Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group

Background/PurposeA small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.MethodsRetrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale.ResultsThere were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD.ConclusionMore than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment.Level of EvidenceLevel 4     

Teaching Family Policy in Undergraduate and Graduate Classrooms: Why It's Important and How to Do It Better*

Abstract: As newcomers on college campuses, family policy courses have the potential to benefit policymaking, fill a void in undergraduate and graduate education, strengthen families, and prepare students for lifelong political engagement during a pivotal period in their development. Yet, family policy has proven a challenging course to teach. Family policy is an esoteric concept, which makes courses difficult to distinguish from other policy courses. The content of a family policy course is fluid and inherently value laden. This paper proposes course content and teaching techniques to transform these challenges into learning opportunities. The author discusses similarities and differences in teaching undergraduate and graduate courses and recommends cross‐university dialogue and resource exchange to improve the teaching of family policy in college classrooms.     

Sensitivity and specificity of a self-administered questionnaire for familial screening of adult-onset dystonia.

We developed a self-administered questionnaire for screening the most common adult-onset dystonias. It was tested in 90 first-degree relatives of 22 adult-onset dystonia patients, yielding 79% sensitivity and 94% specificity. Simulation of a case-finding procedure based on serial application of the questionnaire and clinical examination of both subjects screening positive and subjects screening negative who had < 8 years of schooling increased sensitivity to 95% and specificity to 100%. This questionnaire may be an important screening resource for familial aggregation studies to be used in the context of a complex case-finding procedure.     

Family relationships and the development of social competence in adolescence

Abstract Resilient adolescents are notable for their social competence, which enables them to form and maintain close relationships. The evidence is that adolescents' social competence is derived from their experience of close relationships within their family. On the basis of structured interviews, adolescents' working models of attachments can be categorized into secure, dismissive, or pre-occupied. These attachment styles are associated with very divergent beliefs about the self and others, with differing patterns of emotion regulation and with differing risk profiles for maladjustment. Parenting styles and family relationships appear to have considerable influence on attachment behaviour. Further evidence for the importance of the family comes from research on ego development. Family level behavioural patterns have been discerned from family research interviews which are associated with stagnation or advancement in ego development during adolescence. Though the results suggest causal connections, the direction of effects is far from clear. Longitudinal research underpins the importance of childhood temperament as a contributing factor to the quality of the family environment that the child and then adolescent experiences.     

Parental alcohol use and brain volumes in early- and late-onset alcoholics.

BACKGROUND: Studies have shown that alcoholics have smaller brain volumes than non-alcoholic cohorts, but an effect of family history (FH) of heavy drinking on brain volume has not been demonstrated. We examined the relationship between an FH of heavy drinking and both brain shrinkage as measured by the ratio of brain volumes to intracranial volume (ICV) as well as maximal brain growth as measured by ICV in early-onset and late-onset alcoholics. METHODS: With T1-weighted resonance imaging, we measured ICV, brain volume, and white and gray matter volume in adult treatment-seeking late-onset and early-onset alcoholics with either a positive or a negative FH of heavy alcohol use, and in healthy control subjects. We also calculated brain shrinkage using a ratio of soft tissue volumes to ICV. RESULTS: The FH positive alcoholic patients had significantly smaller ICVs than FH negative patients, suggesting smaller premorbid brain growth. Brain shrinkage did not correlate with FH. Late-onset alcoholics showed a greater difference in ICV between FH positive and FH negative patients than early-onset alcoholics. Late-onset FH positive patients also had significantly lower IQ scores than late-onset FH negative patients, and IQ scores were correlated with ICV. CONCLUSIONS: These data provide evidence that parental alcohol use might increase risk for alcoholism in offspring in part by a genetic and/or environmental effect that might be related to reduced brain growth.     

The use of the Impact on Sibling scale with families of children with chronic illness and developmental disability

Objective   This study evaluated the Impact on Sibling scale, a six-item measure of parents' perception of the effects of a child's illness on healthy siblings.
Methods   Participants were 122 parents of a child with chronic illness, developmental disability, or autism spectrum disorder, and a well sibling aged 4–13 years. Parents completed the Impact on Sibling scale and the Child Behavior Checklist about the sibling, and completed the revised Impact on Family scale and the Brief Symptom Inventory about themselves.
Results   The Impact on Sibling score was correlated with measures of sibling, parent and family functioning. The internal consistency of the Impact on Sibling scale was higher for families with children with chronic illness compared with the other two diagnostic groups.
Conclusion   The Impact on Sibling scale is a brief set of items that can help identify siblings who are negatively affected by a brother/sister's illness. Findings support further research on the Impact on Sibling scale, particularly with families of children with chronic illnesses.     

When work strain transcends psychological boundaries: an inquiry into the relationship between time pressure,irritation, work–family conflict and psychosomatic complaints

Against the theoretical background of the effort–recovery model and the action regulation theory, the author presents a cross‐sectional questionnaire study testing hypotheses about the relationship between work‐related time pressure, cognitive and emotional irritation, work–family conflict and psychosomatic complaints. Subjects were 576 female home care nurses. Results of a path analysis show that the relation of time pressure and psychosomatic complaints is partially mediated by experiencing a work–family conflict; also the relation of time pressure and work–family conflict is partially mediated by cognitive and emotional irritation. It is argued that cognitive and emotional irritation are fruitful concepts for a more comprehensive understanding of the relationship between work stressors and the development of strain‐based work–family conflict. Implications for the prevention of work–family conflict are outlined. Copyright © 2008 John Wiley & Sons, Ltd.