Solitary fibrous tumour of the cheek: An unusual presentation of a rare soft tissue tumour

This case report discusses the unusual presentation and ultrasound features of a solitary fibrous tumour of the face. Solitary fibrous tumour is an uncommon form of soft tissue tumour which, although seen predominantly within the lung pleura, can occur throughout the body in sites such as the peritoneum, mediastinum and head and neck. Ultrasound is an excellent imaging modality in the assessment of soft tissue masses in the head and neck. The ultrasound features demonstrated by this example of solitary fibrous tumour are reviewed. This report also highlights that ultrasound alone is ultimately limited in reaching a definitive diagnosis. The roles of other investigations such as ultrasound-guided biopsy and cross-sectional imaging are discussed.     

Beta thalassemia in Melanesia: association with malaria and characterization of a common variant (IVS-1 nt 5 G----C)

Data on the distribution of beta thalassemia among over 6,000 Melanesians reveals a major difference in the carrier rates between populations in the malarious coastal regions of New Guinea and those living in the historically malaria-free Highlands. The island of Maewo in Vanuatu has a particularly high incidence of beta + thalassemia associated with a single restriction enzyme haplotype. Direct cloning into a plasmid vector and sequence analysis demonstrate that the mutation is a G to C transversion at position 5 of intron 1 of the beta- globin gene. Oligonucleotide probe surveys indicate that this variant accounted for all cases of beta thalassemia studied from Maewo. It is also common in coastal Papua New Guinea where haplotype and oligonucleotide probe data suggest that the molecular basis of beta thalassmia is more heterogeneous.     

Delayed presentation of vernix caseosa peritonitis

INTRODUCTION

Vernix caseosa peritonitis (VCP) is a rare and poorly recognised condition resulting from a sustained foreign body reaction to the vernix caseosa of the baby. This case-based review aims to highlight its importance for any medical team managing patients with peritonitis who have undergone a recent Caesarean section.

CASE REPORT

A 31-year-old woman presented 5 weeks after a Caesarean section with symptoms and signs of peritonitis.

CONCLUSIONS

Laparotomy and peritoneal lavage is the mainstay of treatment for VCP. Knowledge of the condition may stop inadvertent resection of normal intra-abdominal organs. Greater awareness of VCP is required to ensure earlier recognition as patients can recover well following timely operative intervention.     

Protective effect of mitochondria-targeted antioxidants in an acute bacterial infection

Acute pyelonephritis is a potentially life-threatening infection of the upper urinary tract. Inflammatory response and the accompanying oxidative stress can contribute to kidney tissue damage, resulting in infection-induced intoxication that can become fatal in the absence of antibiotic therapy. Here, we show that pyelonephritis was associated with oxidative stress and renal cell death. Oxidative stress observed in pyelonephritic kidney was accompanied by a reduced level of mitochondrial B-cell lymphoma 2 (Bcl-2). Importantly, renal cell death and animal mortality were both alleviated by mitochondria-targeted antioxidant 10(6′-plastoquinonyl) decylrhodamine 19 (SkQR1). These findings suggest that pyelonephritis can be treated by reducing mitochondrial reactive oxygen species and thus by protecting mitochondrial integrity and lowering kidney damage.Normally, the kidney and urinary tract are germ-free. However, during their lifetimes, about 40% of women and 12% of men experience urinary tract infections (UTIs) (1). Acute pyelonephritis (APN) is a potentially life-threatening complication of UTI that occurs when infection progresses to the upper urinary tract. The uropathogen most frequently associated with this disease is the pyelonephritogenic subset of Escherichia coli, which is responsible for up to 85% of both complicated and uncomplicated UTIs (2). This disease is frequently accompanied by bacterial invasion and stimulation of acute inflammatory response (3, 4). Toxin-induced epithelial damage and bladder hemorrhage contribute further to the pathogenicity of uropathogens in the kidney, with progression leading to renal damage including renal scarring and in extreme cases septicemia (5, 6). Ultimately, renal scarring is a cause of substantial morbidity (7, 8).Leukocyte infiltration in response to bacterial invasion is an important contributor to renal tissue damage (9, 10). Production and extracellular release of reactive oxygen species (ROS) by infiltrating leukocytes can lead to kidney injury and dysfunction (11, 12). Consequently, oxidative stress in renal cells may be a critical factor in the pathogenesis of pyelonephritis whereas pharmacological management of the oxidative stress response may provide a therapeutic effect in preventing renal pathologies (13–18). However, the issue is complicated by the diversity of ROS-generating mechanisms and their differential contribution to host defense from infection and collateral tissue damage. Mitochondria and NADPH oxidases (19) are the two principle sources of ROS although their relative contribution to inflammatory pathologies is not well-defined. A new class of antioxidants that specifically target mitochondrial ROS (hereafter referred to as SkQ1 and SkQR1) have been recently developed and shown to have a beneficial effect in a number of cell pathologies (20–28).In this study, we explored mechanisms of APN progression considering various aspects of interaction of renal cells with leukocytes and bacterial pathogens. The goal was to gain insight into the role and mechanisms of induction of oxidative stress in eukaryotic components of the system and find an approach of directed correction of the pathological oxidative changes in renal tissue. We analyzed the relevance of the strategy of protecting the kidney based on the activation of prosurvival and blockage of prodeath signaling pathways involving mitochondria. We evaluated chimeric compounds carrying an antioxidant moiety as potential agents to efficiently alleviate the deleterious consequences of APN. To facilitate the future design of directed pharmacologic interventions to normalize renal function subsequent to APN, we explored the role of mitochondria and oxidative stress in this pathology using positively charged membrane-permeable, mitochondrial-targeted compounds (29). We demonstrated that specific targeting of mitochondrial ROS by antioxidant resulted in a significant protective effect in animal models of APN. These results illustrate the role of mitochondrial ROS in renal tissue damage in the context of acute infection and suggest a therapeutic potential of mitochondrial antioxidants.     

Failure of cyclophosphamide to prevent cerebritis in Wegener's granulomatosis

Two patients with diffuse cerebritis due to Wegener's granulomatosis are described. The cerebritis developed despite prolonged treatment with cyclophosphamide. This may be the first report of such an occurrence. Both patients had excellent functional recovery with intravenous corticosteroids.