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PERFORATION OF THE GREAT VESSELS DURING TUNNELED INTERNAL JUGULAR DIALYSIS CATHETER PLACEMENT
NKF 2015 Spring Clinical Meetings Abstracts
257 BRACHIO-OTO-RENAL (BOR) SYNDROME Ranjeet Singh, Elwaleed Elnagar, Aziz Bakhous, Ravinder Pal Bhatti Introduction: Brachio-Oto-Renal syndrome is an uncommon cause of ESRD. It should be considered along with other possibilities in right clinical scenario. Clinician’s awareness of the diagnosis is important because of significant implication in prenatal testing and regular screening of affected individuals and at risk family members. Case description: We report a 21 year old male sent to hospital by primary care physician because routine labs showed Blood urea nitrogen=70, and Creatinine=10. Patient had history of worsening fatigue and occasional nausea for 2yrs. Past medical history included recent diagnosis of depression; conductive hearing loss in left ear since early childhood secondary to ossicular fixation. There was no history of recent nephrotoxic substance use. No history ofillicit drug abuse or any recent infections. His father had congenital unilateral renal agenesis; hearing loss, preauricular pit, and unilateral neck mass. Two sisters were stillborn, both had renal agenesis. Significant findings on physical exam including preauricular pit, thick helix of ear and hypertension. Lab reported 7gm/day proteinuria, anemia of chronic disease in addition to confirming outpatient labs. Ultrasound showed atrophic kidneys. Clinical diagnosis of BOR syndrome was made. Patient was started on peritoneal dialysis. Renal biopsy showed FSGS. Genetic analysis was positive for EYA1 gene mutation. Discussion: BOR syndrome is an autosomal dominant disorder characterized by triad of Brachial arch, Otological, and Renal anomalies. Renal malformations may range from mild unilateral hypoplasia to bilateral renal agenesis. Brachio-oto Syndrome (BOS) is a variant without renal involvement. Penetrance is 100%, though expressivity is highly variable among members of same family. True prevalence is unknown as data from studies varies widely. Diagnosis is made based on clinical criteria and requires high clinical suspicion. Accurate diagnosis is important as it has potential implications in management and prenatal genetic testing and screening of family members. Prenatal Diagnosis with genetic analysis is available for families with known EYA1 mutation.
258 PRIMARY RENAL CELL CARCINOMA IN A TRANSPLANTED ALLOGRAFT KIDNEY Siriki R, Syed S, Teran FJ. Tulane University School of Medicine, New Orleans, LA. Recipients of transplanted kidneys are susceptible to malignancy due to their immunocompromised state. Primary cancers involving the kidneys occur mostly in the native. We report a rare case of primary renal cell carcinoma (RCC) in a transplanted kidney 22 years after transplantation. 51 y/o African American male, with a living related kidney in 1992, presented with a history of weight loss and fatigue for a few months. His immunosuppressant therapy included prednisone 5mg and tacrolimus 1mg twice daily. Examination was unremarkable with no tenderness over the transplanted kidney. Pertinent labs included creatinine of 3.51 mg/dL (baseline of 1.7 mg/dL) and sub-therapeutic levels of tacrolimus. Workup for his graft failure included an ultrasound which showed a 6 cm solid, non-calcified mass through the mid kidney and a 1.7 cm mass in the lower pole. Further evaluation with an MRI showed changes within the 2 masses concerning for RCC. A biopsy showed histological and cytological pattern of tumor cells favoring papillary RCC, type 2. The patient will undergo a radical allograft nephrectomy. Recipients of kidney allografts are two to three times more likely to develop a malignancy compared to the general population. They are at an increased risk of developing a carcinoma in the native kidneys which increases up to 100 fold if they were on dialysis prior to transplantation. RCC accounts for 2% of all cancers in the general population. The majority of primary RCC in transplant recipients develop in the native kidneys, but primary RCC in the transplanted allograft is rare with an incidence of only 0.19% to 0.5%; occurring at a mean of 12.6 years after transplantation. The transplant population has a higher risk of post-transplant malignancy which can lead to graft loss and death. It is highly recommended that transplant recipients have close monitoring for malignancies. Early detection in these patients is essential with the goal of early treatment to prevent graft loss and death.
Am J Kidney Dis. 2015;65(4):A1-A93
259 PYRIDORINTM TREATMENT IN MOUSE MODELS OF AKI: Nataliya Skrypnyk1, J. Wesley Fox2, Carrie Taylor2, Ray Harris1, Billy Hudson1, Paul Voziyan1, Mark de Caestecker1 1 Vanderbilt University Medical Center (VUMC), Nashville, TN, USA; 2 NephroGenex, Inc., Raleigh, NC, USA Pyridorin (PYR), an inhibitor of pathogenic oxidative chemistries, has a favorable safety profile in clinical trials and has been shown to scavenge reactive oxygen species (ROS) and reactive carbonyl species (RCS) in vitro and in vivo animal studies. ROS and RCS are highly up regulated during kidney injury. In this study we propose that PYR treatment will reduce injury and prevent long-term fibrosis after ischemia-reperfusion acute kidney injury (IR-AKI). Mouse IR-AKI models: moderate ischemia-reperfusion AKI (unilateral ischemia time 26 min and simultaneous contralateral nephrectomy) and severe ischemia–reperfusion AKI (unilateral ischemia time 31 min and delayed contralateral nephrectomy on day 8). PYR was provided to mice in drinking water at 500 mg/kg BW/day starting 72 h prior to injury and continued until sacrificed, except for mice with moderate IR-AKI where PYR was administered 1 day after injury induction. PYR was given to mice via gavage feeding twice a day at 200 mg/kg for 72 h after AKI induction to ensure proper postoperative dosing. Renal function was assessed by serum creatinine, renal tubular injury with urinary Kim1 (days 1 and 3), and post-injury fibrosis with qRT-PCR for renal fibrosis markers (aSMA, Col1a1, Col3a1) on day 28 after injury. PYR ameliorated the increase in serum creatinine on days 9 and 14 after injury, in urinary Kim1 expression on days 1 and 3, and in postinjury fibrosis markers on day 28 after severe I/R-AKI. PYR had no effect on serum creatinine (day 3, 5 and 7) in moderate I/R-AKI when treatment was delayed 24 hours after injury. PYR ameliorates injury and reduces post-injury fibrosis in severe IRAKI when administered 3 days before injury, but has no effect on functional recovery or renal injury when administered 24 hours after moderate injury. These data suggest that pre-treatment with PYR can ameliorate injury and prevent progression to chronic kidney disease in patients with AKI. Supported by research grant from NephroGenex, Inc. JWF, BH and VUMC are stockholders of NephroGenex, Inc.
260 PERFORATION OF THE GREAT VESSELS DURING TUNNELED INTERNAL JUGULAR DIALYSIS CATHETER PLACEMENT Lindsay M Smith, Dan Bucaloiu, Jamie Green, Susan Trocciola, Geisinger Medical Center, Danville, PA, USA Complications related to tunneled dialysis catheter placement are relatively rare in the era of ultrasound and fluoroscopy guidance. We present a case of transection of the great vessels during fluoroscopic tunneled dialysis catheter placement under general anesthesia. A 69-year-old woman with end stage renal disease on peritoneal dialysis underwent an elective umbilical and inguinal hernia repair. At the end of surgery, a right internal jugular tunneled dialysis catheter (28 cm PermCath) was inserted under fluoroscopic guidance while the patient was under general anesthesia. After completion of a dialysis session, the patient experienced transient confusion and dysarthria prompting referral to our hospital. Brain imaging revealed no acute pathology. A chest x-ray revealed the catheter overlying the heart shadow with the tip overlying the left atrium. Additional imaging confirmed the presence of the catheter tip within the left atrium. The patient underwent urgent sternotomy, which exposed the catheter completely transecting the main pulmonary artery, perforating and entering the roof of the left atrium. The catheter was surgically removed on cardiopulmonary bypass and the holes were repaired. The patient tolerated the procedure well and made a complete recovery. We would like to use this illustrative case to raise awareness of this complication of a relatively common procedure in Nephrology. Careful review of post procedure chest x-ray and fluoroscopy imaging prior to initiating hemodialysis are crucial in identifying dialysis catheter malposition and preventing major adverse outcomes.
A79
257 BRACHIO-OTO-RENAL (BOR) SYNDROME Ranjeet Singh, Elwaleed Elnagar, Aziz Bakhous, Ravinder Pal Bhatti Introduction: Brachio-Oto-Renal syndrome is an uncommon cause of ESRD. It should be considered along with other possibilities in right clinical scenario. Clinician’s awareness of the diagnosis is important because of significant implication in prenatal testing and regular screening of affected individuals and at risk family members. Case description: We report a 21 year old male sent to hospital by primary care physician because routine labs showed Blood urea nitrogen=70, and Creatinine=10. Patient had history of worsening fatigue and occasional nausea for 2yrs. Past medical history included recent diagnosis of depression; conductive hearing loss in left ear since early childhood secondary to ossicular fixation. There was no history of recent nephrotoxic substance use. No history ofillicit drug abuse or any recent infections. His father had congenital unilateral renal agenesis; hearing loss, preauricular pit, and unilateral neck mass. Two sisters were stillborn, both had renal agenesis. Significant findings on physical exam including preauricular pit, thick helix of ear and hypertension. Lab reported 7gm/day proteinuria, anemia of chronic disease in addition to confirming outpatient labs. Ultrasound showed atrophic kidneys. Clinical diagnosis of BOR syndrome was made. Patient was started on peritoneal dialysis. Renal biopsy showed FSGS. Genetic analysis was positive for EYA1 gene mutation. Discussion: BOR syndrome is an autosomal dominant disorder characterized by triad of Brachial arch, Otological, and Renal anomalies. Renal malformations may range from mild unilateral hypoplasia to bilateral renal agenesis. Brachio-oto Syndrome (BOS) is a variant without renal involvement. Penetrance is 100%, though expressivity is highly variable among members of same family. True prevalence is unknown as data from studies varies widely. Diagnosis is made based on clinical criteria and requires high clinical suspicion. Accurate diagnosis is important as it has potential implications in management and prenatal genetic testing and screening of family members. Prenatal Diagnosis with genetic analysis is available for families with known EYA1 mutation.
258 PRIMARY RENAL CELL CARCINOMA IN A TRANSPLANTED ALLOGRAFT KIDNEY Siriki R, Syed S, Teran FJ. Tulane University School of Medicine, New Orleans, LA. Recipients of transplanted kidneys are susceptible to malignancy due to their immunocompromised state. Primary cancers involving the kidneys occur mostly in the native. We report a rare case of primary renal cell carcinoma (RCC) in a transplanted kidney 22 years after transplantation. 51 y/o African American male, with a living related kidney in 1992, presented with a history of weight loss and fatigue for a few months. His immunosuppressant therapy included prednisone 5mg and tacrolimus 1mg twice daily. Examination was unremarkable with no tenderness over the transplanted kidney. Pertinent labs included creatinine of 3.51 mg/dL (baseline of 1.7 mg/dL) and sub-therapeutic levels of tacrolimus. Workup for his graft failure included an ultrasound which showed a 6 cm solid, non-calcified mass through the mid kidney and a 1.7 cm mass in the lower pole. Further evaluation with an MRI showed changes within the 2 masses concerning for RCC. A biopsy showed histological and cytological pattern of tumor cells favoring papillary RCC, type 2. The patient will undergo a radical allograft nephrectomy. Recipients of kidney allografts are two to three times more likely to develop a malignancy compared to the general population. They are at an increased risk of developing a carcinoma in the native kidneys which increases up to 100 fold if they were on dialysis prior to transplantation. RCC accounts for 2% of all cancers in the general population. The majority of primary RCC in transplant recipients develop in the native kidneys, but primary RCC in the transplanted allograft is rare with an incidence of only 0.19% to 0.5%; occurring at a mean of 12.6 years after transplantation. The transplant population has a higher risk of post-transplant malignancy which can lead to graft loss and death. It is highly recommended that transplant recipients have close monitoring for malignancies. Early detection in these patients is essential with the goal of early treatment to prevent graft loss and death.
Am J Kidney Dis. 2015;65(4):A1-A93
259 PYRIDORINTM TREATMENT IN MOUSE MODELS OF AKI: Nataliya Skrypnyk1, J. Wesley Fox2, Carrie Taylor2, Ray Harris1, Billy Hudson1, Paul Voziyan1, Mark de Caestecker1 1 Vanderbilt University Medical Center (VUMC), Nashville, TN, USA; 2 NephroGenex, Inc., Raleigh, NC, USA Pyridorin (PYR), an inhibitor of pathogenic oxidative chemistries, has a favorable safety profile in clinical trials and has been shown to scavenge reactive oxygen species (ROS) and reactive carbonyl species (RCS) in vitro and in vivo animal studies. ROS and RCS are highly up regulated during kidney injury. In this study we propose that PYR treatment will reduce injury and prevent long-term fibrosis after ischemia-reperfusion acute kidney injury (IR-AKI). Mouse IR-AKI models: moderate ischemia-reperfusion AKI (unilateral ischemia time 26 min and simultaneous contralateral nephrectomy) and severe ischemia–reperfusion AKI (unilateral ischemia time 31 min and delayed contralateral nephrectomy on day 8). PYR was provided to mice in drinking water at 500 mg/kg BW/day starting 72 h prior to injury and continued until sacrificed, except for mice with moderate IR-AKI where PYR was administered 1 day after injury induction. PYR was given to mice via gavage feeding twice a day at 200 mg/kg for 72 h after AKI induction to ensure proper postoperative dosing. Renal function was assessed by serum creatinine, renal tubular injury with urinary Kim1 (days 1 and 3), and post-injury fibrosis with qRT-PCR for renal fibrosis markers (aSMA, Col1a1, Col3a1) on day 28 after injury. PYR ameliorated the increase in serum creatinine on days 9 and 14 after injury, in urinary Kim1 expression on days 1 and 3, and in postinjury fibrosis markers on day 28 after severe I/R-AKI. PYR had no effect on serum creatinine (day 3, 5 and 7) in moderate I/R-AKI when treatment was delayed 24 hours after injury. PYR ameliorates injury and reduces post-injury fibrosis in severe IRAKI when administered 3 days before injury, but has no effect on functional recovery or renal injury when administered 24 hours after moderate injury. These data suggest that pre-treatment with PYR can ameliorate injury and prevent progression to chronic kidney disease in patients with AKI. Supported by research grant from NephroGenex, Inc. JWF, BH and VUMC are stockholders of NephroGenex, Inc.
260 PERFORATION OF THE GREAT VESSELS DURING TUNNELED INTERNAL JUGULAR DIALYSIS CATHETER PLACEMENT Lindsay M Smith, Dan Bucaloiu, Jamie Green, Susan Trocciola, Geisinger Medical Center, Danville, PA, USA Complications related to tunneled dialysis catheter placement are relatively rare in the era of ultrasound and fluoroscopy guidance. We present a case of transection of the great vessels during fluoroscopic tunneled dialysis catheter placement under general anesthesia. A 69-year-old woman with end stage renal disease on peritoneal dialysis underwent an elective umbilical and inguinal hernia repair. At the end of surgery, a right internal jugular tunneled dialysis catheter (28 cm PermCath) was inserted under fluoroscopic guidance while the patient was under general anesthesia. After completion of a dialysis session, the patient experienced transient confusion and dysarthria prompting referral to our hospital. Brain imaging revealed no acute pathology. A chest x-ray revealed the catheter overlying the heart shadow with the tip overlying the left atrium. Additional imaging confirmed the presence of the catheter tip within the left atrium. The patient underwent urgent sternotomy, which exposed the catheter completely transecting the main pulmonary artery, perforating and entering the roof of the left atrium. The catheter was surgically removed on cardiopulmonary bypass and the holes were repaired. The patient tolerated the procedure well and made a complete recovery. We would like to use this illustrative case to raise awareness of this complication of a relatively common procedure in Nephrology. Careful review of post procedure chest x-ray and fluoroscopy imaging prior to initiating hemodialysis are crucial in identifying dialysis catheter malposition and preventing major adverse outcomes.
A79