The multiple lentigines syndrome or LEOPARD syndrome is an autosomal dominantly inherited disorder with a variety of abnormalities and a familial occurrence. This syndrome is characterized by the presence of numerous dark brown macules on the skin but not the mucous surface, and by a marked increase in the number of lentigines from birth to puberty. The eponym LEOPARD stands for lentigines, EKG abnormalities, ocular hypertelorism, pulmonary stenosis, abnormalities of the genitalia, retardation of growth and deafness. We report a case of multiple lentigines syndrome in 7-year-old boy. He had numerous pinhead to pea sized, dark brownish macules scattered on the entire body and also had pulmonary stenosis, EKG abnormality, ocular hypertelorism and right exotropia. Interestingly, he also had a labial melanotic macule on the lower lip, which is usually spared in the multiple lentigines syndrome. Histologically, the biopsy specimen taken from the macule revealed an elongation of rete ridges, an increase of melanin pigments in the basal layer and mild inflammatory infiltrates intermingled with the melanophages in the upper dermis.
Adolescent ; Biopsy ; Child ; Deafness ; Dermis ; Electrocardiography ; Eponyms ; Exotropia ; Genitalia ; Humans ; Hypertelorism ; Lentigo ; LEOPARD Syndrome* ; Lip ; Male ; Melanins ; Panthera ; Parturition ; Peas ; Puberty ; Pulmonary Valve Stenosis ; Skin

Traditionally the mast cell has been known to he a cell component dwells in annective tissue. But oocasional epithelial mast cells were presented both in light microscopic and electron microscapic examinations. This study was undertaken to evaluate the presence of mast ceil in epidermis of diseased skin. A total of 93 biopsy specimes obtained from the skin lesions of patients with various dermatological conditions were examined with light microacope after toluidine blue stain. The results are as follows: 1. Mast cells were shown in the epidermis of 2 patients with chronic eczema and one patient with pustular psoriasis. 2. Most of the epidermal mast cells were located in ar nearby the basal ceII layer. 3. Epidrmal mast cells have an oval or round nucleus and variable amounts of granulesin the cytoplasm. Some of the cells have a halo-like clear zone around them.
Biopsy ; Cellular Structures ; Cytoplasm ; Eczema ; Epidermis ; Humans ; Lichens* ; Mast Cells ; Psoriasis ; Skin* ; Tolonium Chloride

No abstract available.
Glomerulonephritis, IGA* ; Immunity, Cellular* ; Immunoglobulin A*

No abstract available.
Glomerulonephritis, IGA* ; Immunity, Cellular* ; Immunoglobulin A*

No abstract available.
Acute Kidney Injury* ; Nifedipine* ; Plasma* ; Renin*

Endodermal sinus tumor of the mediastinum is a very rare malignant tumor, usually affecting young adolescents, and its histologic findings are characteristic as that of gonadal germ cell origin. We describe the cytologic finding of fine needle aspiration of mediastinal endodermal sinus tumor in a 19-year-old male patient, comparing with tissue section. The tumor cells in smears were usually in tight clusters with large overlapping cells, which were arranged in a papillary or microacinar pattern, suggesting carcinoma. The tumor cells were large, round or oval with a small amount of cytoplasm which was occasionally vacuolated and had indistinct border. The nuclei were large, pleomorphic, and vesicular with large promi- nent nucleoli. The tissue sections showed typical findings of endodermal sinus tumor. Careful correlation of cytological findings and the serum alpha-fetoprotein level would be helpful to confirm the diagnosis.
Adolescent ; alpha-Fetoproteins ; Biopsy, Fine-Needle ; Biopsy, Needle ; Carcinoma, Papillary ; Carcinoma, Squamous Cell ; Child* ; Clonorchiasis ; Cytoplasm ; Diagnosis ; Dietary Proteins* ; Endodermal Sinus Tumor ; Female ; Germ Cells ; Giant Cells ; Gonads ; Humans ; Kidney Failure, Chronic* ; Male ; Mediastinum ; Middle Aged ; Thyroid Gland ; Urinary Tract Infections* ; Urinary Tract* ; Vesico-Ureteral Reflux* ; Young Adult

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

BACKGROUND: The Cobas(R) Integra is a new automated chemistry analyzer with continuous and random-access features for routine chemistries, specific proteins, electrolytes, therapeutic drugs and drugs of abuse. The system maintains 68 test-specific reagent cassettes on board and have test principles of absorbance photometry, immunoturbidometry, fluorescence polarization and ISE measurements. We evaluated the usefulness of Cobas(R) Integra in the aspect of accuracy, precision, linearity, comparison study, test speed and general performance. METHODS: We evaluated the analytical performance of the Cobas(R) Integra for T. bilirubin, CRP, BUN, creatinine, sodium, potassium, chloride, valproic acid, phenytoin and carbamazepine according to NCCLS guidelines (EP5-T2, EP6-P, EP9-T and GP10-T). RESULTS: In accuracy study, error ranges(%) of most test items, except sodium and chloride, were within analytic goals. In within-run and between-run precision study, Coefficient of variation (CV, %) of most items, except sodium, were lower than limits suggested by College of American Pathologists Survey 1991. The linearities were maintained well in the range of medically significant levels and were statistically acceptable (p<0.001). The comparison study for most items, except sodium and chloride, indicated good correlation with Hitachi 747 and TDx FLx(TM) and correlation coefficients (r) were above 0.98. Throughput was about 450 tests/hr. CONCLUSIONS: Cobas(R) Integra showed satisfactory accuracy, precision, linearity, good correlation with other analyzers and high throughput. So, we concluded that Cobas(R) integra is highly suitable for not only routine use but also emergency tests in medium to large-sized hospitals because of its high productivity and reliability.
Bilirubin ; Carbamazepine ; Chemistry* ; Creatinine ; Efficiency ; Electrolytes ; Emergencies ; Fluorescence Polarization ; Phenytoin ; Photometry ; Potassium ; Sodium ; Street Drugs ; Valproic Acid

BACKGROUND: The Cobas(R) Integra is a new automated chemistry analyzer with continuous and random-access features for routine chemistries, specific proteins, electrolytes, therapeutic drugs and drugs of abuse. The system maintains 68 test-specific reagent cassettes on board and have test principles of absorbance photometry, immunoturbidometry, fluorescence polarization and ISE measurements. We evaluated the usefulness of Cobas(R) Integra in the aspect of accuracy, precision, linearity, comparison study, test speed and general performance. METHODS: We evaluated the analytical performance of the Cobas(R) Integra for T. bilirubin, CRP, BUN, creatinine, sodium, potassium, chloride, valproic acid, phenytoin and carbamazepine according to NCCLS guidelines (EP5-T2, EP6-P, EP9-T and GP10-T). RESULTS: In accuracy study, error ranges(%) of most test items, except sodium and chloride, were within analytic goals. In within-run and between-run precision study, Coefficient of variation (CV, %) of most items, except sodium, were lower than limits suggested by College of American Pathologists Survey 1991. The linearities were maintained well in the range of medically significant levels and were statistically acceptable (p<0.001). The comparison study for most items, except sodium and chloride, indicated good correlation with Hitachi 747 and TDx FLx(TM) and correlation coefficients (r) were above 0.98. Throughput was about 450 tests/hr. CONCLUSIONS: Cobas(R) Integra showed satisfactory accuracy, precision, linearity, good correlation with other analyzers and high throughput. So, we concluded that Cobas(R) integra is highly suitable for not only routine use but also emergency tests in medium to large-sized hospitals because of its high productivity and reliability.
Bilirubin ; Carbamazepine ; Chemistry* ; Creatinine ; Efficiency ; Electrolytes ; Emergencies ; Fluorescence Polarization ; Phenytoin ; Photometry ; Potassium ; Sodium ; Street Drugs ; Valproic Acid

A simple and convenient management system in coding and storage of the pathologic diagnoses was developed by generating the dBASE III with IBM-PC microcomputer. The program named "Pathology Slide Bank" can store about 3,600 data according to the SNOP coding in a standard 51/4 inch floppy disk and retrieve the data within two minutes.