Iron and CF-related anemia: expanding clinical and biochemical relationships.

INTRODUCTION: This cross-sectional study was conducted to assess the relationship between iron levels in the plasma and sputum of cystic fibrosis (CF) patients. METHODS: Demographic, clinical, and iron-related laboratory data were prospectively obtained from 25 patients with stable clinical features and 14 patients with worsened clinical features since their most recent evaluations. RESULTS: Compared to patients with stable clinical features, those who experienced clinical deterioration demonstrated significantly worse lung function and were more frequently malnourished and diabetic. Members of the latter group were also significantly more hypoferremic and had higher sputum iron content than patients with stable clinical features. No significant correlation was found between plasma and sputum iron levels when the groups were analyzed together and separately. CONCLUSIONS: Sputum iron content does not correlate with iron-related hematologic tests. Hypoferremia is common in CF and correlates with poor lung function and overall health.

Erythrocyte membrane ATP binding cassette (ABC) proteins: MRP1 and CFTR as well as CD39 (ecto-apyrase) involved in RBC ATP transport and elevated blood plasma ATP of cystic fibrosis.

In addition to the better-known roles of the erythrocyte in the transport of oxygen and carbon dioxide, the concept that the red blood cell is involved in the transport and release of ATP has been evolving (J. Luthje, Blut 59, 367, 1989; G. R. Bergfeld and T. Forrester, Cardiovasc. Res. 26, 40, 1992; M. L. Ellsworth et al., Am. J. Physiol. 269, H2155, 1995; R. S. Sprague et al., Am. J. Physiol. 275, H1726, 1998). Membrane proteins involved in the release of ATP from erythrocytes now appear to include members of the ATP binding cassette (ABC) family (C. F. Higgins, Annu. Rev. Cell Biol. 8, 67, 1992; C. F. Higgins, Cell 82, 693, 1995). In addition to defining physiologically the presence of ABC proteins in RBCs, accumulating gel electrophoretic evidence suggests that the cystic fibrosis transmembrane conductance regulator (CFTR) and the multidrug resistance-associated protein (MRP1), respectively, constitute significant proteins in the red blood cell membrane. As such, this finding makes the mature erythrocyte compartment a major mammalian repository of these important ABC proteins. Because of its relative structural simplicity and ready accessibility, the erythrocyte offers an ideal system to explore details of the physiological functions of ABC proteins. Moreover, the presence of different ABC proteins in a single membrane implies that interaction among these proteins and with other membrane proteins may be the norm and not the exception in terms of modulation of their functions.

Endoscopic intervention for biliary leaks after laparoscopic cholecystectomy: a multicenter review.

BACKGROUND: Endoscopic therapy of biliary tract leaks was uncommon before laparoscopic cholecystectomy. Studies have demonstrated the efficacy of endoscopic drainage by endoscopic sphincterotomy or stent placement. Various endoscopic therapeutic modalities and long-term follow-up of this problem were studied. METHODS: Members of the Midwest Pancreaticobiliary Group reviewed all patients referred for endoscopic therapy of biliary leaks after laparoscopic cholecystectomy from 1990 to 1994. Long-term follow-up was by direct patient contact. RESULTS: Fifty patients were referred for endoscopic therapy of biliary leaks. Abdominal pain was present in 94%. The mean time from laparoscopic cholecystectomy to referral was 6.9 days. Therapy consisted of sphincterotomy only in 6 patients, stent only in 13, and sphincterotomy with stent in 31. Biliary leaks were healed in 44 patients at a mean of 5.4 weeks. A second or third endoscopic procedure was necessary to achieve healing in five patients. Two stent-related complications were noted. Percutaneous or surgical drainage of biliary fluid collections was required in 16 patients. The mean hospital stay for treatment of the leak was 11.1 days after endoscopic therapy. On follow-up (mean 17.5 months), all patients were well except two with mild abdominal discomfort. CONCLUSIONS: Endoscopic sphincterotomy, stent placement, or sphincterotomy with stent are effective in healing biliary leaks after laparoscopic cholecystectomy. Despite prolonged treatment for the leak, patients did well on long-term follow-up.

Treatment of cystic fibrosis in adults.

More patients with cystic fibrosis are surviving into adulthood. Primary care physicians need a basic understanding of adult cystic fibrosis and the evaluation of patients with acute decompensation. Respiratory decompensation is usually the result of infective agents, including Pseudomonas aeruginosa, Stentrophomonas maltophilia and Burkholderia cepacia, and requires treatment with intravenous antibiotics. If symptoms worsen, the possibility of another complication, such as pneumothorax, hemoptysis, mycobacterial infection and allergic bronchopulmonary aspergillosis, must be considered. Family physicians can play an important role in the birth to adult care of patients with cystic fibrosis.

Skin testing with Mycobacterium avium sensitin to identify infection with M. avium complex in patients with cystic fibrosis.

We sought to determine if patients with cystic fibrosis and sputum cultures positive for Mycobacterium avium complex (MAC) have delayed-type hypersensitivity to an M. avium sensitin. Seventeen (33%) of 51 selected patients had MAC isolated from at least one sputum culture. Skin tests with purified protein derivative and M. avium sensitin demonstrated that five (10%) of 51 patients were anergic, and anergy was correlated with use of systemic steroids. Sixteen (35%) of 46 nonanergic patients had M. avium-dominant skin test reactions. Twelve (75%) of these 16 patients with cultures positive for MAC had M. avium-dominant skin tests; the specificity of skin testing was 87%. These data suggest that most patients with cystic fibrosis and sputum cultures positive for MAC have infection rather than colonization with MAC. Skin testing with M. avium sensitin is a sensitive and specific method for screening these infections.

Chronic obstructive airway disease. Impact of health education.

We conducted a controlled study of the impact of an education program on the health outcomes and perceptions of patients with varying degrees of airway obstruction in two communities. In one community, patients with chronic obstructive airway disease (COAD) were identified, assessed and offered an educational program. In the other community, patients were identified, assessed and advised of the findings only. Patients at both sites were given the same pretest and, one year later, the same posttest. These tests measured physical and social function, and health outcomes (eg, respiratory symptoms, exercise tolerance, mental health), plus patients' health perceptions. Posttest results showed a significant difference between groups on a health perception measure and locus of control, but no difference on health outcomes. We conclude that education programs for COAD are unlikely to improve patients' health status unless they are part of a comprehensive medical program that includes physical reconditioning.

Physiologic changes in rowing performance associated with training in collegiate women rowers.

To evaluate the physiologic changes in rowing performance during the training season, selected cardiorespiratory variables were measured three times at 3-month intervals in seven collegiate women rowers during incremental exercise on the rowing ergometer. Values for maximal oxygen consumption (VO2 max) and peak power production increased by 14% and 18%, respectively, over the 6-month period. Maximal heart rate was unchanged with training. Oxygen-pulse increased significantly (+ 14%) during the training season, while the ventilatory equivalent for oxygen did not change. Oxygen consumption as a percent of VO2 max and heart rate at the anaerobic threshold (AT) decreased during the first 3 months of predominantly aerobic training, but increased significantly in the last 3 months with greater anaerobic conditioning. The changes demonstrated by physiologic testing corresponded to the particular type of training emphasized during the 6-month period. Serial measurements of VO2 max and AT can be used to assess the benefits of specific training. Based on these results, individual guidelines for aerobic and anaerobic conditioning can be developed using the heart rate response at the AT.

Locating community residents with chronic airway obstruction. A comparison of four strategies.

In the course of establishing and evaluating a patient education program, we compared 4 strategies for locating patients with chronic obstructive airway disease (COAD): (1) search of hospital discharge records (HOSP), (2) referral by physicians (MD), (3) an advertising campaign (AD), and (4) a respiratory symptom questionnaire mailed to households (QUEST). Of 1,834 persons assessed, 923 (50%) had airway obstruction; 43% of the confirmed cases (396 of 923) reported no previous diagnosis of COAD. The HOSP strategy accounted for 75 assessments (4%) and 63 confirmed cases (7%), MD produced 352 assessments (19%) and 247 cases (27%), AD generated 475 assessments (26%) and 204 cases (22%), and QUEST resulted in 932 assessments (51%) and 409 cases (44%); MD was the least expensive strategy ($17.00/case). The mailed questionnaire located the largest number of cases not previously diagnosed.

Venereal transmission of hepatitis B virus. The possible role of vaginal secretions.

The presence of Type B viral (Australia antigen related serum) hepatitis in vaginal specimens was investigated and compared with that in saliva specimens in a group of patients during the acute phase of their illness. Hepatitis B surface antigen was detectable in most vaginal and salivary secretions during the phase of antigenemia and not in hepatitis B surface antigen-negative controls. Despite care in collection, occult blood was detected in the majority of specimens, but the positivity for hepatitis B surface antigen did not depend on the presence of occult blood. Recovery studies were performed in both vaginal fluids and saliva to determine if inhibitors to hepatitis B surface antigen were similar to those observed in stool samples. Significant inhibition was not observed. Although infectivity of the specimens was not established from this study, handling of these specimens as potentially infective material is recommended.

Double duodenal diaphragms in an adult: endoscopic, radiographic, and operative findings.

Hypotonic duodenography, duodenoscopy, and operative exploration were performed in an adult with double duodenal webs and symptoms suggesting gastric outlet obstruction. The spectrum of diagnostic features associated with this rare entity is described in this report.