Perceived Body Image and Eating Behavior in Young Adults with Cystic Fibrosis and Their Healthy Peers

Treatment aimed at achieving an ideal nutritional status is an integral part of the management of patients with cystic fibrosis (CF). Emphasis is continually placed upon dietary intake and weight. The effects of this on eating behavior and self-perceptions are unclear. This work compared male and female CF adults with a healthy male and female control population with regard to (a) clinical variables, (b) actual, perceived, and desired body shape/body mass index (BMI), and (c) body satisfaction, eating behaviors and attitudes, and self-esteem. Clinical data were recorded for 221 adults with CF and 148 healthy controls. All subjects completed BMI Charts (perception of body weight/BMI), the Eating Attitudes Test, and scales of body satisfaction and self-esteem. CF patients had poorer lung function and nutritional status than controls. Control males accurately perceived their body shape/BMI and were content with it, whereas CF males viewed their BMI as greater than it actually was and desired to be much heavier. Control females viewed their body shape/BMI as less than it actually was and desired to be even slimmer, in comparison with CF females, who perceived their BMI as less than it actually was but were happy with their perceived shape/weight. Control subjects, especially females, dieted to a greater extent and were more preoccupied with food (with binge eating and intended vomiting) than CF patients. Conversely, those with CF reported greater pressure from others to eat than did controls. More problems with food/eating behavior were associated with less body satisfaction and reduced self-esteem. In comparison with a healthy control population, the perceptions and behaviors of CF adults relating to eating, weight, and body image are not abnormal. Indeed, females with CF have fewer problems than their healthy peers.     

Behavioral adjustment of latency age children with cystic fibrosis

One hundred and twenty-six 6 to 11-year-old latency age children with Cystic Fibrosis regularly attending the CF clinic of the Hospital for Sick Children were asked to complete the Piers-Harris Self-Concept Scale and the Children's Health Locus of Control. Their parents were requested to complete the Child Behavior Checklist and the Family Assessment Measure. One hundred and eight (86%) participated in the study. Twenty-three percent of the children were found to have sufficient behavior problems to indicate a significant degree of maladjustment. Latency-age CF children show an increase in problems compared to a pre-school group, suggesting that leaving the protection of the family is problematic for a child with a chronic physical disorder. Males show more behavior problems than females. Males' behavior is characterized by somatic complaint profile. In spite of difficulties, CF latency children are able to maintain good social competence and self-concept suggesting compensatory mechanisms. These mechanisms are different for males and females. Females' self-concept and social competence are supportive of each other, whereas for males, this is not the case. Similarly, female behavior is relevant to family functioning. Males and females adjust to difficulty as indicated by differences in behavior profiles.     

Differential cellular expression of cystic fibrosis transmembrane regulator in human reproductive tissues. Clues for the infertility in patients with cystic fibrosis.

Cystic fibrosis (CF) is characterized by a wide spectrum of clinical manifestations, including reproductive problems. Practically all males affected by the disease are infertile due to azoospermia associated with pathology of the male ducts, whereas females with CF have reduced fertility. To study the mechanism of reproductive pathology in CF patients, we analyzed the levels and localization of expression of the cystic fibrosis transmembrane regulator (CFTR) gene in relevant postnatal tissues. Significant expression was detected in the epithelium of the epididymis and vas deferens. Minimal expression, not associated with specific cell types, was seen in the mature testis. In female genitalia, variable levels of expression were seen in the cervical epithelium and fallopian tube. The endometrial epithelium and glands expressed CFTR at high levels only after puberty. No expression was seen in ovaries. Deficient secretory function of CFTR in males but not in females may lead to organ damage probably as a consequence of excessive concentration of viscid luminal contents.     

Growing older with cystic fibrosis: psychologic adjustment of patients more than 16 years old

The 79 female and 147 male patients constituting the population with cystic fibrosis (CF) aged 16 years and older attending The Hospital for Sick Children were asked to complete the Cornell Medical Index (CMI) and Tennessee Self-Concept Scale (TSCS); 64 female (81%) and 112 male (76%) subjects participated. Analysis of CMI results showed 43% of female subjects to have moderate to severe emotional disturbance compared to 19% of male subjects. This female : male ratio for severity of emotional disturbance is found in ostensibly healthy groups, but the percentages of disturbance approach values for medical patient populations. The frequency of emotional disability is greater in those more than 20 than in those 16-19 years old. The TSCS results portray a generally normal self-concept except for scores of positive physical self and psychosis for patients aged 20 years and older; these scores approach psychiatric values, suggesting that some reality distortion facilitates emotional adjustment to adult life with CF. The TSCS and CMI results correlate significantly, indicating a connection between self-concept and emotional status. However, TSCS and CMI scores do not correlate with measures of disease severity except for correlations between lung function and physical self-concept in older male patients. These results suggest that psychologic functioning is independent of the degree of physical impairment in older patients with CF, with the long-surviving male patients more realistically appraising the limitations their disease imposes and utilizing denial and minimization to a lesser degree. Demographic data on the clinic population reveal that most patients aged 16 years and older cope with their intellectual, developmental, and socioeconomic tasks commensurate with normal age expectations.     

Mealtime Behavior and Parent-Child Interaction: A Comparison of Children with Feeding Problems, and Nonclinic Controls

Examined the role of family interaction factors in dietary complianceproblems reported by parents of children with cystic fibrosis(CF). The family mealtime interactions of children with CF,children with feeding problems and nonclinic controls were observed,and parents monitored children's eating behavior at home. Parentsof children with CF reported more concern about feeding problemsand recorded more disruptive mealtime behavior than parentsof nonclinic children. Observational data showed children withCF to display overall rates of disruptive mealtime behaviornot significantly different from either comparison group. Mothersof children with CF were observed to engage in higher ratesof aversive interaction with their child than did mothers ofnonclinic controls. Fathers of children with CF reported lowermarital satisfaction than fathers of controls. Both mothersand fathers of children with CF reported lower parenting self-efficacythan non-CF families. Clinical implications are discussed.     

Fertility and pregnancy outcomes in men and women with cystic fibrosis in the United Kingdom

BACKGROUND: The survival of patients with cystic fibrosis (CF) now occurs into the fourth decade of life. Our aim was to evaluate the fertility and pregnancy outcomes of men and women with cystic fibrosis within a large British cohort. METHODS: A population based cohort study of 4659 males and females registered with the UK Cystic Fibrosis Database in 2001. RESULTS: Poor respiratory health (forced expiratory volume in 1 s FEV1 <50%) and CF-related diabetes increased significantly post-puberty compared with childhood. Few individuals with CF sought fertility treatment (1% men, 0.5% women) or achieved pregnancies (1.3% of partners of men, 5.7% women). However, the majority of pregnancies had a good outcome (67% live term birth among male partners, 74% among women). Women who achieved a pregnancy were less likely to suffer from poor respiratory health (FEV1 <50%), age adjusted Odds Ratio 0.6 (95% CI 0.3-0.9), less likely to be homozygous for the DF508 genotype OR 0.5 (95% CI 0.3-0.9) and had a significantly earlier median age at diagnosis (0 years vs 2 years, P=0.001). CONCLUSIONS: Increasing numbers of males and females with cystic fibrosis are reaching reproductive age but currently very few have a child. Optimal adult health should improve the reproductive prognosis for both men and women.     

Pregnant female guinea pigs adapt easily to a new social environment irrespective of their rearing conditions

For male guinea pigs, the social rearing conditions are crucial for the course of social interactions and endocrine stress responses in later life. While colony-reared males adjust easily to new social situations, high levels of aggression and extreme endocrine responses are found in males that were raised in pairs [Psychoneuroendocrinology 23 (1998) 891]. This study investigated whether the same finding also applies to females. Therefore, 8 females reared in mixed-sexed colonies of 8-10 males and 12-13 females (designated as CF) and 8 females reared in mixed-sexed pairs (designated as PF) were transferred to an unknown colony for 3 days. Control females remained in their respective housing conditions. Serum cortisol concentrations (CORT) were determined before, during and after the transfer. The behavior of the CF and PF was recorded on the first, second and third day of transfer. All females adjusted easily to the new social situation, but frequencies of some behavioral patterns were significantly different between CF and PF. In the new social situation, PF displayed social orientation as well as defensive aggressive and avoidance behavior more frequently than CF. On the day of transfer, resident males displayed courtship behavior more frequently towards PF than CF. CORT did not differ between PF and CF either before, during or after the transfer. Furthermore, the transfer to the new social situation did not result in significantly increased CORT 4 h later either in CF or PF. We conclude that-in contrast to male guinea pigs-females are able to adapt to unfamiliar conspecifics independently of their social rearing conditions.     

Linkage between the loci for cystic fibrosis and paraoxonase

In a material of 22 Danish, 26 Canadian, 10 Australian, 5 English and 5 American families with at least 2 children affected with cystic fibrosis (CF) a combined positive LOD score of 3.46 was found for the relationship cystic fibrosis-paraoxonase (PON) at recombination fraction theta = 0.07 in males and theta = 0.13 in females. Assuming a three allele model for PON the LOD score was 4.50 at the same recombination fractions. This confirms our earlier finding of an indication of CF-PON synteny.     

Modifying Problematic Mealtime Interactions of Children with Cystic Fibrosis and Their Parents Via Behavioral Parent Training

Implemented behavioral parent training targeting maladaptivemealtime behavior with two children with cystic fibrosis (CF)and their parents. Treatment was implemented in multiple baseline fashion across the two families. Primary dependent measurewas coding of parent and child behaviors from videotaped dinners.Data were also collected on the children's daily calorie intakeand weight. During treatment and at the posttreatment follow-ups,parents' attention to disruptive behavior decreased, attentionto appropriate eating increased, and parental control at mealsincreased. The children showed an increase in appropriate behaviorand a decrease in disruptive behavior; caloric intake and weightalso improved. Results are discussed in terms of the applicabilityof behavioral intervention with feeding problems in childrenwith CF.     

父母教养方式与青少年行为问题关系的研究

目的　探讨父母教养方式与青少年行为问题的关系。方法　抽取 2 0 6名青少年为本研究的对象 ,其中男生 1 0 4名 ,女生 1 0 2名 ,每位被试完成父母教养方式评价量表 ( EMBU) ,其父母完成 Achenbach儿童行为量表 ( CBCL)。结果　青少年中存在着一定程度的行为问题 ,男女生的行为问题有所不同。父母教养方式中的情感温暖、理解与女生的行为问题有显著的负相关。父母教养方式与男生的行为问题有一定关联。结论　父母教养方式与青少年的行为问题有关。父母亲要重视改善教养方式 ,减少青少年的行为问题     

Female and male antisocial trajectories: from childhood origins to adult outcomes

This article reports on the childhood origins and adult outcomes of female versus male antisocial behavior trajectories in the Dunedin longitudinal study. Four antisocial behavior trajectory groups were identified among females and males using general growth mixture modeling and included life-course persistent (LCP), adolescent-onset, childhood-limited, and low trajectory groups. During childhood, both LCP females and males were characterized by social, familial and neurodevelopmental risk factors, whereas those on the adolescent-onset pathway were not. At age 32, women and men on the LCP pathway were engaging in serious violence and experiencing significant mental health, physical health, and economic problems. Females and males on the adolescent-onset pathway were also experiencing difficulties at age 32, although to a lesser extent. Although more males than females followed the LCP trajectory, findings support similarities across gender with respect to developmental trajectories of antisocial behavior and their associated childhood origins and adult consequences. Implications for theory, research, and practice are discussed.     

Cystic fibrosis in Ontario

The incidence of cystic fibrosis in Ontario, Canada has been determined from clinical data, from the cystic fibrosis database of the Hospital for Sick Children, Toronto, and from population statistics in the Province of Ontario. The survey included 420 confirmed cases of cystic fibrosis born during the period 1966–1980. The mean incidence during this period was one in 2,927. In the last 5-year period, a decline was noted in incidence that may have reflected in part the effectiveness of early diagnosis and genetic counseling in affected families. During the period of the survey, over 60% of cases were diagnosed within the first year of life, 74% by age 2 years, and 90% by age 5 years. Clinical diagnosis in the first year of life was more common in males (65%) than in females (54%), a consistent finding during the period of the survey. The incidence of meconium ileus was 15.7% of ascertained cases of cystic fibrosis, with similar incidences in males (16.4%) and females (14.4%). Although survival has not been the subject of this survey, mortality in the neonatal period was significantly higher in males than in females with cystic fibrosis.     

Cystic fibrosis in Ontario

The incidence of cystic fibrosis in Ontario, Canada has been determined from clinical data, from the cystic fibrosis database of the Hospital for Sick Children, Toronto, and from population statistics in the Province of Ontario. The survey included 420 confirmed cases of cystic fibrosis born during the period 1966-1980. The mean incidence during this period was one in 2,927. In the last 5-year period, a decline was noted in incidence that may have reflected in part the effectiveness of early diagnosis and genetic counseling in affected families. During the period of the survey, over 60% of cases were diagnosed within the first year of life, 74% by age 2 years, and 90% by age 5 years. Clinical diagnosis in the first year of life was more common in males (65%) than in females (54%), a consistent finding during the period of the survey. The incidence of meconium ileus was 15.7% of ascertained cases of cystic fibrosis, with similar incidences in males (16.4%) and females (14.4%). Although survival has not been the subject of this survey, mortality in the neonatal period was significantly higher in males than in females with cystic fibrosis.     

Life events, psychopathology, and suicidal behavior in Chinese adolescents

BACKGROUND: Little is known about risk factors of suicidal behavior among Chinese adolescents. This study examined the associations between negative life events, psychopathology, and suicidal behavior in rural adolescents of China. METHODS: A total of 1362 adolescent students in a rural prefecture of China completed a self-administered questionnaire concerning suicidal behavior, psychopathology, life events, and demographics. Data analyses were conducted using multivariate logistic regression modeling. RESULTS: Females were more likely to report suicidal ideation than males (22.0% vs. 17.5%). While the rate of suicide attempt was slightly higher in younger males (12-15 years) than in females (4.7% vs. 3.1%), the rate was higher in older females (16-18 years) than in males (12.7% vs. 9.5%). Suicide attempters reported more negative life events during the past year than suicidal ideators and non-suicidal adolescents. Academic stress and family conflicts were the major stress domains of adolescents at risk for suicidal behavior. A significant dose-response relationship was observed between the number of life events and suicidal behavior. Negative life events were also associated with increased risk for internalizing and externalizing problems. Both internalizing and externalizing problems were significantly associated with elevated risk for suicidal behavior after negative life events were controlled. LIMITATIONS: This is a cross-sectional study. Longitudinal study is warranted to examine the roles of life stress in adolescent suicidal behavior. CONCLUSIONS: Adolescents who experienced more negative life events are at increased risk for suicidal behavior. Both internalizing and externalizing problems mediate the effect of life events on adolescent suicidal behavior.     

Translation and linguistic validation of a disease-specific quality of life measure for cystic fibrosis

OBJECTIVE: To develop a conceptually and semantically valid English version of a French disease-specific measure of quality of life for children, adolescents, and adults with cystic fibrosis (CF). METHODS: Following a backward and forward translation of the measure, 60 participants, including 20 children, 20 parents, and 20 adolescents/young adults completed the Cystic Fibrosis Questionnaire (CFQ) and a series of cognitive probes evaluating their understanding of the items and response choices. RESULTS: Semantic and conceptual problems with the items were identified and modified for the second set of cognitive interviews. Response distributions across items and ages were adequate, and the predicted associations between disease severity and quality of life were obtained. CONCLUSIONS: The English version of the CFQ appears to be a linguistically valid measure of quality of life for patients with CF. A national validation study is now under way to test the psychometric properties of the measure.     

Association of observed family relationship quality and problem-solving skills with treatment adherence in older children and adolescents with cystic fibrosis

OBJECTIVE: To examine associations between observations of the quality of family relationships and problem-solving skills and reported adherence to medical treatments for older children and adolescents with cystic fibrosis (CF). METHODS: Reports of adherence were obtained from 96 youth with CF and their parents recruited from six CF centers in the Midwest and southeastern United States. Videotaped observations of family discussions of high conflict issues were used to assess quality of relationships and problem-solving skills. RESULTS: Hierarchical regression analyses indicated that observed family relationship quality (RQ) was related to parent and child reports of adherence to airway clearance and aerosolized medications after controlling for demographic variables and illness severity. Observed family problem solving was not a significant predictor after controlling for RQ. CONCLUSIONS: Older children and adolescents who come from families experiencing unhappy and conflicted relationships may be at greater risk for poor adherence to treatments; thus, family relationships are appropriate targets for interventions aimed at improving adherence.     

The pathology of cystic fibrosis

Cystic fibrosis (CF) is one of the commonest lethal inherited conditions among Caucasians. It affects multiple organ systems and exhibits a range of clinical problems of varying severity. Life expectancy has improved in recent years as treatment regimes have become more intensive, but current treatments are expensive, often time consuming and may affect quality of life. New treatments for the pulmonary disease are under clinical trial and include antiproteases, amiloride, a sodium channel blocker, DNase and gene therapy. The gene for cystic fibrosis was identified in 1989 and this together with the emerging technology of gene therapy heralded a new dawn for the treatment of genetic disease. The lung is considered an ideal organ to target due to ease of access, but subsequent research has shown that the airway surface provides an efficient barrier to topically applied gene transfer agents. A number of Phase I clinical safety trials were carried out through the 1990s and provided proof of concept evidence that delivery of DNA by either viral or non-viral means was safe though not clinically efficacious. Current research is now focusing more on the barriers faced by delivery agents, with the aim that more efficient gene delivery will lead to a gene therapy for cystic fibrosis. The histopathologist is rarely called upon to make the initial diagnosis as cystic fibrosis is usually diagnosed clinically, being characterized by chronic bronchopulmonary infection, malabsorption due to pancreatic insufficiency and a high sweat-sodium concentration on sweat testing. Most information concerning both macroscopic and microscopic findings in cystic fibrosis has come from autopsy studies, so the pathological features are often extreme. However, with increasing survival of patients with cystic fibrosis, we are seeing more subtle changes in other organs and in addition, more aggressive drug therapy, gene therapy and lung transplantation are bringing with them new disease entities and complications.     

Physical and Psychological Health of Young Adolescents: The Relationship to Gender and Marital Conflict

The response of early adolescent boys and girls to parentalmarital conflict was studied. Self-, mother-, and teacher-reportedphysical, externalizing (e.g., acting out), and internalizing(e.g., anxiety) problems were examined. Although prior studieshave examined the relationship between marital con flict andpsychological adjustment, particularly externalizing problems,the association of marital conflict and physical symptoms hasbeen ignored. Thirty-nine adolescents from intact families wereassigned to one of four groups based on gender and the mother'sreport of marital conflict: males, high-conflict homes; females,high-conflict homes; males, low-conflict homes; and females,low-conflict homes. The hypothesis that females would respondto marital conflict by the demonstration of physical and/orinternalizing difficulties was not supported. However, as predicted,males, but not females, demonstrated more externalizing problemsin high than in low-conflict homes. Several explanations formales' responsiveness to marital conflict by the manifestationof externalizing problems and for the absence of a relationship between physical health and marital conflict were proposed.     

Frequency of CFTR gene mutations in males participating in an ICSI programme.

A higher prevalence of cystic fibrosis transmembrane regulator (CFTR) gene mutations has been suggested both in men affected by congenital aplasia of the vas deferens, and in individuals presenting with reduced sperm quality. In this case, an increased risk for offspring being affected by cystic fibrosis (CF) can be expected in couples who are planning to undergo intracytoplasmic sperm injection (ICSI), since most of the male partners suffer from infertility. In order to determine the risk for these couples more precisely, we offered them a test for the most frequent CF mutations prevalent in the German population. The frequency of mutations within the CFTR gene in the female group was in the same range as expected for the general population (six out of 150). In 10 out of 207 males tested, infertility could be explained by exogenous factors not related to CFTR. Among the remaining 197 males with idiopathic infertility, we detected 13 heterozygotes for a mutation within the CFTR gene. This slightly, but significantly (P = 0.014), elevated rate could indicate that infertile males have, compared with the general population, an increased risk of being a carrier of a CFTR gene mutation.     

Mutation analysis in 600 French cystic fibrosis patients.

The cystic fibrosis transmembrane conductance regulator (CFTR) gene of 600 unrelated cystic fibrosis (CF) patients living in France (excluding Brittany) was screened for 105 different mutations. This analysis resulted in the identification of 86% of the CF alleles and complete genotyping of 76% of the patients. The most frequent mutations in this population after delta F508 (69% of the CF chromosomes) are G542X (3.3%), N1303K (1.8%), W1282X (1.5%), 1717-1G-->A (1.3%), 2184delA + 2183 A-->G (0.9%), and R553X (0.8%).