Fabry disease with early-onset ventricular dilation: A case report.

RATIONALE: The most common cardiac involvement of Fabry disease (FD) is left ventricular hypertrophy (LVH), which usually occurs in male patients over the age of 30. In rare cases, it can progress to ventricular dilation in the late stage of the disease. PATIENT CONCERNS: A 16-year-old boy presenting with recurrent extremity pain and chest distress was admitted to our hospital. Imaging examinations revealed ventricular dilation. DIAGNOSIS: α-Galactosidase A enzyme assay and GLA gene sequencing confirmed the diagnosis of FD and revealed a novel mutation c.76_77insT. INTERVENTIONS: The patient was treated using metoprolol (23.75 mg qd) and angiotensin-converting enzyme inhibitor (fosinopril sodium 5 mg qd). He refused enzyme replacement therapy for financial reasons. OUTCOMES: The echocardiography, electrocardiography, renal function, and routine blood and urine tests performed 20 months after the patients discharge from hospital showed no significant changes. The patient reported a slow and gradual decrease in the frequency and degree of pain and chest distress, starting approximately 24 months after discharge. LESSONS: Cardiac involvement of FD can progress rapidly in some cases. Screening for FD should be considered in patients with unexplained ventricular dilation, especially in those with a history of typical FD manifestations.

Quality of life improves in vasovagal syncope patients after clinical trial enrollment regardless of fainting in follow-up.

BACKGROUND: Frequent syncope is linked to poorer health-related quality of life (HRQoL). Recurrent syncope has been observed to reduce in all groups after seeing a syncope expert and enrolling in a clinical trial. It is unknown if HRQoL improves with this reduction in syncope recurrence. OBJECTIVES: We examined the change in HRQoL over time in vasovagal syncope (VVS) patients seen by a syncope expert and enrolled in a trial. We also explored whether change differed with treatment or the frequency of fainting during follow up. METHODS: The Short Form Health Survey (SF36) was completed at baseline (BL), 6 m, and 12 m post-enrollment by VVS patients in the 1st and 2nd Prevention of Syncope Trials, which were multi-centered, randomized, placebo-controlled trials of metoprolol (POST) and fludrocortisone (POST2). Differences in HRQoL at BL, 6 m, and 12 m were analyzed and compared by faints in follow-up and randomization group. RESULTS: Complete study data were available for 143 VVS patients (40 ±â€¯17 years, 62% F). Over 12 months, patients reported improvement in all SF36 dimensions except for bodily pain. Post hoc analyses indicated that differences first occurred between BL and 6 m for all but general health. Fainting in follow-up or drug randomization group did not diminish the improvements. The baseline syncope burden was not different whether patients' HRQoL improved or not. CONCLUSION: HRQoL of VVS patients improves over time after enrolling in a clinical trial, even with recurrent faints or randomization to placebo. Improvements may result from alternative factors, such as interaction with experts or patient adjustment.

Assessment of patients' satisfaction in carotid surgery under local anaesthesia by psychometrical testing--a prospective cohort study.

METHODS: Between July 2008 and December 2008 102 consecutive patients undergoing CEA under local anaesthesia (LA) were prospectively evaluated. All patients were psychometrically assessed by the Hospital Anxiety and Depression Scale (HADS), the EuroQol and the Heidelberg peri-anaesthetic questionnaire (HPQ). Furthermore technical issues of cervical plexus block were assessed. RESULTS: Multivariate analysis with an HPQ sum-score of 98 points as a cut-off level for reduced patients' satisfaction demonstrated that HADS-D scores of >9 (OR: 7.228; p = 0.003), insufficient intra-operative pain control (OR: 3.264; p = 0.0322) and complications due to plexus anaesthesia (OR: 3.794; p = 0.0370) were associated with a low patients' satisfaction in carotid surgery under LA. CONCLUSION: The efficacy of the plexus blockade in terms of pain control and side effects affects patients' satisfaction in carotid surgery under LA. When choosing LA for patients undergoing carotid endarterectomy altered states of anxiety and mood reduce satisfaction in carotid surgery under LA and might compromise patients' suitability for LA.

Opiate addiction in China: current situation and treatments.

BACKGROUND: Historically, China has had extraordinarily high rates of opiate dependence. These rates declined drastically following the 1949 revolution; however, opiate abuse has re-emerged in the late 1980's and has spread quickly since then. AIMS: To describe the current situation of opiate addiction and treatments in China and make some suggestions. DESIGN: A descriptive study based on literature searched from Medline and the China National Knowledge Infrastructure database (1996 to 2004) and hand-picked references. FINDINGS: The number of registered addicts in 2004 was 1.14 million (more than 75% of them heroin addicts), but the actual number is probably far higher. Opiate abuse contributes substantially to the spread of HIV/AIDS in China, with intravenous drug use the most prevalent route of transmission (51.2%). Currently, the main treatments for opiate dependence in China include short-term detoxification with opiate agonists or non-opiate agents, such as clonidine or lofexidine; Chinese herbal medicine and traditional non-medication treatments are also used. Methadone maintenance treatment (MMT) has not been officially approved by the Chinese government for widespread implementation, but some pilot studies are currently underway. CONCLUSION: China faces substantial drug abuse problems that appear to be worsening with time. Opiate dependence is a major threat to the public health and social security of China because of its devastating medical effects, its impact on risk for HIV/AIDS and criminal behaviors, low rates of recovery and high rates of relapse. There is an urgent need to implement MMT and other modern treatments for opiate dependence more widely in China.

Clonidine poisoning--an emerging problem: epidemiology, clinical features, management and preventative strategies.

OBJECTIVE: To ascertain whether the incidence of clonidine poisoning in children has increased given the probable increase in clonidine use for treatment of childhood behavioural disorders. METHODS: Cases of clonidine poisoning requiring hospital admission between 1985-95 inclusive were reviewed and demographic data pertinent to each admission were recorded. A literature review was also performed, with particular emphasis on incidence, clinical presentation and management of clonidine poisoning. RESULTS: There were 14 cases of clonidine poisoning during the specified period eight cases presenting in the last 2 years. These eight children or their siblings had been prescribed clonidine for behavioural disorders. The most common signs at presentation were alteration of conscious state (71%) and bradycardia (50%). Nine children were given activated charcoal while seven cases underwent gastric lavage or induced emesis. Although six children were admitted to intensive care, length of hospital stay was less than 24 h in all cases and all had a favourable outcome. CONCLUSION: We concluded that the incidence of clonidine poisoning had increased over the specified period and that, based on our results, this was likely to be due to an increase in clonidine use in childhood behavioural disorders. Based on our data and that from literature review it was evident that there are inconsistencies in the management of clonidine poisoning and that safety measures, namely packaging and education, are inadequate given the increasing profile of clonidine use.

Cost-minimization analysis of initial antihypertensive therapy in patients with mild-to-moderate essential diastolic hypertension.

In addition to efficacy and safety, the cost of therapy has become an increasingly important factor to consider when selecting drugs to treat patients with mild-to-moderate hypertension. However, acquisition prices alone do not determine the total cost of therapy. To better assess total costs, we conducted a systematic, retrospective, cost-minimization analysis of drugs used to treat 673 patients with newly diagnosed, mild-to-moderate (> 95 to < 110 mmHg) diastolic hypertension between the years 1985 and 1992. Patients included in the study had started antihypertensive monotherapy, and a minimum of one dose titration was required before adding another antihypertensive agent to the regimen. A patient had to have a diastolic blood pressure of < or = 90 mmHg while undergoing therapy to be included in the analysis. Drug classes included diuretics, beta-adrenergic blockers, centrally acting alpha 2-agonists, alpha 1-adrenergic blockers, calcium channel blockers, and angiotensin-converting enzyme (ACE) inhibitors. Costs, adjusted to 1992 price levels, were analyzed for 32 individual agents for each of the following five cost variables: initial drug acquisition, supplemental drug acquisition, laboratory monitoring, clinic visits, and treatment of side effects. Mean total costs per patient for all five variables by drug class were $895 for beta-blockers, $1043 for diuretics, $1165 for centrally acting alpha 2-agonists, $1243 for ACE inhibitors, $1288 for alpha 1-blockers, and $1425 for calcium channel blockers. However, costs within each class varied considerably. Acquisition cost was often a poor predictor of the total cost of treatment. Therefore, acquisition cost must be considered in conjunction with a number of outcome variables to assess the true cost of antihypertensive therapy.

Prescription patterns and costs of antihypertensive drugs in two outpatient clinics: Zürich (Switzerland) and Münster (FRG), 1975-1985.

In the present study the prescription patterns and cost of antihypertensive drugs in two outpatient clinics located in two different countries (Zürich, Switzerland, and Münster, FRG), were analyzed from 1975 till 1985 by using representative random samples of hypertensive outpatients. Throughout the observed period the leading positions were held by diuretics and beta-blockers, whereas central sympatholytics and ganglion blockers nearly disappeared. A rapid increase in the use of calcium antagonists occurred within the last 2 analyzed years. Drugs in fixed combination containing reserpin showed a constant decrease during the observed period, whereas beta-blocker-containing combination drugs increased in both clinics. The comparison between the two clinics revealed only minor differences in the prescription pattern of the various known classes of antihypertensive drugs. However, marked differences were observed within given classes between the preferred products. Both in Zürich and Münster the mean annual drug cost per patient doubled from 1975 to 1985. Especially during the last few years these changes took place with a remarkable rapidity, obviously the result of a more intense promotion of new antihypertensive drugs.

Metastatic pheochromocytoma in pregnancy and fetal biophysical assessment after maternal administration of alpha-adrenergic, beta-adrenergic, and dopamine antagonists.

Metastatic pheochromocytoma, a rare complication of pregnancy, was managed from 30 weeks' gestation until delivery three weeks later with a combination of alpha-adrenergic blockade (Minipres) beta-adrenergic blockade (Timolol), and dopamine synthesis inhibition (Demser). The biophysical parameters of fetal heart rate (FHR) baseline, variability, and reactivity, as well as fetal breathing movements, body movements, tone, and amniotic fluid volume were followed sequentially during this period. A 1450-g growth-retarded infant, who subsequently did well, was delivered by cesarean section; the mother received combined surgical and medical therapy for her metastatic disease in the postpartum period. The initial fetal biophysical alteration observed was a reduction in mean FHR baseline rate; further biophysical test abnormalities appeared only after overt fetal compromise was evident. Sequential multiple parameter biophysical testing in such circumstances appears to be a valid and valuable approach to antepartum management.