Acrodermatitis continua of Hallopeau is a clinical phenotype of DITRA: evidence that it is a variant of pustular psoriasis.

Acrodermatitis continua of Hallopeau (ACH) is a rare, chronic, sterile, pustular eruption that predominantly affects the fingertips with nail involvement. While some consider ACH a distinct entity, many believe it to be a variant of pustular psoriasis, especially as cases of ACH progressing to generalized pustular psoriasis (GPP) have been reported. Recently, recessively inherited mutations in the IL36RN gene, which encodes interleukin-36 receptor antagonist (IL-36Ra), have been demonstrated to be the cause of familial GPP, a condition termed DITRA (deficiency of IL-36Ra). Here, we identified a homozygous missense mutation c.338C>T (p.Ser113Leu) in the IL36RN gene in a male patient with ACH, as well as in his sister who had a history of GPP.

Evaluation of the appropriateness criteria for coronary computed tomography angiography in an academic medical center in a developing country: comparison of the 2006 and 2010 criteria.

BACKGROUND: The aims of this study are to analyze the risk profile and evaluate the appropriateness of patient referral to coronary CT angiography in a new MSCT laboratory in an academic medical center in a developing country. METHODS AND RESULTS: The study population consisted of 100 consecutive patients (mean age 53 ± 13 years, 83% males) who were referred to the new 64 slice MSCT laboratory at the American University of Beirut between July 2008 and July 2009. 52% of patients had chest pain and 48% were asymptomatic. By the 2006 ACCF criteria, only 8% of the referrals fitted the "Appropriate" criteria, 49% were "Inappropriate", and 43% were "Uncertain". The percentage of "Appropriate" indications increased from 8% by the 2006 criteria to 38% by the 2010 ACCF criteria. Framingham risk score (FRS) calculation showed a substantial percentage (46%) of patients to be at low risk, with 32% being at intermediate risk, and 22% at high risk. The prevalence of high calcium score (>400) (0% vs 22%, vs 27%, P < .001) and of obstructive CAD (4% vs 23% vs 40%, P < .001) increased steadily across the low, intermediate, and high FRS groups, respectively. CONCLUSIONS: There was a significant increase in the percentage of "Appropriate" indications for MSCT when comparing the 2010 to the 2006 ACCF appropriateness criteria. However, in spite of that, there was still significant deviation from the ACCF appropriateness criteria among patients referred for coronary CT angiography in this new MSCT laboratory in a developing country.

Prognostic factors of multiple sclerosis in Lebanon.

BACKGROUND: Multiple sclerosis (MS) has a variable disease course. Identifying early predictive prognostic factors is of paramount importance. Most of the data on these factors however comes from studies performed in western countries. Such data is lacking in the Arab World. The objective of this study is to identify early predictors of disability among MS patients in Lebanon. METHODS: 75 relapsing-remitting MS patients with 5 year follow-up from disease onset were selected from Project MS Lebanon database. The following parameters were studied as potential causes of early disability as defined by an EDSS > or = 3, after five years from disease onset: age at onset of MS, gender, interval between first and second attack, residual deficit after first attack, initial symptoms, treatment for at least 1 year in the first 5 years, and the number of relapses in the first 2 and 5 years. RESULTS: Patients with incomplete recovery from the first relapse were 11.66 times more likely to have a higher EDSS after 5 years (CI = 2.02-67.31, p = .001). Furthermore, the number of relapses during the first 5 years was also an independent predictor of EDSS > or = 3 at 5 years (p = .024). Other factors were not shown to predict a worse outcome. CONCLUSION: Overall, early predictors of disability in MS among the Lebanese population were not very different from similar predictors in western countries.

Celiac disease, Behçet, and idiopathic thrombocytopenic purpura in siblings of a patient with multiple sclerosis.

Multiple sclerosis (MS) is a demyelinating disease of uncertain etiology. Many genetic and environmental risk factors have been associated with this disease including certain human leukocyte antigen haplotypes, Epstein-Barr virus infection, and vitamin D deficiency. We report a 30-year-old woman with MS, the product of consanguineous marriage, and three siblings with three different autoimmune diseases: idiopathic thrombocytopenic purpura, celiac disease, and Behçet's disease.

Variations in the referral patterns to stress nuclear imaging and stress echocardiography scans.

BACKGROUND: Stress myocardial perfusion imaging (MPI) and stress echocardiography (Echo) are commonly used for the noninvasive evaluation of patients with suspected coronary artery disease (CAD). Very few studies have compared the referral patterns to these imaging modalities in terms of the clinical profile of patients, reasons for referral, and type of referring physicians. METHODS AND RESULTS: This was a prospective study of 1,020 consecutive patients who were referred for stress MPI (429 patients) or stress Echo (591 patients) at the American University of Beirut Medical Center in the year of 2008. Patients referred to MPI were older and had a higher prevalence of diabetes, hypertension, hypercholesterolemia, smoking, and previous myocardial infarction, coronary angioplasty, or bypass surgery. There were more abnormal scans in the stress MPI group (24% vs 15%, P < 0.001), as well as a higher prevalence of ischemia (15% vs 7.6%, P < 0.001) and impaired left ventricular function with an ejection fraction <50% (11% vs 1.7%, P < 0.001). A higher percentage of stress Echo studies were self-referred by physicians who themselves interpret the scans (31% vs 19%, P < 0.001). CONCLUSION: Patients referred for stress MPI are at a higher risk than those referred for stress Echo having more CAD risk factors, more prior history of coronary events, and an older age. These findings have important implications in the interpretation of studies that compare the diagnostic and prognostic power of these two imaging modalities.

High prevalence of metabolic syndrome in patients with psoriasis in Lebanon: a prospective study.

BACKGROUND: Psoriasis is a chronic inflammatory disease that affects not only the skin but also other organs as well. Genetic factors play an important role in individual predisposition. Lately, a positive association has been confirmed between psoriasis and metabolic syndrome (MBS), in western as well as in Middle Eastern countries. AIM: Assess the prevalence of MBS in Lebanese patients with psoriasis and the differential effect according to types and disease severity. METHODS: This was a case-control study including 150 psoriasis patients and 150 age- and gender-matched controls admitted to the dermatology clinics at the American University of Beirut-Medical Center, a tertiary care center in Beirut. Psoriasis severity was assessed by the Psoriasis Area Severity Index (PASI). Blood samples were collected from fasting subjects and tested for glucose, HDL cholesterol, triglycerides, and C-reactive protein (CRP). Multivariate binary logistic regression models were built to assess the relationship between MBS and psoriasis, after adjustment for smoking as a possible confounding variable. RESULTS: Patients with psoriasis were two times more likely to have MBS as compared to controls (35.3% vs 18.0%, P < 0.001) with an odds ratio (OR) of 2.4. All components of MBS were more prevalent in psoriasis patients than in controls. PASI score was greater in patients with MBS than those without MBS (10.5 ± 11.5 vs. 7.0 ± 8.1, P = 0.05). MBS prevalence tended to be higher in the inverse type than in others (52.2% versus 32.3%; P = 0.06) and in patients with nail pitting versus those without (45.3% vs. 28.2%; P = 0.03). CONCLUSIONS: This was the first study to assess the prevalence of MBS in Lebanese subjects with psoriasis and, to our knowledge, the first study that showed a higher likelihood of MBS in patients with inverse psoriasis and with nail pitting.

Current reperfusion strategies for ST-elevation myocardial infarction in an academic medical center in a developing country: efficacy of primary percutaneous coronary intervention.

OBJECTIVES: To study the reperfusion strategies currently being used in the treatment of ST-elevation myocardial infarction (STEMI) at an academic medical center in a developing country and to analyze the door-to-balloon time (DBT) in those patients undergoing primary percutaneous coronary intervention (PCI). METHODS: The study included all patients presenting with STEMI to the emergency department at the American University of Beirut Medical Center between July 2008 and February 2010. Data were collected prospectively from the patients' medical records. RESULTS: The study population consisted of 100 consecutive patients. Compared with an earlier study from American University of Beirut Medical Center done in 2002-2005, there was a significant increase in the utilization of primary PCI for reperfusion (81% vs. 2.5%; P < 0.001). However, the median DBT was 110 minutes, with only 30% of patients achieving a DBT ≤90 minutes. The predictors of delayed DBT (>90 minutes) were culprit lesions in the circumflex artery (P = 0.007) and delayed time from electrocardiogram to arrival in the catheterization laboratory (P < 0.001). CONCLUSIONS: There was a significant increase in the utilization of primary PCI for reperfusion of STEMI in this academic medical center in a developing country. However, achieving a target DBT ≤90 minutes was suboptimal. Future studies are needed to analyze the logistic factors associated with delayed reperfusion to institute policies and systems that can enhance the efficacy of primary PCI as a reperfusion modality in these countries.

The effect of war stress on multiple sclerosis exacerbations and radiological disease activity.

OBJECTIVE: The relationship between stressful life events and multiple sclerosis (MS) exacerbations or radiological disease activity is at best controversial. The aim of this study is to examine the relationship between exposure to war-related events incurred during the July 2006 Israeli-Lebanese war and clinical relapses and MRI disease activity among Lebanese MS patients. METHODS: We studied a group of 216 patients with clinically definite relapsing remitting MS (RRMS), on whom clinical data was available for the war period and for the preceding and following year(s). The number of relapses was determined during the war period and during similar periods over a 3-year span. All patients with brain MRI during the war period had their scans reviewed for evidence of disease activity as defined by the presence of gadolinium enhancing (Gd+) lesions. A group of patients with brain MRI performed outside the war period was used for comparison. RESULTS: The total number of relapses during the war period (n=23) was significantly higher than during non-war periods (mean=8.4, SD=0.86) (p=0.006). Of the 18 patients with brain MRI during the war, 5/7 with relapses and 1/11 without relapses had Gd+ lesions (p=0.013). More patients had Gd+ lesions during the war period (33%) compared to controls (13%) (p=0.075). INTERPRETATION: Our study shows that exposure to war-related events is likely to lead to an increase in both clinical relapses and MRI disease activity in patients with MS.

The interaction of gender and clinical risk profile in patients referred for exercise nuclear imaging versus exercise echocardiography.

BACKGROUND: Exercise myocardial perfusion imaging (E-MPI) and exercise echocardiography (E-Echo) are thought to be compatible for the non-invasive evaluation of patients with suspected coronary artery disease (CAD). The interaction of gender and clinical risk profile in the referral of patients to either of these two imaging modalities has not been well studied. METHODS AND RESULTS: This was a prospective study of 922 consecutive patients who were referred for either E-MPI (331 patients) or E-Echo (591 patients) at the American University of Beirut Medical Center in the year of 2008. Men undergoing E-MPI had a higher risk profile than those undergoing E-Echo. They were older (58±12 years versus 55±12 years, p=0.002) and had a higher prevalence of multiple (>2) CAD risk factors (55% versus 37%, p<0.001) as well as a higher prevalence of prior PCI (22% versus 15%, p=0.017) or CABG (12% versus 7%, p=0.016). Furthermore, they achieved lower METS in their exercise (9.4±2.0 versus 10.4±2.3, p<0.001) and had a higher incidence of ischemia and on their scans (16% versus 9.1%, p=0.008). In contrast, women undergoing E-MPI had a similar profile to those undergoing E-Echo in terms of prevalence of risk factors, prior history of coronary events and the prevalence of ischemia or impaired ejection fraction on their scans. CONCLUSION: There is an important interaction between gender and clinical risk profile in patients undergoing E-MPI versus E-Echo. In men, E-MPI seems to be the preferred test for the higher risk profile patients, whereas in women the two tests are interchangeable.

Acute coronary syndromes: clinical characteristics, management, and outcomes at the American University of Beirut Medical Center, 2002-2005.

OBJECTIVES: Data on acute coronary syndromes (ACS) in developing countries is scarce. In this report, we analyze the temporal trends in the management and outcomes of a large series of ACS patients hospitalized at the American University of Beirut Medical Center (AUBMC), a tertiary referral university hospital located in a middle income Middle Eastern developing country. METHODS: A total of 1025 consecutive patients hospitalized and discharged with the diagnosis of ACS were enrolled between 2002 and 2005. The utilization of evidence-based therapies and in-hospital outcomes were determined. RESULTS: The study enrolled 228 patients (22%) with ST-elevation myocardial infarction (STEMI), 275 patients (27%) with non-ST-elevation myocardial infarction (NSTEMI), and 522 patients (51%) with unstable angina. The STEMI group was younger and had a higher percentage of men. The utilization rates of coronary angiography and percutaneous coronary intervention (PCI) were highest in the STEMI group. Comparison to earlier ACS data (1997-1998) from the same hospital, showed an increase in the utilization of reperfusion therapy, coronary angioplasty, bypass surgery, aspirin, beta-blockers, angiotensin-converting enzymes (ACE), angiotensin receptor blockers (ARB), and statins over the past decade (P < .05). This was associated with a significant decrease in hospital mortality (13%-7.7%, P < .01). CONCLUSIONS: This study analyzes one of the largest series of ACS patients reported from a single center in a developing country. The utilization of evidence-based therapies in the management of ACS at AUBMC has improved significantly over the past decade with an associated decrease in hospital mortality.