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

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

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*

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

Experinece in the management of 74 patients with delayed traumatic intracranial hemorrhage(DTICH) of 474 head injury from January 1996 to December 1996 is poresented with emphasis on the incidence, occurring time, risk factors and outcome. The incidence of DTICH was 15.6% of all hospitalized head-injury patients. After an injury, every patient had an immediate computerized tomography(CT) scan to diagnose intracranial pathology and then CT follow-up was carried out according to intial CT finding and reurological deficit. The lesion was almost occurred in patients with initial abnormal CT finding(85.1%). 82.4% of DTICH were noted within 72 hours after injury. The delayed epidural hematoma and intracerebral hemorrhage were almost noted in first 72 hours(>90%), but the delayed subdural hemorrhage was found after a time interval varying from 6 hours to 10 days. So we strongly recommend CT follow-up in 4-8hour, 24-72hour, and then 7th day after head injury, especially in patients with initial abnormal CT findings. The risk factor of the delayed lesion was not hypotension, hypoxia, and consciousness level, but age of patients and the initial CT finding. The development of DTICH was not heralded by neurological deterioration. The prognosis of DTICH was not worse than non-DTICH. The patient with delayed subdural hemorrhage was better than the patient with non-delayed lesion(including hemorrhage and normal CT finding).
Anoxia ; Cerebral Hemorrhage ; Consciousness ; Craniocerebral Trauma* ; Follow-Up Studies ; Head* ; Hematoma ; Hematoma, Subdural ; Hemorrhage ; Humans ; Hypotension ; Incidence ; Intracranial Hemorrhages* ; Pathology ; Prognosis ; Risk Factors