Creutzfeldt-Jakob disease(CJD) is characterized clinically by rapidly progressive dementia with pyramidal, extrapyramidal, and cerebellar symptoms and signs, and histologically by spongiform change, neuronal loss and reactive gliosis. We have experienced 2 cases of CJD. Case 1 was a 36-year-old male who had suffered from myoclonus and cerebellar symptoms including sluggish speech, gait and balance disturbance. Case 2 was a 70-year-old female who had showed cognitive dysfunction, ataxic gait and disturbance of extraocular movement. Both patients, underwent brain biopsy. Case 1 revealed marked cortical atrophy, 2mm in thickness, with neuronal loss and astrocytic proliferation extending into white matter. The spongiform change, made up of many small, usually rounded or oval, vacuoles was noted mainly in the neuropil. Case 2 revealed remarkable spongiform change throughout the cortex and cytoplasmic vacuoles compressing the nuclei of neuronal cells were numerous. Neuronal loss and gliosis were also found without considerable change in the white matter. On double immunostaining against GFAP and PrP(Prion Protein), there was a weak positive reaction for PrP in the perinuclear cytoplasm in case 1, and a strongly positive reaction in case 2. The electron microscopic examination showed numerous membrane-bound vacuoles in neuropil and perikarya of neurons. The majority of the vacuoles were multiseptated by thin membranous structures. They demonstrated curled, or disrupted membrane, that had foldings and protrusions into the vacuolar clear spaces. There were neither identifiable virus-like particles nor amyloid deposition.
Female ; Male ; Humans

The Kidd blood group is clinically significant since the Jk antibodies can cause acute and delayed transfusion reactions as well as hemolytic disease of newborn (HDN). In general, HDN due to anti-Jk(b) incompatibility is rare and it usually displays mild clinical symptoms with a favorable prognosis. Yet, we apparently experienced the second case of HDN due to anti-Jk(b) with severe clinical symptoms and a fatal outcome. A female patient having the AB, Rh(D)-positive boodtype was admitted for jaundice on the fourth day after birth. At the time of admission, the patient was lethargic and exhibited high pitched crying. The laboratory data indicated a hemoglobin value of 11.4 mg/dL, a reticulocyte count of 14.9% and a total bilirubin of 46.1 mg/dL, a direct bilirubin of 1.1 mg/dL and a strong positive result (+++) on the direct Coomb's test. As a result of the identification of irregular antibody from the maternal serum, anti-Jk(b) was detected, which was also found in the eluate made from infant's blood. Despite the aggressive treatment with exchange transfusion and intensive phototherapy, the patient died of intractable seizure and acute renal failure on the fourth day of admission. Therefore, pediatricians should be aware of the clinical courses of hemolytic jaundice due to anti-Jk(b), and they should be ready to treat this disease with active therapeutic interventions.
Bilirubin/blood ; Erythroblastosis, Fetal/*blood ; Fatal Outcome ; Female ; Humans ; Infant, Newborn ; Isoantibodies/blood ; Kidd Blood-Group System/*immunology

The use of osseointegrated implants is an accepted procedure for the treatment of Total, or partial edentulism and offers good predictability of long-term success. Osseointegration implies a firm and direct interlocking between vital bone and screw-shaped titanium implants. There should be not to interposed tissue between fixture and bone. This study was undertaken to assess the clinical condition, complication, and prosthodontic aftercare of different implant systems. One hundred fifty-nine patients treated with a total of 503 endosseous implants (364 Steri-oss threaded type, 69 Integral cylinder with HA coated type, 35 Steri-oss threaded with HA coated type, 21 Steri-oss cylinder with HA coated type and 14 3i implant type), Most of the implant were placed in type B and C bone quantity and type 2 and 3 bone quality according to Lekhorm and Zarb. The success rate of Steri-oss threaded type during healing and function was 92%, Steri-oss threaded type with hydroxyapatite coated was 91%, Steri-oss cylinder type with hydroxyapatite coated was 90%, Integral cylinder type with hydroxyapatite coated was 90% and 3i implant type was 93%. One hundred twenty-nine patients had been treated with implant prosthesis. 79 of these patients had received a fixed type prosthesis and 50 patients had received a removable type prosthesis. There were no differences between the implant systems with regard to age, gender. Failures were associated with poor bone quality, smaller implant sizes, a surgical installation technique and stress distribution when in function. Visual analgoue scales recorded as satisfied results functionally and esthetically, but 15% dissatified with chewing ability.
Aftercare ; Dental Implants* ; Durapatite ; Humans ; Mastication ; Mouth Rehabilitation* ; Mouth* ; Osseointegration ; Prostheses and Implants ; Prosthodontics ; Titanium ; Weights and Measures

Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait spots and neurofibrobmas, or only neurofibroma, limited to one region of the body. Disease-associated systemic involvement is uncommon. Most patients with segmental neurofibromatosis do not have a family history of neurofibromatosis. In Korea, there have been 3 reported cases of segmental neurofibromatosis. None of them had caf6-au-fait spots, systemic involvement, or family history. This report describes a case of segmental neurofibromatosis in a 25-year-old woman who had Becker's nevus-like cafe-au-lait spots and this was linked to the presence of Fanconi's syndrome in a second degree relative of the patient.
Adult ; Cafe-au-Lait Spots ; Female ; Humans ; Korea ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1

BACKGROUND: Psoriasis is a chronic skin disease characterized histologically by prominent keratinocyte hyperplasia and an early inflammatory cell infiltrate. However, the pathogenesis is not fully understood yet. Recently, there has been growing interest in the probable role of a T cell mediated immune response in the pathogenesis. Especially, cytokine network involved in psoriasis has been studied. OBJECTIVE: This study was done to investigate the role of Interleukin-8(IL-8) and its inducer, Tumor necrosis factor-alpha(TNF-alpha) for pathogenesis of psoriasis. METHODS: We performed immunohistochemical staining using antibodies for IL-8 and TNF-alpha in frozen skin samples of 14 psoriatic patients who had not been treated for psoriatic lesion for 1 month and 3 normal skin samples as controls. RESULTS: There was no detectable discrete immunoreactivity for either TNF-alpha and IL-8 in epidermis and dermis of normal controls. In psoriatic lesions, the expression of TNF-alpha was found in dermal cells within the papillary dermis, particularly around blood vessels. The expression of IL-8 was presented on suprabasal keratinocytes above TNF-alpha-positive dermal tissue. CONCLUSION: Our results strongly suggest that histologic expression and functional interaction between IL-8 and its inducer, TNF-alpha have significant roles in the pathogenesis of psoriasis.
Antibodies ; Blood Vessels ; Dermis ; Epidermis ; Humans ; Hyperplasia ; Interleukin-8* ; Keratinocytes ; Necrosis ; Psoriasis ; Skin ; Skin Diseases ; Tumor Necrosis Factor-alpha*

Common peroneal neuropathy during normal delivery is associated with instrumentation and prolonged time spent in a certain position. We report a case of unilateral common peroneal neuropathy in a vaginal delivery patient under labor analgesia with the combined spinal-epidural technique. A 29-year-old woman complained of weakness and numbness of the left foot two days after vaginal delivery. Neurologic examination and electrodiagnostic testing confirmed left common peroneal neuropathy. The patient was referred for rehabilitation, which substantially improved her mobility and function, and prevented secondary damage. The patient had not fully recovered at three months after the delivery. The possible etiology of the postpartum common peroneal neuropathy was most likely prolonged and excessive pressure around the knee by assistants. Excessive compression during delivery should be minimized or avoided. Patient education and awareness among the labor and delivery team will prevent this complication. Neurologic complications related to epidural analgesia should also be ruled out in these patients.
Adult ; Analgesia* ; Analgesia, Epidural ; Female ; Foot ; Humans ; Hypesthesia ; Knee ; Labor, Obstetric ; Neurologic Examination ; Patient Education as Topic ; Peroneal Neuropathies ; Postpartum Period ; Pregnancy ; Rehabilitation

Letterer-Siwe disease is a one of Langerhans cell histiocytosis and characterized by proliferation of Langerhans cells. It's clinical features are onset of infancy, hemorrhagic crusted papules and petechiae, hepatomegaly, lymphadenopathy, localized bone defect, and fatal outcome. We report a 38-year-old woman with hepatomegaly, lymphadenopathy, scaly papules and petechiae on the trunk. The eletronmicroscopy showed a tennis racquet shaped Birbeck granules in cytoplasm of histiocyte. Systemic treatment with etoposide, cyclophosphamide, prednisone, and vincristine was effective.
Adult* ; Cyclophosphamide ; Cytoplasm ; Etoposide ; Fatal Outcome ; Female ; Hepatomegaly ; Histiocytes ; Histiocytosis, Langerhans-Cell* ; Humans ; Langerhans Cells ; Lymphatic Diseases ; Prednisone ; Purpura ; Tennis ; Vincristine

Dilated cardiomyopathy (DCM) is a multifactorial disease with enlargement and systolic dysfunction of one or both ventricles. Most of parturients having DCM would be advised to avoid or terminate her pregnancy owing to the high risk of cardiac overload that is likely associated with pregnancy. When maternal cardiac disease is observed, effective neuraxial analgesia may play an important role because a pain induced activation of the sympathetic nervous system during labor is associated with cardiovascular adverse effects, which may endanger the maternal and/or fetal well-being. Herein we report a case of a parturient with known DCM who received combined spinal-epidural anesthesia for labor and vaginal delivery. The maternal cardiac function and the effect of analgesia were evaluated by echocardiography during labor.
Analgesia* ; Anesthesia ; Cardiomyopathy, Dilated* ; Echocardiography ; Heart Diseases ; Humans ; Pregnancy ; Sympathetic Nervous System

We analyzed 13 cases of adult intussusceptions, which has been surgically treated, during the period from January 1987 to June 1997. There were 6 benign tumors, 2 malignant tumors and 5 others. The most common presenting symptom was abdominal pain. The types of intussusception were 3 jejuno- jejunal, 4 ileo-ileal, 1 ileocolic, 2 ileo-ileo-colic, and 1 colo-colic, intussusception of the vermiform appendix, and 1 combined type. In the pathlogic findings of the tumors, lipoma was the most common benign tumor and metastatic malignant melanoma was the most common malignant tumor. Other benign conditions were adhesion and granuloma due to tuberculosis. Surgical procedures were manual reduction, small bowel resection and anastomosis, a right hemicolectomy, a partial colectomy, and a polypectomy.
Abdominal Pain ; Adult* ; Appendix ; Colectomy ; Granuloma ; Humans ; Intussusception* ; Lipoma ; Melanoma ; Tuberculosis

Prurigo pigmentosa is a distinct type of inflammatory dermatosis characterized by recurrent pruritic erythematous papules that resolve, leaving a peculiar, reticulate hyperpigmentation. Although the cause of prurigo pigmentosa is unknown, exogenous factors, such as friction from clothing, have been suggested as possible triggers in some cases. In other cases, however, systemic conditions such as diabetes mellitus, pregnancy, fasting, and dieting were associated with prurigo pigmentosa, suggesting involvement of endogenous factors in the pathogenesis. There have been reported cases of prurigo pigmentosa associated with ketosis. Because ketosis is commonly observed in association with fasting, dieting, and insulin dependent diabetes melitus, it may be involved in the pathogenesis of prurigo pigmentosa. Here, we describe a patient with prurigo pigmentosa associated with diabetes mellitus and ketosis. In this case, the prurigo pigmentosa improved with the disappearance of the ketosis, but the blood sugar levels did not correlate with the improvement.
Blood Glucose ; Clothing ; Diabetes Mellitus ; Diet ; Fasting ; Friction ; Humans ; Hyperpigmentation ; Insulin ; Ketosis* ; Pregnancy ; Prurigo* ; Skin Diseases