Adult Polycystic Kidney Disease Research Articles

Pregnancy outcomes for women with pre-existing renal disease and the role of a dedicated joint maternal medicine and renal clinic: A retrospective cohort study.

To reaffirm the value of a joint obstetric and renal clinic on obstetric outcomes in patients with high-risk pregnancies due to chronic kidney disease (CKD). This was a retrospective cohort study of patients who attended the clinic between 2005 and December 2021. The hospital is a regional tertiary unit for renal medicine and a maternal medicine hub. The data included all women with pre-existing renal conditions who were cared for in a dedicated renal and obstetric clinic. Datasets were extracted from hospital notes, the renal database, clinical data and maternity electronic health records. The data analyzed included pre-existing renal conditions, biochemical parameters related to the renal condition, pregnancy outcomes included miscarriages, gestation, mode of delivery, postpartum hemorrhage (PPH), loss, birth weight and neonatal admission. The results were as follows: Lupus nephritis: four term deliveries; three had pre-eclampsia; two PPH and two miscarriages. Four estimated glomerular filtration rates (eGFRs) returned to baseline levels within 12 months. With regard to IgA nephropathy there were five live births, four term deliveries, two pre-eclampsia (PE) and five cesarean sections (CS). All eGFRs returned to baseline within 12 months. With regard to patients with adult polycystic kidney disease (APKD), there were six live births, two had pre-eclampsia and there were five term vaginal deliveries. Patients with lupus nephritis, APKD, and IgA demonstrated a higher incidence of adverse pregnancy outcomes as compared with our local pregnant population. Our findings reflect those of larger studies and support the role of combined renal/obstetric clinics. More research and larger scale studies are needed into specific CKD conditions and their outcomes.

Monoallelic IFT140 Pathogenic Variants in Adult Polycystic Kidney Disease Patients Without Family History

Monoallelic IFT140 Pathogenic Variants in Adult Polycystic Kidney Disease Patients Without Family History

The crucial role of the nephrologist in the management of pregnancy in women with adult autosomal dominant polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is a genetic kidney disease characterized by gradual kidney enlargement and progressive renal function loss. Pregnancy is a significant risk factor for adverse maternal and fetal outcomes in chronic kidney disease (CKD), regardless of the nephropathy. Women with ADPKD often face concerns about worsening their renal condition and passing the disease on to their offspring. Recent studies show better outcomes due to improved pregnancy surveillance and prior counseling. Risk factors for poor fetal and maternal outcomes include advanced maternal age, pre-existing hypertension, urinary infections, proteinuria, and renal failure. Collaboration between nephrologists and gynecologists is essential for addressing clinically significant concerns in pregnant women with ADPKD. Nephrologists should play an active role in assisting and supporting pregnant ADPKD patients as part of a multidisciplinary team.

Wall Tension and Tubular Resistance in Kidney Cystic Conditions.

The progressive formation of single or multiple cysts accompanies several renal diseases. Specifically, (i) genetic forms, such as adult dominant polycystic kidney disease (ADPKD), and (ii) acquired cystic kidney disease (ACKD) are probably the most frequent forms of cystic diseases. Adult dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by multiple kidney cysts and systemic alterations. The genes responsible for the condition are known, and a large amount of literature focuses on the molecular description of the mechanism. The present manuscript shows that a multiscale approach that considers supramolecular physical phenomena captures the characteristics of both ADPKD and acquired cystic kidney disease (ACKD) from the pathogenetic and therapeutical point of view, potentially suggesting future treatments. We first review the hypothesis of cystogenesis in ADPKD and then focus on ACKD, showing that they share essential pathogenetic features, which can be explained by a localized obstruction of a tubule and/or an alteration of the tubular wall tension. The consequent tubular aneurysms (cysts) follow Laplace's law. Reviewing the public databases, we show that ADPKD genes are widely expressed in various organs, and these proteins interact with the extracellular matrix, thus potentially modifying wall tension. At the kidney and liver level, the authors suggest that altered cell polarity/secretion/proliferation produce tubular regions of high resistance to the urine/bile flow. The increased intratubular pressure upstream increases the difference between the inside (Pi) and the outside (Pe) of the tubules (∆P) and is counterbalanced by lower wall tension by a factor depending on the radius. The latter is a function of tubule length. In adult dominant polycystic kidney disease (ADPKD), a minimal reduction in the wall tension may lead to a dilatation in the tubular segments along the nephron over the years. The initial increase in the tubule radius would then facilitate the progressive expansion of the cysts. In this regard, tubular cell proliferation may be, at least partially, a consequence of the progressive cysts' expansion. This theory is discussed in view of other diseases with reduced wall tension and with cysts and the therapeutic effects of vaptans, somatostatin, SGLT2 inhibitors, and potentially other therapeutic targets.

Diet and Physical Activity in Adult Dominant Polycystic Kidney Disease: A Review of the Literature.

Autosomal polycystic kidney disease is the most common inherited kidney disease determining 5% of all end-stage kidney disease. The only therapy approved for this condition is Tolvaptan, which, with its aquaretic effect, has a strong effect on patients' daily life. Recently, the literature has been enriched with new works that analyze possible non-pharmacological therapeutic strategies to slow cysts' enlargement and chronic kidney disease progression. Among them, dietary schemes reducing carbohydrate intake and inducing ketoses have been demonstrated to have efficacy in several pre-clinical and clinical studies. A ketogenic diet, calorie restriction, intermittent fasting, and time-restricted feeding can reduce aerobic glycolysis and inhibit the mTOR pathway, producing a reduction in cyst cell proliferation, a reduction in kidney volume, and helping to preserve kidney function. ADPKD's burden of disease has an impact on patients' quality of life, and the possibility to play sports or carry out physical exercise can help people in everyday life. The multisystemic character of the disease, especially cardiovascular involvement, needs to be carefully evaluated to establish the quality and quantity of physical activity that patients can safely carry out.

Rapid Diagnosis of Disseminated Tuberculosis Using Cell-free DNA Sequencing in a Kidney Transplant Recipient.

Here, we report a case of smear-negative disseminated tuberculosis (TB) in a kidney transplant recipient diagnosed using plasma cell-free DNA (cfDNA). A 68-y-old woman presented with 2 mo of fevers and a 15-pound weight loss. Six years earlier, she received a living donor kidney transplant from her husband for adult polycystic kidney disease. She received standard induction with antithymocyte globulin and maintenance immunosuppression with tacrolimus, mycophenolate mofetil, and low-dose prednisone. Her posttransplant course was uncomplicated. She was born and lived in Massachusetts and had not traveled internationally in many years. She worked in finance until she retired a few years prior. She had no history of incarceration or homelessness. Her hobbies included volunteering at a local food bank. On presentation, she was febrile to 38.8 °C and diaphoretic but otherwise appeared well. Her laboratory values were notable for a leukocytosis of 16 000 K/µL and a urinalysis with >100 white blood cells per high power field. There was no growth in the urine or blood cultures. Her nasopharyngeal multiplex polymerase chain reaction (PCR) was negative for common respiratory viruses. She had negative test results for Epstein-Barr virus DNA, cytomegalovirus DNA, HIV 1/2 antigen/antibody, cryptococcal and Histoplasma antigens. Chest computed tomography showed innumerable 1- to 3-mm bilateral nodules (Figure 1A) and lytic lesions of the T6 and T7 vertebral bodies. Spine magnetic resonance imaging demonstrated enhancing lesions involving the entire T6 and T7 bodies (Figure 1B).FIGURE 1.: Abnormal imaging findings. A, Chest CT scan with diffuse bilateral pulmonary nodules in a miliary pattern. B, MRI of the thoracic spine with enhancing lesions of the T6 and T7 vertebral bodies. CT, computed tomography.Further workup included bronchoalveolar lavage with multiple negative studies: bacterial cultures, Pneumocystis direct fluorescent antibody, galactomannan antigen, acid-fast bacilli (AFB) and modified AFB smear, and Mycobacterium tuberculosis PCR. Transbronchial biopsy was nondiagnostic, with negative AFB and fungal stains. Mycobacterial blood and urine cultures were also obtained. A biopsy of her T7 vertebral body noted chronic inflammation and 1 nonnecrotizing granuloma. On hospital day (HD) 13, while she remained febrile and without a definitive diagnosis, we sent a quantitative cfDNA test (Karius, Inc, Redwood City, CA). On HD 15, the Karius cfDNA test returned positive for M tuberculosis. Subsequently, the mycobacterial blood, urine, and bronchoalveolar lavage cultures grew M tuberculosis on HD 17, 17, and 21, respectively. Bone cultures grew M tuberculosis at 34 d after biopsy after the patient had been discharged from the hospital. Cell-free DNA tests have become increasingly prevalent in recent years, including donor-derived cfDNA, which is routinely used to detect allograft rejection. The Karius cfDNA test uses metagenomic next-generation sequencing to specifically detect microbial nucleic acid from over a thousand clinically relevant species in plasma.1 This was the first case of TB diagnosed by plasma cfDNA sequencing in our institution. The turnaround time of 48 h is a promising characteristic of this test, especially when slow-growing pathogens are suspected. Had it been obtained earlier in the course, this test would have not only expedited diagnosis and treatment initiation but also eliminated the need for invasive diagnostics. Nevertheless, like other molecular tests, cfDNA sequencing does not provide antimicrobial susceptibilities to guide antimicrobial therapy and thus does not eliminate the need for cultures. In a recent meta-analysis, cfDNA sequencing showed excellent specificity but poor pooled sensitivity for the diagnosis of TB.2 The very low-risk epidemiologic history for TB and disease onset in the late posttransplant period were other interesting features of this case. Her pretransplant T spot TB was negative. We considered donor-derived TB, but this was deemed unlikely because her donor was her husband, who was healthy and had a negative TB screening. She had no known exposures to TB, and her exposure to higher-risk populations via volunteerism was her only epidemiologic risk factor.

Unusual Presentation of Bladder Tumour: Haematuria only after Administration of Anti-Platelet Drugs

Anti-thrombotic agents are used for the treatment and prevention of cardiovascular diseases. Bleeding is the most important side-effect of these agents. The common causes of haematuria after anti-thrombotic administration are benign prostatic hyperplasia, haemorrhagic cystitis, renal calculi, renal infarction, infection, and adult polycystic kidney disease. Haematuria only after anti-thrombotic administration can be the presentation of a bladder tumour. Here, 71 years old male patient, with a bladder tumour was reported, who presented with haematuria only after he took dual anti-platelet drugs (tablet Ecosprin and tablet Clopidogrel). He took these drugs after he underwent the insertion of cardiac stents. He never had any previous history of haematuria. After the complaint, ultrasonography was performed and it reported a normal urinary system. So, the anti-platelet drugs were suspected to be the cause of haematuria. Tablet Clopidogrel was stopped and patient was sent home on Tablet Ecosprin. However, one month later, he again developed haematuria. Repeat imaging studies (ultrasonography and computed tomography) detected a urinary bladder mass. Transurethral resection of the mass was performed and a biopsy reported high-grade, muscle-invasive transitional cell carcinoma. This case highlights the fact that all cases of haematuria, irrespective of anti-thrombotic agents’ administration, should be suspected to be due to urological malignancy and the patients should undergo complete urological investigations, including cystoscopy.

Adult human kidney organoids originate from CD24+ cells and represent an advanced model for adult polycystic kidney disease.

Adult kidney organoids have been described as strictly tubular epithelia and termed tubuloids. While the cellular origin of tubuloids has remained elusive, here we report that they originate from a distinct CD24+ epithelial subpopulation. Long-term-cultured CD24+ cell-derived tubuloids represent a functional human kidney tubule. We show that kidney tubuloids can be used to model the most common inherited kidney disease, namely autosomal dominant polycystic kidney disease (ADPKD), reconstituting the phenotypic hallmark of this disease with cyst formation. Single-cell RNA sequencing of CRISPR-Cas9 gene-edited PKD1- and PKD2-knockout tubuloids and human ADPKD and control tissue shows similarities in upregulation of disease-driving genes. Furthermore, in a proof of concept, we demonstrate that tolvaptan, the only approved drug for ADPKD, has a significant effect on cyst size in tubuloids but no effect on a pluripotent stem cell-derived model. Thus, tubuloids are derived from a tubular epithelial subpopulation and represent an advanced system for ADPKD disease modeling.

Simultaneous Robotic-Assisted Bilateral Native Nephrectomy and Kidney Transplantation for Autosomal Dominant Polycystic Kidney Disease in Recipients With High Body Mass Index: Report of 2 Cases

Robotic kidney transplantation is a safe, reproducible, and less morbid technique in high body mass index and end-stage renal disease. Polycystic kidney disease is a relative contraindication to robotic-assisted kidney transplantation because of the mass effect of the native kidneys on the patient's pelvis that prevents ideal exposure. We report the first 2 cases of robotic-assisted simultaneous bilateral nephrectomy and kidney transplantation for patients with obesity and adult polycystic kidney disease. The recipients were 2 males, 50 and 53 years old, with a body mass index of 35.1 41.6 kg/m2 and 41.6 kg/m2, respectively. Both recipients had suitable living donors. The average operating time was 395 minutes and the estimated blood loss was on average 250 mL. The postoperative course was uneventful and the patients were discharged home on days 4 and 5. Performing robotic nephrectomies simultaneously with kidney transplantation can be done safely, allowing patients with obesity and polycystic kidney disease needing bilateral nephrectomy, to take full advantage of minimally invasive kidney transplantation.

Ttc21b deficiency attenuates autosomal dominant polycystic kidney disease in a kidney tubular- and maturation-dependent manner

Primary cilia are sensory organelles built and maintained by intraflagellar transport (IFT) multiprotein complexes. Deletion of several IFT-B genes attenuates polycystic kidney disease (PKD) severity in juvenile and adult autosomal dominant polycystic kidney disease (ADPKD) mouse models. However, deletion of an IFT-A adaptor, Tulp3, attenuates PKD severity in adult mice only. These studies indicate that dysfunction of specific cilia components has potential therapeutic value. To broaden our understanding of cilia dysfunction and its therapeutic potential, we investigate the role of global deletion of an IFT-A gene, Ttc21b, in juvenile and adult mouse models of ADPKD. Both juvenile (postnatal day 21) and adult (six months of age) ADPKD mice exhibited kidney cysts, increased kidney weight/body weight ratios, lengthened kidney cilia, inflammation, and increased levels of the nutrient sensor, O-linked β-N-acetylglucosamine (O-GlcNAc). Deletion of Ttc21b in juvenile ADPKD mice reduced cortical collecting duct cystogenesis and kidney weight/body weight ratios, increased proximal tubular and glomerular dilations, but did not reduce cilia length, inflammation, nor O-GlcNAc levels. In contrast, Ttc21b deletion in adult ADPKD mice markedly attenuated kidney cystogenesis and reduced cilia length, inflammation, and O-GlcNAc levels. Thus, unlike IFT-B, the effect of Ttc21b deletion in mouse models of ADPKD is development-specific. Unlike an IFT-A adaptor, deleting Ttc21b in juvenile ADPKD mice is partially ameliorative. Thus, our studies suggest that different microenvironmental factors, found in distinct nephron segments and in developing versus mature stages, modify ciliary homeostasis and ADPKD pathobiology. Further, elevated levels of O-GlcNAc, which regulates cellular metabolism and ciliogenesis, may be a pathological feature of ADPKD.

Deletion of STAT3 from Foxd1 cell population protects mice from kidney fibrosis by inhibiting pericytes trans-differentiation and migration.

Signal transduction and activator of transcription 3 (STAT3) is a key transcription factor implicated in the pathogenesis of kidney fibrosis. Although Stat3 deletion in tubular epithelial cells is known to protect mice from fibrosis, vFoxd1 cells remains unclear. Using Foxd1-mediated Stat3 knockout mice, CRISPR, and inhibitors of STAT3, we investigate its function. STAT3 is phosphorylated in tubular epithelial cells in acute kidney injury, whereas it is expanded to interstitial cells in fibrosis in mice and humans. Foxd1-mediated deletion of Stat3 protects mice from folic-acid- and aristolochic-acid-induced kidney fibrosis. Mechanistically, STAT3 upregulates the inflammation and differentiates pericytes into myofibroblasts. STAT3 activation increases migration and profibrotic signaling in genome-edited, pericyte-like cells. Conversely, blocking Stat3 inhibits detachment, migration, and profibrotic signaling. Furthermore, STAT3 binds to the Collagen1a1 promoter in mouse kidneys and cells. Together, our study identifies a previously unknown function of STAT3 that promotes kidney fibrosis and has therapeutic value in fibrosis.

Incidental finding of autosomal dominant polycystic kidney disease in a 52-year-old man: a case report.

Background: Autosomal dominant polycystic kidney disease (ADPKD) also known as Adult Polycystic Kidney Disease (APKD) is one of the most common systemic hereditary diseases. The disease usually presents between 20 to 39 years of age, although milder forms may not present until over 60 years and absence of renal failure has been rarely observed in some patients up to 80 years of age. Three distinct gene defects have been implicated in the pathogenesis of APKD designated as PKD1, PKD2, and PKD3. Patients with APKD may be asymptomatic, or may usually present with hypertension (in 50-70% of cases), renal insufficiency, and the complications of multiple cysts (haematuria, pain and infection) or as an abdominal mass discovered on incidental clinical or imaging examination. APKD is said to be rare in Africans. Case report: We report a case of an incidental finding of autosomal dominant polycystic kidney disease (ADPKD) in a 52-year-old normotensive man with a normal renal function test. Haematuria, dysuria and low-grade fever were the presenting complaints. The role of radiology in the diagnosis and management of ADPKD was highlighted. Conclusion: Although APKD is rare in Africans, a high index of suspicion for the disease is essential for the diagnosis of the disease especially in elderly patients with a family history of APKD who present with haematuria associated with multiple renal cysts.

Anaesthetic Management of a Patient with Von-Hippel-Lindau Syndrome: A Conglomeration of Perplexities

Von-Hippel-Lindau (VHL) syndrome is an autosomal dominant disease with multisystem involvement. Tumours associated with VHL include, hemangioblastomas of the spine and brain, retinal capillary hemangioblastomas, renal cell carcinoma, pheochromocytoma, and neuroendocrine tumours of pancreas. We present a case of a 54 year old female second-degree uterovaginal prolapse scheduled for laparoscopic assisted vaginal hysterectomy. She was diagnosed with adult polycystic kidney disease, pheochromocytoma, parathyroid adenoma, vertebral hemangioma and multiple renal and hepatic cysts. Though genetic testing was not feasible, the co-existing systemic manifestations in this patient were strongly suggestive of Von Hippel Lindau syndrome. Owing to the multi-morbidities, central neuraxial block was deemed appropriate as opposed to general anaesthesia. In concert with the surgeons, it was decided to change the surgical plan to vaginal hysterectomy and avoid adrenaline infiltration at the surgical site, as it is known to precipitate arrythmias. We placed an intra-arterial catheter for continuous blood pressure monitoring before administration of the subarachnoid block and co-loaded the patient with fluids to avoid any drastic hypotension. The patient remained hemodynamically stable throughout the procedure, which could be attributed to extensive pre-operative work-up multi-disciplinary pre-operative optimization and synergistic management with surgical team. Providing anaesthesia to patients with multisystem involvement such as Von Hippel Lindau syndrome can be very challenging. Meticulous pre-anaesthetic evaluation and pre-operative optimization are often crucial. A multidisciplinary approach is often employed to achieve the best possible pre-operative functional status of these patients. Well-coordinated team with flexibility in management tailored to the patient helps achieve the best possible outcomes. Disclaimer: Informed consent was obtained from the patient prior to publication Main Points • Von-Hippel-Lindau (VHL) syndrome is an autosomal dominant disease with multisystem involvement; associated tumours include hemangioblastomas of the CNS, retinal capillary hemangioblastomas, renal cell carcinoma, pheochromocytoma, endolymphatic sac tumours of the middle ear, serous cystadenomas and neuroendocrine tumours of pancreas. • Providing anaesthesia to patients with multisystem involvement such as Von Hippel Lindau syndrome can be very challenging. Meticulous pre-anaesthetic evaluation and pre-operative optimization are often crucial. • A detailed anaesthetic plan, tailored to each patient, has to be formulated in elective cases, after an extensive work-up of the associated tumours. • Extensive pre-operative work-up, a multi-disciplinary approach towards pre-operative optimization and synergistic management with surgical team remains vital in minimizing peri-operative complications. • Anaesthetic implications are often at the nexus of surgical outcomes, hence, proper communication with the surgical team and arrival at a joint plan is crucial.

Robotic-assisted Kidney Transplantation With Simultaneous Bilateral Nephrectomies Is an Efficient, Feasible, and Safe Way to Manage Patients With Renal Failure Secondary to Adult Polycystic Kidney Disease.

Kidney transplantation has become the standard of care for end-stage renal disease secondary to adult polycystic kidney disease. Open surgical techniques remain the gold standard, although minimally invasive methods have gained traction in recent years. Native nephrectomy is frequently needed secondary to size or symptoms. Continued developments in surgical technology have allowed for the introduction of computer-assisted surgery (Robotics). We aim to describe the feasibility, safety, and efficiency of simultaneous laparoscopic bilateral nephrectomy and robotic-assisted kidney transplantation to treat end-stage renal kidney transplantation secondary to polycystic kidney disease. In this initial experience, 3 patients underwent kidney transplantation with a simultaneous bilateral nephrectomy. All patients tolerated the procedure well with no postoperative blood transfusions, dialysis, or surgical site infections. Simultaneous laparoscopic bilateral nephrectomy and robotic-assisted kidney transplantation may be feasible, safe, and efficient techniques. Complications were minimal, with short hospital stays.Supplemental Video; http://links.lww.com/TXD/A352

Functional megalin is expressed in renal cysts in a mouse model of adult polycystic kidney disease.

BackgroundAutosomal dominant polycystic kidney disease (ADPKD) is characterized by the progressive growth of cysts and a decline of renal function. The clinical feasibility of the number of potential disease-modifying drugs is limited by systemic adverse effects. We hypothesize that megalin, a multiligand endocytic receptor expressed in the proximal tubule, may be used to facilitate drug uptake into cysts, thereby allowing for greater efficacy and fewer side effects.MethodsThe cyst expression of various tubular markers, including megalin and aquaporin 2 (AQP2), was analysed by immunohistochemistry (IHC) of kidney sections from the ADPKD mouse model (PKD1RC/RC) at different post-natal ages. The endocytic function of megalin in cysts was examined by IHC of kidney tissue from mice injected with the megalin ligand aprotinin.ResultsCyst lining epithelial cells expressing megalin were observed at all ages; however, the proportion decreased with age. Concomitantly, an increasing proportion of cysts revealed expression of AQP2, partial expression of megalin and/or AQP2 or no expression of the examined markers. Endocytic uptake of aprotinin was evident in megalin-positive cysts, but only in those that remained connected to the renal tubular system.ConclusionsMegalin-expressing cysts were observed at all ages, but the proportion decreased with age, possibly due to a switch in tubular origin, a merging of cysts of different tubular origin and/or a change in the expression pattern of cyst lining cells. Megalin expressed in cysts was functional, suggesting that megalin-mediated endocytosis is a potential mechanism for drug targeting in ADPKD if initiated early in the disease.

Trichosporonosis Causing Mycotic Aortic Root Pseudoaneurysm after Cardiac Transplantation

<h3>Introduction</h3> Trichosporonosis and complications at the aorto-allograft anastomosis are rare after cardiac transplantation (CTx). We report a case of Trichosporon endocarditis and mycotic pseudoaneurysm. <h3>Case Report</h3> A 51 year old female with dilated cardiomyopathy and adult polycystic kidney disease underwent bilateral nephrectomy and was listed for renal transplant (RTx). While waiting, her cardiac status progressed and she was assessed for combined CTx-RTx. Staged surgery was planned, with CTx followed by urgent RTx. She underwent orthotopic CTx in 2017. Early complications (including 2R rejection, drug-induced leucopenia, cytomegalovirus viraemia, and an infected fistula) delayed listing for RTx. Eventually, she underwent cadaveric RTx in 2019. Two weeks after RTx she was admitted with fevers, skin lesions and renal graft dysfunction. She had erythematous papules developing into bullae with ulceration and central necrosis, and loss of vision due to endophalmitis. Mycology from her skin and eye identified fungus of the Trichosporon species and she was treated with antifungal therapy comprising liposomal amphotericin B, Voriconzaole and Flucytosine. Transoesophageal echocardiography showed a large pseudoaneurysm at the aortic anastomosis with vegetations on the suture line and in the cavity. Serial computed tomography showed the pseudoaneurysm increasing in size despite medical therapy, and she underwent aortic valve and root replacement. Explanted tissue confirmed endocarditis with Trichlosporon species. She made a good surgical recovery. Five months after admission she was discharged with good cardiac and renal graft function, on lifelong voriconazole. <h3>Summary</h3> This is the first report of Trichosporon endocarditis and suture-line pseudoaneurysm after CTx. Haemodialysis was a key risk factor for infection, potentially avoided by earlier RTx. She remains free of recurrent fungaemia on long term suppression after 1 year, but there remains a risk of recurrent infection in the aortic root.

Mirror aneurysm of ICA terminus associated with adult polycystic kidney disease

Introduction. Bilatereal saccular cerebral aneurysms (SCAs) that developed symmetrically on the same named vessels are defined as mirror aneurysms and account for a small subset of multiple cerebral aneurysms. The internal carotid artery (ICA) bifurcation is a rare location for mirror aneurysms. Aim. We aimed to present the importance of risk status assessment for SCAs and screening in all ADPKD patients for timely detection and managementof SCAs before catastrophic complications occur Description of the case. We present mirror aneurysms of bilateral ICA bifurcation that appear like a couple of dancing men on coronal computed tomography angiography (CTA) images, which were successfully treated with single stage coil embolization in a 45 year old female patient with medical history of autosomal dominant policystic kidney disease (ADPKD). Conclusion. SCAs are more frequent in patients with ADPKD than in general population and also these aneurysms are more likely to rupture at earlier ages. Mirror aneurysms of ICA terminus can be treated effectively and safely by single stage coil embolization.

Management of end-stage kidney failure in patients with adult polycystic kidney disease

Management of end-stage kidney failure in patients with adult polycystic kidney disease

A Comparison of Urine Dilution Ability between Adult Dominant Polycystic Kidney Disease, Other Chronic Kidney Diseases, and Healthy Control Subjects: A Case-Control Study.

The final dilution of urine is regulated via aquaporin-2 water channels in the distal part of the nephron. It is unclear whether urine dilution ability in autosomal dominant polycystic kidney disease patients (ADPKD patients) differs from other patients with similar degree of impaired renal function (non-ADPKD patients). The purpose of this case control study was to measure urine dilution ability in ADPKD patients compared to non-ADPKD patients and healthy controls. Methods. Eighteen ADPKD, 16 non-ADPKD patients (both with chronic kidney disease, stage I-IV), and 18 healthy controls received an oral water load of 20 ml/kg body weight. Urine was collected in 7 consecutive periods. We measured free water clearance (CH2O), urine osmolality, urine output, fractional excretion of sodium, urine aquaporin2 (u-AQP2), and urine epithelial sodium channel (u-ENaC). Blood samples were drawn four times (at baseline, 2 h, 4 h, and 6 hours after the water load) for analyses of plasma osmolality, vasopressin, renin, angiotensin II, and aldosterone. Brachial and central blood pressure was measured regularly during the test. Results. The three groups were age and gender matched, and the patient groups had similar renal function. One hour after water load, the ADPKD patients had an increased CH2O compared to non-ADPKD patients (2.97 ± 2.42 ml/min in ADPKD patients vs. 1.31 ± 1.50 ml/min in non-ADPKD patients, p0.029). The reduction in u-AQP2 and u-ENaC occurred earlier in ADPKD than in non-ADPKD patients. Plasma concentrations of vasopressin, renin, angiotensin II, and aldosterone and blood pressure measurements did not show any differences that could explain the deviation in urine dilution capacity between the patient groups. Conclusions. ADPKD patients had a higher CH2O than non-ADPKD patients after an oral water load, and u-AQP2 and u-ENaC were more rapidly reduced than in non-ADPKD patients. Thus, urine-diluting capacity may be better preserved in ADPKD patients than in non-ADPKD patients.

S0034 Epidemiology and Risk of Pancreatic Cyst Among Adult Polycystic Kidney Disease

S0034 Epidemiology and Risk of Pancreatic Cyst Among Adult Polycystic Kidney Disease