Myocardial disarray in Noonan syndrome

Objective—To characterise the histopathology of the left ventricular hypertrophy commonly associated with Noonan syndrome by assessing the extent of myocyte disarray and therefore to define one aspect of the relation between this disease and idiopathic hypertrophic cardiomyopathy.

Design—Blinded histological analysis.

Setting—Hospital medical school.

Patients—Six hearts of children with the Noonan phenotype and isolated ventricular hypertrophy were compared with age and sex matched controls.

Methods—Histological analysis was performed with an image analyser under light microscopy. Representative sections from the entire left ventricular free wall were examined. Results were expressed as the percentage of fields showing disarray related to the number of fields evaluated: 100 fields were examined for each patient.

Results—In the patients with Noonan syndrome myocardial disarray was present in the ventricular septum in 24 (5·7)% (mean (SD)) of fields and in the free wall in 22·2 (6·8)%. In the controls disarray was present in the septum in 3·8 (2·3)% of fields and in the free wall in 2·4 (2·8)%. In both regions the extent of disarray was significantly greater in patients with Noonan syndrome (p < 0·0005; 95% confidence interval 14 to 26·3 for the septum: p < 0·005, 95% confidence interval 11·4 to 28·2 for the free wall).

Conclusions—The ventricular hypertrophy associated with Noonan syndrome is histologically similar to hypertrophic cardiomyopathy but whether the two diseases are the expression of the same genetic defect remains to be determined.

     

The Incidence and Outcomes of Ischemic Hepatitis: A Systematic Review with Meta-analysis

Background

Ischemic hepatitis is a devastating cause of acute liver injury. Data are limited regarding its incidence and outcomes.

Methods

Systematic review and meta-analysis of studies from PubMed, EMBASE, and Web of Science with specific search terms. Inclusion criteria included case series with >10 patients and clear case definition (especially liver enzyme levels >10 times the upper limit of normal).

Results

Twenty-four papers met inclusion criteria. A total of 1782 cases were identified in these papers (mean 78 per paper, range 12-322). The pooled average age of the included patients was 64.2 years, and their mean peak aspartate aminotransferase level, alanine aminotransferase level, and total bilirubin were 2423 IU/L, 1893 IU/L, and 2.55 mg/dL, respectively. Ischemic hepatitis was present in 2 of every 1000 admissions; including 2.5 of every 100 intensive care unit admissions and 4 of 10 admissions associated with an aminotransferase level >10 times the upper limit of normal. The pooled proportions of patients with ischemic hepatitis who had a predisposing acute cardiac event or sepsis were 78.2% and 23.4%, respectively. The proportion of patients with a documented hypotensive event of any duration was 52.9%. Overall, the pooled rate of survival to discharge was 51% (range 23.1%-85.7%).

Conclusions

Ischemic hepatitis is a common cause of severe acute liver injury and is associated with a significant risk of in-hospital death. A major opportunity in the management of ischemic hepatitis is recognition of the condition without documented hypotension.     

Esthesioneuroblastoma: An Update on the UCLA Experience, 2002–2013

Objectives To profile the clinical presentation and treatment results of esthesioneuroblastomas at the University of California, Los Angeles (UCLA), from 2002 to 2013. Design Retrospective review. Setting Tertiary academic institution. Participants Forty-one patients with esthesioneuroblastomas treated at UCLA. Main Outcome Measures Overall survival (OS) and recurrence-free survival (RFS). Results Thirty-six patients were included with a mean age of 50.1 years and a median duration of follow-up of 33 months. The 5-year RFS and OS were 54% and 82%, respectively. Modified Kadish stage was the only factor identified to affect OS. Multivariate analysis demonstrated that tumor grade was the only factor that had an independent impact on RFS. There was no statistical difference in survival among the surgical approaches chosen. Conclusions The updated data on the UCLA experience reveals that all three surgical approaches chosen provide comparable survival, although longer follow-up will be needed to ascertain if these findings hold true. The endoscopic approach had a statistically significant decrease in length of hospital stay and a trend toward reduced blood loss, intensive care unit admission, and complications. The modified Kadish staging was the only factor identified to predict OS. Multivariate analysis revealed that tumor grade was an independent predictor of recurrence; therefore, its importance should be emphasized in future staging systems.     

Case Report: Conversion of a Low-Flow to High-Flow Priapism

Priapism is defined as an erection lasting for more than 4 hours and can be grouped into 3 distinct subtypes: ischemic (low-flow), stuttering and non-ischemic (high-flow). Herein, we present an interesting case of conversion from a low-flow to high-flow priapism after a distal shunting procedure. This is a rare phenomenon that has a paucity of documented cases. Diagnosis requires prompt clinical suspicion and confirmatory testing including penile cavernosal blood gases and Doppler ultrasound.Key Words: Priapism, Ischemic priapism, Non-Ischemic priapism     

Human Mate Selection and Addiction: a Conceptual Critique

The authors review past work on modeling human mate selection, and suggest, using illustrations from existing literature on the impact of alcoholism on relationship formation and dissolution and reproduction, that the challenges of adequately characterizing human mate selection have not yet been overcome. Some paths forwards are suggested.