Imaging of vascular biology in the heart

The potential of molecular imaging in visualizing pathophysiologic processes underlying the development, progression, and regression of disease makes it a powerful tool for early detection, risk stratification, and tracking response to therapy. We have seen remarkable advances in molecular imaging of vascular diseases in recent years. To date, most studies have addressed the feasibility of molecular imaging in animal models of human disease, and translation into clinical practice is expected in the near future. Although there is a large body of literature, some groundbreaking, on cardiovascular molecular imaging going back to a decade ago, this review mainly focuses on recent advances in molecular imaging of vascular diseases of the heart.     

Mortality and morbidity in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by hypogammaglobulinemia, defective specific-antibody production resulting in recurrent bacterial infections. Delay in diagnosis and inadequate treatment result in increased irreversible complications and mortality. To determine persistent morbidities, mortality rate and survival in Iranian patients with CVID, hospital records of 72 (39 males and 33 females) diagnosed CVID patients were reviewed. Probabilities of survival after diagnosis of CVID were estimated from Kaplan-Meier life tables. Studied patients were enrolled over a 20-year period (1984-2005). The most commonly observed complication was bronchiectasis (24 cases), followed by splenomegaly, intestinal villous atrophy (11 cases), and failure to thrive (10 cases). Post-diagnosis survival was estimated as 65% for the first 6.5 years, which remains the same until 14 years after diagnosis when the survival curve drops to nearly 45%. The mortality rate among patients who had no regular visits and did not receive periodical IVIG was more remarkable when compared with those who had been followed up timely (p-value = 0.001).The most common cause of death was respiratory failure. Based on our observation, it can be highlighted that all patients with CVID, even under regular immunoglobulin replacement, need close monitoring for early detection of complications and introduction of appropriate management.     

The urethral valve of Guérin and lacuna magna: clinical presentations and urodynamic findings

We described the clinical presentations, radiological findings, urodynamic patterns and endoscopic management in a series of patients with symptomatic lacuna magna (LM). The medical records of 14 boys diagnosed as LM were reviewed, retrospectively. The presence of LM was first diagnosed based on clinical history, physical exam, and radiological findings; then confirmed by urethroscopy. Urodynamic study was performed in 10 boys before and after the procedure. Transurethral incision and fulguration of the valve was performed in all patients. The mean (range) age of patients was 3.1 years (6 months to 8 years). The boys presented with dysuria (n = 9), postvoid dribbling (n = 4), hematuria (n = 3), bloody spotting (n = 3), enuresis (n = 4), and glanular irritation and pain (n = 6). The post-operative period was uneventful in all patients. The symptoms were relieved and repeat radiological exam showed complete resolution of LM in all children by the 3rd post-surgical month. Before the procedure, main urodynamic abnormalities in children were high P(detmax) and detrusor instability that significantly improved within 3-6 months after treatment. Despite evidence suggesting that a LM is a frequent, normal anatomical variant, the valve may cause symptoms such as intermittent hematuria, dysuria, and bloody spotting of underwear. The lesion may be associated with obstructive urodynamic patterns in some children. Endoscopic treatment of patients effectively relieves the symptoms and corrects the urodynamic abnormalities.     

A neonate with cleft palate and a fetal mass in the oral cavity: a rare case of an oral fetus-in-fetu

Fetus-in-fetu is a rare congenital condition in which a malformed fetus-like structure is found in the body of its twin. We report a unique case of a male neonate with cleft palate and a fetus-like structure arising in his oral cavity. The neonate underwent emergent surgical removal of the mass immediately after delivery. Radiological and pathological studies of the resected mass provided supportive evidence for the case of an oral fetus-in-fetu. To our knowledge, there are few cases of oral fetus-in-fetu in the literature. Moreover, the presence of cleft palate in this neonate is of potential interest and clinical importance.     

Acne keloidalis: a novel presentation for tinea capitis

Acne keloidalis is characterized by firm skin-colored papules on the surface of the neck and scalp. We report a case of tinea capitis in 60-year-old female which mimicked acne keloidalis. The lesions resolved following oral antifungal medication.     

Open-label study of valacyclovir 1.5 g twice daily for the treatment of uncomplicated herpes zoster in immunocompetent patients 18 years of age or older

BACKGROUND: Herpes zoster (shingles) is a common disease caused by a reactivation of the latent varicella-zoster virus (chickenpox), which resides in the dorsal root ganglia. Valacyclovir HCl, the L-valyl ester of acyclovir, is an antiviral drug that is used to accelerate the resolution of the herpes zoster rash and associated pain and reduce the duration of postherpetic neuralgia. OBJECTIVE: To demonstrate the safety and efficacy of oral valacyclovir 1.5 g twice daily (bid) for the treatment of uncomplicated herpes zoster in immunocompetent patients over 18 years of age. The dosing schedule of bid versus three times daily is desirable for enhancing patient compliance and to subsequently reduce the incidence of viral resistance. METHODS: One treatment group of 125 patients was administered oral valacyclovir 1.5 g bid for 7 days. Administration of the first dose occurred within 72 hours after onset of rash. Patients were seen and assessed for cutaneous healing, zoster-associated pain (ZAP), and/or zoster-associated abnormal sensations (ZAAS). Patients under 50 years of age were followed for 4 weeks and patients 50 years of age and older were followed for a total of 24 weeks. Patients >or= 50 years were also asked to record a daily diary on pain and abnormal sensations throughout the 24-week study period. Responses to resource use and quality of life questions were also collected. Safety was monitored by means of routine hematologic and biochemical assessments and reporting of adverse experiences. RESULTS: Data from this study were compared with historical control groups both for three times daily antiviral therapy and for placebo. The results showed that twice-daily dosing was as safe and effective as three times daily dosing for the reduction of ZAP and ZAAS. Adverse-effect profiles were similar between the two different regimens, and both treatment groups showed better outcomes than the historical placebo group. Because it is standard of care to administer antivirals for the treatment of acute herpes zoster, a placebo-controlled trial is not possible, necessitating the use of historical controls. CONCLUSION: Oral valacyclovir 1.5 g bid is safe and effective for the treatment of uncomplicated herpes zoster in immunocompetent patients over 18 years of age. Twice-daily dosing may help increase patient compliance and therefore increase the effectiveness of treatment of the acute herpes zoster rash and the prevention of ZAP.     

Involvement of vacuolar protein sorting pathway in Ebola virus release independent of TSG101 interaction

Budding of Ebola virus (EBOV) particles from the plasma membrane of infected cells requires viral and host proteins. EBOV virus matrix protein VP40 recruits TSG101, an ESCRT-1 (host cell endosomal sorting complex required for transport-1) complex protein in the vacuolar protein sorting (vps) pathway, to the plasma membrane during budding. Involvement of other vps proteins in EBOV budding has not been established. Therefore, we used VP40 deletion analysis, virus-like particle-release assays, and confocal microscopy to investigate the potential role of ESCRT-1 proteins VPS4, VPS28, and VPS37B in EBOV budding. We found that VP40 could redirect each protein from endosomes to the cell surface independently of TSG101 interaction. A lack of VPS4 adenosine triphosphatase activity reduced budding by up to 80%. Inhibition of VPS4 gene expression by use of phosphorodiamidite morpholino antisense oligonucleotides protected mice from lethal EBOV infection. These data show that EBOV can use vps proteins independently of TSG101 for budding and reveal VPS4 as a potential target for filovirus therapeutics.