Gastrointestinal Stromal Tumor Patients with Molecular Testing Exhibit Superior Survival Compared to Patients without Testing: Results from the Life Raft Group (LRG) Registry.

Mutational testing for Gastrointestinal Stromal Tumor (GIST) patients remains underutilized. In this retrospective analysis, the target population (n = 1556) reported: 904 had molecular testing ("Tested") vs. 652 without testing ("Untested"). Overall survival (OS) was 14.7 vs. 12.7 years (p < 0.00001), in metastatic patients 1st line OS was 8.9 vs. 5.9 years in the Tested vs. Untested group (n = 416 vs. n = 254), respectively. From 1st - 3rd-line, no difference has been (self-)reported for progression-free survival (PFS). Dropout to/for further lines of treatment was 15% for patients with a Tested mutation vs. 47% in Untested patients.

Barriers to mutational testing in patients with gastrointestinal stromal tumors (GIST) - a survey of life raft group members.

BACKGROUND: Due to the low mutational testing rate in patients with Gastrointestinal Stromal Tumors (GIST), The Life Raft Group (LRG), a non-profit organization that provides support, advocacy and conducts research for patients with GIST, analyzed various factors that may have an impact on patients' ability to receive mutational testing. METHODS: A survey about mutational testing for patients with GIST or their caregivers, was conducted in June 2020. The survey, sent to 1004 GIST patients and caregivers through email, was promoted through social media with instructions to contact the LRG to participate. The survey was designed by the LRG Patient Registry Department. Members of the LRG, regardless of Patient Registry status, were eligible to participate. RESULTS: A total of 295 patients/caregivers participated in this study (response rate: 29.4%). The percentage of patients who indicated they had received mutational testing was much higher in this survey (80%) than in the general GIST community (26.7%). Several reasons were cited for having a test, including: "My doctor ordered/suggested that I have it done" (54%); "The Life Raft Group advised/suggested I have it done" (25%); "I asked my doctor to have it done" (22%); "I had it done as part of a clinical trial" (5%); "I am not sure" (3%) and "Other" (14%). Mutational testing resulted in a treatment change in 25% of cases. Patients were able to select more than one option when completing this question resulting in a percentage greater than 100. CONCLUSIONS: The LRG membership is voluntary and proactive; patients who join are more likely to participate in surveys and mutational testing, as well as more likely to have a GIST specialist. Mutational testing can influence understanding of a patient's GIST and the treatment best suited to each case. These are extremely important findings, as it helps ensure that patients are on the proper treatment, which should lead to better outcomes.

Course and Outcome of Children with Convulsive Status Epilepticus Admitted to a Pediatric Intensive Care Unit.

Introduction The objective of this study was to describe the course and the outcomes of children with convulsive status epilepticus and to evaluate the differences between two groups of children with new-onset seizures and known seizure disorders. Methods This is a retrospective, single-center study. Children with convulsive status epilepticus admitted to our tertiary care pediatric intensive care unit were included in the study. Medical records were reviewed to obtain the demographic- and seizure-related variables. Results Among 139 children with status epilepticus, 69.7% (n = 99) had a known seizure disorder. Focal seizures were present in 23.9% of children, and 34.6% required intubation; there was an overall mortality rate of 1.2%. The children with new-onset seizures were younger and received electroencephalography (EEG) and neuroimaging more often compared to children with known seizure disorders (p < 0.05). However, an abnormal EEG was more common among children with known seizure disorders (p < 0.001). Conclusions Sub-therapeutic anti-epileptic drugs levels were common among children with known seizure disorders presenting with status epilepticus. Gender, race, insurance status, type of seizures, intubation requirement, lengths of stay, and mortality were not significantly different between the two groups.

What Is the Utility of Routine Complete Blood Count, Liver Function Tests, and Chest X-ray in the Evaluation of Patients with Clinically Node-Negative Breast Cancer?

National Comprehensive Cancer Network guidelines suggest a selective workup of early-stage breast cancer with complete blood count (CBC) and liver function tests (LFTs) and no longer recommend chest X-ray (CXR) to screen for occult metastasis. We evaluated the utility of routine preoperative screening tests in patients with clinically node-negative (cN0) breast cancer. We identified 1611 patients with cN0 breast cancer treated at our institution between October 1998 and December 2015; 94.4 per cent of the patients underwent at least one preoperative screening test: 90.8 per cent CBC, 89.4 per cent LFTs, and 63.6 per cent CXR. Thirty-six per cent of CBCs, 13.1 per cent of LFTs, and 6.7 per cent of CXRs were abnormal. Abnormalities led to 17 CT scans, and one found an occult metastasis. Eleven patients (0.7%) were diagnosed with metastatic disease postoperatively, but only half had abnormal preoperative screening tests. Positive predictive values of preoperative CBC, LFTs, and CXR for occult metastasis are 1.3 per cent, 1.1 per cent, and 1.5 per cent, respectively. Preoperative screening in cN0 breast cancer is of low yield. Even when abnormalities are found, metastasis is present in less than 1 per cent of patients, and a normal study does not rule out metastasis. Routine preoperative determination is not warranted, and staging studies should be limited to patients with signs or symptoms.

The impact of obesity on outcomes for patients undergoing mastectomy using the ACS-NSQIP data set.

PURPOSE: According to the World Health Organization (WHO), 34.7% of females in the United States are obese (BMI ≥ 30) in 2014, compared to 32.5% in 2010. The previous research has demonstrated high BMI as an independent risk factor for surgical complications after breast surgery. As more patients become obese, we sought to examine whether increasing obesity had an effect on outcomes of women who underwent a unilateral mastectomy without breast reconstruction. METHODS: The study reviewed the 2007-2012 ACS-NSQIP database and identified all patients who underwent a unilateral mastectomy without reconstruction. Patients were then categorized and compared according to the World Health Organization obesity classification. Data were analyzed for minor complications (e.g., UTI and SSI) and major complications (e.g., renal failure, sepsis, deep vein thrombosis, return to operating room [RTOR], and cardiac arrest). RESULTS: A total of 7207 women were identified. Median BMI was 27.3 kg/m2. From the cohort, 453 patients (6.29%) had a major complication and 173 patients (2.40%) had a minor complication. 53 (0.74%) had bleeding complications, 148 (2.05%) had a surgical site infection (SSI), 352 (4.88%) RTOR, and 7 (0.01%) died within 30 days. Major complications (p = 0.005) and minor complications (p < 0.001) significantly increased as BMI increased. SSI and RTOR had increasing trends, but were not statistically significant. CONCLUSIONS: This study characterizes the risk of complications in women undergoing unilateral mastectomies and shows that increasing obesity is associated with major and minor postoperative complications. Our finding highlights the need for personalized preoperative risk assessment and counseling of obese patients.

Optimal Modified Frailty Index Cutoff in Older Gastrointestinal Cancer Patients.

The newly characterized modified frailty index (mFI) is a robust predictor of postoperative outcomes for surgical patients. The present study investigates the optimal cutoff for mFI specifically in older gastrointestinal (GI) cancer patients undergoing surgery. All patients more than 60 years old who underwent surgery for a GI malignancy (esophagus, stomach, colon, rectum, pancreas, liver, and bile duct) were identified in the 2005 to 2012 National Surgical Quality Improvement Program, Participant Use Data File (NSQIP PUF). Patients undergoing emergency procedures, of American Society of Anesthesiologists (ASA) five status, or diagnosed with preoperative sepsis were excluded. Logistic regression modeling and 10-fold cross validation were used to identify an optimal mFI cutoff. A total of 41,455 patients (mean age 72, 47.4% female) met the eligibility criteria. Among them, 19.0 per cent (n = 7891) developed a major postoperative complication and 2.8 per cent (n = 1150) died within 30 days. A random sampling by a cancer site was performed to create 90 per cent training and 10 per cent test sample datasets. Using 10-fold cross validation, logistical regression models evaluated the association between mFI and endpoints of 30-day mortality and major morbidity at various cutoffs. Optimal cutoffs for 30-day mortality and major morbidity were mFI ≥ 0.1 and ≥0.2, respectively. After adjusting for age, sex, ASA, albumin ≥3g/dl, and body mass index ≥ 30 kg/m2, mFI ≥ 0.1 was associated with increased mortality (odds ratio (OR) 1.49, 1.30-1.71 95% confidence interval (CI), P < 0.001) and mFI ≥ 0.2 was associated with increased morbidity (OR 1.52, 1.39-1.65 95% CI, P < 0.001). For older GI cancer patients, a very low mFI was a predictor of poor postoperative outcomes with an optimal cutoff of two or more mFI characteristics.

Umbilical Negative Pressure Dressing for Transumbilical Appendectomy in Childern.

BACKGROUND AND OBJECTIVES: Transumbilical laparoscopic-assisted appendectomy (TULAA) carries a high risk for surgical site infection. We investigated the effect of a bio-occlusive umbilical vacuum dressing on wound infection rates after TULAA for patients with acute appendicitis and compared to it with a conventional 3-port appendectomy with a nonvacuum dressing. METHODS: This study was a retrospective chart review of 1377 patients (2-20 years) undergoing laparoscopic appendectomy for acute appendicitis in 2 tertiary care referral centers from January 2007 through December 2012. Twenty-two different operative technique/dressing variations were documented. The 6 technique/dressing groups with >50 patients were assessed, including a total of 1283 patients. RESULTS: The surgical site infection rate of the 220 patients treated with TULAA and application of an umbilical vacuum dressing with dry gauze is 1.8% (95% CI, 0.0-10.3%). This compares to an infection rate of 4.1% (95% CI, 1.3-10.5%) in 97 patients with dry dressing without vacuum. In the 395 patients who received an umbilical vacuum dressing with gauze and bacitracin, the surgical site infection rate was found to be 4.3% (95% CI, 2.7-6.8%). CONCLUSIONS: Application of an umbilical negative-pressure dressing with dry gauze lowers the rate of umbilical site infections in patients undergoing transumbilical laparoscopic-assisted appendectomy for acute appendicitis.

Loss of central inhibition: implications for behavioral hypersensitivity after contusive spinal cord injury in rats.

Behavioral hypersensitivity is common following spinal cord injury (SCI), producing significant discomfort and often developing into chronic pain syndromes. While the mechanisms underlying the development of behavioral hypersensitivity after SCI are poorly understood, previous studies of SCI contusion have shown an increase in amino acids, namely, aspartate and glutamate, along with a decrease in GABA and glycine, particularly below the injury. The current study sought to identify alterations in key enzymes and receptors involved in mediating central inhibition via GABA and glycine after a clinically-relevant contusion SCI model. Following thoracic (T8) 25.0 mm NYU contusion SCI in rodents, significant and persistent behavioral hypersensitivity developed as evidenced by cutaneous allodynia and thermal hyperalgesia. Biochemical analyses confirmed upregulation of glutamate receptor GluR3 with downregulation of the GABA synthesizing enzyme (GAD65/67) and the glycine receptor α3 (GLRA3), notably below the injury. Combined, these changes result in the disinhibition of excitatory impulses and contribute to behavioral hyperexcitability. This study demonstrates a loss of central inhibition and the development of behavioral hypersensitivity in a contusive SCI paradigm. Future use of this model will permit the evaluation of different antinociceptive strategies and help in the elucidation of new targets for the treatment of neuropathic pain.

Peripheral neuropathy of the extremity after hyperbaric exposure.

BACKGROUND: Neurological deficits occurring after diving or hyperbaric exposure are typically due to central nervous system decompression illness (DCI). However, consideration of alternate diagnoses is sometimes warranted. CASE REPORT: A 47-yr-old female hyperbaric technician developed foot drop 2 d following her last hyperbaric exposure. She had worked in chamber once daily at 45 fsw for 90 min. The breathing gas was air until the last 15 min, during which she breathed oxygen. Her history was complicated by a gastric bypass and weight loss totaling 160 pounds within the preceding 9 mo. She was treated for presumed neurological DCI without improvement. The adherence to safe hyperbaric protocols, delayed presentation, ongoing medical issues, and lack of response to therapy brought the diagnosis into question. Extensive neurological evaluation, including nerve conduction studies, revealed a common peroneal mononeuropathy. We concluded that her neuropathy was more likely due to extreme weight loss and malnutrition than peripheral nervous system DCI. DISCUSSION: There are rare reports of DCI affecting the peripheral nerves of the extremities. A literature search revealed only three such cases. Two further reports describe peripheral neuropathy in the extremities of divers that were attributed to other causes: compression by a weight belt and vasculitis. However, peroneal neuropathy is one of the most common focal mononeuropathies of the lower extremity and has been reported in association with extreme weight loss and malnutrition. We attributed our diver's neuropathy to extreme weight loss and malnutrition rather than DCI.