No abstract available.
Neurofibromatoses*

A case of porphyria cutanca tarda is reported, in which the photosensitive cutaneous symptoms developed in association with impaired hepatic function possibly related to chronic consumption of alcohol. Wood's light examination of the urine revealed coral red fluorescence, and qualitative chemical urinalysis was strong positive for uroporphyrin and coproporphyrin. Biopsy of the skin showed subepidermal bulla with festooning arrangement of the naked papillae and basophilic degeneration of the dermis. The patient's favorable response to alkalinization therapy by sodium bicarbonate per os is encouraging, even for a short period's observation. Clinical, histopathological, and biochemical features of this disease are also discussed.
Anthozoa ; Basophils ; Biopsy ; Dermis ; Fluorescence ; Porphyrias ; Skin ; Sodium Bicarbonate ; Urinalysis

No abstract available.
Humans

Two cases of tuberous sclerosis are reported. Case 1: A 13 years-old girl had a triad of adenoma sebaceum, mental deficiency and epileptic seizures associated with ichthyosis vulgaris, but family history revealed no evidences of the disease except ichthyosis in her mother. Case 2 : A 21 year-old woman had a adenoma sebaceum, shagreen patch, periungual fibroma and fihromatous plaque, showing intracranial calcificatins on roentgenographic examination, but has not demonstrated any neurological manifestations as yet. Family history also revealed adenoma sebaceum in her father and two younger sisters. We feel this case is a "forme fruste" of tuberous sclerosis.
Adolescent ; Epilepsy ; Fathers ; Female ; Fibroma ; Humans ; Ichthyosis ; Ichthyosis Vulgaris ; Intellectual Disability ; Korea* ; Mothers ; Neurologic Manifestations ; Siblings ; Tuberous Sclerosis* ; Young Adult

A case of dermatitis herpetiformis assaciated with erythema annulare centrifugum is reported. The patient was a 21 year-old male, who had papulovesieles, erythematous lesions, pigmentation, and erythema annulare centrifugum lesions on the trunk, shoulders, buttocks and extensor surfaces of the extremities, and severe itching sensation of 9 months' duration. The skin biopsy from the vesicular and erythema annulare centrifugum lesion showed subepidermal bulla and vesicles with neutrophil and eosinophil infiltrations.
Biopsy ; Buttocks ; Dermatitis Herpetiformis* ; Dermatitis* ; Eosinophils ; Erythema* ; Extremities ; Humans ; Male ; Neutrophils ; Pigmentation ; Pruritus ; Sensation ; Shoulder ; Skin ; Young Adult

No abstract available.

Cubitus varus and cubitus valgus is one of late complications of elbow fracture. Tardy ulnar nerve palsy often accompanies with cubitus valgus. The authors reviewed 40 supracondylar osteotomies (on cubitus varus 31, cubitus valgus 9) performed at the Department of Orthopedic Surgery, Seoul National University Hospital, during the period of 15 years, from 1962 till 1976. During long term follow up, correction of elbow deformity and its maintenance is possible even in growing child. So early operation is recommended for the prevention of neurologic compIication and elbow stiffness.
Child ; Congenital Abnormalities ; Elbow ; Follow-Up Studies ; Humans ; Orthopedics ; Osteotomy ; Seoul ; Ulnar Neuropathies

Forty-five patients with primary neoplasm of the spine, disgnosed and operated at Dept. of Orthopedic Surgery, Seoul National University Hospital, from 1970 to 1988, were reviewed in an attempt to analyze the incidence of the primary spine tumors and effectiveness of surgical treatment. Mean follow-up was 2.5 years. Among 29 benign tumors, 7 cases of osteoid osteoma, 6 cases of giant cell tumor, 4 cases of osteoblastoma and aneurysmal bone cyst, and 2 cases of fibrous dysplasia and osteochondroma, and others were identified. Of 16 malignant tumors, 6 cases of eosinophilic granuloms, 4 cases of chordoma, 3 cases of solitary plasmacytoms, and others were noted. 17 cases occurred in the anterior compartment, 18 cases in the posterior compartment, and 10 cases involved both compartments. Surgical treatment consisted of complete or near complete excision, decompression, and additional stabilization procedure when the stability of the vertebral column was compromised. Stablization was achieved either by fusion, strut bone graft, or by additional Zielke, Cotrel-Dubousset, or Luque-Harringtion instrumentation. The results were astisfactory in most of the cases with benign tumors except cases of giant cell tumor. The results in malignant tumor were satisfactory especially in eosinophilic granuloma and solitary plasmacytoma with respects to symptoms and survival, and this fact was attaibuted to good response to adjuvant chemotherapy and radiation therapy. Recent application of Cotrol-Dubousset or Zielke instrumentation after agressive resection made early ambulation a clinical reality.
Aneurysm ; Bone Cysts ; Chemotherapy, Adjuvant ; Chordoma ; Decompression ; Early Ambulation ; Eosinophilic Granuloma ; Eosinophils ; Follow-Up Studies ; Giant Cell Tumors ; Humans ; Incidence ; Orthopedics ; Osteoblastoma ; Osteochondroma ; Osteoma, Osteoid ; Plasmacytoma ; Seoul ; Spine ; Transplants

No abstract available.
Ascites* ; Intussusception*

Adenoid cystic carcinoma is a rare disease of which incidence is 10-15 % of bartholins gland carcinoma. The clinical presentation is characterized by a vulval mass that existed for a prolonged period to the onset of symptoms, usually infection, pain, and burningsensation Histologically, this tumor is characterized by cribriform pattern cell growing and perineural invasion. The obtaining of clear surgical margins is the most important aspect of treatment. Here we report a case of adenoid cystic carcinoma of the Bartholins gland with a brief review of literature.
Adenoids* ; Carcinoma, Adenoid Cystic* ; Incidence ; Rare Diseases