Primary Immunodeficiency Diseases in Latin America: The Second Report of the LAGID Registry

This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras, Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reveals that from a total of 3321 patients registered, the most common form of primary immunodeficiency disease was predominantly antibody deficiency (53.2%) with IgA deficiency reported as the most frequent phenotype. This category was followed by 22.6% other well-defined ID syndromes, 9.5% combined T- and B-cell inmunodeficiency, 8.6% phagocytic disorders, 3.3% diseases of immune dysregulation, and 2.8% complement deficiencies. All countries that participated in the first publication in 1998 reported an increase in registered primary immunodeficiency cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic granulomatous disease, and severe combined immunodeficiency between the first report and the present one shows an increase in the reporting of these diseases in all countries. In this report, the estimated minimal incidence of chronic granulomatous disease was between 0.72 and 1.26 cases per 100,000 births in Argentina, Chile, Costa Rica, and Uruguay and the incidence of severe combined immunodeficiency was 1.28 and 3.79 per 100,000 births in Chile and Costa Rica, respectively. However, these diseases are underreported in other participating countries. In addition to a better diagnosis of primary immunodeficiency diseases, more work on improving the registration of patients by each participating country and by countries that have not yet joined LAGID is still needed. Latin American Group for Primary Immunodeficiency Diseases     

Effects of Septal Pacing on P Wave Characteristics: The Value of Three-Dimensional Echocardiography

SZILI-TOROK, T., et al .: Effects of Septal Pacing on P Wave Characteristics: The Value of Three-Dimensional Echocardiography. Interatrial septum (IAS) pacing has been proposed for the prevention of paroxysmal atrial fibrillation. IAS pacing is usually guided by fluoroscopy and P wave analysis. The authors have developed a new approach for IAS pacing using intracardiac echocardiography (ICE), and examined its effects on P wave characteristics. Cross-sectional images are acquired during pullback of the ICE transducer from the superior vena cava into the inferior vena cava by an electrocardiogram- and respiration-gated technique. The right atrium and IAS are then three-dimensionally reconstructed, and the desired pacing site is selected. After lead placement and electrical testing, another three-dimensional reconstruction is performed to verify the final lead position. The study included 14 patients. IAS pacing was achieved at seven suprafossal (SF) and seven infrafossal (IF) lead locations, all confirmed by three-dimensional imaging. IAS pacing resulted in a significant reduction of P wave duration as compared to sinus rhythm (   99.7 ± 18.7   vs   140.4 ± 8.8  ms; P < 0.01   ). SF pacing was associated with a greater reduction of P wave duration than IF pacing (   56.1 ± 9.9   vs   30.2 ± 13.6  ms; P < 0.01   ). P wave dispersion remained unchanged during septal pacing as compared to sinus rhythm (   21.4 ± 16.1   vs   13.5 ± 13.9  ms; NS   ). Three-dimensional intracardiac echocardiography can be used to guide IAS pacing. SF pacing was associated with a greater decrease in P wave duration, suggesting that it is a preferable location to decrease interatrial conduction delay. (PACE 2003; 26[Pt. II]:253–256)     

Coronary Sinus Aneurysm and Left Ventricular‐Coronary Sinus Fistula. Implantation of CRT Device

We describe a 72‐year‐old man referred for implantation of a cardiac resynchronization therapy device who had previously undergone repeated operations to replace the mitral valve. Retrograde venography of the coronary sinus (CS) to implant the left ventricular (LV) pacing lead revealed aneurysmal dilatation of the CS with LV‐CS fistula that hindered—but did not prevent—complete implantation of the system. (PACE 2013; 36:e38–e40)     

Usual sleep duration and cognitive function in older adults in Spain

The few studies that have examined the association between usual sleep duration and cognitive function have shown conflicting results. This cross‐sectional study examined the association between sleep duration and cognitive function among 3212 people, representative of the non‐institutionalized population aged 60 years and over in Spain. Sleep duration was self‐reported, and cognitive function was measured with the Mini‐Examen Cognoscitivo (MEC), a version of the Mini‐Mental State Examination that has been validated in Spain. Linear regression, with adjustment for the main confounders, was used to obtain mean differences in the MEC between the categories of sleep duration (≤5, 6, 7, 8, 9, 10, ≥11 h day^?1). The MEC score decreased progressively (became worse) across sleep categories from 7 to ≥11 h (P for linear trend <0.001). People who slept for ≥11 h had a significantly lower MEC score than those who slept for 7 h (mean difference ?1.48; 95% confidence interval ?2.12 to ?0.85). This difference in the MEC was similar to that observed for a 10‐year increase in age. The results did not vary significantly by sex (P for interaction >0.05). No association was observed between short sleep duration (<7 h) and cognitive function. We conclude that long sleep duration is associated with poorer cognitive function in older adults from the general population.     

Bacteraemia as Predictor of HIV Infection in African Children

ABSTRACT. In Rwanda, both HIV infection and bacteraemia represent major health problems among paediatric populations. We carried out a prospective study to determine if bacteraemia is a marker of HIV infection among ambulatory and hospitalized Rwandese children. All children presenting at the Department of Paediatrics of the Centre Hospitalier de Kigali and who had their blood cultured during a two-month period were eligible for the study. One hundred and thirty-five children were included in the study. A pathogen was isolated from 36 children (26.7 %): S. typhimurium (10 cases), S. enteritidis (6), S. typhi (4), Str. pneumoniae (9). H. influenzae (6) and S. aureus (1). No association was found between bacteraemia and HIV seropositivity when all the children were considered. However, among patients less than 2 years old, bacteraemic subjects were more frequently ( p ≤0.05) HIV seropositive (44 %) than those with negative blood cultures (19 %). Our study shows that in young children in Central Africa, the presence of bacteraemia may be an important marker of HIV seropositivity.     

Chronic fatigue syndrome: a clinical and laboratory study with a well matched control group

Abstract. Objective. To investigate the relation between severity of complaints, laboratory data and psychological parameters in patients with chronic fatigue syndrome (CFS). Subjects. Eighty-eight patients with CFS and 77 healthy controls matched for age, sex and geographical area. Methods. Patients and controls visited our outpatient clinic for a detailed medical history, physical examination and psychological tests: Checklist Individual Strength (CIS), Beck Depression Inventory (BDI) and Sickness Impact Profile (SIP). Venous blood was drawn for a complete blood cell count, serum chemistry panel, C-reactive protein and serological tests on a panel of infectious agents. Results. All patients fulfilled the criteria for CFS as described by Sharpe et al. (J R Soc Med 1991; 84 : 118–21), only 18 patients (20.5%) fulfilled the CDC criteria. The outcome of serum chemistry tests and haematological tests were within the normal range. No significant differences were found in the outcome of serological tests. Compared to controls, significant differences were found in the results on the CIS, the BDI, and the SIP. These results varied with the number of complaints (CDC criteria). When the number of complaints was included as the covariate in the analysis, there were no significant differences on fatigue severity, depression, and functional impairment between patients who fulfilled the CDC criteria and patients who did not. Conclusion. It is concluded that the psychological parameters of fatigue severity, depression and functional impairment are related to the clinical severity of the illness. Because the extensive panel of laboratory tests applied in this study did not discriminate between patients and controls, it was not possible to investigate a possible relation between the outcomes of psychological and laboratory testing.