Cystic fibrosis and the phenotypic expression of autosomal dominant polycystic kidney disease

Recent experiments in cultured cyst epithelial cells from kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) have shown that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is present in the apical surface of these cells and mediates chloride (Cl-) and fluid secretion in vitro. To determine whether the presence of CF with the expression of mutated CFTR proteins modifies cyst formation in ADPKD, we studied a large family with both inherited diseases. ADPKD in this family is linked to PKD1. The family is composed of 26 members; 11 members with ADPKD, 4 members with CF, and 2 members with both diseases. Renal volumes measured by computerized tomography (CT), calculated creatinine clearances, and other clinical parameters in the family members with ADPKD and CF were compared with those in the family members with ADPKD alone, as well as to a large population of patients with ADPKD. The patients with CF and ADPKD, but not the CF heterozygote carriers with ADPKD, had less severe polycystic kidney and liver disease, as indicated by normal renal function; smaller renal volume, even when corrected for height and body surface area; and the absence of hypertension and liver cysts. These observations suggest that the coexistence of CF may reduce the severity of ADPKD.     

HIV/AIDS infection control enforcement: a comparison between Nigeria and the United States

We examined differences in approaches to HIV-related infection control practices in two university teaching hospitals in the United States and Nigeria. Health care workers (n = 202 in Nigeria and 186 in the USA) responded to a previously validated measure of infection control practices. There were significant differences in the estimated probability of treating a person with HIV disease (higher in USA), and a greater probability of peer ridicule as a way of enforcing group norms on infection control in Nigeria. Peer enforcement of norms was significantly lower in the USA. In both countries, more precautions would be taken if it was known that the patient was HIV infected. Infection control practices were more likely to be followed in the USA compared with Nigeria if they were praised for this activity, if appropriate facilities (sharps containers, gloves, etc.) were nearby, and if they felt that infection control procedures were effective. These data point to the importance of normative social pressures in Nigeria and of knowing the patient is HIV infected in the USA and feeling that infection control procedures are effective ways of avoiding occupational HIV infection. The role of normative pressures and assumptions about HIV infection status as well as cues and availability of facilities for infection control appear to differ between these health care workers in Nigeria and the USA.     

Iopamidol and meglumine diatrizoate: comparison of effects on patient discomfort during aortofemoral arteriography

Iopamidol was compared with Renografin-60 (meglumine diatrizoate, Squibb) in a controlled, randomized double-blind study of 40 patients undergoing peripheral arteriography for arteriosclerotic occlusive disease to determine which agent caused less discomfort. Each patient was evaluated for objective signs of discomfort and subjective feelings of pain and heat. Monitoring was achieved by multiple physical examinations, chemical tests, electrocardiograms, and intra-arterial pressure recordings. It is concluded that iopamidol is safe and causes significantly less patient discomfort than Renografin-60.     

Solid phase red cell adherence immunoassay for anti-HIV 1: a simple, rapid, and accurate method for donor screening

In technically developed countries in which acquired immunodeficiency syndrome is a risk to the recipients of blood or tissue, it is mandatory to screen the donor for evidence of HIV (human immunodeficiency virus) infection. Current tests, based on enzyme-linked immunoassay, are time-consuming and expensive and as such are unsuitable for developing countries. We describe a second generation test using anti-human IgG coupled to red cells as the indicator of antibody having reacted with test antigen (1). The test is complete within ten minutes, simple to perform and to read and has 100% sensitivity and 99% specificity compared with Western blot. It is ideal for the rapid screening of organ donors and for the screening of blood donors where cost is a major consideration.     

Evaluation of myocardial viability with technetium-99m hexakis-2-methoxyisobutyl isonitrile and iodine-123 phenylpentadecanoic acid and single photon emission tomography

The detection of viable myocardium in infarcted regions, i.e. hibernating myocardium, is a major goal in clinical cardiology today. We applied combined planar and single photon emission tomography (SPET) to the non-invasive estimation of the left ventricular ejection fraction (LVEF), myocardial blood flow and free fatty acid uptake in the heart. Of the 31 patients with coronary artery disease, 25 (81%) had had a previous myocardial infarction. All patients had at least one persistent perfusion defect on the stress-rest technetium-99m hexakis-2-methoxyisobutyl isonitrile (Sestamibi) study, and the results revealed 57/124 (46%) persistent perfusion defects. As a part of the perfusion study, the LVEF was measured at rest using the first-pass ^99mTc-Sestamibi injection, and the mean LVEF was 47% ±9% (mean ± 1 standard deviation). Iodine-123 phenylpentadecanoic acid (¹²³I-pPPA) imaging at rest was performed within 2 weeks from the perfusion study. Then 6-mm transaxial, sagittal and coronal slices of the perfusion and ¹²³I-pPPA studies were reconstructed. The bull's eye displays of the coronal slices were visually surveyed and divided into 4 quadrants: anterior, lateral, posterior and septal. The following image score was used: 0 = fixed defect, 1 = partial uptake and 2 =normal uptake. Moreover an index of metabolic reserve (MR) was calculated by dividing the bull's eye of the ¹²³I-pPPA study by the bull's eye of resting ^99mTc-Sestamibi, and its maximum value was normalized to 100%. Fourteen segments (25%) had a normal ¹²³I-pPPA uptake with a MR value of 96% ±8%. Twenty-two segments (39%) had a partial ¹²³I-pPPA uptake with a MR of 74% ±20%, whereas 21 segments (36%) had no ¹²³I-pPPA uptake and a very low MR of 36%±34%. There was a highly significant correlation (r =0.70) between LVEF and MR. These findings suggest that it is possible to identify viable myocardium by measuring contractile function (first-pass, multiple-gated ^99mTc-Sestamibi) and myocardial perfusion (stress-rest ^99mTc-Sestamibi) and by combining these parameters with myocardial fatty acid uptake (1231-pPPA) studies. Correspondence to: IT Kuikka