In 1908 Leiner described the peculiar skin condition among infants to which he applied the name erythroderma desquamativa. The four cardinal features of Leiners disease are generalized seborrheic dermatitis, intractable severe diarrhea, recurrent local and systemic infections usually of gram negative etiology, and marked wasting and cyst rophy. A 2-month-old female presented to our clinic with generalized seborrheic erythrodermic eruption and intractable diarrhea. She had sufferd from these symptoms since birth. Her body weight was 3.4kg (below 3rd percentile). Her scalp was covered with thick yellowish crsts. There was generalized erythroderma covered vith scales and crusts. Laboratory result; revealed gram negative sepsis and DIC. Chest X-ray showed the findings of minimal aspir ition pneumonia in the right upper lobe. She expired on the following day.
Body Weight ; Dacarbazine ; Dermatitis, Exfoliative ; Dermatitis, Seborrheic ; Diarrhea ; Female ; Humans ; Infant ; Parturition ; Pneumonia ; Scalp ; Sepsis ; Skin ; Thorax ; Weights and Measures

No abstract available.
Hepatitis* ; Humans

The most common malignant renal neoplasm is renal cell carcinoma. It is estimated that renal cell carcinoma accounts for 1% of all primary malignancies in Korea. Rell cell carcinoma presents diverse clinical courses with gross, histopathologic features. It has been known to be very difficult tumor to predict its clinical prognosis. In Korea, many studies have been reported concerning the clinical aspects of renal cell carcinoma. However, pathological studies of renal cell carcinoma are very few even though studies of nuclear grade have been attempted recently. We reviewed 93 cases of renal cell carcinoma examined in the period from 1978 to 1987 in the department of pathology, Yonsei university college of medicine, Yongdong Severance hospital, Wonju college of medicine and analyzed the histopathologic classification, including nuclear grade according to the Fuhrman's method. We abtained the following results by studying the relationship of the factors which had been known as correlated with the prognosis. 1) The ages of patients ranged from 9 to 74 years with a peak in the 6th decade. 2) The most common symptoms of the patients were hematuria, mass and pain, in that oder, and 7 patients complained to specific symptoms. The incidentally found cases characterized stage I, nuclear grade 2 small tumor size (not more than 4 cm) and clear cell type. 3) The renal cell carcinoma was more frequently located in the left kidney than the right by a ratio of 1.25 : 1. The incidence of intrarenal location was divided to the upper pole, 40% : mid portion, 29% : lower pole, 23% : diffuse involvement, 8%. The tumor shoing diffuse growth pattern had a large size, high nuclear grade and mixed cells. 4) The tumor size averaged 8 cm and there was no significant relationship between the size and stage. Seven cases of neoplasms not more than 3 cm were seen, of which 2 cases revealed an outcome of distant metastasis. 5) The histological pattern showed major solid, 53% : tubular, 11% : mixed, 18% : papillary, 9% and sarcomatoid type 9%. The sarcomatoid type was characterized by grade 4, a larger size(more than 10 cm), advanced stage. 6) There was no special relationship between the stage and grade but mostly grade 2 occupied the stage I. 7) The clear cell type was predominantly noted at grade 2 (65%), at the stage I (63%), granular or mixed cell type at grade 3 (87%), 4 (70%). According to these results, the tumors showing a sarcomatoid histologic pattern, diffuse growth pattern had unfavorable prognostic factors, and are thus estimated to have a poor prognosis. But the case which were incidentally found have favorable prognostic factors and probably a better prognosis. The tumor size alone can not exactly predict the metastasis and is not correlated with the stage. Small renal cell neoplasm (not more than 3 cm) generally has unfavorable prognostic factors and should be considered potentially malignant. The high grade frequently has granular cytoplasm. This represents the relationship between grade and cytoplasm, poor prognosis in the granular cell than the clear. The renal cell carcinoma shows variable prognosis and thus the prognosis should be estimated by all the factors. Nuclear grade can be used as one of the useful prognostic factors.
Incidence ; Neoplasm Metastasis

Hamartoma of the breast is relatively rare benign tumor, which is a well-circumscribed mass mainly composed of fibrous stroma, a(tipose tissue, ducts and acini. Muscular hamartoma of the breast is mainly composed of smooth muscle, and is extremely rare because proper smooth muscle is normaly absent in the breast except in the nipple. We describe a rare case of muscular hamartoma of the breast in a 38-year-old woman. This tumor was located in the upper outer quadrant and a 3 x 2.5 x 2 cm sized, well-demarcated but not encapsulated mass, The mass consisted mainly of irregularly arranged smooth muscle bundles in the fibrous stroma with lobular units and admixed fat cells. The origin of smooth muscle in hamartoma is not well known.
Female ; Humans ; Hamartoma

The immunoreactivities of surgically removed 16 cardiac myxomas were studies for factor VIII-related Ag (F VIII-RA), Ulex europaeus agglutinin I (UEA-I) and desmin alpha1-antichymotrypsin, and this study was accompained by a clinicopathologic review. More than 50% of the patients with cardiac myxomas were in their fourth and fifth decades, and cardiac myxomas were much more common in women than in men. All but on occurred in the left atrium, and the majority were attached to the atrial septum, usually in the region corresponding to the fossa ovalis. In one case, an atrial myxoma recurred 37 months after the initial excision. Microscopically, the myxomas contained a myxoid matrix composed of acid mucopolysaccharides within which were embedded polygonal cells. The cells forming both the surface and complicated vascular like channels throuhout the myxoid stroma tested positive for F VIII-RA and UEA-I. The outer cell layers of the complex vascular structures demonstrated variable staining for F VIII-RA, while isolated bundles of smooth muscle cells were present and stained for desmin. A small number of the so-called myxoma cells, immunoreactive for alpha1-antichymotrypsin which were not laden with hemosiderin pigment but were similar to histiocytes, were present particularly around the areas of hemorrhage. These findings support the current view that cellular and histologic heterogeneity arose from the divergent differentiation of multipotential mesenchymal cells. In particular, it remains to be confirmed by further study whether or not true histiocytic differentiation occurs.
Female ; Male ; Humans

No abstract available.
Lipoma*

BACKGROUND/AIMS: For laboratory diagnostics in liver diseases, many enzymes have been used for the assessment of hepatocellular function. Among them, two transaminases, alanine and aspartate aminotransferase, have been regarded as the most sensitive indicators of hepatocellular damage. However, the enhanced enzyme activities of the enzymes do not exactly indicate or represent the cause and progression of diseases in the patients with liver disease. To overcome such limitations, immunological methods have been suggested as one of the alternatives for the replacement or supplement of the conventional enzymatic analysis. METHODS: In the hope of developing a new assay system for measuring the AST concentration rather than its activity, we have developed a new assay using fluorescence labeled anti-AST monoclonal antibodies. Blood was obtained from a normal population of 234 patients and 43 liver disease patients. The linearity, limit of detection, and performance of the new assay system were tested and evaluated. The comparability of assay was examined with an ELISA and biochemical assays. RESULTS: The linearity fell in the range of 0-1 mg/L of AST (R=0.995), and the analytical detection limit was 12 microgram/L of AST. The mean recovery of the control was 102.4 % in a working range. The precision of the intra- and inter-assay in a range of 50-800 microgram/L was CVs < 7% and CVs < 6%, respectively. In the normal population, the mean AST concentration was 35.5 microgram/L. The mean AST concentration in patients with liver disease was 266.5 microgram/L. The new assay system correlated well with an ELISA and biochemical assay for quantification of AST concentration (R=0.92 and 0.88, respectively; N=43). CONCLUSIONS: We have developed a new immunological assay using generated monoclonal antibodies to human cytosolic AST and used them for the development of a fluorescent assay measuring the enzyme mass. Cytosolic AST mass in sera could be measured reproducibly by the immunological method. In conclusion, this study has provided us with a new type of tool for an accurate measurement of the enzyme amount in circulation.
Antibodies, Monoclonal ; Aspartate Aminotransferase, Cytoplasmic/*blood/immunology ; Fluorescent Antibody Technique/*methods ; Humans ; Liver Diseases/diagnosis

No abstract available.
Blood Pressure* ; Metoclopramide* ; Rabbits*

A total of 127 cases of seborrheic keratosis examind at the Dept. of Pathology, Yonsei University School of Medicine during 1980~1986 was reviewed. The results were as follow; 1) Sex distribution showed without preponderance with male; Female ratio of 1.05:1. 2) Age distrubution were 10~19 years 0.8%, 20~29 years 4.7%, 30~39 years 14.2%, 40-49 years 25.2%, 50~59 years 29.1%, 60~69 years 17.3%, 70~79 years 6.3% and neck (3.7%). 3) The most common site was face (44.8%), followed by trunk (29.8), lower extremities (10.4%), scalp (6.7%) and neck (3.7%). 4) The clinical diagnoses were sebrorrheic keratosis (47%), nevus (15.4%), verruca (12%), malignant melanoma (7.7%), actinic keratosis (3.4%), pyogenic granuloma (2.6%), mass (2.6%), basal cell epithelioma (0.9%), corn (0.9%), leproy (0.9%), and angiokeratoma (0.9%). 5) The distributions of the light microscopic diagnosis were acanthotic 52 cases (42.9%), hyperkeratotic 33 cases (30%), irritated 17 cases (13.4%), adenoid 15 cases (11.8%), clonal 3 cases (2.4%), melanoacanthoma 1 case (0.8%) and mixed 6 cases (4.7%). 6) The lesions measured from 0.2 cm to 3 cm in maximum diameter and its color was light brown to black with sharply demarcated papule, pladque or nodule.
Female ; Male ; Humans

No abstract available.