First description of three patients with multifocal lymphangiomatosis and protein-losing enteropathy following palliation of complex congenital heart disease with total cavo-pulmonary connection.

The objective was to identify abdominal lymphatic malformations in pediatric patients with protein-losing enteropathy after palliation of complex congenital heart disease with total cavo-pulmonary connection (TCPC). In 2006, we performed complete hemodynamic and laboratory workup and thoracic and abdominal MRT screens in three patients who newly presented with symptoms of protein-losing enteropathy. All three patients, aged 3, 5, and 7 years, showed excellent TCPC hemodynamics with central venous pressures of 10-13 mm Hg. None of the patients had right-to-left overflow. All three patients showed extensive thoracic and mesenterial lymphangiomatosis. One patient died after 18 months of therapy, which included long-term parenteral nutrition, somatostatin, subcutaneous heparin injections, and frequent albumin and immunoglobulin substitution. The other two patients are in stable condition. Lymphangiomatosis might play an unknown role in the pathogenesis of protein-losing enteropathy after TCPC. It remains unclear whether lymphangiomatosis is a primary congenital disease related to the cardiac disease or if it is triggered by repeated surgery or venous congestion. The presence of lymphangiomatosis should be given diagnostic and therapeutic consideration in TCPC patients in the future.

Chronic colitis due to an epithelial barrier defect: the role of kindlin-1 isoforms.

Kindlin-1 is an epithelium-specific phosphoprotein and focal adhesion adaptor component. Mutations in the corresponding gene (KIND1) cause Kindler syndrome (KS), which is manifested by skin blistering, poikiloderma, photosensitivity and carcinogenesis. Some patients also exhibit gastrointestinal symptoms, but it has remained unclear whether these represent a feature of Kindler syndrome or a coincidence. We examined kindlin-1 in human gastrointestinal epithelia and showed that it is involved in the aetiopathology of Kindler syndrome-associated colitis. Kindlin-1 expression was assessed by indirect immunofluorescence, western blot and RT-PCR. Kindlin-1 is expressed in oral mucosa, colon and rectum. Both the full-length 74 kDa kindlin-1 protein and a 43 kDa isoform were detected in CaCo2 cells, the latter resulting from alternative splicing. In the first months of life, patients (homozygous for null mutations) had severe intestinal involvement with haemorrhagic diarrhoea and showed morphological features of severe ulcerative colitis. Later in childhood, histopathology demonstrated focal detachment of the epithelium in all segments of the colon, chronic inflammation and mucosal atrophy. These findings define an intestinal phenotype for Kindler syndrome as a consequence of a primary epithelial barrier defect. The different clinical intestinal manifestations in Kindler syndrome patients may be explained by partial functional compensation of kindlin-1 deficiency by the intestinal isoform or by the presence of truncated mutant kindlin-1.

Dieulafoy lesion in a one-year-old child.

The Dieulafoy lesion is a rare cause of severe upper gastrointestinal bleeding associated with a significant mortality. Rebleeding from undiscovered lesions is frequent and often fatal. The outcome depends on a high degree of suspicion for the condition. In any age group, the entity may be underdiagnosed rather than truly rare. It is particularly uncommon in infants. The authors report the case of the youngest patient so far to suffer from a Dieulafoy lesion.

Late onset group B streptococcal disease manifested by isolated cervical lymphadenitis.

Sepsis and meningitis are the major clinical manifestations of group B streptococcal (GBS) infections in neonates, but GBS can cause a wide spectrum of presentations ranging from asymtomatic bacteraemia to fulminate septicaemia and shock. To our knowledge this is the first report of isolated neonatal lymphadenitis as a manifestation of late onset GBS disease.

Hitting the mark in community health outreach: what counts?

This article describes a nurse-managed community health outreach project, at the Health Promotion Center (HPC) of Fairfield University School of Nursing, to prevent cardiovascular disease in an inner-city community. Flexibility in the project's grant funding has enabled the HPC to use a customer-oriented approach to develop programs. The article details the application of community health nursing and quality management practices to the development of HPC's cardiovascular-disease-screening program.

Self-rated function, self-rated health, and postmortem evidence of brain infarcts: findings from the Nun Study.

OBJECTIVES: Self-rated function is a new global measure. Previous findings suggest that self-rated function predicts future functional decline and is strongly associated with all-cause mortality. We hypothesized that the strength of the relationship of self-rated function to all-cause mortality was in part due to functional decline, such as would occur with brain infarcts. METHODS: Self-ratings of function and health (on a 5-point scale, ranging from excellent to poor) were assessed annually on 630 participants in the Nun Study. Mortality surveillance extended from October 31, 1991 to March 1, 1998, and, among those who died, neuropathological examination determined postmortem evidence of brain infarcts. Cox regression modeling with self-rated function and health as time-dependent covariates and stratification by assessment period were used in these analyses. RESULTS: Self-rated function and health ratings of good, fair, and poor were significantly associated with doubling of the risk of mortality, compared with ratings of very good and excellent. Self-rated function ratings of fair or poor were associated with a threefold increase in the risk of mortality with brain infarcts, but self-rated function and health ratings of fair and poor were comparable in their association with all-cause mortality and mortality without brain infarcts. DISCUSSION: Self-rated function was significantly associated with mortality with brain infarcts, suggesting that brain infarcts may be experienced as functional loss but not recognized or labeled as disease. Our results suggest that self-rated function and health should be explored simultaneously in future research.

Creative educational strategies for health promotion.

The dramatic changes in health care delivery present challenges to nurses in practice and to the educators who must prepare professional nurses for the uncertain world of tomorrow's managed care environment. In light of the increased emphasis on health promotion occurring with current changes in health care, the article proposes numerous creative strategies that nurse educators can use to prepare students to fulfill their health promotion responsibilities effectively. The concepts of health promotion and creativity are examined, and teaching approaches needed to facilitate community-based, health promotion-focused activities are presented. Specific strategies, particularly clinical teaching/learning opportunities, are suggested.

Brain infarction and the clinical expression of Alzheimer disease. The Nun Study.

OBJECTIVE: To determine the relationship of brain infarction to the clinical expression of Alzheimer disease (AD). DESIGN: Cognitive function and the prevalence of dementia were determined for participants in the Nun Study who later died. At autopsy, lacunar and larger brain infarcts were identified, and senile plaques and neurofibrillary tangles in the neocortex were quantitated. Participants with abundant senile plaques and some neurofibrillary tangles in the neocortex were classified as having met the neuropathologic criteria for AD. SETTING: Convents in the Midwestern, Eastern, and Southern United States. PARTICIPANTS: A total of 102 college-educated women aged 76 to 100 years. MAIN OUTCOME MEASURES: Cognitive function assessed by standard tests and dementia and AD assessed by clinical and neuropathologic criteria. RESULTS: Among 61 participants who met the neuropathologic criteria for AD, those with brain infarcts had poorer cognitive function and a higher prevalence of dementia than those without infarcts. Participants with lacunar infarcts in the basal ganglia, thalamus, or deep white matter had an especially high prevalence of dementia, compared with those without infarcts (the odds ratio [OR] for dementia was 20.7, 95% confidence interval [95% CI], 1.5-288.0). Fewer neuropathologic lesions of AD appeared to result in dementia in those with lacunar infarcts in the basal ganglia, thalamus, or deep white matter than in those without infarcts. In contrast, among 41 participants who did not meet the neuropathologic criteria for AD, brain infarcts were only weakly associated with poor cognitive function and dementia. Among all 102 participants, atherosclerosis of the circle of Willis was strongly associated with lacunar and large brain infarcts. CONCLUSION: These findings suggest that cerebrovascular disease may play an important role in determining the presence and severity of the clinical symptoms of AD.

The relationship of self-rated function and self-rated health to concurrent functional ability, functional decline, and mortality: findings from the Nun Study.

We investigated the relationship of self-rated function (i.e., the ability to take care of oneself) and self-rated health to concurrent functional ability, functional decline, and mortality in participants in the Nun Study, a longitudinal study of aging and Alzheimer's disease. A total of 629 of the 678 study participants self-rated their function and health and completed an initial functional assessment in 1991-93. Survivors completed a second assessment in 1993-94. Overall, self-rated function had a stronger relationship to functional ability at the first assessment and to functional decline between the first and second assessments than did self-rated health. Self-rated function also had a stronger relationship to mortality than did self-rated health. Self-rated function may be a better marker of global function than is self-rated health and may be a useful addition to clinical assessment and scientific investigation of the relationships among function, health, and disease.

The loss of independence in activities of daily living: the role of low normal cognitive function in elderly nuns.

OBJECTIVES: This study investigated the role of low normal cognitive function in the subsequent loss of independence in activities of daily living. METHODS: Of the 678 elderly nuns who-completed cognitive and physical function assessments in 1992/93, 575 were reassessed in 1993/94. Mini-Mental State Examination scores were divided into three categories and related to loss of independence in six activities of daily living. RESULTS: Participants with low normal cognitive function at first assessment had twice the risk of losing independence in three activities of daily living by second assessment relative to those with high normal cognitive function. This relationship was largely due to a progression from low normal cognitive function at first assessment to impaired cognitive function at second assessment and was associated with an elevated risk of losing independence in the six activities. CONCLUSIONS: Progression from low normal to impaired cognitive function was associated with loss of independence in activities of daily living. Thus low normal cognitive function could be viewed as an early warning of impending cognitive impairment and loss of physical function.

Impaired resistance to oxidation of low density lipoprotein in cystic fibrosis: improvement during vitamin E supplementation.

Antioxidants such as vitamin E protect unsaturated fatty acids of LDL against oxidation. In the ex vivo model used, LDL was exposed to Cu2+ ions, a potent prooxidant capable of initiating the oxidation of LDL. The lag time, indicating the delay of conjugated diene formation in LDL due to antioxidant protection, was measured in 54 cystic fibrosis (CF) patients with plasma alpha-tocopherol levels below (Group A, n = 30) or above (Group B, n = 24) 15.9 mumol/L (mean - 2 SD of Swiss population). Patients were reevaluated after 2 months on 400 IU/d of oral RRR-alpha-tocopherol. In group A, alpha-tocopherol concentrations in LDL increased significantly from 3.2 +/- 1.6 mol/mol LDL to 8.2 +/- 2.8 mol/mol (P < 0.001) and lag times increased from 79 +/- 33 min to 126 +/- 48 min (P < 0.001), whereas in the vitamin E sufficient group B no further increase neither in LDL alpha-tocopherol concentrations or in lag times was observed. LDL oleic acid concentrations were higher, and linoleic acid concentrations were lower in patients than in controls. After efficient vitamin E supplementation, lag times were positively related to LDL alpha-tocopherol (P < 0.01) and negatively to LDL linoleic and arachidonic acid content (P < 0.001). The maximum rate of oxidation correlated positively with linoleic and arachidonic acid concentrations, as did the maximum conjugated diene absorbance. These results indicate that LDL resistance to oxidation is impaired in vitamin E deficient CF patients but can be normalized within 2 months when alpha-tocopherol is given in sufficient amounts. Linoleic and arachidonic acid content exhibit a major influence on the LDL resistance to oxidation.

Neuropharmacological study of aged MAM-treated rats.

After evaluation of activity in an open field, norepinephrine (NE), serotonin (5HT), 5-hydroxyindolacetic acid (5HIAA), homovanillic acid (HVA), and choline acetyltransferase (CAT) were investigated in cortex of 26-month-old rats poisoned with methylazoxymethanol (MAM) as compared to control rats of the same age. NE and 5HT concentrations showed a marked increase, but levels were normal when expressed as total content, just as in MAM-exposed young adults. Concentrations of 5HIAA were also increased but to a lesser extent than 5 HT. Aged MAM rats did not show any modification of spontaneous activity although hyperactivity is characteristic of young adults exposed to MAM. Together with this behavioral observation, a significant decrease in total HVA content was measured. Because HVA levels seem correlated with activity in MAM-exposed rats, we speculate that the behavioral abnormality recovers in old age. Total CAT activity was also reduced. These results indicate that the neurochemical pattern of young adult MAM-poisoned rats is conserved in aged rats except for some changes in the dopaminergic and cholinergic systems.