Juvenile lupus: Different clinical and serological presentations compared to adult lupus in Egypt

Aim of the work: We aimed to evaluate the differences in clinical presentation, serological pattern and disease activity between juvenile and adult-onset of Egyptian systemic lupus erythematosus (SLE) patients. Patients and methods: 160 Egyptian SLE patients (80 Adult-onset and 80 juveniles) were included. Patients records were reviewed for clinical and laboratory evaluation on presentation. Disease activity at onset was assessed using SLE Disease Activity Index (SLEDAI). Results: The mean age of the adult patients was 29.9 ± 7.2 years and of the juvenile cases (12.8 ± 2.1 years). The female:male ratio of the adults was 10:1 while it was 39:1 in the SLE children. The most common clinical presentation among adult SLE was malar rash (75%) followed by articular manifestations (62.5%), while in juveniles, nephritis (78.8%) followed by articular manifestations (71.2%) were the most common. Juvenile patients had more frequent neuropsychiatric (p = 0.015) and hematologic abnormalities (p < 0.001) at onset; and lupus nephritis (72.5%) compared to adults (36.2%) (p < 0.001) during the first year of presentation. Juvenile SLE showed higher frequency of proteinuria (p < 0.001), hematuria (p = 0.02) and active urinary sediments (p = 0.016). Proliferative nephritis was the most common form among both juveniles and adults. Positivity and titres of both anticardiolipin antibodies and lupus anticoagulant were significantly higher in juvenile SLE. Juvenile SLE patients had significantly higher SLEDAI [median (IQR): 12 (10–22)] compared to adults [median (IQR): 8 (4–12)], p < 0.001. Conclusion: Juvenile SLE patients differ from adult SLE with more frequent major organs affection and significantly higher serological activity. Earlier and more careful assessment with strict management plan and follow-up are needed in juvenile SLE patients.     

Focal liver lesions found incidentally

Incidentally found focal liver lesions are a commonfinding and a reason for referral to hepatobiliary service.They are often discovered in patients with history of liver cirrhosis,colorectal cancer,incidentally during work up for abdominal pain or in a trauma setting.Specific points should considered during history taking such as risk factors of liver cirrhosis;hepatitis,alcohol consumption,substance exposure or use of oral con-traceptive pills and metabolic syndromes.Full blood count,liver function test and tumor markers can act as a guide to minimize the differential diagnosis and to categorize the degree of liver disease.Imaging should start with B-mode ultrasound.If available,contrast enhanced ultrasound is a feasible,safe,cost effective option and increases the ability to reach a diagnosis.Contrast enhanced computed tomography should be considered next.It is more accurate in diagnosis and better to study anatomy for possible operation.Contrast enhanced magnetic resonance is the gold standard with the highest sensitivity.If doubt still remains,the options are biopsy or surgical excision.     

A guide to fluoroscopic identification and design of bioprosthetic valves: A reference for valve‐in‐valve procedure

Surgical aortic valve replacement remains the therapy of choice in majority of patients with aortic stenosis. Bioprosthetic heart valves are often preferred over mechanical valves as they preclude the need for anticoagulation with its associated risks of bleeding and thromboembolism. However, bioprosthetic heart valves undergo structural deterioration and eventually fail. Reoperation is the standard treatment for structural failure of the bioprosthetic valve, stenosis or regurgitation but can carry a significant risk, especially in elderly patients with multiple comorbidities. Transcatheter aortic valve implantation has recently been established as a feasible alternative to conventional valve surgery for the management of high‐risk elderly patients with aortic stenosis. This treatment modality has also been shown to be of benefit in the management of degenerated aortic bioprosthesis as a valve‐in‐valve procedure. The success of this procedure depends on a good understanding of the failing bioprostheses. This not only includes the device design but its radiological/fluoroscopic appearance and how it correlates with the implanted valve, as transcatheter aortic valve implantation is performed under fluoroscopic guidance. Here we illustrate the fluoroscopic appearance of 11 commercially available surgical bioprostheses and two commercially available transcatheter heart valves and discuss important aspects in their design which can influence outcome of a valve‐in‐valve procedure. We have also collated relevant information on the aspects of the design of a bioprosthetic valve, which are relevant to the valve‐in‐valve procedure. © 2012 Wiley Periodicals, Inc.     

Comparison of modified short axis view and apical four chamber view in evaluating right ventricular function after repair of tetralogy of Fallot

BACKGROUND: In clinical settings an easy and reliable method for following up right ventricular (RV) function in patients after repair of tetralogy of Fallot (TOF) is needed. It is, however, unclear whether the novel modified short axis view from echocardiography is superior to the apical four chamber view in this aspect. MATERIALS AND METHODS: Thirty postoperative TOF patients with median age 17 years (range 6-45 years) and follow up period of 10 years (range 0.5-40 years) were examined echocardiographically using the apical four chamber view and the novel modified short axis view. RV areas in end-systole (Amin) and end-diastole (Amax) were measured and an area fraction [(Amax - Amin)/Amax * 100%] was calculated from the respective view. RV ejection fraction was assessed through magnetic resonance imaging (MRI). The RV area fractions from echocardiography were compared to the RV ejection fraction. RESULTS: The right ventricular area fraction derived from the modified short axis view was significantly lower than that from the apical four chamber view (34.3+/-9.1% vs. 42.5+/-10.2%, p=0.007). Both the RV area fractions obtained from the modified short axis view (r=0.674, p<0.001) and from the apical four chamber view (r=0.512, p=0.025) correlated significantly with the MRI derived RV ejection fraction. CONCLUSION: The novel modified short axis view from echocardiography may be superior to the apical four chamber view for routine follow up of patients after TOF repair, in whom the right ventricular outflow tract plays an important role in the right ventricular systolic function.     

A remarkable female cranium of the early Oligocene anthropoid Aegyptopithecus zeuxis (Catarrhini, Propliopithecidae)

The most complete and best-preserved cranium of a Paleogene anthropoid ever found, that of a small female of the early Oligocene ( approximately 29-30 Ma) stem catarrhine species Aegyptopithecus zeuxis, was recovered from the Jebel Qatrani Formation (Fayum Depression, Egypt) in 2004. The specimen is that of a subadult and, in craniodental dimensions, is the smallest Aegyptopithecus individual known. High-resolution computed tomographic (microCT) scanning of the specimen's well preserved cranial vault confirms that Aegyptopithecus had relatively unexpanded frontal lobes and a brain-to-body mass ratio lower than those of living anthropoids. MicroCT scans of a male cranium recovered in 1966 [Egyptian Geological Museum, Cairo (CGM) 40237] reveal that previous estimates of its endocranial volume were too large. Thus, some amount of encephalization evolved independently in platyrrhine and catarrhine anthropoids, and the relative brain size of the last common ancestor of crown Anthropoidea was probably strepsirrhine-like or smaller. A. zeuxis shows extreme sexual dimorphism in craniodental morphology (apparently to a degree otherwise seen only in some highly dimorphic Miocene catarrhines), and the crania of female Aegyptopithecus lack a number of morphological features seen in larger males that have been accorded phylogenetic significance in catarrhine systematics (e.g., a well developed rostrum, elongate sagittal crest, and frontal trigon). Although a unique pattern of craniofacial sexual dimorphism may have characterized advanced stem and basal crown catarrhines, expression of various allegedly "discrete" craniofacial features may have been intraspecifically variable in early catarrhine species due to high levels of dimorphism and so should be treated with caution in phylogenetic analyses.     

Soluble urokinase plasminogen activator receptor in type 1 diabetic children,relation to vascular complications

BackgroundEndothelial dysfunction caused by chronic inflammation is the cornerstone of vascular complications in type 1 Diabetes-Mellitus (T1DM). Soluble Urokinase Plasminogen Activator Receptor (SuPAR) is a novel marker of inflammation and endothelial dysfunction.AimTo evaluate SuPAR in T1DM children and correlate it to diabetic vascular complications.MethodsSeventy T1DM children and 40 matched healthy controls were studied focusing on disease duration, insulin therapy and symptoms of diabetic complications. Blood-pressure, fundus and screening for peripheral-neuropathy were done. Fasting lipid profile, fraction-C of glycosylated hemoglobin (HbA1c%), Urinary albumin excretion (UAE), estimated-glomerular filtration rate (eGFR) and SuPAR were measured. Internal aortic diameter was measured with calculation of aortic distensibility and stiffness index.ResultsSixteen T1DM patients(22.9%) had peripheral neuropathy, 12(17%) had nephropathy and none had retinopathy. SuPAR was significantly elevated in diabetic nephropathy (p < 0.01) and neuropathy (p < 0.01). Aortic stiffness index was significantly higher (p < 0.01) whereas, aortic strain and distensibility were significantly lower (p < 0.01) in T1DM than controls. SuPAR was significantly correlated to disease duration (p < 0.01), systolic blood pressure (p < 0.01), total cholesterol (p < 0.01), triglycerides (p < 0.01), UAER (p < 0.01) and aortic strain (0.013).ConclusionIncreased SuPAR early in diabetes might become a useful indicator of developing vascular complications. Further prospective studies are needed to determine the cut-off level of SuPAR for detection of T1DM and its complications.     

The all-cause mortality and risk factors for mortality within five years among prevalent Type 1 Diabetes Mellitus Patients

Despite new comprehensive approach in diabetes care, type 1 diabetes is still facing considerable premature mortality. This study aims to examine associated risk factors for all-cause mortality among prevalent patients with type 1 diabetes mellitus (T1DM) within 5 years’ period and subsequently develop a logistic regression model to predict the outcome. This was a cohort study where prevalent patients diagnosed with T1DM were notified in a national diabetes registry. Patients’ particulars were recorded between 1 January 2009 and 31 December 2009. Their records were matched with national death record at the end of year 2013 to determine the status of mortality within 5 years. The factors associated with mortality were investigated, and a prognostic model was developed based on logistic regression model. There were 665 patients included in the study, and 105 patients died within 5 years. The mortality rate was 1.6 persons per 100 person-years and the standardized mortality ratio was 10.04. Majority causes of death were due to circulatory system (33.8%) and infection (32.5%). Multivariate analysis suggested that gender, age group, and ischemic heart disease (IHD) were the major contributing factors towards the outcome. Elderly male with IHD has a significant risk of mortality within 5 years with probability of event of 0.755, while elderly female with IHD has probability of event of 0.612. The main causes of death among prevalent T1DM patients were heart disease and infection. Male gender, elderly age group, and having IHD were significant risk factors of mortality in prevalent T1DM patients within 5 years.

     

Clinical outcome of Crohn's disease treated with infliximab

BACKGROUND/AIMS: Controlled studies in humans have shown the role of antibodies to tumor necrosis factor-alpha in the treatment of both fistulizing and inflammatory Crohn's disease. The aim of this study is to report the results of a multicenter clinical trial to evaluate efficacy of infliximab in Crohn's disease patients who are refractory to conservative drugs or fistulizing Crohn's disease. METHODOLOGY: This trial was carried out at 5 university and community hospitals, in Turkey. A total of 25 patients with Crohn's disease that were unresponsive to conventional medical therapy, participated; 17 of the 25 were in the fistulizing disease group and 8 were in the inflammatory disease group. Clinical response was classified according to fistula drainage, diarrhea as positive response or no response. RESULTS: Overall response rate was 92% (23/25), regardless of the disease group, after first infusion of infliximab. Sixteen out of 17 patients in the fistulizing disease group had a positive response. Fourteen of the 16 positive responders later relapsed. Median duration of response was 8 weeks (range, 2-35 wk). Active inflammatory disease patients had a positive response rate of 75% (6/8) and two of the patients were nonresponders. A further two patients relapsed at week 14. Two patients in both arms of the study were still in remission at the end of the study. Major adverse events were: pneumonia in one patient, skin infections in two patients, pulmonary thromboembolism and death in one patient. CONCLUSIONS: Infliximab treatment seems to be more effective in Crohn's disease patients especially in those with fistulizing disease than those with non-fistulizing, inflammatory disease. It is evident that maintenance of remission might be achieved with ongoing maintenance therapy. We suggest maintenance of infliximab therapy.     

Diagnostic potential of serum matrix metalloproteinase-2 and tissue inhibitor of metalloproteinase-1 as non-invasive markers of hepatic fibrosis in patients with HCV related chronic liver disease

As chronic liver disease progresses, an imbalance occurs between synthesis and breakdown of extracellular matrix (ECM). Matrix metalloproteinases (MMPs) are involved in degrading ECM while tissue inhibitors of metalloproteinases (TIMPs) prevent their fibrolytic action. In the present study, serum levels of matrix metalloproteinase-2 (MMP-2) and tissue inhibitor of metalloproteinase-1 (TIMP-1) were investigated as non-invasive parameters for the diagnosis of hepatic fibrosis in patients with HCV related chronic liver disease. Their diagnostic potential was evaluated in comparison to hepatic histology and standard liver function tests. A sandwich enzyme immunoassay technique was used to study circulating values of MMP-2 and TIMP-1 in forty-one patients with HCV antibodies in their sera (27 patients with biopsy ascertained chronic hepatitis C and 14 patients with histologically proven liver cirrhosis. Hepatic histology was evaluated using the hepatitis-activity-index according to Ishak et al. (1995), quantifying separately inflammatory activity and fibrosis. Ten healthy individuals were also included in the study as controls. Serum levels of MMP-2 were similar in controls and in chronic hepatitis C patients with (n = 15) and without (n = 12) fibrosis, but increased significantly in cirrhosis. TIMP-1 serum values showed a steady increase from normal controls to chronic hepatitis C without fibrosis, hepatitis C with fibrosis, and cirrhosis. The diagnostic potential of MMP-2 to detect fibrosis was low with a sensitivity of 7% and a diagnostic efficiency of 56%. The diagnostic potential of circulating MMP-2 to detect cirrhosis was higher with a sensitivity of 83% and a specificity of 96% resulting in a diagnostic efficiency of 92%. Serum TIMP-1 values detected fibrosis with a sensitivity of 67% and a specificity of 69% resulting in an efficiency rate of 70%. TIMP-1 values detected cirrhosis with 100% sensitivity but only 75% specificity. The diagnostic potential of circulating TIMP-1 was higher than that of serum ALT, AST or albumin values. In conclusion, serum values of MMP-2 and TIMP-1 are able to detect cirrhosis with a high sensitivity. Moreover, TIMP-1 values can detect fibrosis with comparable efficiency. Regular determinations of both TIMP-1 and MMP-2 in patients with chronic hepatitis C may be used as indicators of increasing fibrosis and the development of cirrhosis.     

Prevalence of cytomegalovirus (CMV) infection among neonatal intensive care unit (NICU) and healthcare workers

CMV is the most common cause of congenital and perinatal infection, most infections are asymptomatic at birth but later on develop handicaps, mainly neurological disturbances. The aim of the present work is to study the prevalence of CMV infection in NICU, to detect possible nosocomial transmission of CMV infection and determine possible risk factors for neonatal CMV infection. This study was carried on 175 neonates in NICU and 19 employees in the same unit. All members of the study were investigated for serum CMV-IgG and IgM by ELISA and CMV - DNA by PCR. The overall prevalence of CMV was 12.57%, 10 (5.71%) had congenital infection, while 12 cases (6.86%) had perinatal infection. In neonates with congenital CMV infection, the prevalence of breast milk feeding, congenital anomalies and blood transfusion were 80%, 30% and 60%, respectively. In neonates with perinatal CMV infection the prevalence of breast milk feeding, congenital anomalies and blood transfusion were 75%, 16.67% and 50%, respectively. On the other hand from the 19 employees, 2 (10.53%) were CMV-DNA positive by PCR, none of them was CMV-lgM positive and all of them were CMV-IgG positive. The risk factors related to CMV infection among neonates in NICU were, low birth weight, congenital anomalies and breast milk feeding, while CMV infection among employee was related to blood transfusion and employment period. In our results there was no statistical correlation between neonates in NICU and employee in the same unit. CMV infections are of more prevalence in premature and low birth weight neonates in NICU. No evidence of nosocomial CMV transmission to employee in NICU.