Hypophosphatemic rickets and breast milk.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to J Fisher.
Explore the source record for details and available documents.
Four adolescents with Hodgkin's disease also developed autoimmune diseases. There were two idiopathic thrombocytopenic purpura (ITP), one polymyositis, and one scleroderma. The first two patients developed ITP in the absence of a spleen, and with their Hodgkin's disease in remission. The first patient with Hodgkin's disease has been continuously free of cancer for over five years. The second patient was a 17-year-old male whoe Hodgkin's disease recurred, but whose disease was in remission at the time the ITP occurred. The polymyositis occurred in an 18-year-old youth when he was in his initial remission for his Hodgkin's disease, but his disease subsequently recurred two years later. This youth presented with Coombs positive autoimmune hemolytic anemia. The polymyositis did not respond to therapy, and he is left with severe muscle wasting and weakness; however, the polymyositis is now quiescent. The scleroderma occurred in an 18-year-old female who had been continuously free of Hodgkin's disease for eight years. The scleroderma did not respond to drug therapy and she now has moderate skin changes, but remains in continuous remission of her Hodgkin's disease. Although there are a few reports of Hodgkin's disease and concurrent autoimmune disorders, physicians dealing with cancer in adolescents should be aware of this association.
The effects of doxapram infusion (0.25 mg.kg-1. min-1) were studied in cats anaesthetized with pentobarbitone (35 mg . kg-1 intraperitoneally). Cats were studied breathing 50 per cent oxygen and the responses to two concentrations of inspired carbon dioxide were measured. Doxapram infusion increased pulmonary ventilation by increasing both tidal volume and respiratory frequency, and also caused increases in the volume inspired in the first 0.5 second after the onset of an inspiration (V0.5) and the pressure generated in the airway 0.5 second after the onset of an inspiration when the airway had been occluded (P degrees 0.5). V 0.5, P degrees 0.5 and the mean inspiratory flow rate (VT/VI) were essentially equivalent indices of inspiratory drive. Doxapram infusion did not alter the effective impedance of the respiratory system (P degrees 0.5/V 0.5). Doxapram infusion increased the ventilatory response to carbon dioxide. The slope of the ventilatory response to carbon dioxide was increased and the response line was shifted to the left. We conclude that the increase in pulmonary ventilation caused by doxapram infusion is due almost entirely to increased inspiratory neuromuscular drive (P degrees 0.5).
Explore the source record for details and available documents.
Two methods for quality control of automated blood counters have been compared: (1) monitoring results from patients' samples, and (2) analysis of results of a stable whole-blood control. The latter method proved better able to distinguish calibration changes from patient variation.
Sarcoidosis has been characterized by a variety of clinical presentations ranging from complete lack of symptoms to incapacitating multisystem disease. Bilateral thoracic lymph node enlargement with or without pulmonary disease is the most common presentation. An elderly woman with eye pain, visual disturbance, nonproductive cough, and weight loss was found to have unilateral hilar enlargement on roentgenographic examination of the chest. An aggressive search for a neoplastic process disclosed only noncaseating granulomas consistent with sarcoidosis. The patient completely recovered on corticosteroid therapy alone.
Fungus balls of the urinary tract are rare and usually associated with infection by Candid albicans. Since 1968 five patients seen at the Medical College of Virginia Hospitals presented with this peculiar manifestation of candidiasis. Summaries of their epidemiologic clinical, pathologic, and mycologic data are presented. All Candida fungus balls involved the upper collecting system and were detected by radiography and confirmed by culture and/or pathologic section. Two of the five patients completely recovered. Three patients were treated with flucytosine and/or local irrigation with a polyene antifungal agent. Two recovered and the third died of probable bacterial sepsis. One patient was treated successfully with surgical removal of the fungus ball and a brief period of local irrigation with amphotericin B (AMB). The fifth patient recovered after 28 days of parenteral AMB. Predisposing factors and pathogenetic mechanisms are discussed, and a rational approach to therapy is outlined.
Explore the source record for details and available documents.
The design of a simple calibrator for checking the performance of respiratory measuring equipment is described. It is particularly suitable for calibrating the equipment used for obtaining flow/volume curves.
Investigation of a series of falls from hospital beds showed that the cause was the poor design of the particular mattresses. Interior sprung mattresses of this construction are unstable at their edges and are particularly hazardous when used with the modern, rigid base hospital bed.
Explore the source record for details and available documents.
Nutritional, racial, cultural, and environmental factors have combined to produce a resurgence of vitamin D deficiency rickets in urban Philadelphia. Between January 1974 and June 1978, 24 cases were diagnosed at the Children's Hospital of Philadelphia. Patients' ages ranged from 4 to 58 months. Presenting complaints included seizures, swollen wrists, pathologic fractures, and developmental regression. Sixteen patients were below the third percentile for length and weight. Laboratory results indicated vitamin D deficiency in nursing mothers as well as in infants. All infants had been breast-fed and all were black. Ingestion of vitamin D was limited by exclusion of meat and/or dairy products in 21, and no infants had consistently taken supplemental vitamins. Nineteen were members of Muslim or Seventh Day Adventist faiths. Endogenous synthesis of vitamin D was limited by dark skin, by dressing in long garments with hoods and veils, and by air pollution in a densely populated northern city. The return to a more "natural" diet, free of food additives, has been accompanied by the return of a classic disease of industrial society. Effective management required patience and respect for religious convictions. With treatment, there was correction of chemical and skeletal abnormalities, but few patients showed catch-up growth.
The kinetic details of the irreversible inactivation of the Escherichia coli RTEM beta-lactamase by clavulanic acid have been elucidated. Clavulanate is destroyed by the enzyme and simultaneously inhibits it by producing two catalytically inactive forms. One of these is transiently stable and decomposes to free enzyme (k = 3.8 X 10(-3) S-1), while the other corresponds to an irreversibly inactivated form. The transient complex is formed from the Michaelis complex at a rate (k approximately 3 X 10(-2) S-1) which is some threefold faster than the rate of formation of the irreversibly inactivated complex. The transient complex is, therefore, the principle enzyme form present after short time periods. In the presence of excess clavulanate, however, all the enzyme accumulates into the irreversibly inactivated form. The number of clavulanate turnovers that occur prior to complete enzyme inactivation is 115.
Incubation of clavulanic acid with the beta-lactamase from Escherichia coli RTEM leads to enzyme-catalyzed depletion of clavulanic acid, to transient inhibition, and to irreversible inactivation of the enzyme. Both the transiently inhibited and the irreversibly inactivated species show a marked increase in the absorbance at 281 nm that is proportional to the decrease in enzyme activity. Hydroxylamine treatment of irreversibly inactivated enzyme restores about one-third of the catalytic activity, with a concomitant decrease in absorbance at 281 nm. Polyacrylamide isoelectric focusing of the irreversibly inactivated enzyme shows three bands of approximately equal intensity, different from native enzyme. Upon hydroxylamine treatment, one of the three bands disappears and now focuses identically with native enzyme. It is evident that the irreversible inactivation of enzyme by an excess of clavulanic acid generates three products, one of which can be reactivated by hydroxylamine.
The chemical and enzymatic properties of 26 analogues of riboflavin are presented. These analogues include both endo- and exocyclically substituted isoalloxazines with redox potentials from -370 to -128 mV. Physical and chemical data such as the electronic absorption spectra, pKas, and redox potentials of the analogues are presented and are discussed with respect to preferred tautomeric and resonance forms. Like riboflavin, most of the analogues are shown to be catalytic oxidants of dihydro-5-deazaflavins. Analogue binding to egg white binding apoprotein has been quantitated and serves to determine the origins of binding site specificity for this protein. Nearly all of the analogues that possess D-ribityl groups are found to be processed to the FAD level by the flavokinase/FAD synthetase system of Brevibacterium ammoniagenes. Most extensively studied are the reactivities of the analogues with the NAD(P)H:flavin oxidoreductase of Beneckea harveyi. Many of the analogues are substrates in this enzymatic redox reaction, and a linear free energy-rate relation (log Vmax vs. E0' of the analogue) is seen that parallels similar relationships in the nonenzymatic oxidation of dihydro-5-deazaflavins. This suggests a common mechanism for the reactions of such diverse flavins as riboflavin, 5-deazariboflavin, and 1-deazariboflavin.
The white cell count, red cell count, haemoglobin, and mean cell volume of a new preserved whole blood preparations used as a control material for the Coulter Model S were found to be stable for at least two months. This material provides a cheap and stable whole blood control which is now in routine use in 18 laboratories throughout Wales.
The clinical and pathology findings in 2 sibs with xeroderma pigmentosum (XDP), complicated by metastatic malignant melanoma which underwent spontaneous regression, are described. The pathology of one of these patients showed features of possible spontaneous regression, namely foamy histiocytes, capillary proliferation, and a chronic inflammatory infiltrate which was devoid of malignant cells, suggesting the possibility that an immunological mechanism was at work. It was of interest that a recent review of 27 cases of spontaneous regression of metastatic melanoma since 1900 contained a patient with XDP. Adding our 2 cases, at least 10% of the spontaneous regressions of metastatic melanoma occurred in patients with XDP. This unusual association raised the question that the genotype for XDP may possibly foster control of metastatic malignant melanoma in some as yet unknown way.
Three cases of spontaneous umbilical hernia rupture in patients with alcoholic liver disease and ascites are reported. Eighteen cases have been previously reported in the literature. These 21 cases are reviewed in an attempt to determine precipitating events, prognostic factors, complications, and the results of therapy. All patients had significant ascites prior to umbilical rupture. Ulceration of the umbilicus prior to rupture was common (81%). The subsequent presence of peritonitis, hypotension, renal failure, gastrointestinal hemorrhage or hepatic coma was associated with significant mortality (80%). All patients with a serum albumin above 2.4 gm./dl. survived. There were no survivors in those patients who did not receive surgical treatment. Based upon the outcome of these 21 patients optimal treatment of this disorder would seem to be early surgical repair of the umbilical hernia, appropriate fluid and electrolyte replacement, antiobiotics and measures to reduce intraabdominal pressure.