Israel Medical Association Journal

background: many electrophysiologists recommend implantable cardioverter defibrillators for patients with Brugada syndrome who are cardiac arrest survivors or presumed at high risk of sudden death (patients with syncope or a familial history of sudden death or those with inducible ventricular fibrillation at electrophysiologic study).

objectives: To assess the efficacy and complications of ICD therapy in patients with Brugada syndrome.

Methods: The indications, efficacy and complications of ICD therapy in all patient with Brugada syndrome who underwent ICD implantation in 12 Israeli centers between 1994 and 2007 were analyzed.

Results: there were 59 patients (53 males, 89.8%) with a mean age of 44.1 years. At diagnosis 42 patients (71.2%) were symptomatic while 17 (28.8%) were asymptomatic. The indications for ICD implantation were: a history of cardiac arrest (n=11, 18.6%), syncope (n=31, 52.5%), inducible VF in symptomatic patients (n=14, 23.7%), and a family history of sudden death (n=3, 0.5%). The overall inducibility rates of VF were 89.2% and 93.3% among the symptomatic and a symptomatic patients, respectively (P=NS). During a follow-up of 4-160 (45+-35) months, all patients (except one who died from cancer) are alive. Five patients (8.4%), all with a history of cardiac arrest, had appropriate ICD discharge. Conversely, none of the patients without prior cardiac arrest had appropriate device therapy during 39+-30 month follow-up. Complications were encountered in 19 patients (32%). Inappropriate shocks occurred in 16 (27.1%) due to lead failure/dislodgment (n=5), T wave oversensing (n=2), device failure (n=1), sinus tachycardia (n=4), and supraventricular tachycardia (n=4). One patient suffered a pneumothorax and another a brachial plexus injury during the implant procedure. One patient suffered a late (2 months) perforation of the right ventricle by the implanted lead. Eleven patients (18.6%) required a reintervention either for infection (n=1) or lead problems (n=10). Eight patients (13.5%) required psychiatric assistance due to complications related to the ICD (mostly inappropriate shocks in 7 patients).

Conclusions: In this Israeli population with Brugada syndrome treated with ICD, appropriate device therapy was limited to cardiac arrest survivors while none of the other patients including those with syncope and/or inducible VF suffered an arrhythmic event. The overall complication rate was high.
 

Background The temporal behavior of the coronary microcirculation in acute myocardial infarction may affect outcome. Diastolic deceleration time and early systolic flow reversal derived from coronary artery blood flow velocity patterns reflect microcirculatory function.

Objectives To assess left anterior descending coronary artery flow velocity patterns using Doppler transthoracic echocardiography after primary percutaneous coronary intervention, in patients with anterior AMI[1].

Methods Patterns of flow velocity patterns of the LAD[2] were obtained using transthoracic echocardiography-Doppler in 31 consecutive patients who presented with anterior AMI. Measurements were done at 6 hours, 36–48 hours, and 5 days after successful PPCI[3]. Measurements of DDT[4] and pressure half times (Pt½), as well as observation for ESFR[5] were performed.

Results In the first 2 days following PPCI, the average DDT, 600 ± 340 msec, were shorter than on day 5, 807 ± 332 msec (P < 0.012). FVP[6] in the first 2 days were dynamic and bidirectional: from short DDT (< 600 msec) to long DDT (> 600 msec) and vice versa. On day 5 most DDTs became longer. Pt½ at 6 hours was not different than at day 2 (174 ± 96 vs. 193 ± 99 msec, P = NS) and became longer on day 5 (235 ± 98 msec, p = 0.012). Bidirectional patterns were also observed in the ESFR in 6 patients (19%) at baseline, in 4 (13%) at 36 hours, and in 2 (6.5%) on day 5 after PPCI.

Background: Dupuytren’s disease is a fibroproliferative disorder of the palmar fascia that can cause disabling digital contractures. The pathogensis of the disease is still unclear, and it afflicts predominantly white males of northern European origin. Gender-related differences of Dupuytren's disease and the distinctive characteristics of the disease in females are not yet well defined.

Objectives: To evaluate and illustrate the distinctive characteristics of Dupuytren's disease in females.

Methods: A retrospective study was performed of all female patients with Dupuytren’s disease seen and followed at our Hand Surgery Unit over a 20 year period. The study group consisted of 48 women (56 hands). The collected data included clinical and epidemiological features on admission, and outcome of surgical intervention.

Results: Of the 48 women (56 hands) with Dupuytren's disease, 23 (26 hands) underwent limited fasciectomy. The average age at presentation was 60.1 years. A few of the patients originated from Asia and Africa. Manifestations and pattern of the disease were nearly comparable to those observed in the male group, except for a slightly higher incidence of proximal interphalangeal joint contracture in female patients. Generally, females expressed less severe contractures on presentation, and a slow progression thereafter. A favorable functional postoperative outcome was observed. Seven patients had minor complications including local hematoma and painful scars. Two patients developed moderate signs of complex regional pain syndrome.

Conclusions: Further investigations are needed to assess the potential role of androgens in the pathogenesis of Dupuytren's disease, and a possible protective role of estrogenic hormones, rendering Dupuytren’s contracture a postmenopausal affliction.
 

Background: Occult breast cancer without clinically or mammographically detectable breast tumor is an uncommon presentation.

Objectives: To assess the role of breast MRI in women with metastatic carcinoma and an occult primary, and to define the MRI characteristics of the primary breast tumor.

Methods: This retrospective study evaluated 20 women with metastatic carcinoma of unknown origin who underwent breast MRI between 2000 and 2006. Four women were excluded, leaving 16 in the study group. Probability of malignancy was assessed according to BIRADS classification. MRI performance in detecting lesions and evaluating disease extent was assessed, with the gold standard being surgical or biopsy pathology.

Results: MRI detected suspicious lesions in 15 patients. Lesion size ranged from 0.4 to 7 cm (median 1.5 cm). MRI detected a single lesion in 6 patients (40%), multifocal disease in 3 (20%), multicentric disease in 4 (27%), and bilateral breast lesions in 2 (13%). In 13 patients MRI depicted the primary breast cancer. Initial treatment was surgical in nine; MRI correctly estimated disease extent in 6 (67%), underestimated disease extent in 1 (11%), and overestimated it in 2 (22%). Four patients had biopsy followed by chemotherapy; one had multicentric disease and one had multifocal disease. MR findings were false positive in two patients and false negative in one.

Conclusions: MRI is sensitive in detecting the primary tumor and beneficial in assessing tumor extent. Small size and multiple foci are common features. We suggest that bilateral breast MRI be part of the evaluation of women with metastatic carcinoma and an occult primary.
 

Background Voiding cystourethrogram is performed 3–6 weeks after urinary tract infection. This prolongs the interval of prophylactics, reducing the likelihood of performing the procedure.

Objectives To investigate the yield and potential risks/benefits of early compared to late-performance VCUG[1] after UTI[2].

Methods We conducted a prospective study of 84 previously healthy children < 5 years old admitted from October 2001 to November 2002 with first documented UTI. We then divided the 78 patients who had VCUG into two groups and compared them to a control group:  group A – 49 children in whom VCUG was performed within 10 days, group B – 29 children in whom VCUG was performed > 10 days after UTI, and a historical control group C – 82 children in whom VCUG was performed > 4 weeks following UTI.

Results VCUG was performed in 48/48 (100%), 6/35 patients (17.1%), 34/116 patients (29.3%) and vesicoureteral reflux was demonstrated in 38.8%, 37.9%, 39% in groups A, B, C respectively. No significant difference was found between these groups in terms of incidence of VUR[3] and severity and grading of reflux within each group. One case of UTI secondary to VCUG occurred in a patient in whom the procedure was performed 4 months after the diagnosis.

Conclusions Performing VCUG early does not influence detection rate, severity of the VUR, or risk of secondary infection; it shortens the period of prophylactic use and increases performance rate of VCUG, thereby minimizing the risk of failure to detect VUR. The traditional recommendation of performing VCUG 3–6 weeks after the diagnosis of UTI should be reevaluated.

Background: A substantial number of premature deliveries occur in hospitals lacking neonatal intensive care facilities. We previously demonstrated a comparable outcome of very low birth weight infants delivered in a level II nursery to that of inborn infants delivered in our tertiary care center, but a similar comparison of extremely low birth weight infants has not been done.

Objectives: To compare the neonatal outcome (mortality, severe intraventricular hemorrhage/periventricular leukomalacia, bronchopulmonary dysplasia and intact survival) of inborn and outborn ELBW[1] infants, accounting for sociodemographic, obstetric and perinatal variables.

Methods: We compared 97 ELBW infants (birth weight ≤ 1000 g.) delivered between the years 2000 and 2004 in a hospital providing neonatal intensive care to 53 ELBW babies delivered in a referring hospital. A univariate model was first applied to examine the associations of the individual independent variables with the outcome variable, followed by a logistic stepwise regression analysis for each of the outcome variables. The odds ratios for each predictor were reported as well as their P values and 95% confidence intervals.

Results: In the stepwise logistic regression analysis, accounting for a possible confounding effect of the independent variables, ‘hospital of birth’ remained a statistically significant predictor in the final step only for mortality, with odds ratio (inborns relative to outborns) of 3.32 (95%CI[2] 1.19–9.28, P = 0.022). No statistically significant associations with the other outcome variables were found (severe IVH[3]/PVL[4] odds ratio = 1.99, 95%CI = 0.77–5.14, P = 0.155; BPD[5] odds ratio = 0.60, 95%CI = 0.19–1.91, P = 0.384; intact survival OR[6] = 0.56, 95%CI = 0.23–1.35, P = 0.195).

Neonatal lupus erythematosus is an uncommon transplacentally Acquired Autoimmune Disorder. The most common clinical manifestations are skin rash, congenital atrioventricular block, thrombocytopenia, leucopenia, anemia, and hepatosplenomegaly. Usually, the skin rash resembles subacute cutaneous lupus, but different forms of rash have been reported in Neonatal lupus erythematosus and some are rare forms. NLE should be suspected in babies with atypical skin lesions, even if present at birth.