Letterer-Siwe disease is a non-lipid reticuloendotheliosis of unknown etiology and usually grouped with Hand-Schuller-Christian disease and eosinophilic granuloma as components of histiocytosis-X. It occurs mostly in infancy and, clinically it is the most generalliaed and serious. In recent years, the prognosis of infants with Letterer-Siwe disease has improved, primarily due to more aggressive therapy with corticosteroids and antimetabolic agents. The 14 months old male patient was admitted into the Severance Hospital on 15th, July 1974 for evaluation of a chronic and refractory skin eruptions, abdominal distension with diarrhea and edema on extremities. He had been well until 3 months old age when he developed seborrhea-like skin eruptions which did not improve on treatment for seborrhea. Vigorous treatment was started for the baby from the beginning of admission day. Routine laboratory tests, skin biopsy, bone marrow aspiration and radiologic studies for bony structures wer done. All those studies had showed severe anemia, thrombocytopenia, multiple bony defects in skull and numerous non-lipid histiocytic infiltration in skin and bone marrow all those were consist with Letterer-Siwe disease.
Adrenal Cortex Hormones ; Anemia ; Biopsy ; Bone Marrow ; Dermatitis, Seborrheic ; Diarrhea ; Edema ; Eosinophilic Granuloma ; Extremities ; Histiocytosis, Langerhans-Cell* ; Humans ; Infant ; Male ; Prognosis ; Skin ; Skin Tests ; Skull ; Thrombocytopenia

Congenital ichthyosiform erythroderma is most severe type of ichthyosis. This disease is characterized clinically by generallized erythroderma covered with flaccid bullae and verruciform scales which are especially prominent on the flexural areas. This disease demonstrates an autosomal dominant mode of inheritance. The distinctive histologic change is the extreme hyperkeratosis associated with granular and vacuolar degeneration of the mid and upper malphigian layers. Cell kinetic studies indicate an increase in the epidermal mitotic turnover tirne. This 8 months old male patient visited to Dermatologic Department of Severance Hospital on April-28, 1973. Seven days after birth the scales were appeared on his flexural region of limbs and shed to leave a raw surface which forms scales anew. And there were the intermittent appearance of flaccid bullae prominantly involve the flexural area of limb but thereafter appearing the other region of body. Skin biopsy shows that of bullous type of congenital ichthyosiform erythroderma. This baby was expired 4 days after the admission day in spite of vigorous systemic and local trea- tments. The autopsy findings had exhibited fatty necrotic degenerative changes of hepatic cells and septic inflammatory changes of meningeal vessels and meninges itself as the causes of death of this pitiful victim.
Autopsy ; Biopsy ; Cause of Death ; Dermatitis, Exfoliative ; Extremities ; Hepatocytes ; Humans ; Ichthyosiform Erythroderma, Congenital ; Ichthyosis ; Infant ; Male ; Meninges ; Parturition ; Skin ; Weights and Measures ; Wills

No abstract available.
Lung* ; Oxygen* ; Rabbits* ; Ventilation*

No abstract available.
Humans

No abstract available.
Humans

No abstract available.
Biopsy* ; Child* ; Humans

PURPOSE: To evaluate the indication for intracavitary Urokinase(UK) in the treatment of Ioculated pleural effusion. MATERIALS AND METHODS: We analyzed CT and US in 31 patients who were treated with intracavitary UK in Ioculated pleural effusion. In each patient, a single chest catheter (10-12F) was insected under imaging guidance. When the amount of drainage was less than 100ml/day, UK was instilled through the catheter until less than 50ml/day was drained. On follow-up chest radiographs of more than 1 month, we classified the results of treatment into 3 groups:(1) completely effective (lung expansion, over 80%);(2) partially effective (20-80%); (3) ineffective (below 20%) group. Sonographic pattern of pleural fluid was classified into anechoic, septated, and honeycomb appearances and the thickness of parietal pleura was measured on CT. RESULTS: Sixteen patients were completely effective, nine were partially effective, and six were ineffective. patients with completely or partially effective outcome had anechoic and linear septated appearance on had less than 4mm of parietal pleural thickness on CT. Of six ineffective patients, US showed linear septated in one patient and honeycomb appearance in five patients and the thickness of parietal pleura on CT was 3 mm in one patient, 4ram in two patients, 5mm in one patient, and 6mm in two patients. CONCLUSION: UK instillation through percutaneous catheter was an effective method in the treatment of Ioculated pleural effusion. However, we found near complete reaccumulation of pleural fluid when honeycomb appearance of pleural fluid on US or more than 5mm parietal pleural thickness on CT was observed, which might suggest that we should consider the other kinds of treatment method in those patients.
Catheters ; Drainage ; Follow-Up Studies ; Humans ; Insects ; Pleura ; Pleural Effusion* ; Rabeprazole ; Radiography, Thoracic ; Thorax ; Ultrasonography ; Urokinase-Type Plasminogen Activator*

No abstract available.
Antigen-Antibody Complex* ; Humans ; Interleukin-2* ; Ovarian Neoplasms*

The transarterial embolization has been widely used to control bleeding. It has a variety of clinical utility; to reduce bleeding on the surgical field, to reduce the size of malignant tumor as a preopearative treatment, to treat arteriovenous malformation or arterial aneurysm as a curative method and to promote life qua' ity of patient with diffuse or multiple hepatocellular carcinoma as a palliative treatment, etc. With the advance of modem technology, various embolic materials have been also developed. IIowever, it has not- been -fully investigated of histopathologic changes of the embolized organs according to the embolic materials used. This study was undertaken to investigate the histopathologic changes of embolized renal artery in rabbit by various embolic materials, according to each embolic material and to time passed by after embolization. Of the 5 arteries embolized by ethylene vinyl alcohol copolymer(EVAL), one showed abscess formation in embolized kidney. The other 4 allowed to perform further pathologic study: within a week after embolization there was no any specific change in vessels, however, r,unimal endothelial hypertrophy was observed following 2 weeks of embolization. Of the 8 renal arteries embolized by N-buthyl-2-cyanoacrylate(Histoacryl), 4 showed total occlusion of the main renal arteries as well as renal infarction, which reflects the strong adhesiveness of Histoacryl to vascular wall. The other 4 showed fibrinoid degeneration in vascular wall within a week. However, further change was not observed thereafter. In all the 5 renal arteries embolized by polyvinyl
Abscess ; Adhesiveness ; Aneurysm ; Arteries ; Arteriovenous Malformations ; Carcinoma, Hepatocellular ; Enbucrilate ; Hemorrhage ; Humans ; Hypertrophy ; Infarction ; Kidney ; Modems ; Palliative Care ; Polyvinyls ; Renal Artery*

While a giant cell tumor most frequently involves the distal end of the femur and proximal end of the tibia, involvement of the cervical spine is quite rare. Recently we observed a patient with a giant cell tumor affecting the sixth cervical vertebra. This is regarded as a clinically malignant tumor because its nature and difficulty in diagnosis and treatment We easily confirmed the extent of the lesion by CT scanning with Metrizamide. The treatment was palliative tumor ressection. This case of a giant cell tumor affecting the sixth cervical vertebra is reported with a brief review of the literature.
Diagnosis ; Femur ; Giant Cell Tumors ; Giant Cells ; Humans ; Metrizamide ; Spine ; Tibia ; Tomography, X-Ray Computed