Studies of the rectal mucosa in coeliac sprue: the intraepithelial lymphocyte.

The dynamics of the rectal surface epithelial lymphocyte and leucocyte response to wheat, gluten, and gliadin enema challenges in control individuals and in patients with coeliac sprue in remission is shown. There is a clear increase in intraepithelial lymphocytes and polymorphonuclear (PMN) leucocytes in response to these enemas in coeliac sprue, but not in controls. The peak response was at eight hours and cleared within 24 hours. There was no change in the crypt epithelium. These data add further support to the role of wheat, gluten, and gliadin in the pathogenesis of coeliac sprue, at least in the rectal mucosa.     

The cytoskeleton in Chediak-Higashi syndrome fibroblasts

The Chediak-Higashi syndrome (CHS) trait is expressed in cultured human skin fibroblasts as an abnormal perinuclear concentration of moderately enlarged lysosomes. The cytoskeleton of CHS fibroblasts appears intact. Microtubules are normal in number and morphology, as assessed by colchicine binding studies, antitubulin immunofluorescence, and electron microscopy. Deformability by shear force is unaltered and microfilaments are abundant. However, CHS lysosomes appear to interact abnormally with the cytoskeleton, since the perinculear aggregation partially disperses after depolymerization of cell microtubules with colchicine. These results suggest that CHS is associated with a defect of either the lysosomal membrane itself or of lysosomal membrane- microtubule interaction.     

Specific Unwillingness to Donate Eyes: The Impact of Disfigurement,Knowledge and Procurement on Corneal Donation

Although willingness, attitudes and beliefs surrounding solid‐organ donation have been extensively investigated, much less is known about corneal donation. Despite evidence that a substantial number of families who agree to multiorgan donation also specifically refuse corneal donation, it is unclear why this occurs and what can be done to increase rates of corneal donation. We conducted a survey of 371 Australian adults regarding their views on corneal donation. Although willingness to donate corneas generally reflected a person's willingness to donate all of one's organs, unwillingness to donate corneas appeared to be due to other factors. Specifically, decisions not to donate appear to be driven by a range of concerns surrounding disfigurement. The survey also provides eye banks with reassurance about the acceptability of whole globe procurement, and recognition that research into blindness is a highly valued part of corneal donation. Finally, the survey identifies that many individuals see benefit in having their family engaged in the decision‐making process, suggesting that decisions about donation are more complex than a simple appeal to the autonomy of the deceased.     

The impact of end-of-life curriculum content on the attitudes of associate degree nursing students toward death and care of the dying

Because end-of-life care competes with other nursing content for a place in the curriculum, nurse educators are challenged to select effective teaching strategies to prepare graduates to care for dying patients. An elective course using the End-of-Life Nursing Education Consortium curriculum was compared with a 3-hour lecture embedded in a larger medical–surgical course. Results suggested that both content-delivery methods positively impacted the attitude of students toward death and care of the dying to some extent; however, significant positive changes were noted in both intervention groups and in the control group that viewed the film Wit.     

Visual impairment in severe and profound sensorineural deafness

The frequency of reversible and irreversible visual impairment was determined in children with severe and profound sensorineural deafness, as subnormal vision can adversely affect their educational and social development. Eighty three of 87 such children attending an audiology service were examined to assess the incidence and severity of visual impairment. Each child underwent a detailed ophthalmic assessment. The criteria for visual impairment were visual acuity < 6/9 Snellen or equivalent and/or abnormal binocular vision. Forty five had a normal ophthalmic examination (54.2%). Twenty nine had visual impairment (34.9%) and nine had ophthalmological abnormalities that did not interfere with vision (10.9%). A higher proportion of children with risk factors for visual pathology demonstrated visual impairment than those in whom there were no risk factors. None the less, 44% of visual impairment was among patients without risk factors. The results underline the need to examine all children with severe and profound sensorineural deafness soon after diagnosis and indicate that children with multiple handicaps have a greater likelihood of visual impairment (11 of 14 cases).     

Worldwide experience in newborn screening for classical congenital adrenal hyperplasia due to 21-hydroxylase deficiency

The need for a reliable screening test for classical congenital adrenal hyperplasia prompted development of newborn screening programs. Worldwide incidence of classical congenital adrenal hyperplasia in this report was taken from newborn screening programs in France, Italy, Japan, New Zealand, Scotland, and the United States. Two populations in which the occurrence of congenital adrenal hyperplasia among live births has been reported with greater than usual frequency are the Yupik Eskimos of southwestern Alaska (1:282) and the people of La Reunion, France (1:2,141). Aside from these populations, 1,093,310 newborns were screened between 1980 and 1988, of whom 77 had congenital adrenal hyperplasia. Thus, worldwide incidence of this disorder was estimated at 1:14,199 live births for homozygous patients, 1:60 for heterozygous subjects, with a gene frequency of 0.0083. Incidence of congenital adrenal hyperplasia among whites was estimated to be 1:11,909 (41:488,279) for homozygous patients, 1:55 for heterozygous subjects with a gene frequency of 0.0091. Incidence for the salt-wasting form of congenital adrenal hyperplasia was 1:18,850 (58:1,093,310) compared with 1:57,543 (19:1,093,310) for congenital adrenal hyperplasia in the simple virilizing form. Thus, salt-wasting congenital adrenal hyperplasia was three times more common than simple virilizing congenital adrenal hyperplasia. Estimated incidence of congenital adrenal hyperplasia in white populations in Italy and France (1:10,866) was higher than in Scotland (1:17,098), New Zealand (1:14,500). The incidence in an Asian population (Japan) (1:15,800) did not differ significantly from that of the white population. In four of five populations, overall incidence was higher than previously reported, as was the frequency of the salt-wasting form (75% v 50% to 66%), suggesting improved case detection by newborn screening.(ABSTRACT TRUNCATED AT 250 WORDS)     

The negative impact of the repeal of the Arkansas motorcycle helmet law

BACKGROUND: On July 1, 1997, Arkansas became the first state in 14 years to repeal their adult helmet law. We examined the clinical and financial impact of this repeal. METHODS: A 6-year retrospective review was conducted of the University of Arkansas for Medical Sciences trauma registry including the 3 years before and the 3 years after the repeal of the helmet law. A head and neck Abbreviated Injury Scale (AIS) score >or= 3 was considered severe. All patients admitted to the hospital or who died in the emergency department were included in the study. The database of the Arkansas Highway and Transportation Department was also used to determine the number of crashes and fatalities occurring statewide (1995-1999). RESULTS: Although total and fatal crashes in Arkansas were not significantly different (1995-1996 vs. 1998-1999), nonhelmeted deaths at the scene of a crash significantly increased from 19 of 48 (39.6%) (1995-1996) to 40 of 53 (75.5%) (1998-1999) (p < 0.0001). Before repeal, 25% of nonfatal crash admissions were nonhelmeted (18 of 73). This significantly increased to 54% (52 of 96, p< 0.001) after repeal. Overall, patients who were nonhelmeted had significantly higher AIS scores for head and neck, significantly more severe head injuries (AIS score >or= 3), 47% (33 of 70) versus 20% (20 of 99), and significantly longer length of intensive care unit stay. Financially, patients without helmets had significantly higher unreimbursed charges compared with their helmeted counterparts, resulting in a total of 982,560 dollars of additional potentially lost revenue over the length of the study. CONCLUSION: Repeal of the mandatory helmet law was associated with an increase in the nonhelmeted crash scene fatality rate. After the repeal, there was a disproportionately higher admission rate for nonhelmeted motorcycle crash survivors. These patients had an increased use of hospital resources and poorer reimbursement of charges compared with their helmeted counterparts. This resulted in significantly higher unreimbursed charges. States considering repeal of their mandatory adult helmet laws should consider the potential negative financial impact on their health care system and the increased morbidity associated with nonhelmeted motorcycle riders involved in a crash.     

The composition of dietary fat directly influences glucose-stimulated insulin secretion in rats

Acute elevations of plasma free fatty acid (FFA) levels augment glucose-stimulated insulin secretion (GSIS). Prolonged elevations of FFA levels reportedly impair GSIS, but no one has previously compared GSIS after prolonged exposure to saturated or unsaturated fat. Rats received a low-fat diet (Low-Fat) or one enriched with either saturated (Lard) or unsaturated fat (Soy) for 4 weeks. Insulin responses during hyperglycemic clamps were augmented by saturated but not unsaturated fat (580 +/- 25, 325 +/- 30, and 380 +/- 50 pmol x l(-1) x min(-1) in Lard, Soy, and Low-Fat groups, respectively). Despite hyperinsulinemia, the amount of glucose infused was lower in the Lard compared with the Low-Fat group. Separate studies measured GSIS from the perfused pancreas. Without fatty acids in the perfusate, insulin output in the Lard group (135 +/- 22 ng/30 min) matched that of Low-Fat rats (115 +/- 13 ng/30 min), but exceeded that of Soy rats (80 +/- 7 ng/30 min). When FFAs in the perfusate mimicked the quantity and composition of plasma FFAs in intact animals, in vivo insulin secretory patterns were restored. Because the GSIS of rats consuming Lard diets consistently exceeded that of the Soy group, we also assessed responses after 48-h infusions of lard or soy oil. Again, lard oil exhibited greater insulinotropic potency. These data indicate that prolonged exposure to saturated fat enhances GSIS (but this does not entirely compensate for insulin resistance), whereas unsaturated fat, given in the diet or by infusion, impairs GSIS. Inferences regarding the impact of fatty acids on GSIS that are based on models using unsaturated fat may not reflect the effects of saturated fat.