Longitudinal impedance is independent of outflow resistance

BACKGROUND: Many investigators have measured outflow resistance (R) following peripheral bypass procedures, but correlations with graft patency have been weak. This is because the primary determinants of graft patency are the size and quality of the conduit, not its outflow bed. Efforts at separating conduit resistance from outflow resistance have been unsuccessful. Recently, the concept of longitudinal impedance ( integral Z(L)) has been suggested as a measure of conduit resistance independent of outflow resistance. The purpose of this in vitro experiment was to test the hypothesis that integral Z(L) is independent of R within physiologically relevant ranges. METHODS: Rigid polyethylene tubing of known internal diameter and length (4.3 mm, 375 cm) was perfused with a glycerin/saline mixture mimicking the viscosity of blood (4.1 cp), utilizing a variable pulsatile pump and Windkessel, with outflow into multiply branched tubes of decreasing diameter simulating the hemodynamic conditions of arterial bypass. Flow and pressure were measured using ultrasonic transit time and catheter transduction, respectively, and waveforms digitized at 200 Hz. Flow was varied while maintaining "systemic" pressure and resistance. After Fourier transformation, integral Z(L) was calculated as deltaP/Q at each harmonic and integrated over 4 Hz. RESULTS: integral Z(L) calculations were remarkably reproducible within the same day with a coefficient of variation (CV) = 4.0% (at 100 dyne. s/cm(5); n = 4) or over 4 successive days (CV = 4.3%). Furthermore, integral Z(L) was largely independent of R over the physiologic range tested, with integral Z(L) remaining relatively constant as R was increased sixfold. CONCLUSION: integral Z(L) is a consistent and reproducible measure of conduit resistance independent of R over a wide physiologic range. It may be useful for measuring the adequacy of bypass graft conduits.     

Surveillance methods to monitor the impact of HIV therapy programmes in resource-constrained countries

To monitor the collective national impact of initiatives to expand the availability of HIV therapy including antiretroviral treatment (ART) countries need to monitor the proportion of HIV-infected individuals who are receiving HIV therapy, whether morbidity is decreasing, and HIV-infected individuals are experiencing increased survival, and if there is an overall decrease in the number of individuals dying of HIV. However, in many resource-constrained countries these data are limited or unavailable. Morbidity surveillance relies primarily on AIDS case reporting, but severe under-reporting limits the usefulness of these data. A variety of AIDS case definitions are in use and case definitions do not concur with clinical staging definitions. Harmonizing AIDS case definitions with clinical staging, providing resources and training to improve reporting, and using other surveillance systems, such as tuberculosis programme data to monitor morbidity are urgently needed. A cohort analysis of individuals in ART programmes to follow the progress and outcomes of these patients longitudinally is important to monitor quality of care and impact. Because the rapid scale-up of ART programmes may result in HIV drug resistance, surveillance for drug resistant viruses is also required. Very few resource-constrained countries have well-functioning vital registration systems to assess mortality trends and cause-specific mortality. Alternative approaches to measuring mortality trends, such as sample vital registration with verbal autopsy should be considered. Strong commitments from governments, international organizations and other partners are needed to establish and strengthen the HIV morbidity and mortality monitoring surveillance systems.     

Transplantation of Hematopoietic Stem Cells for Primary Immunodeficiencies in Brazil: Challenges in Treating Rare Diseases in Developing Countries

The results of hematopoietic stem cell transplant (HSCT) for primary immunodeficiency diseases (PID) have been improving over time. Unfortunately, developing countries do not experience the same results. This first report of Brazilian experience of HSCT for PID describes the development and results in the field. We included data from transplants in 221 patients, performed at 11 centers which participated in the Brazilian collaborative group, from July 1990 to December 2015. The majority of transplants were concentrated in one center (n?=?123). The median age at HSCT was 22 months, and the most common diseases were severe combined immunodeficiency (SCID) (n?=?67) and Wiskott-Aldrich syndrome (WAS) (n?=?67). Only 15 patients received unconditioned transplants. Cumulative incidence of GVHD grades II to IV was 23%, and GVHD grades III to IV was 10%. The 5-year overall survival was 71.6%. WAS patients had better survival compared to other diseases. Most deaths (n?=?53) occurred in the first year after transplantation mainly due to infection (55%) and GVHD (13%). Although transplant for PID patients in Brazil has evolved since its beginning, we still face some challenges like delayed diagnosis and referral, severe infections before transplant, a limited number of transplant centers with expertise, and resources for more advanced techniques. Measures like newborn screening for SCID may hasten the diagnosis and ameliorate patients’ conditions at the moment of transplant.     

Anti-arrhythmia efficacy of propafenone in children. Apropos of 30 cases

Thirty children aged from 3 months to 20 years were treated with propafenone 250 to 650 mg/m2 divided into 2 to 4 daily doses, for a mean period of 14 months (range: 4 days to 5 years); 8 had chronic atrial tachycardia, 9 had junctional arrhythmia and 13 had ventricular arrhythmia. There were 17 good results (suppression of the arrhythmia), 7 fair results (good clinical effect but partial persistence of the arrhythmia) and 6 failures, either because the drug proved ineffective (3 cases) or on account of side-effects (3 cases). In the treatment of chronic atrial tachycardia propafenone seemed to be more effective than amiodarone in 3 cases and as effective as that drug in 2 cases. In junctional arrhythmia propafenone was certainly effective but unpredictably so (3 good results, 2 fair results, 4 failures). Among ventricular arrhythmias, ventricular tachycardia in bursts was the one which benefited most regularly from treatment with propafenone: the results in 8 patients were better than those obtained with other antiarrhythmic agents (class I drugs, beta-blockers, calcium antagonists); only amiodarone proved superior to propafenone in this type of arrhythmia. Despite a 27% incidence of side-effects, propafenone was generally well tolerated by the children, with no significant gastrointestinal disorders. No depressive effect on the myocardium was noted in 6 children with moderate heart failure well controlled by digitalis and diuretics. However, since overdosage may cause severe disorders of conduction with widened ventriculogram, we recommend regular ECG monitoring during the first 3 days of treatment at least: although there is little slowing down of sinus rate (12%) and little modification of the slow phase under treatment, serious toxicity is possible. Thus, propafenone is a drug that should be handled with caution, but it constitutes a major addition to the range of antiarrhythmic agents which can be used in paediatrics.     

Unhealthy Weight‐control Behaviours,Dieting and Weight Status: A Cross‐cultural Comparison between North American and Spanish Adolescents

The aim of the current study was to examine and compare dieting and unhealthy weight‐control behaviours (UWCB) in population‐based samples in two large urban areas in Spain (Barcelona) and in the USA (Twin Cities of Minneapolis and St. Paul, Minnesota). Additionally, use of UWCB across weight categories was explored in both samples. Participants included 1501 adolescents from Barcelona (48% girls, 52% boys) and 2793 adolescents from the Twin Cities (53% girls, 47% boys). The main outcome measures were dieting, UWCB (less extreme and extreme) and weight status. Although dieting and UWCB were prevalent in both samples, particularly among girls, the prevalence was higher in the US sample. In both countries, the report of dieting and use of UWCB was highest among overweight and obese youth. Prevention interventions that address the broad spectrum of eating and weight‐related problems should be warranted in light of the high prevalence and co‐occurrence of overweight and unhealthy weight‐related behaviours. Copyright © 2012 John Wiley & Sons, Ltd and Eating Disorders Association.     

Genetic aspects of familial multiple myeloma

To elucidate the role of genetics in familial multiple myeloma, two sisters having plasma cell dyscrasia were studied. The women were 58 and 56 years old, and the diagnoses were made 22 months apart. Specific antisera for patient 1's lambda light chains produced in rabbits had no cross-reactivity with her sister's lambda light chains. Karyotypic analysis by G.T.G. banding revealed abnormalities in both patients, but no common abnormalities. HLA typing disclosed identical tissue types (AW24, A26, B13, BW55). An immunologic epidemiologic study performed on 26 family members, encompassing four generations, disclosed no additional cases of paraproteinemia.