A New Variant of Hereditary Hemolytic Anemia With Stomatocytosis and Erythrocyte Cation Abnormality

A new variant of congenital hemolyticanemia associated with stomatocytosis,reticulocytosis, decreased osmotic fragility, type I autohemolysis and shortened erythrocyte survival without specific splenic sequestration was discoveredin three siblings of Swiss-German ancestry. Increased intracellular sodium(two to three times normal) and slightlydecreased intracellular potassium weredetected. Total sodium efflux was eight-fold greater than normal but total potassium influx was normal and ouabain-sensitive potassium influx was decreased.The ouabain-sensitive sodium efflux:potassium influx ratio was 26:1 ratherthan the 3:2 ratio noted in normal cells.The consanguineous parents, four othersiblings, and 44 other family membershad mild stomatocytosis, reticulocytosis,and, when studied, decreased osmoticfragility, increased autohemolysis, intermediate abnormalities of cation content,cation flux, and moderate shortening oferythrocyte survival. Autosomal dominant inheritance was suggested. Noabnormalities of RBC enzymes, hemoglobin or lipids were observed. No abnormalities of membrane protein weredetected on acrylamide gel. Substratedepletion of these hypermetabolic cellsresulted in intracellular dehydrationwith potassium loss in excess of sodiumgain and decreased deformability. Although the exact nature of the defectresponsible for hemolysis is unknown,this syndrome differs from other hereditary hemolytic anemias associated withstomatocytosis.

Submitted on December 21, 1970 Revised on March 16, 1971 Accepted on March 29, 1971     

TREATMENT OF SUPERFICIAL MYCOSES IN THE TROPICS: WHITFIELD'S OINTMENT VERSUS CLOTRIMAZOLE

Background. In tropical primary health care, essential drugs should be safe, effective, and as inexpensive as possible. To treat the very common dermatophyte infections of the skin, one may use inexpensive Whitfield's preparations, more expensive topical imidazole derivatives, or extremely expensive oral antifungals. Because a cream base is felt to be more appropriate than an ointment in tropical conditions, we wanted to compare the effectiveness of Whitfield's cream and a topical imidazole derivative in field conditions in the tropics. Methods. A double-blind trial was performed involving 153 patients with a dermatophyte infection of the skin in Karonga District, Northern Malawi, including 25 patients who were Hiv-i-seropositive, comparing Whitfieid's cream with clotrimazole cream. Results. 75 patients were treated with Whitfield's cream and 78 with clotrimazole cream for a period of 6 weeks. Cure rates ranged from 80% to over 90% depending on the definition of cure. If positive cultures after treatment were used as criterion for treatment failure, six were found in each treatment group. One in each treatment failure group was an mv-i-seropositive patient. Conclusions. The great majority of patients in the tropics with a dermatophyte infection of the skin can be cured with a topical antimycotic preparation and do not need expensive oral therapy. This also proved to be valid for HIV-I-seropositive patients. Whitfield's cream and clotrimazole cream are both very effective. The lower cost makes Whitfield's cream the treatment of choice in dermatophyte infections of the skin in tropical primary health care.     

Left Septal Atrial Tachycardias: Electrocardiographic and Electrophysiologic Characterization of a Paraseptal Focus

Left Septal Atrial Tachycardias. Objective: The objective was to characterize the electrocardiographic and electrophysiological features of focal atrial tachycardia (FAT) originating from the left septum (LS). Background: FAT is recognized to occur at predefined anatomic locations rather than randomly throughout the atria. We describe the ECG and EP features of ATs originating from the LS as an important site for apparent perinodal tachycardias. Methods: Nine patients presenting with LS FAT from a consecutive series of 384 underwent EP/RFA for symptomatic FAT. Results: The mean age was 56 ± 12 years; 7 female with symptoms for 36 ± 28 months. P wave morphology (PWM) was negative/positive in lead V1 and across the precordial leads and negative or negative/positive in inferior leads in all patients. Tachycardia was incessant in 6 out of 9 patients with a mean tachycardia cycle length 421 ± 56 milliseconds. His A was ahead of P wave in all patients (mean ?15 ± 5 milliseconds) and earlier than CS proximal (mean 4 ± 9 milliseconds). Successful acute focal ablation achieved at a mean of 31 ± 12 milliseconds ahead of P wave with no recurrences at a mean follow‐up of 30 ± 28 months. Conclusion: Although the left septum is an uncommon site for focal AT an awareness of this location for harboring foci is particularly important when mapping apparently right‐sided septal tachycardias. (J Cardiovasc Electrophysiol, Vol. 24, pp. 413‐418, April 2013)     

Pulmonary Vein Isolation Alone in Patients with Persistent Atrial Fibrillation: An Ablation Strategy Facilitated by Antiarrhythmic Drug Induced Reverse Remodeling

PVI Alone in Patients with Persistent AF . Introduction: Pulmonary vein isolation (PVI) alone has been thought to be insufficient in patients with persistent atrial fibrillation (PersAF). We hypothesized that preablation treatment of PersAF with a potent antiarrhythmic drug (AAD) would facilitate reverse atrial remodeling and result in high procedural efficacy after PVI alone. Methods and Results: Seventy‐one consecutive patients (59.4 ± 9.8 years) with PersAF and prior AAD failure were treated with oral dofetilide (768 ± 291 mcg/day) for a median of 85 days pre‐PVI. P‐wave duration (Pdur) on ECG was used to assess reverse atrial remodeling. Thirty‐five patients with paroxysmal (P) AF not treated with an AAD served as controls. All patients underwent PVI alone; dofetilide was discontinued 1–3 mos postablation. In the PersAF patients, the Pdur decreased from 136.3 ± 21.7 ms (assessed postcardioversion on dofetilide) to 118.6 ± 20.4 ms (assessed immediately prior to PVI) (P < 0.001). In contrast, no change in Pdur (122.6 ± 11.5 ms vs. 121.3 ± 13.7 ms, P = NS) was observed in PAF patients. The 6 and 12 mos AAD‐free response to ablation was 76% and 70%, respectively, in PersAF patients, similar to the 80% and 75%, response in PAF patients (P = NS). A decline in Pdur in response to dofetilide was the only predictor of long‐term clinical response to PVI in patients with PersAF. Conclusions: Pre‐treatment with AAD resulted in a decrease in Pdur suggesting reverse atrial electrical remodeling in PersAF patients. This may explain the excellent clinical outcomes using PVI alone, and may suggest an alternative ablation strategy for PersAF. (J Cardiovasc Electrophysiol, Vol. 22, pp. 142‐148, February 2011)     

Titration of Power Output During Radiofrequency Catheter Ablation of Atrioventricular Nodal Reentrant Tachycardia

Radiofrequency lesions in the anterior, superior aspect of the tricuspid annulus result in selective elimination of fast pathway function in patients with typical atrioventricular (AV) nodal reentry tachycardia. This technique is simple and effective, but has been associated with a significant risk of inadvertent complete AV block. The purpose of this study was to compare the safety and effectiveness of two different techniques for radiofrequency catheter ablation of the fast AV nodal pathway. Initially, a fixed power output was used at each target site. This method was compared retrospectively to a newer technique where power output was gradually incremented at each site. Radiofrequency power was initially applied at 10 watts for 10–15 seconds. If no junctional ectopy or a change in PR intervoi was seen, power output was incremented by 2 to 4 watts every 10 to 15 seconds up to a maximum of 30 watts. Thirty seven of 38 (96%) patients treated using this incremental power output were cured of their AV nodal reentry tachycardia. None of these patients developed inadvertent complete AV block. In contrast, 92% of historic controls treated with a fixed power output between 20 and 30 watts achieved a primary success and nine of these 89 (10%) historic controls developed inadvertent complete AV block (P = 0.04). There was no difference in the amplitudes of atrial, His, or ventricular electrograms at the effective sites between the two groups. Conclusions: (1) the anterior approach to radiofrequency catheter ablation of typical AV nodal reentry is associated with a significant risk of inadvertent complete AV block if a fixed power output is used; (2) starting at low power and gradually incrementing the output during radiofrequency energy application reduces the risk of complete AV block; (3) this incremental technique does not compromise efficacy.     

Long-term Survival of VDD Pacing

All patients with VDD systems implanted at a tertiary pacing center were identified from a computer database and data collected on pacing indications, follow-up duration, rate response, reasons for programming changes, and implant P wave amplitudes. Results: 366 implants were identified for which complete data were available for 335 leads implanted in 316 patients. The mean follow-up period was 24.1 months, and age at implant was 73.5 ± 11.8 years. During follow-up, 19 patients died (6%) and 62 (19.6%) were followed elsewhere. Indications for pacing were complete heart block, 56.6%; intermittent AV block, 21.8%; postablation complete heart block, 5.4%; 2:1 AV block, 13%; and others, 3.2%. Two groups: no mode change (NMC, n = 280) and mode change (MC, n = 36) were identified. Reasons for reprogramming in the MC group were as follows: atrial sensing, 11; AF/atrial flutter, 18; chronotropic incompetence, 3; and others 4. Significantly more MC patients had rate response programmed ON (44.4% vs 22.1%, P < 0.05). No significant differences between the two groups were found in other variables, including male gender (55.5% vs 54.6%), length of follow-up (27.1 ± 17.8 vs 23.8 ± 20.6 months), age at last follow-up (72 ± 12.3 vs 75.9 ± 11.9years), and P wave amplitude (1.7 ± 0.9 vs 1.8 ± 0.9mV). Conclusion: Reprogramming of VDD systems is infrequent. When necessary, it is usually prompted by atrial arrhythmias or failure of atrial sensing. When adequate atrial chronotropy has been verified, VDD is an acceptable alternative to DDD pacing and survives well over the long term.