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Glomerulopathy with Fibronectin Deposits (GFND) is a rare, autosomal dominant disease characterized by proteinuria, hematuria and progressive renal failure associated with glomerular deposition of fibronectin, frequently resulting in end-stage renal disease (ESRD). There is no established treatment for this condition beyond conservative measures such as blood pressure control and the use of angiotensin-converting enzyme (ACE) inhibitors. We present a case of GFND associated with progressive chronic kidney disease (CKD) and nephrotic range proteinuria showing a sustained response to prednisone treatment. A 27-year-old G2P2 Caucasian female presented with 3 g/day of proteinuria, serum creatinine (Cr) 0.7 mg/dL, inactive urinary sediment and normotension without medication. She was part of a large family with glomerular disease, including three members who died of cerebral hemorrhage or stroke in their thirties. The patient's kidney biopsy showed mesangial deposition of fibronectin consistent with GFND. No interstitial fibrosis was seen. Genotyping revealed the Y973C FN1 gene mutation. Despite maximal tolerable ACE inhibition, proteinuria increased to 4-6 g/g Cr and serum Cr increased to 1.0 mg/dL. She was treated with prednisone 60 mg (~ 1 mg/Kg) daily for 2 mos and then tapered by ~ 0.2 mg/Kg every month for 6 mos of total therapy. Proteinuria decreased to ~ 1 g/g Cr for > 5 years and serum Cr stabilized in the 1.2 mg/dL range with treatment. No significant side effects were encountered. In conclusion, this protocol should be considered in GFND patients with nephrotic range proteinuria despite maximal angiotensin system inhibition who have relatively preserved renal function.
The recommended rocuronium dose for rapid sequence intubation is 1.0mg/kg; however, the optimal dose for emergency airway management is not clear. We assessed the relationship between rocuronium dose and first-attempt success among emergency department (ED) patients undergoing rapid sequence intubation.
This is a secondary analysis of the National Emergency Airway Registry (NEAR), an observational 25-center registry of ED intubations. Ninety percent recording compliance was required from each site for data inclusion. We included all patients > 14years of age who received rocuronium for rapid sequence intubation from 1 Jan 2016 to 31 Dec 2018. We compared first-attempt success between encounters using alternative rocuronium doses (< 1.0, 1.0-1.1, 1.2-1.3 and ≥1.4mg/kg). We performed logistic regressions to control for predictors of difficult airways, indication, pre-intubation hemodynamics, operator, body habitus and device. We also performed subgroup analyses stratified by device (direct vs. video lcess 94.9% versus 88.6% when higher doses of rocuronium were used. The rates of all peri-intubation adverse events and desaturation were similar between dosing groups, laryngoscope type utilized and varying pre-intubation hemodynamics.
Rocuronium dosed ≥1.4mg/kg was associated with higher first attempt success when using direct laryngoscopy and among patients with pre-intubation hypotension with no increase in adverse events. We recommend further prospective evaluation of the dosing of rocuronium prior to offering definitive clinical guidance.
Rocuronium dosed ≥1.4 mg/kg was associated with higher first attempt success when using direct laryngoscopy and among patients with pre-intubation hypotension with no increase in adverse events. We recommend further prospective evaluation of the dosing of rocuronium prior to offering definitive clinical guidance.Robotic-assisted simple prostatectomy (RASP) has proven to be an effective minimally invasive option for benign prostatic enlargement (BPE) in recent years. Single-site surgery is theorized to reduce post-operative pain beyond traditional minimally invasive approaches. We sought to assess whether use of a single-port robotic platform decreases post-operative opioid use in patients undergoing robotic-assisted simple prostatectomy (RASP). A retrospective review was performed of all patients undergoing RASP our institution from November 2017 to July 2019. Demographic, intraoperative, and post-operative data, including morphine equivalent (ME) use, were collected. Patients were stratified by robotic platform utilized. Propensity score matching using nearest neighbor method was performed using prostate volume, Charlson comorbidity index (CCI), and post-op ketorolac use in 41 fashion. Chi-squared analysis and Kruskal-Wallis analyses were utilized. Two-hundred-and-seven men underwent RASP. After matching, 80 patients (64 multi-port, 16 single-port) were included in the analysis. Groups were comparable for age, body mass index, CCI, prostate volume, prior opioid use, and use of scheduled ketorolac post op. The single-port approach was associated with a reduction in MEs once admitted to the floor (5 vs. 11 mg, p = 0.025) and an increase in the proportion of patients who did not require any narcotics post-operatively (44 vs. 19%, p = 0.036). In a propensity matched cohort of patients undergoing RASP at a single institution, use of the single-port robotic system conferred a significant decrease in post-operative narcotic use by approximately 50%.Technique, indications and outcomes of laparoscopic splenectomy in stable trauma patients have not been well described yet. All hemodynamically non-compromised abdominal trauma patients who underwent splenectomy from 1/2013 to 12/2017 at our Level 1 trauma center were included. Saracatinib Demographic and clinical data were collected and analysed with per-protocol and an intention-to-treat comparison between open vs laparoscopic groups. 49 splenectomies were performed (16 laparoscopic, 33 open). Among the laparoscopic group, 81% were successfully completed laparoscopically. Laparoscopy was associated with a higher incidence of concomitant surgical procedures (p 0.016), longer operative times, but a significantly faster return of bowel function and oral diet without reoperations. No significant differences were demonstrated in morbidity, mortality, length of stay, or long-term complications, although laparoscopic had lower surgical site infection (0 vs 21%).The isolated splenic injury sub-analysis included 25 splenectomies,76% (19) open and 24% (6) laparoscopic and confirmed reduction in post-operative morbidity (40 vs 57%), blood transfusion (0 vs 48%), ICU admission (20 vs 57%) and overall LOS (7 vs 9 days) in the laparoscopic group.