1/20. A patient with type 2 Gaucher's disease with respiratory disease.A 5-month-old boy had respiratory problems and gastroesophageal reflux. Electron microscopy of a tracheal biopsy specimen showed accumulation of lamellar bodies in the columnar cells indicative of lysosomal storage disease. Subsequently, the child had neurologic symptoms and hepatosplenomegaly, and the diagnosis of Gaucher's disease type 2 was made.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
2/20. Predicted survival in peptic ulcer patients based on computer analysis of preoperative variables.A prospective study designed to emphasize and quantitate the operative risk of patients preparing to undergo surgery for the treatment of complicated peptic ulcer disease is presented. Data were gathered from 347 consecutive patients operated on with benign gastric and/or duodenal ulcers in a veterans Hospital over an 8-year period. Resident surgeons performed all operations and for the most part decided on the operative procedure used, with advice from attending faculty. Preoperative factors influencing the operative mortality in 34 patients were compared with those in surviving patients and subjected to a multivariant discriminant function analysis by computer. Ten variables were identified as being significantly different (P less than 0.05-P less than 0.01) between the survivor and non-survivor groups. Using the discriminant weights of these variables, a computer program was written to calculate the 30-day operative mortality of any preoperative patient based on this past experience. The accuracy of the program is excellent in good risk patients; i.e., a predicted greater than 90% chance of survival was correct 98.9% of the time with 3 deaths in 279 patients. patients at the low end of the scale (less than 10%) were predicted with 85% accuracy. In the last 8 months, 29 patients have undergone surgery after prospective computer assessment of their operativ risk. All have survived with a predicted chance of greater than 50%. Four patients died with survival chances predicted at 4, 2, 1, and 1%. The computer may be used as an educational vehicle for sharpening our preoperative assessment of a patient with ulcer disease, particularly regarding operative risk.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
3/20. Aplasia of respiratory tract cilia.We report on ciliary aplasia of the respiratory tract, a rare disorder of the mucociliary apparatus, that is insufficiently recognized as a distinct entity. A culture method for ciliogenesis was developed by our laboratory and offers the advantage of studying cilia free of secondary changes associated with infection. Three cases of primary ciliary aplasia were documented histologically in direct biopsy specimens and also in biopsy specimens cultured specifically for ciliogenesis. Primary ciliary aplasia should be differentiated from secondary ciliary aplasia in which basal bodies are present and ciliogenesis takes place in specific culture. Only hereditary ciliary abnormalities are expressed in cell cultures. We critically review the cases of ciliary aplasia reported to date.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
4/20. Pulmonary manifestations of the eosinophilia-myalgia syndrome associated with tryptophan ingestion.Pulmonary manifestations are not infrequent in the L-tryptophan-induced eosinophilia-myalgia syndrome (EMS). However, previous reports have not described the results of longitudinal pulmonary function, exercise testing, high-resolution computerized tomographic (HRCT) scanning of the chest, or detailed bronchoalveolar lavage (BAL) analysis. We report six patients with EMS who had dyspnea. The diffusing capacity for carbon monoxide was decreased in five patients tested. exercise testing with arterial blood gas sampling in three patients was consistent with pulmonary vascular or parenchymal disease. Serial exercise testing in two of these patients demonstrated marked improvement temporally associated with corticosteroid treatment. In four patients, HRCT scanning of the chest was abnormal. One of these patients showed no abnormality on routine chest roentgenogram. Two patients undergoing BAL exhibited increased eosinophils in the lavage fluid; a third had elevated lymphocytes. Serial measurements of fibroblast proliferation-stimulating-activity in samples of BAL fluid obtained from serial examinations in two patients exhibited heightened pretreatment activity that returned to the normal range following corticosteroid therapy. In these two patients, increased proportions of T-suppressor/cytolytic (CD8 ) cells were observed in the BAL fluid. Despite aggressive immunosuppressive therapy, one of the patients died of respiratory failure. Another remains markedly dyspneic with pulmonary hypertension. Of the remaining four patients, two exhibited resolution of pulmonary symptoms after systemic corticosteroid therapy, and two experienced partial improvement.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
5/20. Benign mucous membrane pemphigoid of the upper aero-digestive tract: rare paraneoplastic syndrome presentation in renal cell carcinoma.Benign mucous membrane pemphigoid is a rare autoimmune disorder affecting the upper aero-digestive tract and conjunctivae. This is a case presentation of benign mucous membrane pemphigoid affecting the oral mucosa, pharynx, oesophagus and larynx, leading to cicatricial lesions in the pharynx and larynx, causing dysphagia, hoarseness and stridor. The alternative forms of management for laryngeal scarring due to this disease are explained. The patient was later diagnosed with advanced renal cell carcinoma, raising the possibility of cicatricial pemphigoid manifesting as a paraneoplastic syndrome of underlying renal cell carcinoma.- - - - - - - - - - ranking = 6keywords = cell (Clic here for more details about this article) |
6/20. Griscelli syndrome.A 4-month-old child had silvery gray hair, light-colored skin, recurrent chest infections, hepatosplenomegaly, and episodes of pancytopenia and hemophagocytosis in the liver, spleen, and bone marrow. light microscopy of hair showed characteristic large aggregates of pigment granules distributed irregularly along the hair shaft. Peripheral blood smear examination did not show giant granules in granulocytes. Enlarged hyperpigmented basal melanocytes with sparsely pigmented adjacent keratinocytes were seen on the skin biopsy specimen. On the basis of these clinical and laboratory findings, Griscelli syndrome was diagnosed. The child succumbed to infection during an accelerated phase of the disease.- - - - - - - - - - ranking = 5keywords = cell (Clic here for more details about this article) |
7/20. Ultrastructure of bronchial submucosal glands in children with chronic respiratory diseases.The ultrastructure of bronchial submucosal glands in 4 children with chronic respiratory diseases was studied. Based on their ultrastructure the glandular secretory cells were divided into four categories: 1. mucous cells packed with large electron-lucent coalescing granules with fibrogranular matrix, 2. mucous-like cells with abundant granular endoplasmic reticulum and fewer smaller electron-lucent granules, 3. serous cells containing small to moderately large membrane-bound electron-dense granules with homogenous matrix and prominent granular endoplasmic reticulum, 4. sero-mucous cells similar to the serous ones but containing granules of either serous or mucous morphology. In our patients the mucous and mucous-like cells prevailed, forming 54.6%-100% of the glands' secretory area. Only in 2 children all four types of secretory cells were detected. Signs of pathological alteration were discovered in secretory cells of all patients. The system of intra- and interlobular ducts was lined by tall mucous cells.- - - - - - - - - - ranking = 9keywords = cell (Clic here for more details about this article) |
8/20. Pulmonary manifestations of juvenile laryngotracheal papillomatosis.Juvenile laryngotracheal papillomatosis spreads to involve the lungs in less than 1% of cases, and when this occurs, the prognosis is poor. In seven such cases, the lung lesions, which appeared either solid or cystic on radiographs, proved to be benign squamous cell proliferations or papillomas, with central cavities containing debris or air. They seemed to grow centrifugally, using the alveolar walls as scaffolding with central coalescence and lung destruction. Papillomas spread inferiorly from the larynx by direct extension as far as the major bronchi, but rarely beyond. However, the parenchymal lesions were widely scattered, and some were subpleural. This discrepancy suggests that fragments become detached, particularly during endoscopic resection, and are carried down the airways by airflow. Those that lodge proximal to the respiratory bronchioles may be removed by mucociliary action and cough. Those that travel more distally are poorly cleared and may grow. If enough lung parenchyma is destroyed, the patient can develop symptoms of restrictive lung disease in addition to signs of upper airway obstruction.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
9/20. Gamma heavy-chain disease involving upper airway.Gamma heavy-chain disease is a rare disorder. It is a plasma cell dyscrasia, with the patient's age at onset being 9 to 87 years. Identification of IgG heavy chains in the serum and urine is diagnostic. Histopathologic features are nonspecific. Although the clinical presentation is diverse, many signs and symptoms lead to early otorhinolaryngologic consultation. For this reason gamma-heavy-chain disease should be considered in the differential diagnosis of inflammatory lesions of the upper aerodigestive tract.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
10/20. munchausen syndrome by proxy: definition of factitious bleeding in an infant by 51Cr labeling of erythrocytes.The munchausen syndrome by proxy is a phenomenon in which symptoms of a disease are fabricated by some person other than the patient. This report describes and 8-week-old infant with repetitive bleeding episodes, presumably originating from the upper respiratory tract. Extensive investigations, including angiography, several endoscopies under general anesthesia, and reinfusion of the infant's red cells labeled with 51Cr followed by pulmonary and upper airway scanning, failed to reveal the source of bleeding. Within two weeks after initiation of the 51Cr studies, radioactivity of facial blood from two separate bleeding episodes did not exceed background counts. Simultaneous examination of the infant's capillary blood, however, showed moderate to marked radioactivity. The Rh subtype of the facial blood was cc, whereas the infant's type was Cc. These findings indicated that the facial blood was factitious in origin. No further "bleeding" occurred after this information was presented to the parents. This case represents an unusual example of the munchausen syndrome by proxy. awareness of this entity can prevent potentially harmful investigations. Documenting its occurrence and sharing the information with parents in a nonaccusatory manner may prevent future harm to the patient.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
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