Neurofibromatosis is a disease which involves both neuroectodermal and mesodermal tissue, and is characterized by cafe-au-lait spot, multiple subcutaneous neurofibromas, elephantiasis neuromatosa, a positive family history, and specific dystrophic osseous changes such as scoliosis, penciling of ribs, vertebral scalloping, a paravertebral soft tissue tumor, and congenital pseudarthrosis. The classic type of scoliosis in neurofibromatosis was known as a sharp localized short curve that is often rapidly progressive and produces severe deformity with dystrophic changes, but another form with long gentle curve is reported. Since conservative treatment is usually unsuccessful, posterior fusion with or without Harrington instrumentation is the treatment of choice even in young age when the curve is progressive. This paper was aimed to review our experience with 11 patients having neurofibromatosis and scoliosis, who were treated with posterior fusion and Harrington instrumentation from Jan. 1971 to Dec. 1980, and the results were as follows: 1. The average age that spinal deformity was observed was 7.7 years old, but the average age at treatment was 14.4. 2. Cafe-au-lait spot was observed in all cases, subcutaneous nodule in 7 cases, local gigantism in 2 cases, and positive family history in 3 cases. 2 cases were combined with congenital spinal anormalies. 3. Specific pattern in spinal deformity was not significant. There were 7 short curves less than 5 vertebrae involved and 6 long curves more than 6 vertebrae involved. 4. There were 5 kyphosis which had more than 50°, and those were usually combined with severe scoliosis. 5. Preoperative average degree of scoliosis was 93.8, and the final correction was 41.2° (43.9%) with loss of correction 5.3°(5.7%) after 3.2 year follow-up in average. 6. It shouid be educated for early detection at home and school, and for the importance of early treatment for the scoliosis, to prevent rapid increase of scoliosis in neurofibromatosis.
Cafe-au-Lait Spots ; Congenital Abnormalities ; Elephantiasis ; Follow-Up Studies ; Gigantism ; Humans ; Kyphosis ; Mesoderm ; Neural Plate ; Neurofibroma ; Neurofibromatoses ; Neurofibromatosis 1 ; Pectinidae ; Pseudarthrosis ; Ribs ; Scoliosis ; Spine

No abstract available.
Decompression* ; Head* ; Osteonecrosis*

We performed total hip arthroplasty on a young male adult with paralytic dislocation of the hip due to poliomyelitis. Because of extreme shortening, as well as instability and weakness, the patient was unable to bear weight on the limb. Pastoperative course was complicated by ectopic ossification that compromised the hip and knee motion. Another young male adult with severe spastic cerebral palsy underwent total hip arthroplasty because of an intractable pain due to degenerative arthritis. Initial attempt coupled with adductor tenotomy and obtuator neurectomy ended in gross loosening and acetabular protrusion. Revision consisted of extensive soft tissue release and bone grafting of acetabular defect and use of a protrusion cup and an extra-long stem. Postoperative course was complicated by long-standing serous aseptic discharge from the wound which was controlled by antibiotics and prolonged recumbency. Indications for total hip arthroplasty in paralytic hips are rare and should be reserved for the most crippling conditions and one must be prepared fvr technical difficulties and a variety of complications with a prospecs for less than optimum results.
Acetabulum ; Adult ; Anti-Bacterial Agents ; Arthroplasty, Replacement, Hip ; Bone Transplantation ; Cerebral Palsy ; Dislocations ; Extremities ; Hip ; Humans ; Knee ; Male ; Ossification, Heterotopic ; Osteoarthritis ; Pain, Intractable ; Poliomyelitis ; Spectinomycin ; Tenotomy ; Wounds and Injuries

No abstract available.
Ciprofloxacin* ; Mycobacterium tuberculosis* ; Mycobacterium*

Systemic lupus erythematosus is a connective tissue disease which can affect every organ system. Neurologic abnormalities are common, occuring in approximately half of all patients at some time during the course of their illness. But symptoms of nervous system as the sole presenting symptoms occur in less than 1% of lupus patients. In patients initially presenting with neurologic symptoms and signs, differential diagnosis is difficult and sometimes it may be misdiagnosed. Therefore extensive laboratory investigations should be carried out in all patients with unusual neurological symptoms, since early diagnosis of lupus can help in providing effective treatment. We report a patient with systemic lupus erythematosus who presented with dysarthria and dysphagia resembling multiple sclerosis.
Connective Tissue Diseases ; Deglutition Disorders ; Diagnosis, Differential ; Dysarthria ; Early Diagnosis ; Humans ; Lupus Erythematosus, Systemic* ; Multiple Sclerosis* ; Nervous System ; Neurologic Manifestations

Psoriasis is characterized by disregulation of keratinocyte growth with profound epidermal hyperplasia. Keratinocyte hyperplasia in psoriasis may be expained in part by overproduction of growth factor, and by altered metabolism of the epidemal growth factor receptors (EGFR) in affected skin. The expression of epidermal growth f ictor receptor was investigated by Northern blot and slot-blot analysis of total RNA extrated from biopsies of normal skin and psoriatic lesions. In Northern blot analysis, EGFR-specific mRNA transcripts from psoriatic tissues demonstrated the specificity of hybridizarion with a EGFR mDNA probe. The size of EGFR mRNA transcript was 6.7kb in psoriasis lesions which showed no change of quality. In slot-blot analysis, the levels of EGFR mRNA in poriasis revealed a 1.2 fold to 4.1 fold elevation when compared to normal skin. EGFR were present in all epidermal layers by immunoperoxidase staining, whereas in normal skin they were primarily present in the stratum basalis. These results indicate that the increased expresion of the EGFR gene may be, in part, responsible for the hyperproliferation of the epider nis and that retained EGFR may reflect incomplet; abnormal differentiation in active porasis. This altered process of EGFR metabolism may be involved in the pathogenesis of psoriasis.
Biopsy ; Blotting, Northern ; Epidermal Growth Factor* ; Genes, erbB-1 ; Hyperplasia ; Keratinocytes ; Metabolism ; Psoriasis* ; Receptor, Epidermal Growth Factor* ; Receptors, Growth Factor ; RNA ; RNA, Messenger ; Sensitivity and Specificity ; Skin

No abstract available.
Colon* ; Diverticulitis*

Sinus histiocytosis with massive lymphadenopathy(SHML) is a benign, generally selflimited pseudolymphomatous disease that typically appears with cervical massive lymphaclenopathy. Extranodal involvement including skin occurs in the 28% of the cases. We report a case af SHML in 51 year-old male who had several, prominent firm masses ranging from 1-10cm in the cervical, axillary, inguinal areas and multiple, plum colored nodules and plaques in the face, trunk for about 10 years. The histopathological findings of cervical lymph node, facial nodule showed dense heavy infiltration of large histiocytes with abundant pale eosinophilic cytoplasm in the subcapsular and medullary sinuses of lymph node and dermis of skin. No atypical cells suggesting malignancy is seen in the infiltrates. The patient had been treated with combination of prednisolone and vinblasstine, but he expired 1 month later.
Cytoplasm ; Dermis ; Drug Therapy ; Eosinophils ; Histiocytes ; Histiocytosis, Sinus* ; Humans ; Lymph Nodes ; Male ; Middle Aged ; Prednisolone ; Prunus domestica ; Skin

No abstract available.
Anaphylaxis* ; Histamine Release* ; Histamine* ; Mast Cells*

PURPOSE: To evaluate the effects of cheap tinted contact lenses on corneal swelling and ocular surface inflammation, compared to hydrogel and silicone hydrogel contact lenses. METHODS: Forty eyes of 20 New Zealand white rabbits were randomly assigned to 4 groups. Two types of tinted contact lenses, hydrogel lenses, and silicone hydrogel lenses were each applied to 10 rabbit eyes. Corneal thickness and tear lactate dehydrogenase (LDH) activity were measured at 1 and 4 days after contact lens wear, and the inflammation of ocular surface was scored at 4 days after contact lens wear. The internal surface of the cheap tinted lens was examined with a scanning electron microscope to compare the surface quality between the tinted and non-tinted area. RESULTS: Although the corneal swelling of the silicone hydrogel lens group was significantly lower than the other 3 lens groups after contact lens wear (p<0.01), the common hydrogel lens group was not different from the 2 tinted contact lens groups (p>0.1). Tear LDH activity at 1 and 4 days after contact lens wear showed no significant difference among the 4 groups (p>0.29). The scores of ocular surface inflammation in the 2 tinted contact lens groups were greater than the hydrogel and silicone hydrogel lens groups (p=0.03). The scanning electron microscope revealed the internal surface of the tinted area in the tinted contact lens was coarse and irregular though the surface of the non-tinted area was relatively smooth. CONCLUSIONS: Regarding corneal swelling and tear LDH activity, the cheap tinted contact lenses used in Korea were not significantly different from the common hydrogel contact lenses. However, tinted contact lenses showed a greater tendency to provoke ocular surface inflammation than other lenses. The coarse and irregular surface of the tinted area in the tinted contact lens appears to play a role in provoking severe ocular surface inflammation.
Contact Lenses ; Electrons ; Eye ; Hydrogel ; Inflammation ; Korea ; L-Lactate Dehydrogenase ; Rabbits ; Silicones