Elongated styloid process associated with nevoid basal cell carcinoma syndrome

This article presents a case with nevoid basal cell carcinoma syndrome (NBCCS) and an elongated styloid process. Basal cell carcinoma syndrome, also known as Gorlin-Goltz syndrome, is an autosomal dominant inherited syndrome manifested by multiple defects involving the skin, nervous system, eyes, endocrine system, and bones. Elongated styloid process or calcified stylohyoid ligament cause craniofacial or cervical pain. The actual cause of elongation of the styloid process or the calcification of the stylohyoid ligament is unclear. The cause of elongation of styloid process in this case may be the calcification induced by NBCCS. This report is the first case presentation of NBCCS with elongated styloid process. Elongated styloid process might be described as an anomaly of an NBCCS.     

Necrotizing (malignant) otitis externa: an unusual localization of mucormycosis

Malignant otitis externa (MOE) is a severe infection of external auditory canal and skull base. A 17-year-old diabetic girl was admitted with diabetic ketoacidosis. Cellulitis of her right ear occurred on the second day of hospitalization and a black necrotic scar in the same region appeared on the next day. The lesion rapidly invaded to right side of neck and surrounding tissue of the patient. Therefore, antimycotic therapy was started. Unfortunately the patient died on seventh day of hospitalization because of probably extensive fungal invasion. Physicians should suspect MOE connected to mucormycosis especially in patients with cutaneous lesions of ear unresponsive to antibiotic therapy.     

Exploring coronary atherosclerosis with intravascular imaging

Coronary angiography has been widely used for five decades to evaluate a range of vascular pathologies and triage patients to therapeutic interventions. The inability to directly visualize the artery wall with conventional angiographic techniques has stimulated development of a number of intravascular imaging modalities. These approaches have the potential to provide a more comprehensive characterization of the burden, composition and functionality of atherosclerotic plaque, neointimal hyperplasia and allograft vasculopathy that develop within coronary arteries. The ability to use these modalities in vivo and in a serial fashion has provided a greater insight into the factors that underlie the disease process and guide therapeutic interventions.     

Impact of diabetes on long-term mortality following multivessel percutaneous interventions: An insight into optimal statistical analysis

Background

Several studies have demonstrated better long-term outcomes with drug eluting stents (DES) as compared to bare metal stents (BMS) among diabetics with coronary artery disease (CAD). A significant heterogeneity exists with respect to the optimal statistical strategy to analyze stent related data.

Methods

We used our percutaneous intervention (PCI) registry to identify all diabetics with CAD, who underwent PCI on two or more vessel territories between 2003 and 2009. Long-term mortality was assessed using the social security death index. Six different analytical strategies were applied.

Results

A total of 1568 DES and 336 BMS interventions were encountered in 756 diabetics. Considerable differences were observed in the results between the methods applied. Generalized estimating equation (GEE) approach with an autoregressive correlation structure (GEE) was a robust method to account for the cluster structure, since the measurements taken through time on the same person were assumed to be highly correlated, if they were spaced more closely in time. Diabetics undergoing PCI with BMS had a significantly higher long-term mortality as compared to the patients undergoing DES-PCI [Hazard ratio (95% CI): 1.47 (1.04–2.09)].

Conclusion

There is a great potential for erroneous interpretation of PCI data due to complex spatial and temporal clustering. Use of GEE with autoregressive correlation matrix and robust variance is most optimal to account for the clustered nature of the PCI related data. Using GEE, we observed that there is a 47% (4%–119%) higher hazard for mortality among diabetics undergoing BMS-PCI as compared to diabetics undergoing DES-PCI.     

Cellular rejection and rate of progression of transplant vasculopathy: a 3-year serial intravascular ultrasound study.

Intravascular ultrasound (IVUS) is established as the optimal method for early detection of transplant vasculopathy. The association between cellular rejection and development of transplant vasculopathy remains controversial. This study attempts to determine the rate of progression of transplant vasculopathy lesions and its relationship with cellular rejection in a long-term (> 1 year) IVUS serial follow-up.A study cohort of 47 patients undergoing heart transplantation from 1993 to 1995 was evaluated. Intravascular ultrasound was performed at baseline (within 8 weeks) and annually for a period of 3 years to determine maximum intimal thickness and maximum plaque area in each coronary segment. Significant allograft vasculopathy was defined as a site with intimal thickness > 0.5 mm not present at baseline. Biopsy results were scored by assigning a numerical weight to each ISHLT grade during the first year.Donor lesions ranged from 0.86 to 1.1 mm, showing no evidence of progression at serial follow-up. De novo lesions were identified in 30 patients. These lesions appeared yearly but progressed slowly. The average biopsy score in the entire cohort was 1.1 +/- 0.8. Average biopsy score was > 1.0 in 35 patients with significant linear correlation between the rate of intimal progression and biopsy score (r = 0.42, p = 0.01). Multivariate analysis demonstrated that only the biopsy score correlated with the rate of progression.Lesions of donor atherosclerosis do not change significantly after transplantation. However, de novo lesions continue to develop every year. In patients with evidence of rejection, the rate of progression of transplant vasculopathy correlates with the severity of rejection.