The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement

We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. It should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.      

Electrical stimulation of the medial prefrontal cortex supports both 'pure reward' and 'reward-escape' behavior in rats

In female Sprague-Dawley rats, 8 of 12 medial prefrontal cortex (MPFC) sites that yielded criterion self-stimulation behavior supported only self-stimulation, i.e. were 'pure reward' in type. The remaining 4 sites supported behavior to escape from experimenter-administered stimulation of the same parameters as well, i.e. were 'reward-escape' in type. 'Pure reward' and 'reward-escape' sites in the MPFC were distinguished by both the magnitude and temporal form of the escape response functions generated, and by the prevalence of 'pounce-back', a vigorous and repetitive barpressing during the 3-s MPFC stimulation-escape interval produced by an effective barpress. The finding that both 'pure reward' and 'reward-escape' patterns of behavior can be elicited by stimulation of the MPFC provides a basis for further assessment of similarities and differences in medial prefrontal cortical and lateral hypothalamic (LH) 'reward' systems. It is suggested that 'reward-escape' in the MPFC may be mediated by the activity of 'reward' neurons which respond to stimulus offset, rather than by a secondary aversive process as is proposed to underlie 'reward-escape' in the LH.     

Efficacy of right atrial infusion of PGE1 in sepsis-induced pulmonary hypertension

In this study we investigated the effects of right atrial infusion of PGE1 (RAIPGE1) in doses from 40 to 500 ng/kg/min on sepsis-induced pulmonary artery hypertension (SIPAH). Thirteen pigs were randomized into a time-course group (n = 6) and a PGE1-treated group (n = 7). Pulmonary hypertension (PAH) was induced with the infusion of Pseudomonas Aeruginosa (PsAr) at a concentration of 2 X 10(8) CFU/20 kg/min in both groups. The infusion of PsAr caused a significant and persistent rise in mean pulmonary artery pressure (MPA), pulmonary vascular resistance (PVRI), right ventricular compliance (RVC), RV dp/dt, and right ventricular stroke work index (RVSWI), 30 min after the onset of infusion (P less than 0.05 vs baseline). Systemic hemodynamics and gas exchange were not affected throughout the 3-hr period of infusion (P = NS); however, left ventricular compliance (LVC) was depressed at a MPA greater than 35 mm Hg. The RAIPGE1 following SIPAH caused a concentration-dependent reduction above 40 ng/kg/min of MPA, PVRI, RVSWI, and RV dp/dt (P less than 0.05, 120 and 500 ng/kg/min vs PAH). RVC returned to baseline values during the infusion of PGE1. Systemic hemodynamics, including oxygen delivery and extraction, were unaffected by the infusion of PGE1, but LVC was improved (P less than 0.05, PGE1 500 vs PAH). The infusion of PGE1 caused a concentration-dependent rise in shunt fraction (Qs/Qt) and alveolararterial oxygen gradients which reached statistical significance during the infusion of 500 ng/kg/min. Our data show that RAIPGE1 is effective in ameliorating RV and pulmonary hemodynamics, but at the largest dose it negatively affects gas exchange.     

Improving the quality of life in nursing homes

Physicians can help improve the quality of life for persons residing in long-term care facilities. Physician visits offer opportunities to share knowledge and skills with the staff, to influence nursing home policies and procedures, and to serve as advocates for residents and their families. The physician can ensure good medical care and can encourage practices that enable residents to use their remaining capacities and have maximum autonomy over their own lives.     

Multiple personality disorder. A clinical investigation of 50 cases

To study the clinical phenomenology of multiple personality, 50 consecutive patients with DSM-III multiple personality disorder were assessed using clinical history, psychiatric interview, neurological examination, electroencephalogram, MMPI, intelligence testing, and a variety of psychiatric rating scales. Results revealed that patients with multiple personality are usually women who present with depression, suicide attempts, repeated amnesic episodes, and a history of childhood trauma, particularly sexual abuse. Also common were headaches, hysterical conversion, and sexual dysfunction. Intellectual level varied from borderline to superior. The MMPI reflected underlying character pathology in addition to depression and dissociation. Significant neurological or electroencephalographical abnormalities were infrequent. These data suggest that the etiology of multiple personality is strongly related to childhood trauma rather than to an underlying electrophysiological dysfunction.     

STUDIES ON ANTIBODY PRODUCTION : VII. THE EFFECT OF 5-BROMODEOXYURIDINE ON THE IN VITRO ANAMNESTIC ANTIBODY RESPONSE

Incorporation of 5-bromodeoxyuridine (BUDR) in the culture medium for the 2nd, 3rd, or 4th day after in vitro antigen stimulation of rabbit popliteal lymph node fragments suppressed the in vitro anamnestic antibody response described previously. Before or after this 3-day period, BUDR had no measurable effect. The results suggest that the antibody response in this in vitro system depends upon cell multiplication during this period.     

Relative importance of dimensions in the assessment of health-related quality of life for patients with hypertension.

There are many different approaches to quality of life measurement. Most of these methods consider several dimensions of health status. However, few of the current methods evaluate the relative importance of the various dimensions. We suggest that the relative importance dimension is of central importance in the development of a general theoretical conceptualization of health outcome. Further, we offer a General Health Policy Model that can be used to evaluate outcomes in cardiovascular care and to compare the cost/utility of cardiovascular interventions to investments in other aspects of health care. Employing the relative importance concept requires several important assumptions and methodological challenges. For example, we must select between competing measurement methodologies and we must decide whose values to use in the models. As new research addresses these questions, we expect greater use of the models in patient decision making and in health policy research.     

Long-term follow-up of children breast-fed by mothers receiving depot-medroxyprogesterone acetate

A long-term follow-up study compared development and health of 128 breast-fed children whose mothers had received depotmedroxyprogesterone acetate (depot-MPA) while lactating and 142 control children whose mothers had used mechanical contraceptives or no contraceptives or had undergone sterilization. The children, who were approximately 4-1/2 years old at follow-up, showed no ill effects on their growth and development and health status from exposure to depot-MPA. Depot-MPA-treated mothers lactated significantly longer than controls and also had greater parity than controls. These factors apparently contributed to a difference in weight at follow-up. Compared with the SempePedron standard, more of the depot-MPA group were underweight and more controls were overweight.