Histiocytosis X is characterized by aggregates of Langerhans cells with other inflammatory cells. These Langerhans cells are antigen-presenting cells to T lymphocytes and identified by characteristic morphology, ultrastructural demonstration of Birbeck granules and immunologic reactivity with OKT-6 and HLA-DR antibodies. In this report, the tumor arising in a 2-years-old baby was examined byimmunostaining with several macrophage/dendritic cell markers. The main tumor cells showed cytoplasmic and nuclear staining with S-100 protein and ring-like surface and paranuclear staining with PNA. However, they were negative for follicular dendritic cell marker CD21, macrophage markers lysozyme, Mac 387, alpha-1 antitrypsin and CD68, and interdigitating reticulum cell marker ID4 and ID5. These observations demonstrate the usefulness of S-100 protein and PNA for the identification of Langerhans cells in paraffin-embedded tissue.

A total of 197 cases of fungal infections in biopsy specimens obtained from the pathology file of the Department of Pathology, Seoul National University Hospital, for a period of 28 years from 1960 to 1987 were studied by histopathologic investigations with analysis of clinical records. The following results are obtained; 1) While most fungal infections increased in number, annual relative frequency of them were between 0.1% and 0.2%. 2) Among 197 cases of fungal infections, deep-seated mycosis was 175 cases (88.8%). Aspergillosis was the most common mycosis, accounting for 29.4 percent. 3) The age and sex distribution of fungal infection was even throughout the ages, but it was more common in age group over fifty, and the male was more frequently affected than the female. 4) Aspergillosis was encountered in 58 cases (29.4%) and the preferred localization was the lung. The candidiasis was seen in 48 cases (24.4%) and the preferred localization of cryptococcosis was lymph node and skin.
Female ; Male ; Humans ; Biopsy

No abstract available.
Child* ; Humans

The pulmonary cavernous hemangioma is usually from birth and there may be without symptoms until adulthood. Larger or multiple pulmonary angiomata with considerable pulmonary arteriovenous shunts may cause cyanosis, finger clubbing, dyspnea and frequently accompanyingbruit. Recently, we experienced a case of cavernous hemangioma of the lung. A 34-year-old woman was admitted to our hospital for surgical evaluation of a 4 cm solitary, round nodule in the right upper lobe on the chest X-ray and CT scan. She had no symptoms. Laboratory findings are within normal limits except for elevated glucose levels. At surgery, the mass was well encapsulated and easily excised from the peripheral portion of the posterior segment of the right upper lobe. Grossly, it consisted of a 4 cm in diameter, round, soft, sponge-like, hemorrhagic, slightly lobulated mass with a smooth external surface. Microscopically, the mass was composed of vessels, which were thin walled, dilated and filled with blood. The wall of the abnormal vessels was thin and composed of endothelium and fibrous connective tissue with only a little smooth muscle. Immunohistochemically, the wall of the dilated abnormal vessesls showed negative reaction for cytokeratin(low and high) and epithelial membrane antigen but weakly positive reaction for UEA-1 in focal areas.
Adult ; Male ; Female ; Humans ; Hemangioma

Congenital hepatic fibrosis is an inherited, congenital disorder of the liver, and is occasionally associated with cystic disease of the liver and kidney. We present a case of congenital hepatic fibrosis with Caroli's disease. A 21-year-old woman had suffered from an episodic fever with headaches for 3 years. In laboratory examination, the liver function test was within the normal limits. Esophageal varix was noted by an endoscopic examination. Hepatosplenomegaly and multiple dilated bile ducts were seen by abdominal CT scanning. An orthotopic whole liver transplantation was done. The liver was fibrotic and enlarged. Multiple cystically dilated intrahepatic ducts were noted. Microscopically, diffuse portal fibrosis and widening with proliferation of bile ductules were seen. Intrahepatic bile ducts were markedly dilated and tortuous. The liver cell cords were well preserved.
Bile ; Bile Ducts ; Bile Ducts, Intrahepatic ; Caroli Disease* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Esophageal and Gastric Varices ; Female ; Fever ; Fibrosis* ; Headache ; Humans ; Kidney ; Liver ; Liver Function Tests ; Liver Transplantation ; Tomography, X-Ray Computed ; Young Adult

Aniridia or irideremia is characterized by reduction of iris or absence of total iris, but a rudimentary iris is always present in most cases. This congenital anomaly is familial and the transmission is strongly dominant by an autosomal gene. It demonstrates high penetrance and variable expression. Aniridia is mainly due to a primary defect in development of the neural ectoderm and an aberrant development of the mesoderm. Glaucoma and cataract in the eye is frequently complicated with other deformities of the body and the treatment is not satisfactory. The author have experienced a case of aniridic family which is composed of 7 persons. Of these, the father, one son and 3 daughters are affected with aniridia, cataract, nystagmus and amblyopia and the mother and one son are healthy. The author reports this cases with the review of literature.
Amblyopia ; Aniridia* ; Cataract ; Congenital Abnormalities ; Ectoderm ; Fathers ; Glaucoma ; Humans ; Iris ; Mesoderm ; Mothers ; Nuclear Family ; Penetrance

BACKGROUND: Dementia has emerged as a leading public health problem with elderly persons and its early detection is important for treatment in curable cases. Although dementia screening tests are available, they are still complex and time consuming in practice and therefore difficult to use. Our goal was to validate the time and change(T&C) test, a simple, standardized method for detecting dementia in the elderly populations. METHODS: Participants were 59 patients aged 65 years or older at an urban hospital from November 1 to December 31, 2001. The time test evaluated the understanding of clock hands indicating 11:10, and the change test the ability making 1,000 Won from a group of coins consisting one 500 Won, seven 100 Won, seven 50 Won. T&C ratings were validated against a reference standard based on physician`s diagnosis. Test-retest reliability and inter-observer reliability were assessed. RESULTS: The T&C test had a sensitivity of 73.0%, specificity of 90.9%, positive predictive value of 93.1%, and negative predictive value of 66.7%. Test-retest and inter-observer agreement rates were 95% and 95%, respectively. The T&C test was not influenced by education. The time and change tests took a mean of 7.5 seconds and 19.6 seconds to complete respectively, and was acceptable to participants. When timed cut points were added, the T&C test had a sensitivity of 86.5%, specificity of 40.9% and test-retest and inter-observer agreement rates were 77.3% and 81.8%, respectively. CONCLUSION: The T&C test is a simple, accurate reliable, performance-based tool for detection of dementia in the elderly.
Aged* ; Dementia* ; Diagnosis ; Education ; Hand ; Hospitals, Urban ; Humans ; Mass Screening* ; Numismatics ; Public Health ; Sensitivity and Specificity

No abstract available.
Neck*

Recently, we experienced a patient with a B cell leukemia which could not be classified as chronic lymphocytic leukemia (CLL), prolymphocytic leukemia (PL) or follicular lymphoma in leukemic phase. He is a 58-year-old male and suffered from progressive lymphadenopathy at both inguinal and cervical areas. His blood film showed lymphocytosis (79%), and the cells were heterogeneous in size and shape. Twenty-five percent of the lymphocytes had nuclear irregularities or cleavages unlike the lymphocytes of CLL In which the cells often shows monomorphic features, small size with scanty cytoplasm and round nucleus. The bone marrow trephine biopsy specimen showed a diffuse pattern of infiltration of atypical Iymphoid cells. The lymph node histology showed atypical lymphoid colls proliferated as wide mantles around non-neoplastic appearing germinal centers. The immunophenotype of circulating lymphocytes in peripheral blood showed strong reactivity with CD5, CDl9 and CD20 without expression of CD2, CD3, CD7, CD10, CD22 and CD23. Although this case resembled CLL, the laboratory features showed major differences, notably in the Peripheral blood morphology, histology Patterns and the membrane Phenotype. By combining these data, we diagnosed this case as a leukemic phase of mantle cell lymphoma.
Biopsy ; Bone Marrow ; Cytoplasm ; Germinal Center ; Humans ; Leukemia, B-Cell ; Leukemia, Lymphocytic, Chronic, B-Cell ; Leukemia, Prolymphocytic ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphocytosis ; Lymphoma, Follicular ; Lymphoma, Mantle-Cell* ; Male ; Membranes ; Middle Aged ; Phenotype

A fifteen yrs old boy developed divergence paralysis following meningitic attack. The ocular examination revealed homonymous diplopia in all gazes when he fixed at further than 13 cm. He had 18 delta esotropia at distance and 7 delta esophoria at near in prism cover test. His stereopsis was excellent at near in House Fly Stereopsis Test. There wasn't much change in ocular examination in 90 days after initial examination.
Depth Perception ; Diplopia ; Diptera ; Esotropia ; Humans ; Male ; Paralysis*