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Biomedical subjects
Publications and source records attributed to R Miller.
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We studied a case of Wolman's disease that is apparently the first to be reported in a black infant. Culture of the skin fibroblasts showed massive accumulation of neutral lipids, accompanied by decreased esterolytic activity as measured by fluorometric assay. Electrophoresis showed decreased activity of an esterase allotype, with low electrophoretic mobility.
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A 47-year-old woman with right hydroureteronephrosis due to ureterovesical junction obstruction had gross hematuria after being treated for five years wtih cyclophosphamide for cerebral vasculitis. A right nephroureterectomy was required for control of bleeding. The pathology specimen contained clinically occult invasive carcinoma of the renal pelvis. Although the ability of cyclophosphamide to cause hemorrhagic cystitis and urine cytologic abnormalities indistinguishable from high grade carcinoma is well known, it is less widely appreciated that it is also associated with carcinoma of the urinary tract. Twenty carcinomas of the urinary bladder and one carcinoma of the prostate have been reported in association with its use. The present case is the first carcinoma of the renal pelvis reported in association with cyclophosphamide treatment. It is the third urinary tract cancer reported in association with cyclophosphamide treatment for nonmalignant disease. The association of the tumor with preexisting hydroureteronephrosis suggests that stasis prolonged and intensified exposure of upper urinary tract epithelium to cyclophosphamide. Patients who are candidates for long-term cyclophosphamide treatment should be routinely evaluated for obstructive uropathy.
Neutrophil leukocytes are the body's major defence against bacteria, which they phagocytose and kill. It has been found that phagocytosis and killing are accompanied by a dramatic rise in non-mitochondrial respiration; and that the efficiency of killing is impaired in the absence of oxygen. It is also impaired in neutrophils from patients with chronic granulomatous disease (CGD), where the respiratory burst is absent. This has been difficult to reconcile with their normal content of granule proteins that kill bacteria in vitro. Indeed, CGD cells are essentially normal both morphologically and constitutionally except that they lack a functional very low potential cytochrome b (b-245), which is a component of the oxidase system responsible for the respiratory burst of normal cells. Activation of the oxidase is associated with the generation of various reduced oxygen species which have been widely thought to be responsible for the killing of phagocytosed microorganisms either directly, or by acting as substrates for myeloperoxidase-mediated halogenation. We report here, however, that a major consequence of the defective function of this oxidase in neutrophils and monocytes from CGD patients is an absence of the normal initial rise, and an unusually rapid and extensive fall in pH which is itself associated with the impairment of the killing and digestion of intracellular staphylococci.
Flurbiprofen is a nonsteroidal anti-inflammatory (NSAI) agent currently undergoing clinical investigation. Anti-inflammatory steroids have long been known to delay the healing of corneal stromal wounds. This was designed to compare the effects of equipotent anti-inflammatory doses of flurbiprofen and of prednisolone acetate on the inflammation and the healing (as measured by the wound bursting pressure) or 4-mm through-and-through incisions treated four times a day for ten postoperative days. The results suggest that flurbiprofen and prednisolone are not different in their effect on both postoperative inflammation and postoperative wound healing. Since NSAI agents and steroids inhibit prostaglandin formation at different enzymatic steps, it is possible that prostaglandins not only are responsible for postoperative inflammation but also are required for postoperative wound healing.
Models for 8-, 16-, and 32-slit radial keratotomies with both different-sized clear central areas and different incisional scar widths have been produced. Five normal subjects who looked through a series of these models have had their glare sensitivity determined using variable contrast threshold targets surrounded by a constant glare source. Results show that increased glare sensitivity is present with small (less than 3 mm), clear central areas and wide (greater than or equal to 0.3 mm) incisional scars. The study suggests that finer scars, larger clear central areas, and configurations of eight and 16 slits should produce the least increase in glare sensitivity. These suggestions seem to be supported by studies performed on patients who have undergone radial keratotomy.
We have developed a simple procedure that uses a standard keratometer to determine the power of an intraocular lens that is still in its sterile package. The procedure is accurate to within +/-0.5 diopter and may be used in conjunction with any intraocular lens packaging system in which the convex side of a plano-convex intraocular lens is clearly visible through a clear, flat surface.
To study the effect of bronchoconstriction on the activity of pulmonary stretch receptors (PSRs), acetylcholine aerosols (0.05% solution) were delivered continuously into the lungs while the afferent activity of a single PSR was recorded from a filament of the vagus nerve. The relationship between the PSR frequency (fPSR) and the transpulmonary pressure (Ptp) was examined during both constant volume ventilation and hyperinflation. During bronchoconstriction, the peak fPSR for the same tidal volume increased significantly (P less than 0.05) compared to the control response obtained with saline aerosols. However, the fPSR at functional residual capacity decreased in the receptors above the carina but increased in those below. Bronchoconstriction induced a hysteresis in the dynamic Ptp-fPSR relationship during hyperinflation in 11 out of the 21 receptors studied: a clockwise hysteresis was found in those receptors above the carina whereas a counterclockwise one in those below. Results of these studies suggest that the response of PSRs to bronchoconstriction depends on their locations in the tracheobronchial tree.
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The use of ketamine in patients with carcinoid syndrome is controversial because of the catecholamine response to this agent. Prior administration of diazepam modifies the catecholamine response to ketamine and may make its use in such patients safe. The successful use of a diazepam-ketamine technique for anaesthesia of a patient with malignant carcinoid syndrome and associated tricuspid valve disease who underwent laparotomy and hepatic artery ligation is described. The theoretical advantages of safe ketamine anaesthesia in carcinoid patients are discussed and the relevant literature reviewed. To the authors' knowledge the successful use of ketamine anaesthesia in carcinoid syndrome has not previously been reported.
An in-house library system based on a dedicated mini-computer has been in operation in the Israel National Medical Library since the summer of 1979. The integrated system, called MAIMON, features on-line access to bibliographic and circulation records. It replaces manual procedures in cataloging, searching, lending, and reservations. The system provides previously unavailable statistics on items in heavy use and demand, items to be removed from the active collection, and who uses what in the library. It is designed to be user cordial and to save users' time. The system has been very favorably accepted by patrons, and frees professional librarians from time-consuming clerical routine tasks. The system is evaluated in terms of performance, convenience, and cost.
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A system is described in which a SX-70, Alpha Model II, Polaroid, single lens reflex camera, and photoflash unit can be quickly and sturdily mounted to a slit lamp. Such a system can take sharp anterior segment prints of the eye at all slit-lamp magnifications, with white light and in the flourescein mode. The heart of the unit is the modification of an assembly initially designed to attach the camera to a light microscope. The small flash unit and power supply were designed specifically for slit lamp usage. Examples of "instant" photos of the anterior segment at different magnifications are presented.