Elevated resting energy expenditure in adolescents with sickle cell anemia

OBJECTIVE: To examine further the role of the amygdala in the recognition of facial expression in adolescents. METHOD: Twelve healthy adolescents were studied using functional magnetic resonance imaging technology during a task of facial affect recognition and a visual control task. RESULTS: All subjects demonstrated a significant increase in signal intensity in the amygdala for the facial expression recognition task. CONCLUSIONS: The data are consistent with previous work in healthy adult subjects implicating the amygdala as essential for the recognition of fearful facial expression.     

Histopathologic features of neovascularization in sickle cell retinopathy

PURPOSE: To examine the histopathologic and morphometric features of neovascular lesions in human proliferative sickle cell retinopathy. METHODS: Postmortem ocular tissue was obtained from three subjects (aged 20, 28, and 40 years) with SS hemoglobinopathy and prepared for adenosine diphosphatase flat-embedding. Morphometric analysis was performed before serial sectioning. RESULTS: Numerous active and autoinfarcted lesions were found that represented virtually all stages in the life cycle of preretinal neovascularization. These formations ranged from single small loops extending from arteries and veins along the retinal surface to the typical complex, elevated sea fan formations. Sea fans developed at hairpin loops and at arteriovenous crossings. There was an average of 5.6 connections between sea fans and retinal vessels; of these, 45% were arteriolar, 52.5% were venular, and 2.6% were at the capillary level. Six of eight sea fans were located at arteriovenous crossings. Autoinfarction appeared to occur initially within the sea fan capillaries. The average height of sea fans was 123 microns above the retinal surface. CONCLUSIONS: Preretinal neovascularization in sickle cell retinopathy can arise from both the arterial and venous sides of the retinal vasculature and can assume a variety of morphologic configurations. Multiple feeding arterioles and draining venules are common, and autoinfarction appears to occur initially at the preretinal capillary level rather than at feeding arterioles. Arteriovenous crossings may be a preferential site for sea fan development.     

Stress, coping, and psychological adjustment of adults with sickle cell disease.

Examined a transactional model of psychological adjustment to chronic illness with 109 African-American adults with sickle cell disease (SCD). Good psychological adjustment was associated with lower levels of perceived daily stress and stress regarding SCD illness tasks, higher efficacy expectations, less use of palliative coping methods, less use of negative thinking/passive adherence pain-coping strategies, and family functioning characterized by high levels of support and low levels of conflict and control. Overall, the underlying stress and coping conceptual model accounted for 44–50% of the variance in psychological adjustment. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Renal medullary carcinoma: a potential sickle cell nephropathy of children and adolescents

cDNAs of potyviruses from Allium plants cultivated in different parts of the world were cloned by RT-PCR with a common primer for amplifying the 3' terminal genomic RNAs of onion yellow dwarf virus (OYDV), leek yellow stripe virus (LYSV) and, probably, of closely related potyviruses. Their nucleotide sequences bearing the viral coat protein (CP) gene and the 3' non-coding sequence were determined and compared. The degree of their sequence similarities clearly differentiated the respective viruses into 3 groups, namely OYDV "garlic-type", "wakegi-type" and LYSV group. The "garlic-type" included all the garlic isolates and two Indonesian shallot isolates. The "wakegi-type" group consisted of the isolates from Indonesian shallot and previously reported ones from Japanese Allium plants excluding garlic. The LYSV group was represented by LYSV isolates from garlic and leek. The CP sequences of LYSV group viruses differed from those of OYDV "garlic-type" and "wakegi-type" viruses (less than 60% similarities). In contrast, the sequence similarities between the OYDV "wakegi-type" and "garlic-type" isolates were 73.5 to 76.7%, suggesting they were closely related but should be discriminated as distinct species. These findings indicate that at least three distinct potyviruses, clearly distinguishable by the viral CP sequence, are present in Allium species. Finally, we concluded that the "garlic-type" viruses correspond to the typical OYDV and the "wakegi-type" viruses represent the viruses previously identified as Welsh onion yellow stripe virus (WoYSV) and shallot yellow stripe virus (SYSV). We propose the name wakegi yellow dwarf virus (WYDV) for the "wakegi-type" viruses.     

Psychological adjustment of adolescents with sickle cell disease: relations with demographic, medical, and family competence variables

OBJECTIVE: Investigate the hypothesis that family competence in addressing challenges associated with sickle cell disease (SCD) contributes to adolescents' adjustment. METHOD: During routine clinic appointments, 80 adolescents (M age = 14.4 years) and their parents independently completed the Self-Report Family Inventory (SFI), which assesses family competence, and measures of adolescent adjustment problems. Information related to disease severity was obtained from clinic files. RESULTS: Regression analyses controlling for demographic and medical variables revealed that higher family competence was associated with fewer internalizing and externalizing behaviors by the adolescent; these relations were particularly true for younger adolescents and for girls. Parental reports of somatic complaints in girls were predicted by parental ratings of family competence. DISCUSSION: Interventions for adolescents with SCD should be family-centered and should focus on strengthening the family's ability to manage stressors associated with parenting an adolescent with a chronic illness.     

Stability and change in the psychological adjustment of mothers of children and adolescents with cystic fibrosis and sickle cell disease

Found moderate stability in the classification of maternal adjustment in two longitudinal studies of mothers of children and adolescents with cystic fibrosis and sickle cell disease. In terms of the transactional stress and coping model, stable poor maternal adjustment was associated with higher levels of appraisal of daily stress and palliative coping and low levels of family supportiveness. With initial levels of maternal adjustment, demographic parameters, and follow-up interval controlled, concurrent levels of daily stress accounted for significant portions of variance in maternal adjustment at follow-up for both illness groups. In addition, illness severity, child psychological adjustment, and family conflict added significant increments to maternal adjustment at follow-up in the cystic fibrosis group. Findings are discussed in terms of a basis for subsequent intervention studies to enhance the adjustment of mothers of children with chronic illness.     

Health insurance status and the use of emergency and other outpatient services by adults with sickle cell disease

STUDY OBJECTIVE: To evaluate insurance status and frequency of use of emergency services in adults with sickle cell disease. DESIGN: Retrospective analysis of visits. SETTING: Emergency department and outpatient clinics of an urban university hospital. PARTICIPANTS: One hundred seventy-two subjects, who made 771 visits to the ED during 1990. RESULTS: Of the 172 subjects, 31 were covered by commercial insurance, 32 were covered by Medicare, and 109 were covered by Medicaid or were uninsured. Insurance status and frequency of use of emergency services were independent (P > .05). On discriminant analysis, Medicaid-covered and uninsured subjects were correctly classified, but commercially insured and Medicare subjects were not. Medicaid and uninsured subjects were more likely to be younger and to live closer to the hospital (P < .00005). High-frequency users of emergency services were discriminated from low-frequency users. High-frequency users were more likely to be younger, to be users of primary-care services, and to live closer to the hospital (P = .0004). CONCLUSION: Provision of primary-care services or stable insurance in the form of commercial insurance or Medicare did not decrease use of emergency services in subjects with sickle cell disease in a group of patients selected from one urban academic ED.     

Cardiovascular risk factor levels in adults with mental retardation

Cardiovascular risk factors in a population of adults with mental retardation were examined. The subjects resided in three types of settings (16+ bed facility, group home, and natural family) and were measured on the following parameters: blood lipids, obesity, and smoking. The data were compared to a nonretarded population and to the guidelines established by the National Cholesterol Education Program. Results indicated that adults with mental retardation had cardiovascular risk profiles similar to those of individuals without mental retardation in the Framingham Offspring Study and that to the extent that cardiovascular health is a concern to the general population, it must also be a concern for individuals with mental retardation.     

Platelet activation in patients with sickle cell disease

Vascular occlusion and vasculopathy underlie much of the morbidity in patients with sickle cell anaemia. Platelets may play a role in this vasculopathy. Samples from 43 patients with sickle cell disease (SCD) were examined for evidence of platelet activation using fluorescent-labelled monoclonal antibodies and flow cytometry. There was increased expression of activation-dependent antigens on the platelets from patients with SCD compared to those from both Caucasian and African-American controls. In addition, SCD patients had increased levels of platelet microparticles. Platelets are activated in patients with sickle cell disease. The contribution of platelet activation to sickle cell pathophysiology is under active investigation in our laboratories.     

Red cell metabolic and membrane features in haemolytic anaemia of alcoholic liver disease (Zieve's syndrome)

Zieve's syndrome (ZS), which consists of transient haemolytic anaemia, jaundice, hyperlipoproteinaemia, and alcohol-induced liver disease, was studied in male patients during the acute (n = 20) and the remittent (n = 10) phase. Chronic alcoholics (n = 10) without haemolysis and healthy male persons (n = 10) served as controls. Erythrocytes were separated into old and young cells by means of density-layer centrifugation. Those fractions which contained older red cells disclosed a pyruvate-kinase instability which resulted in impaired metabolism. Changes in membrane lipid composition as indicated by increased cholesterol and polyunsaturated fatty acids (PUFA) were also detected in patients during the acute phase of ZS. Alcohol-induced red-cell vitamin-E deficiency with a decrease in PUFA levels may provoke an oxidation of reduced red-cell glutathione which in turn results in the enzyme instability. This study lends further support to the hypothesis that the putative role of the red-cell metabolic injury in the origin of haemolysis in ZS cannot be envisaged without introducing membrane-linked and extracellular cofactors.     

Multiple-risk behaviour in adolescents and young adults

OBJECTIVES: This article examines the prevalence of four risk behaviours among teenagers and young adults: smoking, binge drinking, sex with multiple partners, and sex without a condom. DATA SOURCE: The data are from a Health Canada-sponsored supplement to the 1994/95 National Population Health Survey. The analysis is based on 905 respondents aged 15 to 19 and 1,055 respondents aged 20 to 24. ANALYTICAL TECHNIQUES: Prevalence estimates of the four risk behaviours were calculated for males and females in each age group. An index of multiple-risk behaviour was derived by summing the four risk behaviours. Hierarchical multiple regression was used to examine how sets of variables are related to multiple-risk behaviour. MAIN RESULTS: Multiple-risk behaviour was higher among young people who had never married, who were not students, and who did not live with a parent. Feeling distressed was positively linked with multiple-risk behaviour, while regular attendance at religious services was negatively linked with such conduct.     

Plasma and red cell lipids in sickle cell disease

Lipids, in particular phospholipids, are essential components of membrane systems, and the measurement of phospholipids and cholesterol in plasma and tissues is helpful in diagnosis. Phospholipids represent about 60 to 70% of total red cell (RBC) lipids, while about 25% is free cholesterol. Lipids in RBC are present in a dynamic state of equilibrium, and the RBC have the capacity for rapid exchange of lipids with plasma in several ways. The present study examined the cholesterol and phospholipid levels of plasma and erythrocytes in male patients with sickle cell anemia and in healthy male individuals of comparable age. This was performed with a view to detecting possible differences that might be related to some of the RBC abnormalities which accompany the disease. The results show that plasma lipids are significantly reduced in patients with sickle cell anemia and that RBC cholesterol was higher in sickle cell patients than in normal subjects.     

Hematuria and sickle cell disorders

To examine the relationship between gross hematuria and sickle cell disorders, all patients admitted to Grady Memorial Hospital with the diagnosis of a sickle cell disorder during a 14-month period were reviewed. Of 115 such patients, 65% had sickle cell disease and 35% had sickle cell trait. None of the former but seven of the latter group had gross hematuria. Mean age of the seven was 30 years. Comprehensive examinations and laboratory studies showed that all were free of concomitant disease. Physical findings, diagnostic modalities, and treatment were reviewed. Findings suggest that conservative therapy (bed rest, hydration, and diuresis) is usually effective.     

Follow-up of coping skills training in adults with sickle cell disease: Analysis of daily pain and coping practice diaries.

This study examined the 3-month follow-up effects of a pain coping skills intervention in African American adults with sickle cell disease. Sixty-seven participants were randomly assigned to either a coping skills condition or a disease-eduction control condition. Multivariate analyses applied to summary measures of coping, laboratory pain perception, and clinical measures indicated that participants in the coping intervention reported significantly lower laboratory pain and significantly higher coping attempts at 3-month follow-up in comparison with the control condition. Multilevel random effects models applied to prospective daily diaries of daily pain, health care contacts, and coping practice indicated that on pain days when participants practiced their strategies, they had less major health care contacts in comparison with days when they did not use strategies. (PsycINFO Database Record (c) 2010 APA, all rights reserved)