Calciphylaxis: A condition mimicking necrotizing vasculitis

Summary A patient with end stage renal disease developed ischaemic skin necrosis and digital gangrene. He had diffuse arterial calcification associated with hyperparathyroidism secondary to renal failure. The patient received inappropriate cyclophosphamide therapy as he had been misdiagnosed as having an inflammatory vasculitis. This clinical picture, previously named calciphylaxis should come into the differential diagnosis of systemic vasculitis in a uraemic patient with hyperparathyroidism.     

Germline mutations of the p53 gene in children with malignant solid tumors

Although somatic mutations of p53 are the most common genetic changes observed to date, the frequency of germline p53 mutations is found to be very low in sporadic malignant tumors. It has been postulated that de novo germline p53 mutations may occur in a substantial population of patients in pediatric age group, who die of their disease and do not propagate the mutation. To determine the frequency and type of p53 germline mutations in pediatric patients, we screened 65 children who were consecutively admitted with primary malignant solid tumors.     

The safety and efficacy of Isola-Galveston instrumentation and arthrodesis in the treatment of neuromuscular spinal deformities

BACKGROUND: Implant systems that realign and stabilize a deformed spine continue to evolve. The purpose of the study of this case series was to determine the safety and effectiveness of a system designed to integrate hook, wire, screw, and post anchors for the treatment of a wide spectrum of neuromuscular disorders associated with pelvic deformity or the potential for deformity. METHODS: Forty-seven consecutive patients who had a spinal deformity that was due to cerebral palsy or an upper motor-neuron cerebral palsy-like disease (thirty-one patients), myelomeningocele (nine), Duchenne muscular dystrophy (four), or other disorders (three) were managed with Isola-Galveston instrumentation and arthrodesis. The average age at the time of the operation was fourteen years and three months (range, five years and four months to twenty-three years and nine months). Eight patients (17 percent) had an additional anterior discectomy and arthrodesis without instrumentation, and three (6 percent) had an additional decancellation egg-shell osteotomy. The forty-seven patients were followed for an average of forty-seven months (range, twenty-four to 100 months). The complications were tabulated to assess the safety of the procedure, and the correction of each deformity was calculated to determine the efficacy. RESULTS: There were no deaths, acute wound infections, or serious neurological problems. Reoperation was necessary in five patients (11 percent). One reoperation was performed because of a delayed deep wound infection; one, because of delayed sterile drainage; and one, for a pseudarthrosis repair. The remaining two reoperations were done for removal of an implant because the cephalad portion had become prominent. In addition to the pseudarthrosis that required a reoperation, there were three possible pseudarthroses that did not require a reoperation (overall prevalence of pseudarthrosis, 9 percent). Postoperative bracing was used for eleven patients (23 percent); it did not influence the rate of pseudarthrosis or possible pseudarthrosis. The average preoperative scoliosis of 70 degrees was corrected to 24 degrees (a 66 percent correction) at the time of the latest follow-up, and the average preoperative pelvic obliquity of 27 degrees was corrected to 5 degrees (an 81 percent correction). A survey of the patients, parents, and caregivers indicated that 96 percent of them were satisfied or very satisfied with the result of the operation. CONCLUSIONS: Isola-Galveston instrumentation seems as safe and effective as other types of instrumentation that have been studied in comparable series in the literature. Isola-Galveston instrumentation is probably more effective for the correction of pelvic obliquity and the maintenance of correction. Only a posterior procedure is used, and the instrumentation appears to decrease the need for an additional anterior approach. Spinal hook, wire, screw, and post anchors have been successfully integrated into one posterior spinal implant system.     

Does corticosteroid usage in rhinoplasty cause mood changes?

In this study, the psychological effects of single-dose corticosteroids administered to patients who had undergone rhinoplasty were assessed. A total of 30 rhinoplasty patients were included in the study and were randomly assigned to 1 of 2 groups. Preoperatively, patients completed the Bech Rafaelsen Mania Scale and the Beck Depression Inventory. Dexamethasone 10 mg was given intravenously just before surgery to the first group, but no medication was administered to the second group. On the first postoperative day, patients were seen again, and the Bech Rafaelsen Mania Scale and the Beck Depression Inventory were again completed. Periorbital edema and ecchymosis were graded, and psychological well-being was measured on a standard visual analog scale. All patients and physicians were blinded to treatment until the end of the study. Results show that administration of a single-dose of dexamethasone 10 mg caused neither euphoria nor depression. No significant differences were observed between steroid and control groups in terms of patients’ psychological well-being. With single-dose dexamethasone, periorbital edema was significantly reduced on the first 2 postoperative days, and upper eyelid ecchymosis was significantly decreased only on the first postoperative day. However, preoperative steroid administration had no influence on ecchymosis of the lower eyelid. The authors conclude that single-dose dexamethasone 10 mg can be used safely to reduce periorbital edema and ecchymosis in rhinoplasty patients.     

Hepatitis C virus reactivation in cancer patients in the era of targeted therapies

The purpose of this review is to summarize the evidence of hepatitis C reactivation in cancer patients in the era of targeted therapies.Targeted therapies are novel therapeutics frequently used in cancer patients.During treatment with targeted therapies,viral replication is one of the major problems that can occur.The PubMed database,ASCO,and ASCO Gastrointestinal Cancer Symposium abstracts were searched up until September 15,2013 using the following search keywords:"targeted therapies,rituximab,alemtuzumab,brentuximab,hepatitis,hepatitis C reactivation,tyrosine kinase inhibitors,imatinib,mammalian target of rapamycin(mTOR)inhibitors,everolimus,anti-HER therapies,trastuzumab,pertuzumab,lapatinib,antiepidermal growth factor receptor therapies,cetuximab,panitumumab,and ipilimumab".Papers considered relevant for the aim of this review were selected by the authors.The data about rituximab-induced hepatic flare in hepatitis C virus(HCV)positive patients is controver-sial.However,there is the possibility of life-threatening hepatic flare that can develop after HCV ribonucleic acid(HCV-RNA)viral load increases.Routine followup of liver function tests should be advised.Especially in high-risk patients,such as those with baseline chronic active hepatitis and cirrhosis,and where there are plans to administer rituximab concomitantly with corticosteroids,it is advised to have close follow-up of HCV viral load.The data is insufficient to make accurate statements about the association of alemtuzumab therapy and HCV reactivation.However,alemtuzumab may cause deep immunosuppression.Due to this,it is better to follow up with liver function tests and HCV RNA levels during alemtuzumab therapy.Brentuximab has effects on antibody dependent cellular toxicity and may decrease humoral immunity.Thus,we believe that during brentuximab treatment of HCV infected patients,clinicians may encounter hepatitis C reactivation.There have been no reported cases of hepatitis C reactivation with imatinib therapy.However,there are many reports of hepatitis B reactivation with imatinib treatment.Based on the evidence of hepatitis B reactivation with imatinib and the effects of imatinib on immune system functions,we suggest that imatinib therapy might be a risk factor for HCV reactivation.Anti-human epidermal growth factor receptor 2 therapies are not associated with hepatic flare in HCV infected patients.Post-transplant studies reported that mTOR was safely administered to patients with active hepatitis C without causing hepatic flare.Cetuximab and panitumumab have not been associated with HCV reactivation.Two cases of HCV infected melanoma were safely treated with ipilimumab without any HCV reactivation or hepatic flare.Targeted therapies are a new and emerging area of oncology treatment modalities.While treating HCV infected cancer patients,clinicians should be mindful of the immunosuppressive properties of targeted therapies.Further randomized trials are needed to establish algorithms for this issue.     

In vivo effect of losartan on platelet aggregation in patients with hypertension

The angiotensin II receptor, losartan, has been found to inhibit platelet aggregability to some extent in in vitro experiments. There have been conflicting results about the in vivo effects of losartan. We sought to clarify the in vivo effect of losartan on platelet aggregation. Forty patients with grade I essential hypertension were treated with losartan for 3 weeks. Platelet aggregation tests with adenosine diphosphate (ADP) and ristocetin were analyzed and compared before and at the end of the study. Losartan effectively decreased systolic (SBP) and diastolic (DBP) blood pressure. Mean SBP before and after treatment was 159.6 ± 12.8 and 149.2 ± 17.3mmHg, respectively. Mean DBP decreased from 93.7 ± 8.2 to 87.7 ± 10.3mmHg after treatment. The results of the platelet aggregation tests with ADP and ristocetin were not significantly different when both rate and amplitude of maximal aggregation were included. Peak platelet aggregation with ADP regarding the lowest light transmission in the aggregometer was 59.8% ± 24.3% before and 58.3% ± 18.1% after the treatment. The same variables with ristocetin were 66.8% ± 21.6% and 60.8% ± 23.3%, respectively. In vivo effects of losartan on platelet aggregation with ADP and ristocetin were insignificant.