A case of osteochondrodystrophy is reported, involving many joints of upper and lower extremities. These affections exhibit valgus-flexion deformity of both elbows, extruding distal ulnar end at the wrist and brachiophalangism of both hands, manly thumb and index finger. Varus deformity of left ankle and brachio-phalangism of both feet were also demonstrable. Thorough radiological and histological examination were carried out. Initial treatment was the resection of distal end of ulna, promoting the correction of the deformity and better wrist motion. Some reviews with literatures are supplemented.
Ankle ; Congenital Abnormalities ; Elbow ; Fingers ; Foot ; Hand ; Joints ; Lower Extremity ; Thumb ; Ulna ; Wrist

This study was conducted to set up a common mechanism for varying phases of focal segmental glomerulosclerosis(FSGS) by comparing the morphological differences between human FSGS and changes in 5/6 renal ablation animal model, which has been accepted as experimental prototype for hyperfiltration theory as pathogenesis of FSGS. Both the human and the experimental rats showed very similar changes such as segmental glomerulosclerosis, vacuole formations or inclusion of small granules of podocytes, appearance of foamy cells in the capillary lumina, eosinophilic deposits along the mesangial area, and focal atrophy of tubules with associated interstitial fibrosis. The halo, frequently seen in human FSGS, is due to detachment of visceral epithelium from basement membrane, however, did not appear in the experimental rat specimen. On the other hand, the foamy cells and hyalinization were more frequently noted in the rat series and even involved the arterioles. The mesangial proliferation never appeared in the rat series occasionally found in human FSGS. In conclusion, the pathogenesis of FSGS cannot depend solely on the hyperfiltration theory of hemodynamic derangement, but has complex impairment of visceral epithelium and cells forming the constituents of basement membrane.
Animals ; Arterioles ; Atrophy ; Basement Membrane ; Biopsy ; Capillaries ; Eosinophils ; Epithelium ; Fibrosis ; Hand ; Hemodynamics ; Humans* ; Hyalin ; Models, Animal* ; Nephrectomy* ; Podocytes ; Rats* ; Vacuoles

This study was conducted to set up a common mechanism for varying phases of focal segmental glomerulosclerosis(FSGS) by comparing the morphological differences between human FSGS and changes in 5/6 renal ablation animal model, which has been accepted as experimental prototype for hyperfiltration theory as pathogenesis of FSGS. Both the human and the experimental rats showed very similar changes such as segmental glomerulosclerosis, vacuole formations or inclusion of small granules of podocytes, appearance of foamy cells in the capillary lumina, eosinophilic deposits along the mesangial area, and focal atrophy of tubules with associated interstitial fibrosis. The halo, frequently seen in human FSGS, is due to detachment of visceral epithelium from basement membrane, however, did not appear in the experimental rat specimen. On the other hand, the foamy cells and hyalinization were more frequently noted in the rat series and even involved the arterioles. The mesangial proliferation never appeared in the rat series occasionally found in human FSGS. In conclusion, the pathogenesis of FSGS cannot depend solely on the hyperfiltration theory of hemodynamic derangement, but has complex impairment of visceral epithelium and cells forming the constituents of basement membrane.
Animals ; Arterioles ; Atrophy ; Basement Membrane ; Biopsy ; Capillaries ; Eosinophils ; Epithelium ; Fibrosis ; Hand ; Hemodynamics ; Humans* ; Hyalin ; Models, Animal* ; Nephrectomy* ; Podocytes ; Rats* ; Vacuoles

Crohn's disease is a chronic, nonspecific inflammatory process which most commonly involves the terminal ileum, but which can affect any region of the gastrointestinal tract from the mouth to the anus. When Crohn's disease does involve the upper gastrointestinal tract, there is nearly always concomitant disease in the small bowel or colon. However isolated gastric Crohn's disease is a rare entity, and gastroduodenal involvement in Crohn's disease has been considered unusual, having an incidence of 1.5 to 7.0%. Gastric Crohn's disease usually localizes to antrum of the stomach and or the pyloroduodenal junction. No single feature is pathognomonic of Crohn' disease, and the diagnosis is based on supportive clinical, radiographic, endoscopic, and pathologic findings. Crohn's disease is invariably a diagnosis of exclusion, so infectious, neoplastic, ischemic, infiltrative, and other inflammatory processes must be ruled out. Usually a patient with isolated Crohn's disease of the stomach will have the clinical symptoms of nausea, vomiting, and epigastric pain, and there will be radiographic evidence of a small contracted stomach (or occasionally, a huge dilated stomach). The treatment of stomach Crohn's disease is palliative and symptomatic. In this paper, we describe a patient with Crohn's disease who had diffuse involvement of the entire stomach without any evidence of involvement elsewhere in the gastrointestinal tract.
Anal Canal ; Colon ; Crohn Disease* ; Diagnosis ; Gastrointestinal Tract ; Humans ; Ileum ; Incidence ; Mouth ; Nausea ; Stomach* ; Upper Gastrointestinal Tract ; Vomiting

BACKGROUND: The increase of the incidence of autoimmune diseases and the autoimmune pathogenesis of vitiligo were reported. OBJECTIVE: We studied the frequency of autoimmune disorders and positivity of antinuclear antibody in Korean vitiligo patients. METHODS: Vitiligo patients (439 patients) and control subjects (197 patients) were interviewed about their history of autoimmune diseases. Laboratory studies including complete blood cell count, urine analysis, blood chemistry, fasting blood sugar, thyroid function test (T3, free T4, TSH), and antinuclear antibody were performed for the screening of autoimmune disorders. RESULTS: The diseases associated with vitiligo were microcytic hypochromic anemia (3.64%), non-insulin dependent diabetes mellitus (2.96%), thyroid disease (3.96%), atrophic gastritis, and alopecia areata. In the control subjects, the associated diseases were microcytic hypochromic anemia (1.62%), non-insulin dependent diabetes mellitus (4.65%), and thyroid disease (3.49%). These results show that the frequency of autoimmune disorders in vitiligo patients is not significantly higher than that in control subjects. Six (54.5%) out of 11 vitiligo patients with thyroid disease were diagnosed as having thyroid disease for the first time. Four (0.91%) out of 438 vitiligo patients showed positive to antinuclear antibody. Positivity of antinuclear antibody was not higher in vitiligo patients than that in control subjects (1.16%). CONCLUSION: Frequency of autoimmune diseases and positive reaction to antinuclear antibody in vitiligo patients were not significantly higher than those in control subjects.
Alopecia Areata ; Anemia, Hypochromic ; Antibodies, Antinuclear ; Autoimmune Diseases ; Blood Cell Count ; Blood Glucose ; Chemistry ; Diabetes Mellitus ; Fasting ; Gastritis, Atrophic ; Humans ; Incidence ; Mass Screening ; Thyroid Diseases ; Thyroid Function Tests ; Vitiligo*

No abstract available.
Exanthema* ; Skin*

The extent of excision and necessity of hepatectomy in type IVa choledochal cyst has not been clarified. We performed left lobectomy of the liver for complete excision of the cyst in a thirty-year-old male patient with type IVa choledochal cyst. Postoperative recovery was untroubled and outcome was satisfactory. Complete excision of the cyst including hepatectomy is an acceptable operative management for patients with type IVa choledochal cyst. This procedure can eliminate the possibility of subsequent cancer arising from remaining cyst.
Choledochal Cyst* ; Hepatectomy ; Humans ; Liver ; Male

No abstract available.
Korea* ; Maternal Death*

BACKGROUND: The main therapeutic method currently used for the treatment of vitiligo vulgaris is either photochemotherapy or corticosteroids. But both of the procedures however require lengthy treatment to obtain a satisfactory result. OBJECTIVE: To determine the increased efficacy of the combination therapy of photochemotherapy with corticosteroid and chlorpromazine compared with the usual monotherapy. METHODS: The combination therapy was done in 29 cases and the results were compared with the results of either monotherapy appearing in the literatures. RESULTS: f the 29 patients, 14(48.3%) patients achieved 100% repigmentation, 7(24.1%) patients 75~50%, 5(17.2%) patients 25~50%, and 3(10.4%) patients less than 25%. For the cured patients, 190.3 days and 32.7 times of photochemotherapy were used. CONCLUSION: The combination therapy is more effective, and the duration of treatment can be greatly reduced compared with either monotherapy.
Adrenal Cortex Hormones ; Chlorpromazine ; Humans ; Methods ; Photochemotherapy ; Vitiligo*

Our experience with recurrent dislocation of the patella on ten knees in eight patients seen at Seoul National University Hospital during the last 2 years and eight months is reported. Five were female and three male. The age ranged from seven to 27 years. In four of the patients the onset was attributable to a definite history of trauma, while in one patient the dislocation was considered due to developmental anomalies and in another it was clearly congenital Still another patient had a progressively short limb due to premature closure of the distal femoral epiphysis with accompanying flexion-valgus deformity of the knee, presumably caused by an unrecognized infection during early childhood One adolescent girl had bilateral knock knees. The knock knees were treated successfully by supracondylar osteotomy and one post-traumatic knee by classical Roux-Goldthwait procedure with equally excellent result, The rest were treated by Greens vastus medialis transfer with division of the iliotibial band or reefing of the medial joint capsule when necessary. In one knee dislocation recurred, probably due to avulsion of the transferred vastus, and two knees had residual subluxation and limitation of flexion. The results on the remaining seven knees were excellent.
Adolescent ; Congenital Abnormalities ; Dislocations ; Epiphyses ; Extremities ; Female ; Genu Valgum ; Humans ; Joint Capsule ; Knee Dislocation ; Knee ; Male ; Osteotomy ; Patella ; Quadriceps Muscle ; Seoul