Visual evoked potentials in NIDDM: a longitudinal study

Summary In order to assess the possible progression of neurological abnormalities over time and the value of visual evoked potential alterations in predicting stability and severity of diabetes-related optic pathway disease, a longitudinal study in non-insulin-dependent diabetic patients was performed. Neurological examination, visual evoked potentials with pattern reversal, motor and sensory nerve conduction velocities and metabolic control were studied in 18 non-insulin-dependent diabetic patients and in 35 normal control subjects at baseline and again after 4.6±0.8 years (range 4–6). At the first recording the peak P100 wave latencies were significantly delayed in the diabetic patients compared with the control subjects; signs of peripheral neuropathy were detected in five patients, clinical in three and in two there was only neurophysiological alteration without clinical signs. The second recording revealed no significant alterations of P100 latencies in patients compared with baseline, but the number with clinical signs and/or neurophysiological alterations with no clinical signs of peripheral neurological disease was increased to seven. In conclusion, we observed that visual evoked potential alterations were stable over time whereas peripheral neurological disease progressed and correlated positively with metabolic control.Abbreviations VEP-PR Visual evoked potentials with pattern reversal - NCV nerve conduction velocities - LP100 peak latencies of P100 wave - ILD interocular latencies difference - NIDDM non-insulin-dependent diabetes mellitus - ARI aldose reductase inhibitor     

Normalization of menstrual cycles and pregnancy after liver transplantation

The objective of the present study is to evaluate the effects of successful liver transplantation on menstrual cycles abnormalities and on reproductive function of women with chronic liver disease. Twelve women with age between 17 and 54 years who underwent liver transplantation were evaluated. The following variables were analyzed: age, etiology of chronic liver disease, pattern of menstrual function and period of amenorrhea before and after transplantation, and occurrence of pregnancy after transplantation. The mean age of patients was 36 +/- 12.6 years. Patients with primary biliary cirrhosis did not have menstrual abnormalities before transplantation. The other patients presented amenorrhea for 3 months to 11 years before the transplantation. Rapid recovery of menstrual function was observed in all patients after the transplantation (3.1 +/- 1.2 months). Two patients became pregnant one and three years after the transplantation. It is concluded from this study that most women who present amenorrhea secondary to chronic liver disease have normal menstrual cycles in approximately three months following liver transplantation and they may become pregnant.     

Familial Amyloidotic Polyneuropathy: domino liver transplantation

BACKGROUND/AIMS: The primary cause of Familial Amyloidotic Polyneuropathy is a variant transthyretin gene on chromosome 18. Progressive polyneuropathy followed by fatal cardiac and renal failure commonly manifest during middle age. Within 10 years after onset of clinical symptoms, affected individuals usually die due to malnutrition or heart failure. Currently, liver transplantation is the only available therapeutic option. METHODS: We performed liver transplantation in two patients with Familial Amyloidotic Polyneuropathy carrying the transthyretin-30 mutant. Two patients aged more than 50 years received the two explanted amyloidotic livers. This procedure is called Domino liver transplantation. We report the outcome in the studied subjects and analyze the metabolic consequences of this procedure. RESULTS: We determined the serum half-life of transthyretin-30 as 2.25 days using daily monitoring of transthyretin-30 levels. An affected amyloidotic patient had an increased serum concentration of lipoprotein(a) of 78 mg/dl before transplantation. The tumor patient, who received the organ from this affected patient, developed an almost identical serum concentration of lipoprotein(a) after liver transplantation, confirming the liver as the primary site of synthesis of this lipoprotein. CONCLUSION: Once Domino liver transplantation has been performed, the impact of the liver-dependent metabolism of specific proteins of interest can be studied.     

No evidence of de novo amyloidosis in recipients of domino liver transplantation: 12 to 40 (mean 24) month follow-up.

Domino liver transplantation (DLT) has been performed for selected recipients at several centers, but de novo amyloidosis in recipients of livers from patients with familial amyloid polyneuropathy (FAP) remains a serious concern. AIM: To evaluate the occurrence of de novo amyloidosis in recipients of DLT. PATIENTS AND METHODS: Seven recipients of FAP livers were followed for clinical and electroneuromyographic signs of FAP and also for de novo amyloid deposition in the gut. RESULTS: No signs and symptoms of de novo FAP nor any evidence of amyloid deposits in the gut were observed in recipients of DLT after a mean follow-up of 24 [12-40] months. CONCLUSIONS: Signs and symptoms of FAP do not occur early in recipients of DLT. These livers could therefore be offered to patients suitable for conventional LT, particularly older subjects in whom the event of de novo amyloidosis would seem improbable.     

Long-term results of liver transplantation for Wilson's disease

Wilson's disease is a hereditary defect in hepatic copper metabolism, causing hepatic, neurological and/or psychiatric manifestations. For patients with severe disease, liver transplantation is the treatment of choice. The aim of this study was to report the long-term outcome of patients who underwent liver transplantation for Wilson's disease. PATIENTS AND METHODS: Thirteen patients with Wilson's disease, transplanted in Lyon France between January 1987 and May 2006, were including in this study: eight women and five men, aged eight to 53 years (median 20 years, seven children and six adults). The diagnosis of Wilson's disease was established before liver transplantation. RESULTS: The indication for liver transplantation was chronic (69%) or fulminant liver failure (31%). The median follow-up after liver transplantation was 10 years with 100% patient survival. Copper metabolism returned to normal in all patients. None of the patients with exclusive liver disease required chelation treatment after liver transplantation and none developed neurological symptoms of Wilson's disease. CONCLUSION: Liver transplantation totally reverses the abnormalities of copper metabolism and subsequent hepatic failure, but the course of neurological symptoms remains unpredictable. Long-term patient survival can be excellent without occurrence of neurological complications.     

Partial-liver transplantation to treat familial amyloid polyneuropathy: follow-up of 11 patients.

BACKGROUND: Recently, liver transplantation has been used to treat patients with familial amyloid polyneuropathy (FAP). OBJECTIVE: To describe the clinical course of patients with FAP who received partial-liver transplantation from living donors. DESIGN: Case series. SETTING: University hospital in Matsumoto, Japan. PATIENTS: 11 patients with FAP who underwent partial-liver transplantation. The transthyretin gene abnormality in all 11 patients was the substitution of methionine for valine at position 30. INTERVENTION: Partial liver transplantation from living donors. MEASUREMENTS: Preoperative and follow-up (3 to 64 months) clinical data, including routine laboratory data, nerve conduction velocity tests, and sural nerve histology. RESULTS: All 7 patients who had severe gastrointestinal autonomic disorders or polyneuropathy localized to the lower limbs for less than 4 years showed improvement. Three of 4 patients with polyneuropathy involving both the upper and lower limbs had adverse outcomes, including two deaths. The preoperative duration of their illness was more than 6 years. These 3 patients also had marked decreases in creatinine clearance and nerve conduction velocities and severe loss of myelinated fibers in sural nerves. CONCLUSION: Preoperative clinical severity and duration of illness are associated with outcomes after liver transplantation for FAP.     

Decreased white matter lesion volume and improved cognitive function after liver transplantation

Focal T2-weighted white matter lesions (WML) on brain magnetic resonance imaging (MRI), mimicking those seen in cerebrovascular small-vessel disease described in patients with persistent hepatic encephalopathy, decreased in volume with the improvement of hepatic encephalopathy. This outcome has been interpreted as a decrease in the edema that it is proposed to be involved in the pathogenesis of hepatic encephalopathy. We designed a study to further investigate potential changes in focal WML in the brains of patients with cirrhosis following liver transplantation and to study the relationship between these changes and overall cognitive function. We used MRI to measure the volume of supratentorial focal WML and a neuropsychological examination to assess cognitive function before and after liver transplantation in 27 patients with cirrhosis without signs of overt hepatic encephalopathy. Baseline MRI identified focal T2-weighted lesions in 19 patients (70.3%). The presence of WML was associated with older age but not with vascular risk factors, severity of liver function, or psychometric tests. A significant reduction in lesion volume was observed after liver transplantation (from a median of 1.306 cm(3) to 0.671 cm(3), P = 0.001). This decrease correlated with an improvement in an index of global cognitive function (r = -0.663; P < 0.001). This evolution indicates that lesion volume is partially related to a reversible type of tissue damage, which is compatible with brain edema. CONCLUSION: Focal WML probably induced by age-related microvascular injury can decrease their volume with liver transplantation. The associated improvement of cognitive function supports a relationship between brain edema and minimal hepatic encephalopathy.     

Sexual and reproductive function in female liver transplant recipients

BACKGROUND: Abnormalities in sexual and reproductive functions are common in women with end-stage liver disease and may be reversible after liver transplantation. AIM: Evaluate sexual and reproductive function in female recipients of liver transplantation at the Federal University of Parana, Curitiba, PR, Brazil. PATIENTS AND METHODS: Between September 1991 and December 2001 94 women underwent liver transplantation at "Hospital de Clinicas" of the Federal University of Parana. Twenty-eight female recipients (mean age 44.17 +/- 13.60 years old) fulfilled the following inclusion criteria: age > or = 16 years at liver transplant, post-transplant survival > or = 6 months, be alive and in regular follow-up at our Institution during the period of the study, and agreed to participate of the study. Medical records were reviewed and all subjects were answered by a questionnaire covering the following issues: timing and pattern of menstruation before and after transplant, sexual activity and contraceptive practices before and after transplant, pregnancy after transplant, frequency of cervical cytology and occurrence of gynecological malignant neoplasia after transplant, and aspects of sexuality domain after liver transplantation. RESULTS: The median post-transplant follow-up was 36.5 months (range, 6 to 110 months) and the main indication for liver transplantation was hepatitis C (25%). All patients had normal liver function tests. Excluding six patients who underwent hysterectomy or were in postmenopausal period, 13 of 22 women (59.1%) reported amenorrhea in the year before transplantation. After liver transplantation, 19 of 22 patients (86.4%) promptly recovered menstrual function, after a median period of 1 month (range, 1 to 7 months). Normalization of menstrual function occurred in all women with age 45 or younger. About 71.4% of 28 women were sexually active after transplantation and 70% indicated satisfaction with their relationship. Four successful pregnancies (one gemelar) occurred in three patients and five healthy live-born infants have been delivered, all full term. Most of the patients had cervical cytology at least annually in the post-transplant period. One case of endometrial carcinoma was diagnosed in a 64 year old woman, three years after transplantation and was successfully treated by panhysterectomy. CONCLUSIONS: Most of female liver transplant recipients of child-bearing age recover menstrual function a few months after transplantation and successful pregnancy may occur. As libido and fertility return promptly after liver transplantation, patients should be counseled on safe contraception practices. Most of liver transplant recipients are sexually active and feel satisfied about their relationship. There is a good compliance of patients regarding screening for cervical neoplasm.     

Peripheral nerve function in patients with familial amyloid polyneuropathy after liver transplantation.

To evaluate the therapeutic efficacy of liver transplantation in patients with ATTR Val30Met familial amyloid polyneuropathy (FAP), were repeatedly examined the neurophysiological function of peripheral nerves in nine patients. The maximal motor and sensory conduction velocities (MCV and SCV) of the ulnar and tibial nerves, size of compound muscle action potential (CMAP), terminal latency of CMAP, skin temperature of extremities, CVR-R, blood pressure, heart rate, and Schellong's test were examined before and every 6 months after the operation. Although there were no changes in CVR-R, blood pressure, or heart rate, the skin temperature of foot and hand increased soon after surgery and did not decrease during the period of observation. The temperature-adjusted MCV of tibial nerve gradually increased, but the MCV of ulnar nerve showed no change. The temperature-adjusted tibial nerve SCV worsened slightly soon after transplantation and remained at that level in the distal part. The ulnar nerve SCV worsened and subsequently improved. Liver transplantation is very effective for halting the progression of this type of FAP, but the recovery of peripheral nerve function in patients seems to be very slow and limited, especially the function of large diameter myelinated fibers.     

Liver transplantation in Wilson’s disease: Single center experience from Saudi Arabia

AIM: To determine liver transplantation outcomes in Wilson’s disease (WD) patients, focusing on neurological manifestations.METHODS: This retrospective study assessed data from 16 WD patients (nine males, 56%) who had liver transplants between 1991 and 2007. Survival, graft function, and neurological complications were assessed during a follow-up period of up to 15 years. In addition, each patient’s medical record was reviewed in detail to find the type of Wilson’s disease (hepatic or hepatic plus neurological WD), indication for liver transplantation, use of chelating agents prior to transplantation, immediate and long term complications following transplantation, the donor details, and the pathology of explanted liver.RESULTS: End-stage liver disease was the indication for transplantation in all 16 WD patients. Four patients displayed WD-related neurological symptoms in addition to liver disease. Living-related liver transplantation was done in three cases. One patient died on postoperative day 6 due to primary graft non-function. One-year post liver transplant survival was 94%. Neurological manifestations of all four patients disappeared during their follow-up. Four patients developed acute cellular rejection, but all responded to treatment. One patient developed chronic ductopenic rejection after 15 years post-transplantation and their graft failed; this patient is currently waiting for re-transplantation. Fourteen patients (88%) are still living. The long-term average survival is currently 10.5 years, with a current median survival of 8 years. Long-term graft survival is currently 81%.CONCLUSION: Short- and long-term survival in WD patient liver transplantation was excellent, and neurological and psychological WD manifestations disappeared during long-term follow-up.     

Quality of life in patients with familial amyloidotic polyneuropathy long-term after liver transplantation

Liver transplantation aims to halt the progression of the disease in patients with familial amyloidotic polyneuropathy (FAP) caused by hereditary transthyretin-related (ATTR) amyloidosis. Insight in health-related quality of life of these transplanted FAP-patients can be of help to optimize health care delivery. The aim of this cross-sectional study was to assess the health-related quality of life of patients with FAP long-term after transplantation.

Nine patients with a post-transplant follow-up of 4 years or more were included in the study. During the annual checks, health-related quality of life was measured with the Short Form-36 (SF-36). Data were compared with non-FAP transplanted patients with the same duration of follow-up and with the normal Dutch population. Pre-transplant, all patients had signs of mild to moderate peripheral polyneuropathy.

The results showed that in patients with FAP health-related quality of life was stable in the first 4 years after transplantation. The domain of physical well-being at 4 years after transplantation was significantly lower compared to non-FAP transplanted patients and control Dutch population. The domain of emotional well-being was comparable with non-FAP controls. However, on most health areas patients with FAP scored lower than the non-FAP transplanted patients and the Dutch controls. After four years, the three patients with FAP with longest follow-up (9–12 years) deteriorated in all health domains, except in self-perceived mental health.

This study, including only a small number of patients with FAP, shows a relatively low health-related quality of life after liver transplantation, which may deteriorate further with longer follow-up.     

Neurological complications after orthotopic liver transplantation

BACKGROUND: The number of orthotopic liver transplantation performed each year is increasing due to increased safety and logistic facilities. Therefore, the importance of reducing adverse events is progressively growing. AIM: To review present knowledge on the neurological complications of orthotopic liver transplantation. METHODS: The epidemiology, the clinical features and the pathophysiology of the neurological complications of orthotopic liver transplants, resulting from a systematic review of the literature in the last 25 years, are summarized. RESULTS AND CONCLUSIONS: The review highlights that a relevant variety of neurological adverse events can occur in patients undergoing orthotopic liver transplantation. The knowledge of neurological complications of orthotopic liver transplantation is important for transplantation teams to reduce their prevalence and improve their management. In addition, the likelihood of neurological adverse effects provides evidence for the need of a careful cognitive and neurological work up of patients in the orthotopic liver transplantation waiting list, in order to recognize and interpret neurological dysfunction occurring after orthotopic liver transplantation.     

Early liver transplantation is essential for familial amyloidotic polyneuropathy patients' quality of life.

Nineteen patients, who had undergone liver transplantation for familial amyloidotic polyneuropathy, had answered a quality of life questionnaire including 61 questions on somatic and mental symptoms, social aspects of life, confidence and satisfaction before, one year, and two years after transplantation. We found that patient satisfaction was generally good two years or more after the transplantation. Most of the patients were very or quite satisfied with the result. All of them had the drive to go on and felt hopeful about the future. However, on the second follow-up, 37% of the patients noted that they felt more insecure in their everyday life and there was a significant difference between the two assessments. The diarrhea score became worse between one and two years after the transplantation and was closely related to the duration of the gastrointestinal symptoms and to the duration of the disease before transplantation. The mental symptoms also increased significantly between the evaluations and this related to the severity of the somatic symptoms. Our conclusion is that liver transplantation should be performed before advanced somatic symptoms start to develop in order to improve the patients' chances of a good quality of life following liver transplantation.     

Early liver transplantation is essential for familial amyloidotic polyneuropathy patients' quality of life

Nineteen patients, who had undergone liver transplantation for familial amyloidotic polyneuropathy, had answered a quality of life questionnaire including 61 questions on somatic and mental symptoms, social aspects of life, confidence and satisfaction before, one year, and two years after transplantation.

We found that patient satisfaction was generally good two years or more after the transplantation. Most of the patients were very or quite satisfied with the result. All of them had the drive to go on and felt hopeful about the future. However, on the second follow-up, 37% of the patients noted that they felt more insecure in their everyday life and there was a significant difference between the two assessments. The diarrhea score became worse between one and two years after the transplantation and was closely related to the duration of the gastrointestinal symptoms and to the duration of the disease before transplantation. The mental symptoms also increased significantly between the evaluations and this related to the severity of the somatic symptoms.

Our conclusion is that liver transplantation should be performed before advanced somatic symptoms start to develop in order to improve the patients' chances of a good quality of life following liver transplantation.     

The development of low-grade cerebral edema in cirrhosis is supported by the evolution of (1)H-magnetic resonance abnormalities after liver transplantation.

BACKGROUND/AIMS: Liver failure may cause brain edema through an increase in brain glutamine. However, usually standard neuroimaging techniques do not detect brain edema in cirrhosis. We assessed magnetization transfer ratio and (1)H-magnetic resonance (MR) spectroscopy before and after liver transplantation to investigate changes in brain water content in cirrhosis. METHODS: Non-alcoholic cirrhotics without overt hepatic encephalopathy (n=24) underwent (1)H-MR of the brain and neuropsychological tests. (1)H-MR results were compared with those of healthy controls (n=10). In a subgroup of patients (n=11), the study was repeated after liver transplantation. RESULTS: Cirrhotic patients showed a decrease in magnetization transfer ratio (31.5+/-3.1 vs. 37.1+/-1.1, P<0.01) and an increase in glutamine/glutamate signal (2.22+/-0.47 vs. 1.46+/-0.26, P<0.01). The increase in glutamine/glutamate signal was correlated to the decrease in magnetization transfer ratio and to neuropsychological function. Following liver transplantation, there was a progressive normalization of magnetization transfer ratio, glutamine/glutamate signal and neuropsychological function. Accordingly, correlations between these variables were lost after liver transplantation. CONCLUSIONS: Cirrhotic patients show reversible changes in magnetization transfer ratio that are compatible with the development of low-grade cerebral edema. Minimal hepatic encephalopathy and low-grade cerebral edema appear to be the consequences of the metabolism of ammonia in the brain.     

Subtypes of antimitochondrial antibodies in primary biliary cirrhosis before and after orthotopic liver transplantation

Antimitochondrial antibodies are markers for primary biliary cirrhosis and probably reflect a specific defect in immunoregulation underlying this disease. Antimitochondrial antibodies and their primary biliary cirrhosis-specific subtypes were tested before and up to 6 years after orthotopic liver transplantation. Sera from 31 consecutive patients were tested, 15 patients had primary biliary cirrhosis and 16 non-primary biliary cirrhosis. Antimitochondrial antibodies were investigated under code by immunofluorescence, and primary biliary cirrhosis-specific subtypes were determined by radioimmunoassay (anti-p62, anti-p48) and complement fixation test (anti-M2, anti-M4, anti-M8). Before orthotopic liver transplantation, antimitochondrial antibodies were detected by immunofluorescence in 13 of 15 patients with primary biliary cirrhosis. Of these patients, 12 were positive for anti-p62 and 8 for anti-p48. Ten patients were positive for anti-M2, 4 patients for anti-M4 and 7 patients for anti-M8. Two primary biliary cirrhosis patients and all non-primary biliary cirrhosis patients were negative with all tests. One month after orthotopic liver transplantation, antimitochondrial antibodies titers declined or became negative by antimitochondrial antibodies immunofluorescence, 3 patients became negative by radioimmunoassay for anti-p62 and 1 for anti-p48. With complement fixation test, 4 patients became negative for anti-M2, 2 for anti-M4 and 4 for anti-M8. Antimitochondrial antibody titer reduction observed 1 month after orthotopic liver transplantation remained unchanged in most sera during the following years. A rise was observed in two patients after 4 and 5 years.(ABSTRACT TRUNCATED AT 250 WORDS)     

Long-term outcome of hepatitis C virus infection after liver transplantation

We analyzed the long-term clinical course of 71 patients with RNA-positive hepatitis C virus (HCV) infection after liver transplantation. Patients with reinfection after transplantation for HCV-related liver disease, or de novo infection at transplantation were followed for up to 12 years. Cumulative survival for patients with HCV infection at 2, 5, and 10 years after transplantation was 67%, 62%, and 62%, respectively. It was not significantly different from that in patients transplanted for other nonmalignant diseases without HCV infection. The main factor determining long-term survival was the presence or absence of hepatocellular carcinoma (HCC) at transplantation. The 5-year survival rate for HCV patients with or without HCC was 35% versus 73%, respectively (P < .05). No deaths because of viral hepatitis of the graft were observed. Deaths in the first year after transplantation were caused by infectious complications, cardiovascular problems, or rejection; deaths after more than 12 months were exclusively because of recurrence of HCC. Biochemical and histological evidence of hepatitis was found in the majority of the patients, only 16% had normal alanine aminotransferase (ALT) values throughout. Twenty-two percent of patients complained of symptoms, with hepatitis C being the cause in 82% of these. Two patients lost their HCV-RNA for prolonged, ongoing periods of time. The severity of the posttransplantation hepatitis was unrelated to age, sex, severity of liver disease before transplantation, cold ischemic time of the graft, duration of the operation, transfusions, the number of rejection episodes, or the long-term immunosuppressive regime. Only initial short-term therapy with interleukin 2 (IL2) receptor antibodies adversely influenced inflammatory activity. Viral genotype did not influence the course of the graft hepatitis in our series. Histology showed inflammation in 88% of the biopsies and signs of fibrosis in 24%. Mean ALT values correlated with inflammation but not with fibrosis in the biopsies. Porto-portal bridging was observed in six patients, one patient developed cirrhosis within 2 years after orthotopic liver transplantation (OLT). We conclude that chronic hepatitis develops in the majority of patients with HCV infection after liver transplantation. Carrier states without significant laboratory abnormalities are observed in approximately 16%, biochemical abnormalities without symptoms are seen in 60%, and symptomatic disease develops in a quarter of the patients. The disease course closely resembles that seen in nontransplanted hepatitis C patients. It is generally mild but little over 10% of patients develop signs of fibrosis of the graft during the first decade.(Hepatology 1997 Jan;25(1):203-10)     

Encephalopathy and liver transplantation

Liver transplantation (LT) candidates experience frequently episodic or persistent hepatic encephalopathy. In addition, these patients can exhibit neurological comorbidities that contribute to cognitive impairment in the pre-transplant period. Assessment of the respective contribution of hepatic encephalopathy or comorbidities in the cognitive manifestations is critical to estimate the neurological benefits of restoring liver function. Magnetic resonance imaging and spectroscopy are useful to assess the impact of liver failure or comorbidities. This assessment is critical to decide liver transplant in difficult cases. In the early postoperative period, LT is commonly complicated by a confusional syndrome. The possible role of persisting hepatic encephalopathy in its development has not been clearly established. The origin is usually considered multifactorial and relates to complications following LT, such as infections, rejection, primary liver dysfunction, immunosuppressors, etc.… The diagnosis and treatment is based in the recognition of comorbidities and optimal care of metabolic disturbances. Several studies have demonstrated recovery of cognitive function after LT in patients that have exhibited hepatic encephalopathy. However, some deficits may persist specifically among patients with persistent HE. Other factors present before LT that contribute to a worse neuropsychological outcome after LT are diabetes mellitus and alcohol consumption. Long-term after LT, cognitive function may worsen in relation to vascular risk factors.     

自体骨髓干细胞移植治疗肝衰竭的初步观察

目的观察自体骨髓干细胞移植治疗肝衰竭的初步疗效。方法 12例经常规内科综合治疗无明显效果的肝衰竭患者。在无菌条件下采集骨髓100～200ml,通过密度梯度离心分离纯化出骨髓干细胞,通过介入的方法经肝动脉注射入肝内,静脉输注肝细胞生长素1～2周。观察移植后1、2、3和6月患者症状、体征、肝脏生化和凝血酶原时间等的变化。结果 9例肝衰竭患者的症状、体征改善,白蛋白升高、胆红素降低,肝脏功能改善,所有患者未见明显副作用。结论自体骨髓干细胞移植治疗肝衰竭安全有效。     

Observations on dementias with possibly reversible symptoms

While the evaluation of patients with dementia must also address the possibility of treatable illnesses, recent studies found that reversible diseases are detected in only about 1% of dementia cases. Data on the frequency and evolution of potentially reversible dementias (PRD) in defined clinical settings can be useful in order to optimize diagnostic protocols, thus reducing over-investigation and waste of resources. We reviewed a series of 513 patients (mean age 69.3 +/- 4.2 years, mean education level 7.4 +/- 4.5 years, sex ratio M/F 217/296) referred to a memory clinic by their general practitioner, in order to identify PRD. All the subjects had undergone neurological and neuropsychological examination, and laboratory tests. Patients considered to be demented also underwent CT brain scan. 362 patients (70.6%) met the criteria for dementia. We identified 26 PRD cases (7.2% of dementia cases, 5.1% of the entire sample). In 13 patients (3.6% of dementia cases), a complete clinical and neuropsychological reversal of dementia was seen after treatment. In 5 of them (1.4%), a partial regression was obtained, while 8 showed no improvement. We conclude that potential and actual reversibility do not coincide, and that "true" reversibility is rare (even if not negligible) in clinical practice. Careful clinical history and examination appeared the most useful part of the evaluation to identify PRD. Standard blood tests and vitamin B12 assay were also useful, while CT scan detected PRD causes only in patients with evidence of neurological signs.