Outcome of gastrointestinal complications after liver transplantation for familial amyloidotic polyneuropathy

BACKGROUND: Gastrointestinal disturbances are important prognostic factors for mortality and morbidity after liver transplantation for familial amyloidotic polyneuropathy (FAP). However, the impact of liver transplantation on malabsorption and bacterial small-bowel contamination has not been evaluated. METHODS: Twenty-three FAP patients were available for the study. They were examined for gastrointestinal disturbances as a part of the evaluation for liver transplantation for FAP. Bile acid malabsorption was diagnosed with the [75Se]-homocholic acid taurate (SeHCAT) test; fat malabsorption by measuring faecal fat excretion; and bacterial small-bowel contamination with the hydrogen breath test (HBT). RESULTS: No significant improvement of malabsorption test results were noted from the pre-transplant evaluation 8 months (range, 2-20 months) before transplantation to the post-transplant evaluation performed a median of 20 months (range, 9-62 months) after the procedure. The SeHCAT test result became abnormal in two patients and normal in one, and changes in the test correlated with the time the patients were waiting for transplantation. Faecal fat excretion after transplantation correlated with duration of the disease and with fat excretion before transplantation. A significantly increased fat excretion was noted at the post-transplant evaluation. A change in HBT result was noted in only one patient, in whom the test result became normal; pre-transplant values correlated with those obtained after transplantation. CONCLUSION: For most FAP patients no improvement in gastrointestinal function was found after transplantation. The finding underlines the importance of an early transplantation before the patients have developed gastrointestinal dysfunction.     

艾滋病合并隐球菌脑膜炎18例临床分析

目的　提高对艾滋病 (AIDS)合并隐球菌脑膜炎的认识。方法　对赤道几内亚巴塔地区医院 18例AIDS合并隐球菌脑膜炎患者进行临床综合分析。结果　 18例AIDS合并隐球菌脑膜炎患者的临床主要表现为 :发热、剧烈头痛、极度乏力、肢体痛、脑膜刺激征及消瘦与脱水等。脑脊液 (CSF)培养均为新型隐球菌生长 ;涂片及隐球菌多糖荚膜抗原 (ELISA法 )检测的阳性率分别为 77 8% (14/ 18) ,94 4% (17/ 18)。结论　隐球菌脑膜炎为AIDS常见机会性感染及主要致死病因之一。     

Balancing high accrual and ethical recruitment in paediatric oncology: a qualitative study of the 'look and feel' of clinical trial discussions

Background  

High accrual to clinical trials enables new treatment strategies to be tested rapidly, accurately and with generalisability. Ethical standards also must be high so that participation is voluntary and informed. However, this can be difficult to achieve in trials with complex designs and in those which are closely embedded in clinical practice. Optimal recruitment requires a balance of both ethical and accrual considerations. In the context of a trial of stratified treatments for children with acute lymphoblastic leukaemia (UKALL2003) we examined how recruitment looked to an observer and how it felt to the parents, to identify how doctors' communication could promote or inhibit optimal recruitment.     

Progression of cardiomyopathy after liver transplantation in patients with familial amyloidotic polyneuropathy, Portuguese type

BACKGROUND: Transthyretin amyloidosis is today an accepted indication for orthotopic liver transplantation (OLT). For several mutations progression of the cardiomyopathy has been observed after OLT. The aim of this study was to assess the course of cardiac involvement in Swedish familial amyloidotic polyneuropathy (FAP), Portuguese type, after OLT. By comparison of the echocardiographic findings before OLT with those obtained after, the course of the heart involvement was followed. METHODS: Twenty-three patients, who had undergone OLT and were examined with echocardiography 1-12 months before OLT, were available for the study. Twenty-one patients were examined 12-27 months after OLT, and 12 were re-examined 52-71 months after OLT. Two-dimensional and M-mode echocardiography were performed in accordance with the standards of the American Society of Echocardiography. RESULTS: A significantly increased septal and left ventricular posterior wall thickness and a significantly increased left atrial dimension was observed at the post-OLT examinations, indicating a progression of the amyloid heart disease. This increase of the cardiac involvement was neither correlated to waiting time for OLT or to pre-operative signs of cardiomyopathy. CONCLUSIONS: Even though the production of the amyloidogenic-mutated transthyretin is stopped by OLT, the cardiomyopathy may progress after the operation even for the Portuguese type of FAP. The increase of the septal and left ventricular posterior wall thickness after OLT is not restricted to patients with signs of left ventricular hypertrophy before the transplantation. The findings have important implications for the follow-up of FAP patients after OLT.     

Presence of autoantibody against ATTR Val30 Met after sequential liver transplantation

BACKGROUND: Recently, sequential liver transplantation has been performed with an explanted liver from a patient with familial amyloidotic polyneuropathy (FAP) because of the shortage of donors. However, metabolism of amyloidogenic transthyretin (ATTR), the pathogenic protein of FAP, has not been well studied in patients who have undergone sequential liver transplantation. The purpose of this study was to examine the changes in serum ATTR levels and to investigate the presence of an autoantibody in patients who underwent sequential liver transplantation with an explanted organ from a patient with heterozygotic FAP (FAP ATTR Val30Met). METHODS: This was a case study performed at the Kumamoto University School of Medicine, Kumamoto, Japan, and Kyoto University School of Medicine, Kyoto, Japan. Intervention occurred by sequential liver transplantation with an explanted FAP patient's liver. Levels of normal TTR and ATTR in the two patients who received the transplanted liver were analyzed by means of an enzyme-linked immunosorbent assay (ELISA) and a matrix-assisted laser desorption/time-of-flight mass spectrometry. In addition, the presence of an autoantibody against ATTR Val30Met was evaluated via ELISA using purified ATTR Val30Met from homozygotic FAP patients' sera. RESULTS: After the operation, the variant TTR levels were unexpectedly lower than levels of normal TTR in serum samples from patients with a transplanted liver from the FAP patient. An autoantibody against the variant TTR was detected on day 3 after the operation in the serum of those patients and continued to be present for at least 2 months after the operation. CONCLUSIONS: An autoantibody against the variant TTR may reduce the serum levels of variant TTR. Although the antibody may play a beneficial role in reducing the pathogenic protein, the long-term effect of the antibody must be investigated further.     

"Diagnosis Threat": the effect of negative expectations on cognitive performance in head injury

The present study examined the effect of negative expectations on neuropsychological test performance. It was hypothesized that having attention called to a history of prior head injury and the potential effects of head injury on cognition would result in diminished neuropsychological test performance relative to individuals with a similar head injury history but who did not have their attention called to their head injury history ('diagnosis threat'). Of 36 participants with a history of mild head injury, 17 were randomly assigned to diagnosis threat and 19 to neutral test directions. The diagnosis threat group performed significantly worse on tests measuring general intellect and memory, but were not different from the neutral group in basic attention or psychomotor speed. The diagnosis threat group rated themselves as putting forth less effort on the neuropsychological battery, and self-rated effort correlated with test performance in that group. Overall, results serve as a reminder that neuropsychological tests are measures of behavior, and thus can be influenced by nonneurological factors.     

Psychological symptoms among persons 50 years of age and older living with HIV disease

Although persons 50 years of age and older account for 10% of all US AIDS cases, the mental health needs of this growing group remain largely overlooked. The current study delineated patterns and predictors of psychological symptoms amongst late middle-aged and older adults living with HIV/AIDS in two large US cities. In late 1998, 83 HIV-infected individuals 50-plus years of age (M = 55.2, Range = 50-69) completed self-report surveys eliciting data on psychological symptomatology, HIV-related life-stressor burden, social support, barriers to health care and social services, and sociodemographic characteristics. Based on the Beck Depression Inventory, 25% of participants reported 'moderate' or 'severe' levels of depression. HIV-infected older adults also evidenced an elevated number of symptoms characteristic of somatization. A hierarchical multiple regression analysis revealed that HIV-infected older adults who endorsed more psychological symptoms also reported more HIV-related life-stressor burden, less support from friends, and reduced access to health care and social services due to AIDS-related stigma. As the impact of HIV on older communities continues to increase, geropractitioners must be prepared to provide care to greater numbers of HIV-infected older adults, a substantial minority of whom will present with complex comorbid physical and mental health conditions.     

Executive function deficits associated with symptoms of schizotypy and obsessive-compulsive disorder

Research suggests that executive dysfunction is seen both in disorders of the schizophrenia constellation and in obsessive-compulsive disorder (OCD), but that the patterns of executive deficits may differ. While disorders of the schizophrenia spectrum reflect impairment of functions such as integration of cognitive activities, a tendency to perseverate, and a failure to notice details (e.g. impairment associated with the dorsolateral prefrontal cortex), OCD may involve problems of impulse control and regulation of behavior, and an inability to maintain cognitive set (e.g. impairment associated with the orbitofrontal cortex). The present study examined differences between high-scorers on a measure of schizotypy, high-scorers on a measure of OCD, high-scorers on both schizotypy and OCD and a control group in performance on executive function tests. As expected, the OCD group demonstrated relative deficits on measures thought to reflect orbitofrontal functioning. However, contrary to expectations, the schizotypal group did not demonstrate deficits on neuropsychological tests thought to reflect dorsolateral prefrontal cortex functioning, and the combined group showed no executive impairment.     

Exaggeration index for an expanded version of the auditory verbal learning test: robustness to coaching

Recent research suggests that effort detection measures based on patterns of neuropsychological performance may be more robust to coaching than traditional effort detection measures. In the present study, we evaluated the Exaggeration Index for an extended version of the Auditory Verbal Learning Test (EI-AVLTX), a recently developed effort detection instrument based on patterns of performance on the AVLT using a simulated malingering paradigm. In two independent samples, the EI-AVLTX was1 found to be relatively sensitive and specific to malingering, and robust to the effects of a warning about malingering detection.