Compression of cystic duct caused by focal nodular hyperplasia.

A 29-year-old female presenting with symptoms of biliary colic was found to have a liver mass compressing the cystic duct. Due to the anatomical placement of the growth, the compressed duct produced symptoms mimicking acute cholecystitis. The mass was diagnosed as focal nodular hyperplasia (FNH) upon biopsy. FNH is commonly found incidentally with nonhepatic clinical presentation or during an unrelated surgical procedure. The scope of this paper is to bring awareness to uncommon causes of biliary colic. To our knowledge, there has been one other paper published with FNH being the primary cause of biliary colic.

Is Pre-Hospital CPR a Risk Factor for Early Death in Patients Transferred to an Adult Burn Center?

BACKGROUND: Burn patients who require CPR before admission to a burn center are anecdotally known to suffer higher mortality than those who do not require pre-hospital CPR. STUDY DESIGN: A retrospective chart review identified adult patients admitted to our burn center between 2013 and 2015. Included patients met 1 or both of the following criteria: 20% or more total body surface area burned and need for intubation before admission to our facility. We sought to identify predictors of early death, late death, and survival among burn patients who underwent CPR before admission. RESULTS: Of the 80 patients meeting inclusion criteria, 17.5% underwent CPR before arrival at our facility. Seventy-nine percent of these died, compared with 29% of the patients who did not require CPR (p = 0.0005). Seventy-one percent of CPR patients died within 48 hours of admission, compared with 8% of non-CPR patients (p < 0.0001). The major predictor of death vs survival after CPR was lower initial arterial pH. CONCLUSIONS: Patients who undergo CPR before transfer to a burn center are at high risk for early death. Predictors of death and early death after CPR may include elevated initial lactate and lower initial arterial pH.

T cell infiltrate and outcome following resection of intermediate-grade primary neuroendocrine tumours and liver metastases.

BACKGROUND: Tumour-infiltrating lymphocytes (TILs) have been shown to predict survival in numerous malignancies. The importance of TILs in primary pancreatic neuroendocrine tumours (NETs) and NET liver metastases (NETLMs) has not been defined. METHODS: We identified 87 patients with NETs and 39 with NETLMs who had undergone resection. Immunohistochemistry was performed to determine TIL counts. Recurrence-free survival (RFS) and overall survival (OS) were determined using the log-rank test. RESULTS: The median follow-up time was 62 months in NET patients and 48 months in NETLM patients. Vascular invasion and histologic grade were the only independent predictors of outcome for NETs and NETLMs, respectively. Analysis of intermediate-grade NETs indicated that a dense T cell (CD3+) infiltrate was associated with a median RFS of 128 months compared with 61 months for those with low levels of intratumoral T cells (P= 0.05, univariate analysis). Examination of NETLMs revealed that a low level of infiltrating regulatory T cells (Treg, FoxP3+) was a predictor of prolonged survival (P < 0.01, univariate analysis). CONCLUSIONS: A robust T cell infiltrate is associated with improved RFS following resection of intermediate-grade NETs, whereas the presence of more Treg correlated with shorter OS after treatment of NETLMs. Further study of the immune response to intermediate-grade NETs and NETLMs is warranted.

Interaction of two hereditary spastic paraplegia gene products, spastin and atlastin, suggests a common pathway for axonal maintenance.

Hereditary spastic paraplegia (HSP) is a neurodegenerative disorder that is characterized by retrograde axonal degeneration that primarily affects long spinal neurons. The disease is clinically heterogeneous, and there are >20 genetic loci identified. Here, we show a physical interaction between spastin and atlastin, two autosomal dominant HSP gene products. Spastin encodes a microtubule (MT)-severing AAA ATPase (ATPase associated with various activities), and atlastin encodes a Golgi-localized integral membrane protein GTPase. Atlastin does not regulate the enzymatic activity of spastin. We also identified a clinical mutation in atlastin outside of the GTPase domain that prevents interaction with spastin in cells. Therefore, we hypothesize that failure of appropriate interaction between these two HSP gene products may be pathogenetically relevant. These data indicate that at least a subset of HSP genes may define a cellular biological pathway that is important in axonal maintenance.