Transient Renal Insufficiency Research Articles

Heart Transplantation in Adult Congenital Patients Performed at a Pediatric Center: An Institutional Experience

<h3>Purpose</h3> Advancements in pediatric cardiovascular surgery have improved outcomes of children resulting in more adults with palliated congenital heart disease requiring a heart transplant. There is no standardized practice among adult congenital transplant centers for the location of pre- and posttransplant management of these patients. <h3>Methods</h3> We present the outcomes of 17 adult congenital transplantations performed at our pediatric institution from 2007-2021 as a joint evaluation and management effort between adult and pediatric programs, with pre, peri- and post-operative management occurring at the pediatric center. <h3>Results</h3> Patients aged 18-47 years at the time of heart transplantation. The most common indication for transplant was single ventricle with failing Fontan physiology, followed by DTGV with failing Mustard physiology. Two patients were retransplants for coronary artery vasculopathy. Patients listed were deemed candidates by both the pediatric and adult transplant teams. All patients were admitted prior to transplant at the pediatric hospital for initiation of inotropic therapy. The average pretransplant hospital stay was 76.9 days. Three patients required ECMO following transplant, for an average of 2 days. All patients received thymoglobulin immunosuppression and IV steroids. Maintenance immunosuppression was achieved with tacrolimus, mycophenolate mofetil, and oral steroid wean. Posttransplant length of stay averaged 15.8 days. All patients required temporary pacing. None required permanent pacing post-transplant. Most developed transient liver insufficiency and transient renal insufficiency. To date, 15/17 patients are living. One patient died of rejection at 22 months posttransplant and the other patient had massive post-transplant hemorrhage and developed mediastinitis, liver and renal failure and died 31 days posttransplant. The one year survival in this cohort was 94%. The three and five year survival is 88%. <h3>Conclusion</h3> We recommend a combined approach by adult and pediatric heart transplant teams when caring for adult congenital patients undergoing heart transplantation. Emphasis should be placed on the optimal location for congenital surgical management. Outcomes at or above the national adult heart transplant average can be achieved with such management in a high risk population.

Nephrogenic Systemic Fibrosis in the Setting of Transient Renal Insufficiency

Nephrogenic systemic fibrosis (NSF) is a rare disease that can present in patients with kidney injury who have been exposed to gadolinium-based contrast agents (GBCAs). These patients can easily be misdiagnosed as having another disease because they do not fit the usual presentation of being on hemodialysis. We report a case of NSF occurring in a patient with resolved transient renal insufficiency and no history of chronic renal disease.

The Dilemma after Sealing an Endovascular Aortic Aneurysm - Three Ways Out.

Endovascular aneurysm sealing (EVAS) was commercially introduced in 2013. The initial results of EVAS were positive, leading to its widespread use. The mid- and long-term reports showed greater than expected rates of migration, which led to a recall of the device. In the present article, we describe our experience in managing type Ia endoleaks and migration occurring with the Nellix system in three different ways: open repair with Nellix explantation, Nellix-in-Nellix application (NINA technique), and the use of the multibranched Colt device originally dedicated to the treatment of thoracoabdominal aneurysms. From February 2014 to June 2021, we performed 20 procedures for failed EVAS or ChEVAS (migration, type Ia endoleak, secondary aneurysm rupture). All patients treated for EVAS failure were male, aged 65 - 79. Seven Nellix explantations were performed. Three patients were admitted to our hospital with ruptured aneurysms that occurred 3 weeks to 4 years (mean 124 weeks) after EVAS, and another four with type Ia endoleak. In all but one case, removal of the Nellix system was easy. In two patients, tube grafts were implanted and in the remaining five cases, bifurcated grafts were implanted. In four patients, graft legs were anastomosed with the internal iliac arteries. One patient with secondary aneurysm rupture died from multiorgan failure on the 4th postoperative day. In two cases, transient renal failure was noticed in the perioperative period. All patients were admitted to the intensive care unit for 1 to 4 days (mean 2 days). The mean hospital stay was 9 days. All patients stayed in follow-up (3 - 56 months), but no other complications occurred. Eight patients were treated with the NINA technique: five for distal migration of the Nellix and three for failed ChEVAS. Four patients had a NINA procedure performed with three chimneys, three with two and one with one chimney. In one case, two iliac limbs were implanted to avoid kinking of the external iliac arteries. The median hospitalization time after the procedure was 9 days (range 3 - 12). Four patients developed transient acute renal insufficiency in the perioperative period. The follow-up ranged between 4 and 72 months. In one patient, deterioration of preexisting chronic renal insufficiency developed 5 months after the procedure, but dialysis was not required. One patient died from exacerbation of heart failure 7 months after the NINA procedure. The Colt device was implanted in five patients for the treatment of distal migration with type Ia endoleaks. None of the patients developed any signs of spinal cord ischemia. All patients were admitted to the intensive care unit for 1 or 2 days. In two cases, transient acute renal failure was noticed in the perioperative period. The mean hospital stay was 9 days. All patients remained in follow-up (6 - 22 months). In one case, the occlusion of the celiac trunk branch was found in contrast computed tomography 1 month after implantation of the Colt device, but without any symptoms. No other complications occurred. Normal strategies for the management of complications for late failure of EVAR, including stent-graft extensions, are not suitable after EVAS; therefore, alternatives are necessary. Conversion to open repair carries an extensive burden on the patient, so it is not recommended for patients with high surgical risk. The use of a Nellix-in-Nellix application to treat late failure of EVAS is not within the instructions for use but could be an effective strategy for a type Ia endoleak with or without migration. The use of this technique has been extremely limited since the Nellix system was recalled from the market. The use of the Colt multibranched device may be an alternative option, but due to the small number of patients, this method needs further evaluation.

Wolf-Hirschhorn Syndrome with Hyperparathyroidism: A Case Report and a Narrative Review of the Literature.

Wolf-Hirschhorn syndrome (WHS) is a contiguous gene deletion condition. The WHS core phenotype includes developmental delays, intellectual disabilities, seizures, and distinctive facial features. Various other comorbidities have also been reported, such as hearing loss, heart defects, as well as eye problems and kidney problems. In this report, we present a case of WHS accompanied by hyperparathyroidism and hypercalcemia, which has not been previously reported. A girl was born at 37 weeks of gestation by vaginal delivery. She was small for the gestational age (2,045 g) and admitted to neonatal intensive care unit. She had typical WHS facial features and was found to have bilateral small kidneys associated with transient metabolic acidosis and renal insufficiency. She had right-sided sensorineural hearing loss, a small atrial septal defect, and colpocephaly and hypoplasia of corpus callosum. She had a single seizure which was well controlled with an oral antiepileptic medication. Cytogenetic studies demonstrated a large terminal chromosome 4p deletion (21.4 Mb) and 4p duplication (2.1 Mb) adjacent to the deletion. A unique finding in this patient is her consistently elevated levels of parathyroid hormone and serum calcium, suggesting hyperparathyroidism. We present this rare case along with a review of the literature and hope to draw an attention to a potential relationship between WHS and hyperparathyroidism.

Ibuprofen for the treatment of patent ductus arteriosus in preterm or low birth weight (or both) infants.

Background Indomethacin is used as standard therapy to close a patent ductus arteriosus (PDA) but is associated with reduced blood flow to several organs. Ibuprofen, another cyclo-oxygenase inhibitor, may be as effective as indomethacin with fewer adverse effects. Objectives To determine the effectiveness and safety of ibuprofen compared with indomethacin, other cyclo-oxygenase inhibitor, placebo or no intervention for closing a patent ductus arteriosus in preterm, low birth weight, or preterm and low birth weight infants. Search methods We searched The Cochrane Library, MEDLINE, EMBASE, Clincialtrials.gov, Controlled-trials.com, and www.abstracts2view.com/pas in May 2014. Selection criteria Randomised or quasi-randomised controlled trials of ibuprofen for the treatment of a PDA in newborn infants. Data collection and analysis Data collection and analysis conformed to the methods of the Cochrane Neonatal Review Group. Main results We included 33 studies enrolling 2190 infants. Two studies compared intravenous (iv) ibuprofen versus placebo (270 infants). In one study (134 infants) ibuprofen reduced the incidence of failure to close a PDA (risk ratio (RR) 0.71, 95% confidence interval (CI) 0.51 to 0.99; risk difference (RD) -0.18, 95% CI -0.35 to -0.01; number needed to treat for an additional beneficial outcome (NNTB) 6, 95% CI 3 to 100). In one study (136 infants), ibuprofen reduced the composite outcome of infant mortality, infants who dropped out, or infants who required rescue treatment (RR 0.58, 95% CI 0.38 to 0.89; RD -0.22, 95% CI -0.38 to -0.06; NNTB 5, 95% CI 3 to 17). One study (64 infants) compared oral ibuprofen with placebo and noted a significant reduction in failure to close a PDA (RR 0.26, 95% CI 0.11 to 0.62; RD -0.44, 95% CI -0.65 to -0.23; NNTB 2, 95% CI 2 to 4). Twenty-one studies (1102 infants) reported failure rates for PDA closure with ibuprofen (oral or iv) compared with indomethacin (oral or iv). There was no significant difference between the groups (typical RR 1.00, 95% CI 0.84 to 1.20; I2 = 0%; typical RD 0.00, 95% CI -0.05 to 0.05; I2 = 0%). The risk of developing necrotising enterocolitis (NEC) was reduced for ibuprofen (16 studies, 948 infants; typical RR 0.64, 95% CI 0.45 to 0.93; typical RD -0.05, 95% CI -0.08 to -0.01; NNTB 20, 95% CI 13 to 100; I2 = 0% for both RR and RD). The duration of ventilatory support was reduced with ibuprofen (oral or iv) compared with iv or oral indomethacin (six studies, 471 infants; mean difference (MD) -2.4 days, 95% CI -3.7 to -1.0; I2 = 19%). Eight studies (272 infants) reported on failure rates for PDA closure in a subgroup of the above studies comparing oral ibuprofen with indomethacin (oral or iv). There was no significant difference between the groups (typical RR 0.96, 95% CI 0.73 to 1.27; typical RD -0.01, 95% CI -0.12 to 0.09). The risk of NEC was reduced with oral ibuprofen compared with indomethacin (oral or iv) (seven studies, 249 infants; typical RR 0.41, 95% CI 0.23 to 0.73; typical RD -0.13, 95% CI -0.22 to -0.05; NNTB 8, 95% CI 5 to 20; I2 = 0% for both RR and RD). There was a decreased risk of failure to close a PDA with oral ibuprofen compared with iv ibuprofen (four studies, 304 infants; typical RR 0.41, 95% CI 0.27 to 0.64; typical RD -0.21, 95% CI -0.31 to -0.12; NNTB 5, 95% CI 3 to 8). Transient renal insufficiency was less common in infants who received ibuprofen compared with indomethacin. High dose versus standard dose of iv ibuprofen, early versus expectant administration of iv ibuprofen, echocardiographically guided iv ibuprofen treatment vs. standard iv ibuprofen treatment and continuous infusion of ibuprofen vs. intermittent boluses of ibuprofen and long-term follow-up were studied in too few trials to draw any conclusions. Authors' conclusions Ibuprofen is as effective as indomethacin in closing a PDA and currently appears to be the drug of choice. Ibuprofen reduces the risk of NEC and transient renal insufficiency. Oro-gastric administration of ibuprofen appears as effective as iv administration. To make further recommendations, studies are needed to assess the effectiveness of high-dose versus standard-dose ibuprofen, early versus expectant administration of ibuprofen, echocardiographically guided versus standard iv ibuprofen, and continuous infusion versus intermittent boluses of ibuprofen. Studies are lacking evaluating the effect of ibuprofen on longer-term outcomes in infants with PDA.

Complications Following Nephron-Sparing Surgery for Wilms Tumor

PurposeBecause of the increasing use of nephron-sparing surgery (NSS) in bilateral Wilms tumor, we sought to review the early postoperative complications associated with NSS. MethodsA retrospective review of patients who underwent NSS at our institution from 2000 to 2017 was performed. For comparison, a cohort of patients who underwent radical nephrectomy (RN) was also reviewed. Early (30-day) postoperative complications and oncologic outcomes were assessed. ResultsFifty-five patients underwent either bilateral (46) NSS or unilateral (9) NSS owing to prior resection or congenital solitary kidney. Fifty-four patients who underwent unilateral RN were also evaluated. Twenty NSS patients (36.4%) experienced 21 postoperative complications, including prolonged urine leak (9), infection (8), transient renal insufficiency (1), and intussusception (3). Seven RN patients (13.0%) experienced surgical complications, including infection (4) and intussusception (3). Average intraoperative blood loss was significantly greater in NSS as compared to RN (483.51 ± 337.92 mL and 278.15 mL ± 390.25, respectively, p < 0.001), as was the incidence of positive tumor resection margins (20 [36.4%] and 12 [22.2%], respectively, (p = 0.037). ConclusionsIn our experience, prolonged urine leak, intraoperative blood loss, and positive margins were more frequent in patients undergoing NSS as compared to RN. However, the complications were successfully managed, suggesting that an aggressive approach to NSS in patients with bilateral Wilms tumor is safe and appropriate. Level of evidenceLevel III Type of studyTreatment study.

Association between chronic kidney disease and synergistic, potentially nephrotoxic medication use in elderly hospitalized patients: A single-center cross-sectional study .

In elderly patients, age-related, disease-related, and drug-related factors are associated with chronic kidney disease (CKD). Little is known about which factors are the best predictors for CKD in elderly patients. The study was based on 784 patients aged 75 years or older for whom the clinical and serum creatinine on admission to our hospital were available. Impaired renal function, including CKD and transient renal insufficiency, was defined as a non-indexed glomerular filtration rate (GFR) below 60mL/min. A logistic regression model was developed for predictors of CKD and was internally validated using bootstrapping. Approximately 61% of the patients, who had CKD (46%) and transient renal insufficiency (15%), had a non-indexed GFR <60mL/min. Synergistic use of 3 drugs potentially impairing renal function, diuretics, ACE-I/ARB, and NSAIDs (odds ratio (OR), 4.66; 95% confidence interval (CI), 1.48-17.7, p=0.012) was a significantly associated factor for CKD in a multivariate logistic regression analysis. Age (OR 1.56, 95% CI 1.04-2.33, p=0.03), female gender (OR 1.58, 95% CI 1.04-2.39, p=0.03), any prescription ACE-I/ARB either alone or in combinations with diuretics or NSAIDs (OR 2.74, 95% CI 1.83-4.13, p=0.0001), and proteinuria (OR 1.98, 95% CI 1.27-3.10, p=0.003), were included as the best model for CKD. The area under the curve (AUC) of the best model and the bootstrapping validation were 0.68 and 0.71, respectively. Given the widespread use of ACE-I/ARB for elderly patients, our findings suggest that caution is needed when they are prescribed because of the possibility of the patient developing CKD.

Ibuprofen for the treatment of patent ductus arteriosus in preterm or low birth weight (or both) infants.

Ibuprofen for the treatment of patent ductus arteriosus in preterm or low birth weight (or both) infants.

21 - Gain-of-Function SAMD9L Mutations Cause a Syndrome of Cytopenia, Immunodeficiency, Myelodysplastic Syndrome and Neurological Symptoms

Because of the increasing use of nephron-sparing surgery (NSS) in bilateral Wilms tumor, we sought to review the early postoperative complications associated with NSS.A retrospective review of patients who underwent NSS at our institution from 2000 to 2017 was performed. For comparison, a cohort of patients who underwent radical nephrectomy (RN) was also reviewed. Early (30-day) postoperative complications and oncologic outcomes were assessed.Fifty-five patients underwent either bilateral (46) NSS or unilateral (9) NSS owing to prior resection or congenital solitary kidney. Fifty-four patients who underwent unilateral RN were also evaluated. Twenty NSS patients (36.4%) experienced 21 postoperative complications, including prolonged urine leak (9), infection (8), transient renal insufficiency (1), and intussusception (3). Seven RN patients (13.0%) experienced surgical complications, including infection (4) and intussusception (3). Average intraoperative blood loss was significantly greater in NSS as compared to RN (483.51 ± 337.92 mL and 278.15 mL ± 390.25, respectively, p < 0.001), as was the incidence of positive tumor resection margins (20 [36.4%] and 12 [22.2%], respectively, (p = 0.037).In our experience, prolonged urine leak, intraoperative blood loss, and positive margins were more frequent in patients undergoing NSS as compared to RN. However, the complications were successfully managed, suggesting that an aggressive approach to NSS in patients with bilateral Wilms tumor is safe and appropriate.Level IIITreatment study.

Effect of Fibrinogen Concentrate on Intraoperative Blood Loss Among Patients With Intraoperative Bleeding During High-Risk Cardiac Surgery

Fibrinogen concentrate might partly restore coagulation defects and reduce intraoperative bleeding. To determine whether fibrinogen concentrate infusion dosed to achieve a plasma fibrinogen level of 2.5 g/L in high-risk cardiac surgery patients with intraoperative bleeding reduces intraoperative blood loss. A randomized, placebo-controlled, double-blind clinical trial conducted in Isala Zwolle, the Netherlands (February 2011-January 2015), involving patients undergoing elective, high-risk cardiac surgery (ie, combined coronary artery bypass graft [CABG] surgery and valve repair or replacement surgery, the replacement of multiple valves, aortic root reconstruction, or reconstruction of the ascending aorta or aortic arch) with intraoperative bleeding (blood volume between 60 and 250 mL suctioned from the thoracic cavity in a period of 5 minutes) were randomized to receive either fibrinogen concentrate or placebo. Intravenous, single-dose administration of fibrinogen concentrate (n = 60) or placebo (n = 60), targeted to achieve a postinfusion plasma fibrinogen level of 2.5 g/L. The primary outcome was blood loss in milliliters between intervention (ie, after removal of cardiopulmonary bypass) and closure of chest. Safety variables (within 30 days) included: in-hospital mortality, myocardial infarction, cerebrovascular accident or transient ischemic attack, renal insufficiency or failure, venous thromboembolism, pulmonary embolism, and operative complications. Among 120 patients (mean age; 71 [SD, 10] years, 37 women [31%]) included in the study, combined CABG and valve repair or replacement surgery comprised 72% of procedures and had a mean (SD) cardiopulmonary bypass time of 200 minutes (83) minutes. For the primary outcome, median blood loss in the fibrinogen group was 50 mL (interquartile range [IQR], 29-100 mL) compared with 70 mL (IQR, 33-145 mL) in the control group (P = .19), the absolute difference 20 mL (95% CI, -13 to 35 mL). There were 6 cases of stroke or transient ischemic attack (4 in the fibrinogen group); 4 myocardial infarctions (3 in the fibrinogen group); 2 deaths (both in the fibrinogen group); 5 cases with renal insufficiency or failure (3 in the fibrinogen group); and 9 cases with reoperative thoracotomy (4 in the fibrinogen group). Among patients with intraoperative bleeding during high-risk cardiac surgery, administration of fibrinogen concentrate, compared with placebo, resulted in no significant difference in the amount of intraoperative blood loss. clinicaltrials.gov Identifier: NCT01124981 and EudraCT No: 2009-018086-12.

Long-term efficacy and side effects of low-dose tacrolimus for the treatment of Myasthenia Gravis.

The study evaluated the efficacy of low-dose tacrolimus for treating Myasthenia Gravis (MG). Data were collected from 97 patients treated with low-dose tacrolimus from February 2011 to April 2015. Metabolic analysis was performed to determine more accurate tacrolimus dosing and patients were followed-up within clinic every 6months for up to 4years. The myasthenia gravis-specific activities of daily living scale was used to assess MG symptoms and their effects on patients' daily activities. All side effects and adverse reactions were thoroughly documented. At the end of follow-up, 6 patients were in complete stable remission, 17 patients were in pharmacological remission, 26 patients were in minimal manifestation status, 32 patients were improved, 2 patients were unchanged, 11 patients had worsening symptoms, and 3 patients died. Side effects were reported and/or observed in 24 patients, of which 7 patients experienced elevated blood glucose, 2 patients developed neoplasms, 3 patients developed gastrointestinal symptoms, 3 showed mild increases in aminotransferases, 3 patients suffered from bone marrow suppression, 2 patients suffered from skin rashes and erythema, and 1 patient required discontinuation of therapy. Transient renal insufficiency was also observed in 1 patient and 3 other patients had minor miscellaneous side effects. This study adds some knowledge on the efficacy and side effects of low-dose tacrolimus in the treatment of MG. Tacrolimus immunotherapy is a valid option for the management of MG, and can be gradually reduced in dose once symptoms are improved until complete withdrawal is achieved.

PAROKSIZMALNA NOČNA HEMOGLOBINURIJA - PRIPOROČILA ZA ODKRIVANJE BOLEZNI TER PREGLED POPULACIJE BOLNIKOV V SLOVENIJI

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired disease associated with hemolytic anemia, bone marrow failure, thrombosis, and, frequently, poor quality of life. It is caused by defects in the membrane of blood cells, where there is a lack of protein on the cell surface, which inhibits complement activation. We wanted to know the recognition of the disease in Slovenia and the incidence. We prepared the recommendations for discovering of the disease. Patients and methods: We collected data of 68 patients with prospective analysis of one – year period from 1.10.2013 to 30.9.2014 whose blood was sent to the laboratory of immunology and cytology because of suspected presence of PNH clone. The analysis of peripheral blood was performed with multiparametric high specific flow cytometry in a specialized laboratory KO of Haematology University Medical Centre in Ljubljana. Results: PNH clone was positive in 13/68 (19%) patients, 55/68 (81%) patients had a negative PNH clone, most positive samples were sent from the University Medical Centre Ljubljana (7/13). 4/13 positive patients were newly discovered. In average the incidence through 10-years was 1,3 / 1,000,000 population/year. The most common cause of PNH patient referral to a specialist hematologist was unexplained cytopenia - 12/13 (92.3%), the most common symptoms were fatigue and dyspnea (100%), in 2/13 patients was present dark urine with hemoglobinuria, 2/13 patients had transient renal insufficiency. 11/13 patients with positive PNH clone had associated a bone marrow failure (aplastic anemia or myelodysplastic syndrome). The size of PNH clone varied from patient to patient. Conclusions: Early identification of PNH is a key to effective treatment and survival of patients. We recommend determining PNH clone at Coombs negative hemolytic anemia, hemoglobinuria, an unexplained cytopenia, aplastic anemia, myelodysplastic syndrome with laboratory evidence of haemolysis and unexplained thrombosis.

The IMPACT-CABG trial: A multicenter, randomized clinical trial of CD133+ stem cell therapy during coronary artery bypass grafting for ischemic cardiomyopathy

ObjectivesThe IMPACT-CABG trial is the first North American multicenter phase II randomized study of intramyocardial delivery of autologous CD133+ stem cells in patients with chronic ischemic cardiomyopathy undergoing coronary artery bypass grafting. The primary objective was to demonstrate safety, including freedom from major adverse cardiac events. The secondary objective was to evaluate feasibility of same-day autologous cell preparation. Although the trial was not powered to evaluate LV function, exploratory data were collected. MethodsAfter 7 open-label patients who received cells, patients randomly received stem cells or placebo (N = 40 total, 20 per center). After completion of coronary anastomoses, up to 10 million CD133+, CD34+, CD45+ triple-positive cells or placebo were injected into the infarct and border zones. Patients were followed up clinically and underwent magnetic resonance imaging preoperatively and after 6 months. ResultsThere were no procedural complications from bone marrow isolation and cell injection, no in-hospital mortality, and no protocol-related complications. Four patients had transient renal insufficiency, with 1 death during 6-month follow-up. Magnetic resonance imaging revealed that left ventricular volumes and ejection fractions improved in all patients (no difference between groups). ConclusionsThe trial successfully met both primary and secondary objectives, demonstrating that same-day isolation and autologous CD133+ cell delivery with coronary artery bypass grafting is safe and feasible. The positive findings support a larger randomized, multicenter trial, with higher numbers of transplanted cells to demonstrate beneficial effects. The upcoming IMPACT-CABG II trial will evaluate higher cell doses and pharmacologic enhancement to determine whether these cells improve perfusion and myocardial function.

Transcatheter Arterial Embolization of Angiographically Visible and Occult Renal Capsular Artery Hemorrhage in 28 Patients

PurposeTo evaluate the effectiveness and safety of transcatheter arterial embolization to control bleeding from the renal capsular artery (RCA). Materials and MethodsEmbolization was performed in 28 patients (14 men; mean age, 49.7 y). Presence and type of previous invasive procedures, initial presentation, and coagulation profile were reviewed. Any preceding abdominal computed tomography (CT) findings were analyzed. Angiographic findings were categorized as active bleeding, suspicious for bleeding, or no bleeding. Technical and clinical success and clinical outcomes were evaluated. Changes in hemoglobin level and transfusion volume of packed red blood cells (pBRCs) before and after embolization were evaluated with the paired t test and Wilcoxon signed-rank test, respectively. ResultsTechnical and clinical success rates of therapeutic embolization for active bleeding (n = 11) were 90.9% and 80%, respectively. One case of technical failure (5.9%) and 3 cases of postembolization bleeding (18.7%) were noted in the prophylactic embolization group in patients with suspicion of bleeding (n = 13) or no bleeding (n = 4). Transient renal insufficiency occurred in 4 patients (14.3%). The average hemoglobin level and volume of transfused pBRCs changed from 8.1 g/dL to 9.9 g/dL and from 871 mL to 543 mL, respectively (P < .05). Extravasation of contrast media or acute hematoma in the right subhepatic or perirenal space on CT was noted in 21 patients (78%). ConclusionsEmbolization can provide an effective and safe method to control RCA bleeding. Perirenal invasive procedures and signs of active or recent right subhepatic or perirenal hemorrhage should raise the suspicion of an RCA source.

Ibuprofen and indomethacin for the closure of the patent ductus arteriosus

The ductus arteriosus connects the pulmonary artery with the aorta and allows right ventricular blood to bypass the unexpanded lungs. In mature infants, the ductus arteriosus closes after birth. Patent ductus arteriosus occurs in 70% of preterm infants with a birth weight < 1,000 grams. Failure of the ductus arteriosus to close has been associated with intraventricular hemorrhage, necrotizing enterocolitis, bronchopulmonary dysplasia, periventricular leukomalacia, renal failure, and persistent pulmonary hypertension. The drugs used to treat the patent ductus arteriosus are ibuprofen and indomethacin which are potent non-selective inhibitors of cyclo-oxygenase (COX) and therefore inhibit prostaglandin E2 synthesis. Prostaglandin E2 relaxes smooth muscle and tends to inhibit the closure of the patent ductus arteriosus. Intravenous ibuprofen and indomethacin inhibit prostaglandin E2 synthesis and thereby close the patent ductus arteriosus with similar efficacy. Indomethacin reduces the blood flow velocity in kidneys, intestine and brain. Ibuprofen has less effect on blood flow velocity in these organs. There is a significant increase in serum creatinine after indomethacin administration but not after ibuprofen and infants treated with ibuprofen have higher creatinine clearance. Oliguria (urine output < 1 ml/kg/h) occurs more frequently with indomethacin than with ibuprofen. Indomethacin requires furosemide for urine output more often than ibuprofen. Ibuprofen reduces the risk of necrotizing enterocolitis and transient renal insufficiency and it is the drug of choice for closing the patent ductus arteriosus. Ibuprofen and indomethacin may be administered orally. In conclusion, intravenous ibuprofen and indomethacin close the patent ductus arteriosus at the same rate, but indomethacin is more toxic than ibuprofen.

193 Isolated lung transplantation (LT) for cystic fibrosis patients with portal hypertension (PHT)

Objectives PHT is the main manifestation of liver disease in adults CF patients. The risk of hepatic decompensation after isolated LT is unknown. The model for end-stage liver disease (MELD) score is not relevant for the indication to combined lungs-liver transplantation. We presented the postoperative outcome of isolated lung transplantation in CF patients with asymptomatic PHT. Methods From 2008 to 2014, 14 isolated LT (over 299) were proceeded over CF patients with PHT. Results All Patients had asymptomatic liver disease before LT but all had PHT on the CT scan: splenomegaly (14–19 cm) in 10 patients, portosystemic collaterals in 8, all had portal vein dilatation >14 (diameter 17±2.3 mm). The liver stiffness measurement (LMS) was elevated 18±8 kPa when in our adult CF center median LSM was 5.9 kPa in 241 CF patients. Thirteen patients had normal liver and renal function. Gastroesophageal varices were absents in 10 screened patients. After LT, 4 patients required ECMO, 6 developed easy to treat ascites associated with transient renal insufficiency (4) or intestinal complications (2). One developed transient ascitis 7 months after LT associated with sepsis and renal dysfunction. Hepatocellular function remain normal in all patients. Every patient is still alive (mean time 42±22 months). Conclusion Carefully selected CF patients with end-stage lung disease and portal hypertension can tolerate isolated lung transplantation. The non-invasive evaluation of the severity of PHT has to be evaluated. Prospective multicentric study is need to clearly define the indications of combined lung–liver transplantation.

Ibuprofen for the treatment of patent ductus arteriosus in preterm or low birth weight (or both) infants.

Indomethacin is used as standard therapy to close a patent ductus arteriosus (PDA) but is associated with reduced blood flow to several organs. Ibuprofen, another cyclo-oxygenase inhibitor, may be as effective as indomethacin with fewer adverse effects. To determine the effectiveness and safety of ibuprofen compared with indomethacin, other cyclo-oxygenase inhibitor, placebo or no intervention for closing a patent ductus arteriosus in preterm, low birth weight, or preterm and low birth weight infants. We searched The Cochrane Library, MEDLINE, EMBASE, Clincialtrials.gov, Controlled-trials.com, and www.abstracts2view.com/pas in May 2014. Randomised or quasi-randomised controlled trials of ibuprofen for the treatment of a PDA in newborn infants. Data collection and analysis conformed to the methods of the Cochrane Neonatal Review Group. We included 33 studies enrolling 2190 infants.Two studies compared intravenous (iv) ibuprofen versus placebo (270 infants). In one study (134 infants) ibuprofen reduced the incidence of failure to close a PDA (risk ratio (RR) 0.71, 95% confidence interval (CI) 0.51 to 0.99; risk difference (RD) -0.18, 95% CI -0.35 to -0.01; number needed to treat for an additional beneficial outcome (NNTB) 6, 95% CI 3 to 100). In one study (136 infants), ibuprofen reduced the composite outcome of infant mortality, infants who dropped out, or infants who required rescue treatment (RR 0.58, 95% CI 0.38 to 0.89; RD -0.22, 95% CI -0.38 to -0.06; NNTB 5, 95% CI 3 to 17). One study (64 infants) compared oral ibuprofen with placebo and noted a significant reduction in failure to close a PDA (RR 0.26, 95% CI 0.11 to 0.62; RD -0.44, 95% CI -0.65 to -0.23; NNTB 2, 95% CI 2 to 4).Twenty-one studies (1102 infants) reported failure rates for PDA closure with ibuprofen (oral or iv) compared with indomethacin (oral or iv). There was no significant difference between the groups (typical RR 1.00, 95% CI 0.84 to 1.20; I(2) = 0%; typical RD 0.00, 95% CI -0.05 to 0.05; I(2) = 0%). The risk of developing necrotising enterocolitis (NEC) was reduced for ibuprofen (16 studies, 948 infants; typical RR 0.64, 95% CI 0.45 to 0.93; typical RD -0.05, 95% CI -0.08 to -0.01; NNTB 20, 95% CI 13 to 100; I(2) = 0% for both RR and RD). The duration of ventilatory support was reduced with ibuprofen (oral or iv) compared with iv or oral indomethacin (six studies, 471 infants; mean difference (MD) -2.4 days, 95% CI -3.7 to -1.0; I(2) = 19%).Eight studies (272 infants) reported on failure rates for PDA closure in a subgroup of the above studies comparing oral ibuprofen with indomethacin (oral or iv). There was no significant difference between the groups (typical RR 0.96, 95% CI 0.73 to 1.27; typical RD -0.01, 95% CI -0.12 to 0.09). The risk of NEC was reduced with oral ibuprofen compared with indomethacin (oral or iv) (seven studies, 249 infants; typical RR 0.41, 95% CI 0.23 to 0.73; typical RD -0.13, 95% CI -0.22 to -0.05; NNTB 8, 95% CI 5 to 20; I(2) = 0% for both RR and RD). There was a decreased risk of failure to close a PDA with oral ibuprofen compared with iv ibuprofen (four studies, 304 infants; typical RR 0.41, 95% CI 0.27 to 0.64; typical RD -0.21, 95% CI -0.31 to -0.12; NNTB 5, 95% CI 3 to 8). Transient renal insufficiency was less common in infants who received ibuprofen compared with indomethacin. High dose versus standard dose of iv ibuprofen, early versus expectant administration of iv ibuprofen, echocardiographically guided iv ibuprofen treatment vs. standard iv ibuprofen treatment and continuous infusion of ibuprofen vs. intermittent boluses of ibuprofen and long-term follow-up were studied in too few trials to draw any conclusions. Ibuprofen is as effective as indomethacin in closing a PDA and currently appears to be the drug of choice. Ibuprofen reduces the risk of NEC and transient renal insufficiency. Oro-gastric administration of ibuprofen appears as effective as iv administration. To make further recommendations, studies are needed to assess the effectiveness of high-dose versus standard-dose ibuprofen, early versus expectant administration of ibuprofen, echocardiographically guided versus standard iv ibuprofen, and continuous infusion versus intermittent boluses of ibuprofen. Studies are lacking evaluating the effect of ibuprofen on longer-term outcomes in infants with PDA.

Results of Open Surgical Repair of Chronic Juxtarenal Aortic Occlusion.

Purpose:The aim of study was to review the results of open surgical repair (OSR) of chronic juxtarenal aortic occlusion (JRAO).Materials and Methods:We retrospectively reviewed the results of OSR performed in 47 patients (male, 92%; mean age, 59.9±9.3 years [range, 44–79]) with chronic JRAO during the past 21 years. In order to reduce intraoperative renal ischemic time (RIT), we excised a portion of the occluded segment of the infrarenal aorta without proximal aortic clamping. We then performed suprarenal aortic clamping with both renal arteries clamped, removed the proximal aortic thrombus cap, confirmed both renal artery orifices, and moved the suprarenal aortic clamp to the infrarenal aorta to allow renal perfusion and standard aortoiliac reconstruction. We investigated early (<30 days) postoperative surgical morbidity (particularly renal function), operative mortality, and longterm patient survival. We conducted risk factor analysis for postoperative renal insufficiency.Results:The mean intraoperative RIT was 10.7±5.5 minutes (range, 3–25), including 6 patients who underwent concomitant pararenal aortic thromboendarterectomy. Postoperatively, five (11%) patients had transient renal insufficiency, one had pneumonia, and one patient had an acute myocardial infarction. However, there was no operative mortality or newly developed dialysis-dependent renal failure. Postoperative follow up was available in 36 (77%) patients for a mean period of 6.3 years (range, 1 month-17 years). Kaplan Meier calculations of patient survival at 5 and 10 years after surgery were 91.2% and 83.6%, respectively.Conclusion:We have experienced short RIT, acceptable early postoperative results and long-term survival after OSR of chronic JRAO.

PD7-12 EXTENDED ANTIMICROBIAL USAGE IN PERCUTANEOUS NEPHROLITHOTOMY: INFECTIOUS AND ANTIBIOTIC RELATED COMPLICATIONS

You have accessJournal of UrologyStone Disease: Therapy I1 Apr 2014PD7-12 EXTENDED ANTIMICROBIAL USAGE IN PERCUTANEOUS NEPHROLITHOTOMY: INFECTIOUS AND ANTIBIOTIC RELATED COMPLICATIONS Boyd Viers, Patrick Cockerill, and Amy Krambeck Boyd ViersBoyd Viers More articles by this author , Patrick CockerillPatrick Cockerill More articles by this author , and Amy KrambeckAmy Krambeck More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2014.02.701AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES One-third of upper urinary tract stones harbor microorganisms. As such, current guidelines recommend perioperative antimicrobial agents up to 24-hours at the time of percutaneous renal surgery. Our goal is to evaluate perioperative infectious and antibiotic associated complications in patients receiving extended antimicrobial therapy at percutaneous nephrolithotomy (PCNL). METHODS Between 2009 and 2012, 228 consecutive PCNL patients were retrospectively reviewed. Preoperative urine and stone cultures at time of PCNL were obtained. Patients with positive cultures received extended microbe directed therapy. All others received 7 days of empiric therapy pre and postoperatively. Antibiotic related adverse events and infectious outcomes were recorded. RESULTS There were 67 (31%) infected stones and 64 (31%) patients with positive preoperative urine culture. For sterile and infected stones, mean duration of pre and postoperative antibiotic therapy was 9 vs. 14 and 10 vs. 43 days. Nitrofurantoin, trimethoprim/sulfamethoxazole, or ciprofloxacin was administered pre in 114 (50%), 32 (14%), 42 (18%) and postoperatively in 90 (39%), 42 (18%), 34 (15%) patients. Forty-three (19%) patients had 48 infectious complications: 2 (0.9%) urosepsis, 27 (12%) SIRS, 3 (1%) pneumonia, 10 (4%) UTI (of which 5 (50%) had multi-drug resistant organisms) and 5 (2%) candidiasis. There were 20 (9%) antibiotic associated complications: 8 (4%) drug rash, 2 (0.9%) swelling, 6 (3%) gastrointestinal upset, 1 (0.4%) C. diff colitis, 1 transient renal insufficiency, 1 delirium and 1 shortness of breath. There was no significant difference in antibiotic or infectious related complications based on duration of antibiotic therapy pre (p=0.50 and 0.80) or postoperatively (p=0.76 and 0.23). Patients receiving multiple antibiotics postoperatively were more likely to experience an adverse drug event (11 (19%) vs. 8 (5%); p = 0.005). In those with a positive stone culture, 17 (46%) developed an infectious complication (p = 0.05). Diabetes, struvite stones and preoperative UTI were not significant predictors of infectious or antibiotic related complications. CONCLUSIONS With a prolonged course of antibiotic therapy at the time of PCNL, our data demonstrates a low rate of postoperative infection, specifically urosepsis, and antibiotic related complications in a high risk PCNL cohort. Postoperatively, multi-antibiotic therapy was associated with increased risk of adverse drug event. Furthermore, positive stone culture increased the risk of infectious complications. © 2014FiguresReferencesRelatedDetails Volume 191Issue 4SApril 2014Page: e188 Advertisement Copyright & Permissions© 2014MetricsAuthor Information Boyd Viers More articles by this author Patrick Cockerill More articles by this author Amy Krambeck More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...

Absence of cell surface expression of human ACE leads to perinatal death.

Renal tubular dysgenesis (RTD) is a recessive autosomal disease characterized most often by perinatal death. It is due to the inactivation of any of the major genes of the renin-angiotensin system (RAS), one of which is the angiotensin I-converting enzyme (ACE). ACE is present as a tissue-bound enzyme and circulates in plasma after its solubilization. In this report, we present the effect of different ACE mutations associated with RTD on ACE intracellular trafficking, secretion and enzymatic activity. One truncated mutant, R762X, responsible for neonatal death was found to be an enzymatically active, secreted form, not inserted in the plasma membrane. In contrast, another mutant, R1180P, was compatible with life after transient neonatal renal insufficiency. This mutant was located at the plasma membrane and rapidly secreted. These results highlight the importance of tissue-bound ACE versus circulating ACE and show that the total absence of cell surface expression of ACE is incompatible with life. In addition, two missense mutants (W594R and R828H) and two truncated mutants (Q1136X and G1145AX) were also studied. These mutants were neither inserted in the plasma membrane nor secreted. Finally, the structural implications of these ACE mutations were examined by molecular modelling, which suggested some important structural alterations such as disruption of intra-molecular non-covalent interactions (e.g. salt bridges).