Intrahepatic cholestasis in sickle cell disease: A review of diagnostic criteria, treatments, and case reports.

Objective To delineate the etiology, symptomatology, and treatment of sickle cell intrahepatic cholestasis (SCIC). Sickle cell disease (SCD) is the most frequently inherited hematologic disease, and SCIC is one rare and often fatal complication and comorbid disease. The literature contains only a small number of case reports involving SCIC and hence limited guidance can be obtained. Methods We reviewed the scientific literature to evaluate the science of SCIC to determine if there were consistencies in presentation, evaluation, treatment, and clinical outcomes. Results We reviewed 6 case reports and a limited number of clinical papers on SCIC. We reported consistencies in clinical presentation and treatment outcomes among cases as well as serological and hematological finding. Conclusions While there is some consistency in the symptom presentation of individuals with SCIC, reliable evaluation and clinical procedures were not demonstrated in what we reviewed. Further research is needed to delineate the attributes of this complicated disease that occurs within SCD.

Neuropsychological effects and attitudes in patients following electroconvulsive therapy.

The current study examined the effects of electroconvulsive therapy (ECT) on neuropsychological test performance. Forty-six patients completed brief neuropsychological and psychological testing before and after receiving ECT for the treatment of recalcitrant and severe depression. Neuropsychological testing consisted of the Levin Selective Reminding Test (Levin) and Wechsler Memory Scale-Revised Edition (WMS-R). Self-report measures included the Beck Depression Inventory (BDI), the Short-Term Memory Questionnaire (STMQ), and several other measures of emotional functioning and patient attitudes toward ECT. The mean number of days between pre-ECT and post-ECT testing was 24. T-test revealed a significant decrease in subjective ratings of depression as rated by the BDI, t(45) = 9.82, P < 0.0001 (Pre-BDI = 27.9 +/- 20.2; post-BDI = 13.5 +/- 9.7). Objective ratings of memory appeared impaired following treatment, and patients' self-report measures of memory confirmed this decline. More specifically, repeated measures MANOVA [Wilks Lambda F(11,30) = 4.3, p < 0.001] indicated significant decreases for measures of immediate recognition memory (p < 0.005), long-term storage (p < 0.05), delayed prose passage recall (p < 0.0001), percent retained of prose passages (p < 0.0001), and percent retained of visual designs (p < 0.0001). In addition, the number of double mentions on the Levin increased (p < 0.02). This study suggests that there may be a greater need to discuss the intermittent cognitive risks associated with ECT when obtaining informed consent prior to treatment. Further that self-reports of cognitive difficulties may persist even when depression has remitted. However, patients may not acknowledge or be aware of changes in their memory functioning, and post-ECT self-reports may not be reliable.

Parental substance abuse, reports of chronic pain and coping in adult patients with sickle cell disease.

There is increasing interest from a social learning perspective in understanding the role of parental factors on adult health behaviors and health outcomes. Our review revealed no studies, to date, that have evaluated the effects of parental substance abuse on reports of chronic pain and coping in adult patients with sickle cell disease (SCD). We explored the effects of parental substance (alcohol or drug) abuse on reports of the sensory, affective and summary indices of pain in 67 adult patients, mean age 38.9 (13.5), with SCD. We also explored the effects of parental substance abuse on psychopathology associated with pain and active coping. Twenty-four percent of patients reported that their parent(s) abused substances. Patients whose parent(s) were characterized as substance abusers reported greater sensory (p=0.02), affective (p=0.01) and summary (VAS; p=0.02) indices of pain as compared to their counterparts, whose parent(s) were not characterized as substance abusers. Patients did not differ in average age, education or the propensity to respond in a socially acceptable manner. There was a significant trend towards patients who characterized their parents as abusers scoring higher than their counterparts on active coping. We propose a Social Learning Theory to explain the current findings and suggest a need for additional prospective research to simultaneously explore biological (genetic) and social factors that influence the interpretation, experience and reporting of chronic pain in adult patients with chronic disease.

Relationships of dissociation and childhood abuse and neglect with heart rate in delinquent adolescents.

This study examined the relationship of dissociative symptoms, abuse and neglect, and gender to mean heart rate (HR) in two types of interviews. Participants were 25 female and 16 male delinquent adolescents. Dissociative symptoms and abuse and neglect were assessed by structured interviews. Participants were randomized to one of two conditions, to describe either their most stressful life experience or their free association thoughts. Greater dissociative symptoms were associated with lower mean HR, whereas abuse and neglect, being a girl, and participating in the free association task were associated with higher mean HR. The finding that high levels of dissociative symptoms may be related to a suppression of autonomic physiological responses to stress support Bremner's conceptualization (J. D. Bremner, 1999) that dissociative symptoms comprise one of two subtypes of the acute stress response, differing physiologically as well as subjectively from a predominantly hyperarousal or intrusive symptom response.