Robotic Adrenalectomy in a Patient With Neurofibromatosis Type 1 and Pheochromocytoma.

A 27-year-old female with a past medical history of neurofibromatosis type 1 (NF1) presented with obstructive hydrocephalus due to a thalamic tumor. The neurosurgery team attempted an operative intervention twice, but both times, the patient experienced a hypertensive emergency and unstable supraventricular tachycardia upon induction of anesthesia. After the second failed surgery, a pheochromocytoma was suspected and the workup demonstrated a left pheochromocytoma. Pheochromocytomas in patients with NF1 are known to be more dangerous and labile, requiring in-depth discussion and preparation by personnel in endocrinology, anesthesia, neurosurgery, and minimally invasive surgery. Once the patient was stable and deemed fit for surgery, a robotic adrenalectomy followed by ventriculoperitoneal shunt placement began. After induction of anesthesia, the patient went into hypertensive emergency again. However, the anesthesia team was prepared and quickly resolved this with medical therapy. Minimally invasive surgeons had the patient's live vitals displayed on their robotic monitors to increase their awareness of patient hemodynamics. This provided live feedback on the surgeons' effect as they removed the pheochromocytoma. Surgeons also performed vein clamping to preemptively see the effects of adrenalectomy. When vein clamping demonstrated safety to proceed, adrenalectomy was completed without complication. This case not only highlights the rare pathology of a woman with NF1 with pheochromocytoma, but it also demonstrates the importance of preparedness and communication among a multidisciplinary team in complex cases to ensure a successful outcome. Novel techniques were also used in performing a robotic-assisted adrenalectomy that can aid other adrenal surgeons.

Weakness Due to Anemia? Go Fish! Melena as a Red Herring in the Diagnosis of Statin-Induced Myopathy.

Statins are a ubiquitous medication class in the primary care setting where they provide effective primary and secondary prevention of coronary artery disease by lowering cholesterol. While statins are mostly safe, muscle-related adverse events are well described. Very rarely patients can actually develop elevated creatine kinase (CK) consistent with myonecrosis. We present a case of progressive anti-hydroxymethylglutaryl coenzyme A reductase (anti-HMGCR) inflammatory myopathy, which was misdiagnosed for many months. Our patient was a 67-year-old gentleman sent to the ER by the Internal Medicine Clinic for profound weakness and melena. He had recently undergone esophagogastroduodenoscopy (EGD) for evaluation of progressive dysphagia and was found to be significantly anemic. Repeat EGD demonstrated a bleeding ulcer, and his weakness was attributed to anemia; however, careful examination demonstrated objective muscle weakness which could not be attributed to anemia alone. Subsequent work-up demonstrated myositis due to HMGCR antibody. Statin cessation and treatment with steroids and intravenous immunoglobulin (IVIG) led to a nearly full recovery in strength and resolution of dysphagia over the next several months.

Anesthetic Errors During Procedures in the United States.

OBJECTIVES: The purpose of this study was to identify the incidence of anesthetic errors per discharges in the United States within these errors, the incidence of death. A secondary aim was to identify any association between the mortality and patient comorbidities. METHODS: A retrospective analysis of the hospitals in the United States using the Nationwide Inpatient Sample (NIS) database during 2007-2014 was performed. The study population consisted of patients who were recorded as inpatient discharges who experienced complications as a result of incorrect anesthetic administration resulting from either an overdose or inappropriate medication administration in the United States. RESULTS: Between 2007 and 2014, a total of 17,116 anesthetic errors were reported. There was a substantial decrease in the total number of these errors over time, from 2483 in 2007 to 1391 in 2014 (44% decrease). There were 131 reported deaths in this cohort (0.77% mortality rate), with 61 mortalities in teaching hospitals (0.86% mortality rate) and 57 in nonteaching hospitals (0.73% mortality rate). During the study period, deaths decreased from 21 in 2007 (0.85% mortality rate) to 11 in 2014 (0.79% mortality rate), corresponding with a 7.1% decrease in the mortality rate. Comorbidities associated with a significant increase in mortality from anesthetic substances included fluid and electrolyte disorders (odds ratio 8.82, 95% confidence interval 5.24-14.83, P < 0.001) and coagulopathies (odds ratio 5.26, 95% confidence interval 2.53-10.93, P < 0.001). CONCLUSIONS: Our study showed that although the incidence of anesthetic errors is small, they do still exist in our hospitals. Certain comorbidities appear to predispose patients to increased risk. The subsets of patients who appear to be at the greatest risk include those with preexisting electrolyte and fluid disorders and coagulopathies.

Genetic Separation of Sae2 Nuclease Activity from Mre11 Nuclease Functions in Budding Yeast.

Sae2 promotes the repair of DNA double-strand breaks in Saccharomyces cerevisiae The role of Sae2 is linked to the Mre11/Rad50/Xrs2 (MRX) complex, which is important for the processing of DNA ends into single-stranded substrates for homologous recombination. Sae2 has intrinsic endonuclease activity, but the role of this activity has not been assessed independently from its functions in promoting Mre11 nuclease activity. Here we identify and characterize separation-of-function mutants that lack intrinsic nuclease activity or the ability to promote Mre11 endonucleolytic activity. We find that the ability of Sae2 to promote MRX nuclease functions is important for DNA damage survival, particularly in the absence of Dna2 nuclease activity. In contrast, Sae2 nuclease activity is essential for DNA repair when the Mre11 nuclease is compromised. Resection of DNA breaks is impaired when either Sae2 activity is blocked, suggesting roles for both Mre11 and Sae2 nuclease activities in promoting the processing of DNA ends in vivo Finally, both activities of Sae2 are important for sporulation, indicating that the processing of meiotic breaks requires both Mre11 and Sae2 nuclease activities.