Paraganglioma-like dermal melanocytic tumor: a case report with particular features

Childhood dermal tumors with melanocytic features is an unusual tumor that can create diagnostic confusion. Among them, paraganglioma-like melanocytic tumors- previous included in melanocytic tumors of uncertain malignant potential- has some particular histopathologic and immunohistochemical features. We describe a case of 13 years old girl with a paraganglioma-like dermal melanocytic tumor of the left leg.     

Botulinum toxin improves lid opening delays in blepharospasm-associated apraxia of lid opening

Lid movement and EMG of the orbicularis oculi (OOc) were analyzed in 10 patients with apraxia of lid opening associated with blepharospasm before and after botulinum toxin treatment. The latencies to onset and to complete the eye opening and the time during which eye opening was sustained were studied in relation to OOc activity and compared with control values obtained in 12 healthy subjects. Following treatment there was an improvement of all lid opening measurements, a decrease of the abnormally prolonged OOc activity, and a reduction of the functional disability.     

RPE-rip after intravitreal bevacizumab (Avastin) treatment for vascularised PED secondary to AMD

Background Retinal pigment epithelial (RPE)-rips in age-related macular degeneration (AMD)-associated pigment epithelial detachment (PED) occur in the natural course of the disease but also after therapy (e.g. laser photocoagulation, photodynamic therapy), possibly triggered by the specific therapy. We report here on four patients that received intravitreal bevacizumab (Avastin) for AMD-associated vascularised PED and developed RPE-rips during the follow-up. Methods The case reports of four consecutive patients that developed RPE-rips after intravitreal injection of bevacizumab at 1 mg/0.1 ml were reviewed. Results The RPE-rips occurred in all patients between 1 week and 1 month following intravitreal injection. Two of the four patients improved in vision despite the rip, but 3 months after the initial intervention, three patients suffered deterioration in visual acuity and had to be re-injected. Conclusion Improvement in visual acuity may occur following intravitreal bevacizumab despite RPE-rips, but the patients need close follow-up and eventual re-treatment in the case of deterioration. None of the authors has a propietary interest in the presented cases.     

An experience sampling study of associations between affect and alcohol use and problems among college students

OBJECTIVE: This study used experienced sampling methodology to examine the relationship between affective functioning and alcohol consumption and problems. METHOD: Fifty-six college students provided baseline data on measures of impulsivity and distress tolerance and provided experience sampling data for 2 weeks on measures of negative affect, positive affect, and alcohol consumption and problems. Women made up 54% of the sample. The sample ranged in age from 21 to 23 (mean [SD] = 21.50 [0.57]); 98% were white and 2% were Asian. RESULTS: As predicted, higher levels of both positive and negative affect during the day were associated with higher rates of consumption that night. In contrast, negative, but not positive, affect was associated with alcohol-related problems after controlling for alcohol consumption. Impulsivity was associated with higher consumption and problems and moderated the relationships between negative affect and problems and also between alcohol consumption and problems. Low distress tolerance was associated with a decreased association between positive affectivity and alcohol consumption. CONCLUSIONS: This study replicated and extended previous research on affective models of alcohol use and problems through the use of experience sampling methodology. The results demonstrated associations between affective variables and alcohol consumption and problems measured through near real-time assessment. The results suggest a functional association between alcohol consumption and problems and both within-person changes in affect and between-person differences in impulsivity and distress tolerance.     

Pediatric short bowel syndrome: redefining predictors of success

OBJECTIVE: To determine predictors of survival and of weaning off parenteral nutrition (PN) in pediatric short bowel syndrome (SBS) patients. SUMMARY BACKGROUND DATA: Pediatric SBS carries extensive morbidity and high mortality, but factors believed to predict survival or weaning from PN have been based on limited studies. This study reviews outcomes of a large number of SBS infants and identifies predictors of success. METHODS: Multivariate Cox proportional hazards analysis was conducted on 80 pediatric SBS patients. Primary outcome was survival; secondary outcome was ability to wean off PN. Nonsignificant covariates were eliminated. P < 0.05 was considered significant. RESULTS: Over a mean of 5.1 years of follow-up, survival was 58 of 80 (72.5%) and 51 weaned off PN (63.8%). Cholestasis (conjugated bilirubin > or =2.5 mg/dL) was the strongest predictor of mortality (relative risk [RR] 22.7, P = 0.005). Although absolute small bowel length was only slightly predictive, percentage of normal bowel length (for a given infant's gestational age) was strongly predictive of mortality (if <10% of normal length, RR of death was 5.7, P = 0.003) and of weaning PN (if > or =10% of normal, RR of weaning PN was 11.8, P = 0.001). Presence of the ileocecal valve (ICV) also strongly predicted weaning PN (RR 3.9, P < 0.0005); however, ICV was not predictive of survival. CONCLUSIONS: Cholestasis and age-adjusted small bowel length are the major predictors of mortality in pediatric SBS. Age-adjusted small bowel length and ICV are the major predictors of weaning from PN. These data permit better prediction of outcomes of pediatric SBS, which may help to direct future management of these challenging patients.     

Meropenem activity against staphylococci

Meropenem is a new carbapenem with excellent activity against different Gram-negative and Gram-positive bacteria. Its good antibacterial activity is closely related to high hydrolysis resistance by bacterial beta-lactamases. The aim of this study was to evaluate antimicrobial activity of meropenem by comparison that of oxacillin, gentamicin, clarithromycin and ciprofloxacin against 458 staphylococci collected during 2004 period from healthy individuals. Minimum inhibitory concentrations were determined by agar dilution technique. Meropenem has an activity clearly superior to the other tested agents. Resistance rate is zero for all investigated strains.     

Delayed chemokine receptor 1 blockade prolongs survival in collagen 4A3-deficient mice with Alport disease

Human Alport disease is caused by a lack of the alpha3-, 4-, or 5-chain of type IV collagen (COL4A). Affected humans and COL4A3-deficient mice develop glomerulosclerosis and progressive renal fibrosis in the presence of interstitial macrophages, but their contribution to disease progression is under debate. This question was addressed by treating COL4A3-deficient mice with BX471, an antagonist of chemokine receptor 1 (CCR1) that is known to block interstitial leukocyte recruitment. Treatment with BX471 from weeks 6 to 10 of life improved survival of COL4A3-deficient mice, associated with less interstitial macrophages, apoptotic tubular epithelial cells, tubular atrophy, interstitial fibrosis, and less globally sclerotic glomeruli. BX471 reduced total renal Cll5 mRNA expression by reducing the number of interstitial CCL5-positive cells in inflammatory cell infiltrates. Intravital microscopy of the cremaster muscle in male mice identified that BX471 or lack of CCR1 impaired leukocyte adhesion to activated vascular endothelium and transendothelial leukocyte migration, whereas leukocyte rolling and interstitial migration were not affected. Furthermore, in activated murine macrophages, BX471 completely blocked CCL3-induced CCL5 production. Thus, CCR1-mediated recruitment and local activation of macrophages contribute to disease progression in COL4A3-deficient mice. These data identify CCR1 as a potential therapeutic target for Alport disease or other progressive nephropathies associated with interstitial macrophage infiltrates.