The role of hepatobiliary scintigraphy in cystic fibrosis.

This was a prospective open study that examined the quantitative and qualitative analysis of hepatobiliary scintigraphy (DISIDA) in detecting liver involvement in cystic fibrosis (CF). Forty-four adult and pediatric patients (median age, 12.1 years; range, 1.1-36.3 years) were divided into three groups: group 1, no evidence of liver involvement (n = 8); group 2, biochemical evidence of liver involvement on two or more occasions (n = 26); and group 3, clinical evidence of liver disease (n = 10). In groups 1 and 2, the most common qualitative scintigraphic finding was focal intrahepatic retention of tracer (26/34 patients, 12 of whom had normal findings on ultrasonography). This finding corresponds to focal cholestasis and may warrant treatment with the choleretic agent ursodeoxycholic acid (UDCA). In the group 3 patients, the abnormal qualitative scintigraphic appearances (heterogeneous uptake of tracer and nodular liver outline) added little to the findings on ultrasonography; however, these patients had a prolonged mean hepatic clearance time compared with those in groups 1 and 2 (one-way ANOVA; P < .015). It is proposed that scintigraphy with DISIDA has a role in the detection of early liver involvement in cystic fibrosis.

Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial.

OBJECTIVE: To assess the benefit of nebulised amiloride added to the standard inpatient treatment of a respiratory exacerbation in cystic fibrosis. DESIGN: Prospective, randomised, double blind, placebo controlled trial. SUBJECTS: 27 cystic fibrosis patients (mean age 12.8 years). SETTING: Two hospitals in Leeds, UK. RESULTS: Both forced expiratory volume in one second (FEV1) and forced vital capacity (FVC) showed improvements over the course of treatment, although there was no difference in respiratory function between the two groups at any of three time periods during the study. The time to reach peak FVC was significantly reduced in the amiloride group (4.2 v 7.6 days; 95% CI 0.4 to 6.4 days), but not in the time to reach peak FEV1 (5.7 v 7.9 days; 95% CI -1.2 to 5.6 days). CONCLUSIONS: Amiloride did not result in a greater overall improvement in respiratory function. There was a suggestion that it may have an effect on the rate of improvement, and thus may possibly influence the duration of treatment. This hypothesis deserves further evaluation.

Resting energy expenditure and substrate oxidation rates in cystic fibrosis.

The resting energy expenditure (REE) and substrate oxidation rates in 16 patients with cystic fibrosis who had mild chest disease and 11 healthy controls were measured using indirect calorimetry. The mean REE (% predicted) in the patients with cystic fibrosis was 11% greater than in the controls. Five patients with cystic fibrosis were hypermetabolic but only one of these had a clinically significant reduction of respiratory function. A greater proportion of REE was derived from carbohydrate oxidation in the cystic fibrosis patients (43.5% v 29.9%). However, the 24 hour dietary intake of carbohydrate was greater in the cystic fibrosis group (49.6 v 45.8% of energy intake). These data suggest that a high dietary intake of carbohydrate may contribute to the increased oxidation of carbohydrate in these cystic fibrosis patients. All patients with cystic fibrosis, including those with apparently mild lung disease, should continue to receive a high energy diet.

Stereotypes of women of Asian descent in midwifery: some evidence.

The subject of this paper is part of a larger study which investigated the delivery of maternity care to women of South Asian descent in Britain (Bowler, 1990). An ethnographic approach was used and the main method of data collection was non-participant observation in antenatal clinics, labour and postnatal wards in a teaching hospital maternity unit. These observations were supported by data from interviews with midwives. It was found that the midwives commonly use stereotypes of women in order to help them to provide care. These stereotypes are particularly likely to be used in situations where the midwife has difficulty (through pressure of time or other circumstances) in getting to know an individual woman. The stereotype of women of Asian descent contained four main themes: communication problems; failure to comply with care and service abuse; making a fuss about nothing; a lack of normal maternal instinct. Reasons for stereotyping are explored. Effects on service provision in the areas of family planning and breast feeding are highlighted.

PIP: An ethnographic study of the delivery of maternity care in Britain to women of South Asian descent (predominantly Pakistani or Bangladesh) revealed widespread stereotyping on the part of midwives. Midwives tended to view Asian patients as a homogeneous group, uniformly deviating from the norms of maternal an patient behavior. The fieldwork, which was carried out in a teaching hospital in Southern England in 1988, involved interviews with 25 midwives and non-participant observation in prenatal clinics, labor, and postpartum wards. The stereotyping of Asian patients was organized around 4 themes: communication difficulties, lack of compliance with care and service abuse, a tendency to make a "fuss about nothing." and lack of maternal instinct. Unable to form a personal relationship with Asian patients who spoke little or no English, midwives characterized these women as unresponsive or unintelligent; in other cases, a lack of understanding of colloquial language led midwives to consider these patients rude. Midwives were critical of the large family size of Asian women, yet did not make an effort to suggest family planning due to their assumption there was no interest. These patients were viewed as attention seeking during labor and had a low pain threshold, yet they were not offered pain control. The refusal of the Asian mothers to breastfeed after delivery reflected a cultural norm of waiting for the milk to come in, but was interpreted by midwives as a lack of normal maternal instinct. The midwives appeared to use stereotyping to cope with their feelings of anxiety and ineffectiveness in working with a population whose language and culture interfered with standard norms of care. Given evidence that these stereotypes are negatively affecting the health care received by Asian women in Great Britain, cultural awareness training is recommended.

A double blind lipase for lipase comparison of a high lipase and standard pancreatic enzyme preparation in cystic fibrosis.

A standard acid resistant microsphere pancreatic enzyme preparation was compared with identical capsules half filled with mini-tablets of a new high lipase preparation in a randomised double blind crossover study in children with cystic fibrosis. Each patient received his/her usual number of capsules and the same dose of lipase during each period of the study. Eighteen patients completed the study. There were fewer gastrointestinal symptoms when pancreatic enzyme was supplied as the high lipase preparation. There was also a significant improvement in fat absorption (17%, 95% confidence interval (CI) 6 to 27), reduction in faecal fat output (15.8 g/day, 95% CI 6.4 to 22.5), and faecal energy loss (789 kJ/day, 95% CI 211 to 1384). It is concluded that half filled capsules of the new high lipase preparation are more effective than the standard preparation and it is likely that filled capsules would allow patients to use fewer than half the number of pancreatic enzyme capsules.

Cystic fibrosis in Asians.

The clinical course of cystic fibrosis in nine Pakistani Asians was compared with 18 non-Asian age and sex matched controls. The Asian patients grew Pseudomonas aeruginosa at an earlier age (4.0 v 7.5 years), tended to have lower respiratory function test results (forced vital capacity 58.5 v 76.8% predicted; forced expiratory volume in one second 79.8 v 100.3% predicted), and had significantly greater concentrations of immunoglobulin IgG (13.4 v 10.1 g/l). They had a lower weight for age (78.4 v 95.7%) and weight for height (90 v 98.5%) despite similar intakes of dietary energy. Four of the nine Asians carried the delta F508 mutation compared with 17 of 18 controls. All the Asian patients were born in the UK; seven of their mothers were born in Pakistan and had moderate or severe difficulties with the English language. It is concluded that Asian patients may have a more severe clinical course than matched controls and that genetic and environmental factors may be contributory.