Lesion presenting with a “blue amber” pattern

Atypical fibroxanthoma (AFX) is a spindle cell neoplasm with low metastatic potential but high tendency to recur after surgery. Because of the rarity of this lesion and its aspecific clinical features, AFX could be easily misdiagnosed and undertreated by many clinicians who encounter them. Dermoscopy represents a valuable tool for easily assessing skin lesions, even though histological examination is required for final diagnosis. We report a case of a cheek lesion with dermoscopic “blue amber pattern”, easily recognisable and not observed in others skin tumours, which could represent an additional feature useful in differentiating this tumour from other skin neoplasms.     

Erythrocyte‐exchange with the OPTIA™ cell separator in patients with sickle‐cell disease

Erythrocyte–exchange (EEX) has proven to be a very useful tool in sickle‐cell disease (SCD) patients either during acute painful crisis unresponsive to hydration and/or analgesia or as a prophylactic treatment in high risk patients in those who do not tolerate hydroxyurea (HU), with the aim of lowering HbS levels. EEX may be performed either by using continuous‐ or discontinuous flow devices, the former being of choice in children or in low‐weight patients. Thus, a low extracorporeal blood volume (EBV) could allow for a better and safer procedure management. In this study we compared EEX procedure performed with the recently released OPTIA device with EEX procedures performed using the COBE Spectra device (EBV 185 vs 270 mL, respectively). Twenty‐one EEX (4 as emergency treatment) were performed in 12 patients with the Spectra device and 25 (9 as emergency treatment) in 15 patients with the OPTIA device. All the procedures were well tolerated and uneventful. We did not observe significant differences between the two devices as to pre‐ and post‐EEX parameters, namely in target hematocrit and in HbS reduction. Noteworthy, due to the lowest EBV allowed by the OPTIA device, an EEX procedure performed in a 13 Kg‐ child did not require a preliminary priming of the circuit. In conclusion, the OPTIA device proved to be as effective as the Spectra device in treating SCD patients either during sickling crisis or as prophylactic therapy. The OPTIA device can be safely used in the pediatric setting since it allows a lower EBV. J. Clin. Apheresis, 28:411–415, 2013. © 2013 Wiley Periodicals, Inc.     

Anticoagulant therapy with rivaroxaban in a young patient with paroxysmal nocturnal hemoglobinuria

The new direct oral anticoagulants such us rivaroxaban, could play an important role in the anticoagulant treatment of patients with paroxysmal nocturnal hemoglobinuria where anticoagulant treatment is complex to run, since they have shown a reduction in serious bleeding complications compared to antithrombotic therapy with classical vitamin k antagonist.     

Cervicogenic headache: comparison with migraine without aura; Vågå study

The prevalence of cervicogenic headache (CEH) is only vaguely known. Furthermore, it is a common belief that in migraine without aura (MwoA), neck symptoms frequently occur and that MwoA and CEH may pathogenetically be intimately related. In the Vågå study, 1838 18–65-year-old citizens (88.6% of that age group) were studied with face-to-face interviews and a thorough neck examination. For CEH, the Cervicogenic Headache International Study Group criteria were used, and for MwoA, the IHS criteria. The extent of cervical involvement was assessed by the 'CF' ('features indicative of cervical abnormality'). CEH prevalence was 4.1%. 'CF' was 2.37 in CEH vs 0.93 in M-A. CEH criteria, e.g. mechanical attack provocation, were present many times more frequently in CEH than in MwoA. Conversely, migraine criteria, e.g. photophobia, were ≥ 2.6 times higher in MwoA than in CEH. CEH is unlikely to be a subgroup of MwoA.     

Gene expression profiles of fatigued fibromyalgia patients with different categories of pain and catastrophizing: A preliminary report

Background

Fibromyalgia (FM) is a chronic condition characterized by diffused musculoskeletal pain and overwhelming fatigue.

Purpose

To compare the gene expression profiles of fatigued FM women with different levels of pain and catastrophizing.

Methods

Nine women with FM enrolled in an active Medstar Research Institute protocol were included in the gene expression analyses of peripheral blood RNA via Affymetrix GeneChip Human Genome U133 Plus 2.0 array (Santa Clara, CA). Scores from Brief Pain Inventory, Pain Catastrophizing Scale, and Multidimensional Fatigue Inventory categorized the nine participants into pain (high, n = 3; low, n = 6) and catastrophizing groups (high, n = 5; low, n = 4).

Discussion

Differential expression of 107 genes between the high and low pain groups and 139 genes between the high and low catastrophizing groups (over 2.0-fold change, p < .05) were observed. Network analyses showed interferon signaling and interferon regulatory activation factor pathways distinguished between the pain groups whereas dendritic cell maturation delineated between the catastrophizing groups.

Conclusion

Findings provide preliminary evidence that specific physiological pathways may possibly delineate pain and catastrophizing mechanisms. Further investigation via the use of a larger and more homogenous sample is warranted.     

PET imaging of prostate tumors with 18F‐Al‐NOTA‐MATBBN

Overexpression of the gastrin‐releasing peptide receptor (GRPR) in prostate cancer provides a promising target for detection the disease. MATBBN is a new bombesin analog originating from the GRPR antagonists with a hydrophilic linker. In this study NOTA‐conjugated MATBBN was labeled by the Al¹⁸F method and the potential of ¹⁸F‐Al‐NOTA‐MATBBN for prostate tumor PET imaging was also evaluated. NOTA‐MATBBN was radiolabeled with ¹⁸F using Al¹⁸F complexes. Partition coefficient, in vitro stability and GRPR binding affinity were also determined. PET studies were performed with ¹⁸F‐Al‐NOTA‐MATBBN in PC‐3 tumor‐bearing mice. ¹⁸F‐Al‐NOTA‐MATBBN can be produced within 30 min with a decay‐corrected yield of 62.5 ± 2.1% and a radiochemical purity of >98%. The logP octanol–water value for the Al¹⁸F‐labeled BBN analog was ?2.40 ± 0.07 and the radiotracer was stable in phosphate‐buffered saline and human serum for 2 h. The IC₅₀ values of displacement for the ¹⁸F‐Al‐NOTA‐MATBBN with MATBBN was 126.9 ± 2.75 nm . The PC‐3 tumors were clearly visible with high contrast after injection of the labeled peptide. At 60 min post‐injection, the tumor uptakes for ¹⁸F‐Al‐NOTA‐MATBBN and ¹⁸F‐FDG were 4.59 ± 0.43 and 1.98 ± 0.35% injected dose/g, and tumor to muscle uptake radios for two tracers were 6.77 ± 1.10 and 1.78 ± 0.32, respectively. Dynamic PET revealed that ¹⁸F‐Al‐NOTA‐MATBBN was excreted mainly through the kidneys. GRPR‐binding specificity was also demonstrated by reduced tumor uptake of ¹⁸F‐Al‐NOTA‐MATBBN after coinjection with excess unlabeled MATBBN peptide at 1 h post‐injection. NOTA‐ MATBBN could be labeled rapidly with ¹⁸F using one step method. ¹⁸F‐Al‐NOTA‐MATBBN may be a promising PET imaging agent for prostate cancer. Copyright © 2014 John Wiley & Sons, Ltd.     

Lentivirus‐mediated platelet gene therapy of murine hemophilia A with pre‐existing anti‐factor VIII immunity

Summary. Background: The development of inhibitory antibodies, referred to as inhibitors, against exogenous factor VIII in a significant subset of patients with hemophilia A remains a persistent challenge to the efficacy of protein replacement therapy. Our previous studies using the transgenic approach provided proof‐of‐principle that platelet‐specific expression could be successful in treating hemophilia A in the presence of inhibitory antibodies. Objective: To investigate a clinically translatable approach for platelet gene therapy of hemophilia A with pre‐existing inhibitors. Methods: Platelet FVIII expression in preimmunized FVIII^null mice was introduced by transplantation of lentivirus‐transduced bone marrow or enriched hematopoietic stem cells. FVIII expression was determined with a chromogenic assay. The transgene copy number per cell was quantitated with real‐time PCR. Inhibitor titer was measured with the Bethesda assay. Phenotypic correction was assessed by the tail clipping assay and an electrolytically induced venous injury model. Integration sites were analyzed with linear amplification‐mediated PCR. Results: Therapeutic levels of platelet FVIII expression were sustained in the long term without evoking an anti‐FVIII memory response in the transduced preimmunized recipients. The tail clip survival test and the electrolytic injury model confirmed that hemostasis was improved in the treated animals. Sequential bone marrow transplants showed sustained platelet FVIII expression resulting in phenotypic correction in preimmunized secondary and tertiary recipients. Conclusions: Lentivirus‐mediated platelet‐specific gene transfer improves hemostasis in mice with hemophilia A with pre‐existing inhibitors, indicating that this approach may be a promising strategy for gene therapy of hemophilia A even in the high‐risk setting of pre‐existing inhibitory antibodies.     

Shear‐aggregated fibronectin with anti‐adhesive properties

Biomaterials based on proteins, such as fibronectin, have the potential to guide cell and tissue behaviour during healing as a function of their unique mechanical and bioactive properties. Fibronectin has been reported as a scaffold for attachment of fibroblasts and subsequent deposition of collagen. We have recently developed a derivative process of shear‐aggregated fibronectin that prevents cell attachment without causing cell death. This has potential applications in clinical situations where adhesions form across gliding surfaces and cause loss of function, e.g. peritoneal or flexor tendon adhesions. This in vitro study tested this derivative fibronectin biomaterial and its effects on aggressive adhesion‐forming cells, using rabbit flexor tendon synovial fibroblasts. We investigated degradation of the novel biomaterial, and attachment of fibroblasts to glass coated with the biomaterial, relative to fibroblast attachment to uncoated and fibronectin‐coated glass. We assessed infiltration of the derivative fibronectin biomaterial by fibroblasts and cytotoxicity of the biomaterial to fibroblasts. The interaction between fibroblasts and the derivative fibronectin biomaterial was visualized using time‐lapse photography. The derivative fibronectin biomaterial dissolved by 88% of its mass by 3 weeks. Fibroblast attachment to the novel biomaterial was significantly reduced at 6 h. After 24 h of exposure to the novel biomaterial, fibroblasts did not migrate into it, there was no cell death and no attachment was seen using time‐lapse. This novel derivative fibronectin biomaterial combines inhibition of fibroblast attachment with barrier effects and has suitable mechanical properties for surgical use in preventing adhesions in vivo. Copyright © 2010 John Wiley & Sons, Ltd.     

Parents′ experience of living with a baby with infantile colic – a phenomenological hermeneutic study

Scand J Caring Sci; 2011; 25; 317–324
Parents′ experience of living with a baby with infantile colic – a phenomenological hermeneutic study Background: About 10% of newborn babies have infantile colic which means that they cry more than 3 hours per day. The baby’s crying risks disturbing the early parent–child interaction. Objective: This study aimed to illuminate the meaning of being a parent of a baby with infantile colic. Design: An inductive qualitative interview study. Participants and settings: Twenty‐three parents (12 mothers and 11 fathers) seeking help for infantile colic at a Child Health Clinic in south Sweden, having verified in a diary their babies’ crying to more than 3 hours/day, were individually interviewed between March 2006 and April 2007. Parents were selected to ensure variation in age and gender and if they were first‐time parents. Method: Parent’s narratives were analysed using a phenomenological hermeneutic method. Findings: The main theme found was ‘Colic overshadows everything’. Tired and worried parents experienced living in an inferno. Both fathers and mothers suffered with their babies, felt powerless and overwhelmed by strong feelings and neglected their other needs. To get through this period, parents used various strategies to ease their baby’s pain. Parents forced themselves not to lose control, to keep a stiff upper lip and generally to bear up. Sharing the burden was important. In spite of the suffering, they also felt hope, happiness and gratitude that they had a healthy baby. The results were reflected upon in relation to systems theory, attachment theory and a theory of interpersonal aspects of nursing. Conclusion: It is an important task for professionals to empower parents and help them to endure the colic period and to gain higher self‐esteem as parents. By listening to the parents’ stories they can better understand their situation, offer support and increase self‐efficacy.