XAFS at the Pacific Northwest Consortium-Collaborative Access Team undulator beamline.

The Pacific Northwest Consortium-Collaborative Access Team (PNC-CAT) has begun operating an insertion device beamline at the Advanced Photon Source. The beamline has been extensively used for XAFS studies. This paper summarizes its capabilities, and our initial operational experience. The beamline is based on APS undulator A, and incorporates full undulator scanning. The monochromator is liquid nitrogen cooled and has both Si(111) and Si(311) crystals in a side-by-side configuration. Crystal changes only take a few minutes. The crystals cover the energy range from 3-50 keV with fluxes as high as 2x10(13) ph/sec. Microbeams can be produced using Kirkpatrick-Baez mirrors (spot size 1-3 microm) or tapered capillaries (sub-microm spots). When these optics are combined with a 13-element Ge detector, the beamline provides powerful microbeam imaging and spectroscopy capabilities. Experimental examples from the environmental field and in-situ UHV film growth will be discussed.

Norrie's disease in a French-Canadian kindred: attempt to detect carriers by DNA analysis.

In a French-Canadian kindred four male cousins are affected with Norrie's disease, a rare X-linked recessive disorder. Three have university education, and the fourth has some developmental delay. Only one is microcephalic. All have mild to severe hearing deficit, although only three were aware of their hearing loss. Linkage analysis of DNA from family members with the probe L1.28 failed to detect female carriers.

Effect of hypobaric oxygen and oleic acid on respiration of Staphylococcus aureus.

The effect of hypobaric oxygen, with oleic acid in the nutrient substrate, on respiration and slime production by a pulmonary isolate of Staphylococcus aureus was investigated. Under hypobaric, but not normal oxygen pressure, the addition of oleic acid to the nutrient broth caused the bacteria to drastically diminish their demand for oxygen and initiate the secretion of extrapolymeric substances (slime). The decrease in oxygen demand was found to result from impairment of the capacity to reduce and oxidize the coenzyme NAD. Prior to the initiation of slime production, the rate of oxidation exceeded the rate of reduction of the coenzyme, whereas with slime production the rate of reduction was greatest. This could result in elevation of the cellular NADH, which could stimulate gluconeogenesis and thereby increase the synthesis of the carbohydrate component of the slime. The results suggest that staphylococcal infections, such as those of the pulmonary tract in cystic fibrosis and essential fatty acid deficiency, may occur in response to a peculiar chemical environment.

In vitro response of Staphylococcus aureus from cystic fibrosis patients to combinations of linoleic and oleic acids added to nutrient medium.

The effect of supplementing nutrient substrate with various combinations of concentrations of oleic and linoleic acids on the growth of 11 strains of Staphylococcus aureus was assessed. Whereas increasing the concentration of linoleic acid by itself greatly diminished the growth of all 11 strains, concomitant increases in oleic acid greatly diminished the inhibitory effect of linoleic acid. With oleic acid in the nutrient substrate, most of the strains were induced to produce slime which surrounded the cells. Since the slime incorporated oleic but not linoleic acid, such slime production isolated the cells from direct contact with the growth inhibitor, linoleic acid.

Cor pulmonale in cystic fibrosis.

A VCG and an orthogonal ECG were done on 66 ambulatory patients with cystic fibrosis. Arteriolized pO2, FEV1/VC and MBC were related to electrocardiographic measurements. In this group of adolescent and young adult patients the most helpful electrocardiographic sign of cor pulmonale is a low voltage representing the left ventricle. The four best indicators from the cardiogram are (1) Rx less than .8 mv; (2) Rx/Sx less than 4.0; (3) Tx less than .4 mv; and (4) MLVF + MLVH less than 2.5 mv. If more than one indicator is found cor pulmonale is likely present. Increased voltage over the right ventricle does not identify patients with cor pulmonale.

Intrapancreatic common bile duct compression causing jaundice in an adult with cystic fibrosis.

A 29-yr-old man with cystic fibrosis had a 4-yr history of recurrent episodes of obstructive jaundice. Endoscopic retrograde cholangiography revealed a common bile duct smoothly narrowed in its intrapancreatic portion, with dilatation above. A choledochojejunostomy was performed, but the patient died of septic complications. At autopsy, extensive pancreatic fibrosis caused compression of the distal bile duct in a manner which is analogous to that seen in chronic pancreatitis. This complication of cystic fibrosis, not previously reported, may become more prevalent as more patients with cystic fibrosis are living into adulthood.

Immunoassay of serum conjugates of cholic acid in cystic fibrosis.

Pre- and post-prandial serum conjugates of cholic acid (SCCA) were measured by radioimmunoassay (RIA) in 83 patients with cystic fibrosis (CF), 14 of whom did not have steatorrhoea, and in 25 controls. Of the CF patients with steatorrhoea, 38% had fasting SCCA levels greater than 3 standard deviations above mean fasting control values, whereas no CF patient without steatorrhoea had elevated fasting SCCA levels. Steatorrhoeic patients with palpable livers had higher pre- and post-prandial SCCA levels. Post-prandial SCCA levels failed to discriminate between control and CF groups however. Other serum tests of liver function, including the aspartate amino transferase, alkaline phosphatase, albumin, gamma globulin, and albumin : globulin ratio, failed to correlate with the SCCA. Changes in serum protein constituents correlated strongly with pulmonary dysfunction. The results suggest that elevation of fasting SCCA levels in CF patients is a more sensitive indicator of liver dysfunction than other tests and is a better discriminator than post-prandial SCCA levels between normal and abnormal liver function. The test is recommended for early detection of liver dysfunction in CF patients.

Ventilatory muscle endurance training in normal subjects and patients with cystic fibrosis.

Ventilatory muscles can become fatigued, and this can contribute to respiratory failure. Patients with chronic obstructive lung disease may benefit from improving their ventilatory muscle endurance to improve resistance to fatigue. Ventilatory muscle endurance was measured in 30 normal subjects and 55 patients with cystic fibrosis by finding the highest level of normocapnic hyperpnea that could be sustained for 15 min. Subjects with cystic fibrosis had 36 per cent higher ventilatory muscle endurance than normal subjects, reflecting the chronic training stress of breathing against increased respiratory loads. Four normal subjects and 4 subjects with cystic fibrosis participated in a specific ventilatory muscle endurance training program consisting of 25 min per day of maximal normocapnic hyperpnea 5 days per week for 4 weeks. The cystic fibrosis patients who trained improved their ventilatory muscle endurance by 51.6 per cent, whereas the normal subjects who trained showed a 22.1 per cent increase in ventilatory muscle endurance. Seven subjects with cystic fibrosis participated in a 4-week physical activity training program consisting of at least 1.5 hours per day of intensive swimming and canoeing at summer camp. They increased their ventilatory muscle endurance by 56.7 per cent. There were no other pulmonary function changes. Ventilatory muscle endurance can be readily improved in cystic fibrosis equally well by specific ventilatory muscle endurance exercise.