumor of the right parovarian area. The tumor, measuring 13 cm in diameter, was apart from the ovaries, fallopian tubes and uterus. The cyst wall had well-developed smooth muscle layers. The cyst was lined with mildly atypical ciliated and non-ciliated low columnar cells compatible with those of paramesonephric origin. From the inner surface of the cyst several cauliflower-like structures projected into the lumen. They were of a predominantly papillary architecture covered by atypical epithelial cells with piling-up and occasional glandular growth. No destructive stromal invasion was identified.
Cystadenoma, Serous* ; Epithelial Cells ; Fallopian Tubes ; Female ; Humans ; Hysterectomy ; Lymph Node Excision ; Middle Aged ; Muscle, Smooth ; Ovary ; Parovarian Cyst* ; Uterus

Platelet-derived growth factor(PDGF) is known to accelerate soft tissue fracture and periodontal bone healing, but little information is available for characterizing the healing of articualr cartilage. This study was designed to demonstrate the regeneration potential of pasteurized autogeneous osteochondral graft when PDFG was applied locally in vivo. Eighteen rabbits in two groups were used in the experiment. The free osteochondral fragment(1x1x1 cm) were taken from distal femur. The fragment were pasteurized in 65degrees C for 5 minutes. In experimental group(group II), the fragment were soaked in 0.25 mg/ml of recombinant human PDGF mixed 5 ml normal saline and reimplanted to the resected site, and followed with local administration of 0.25 mg/ml PDGF to knee joint. In control group(group I), the pasteurized fragment were reimplanted in the resected site without any treatments. The histologic characteristics of the transplanted autografts for three rabbits in each group were observed at 3, 5 and 7 weeks postoperatively. The results were as follows; Group II revealed the more favorable regeneration of articular cartilage, less arthritic changes and more mature arrangement of chondrocytes than group I. Even thought the cartilage is avascular and the regeneration capacity of pasteurized cartilage is severely limited, the regeneration of grafted articular cartilage is thought to be accellerated by increased newly formed blood vessels and soft tissues due to the diffusion of PDGF on grafted cartilage, and probably also by differentiation of mesenchymal cells in the initial stage of experiment. The results of this study suggest that local administration of PDGF could be used for the treatment of posttraumatic or degenerative arthritis and various cartilage damages.
Autografts ; Blood Vessels ; Cartilage ; Cartilage, Articular ; Chondrocytes ; Diffusion ; Femur ; Humans ; Knee Joint ; Osteoarthritis ; Platelet-Derived Growth Factor* ; Rabbits* ; Regeneration* ; Transplants*

PURPOSE:Autonomic neuropathy in adult diabetics is known to be common and poor in prognosis. Cardiac autonomic neuropathy increases sudden cardiac death and is present before apparant cardiac symptoms. Subclinical diabetic peripheral neuropathy is common with type 1 diabetes. In children, few studies for subclinical peripheral neuropathy and cardiac autonomic neuropathy were reported but the results were inconsistent. The authors investigated subclinical cardiac and peripheral neuropaties and evaluated correlation between nerve conduction velocity(NCV) and cardiac autonomic function(CAF) test in type 1 diabetic children and adolescents METHODS:Over 5 years from 1997 to 2002, 27 IDDM without clinical neuropathy were registered. Total 57 CAF and NCV were tested every 2 or 3 years. Duration of diabetes, average HbA1c during 1 year prior to the test, and urinary microalbumin excretion for 24 hours were assessed. Diabetic autonomic nervous function was evaluated by cardiovascular reflex test:falling systolic blood pressure in response to standing, heart rate changes in response to standing, beat-to-beat rate variation during deep breathing, RR interval change during and after Valsalva maneuver. Subclinical peripheral neuropathy was evaluated by NCV in both upper and lower extremities. Correlation of parameters according to NCV abnormality and microalbuminuria were analyzed. RESULTS:The age, duration of diabetes and microalbuminuria were not statistically different between children with normal and abnormal NCV. The level of HbA1c was associated with NCV abnormality. In a multivariate logistic model for NCV abnormality, level of HbA1c showed statistical significance after controlling the effects of age, duration of diabetes, and urinary microalbumin level, and the odd ratio was 1.532. The degree of CAF abnormality was marginally significant in the logistic model. Children with microalbuminuria showed older age and longer duration of diabetes than those without microalbuminuria. However, mean HbA1c level was not significantly different between the two groups. Degree of CAF abnormality was not significantly associated with microalbuminuria, either. In a general linear model including urinary albumin level as the dependent variable, none of independent variables was statistically significant. CONCLUSION: Therapy against neuropathy should be considered in patients with high HbA1c and abnormal CAF test even without clinically apparent neuropathy.
Adolescent ; Adult ; Blood Pressure ; Child* ; Death, Sudden, Cardiac ; Diabetes Mellitus, Type 1 ; Diabetic Neuropathies ; Heart Rate ; Humans ; Linear Models ; Logistic Models ; Lower Extremity ; Neural Conduction ; Peripheral Nervous System Diseases ; Prognosis ; Reflex ; Respiration ; Valsalva Maneuver