|
1
|
Castaneda P, Masterson J, Naser-Tavakolian A, Kim I, Najjar R, Gupta A. Surgical outcomes of robotic bilateral nephrectomy compared to open surgery in adult polycystic kidney disease. World J Urol 2025; 43:270. [PMID: 40323445 PMCID: PMC12052875 DOI: 10.1007/s00345-025-05556-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2024] [Accepted: 03/04/2025] [Indexed: 05/08/2025] Open
Abstract
PURPOSE Native nephrectomies for patients with autosomal dominant polycystic kidney disease (ADPKD) have traditionally been performed via an open approach. We have previously described our experience with robotic synchronous bilateral nephrectomies. However, little data is available comparing open nephrectomy (ONx) to robotic nephrectomy (RNx). Here we compare outcomes of ONx and RNx in patients with ADPKD undergoing synchronous bilateral nephrectomy. METHODS We performed a retrospective review of patients with ADPKD undergoing open or robotic synchronous bilateral nephrectomy from January 2015 to November 2023 at a single institution. Patient characteristics, perioperative factors, kidney size, and complication rates were compared. RESULTS Overall, seventeen patients underwent RNx and fifteen patients underwent ONx. There was no significant difference in gender, preoperative BMI, or kidney volume. Patients undergoing ONx had significantly higher estimated blood loss (EBL), length of stay (LOS), and higher rates of non-autologous blood transfusion and complications overall. Complications in the ONx group included 10 patients who required blood transfusions and 2 patients who sustained visceral injuries. Time from nephrectomy to transplant was significantly shorter in the RNx arm. CONCLUSION Robotic synchronous bilateral nephrectomies for ADPKD may have advantages over the traditional open approach, including lower EBL, shorter LOS, decreased overall complication rates, less severe complications and potentially faster time from nephrectomy to transplant.
Collapse
Affiliation(s)
- Peris Castaneda
- Department of Urology, Cedars-Sinai Medical Center, Los Angeles, CA, USA
| | - John Masterson
- Department of Urology, Weill Cornell Medical College, New York, NY, USA
| | | | - Irene Kim
- Department of Surgery, Cedars-Sinai Medical Center, Los Angeles, CA, USA
- Cedars-Sinai Comprehensive Transplant Center, CA, Los Angeles, United States
| | - Reiad Najjar
- Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA
- Cedars-Sinai Comprehensive Transplant Center, CA, Los Angeles, United States
| | - Amit Gupta
- Beverly Hills Urology, 8631 W 3rd St Ste 531, CA, 90048, Los Angeles, United States of America.
| |
Collapse
|
|
2
|
Dugbartey GJ, Alornyo KK, Dapaa-Addo CO, Botchway E, Kwashie EK, Harley Y. Alpha-lipoic acid: A promising pharmacotherapy seen through the lens of kidney diseases. CURRENT RESEARCH IN PHARMACOLOGY AND DRUG DISCOVERY 2024; 7:100206. [PMID: 39524210 PMCID: PMC11550178 DOI: 10.1016/j.crphar.2024.100206] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2024] [Revised: 10/09/2024] [Accepted: 10/21/2024] [Indexed: 11/16/2024] Open
Abstract
Kidney diseases have rapidly increased in prevalence over the past few decades, and have now become a major global public health concern. This has put economic burden on the public healthcare system and causing significant morbidity and mortality worldwide. Unfortunately, drugs currently in use for the management of kidney diseases have long-term major adverse effects that negatively impact the quality of life of these patients, hence making these drugs a "necessary evil". In recent times, antioxidant therapy has been explored as a potential pharmacological avenue for treatment of kidney diseases, and could offer a better therapeutic option with less adverse effect profile. One of such antioxidants is alpha-lipoic acid (ALA), a sulphur-containing multifunctional antioxidant that is endogenously produced by lipoic acid synthase in the mitochondria of many tissues, including the kidney. Burgeoning evidence indicates that ALA is showing clinical promise in the treatment and pharmacological management of many kidney diseases through its antioxidant and other therapeutic properties by activating several protective mechanisms while inhibiting deleterious signaling pathways. In this review, we present ALA as a potent naturally occurring antioxidant, its mitochondrial biosynthesis and pharmacological properties. In addition, we also discuss within the limit of present literature, ALA and its underlying molecular mechanisms implicated in experimental and clinical treatment of various kidney conditions, and thus, may offer nephrologists an additional and/or alternative avenue in the pharmacological management and treatment of kidney diseases while giving hope to these patients.
Collapse
Affiliation(s)
- George J. Dugbartey
- Department of Pharmacology and Toxicology, School of Pharmacy, College of Health Sciences, University of Ghana, Legon, Accra, Ghana
- Department of Physiology and Pharmacology, Accra College of Medicine, East Legon, Accra, Ghana
- Department of Surgery, Division of Urology, London Health Sciences Centre, Western University, N6A 5C1, London, ON, Canada
- Matthew Mailing Centre for Translational Transplant Studies, London Health Sciences Centre, Western University, N6A 5C1, London, ON, Canada
| | - Karl K. Alornyo
- Department of Pharmacology and Toxicology, School of Pharmacy, College of Health Sciences, University of Ghana, Legon, Accra, Ghana
| | | | - Emmanuel Botchway
- Department of Physiology and Pharmacology, Accra College of Medicine, East Legon, Accra, Ghana
| | - Emmanuel K. Kwashie
- Department of Physiology and Pharmacology, Accra College of Medicine, East Legon, Accra, Ghana
| | - Yvonne Harley
- Department of Physiology and Pharmacology, Accra College of Medicine, East Legon, Accra, Ghana
| |
Collapse
|
|
3
|
Trutin I, Oletić L, Nikuševa-Martić T. Unraveling the Complexity of Childhood Polycystic Kidney Disease: A Case Study of Three Sisters. CHILDREN (BASEL, SWITZERLAND) 2023; 10:1700. [PMID: 37892363 PMCID: PMC10605507 DOI: 10.3390/children10101700] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/08/2023] [Revised: 10/11/2023] [Accepted: 10/15/2023] [Indexed: 10/29/2023]
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disorder, estimated to affect 1 in 1000 people. It displays a high level of variability in terms of onset and severity among affected individuals within the same family. In this case study, three sisters (4, 8, and 10 years of age) were suspected of having ADPKD due to their positive family history. While the two younger sisters aged 8 and 4 showed no disease complications and had normal kidney function, the oldest sister was found to have no dipping status on ambulatory blood pressure measurement (ABPM). Two of the sisters were discovered to have a PKD1 mutation, while the third sister aged 8 was heterozygous for TTC21B c.1593_1595del, p. (Leu532del), which is a variant of uncertain significance (VUS). Environmental factors and genetic modifying factors are believed to contribute to the phenotypic variability observed in ADPKD. Identifying and understanding potential genetic and environmental modifiers of ADPKD could pave the way to targeted treatments for childhood ADPKD.
Collapse
Affiliation(s)
- Ivana Trutin
- Department of Pediatrics, Sestre Milosrdnice University Hospital Center, 10000 Zagreb, Croatia; (I.T.); (L.O.)
| | - Lea Oletić
- Department of Pediatrics, Sestre Milosrdnice University Hospital Center, 10000 Zagreb, Croatia; (I.T.); (L.O.)
| | | |
Collapse
|
|
4
|
Bizzoca C, Fiore F, Aquilino F, Fedele S, Salvo MD, Lucarelli G, Vincenti L. A new technique for the laparoscopic treatment of simple hepatic cysts. SURGERY IN PRACTICE AND SCIENCE 2023; 13:100171. [PMID: 39845400 PMCID: PMC11749805 DOI: 10.1016/j.sipas.2023.100171] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/13/2022] [Revised: 03/03/2023] [Accepted: 04/19/2023] [Indexed: 01/24/2025] Open
Abstract
Background Simple hepatic cysts are commonly detected in the general population, both solitary and associated with Adult Dominant Polycystic Kidney Disease (ADPKD). Laparoscopic fenestration is a surgical option adopted as first-line treatment and to treat complications. The techniques reported in the literature are associated with cyst recurrence in up to 41% of cases. Methods From 2012 to May 2021, 19 symptomatic patients diagnosed with simple HCs underwent an innovative technique for laparoscopic fenestration, which includes simultaneous ethanol injection into the residual cavity. The median follow up was 57 (range 4-116) months. We retrospectively analysed symptomatic relief obtained in the short and long term as primary outcome. We also evaluated the postoperative outcome, recurrence and re-intervention rates. Results 11 patients (of 19) were female (58 %), with a median age of 58 (range 31-78) years. Most patients (17 of 19) experienced relief of symptoms after intervention (89,5 %). Radiological recurrence occurred in 21% of patients; nevertheless, only one patient, affected by ADPKD, experienced clinical relapse with abdominal discomfort. No patient needed reintervention. There was no major morbidity (Clavien-Dindo III-IV) nor 90-day mortality. The technique allowed early removal of abdominal drainage (median 2.5 days). Conclusions Laparoscopic fenestration of a simple hepatic cyst, with simultaneous ethanol injection, combines the advantages of the laparoscopic approach with those of injecting sclerosing agent. The described technique is associated with symptomatic relief and a favourable outcome in the postoperative period, as well as with good long term results.
Collapse
Affiliation(s)
- Cinzia Bizzoca
- Department of General Surgery “Ospedaliera”. Polyclinic Hospital of Bari, Piazza G. Cesare, 11, 70124 Bari, Italy
| | - Felicia Fiore
- Department of General Surgery “Ospedaliera”. Polyclinic Hospital of Bari, Piazza G. Cesare, 11, 70124 Bari, Italy
| | - Fabrizio Aquilino
- Department of General Surgery. National Institute of Gastroenterology Saverio de Bellis, Research Hospital, via Turi 27, 70013 Castellana Grotte, Bari, Italy. via Turi 27, 70013 Castellana Grotte, Bari, Italy
| | - Salvatore Fedele
- Department of General Surgery. National Institute of Gastroenterology Saverio de Bellis, Research Hospital, via Turi 27, 70013 Castellana Grotte, Bari, Italy. via Turi 27, 70013 Castellana Grotte, Bari, Italy
| | - Maria Di Salvo
- Department of General Surgery “Ospedaliera”. Polyclinic Hospital of Bari, Piazza G. Cesare, 11, 70124 Bari, Italy
| | - Giuseppe Lucarelli
- Department of Emergency and Organ Transplantation-Urology, Andrology and Kidney Transplantation Unit, University of Bari, 70124 Bari, Italy
| | - Leonardo Vincenti
- Department of General Surgery “Ospedaliera”. Polyclinic Hospital of Bari, Piazza G. Cesare, 11, 70124 Bari, Italy
| |
Collapse
|
|
5
|
Darius T, Bertoni S, De Meyer M, Buemi A, Devresse A, Kanaan N, Goffin E, Mourad M. Simultaneous nephrectomy during kidney transplantation for polycystic kidney disease does not detrimentally impact comorbidity and graft survival. World J Transplant 2022; 12:100-111. [PMID: 35663541 PMCID: PMC9136716 DOI: 10.5500/wjt.v12.i5.100] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2021] [Revised: 08/11/2021] [Accepted: 04/09/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The lack of space, as an indication for a native unilateral nephrectomy for positioning a future kidney graft in the absence of other autosomal dominant polycystic kidney disease-related symptoms, remains controversial. AIM To evaluate the surgical comorbidity and the impact on graft survival of an associated ipsilateral native nephrectomy during isolated kidney transplantation in patients with autosomal dominant polycystic kidney disease. METHODS One hundred and fifty-four kidney transplantations performed between January 2007 and January 2019 of which 77 without (kidney transplant alone (KTA) group) and 77 with associated ipsilateral nephrectomy (KTIN group), were retrospectively reviewed. Demographics and surgical variables were analyzed and their respective impact on surgical comorbidity and graft survival. RESULTS Creation of space for future graft positioning was the main reason (n = 74, 96.1%) for associated ipsilateral nephrectomy. No significant difference in surgical comorbidity (lymphocele, wound infection, incisional hernia, wound hematoma, urinary infection, need for blood transfusion, hospitalization stay, Dindo Clavien classification and readmission rate) was observed between the two study groups. The incidence of primary nonfunction and delayed graft function was comparable in both groups [0% and 2.6% (P = 0.497) and 9.1% and 16.9% (P = 0.230), respectively, in the KTA and KTIN group]. The 1- and 5-year graft survival were 94.8% and 90.3%, and 100% and 93.8%, respectively, in the KTA and KTIN group (P = 0.774). The 1- and 5-year patient survival were 96.1% and 92.9%, and 100% and 100%, respectively, in the KTA and KTIN group (P = 0.168). CONCLUSION Simultaneous ipsilateral native nephrectomy to create space for graft positioning during kidney transplantation in patients with autosomal dominant polycystic kidney disease does not negatively impact surgical comorbidity and short- and long-term graft survival.
Collapse
Affiliation(s)
- Tom Darius
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Sébastien Bertoni
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Martine De Meyer
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Antoine Buemi
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Arnaud Devresse
- Division of Nephrology, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Nada Kanaan
- Division of Nephrology, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Eric Goffin
- Division of Nephrology, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Michel Mourad
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| |
Collapse
|
|
6
|
Torra R, Pérez-Gómez MV, Furlano M. Autosomal dominant polycystic kidney disease: possibly the least silent cause of chronic kidney disease. Clin Kidney J 2021; 14:2281-2284. [PMID: 34754424 PMCID: PMC8573017 DOI: 10.1093/ckj/sfab132] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/21/2021] [Accepted: 07/05/2021] [Indexed: 11/12/2022] Open
Abstract
Pain is the highest prioritized patient-reported outcome in people with autosomal dominant polycystic kidney disease (ADPKD) but it remains infrequently and inconsistently measured across countries, studies and trials. The study by El-Damanawi et al. integrated a network of ADPKD expert clinicians, pain specialists, researchers and patient representatives from the national UK PKD charity, with the aim of addressing the lack of validated ADPKD-specific pain assessment tools (APATs). The APAT designed by the authors included several pain measurement tools and was tested in ADPKD patients, although further validation through assessment in larger cohorts is needed. Establishing a standardized instrument for pain measurement will ensure that pain is measured and reported in a consistent way to inform decision-making and identify effective interventions aimed at managing pain and minimizing the impact pain has on patients with ADPKD. In this context, the APAT established by the authors is to be warmly welcomed.
Collapse
Affiliation(s)
- Roser Torra
- Inherited Kidney Diseases Nephrology Department, Fundació Puigvert IIB-Sant Pau Medicine Department, Universitat Autónoma de Barcelona, REDinREN, Barcelona, Spain
| | | | - Mónica Furlano
- Inherited Kidney Diseases Nephrology Department, Fundació Puigvert IIB-Sant Pau Medicine Department, Universitat Autónoma de Barcelona, REDinREN, Barcelona, Spain
| |
Collapse
|
|
7
|
Philo D. Horseshoe Kidney in Conjunction With Autosomal Dominant Polycystic Kidney Disease: A Case Report. JOURNAL OF DIAGNOSTIC MEDICAL SONOGRAPHY 2021. [DOI: 10.1177/8756479320988290] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
Horseshoe kidney in the presence of autosomal dominant polycystic kidney disease is a rare occurrence of two relatively common and unrelated renal findings. Visualization of multiple, bilateral cysts along with fusion of the kidneys by a midline isthmus can usually isolate these diagnoses. Accurate sonographic evaluation is essential in determining the degree of disease progression and possible complications associated with these diseases. Sonography is also useful in identifying extrarenal involvement and eliminating differential diagnoses.
Collapse
Affiliation(s)
- Dakota Philo
- Diagnostic Medical Sonography Program, UCHealth University of Colorado Hospital, Aurora, CO, USA
| |
Collapse
|
|
8
|
El Chediak A, Degheili JA, Khauli RB. Genitourinary Interventions in Autosomal Dominant Polycystic Kidney Disease: Clinical Recommendations for Urologic and Transplant Surgeons. EXP CLIN TRANSPLANT 2021; 19:95-103. [PMID: 33494664 DOI: 10.6002/ect.2020.0292] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
Autosomal dominant polycystic kidney disease is the fourth most common single cause of end-stage renal disease worldwide with both renal and extrarenal manifestations, resulting in significant morbidity. Approaches to the management of this disease vary widely, with no broadly accepted practice guidelines. Herein, we reviewed the various surgical and interventional management options that are targeted toward treating the symptoms or addressing the resulting kidney failure. Novel treatment modalities such as celiac plexus blockade and renal denervation appear to be promising in pain relief; however, further studies are lacking. Renal cyst decortication seems to have a higher success rate in targeting cyst-related pain compared with aspiration only. In terms of requiring major surgical intervention, such as need and timing of native nephrectomy, there are several considerations when deciding on transplantation with or without a pretransplant native nephrectomy. Patients who are not candidates for native nephrectomy may consider transcatheter arterial embolization. Based on our review of the contemporary indications for genitourinary interventions in the management of autosomal dominant polycystic kidney disease, we propose an algorithm that depicts the decision-making process on assessing the indications and timing of native nephrectomy in patients with end-stage renal disease awaiting transplant.
Collapse
Affiliation(s)
- Alissar El Chediak
- From the Department of Nephrology, Vanderbilt University Medical Center, Nashville, USA
| | - Jad A Degheili
- From the Division of Pediatric Urology, Department of Surgery, Children's Hospital of Eastern Ontario, University of Ottawa, Ontario, Canada.,From the Division of Urology and Renal Transplantation, Department of Surgery, American University of Beirut Medical Center, Beirut, Lebanon
| | | |
Collapse
|
|
9
|
Cansever HN, Sari F, Cevikol C, Cetinkaya R, Süleymanlar G, Ersoy F. Serum uromodulin levels, MR imaging findings, and their relationship with eGFR-based CKD staging in ADPKD patients. Int Urol Nephrol 2021; 53:1383-1389. [PMID: 33389516 DOI: 10.1007/s11255-020-02730-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/06/2020] [Accepted: 12/02/2020] [Indexed: 10/22/2022]
Abstract
PURPOSE Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease that may progress to end-stage renal disease, characterized by increased kidney volume due to cystic formations. In this study, we aimed to investigate the relationship between serum uromodulin levels, total kidney volume and estimated glomerular filtration rate (eGFR) in patients with ADPKD. METHODS This study included a total of 54 ADPKD patients and 18 healthy volunteers (control group). Total kidney volumes were calculated through magnetic resonance images using ellipsoid method. Serum uromodulin measurements were measured using an ELISA method. RESULTS Serum uromodulin levels were lower in patients compared with the control group (2.47 ± 0.16 vs 2.6 ± 0.28, p = 0.021). There was no significant difference in uromodulin values among the patients in chronic kidney disease (CKD) stages 1-2, 3 and 4-5. TKV measurements of CKD stage 4-5 patients were significantly higher than the stage 1-2 patients (p = 0.015). A negative correlation was observed between TKV and eGFR (r = - 0.433, p = 0.001). A positive correlation was observed between uromodulin and eGFR (r = 0.274, p = 0.02). When the serum levels of uromodulin and the level of eGFR were evaluated using simple linear regression analysis, R2 value was found to be 0.075, suggesting that 7.5% change in serum uromodulin values corresponds with the change in eGFR value. CONCLUSION These findings are consistent with previous studies that reported that serum uromodulin may be a good biomarker for demonstrating renal function in the early stages of CKD, before eGFR levels deteriorate. Serum uromodulin level may be useful in demonstrating renal functions in the follow-up of individuals with ADPKD.
Collapse
Affiliation(s)
- Hale Nur Cansever
- Division of Nephrology, Department of Medicine, Akdeniz University Hospital, Dumlupinar Bulvari, Konyaaltı, 07070, Antalya, Turkey
| | - Funda Sari
- Division of Nephrology, Department of Medicine, Akdeniz University Hospital, Dumlupinar Bulvari, Konyaaltı, 07070, Antalya, Turkey.
| | - Can Cevikol
- Division of Nephrology, Department of Medicine, Akdeniz University Hospital, Dumlupinar Bulvari, Konyaaltı, 07070, Antalya, Turkey
| | - Ramazan Cetinkaya
- Division of Nephrology, Department of Medicine, Akdeniz University Hospital, Dumlupinar Bulvari, Konyaaltı, 07070, Antalya, Turkey
| | - Gultekin Süleymanlar
- Division of Nephrology, Department of Medicine, Akdeniz University Hospital, Dumlupinar Bulvari, Konyaaltı, 07070, Antalya, Turkey
| | - Fevzi Ersoy
- Division of Nephrology, Department of Medicine, Akdeniz University Hospital, Dumlupinar Bulvari, Konyaaltı, 07070, Antalya, Turkey
| |
Collapse
|
|
10
|
Kassab GH, Robinson I, Hayes R, Paltiel HJ, Bates DG, Cohen HL, Barth RA, Colleran GCM. Urinary Tract. PEDIATRIC ULTRASOUND 2021:729-833. [DOI: 10.1007/978-3-030-56802-3_17] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
|
|
11
|
Infected hepatic cyst complicating urinary sepsis. Acta Gastroenterol Belg 2021; 84:131-134. [PMID: 33639705 DOI: 10.51821/84.1.319] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
Congenital hepatic cysts are a common disorder. Usually they are asymptomatic and do not have to be treated. However, some serious complications can occur. We report here the case of an 86-year old patient who has been treated by a percutaneous drainage for an infected solitary hepatic cyst due to urinary sepsis. She was admitted to the Department of Internal Medicine for epigastric pain with fever and chills. The patient was treated for a urinary tract infection 3 weeks ago by her General Practitioner. On admission, blood tests showed 21 620 neutrophils per microliter with a C-reactive protein level at 443.7 mg/L, procalcitonin > 200 ng/mL, total bilirubin at 1.43 mg/dL, lactate dehydrogenase at 666 U/L and alanine aminotransferase at 227 U/L. Urinalysis and hemocultures highlighted the presence of Escherichia coli and Streptococcus constellatus. The abdominal tomodensitometry indicated the presence of a left hepatic biliary cyst with banal appearance. She was first treated with intravenous amoxicillin clavulanic acid. After a few days, another abdominal tomodensitometry with contrast pinpointed a large abscess of 11 centimeters in diameter extending to liver segments II and IV with a similar small lesion in segments IV and V. clindamycin per os was added to the treatment because of its good diffusion in tissues. Percutaneous drain was inserted under tomodensitometric control and stayed in place until the follow-up at three weeks. Bacteriologic culture on the fluid sample demonstrated the presence of Escherichia coli and Streptococcus constellatus. The abscess completely regressed after 6 weeks of treatment and the biologic abnormalities resolved at the same time.
Collapse
|
|
12
|
Withey SJ, Verma H, Prezzi D. Multimodality Assessment of Cystic Renal Masses. Semin Ultrasound CT MR 2020; 41:334-343. [DOI: 10.1053/j.sult.2020.05.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
|
|
13
|
Jänigen BM, Hempel J, Holzner P, Schneider J, Fichtner-Feigl S, Thomusch O, Neeff H, Pisarski P, Glatz T. Simultaneous ipsilateral nephrectomy during kidney transplantation in autosomal dominant polycystic kidney disease: a matched pair analysis of 193 consecutive cases. Langenbecks Arch Surg 2020; 405:833-842. [PMID: 32705344 PMCID: PMC7471159 DOI: 10.1007/s00423-020-01939-3] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2020] [Accepted: 07/19/2020] [Indexed: 12/13/2022]
Abstract
BACKGROUND In end-stage renal transplant recipients with autosomal-dominant polycystic kidney disease (ADPKD), the imperative, optimal timing, and technique of native nephrectomy remains under discussion. The Freiburg Transplant Center routinely performs a simultaneous ipsilateral nephrectomy. METHODS From April 1998 to May 2017, we retrospectively analyzed 193 consecutive ADPKD recipients, receiving per protocol simultaneous ipsilateral nephrectomy and compared morbidity, mortality, and outcome with 193 non-ADPKD recipients of a matched pair control. RESULTS The incidence of surgical complications was similar with respect to severe medical, surgical, urological, vascular, and wound-related complications as well as reoperation rates and 30-day mortality. Intraoperative blood transfusions were required more often in the ADPKD (22.8%) compared with the control group (6.7%; p < 0.0001). Early postoperative urinary tract infections occurred more frequent (ADPKD 40.4%/control 29.0%; p = 0.0246). Time of surgery was prolonged by 30 min (ADPKD 169 min; 95%CI 159.8-175.6 min/control 139 min; 95%CI 131.4-145.0 min; p < 0.0001). One-year patient (ADPKD 96.4%/control 95.8%; p = 0.6537) and death-censored graft survival (ADPKD 94.8%/control 93.7%; p = 0.5479) were comparable between both groups. CONCLUSIONS With respect to morbidity and mortality, per protocol, simultaneous native nephrectomy is a safe procedure. Especially in asymptomatic ADPKD KTx recipients, the number of total operations can be reduced and residual diuresis preserved up until transplantation. In living donation, even preemptive transplantation is possible.
Collapse
Affiliation(s)
- Bernd Martin Jänigen
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany.
| | - Johann Hempel
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
| | - Philipp Holzner
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
| | - Johanna Schneider
- Faculty of Medicine Department of Medicine IV, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
| | - Stefan Fichtner-Feigl
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
| | - Oliver Thomusch
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
| | - Hannes Neeff
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
| | - Przemyslaw Pisarski
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
| | - Torben Glatz
- Faculty of Medicine, Department of General and Digestive Surgery, Section of Transplant Surgery, Medical Center, University of Freiburg, Freiburg im Breisgau, Germany
- Department of Surgery, Marien Hospital Herne, Ruhr-University Bochum, Herne, Germany
| |
Collapse
|
|
14
|
Anselmo A, Iaria G, Pellicciaro M, Sforza D, Parente A, Campisi A, Cacciatore C, Calafiore E, Pisani G, Tisone G. Native Nephrectomy in Patients With Autosomal Dominant Polycystic Kidney Disease Evaluated for Kidney Transplantation. Transplant Proc 2019; 51:2914-2916. [PMID: 31711576 DOI: 10.1016/j.transproceed.2019.08.010] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/17/2019] [Revised: 08/02/2019] [Accepted: 08/27/2019] [Indexed: 11/30/2022]
Abstract
Native nephrectomy (NN) in patients with autosomal dominant polycystic kidney disease (ADPKD) is indicated in cases of recurrent urinary tract infections and hematuria, neoplastic degeneration, and encumbrance. Timing, indication, and surgical approach of NN depends on the symptoms or policy of the center. The aim of our study is to evaluate our experience. In our retrospective study, we included 130 patients with a diagnosis of ADPKD from 530 patients evaluated for renal transplantation from 2011 to 2017. We analyzed the etiologic indication, the timing, and the complications of NN. In our cohort, 53 patients underwent open NN, 85% pre-kidney transplantation (KT), 13% post-KT, and only 1 case simultaneous with KT. In the pre-KT group, indications included: major indication was encumbrance in the. In the post-KT group, the major indication was infection followed by encumbrance, which developed after KT. Complications were: 3 cases of bleeding (1 required relaparotomy, 2 evolved into hematoma and radiological derange); 1 iatrogenic iliac artery injury, which was contextually repaired, and 5 cases of incisional hernia. At 35 ± 7.2 months follow-up, patients' survival was 96%; 1 patient died at the induction of anesthesia and 1 patient from sepsis after double NN and removal of nonfunctional transplanted kidney. NN is not without complications and should be performed when clearly indicated. In our experience, we preferred to perform NN before KT.
Collapse
Affiliation(s)
- Alessandro Anselmo
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Giuseppe Iaria
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Marco Pellicciaro
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy.
| | - Daniele Sforza
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Alessandro Parente
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Andrea Campisi
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Chiara Cacciatore
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Eleonora Calafiore
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Gennaro Pisani
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| | - Giuseppe Tisone
- Department of Hepatobiliary Surgery and Transplant Unit, Tor Vergata University of Rome, Italy
| |
Collapse
|
|
15
|
Maxeiner A, Bichmann A, Oberländer N, El-Bandar N, Sugünes N, Ralla B, Biernath N, Liefeldt L, Budde K, Giessing M, Schlomm T, Friedersdorff F. Native Nephrectomy before and after Renal Transplantation in Patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). J Clin Med 2019; 8:jcm8101622. [PMID: 31590248 PMCID: PMC6832478 DOI: 10.3390/jcm8101622] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/13/2019] [Revised: 09/25/2019] [Accepted: 09/27/2019] [Indexed: 01/28/2023] Open
Abstract
The aim of this study was 1) to evaluate and compare pre-, peri-, and post-operative data of Autosomal Dominant Polycystic Kidney Disease (ADPKD) patients undergoing native nephrectomy (NN) either before or after renal transplantation and 2) to identify advantages of optimal surgical timing, postoperative outcomes, and economical aspects in a tertiary transplant centre. This retrospective analysis included 121 patients divided into two groups-group 1: patients who underwent NN prior to receiving a kidney transplant (n = 89) and group 2: patients who underwent NN post-transplant (n = 32). Data analysis was performed according to demographic patient details, surgical indication, laboratory parameters, perioperative complications, underlying pathology, and associated mortality. There was no significant difference in patient demographics between the groups, however right-sided nephrectomy was performed predominantly within group 1. The main indication in both groups undergoing a nephrectomy was pain. Patients among group 2 had no postoperative kidney failure and a significantly shorter hospital stay. Higher rates of more severe complications were observed in group 1, even though this was not statistically significant. Even though the differences between both groups were substantial, the time of NN prior or post-transplant does not seem to affect short-term and long-term transplantation outcomes. Retroperitoneal NN remains a low risk treatment option in patients with symptomatic ADPKD and can be performed either pre- or post-kidney transplantation depending on patients' symptom severity.
Collapse
Affiliation(s)
- Andreas Maxeiner
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Anna Bichmann
- Department of Anesthesiology and Operative Intensive Care Medicine, Campus Charité Mitte, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Natalie Oberländer
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Nasrin El-Bandar
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Nesrin Sugünes
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Bernhard Ralla
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Nadine Biernath
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Lutz Liefeldt
- Department of Nephrology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Klemens Budde
- Department of Nephrology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Markus Giessing
- Department of Urology, Heinrich-Heine-University, 40225 Düsseldorf, Germany.
| | - Thorsten Schlomm
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| | - Frank Friedersdorff
- Department of Urology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Charitéplatz 1, 10117 Berlin, Germany.
| |
Collapse
|
|
16
|
[Arterial embolization of polycystic kidneys as an alternative to ergonomic nephrectomy in renal pre-transplantation. Monocentric retrospective study]. Prog Urol 2019; 29:482-489. [PMID: 31383509 DOI: 10.1016/j.purol.2019.07.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2017] [Revised: 06/03/2019] [Accepted: 07/02/2019] [Indexed: 11/21/2022]
Abstract
PURPOSE This study aims at providing a retrospective assessment of the decrease in renal volume after renal artery embolization (RAE) among a group of patients with autosomal dominant polycystic renal disease and for whom transplantation, for ergonomic reasons was temporarily advised against. MATERIAL AND METHODS Between November 2014 and March 2017, as part of pre-transplantory procedure 15 patients, including 11 men and 4 women benfited from renal embolization (RAE) in a context of preparation for transplant in Lille University Hospital. All of the patients were suffering from autosomal dominant polycystic renal disease (ADPKD) at a severe or terminal stage of renal disease. RESULTS The original mean total kidney volume (TKV) was 2550.6 cm3±1771 (1102 cm3; 7310 cm3), the average TKV at 3 months was 1684 cm3±1539 (648 cm3; 6930 cm3) with an average decrease of 33% in the volume (5.2%; 83.9%) (95% confidence interval [0.229-0.436]) (P<0.01) and 1632±1743 (599 cm3; 6758 cm3) at 6 months with an average decrease of 40.7% (7.6%; 64.1%) (IC95% 0.306-0.508) (P<0.01). Among the 15 patients, 13 had their contraindication removed and to date 7 have had successful transplants. One failure due to a very high initial volume (7310cm3) required a secondary nephrectomy. No post-embolization syndrome has been noticed, 2 minor complications occurred (13%) involving a difficult resumption of transit and there was one case of hematuria. CONCLUSION ERA procedures must have a place in the ergonomic transplant strategy of patients with ADPKD with a low complication rate and a high efficiency. Nephrectomy indications before transplantation must therefore be reconsidered due to a major postoperative risk. Our results are in line with larger scale studies. Nevertheless, these results need to be confirmed by a large-scale randomized prospective study. LEVEL OF EVIDENCE 4.
Collapse
|
|
17
|
Sedaia E, Esanu A, Ivanov V, Dumanschi C, Moiseeva A, Abraș M, Eugen C, Tatiana G. Catecholamine-induced cardiomyopathy in a patient with pheochromocytoma and polycystic kidney and liver disease: a case report. Eur Heart J Case Rep 2019; 3:5485824. [PMID: 31449620 PMCID: PMC6601177 DOI: 10.1093/ehjcr/ytz062] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/08/2018] [Accepted: 04/12/2019] [Indexed: 11/13/2022]
Abstract
Background Clinical manifestations of pheochromocytoma (PCC) frequently are not specific and can be attributed to other pathologies. The most dreaded manifestation is catecholamine-induced cardiomyopathy. A prompt diagnosis, sometimes extremely problematic due to associated conditions of the patient, is essential for clinical outcomes, because early resection of PCC may prevent progression to irreversible cardiac remodelling. Case summary We present a case of 47-year-old woman with suspected acute coronary syndrome but intact coronary vessels. Electrocardiogram examination showed ST depression suggestive for coronary ischaemia. Echocardiography revealed reduced ejection fraction of left ventricle and global hypokinesis. Abdominal ultrasound examination determined multiple cysts in liver and both kidneys. The patient had unclear transient states of sudden sweating, pale skin, nausea, and vomiting accompanied by hypertensive crisis. Fractioned urinary metanephrines were considerably increased. Contrasted computed tomography of abdominal cavity and pelvis revealed in both liver lobes many cysts; both kidneys showed multiple cysts too; in the right adrenal gland was detected a filling defect. Computed tomography findings have established diagnosis of adrenal PCC of right gland associated with liver and kidney polycystic disease. Discussion Phechromocytoma, with primary manifestation as catecholamine-induced cardiomyopathy, in patient with polycystic kidney and liver disease could represent a really challenging diagnosis. Clinical manifestations of PCC frequently are not specific and can be explained by associated pathologies. This is the second case of adrenalectomy due to PCC associated with polycystic kidney and liver disease reported in the medical literature.
Collapse
Affiliation(s)
- Ecaterina Sedaia
- Department of Internal Medicine, Cardiology, State University of Medicine and Pharmacy “Nicolae Testemitanu”, 165 Stefan cel Mare si Sfant, Bd., Chisinau MD-2004, Republic of Moldova
- Department of General Cardiology, Institute of Cardiology, 29/1 Testemitanu Str., Chisinau MD 2025, Republic of Moldova
| | - Andrei Esanu
- Department of Internal Medicine, Cardiology, State University of Medicine and Pharmacy “Nicolae Testemitanu”, 165 Stefan cel Mare si Sfant, Bd., Chisinau MD-2004, Republic of Moldova
- Department of General Cardiology, Institute of Cardiology, 29/1 Testemitanu Str., Chisinau MD 2025, Republic of Moldova
| | - Victoria Ivanov
- Department of Interventional Cardiology, Institute of Cardiology, 29/1 Testemitanu Str., Chisinau MD 2025, Republic of Moldova
| | - Carolina Dumanschi
- Department of Interventional Cardiology, Institute of Cardiology, 29/1 Testemitanu Str., Chisinau MD 2025, Republic of Moldova
| | - Ana Moiseeva
- Department of General Cardiology, Institute of Cardiology, 29/1 Testemitanu Str., Chisinau MD 2025, Republic of Moldova
| | - Marcel Abraș
- Department of Internal Medicine, Cardiology, State University of Medicine and Pharmacy “Nicolae Testemitanu”, 165 Stefan cel Mare si Sfant, Bd., Chisinau MD-2004, Republic of Moldova
- Department of Interventional Cardiology, Institute of Cardiology, 29/1 Testemitanu Str., Chisinau MD 2025, Republic of Moldova
| | - Calenici Eugen
- Department of Interventional Cardiology, Institute of Cardiology, 29/1 Testemitanu Str., Chisinau MD 2025, Republic of Moldova
| | - Globa Tatiana
- Department of Internal Medicine, Cardiology, State University of Medicine and Pharmacy “Nicolae Testemitanu”, 165 Stefan cel Mare si Sfant, Bd., Chisinau MD-2004, Republic of Moldova
| |
Collapse
|
|
18
|
Potts JW, Mousa SA. Recent advances in management of autosomal-dominant polycystic kidney disease. Am J Health Syst Pharm 2019; 74:1959-1968. [PMID: 29167138 DOI: 10.2146/ajhp160886] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
PURPOSE Promising developments in the search for effective pharmacotherapies for autosomal-dominant polycystic kidney disease (ADPKD) are reviewed. SUMMARY The formation and development of cysts characteristic of ADPKD result in inexorable renal and extrarenal manifestations that give rise to more rapid disease progression and more widespread complications than are seen with other forms of chronic kidney disease. To date, no agent has gained Food and Drug Administration marketing approval for use in patients with ADPKD, complicating efforts to meet the medical needs of this population. Although definitive ultrasonographic diagnostic strategies are available, molecular screening approaches lack sufficient evidence and patient outcomes data to support broad clinical application. Recently completed and ongoing clinical trials point to a number of encouraging platforms for evidence-based ADPKD management. Tolvaptan therapy significantly improved cyst burden and slowed disease progression among patients with early-stage ADPKD in a large-scale trial, while somatostatin therapies may also be useful in halting disease progression and managing comorbid polycystic liver disease. Stem cell research and nanomedicine might represent novel approaches to gaining comprehensive insights on ADPKD and, ultimately, to targeting the disease's origins, thereby making restoration of kidney function possible. CONCLUSION A number of pharmacotherapy approaches to ADPKD management show promise but are unlikely to be curative, fueling interest among researchers in finding new applications for nanomedicine and stem cell technologies that can slow ADPKD progression and better control complications of the disease.
Collapse
Affiliation(s)
- Jacob W Potts
- Pharmaceutical Research Institute, Albany College of Pharmacy and Health Sciences, Rensselaer, NY
| | - Shaker A Mousa
- Pharmaceutical Research Institute, Albany College of Pharmacy and Health Sciences, Rensselaer, NY
| |
Collapse
|
|
19
|
Freise J, Tavakol M, Gao Y, Klein O, Lee BK, Freise C, Park M. The Effect of Enlarged Kidneys on Calculated Body Mass Index Categorization in Transplant Recipients With ADPKD. Kidney Int Rep 2019; 4:606-609. [PMID: 30993236 PMCID: PMC6451113 DOI: 10.1016/j.ekir.2019.01.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2018] [Revised: 12/23/2018] [Accepted: 01/07/2019] [Indexed: 11/29/2022] Open
Affiliation(s)
- Jonathan Freise
- School of Medicine, University of California, San Francisco, San Francisco, California, USA
| | - Mehdi Tavakol
- Department of Surgery, University of California, San Francisco, San Francisco, California, USA
| | - Ying Gao
- Department of Medicine, University of California, San Francisco, San Francisco, California, USA
| | - Oana Klein
- Department of Medicine, University of California, San Francisco, San Francisco, California, USA
| | - Brian K Lee
- Department of Medicine, University of California, San Francisco, San Francisco, California, USA
| | - Chris Freise
- Department of Surgery, University of California, San Francisco, San Francisco, California, USA
| | - Meyeon Park
- Department of Medicine, University of California, San Francisco, San Francisco, California, USA
| |
Collapse
|
|
20
|
Ietto G, Raveglia V, Zani E, Iovino D, Parise C, Soldini G, Delfrate NW, Latham L, Saredi G, Benedetti F, Tozzi M, Carcano G. Pretransplant Nephrectomy for Large Polycystic Kidneys in ADPKD (Autosomal Dominant Polycystic Kidney Disease) Patients: Is Peritoneal Dialysis Recovery Possible after Surgery? BIOMED RESEARCH INTERNATIONAL 2019; 2019:7343182. [PMID: 31019972 PMCID: PMC6452549 DOI: 10.1155/2019/7343182] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/19/2018] [Revised: 09/06/2018] [Accepted: 12/05/2018] [Indexed: 11/22/2022]
Abstract
The choice of modality for renal replacement therapy in patients with ADPKD varies, often based on patient choice, physician-related factors, and resource availability. For a long time peritoneal dialysis (PD) was considered as relative contraindication due to the possible limited intraperitoneal space. In recent years, some studies suggested it is a valid option also in patients with ADPKD to be considered as a first line treatment in potentially fit patients. Diuresis volume lowering and potential permanent damage of peritoneal integrity, both leading to a necessary switch to haemodialysis, are the two most important dangers after nephrectomy, especially if bilateral, in PD patients. We performed a retrospective analysis of patient underwent native polycystic kidney nephrectomy in order to state the possibility to recover peritoneal dialysis after surgery.
Collapse
Affiliation(s)
- Giuseppe Ietto
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Veronica Raveglia
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Elia Zani
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Domenico Iovino
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Cristiano Parise
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Gabriele Soldini
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Nicholas Walter Delfrate
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Lorenzo Latham
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Giovanni Saredi
- Urology Department, Ospedale di Circolo e Fondazione Macchi, Varese, Italy
| | - Fabio Benedetti
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Matteo Tozzi
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| | - Giulio Carcano
- General, Emergency and Transplant Surgery Department, Ospedale di Circolo e Fondazione Macchi, University of Insubria, Varese, Italy
| |
Collapse
|
|
21
|
Abstract
The causes of macroscopic and microscopic hematuria overlap; both are often caused by urinary tract infections or urethral/bladder irritation. Coexistent hypertension and proteinuria should prompt investigation for glomerular disease. The most common glomerulonephritis in children is postinfectious glomerulonephritis. In most patients, and especially with isolated microscopic hematuria, the diagnostic workup reveals no clear underlying cause. In those cases whereby a diagnosis is made, the most common causes of persistent microscopic hematuria are thin basement membrane nephropathy, immunoglobulin A nephropathy, or idiopathic hypercalciuria. Treatment and long-term prognosis varies with the underlying disease.
Collapse
Affiliation(s)
- Denver D Brown
- Pediatric Nephrology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, 3415 Bainbridge Avenue, Bronx, NY 10467, USA
| | - Kimberly J Reidy
- Pediatric Nephrology, Children's Hospital at Montefiore, Albert Einstein College of Medicine, 3415 Bainbridge Avenue, Bronx, NY 10467, USA.
| |
Collapse
|
|
22
|
Malekshahabi T, Khoshdel Rad N, Serra AL, Moghadasali R. Autosomal dominant polycystic kidney disease: Disrupted pathways and potential therapeutic interventions. J Cell Physiol 2019; 234:12451-12470. [PMID: 30644092 DOI: 10.1002/jcp.28094] [Citation(s) in RCA: 29] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/24/2018] [Accepted: 12/07/2018] [Indexed: 12/18/2022]
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is a monogenic inherited renal cystic disease that occurs in different races worldwide. It is characterized by the development of a multitude of renal cysts, which leads to massive enlargement of the kidney and often to renal failure in adulthood. ADPKD is caused by a mutation in PKD1 or PKD2 genes encoding the proteins polycystin-1 and polycystin-2, respectively. Recent studies showed that cyst formation and growth result from deregulation of multiple cellular pathways like proliferation, apoptosis, metabolic processes, cell polarity, and immune defense. In ADPKD, intracellular cyclic adenosine monophosphate (cAMP) promotes cyst enlargement by stimulating cell proliferation and transepithelial fluid secretion. Several interventions affecting many of these defective signaling pathways have been effective in animal models and some are currently being tested in clinical trials. Moreover, the stem cell therapy can improve nephropathies and according to studies were done in this field, can be considered as a hopeful therapeutic approach in future for PKD. This study provides an in-depth review of the relevant molecular pathways associated with the pathogenesis of ADPKD and their implications in development of potential therapeutic strategies.
Collapse
Affiliation(s)
- Talieh Malekshahabi
- Department of Stem Cells and Developmental Biology, Cell Science Research Center, Royan Institute for Stem Cell Biology and Technology, ACECR, Tehran, Iran
| | - Niloofar Khoshdel Rad
- Department of Stem Cells and Developmental Biology, Cell Science Research Center, Royan Institute for Stem Cell Biology and Technology, ACECR, Tehran, Iran
| | - Andreas L Serra
- Department of Internal Medicine and Nephrology, Klinik Hirslanden, Zurich, Switzerland
| | - Reza Moghadasali
- Department of Stem Cells and Developmental Biology, Cell Science Research Center, Royan Institute for Stem Cell Biology and Technology, ACECR, Tehran, Iran
| |
Collapse
|
|
23
|
Soroka S, Alam A, Bevilacqua M, Girard LP, Komenda P, Loertscher R, McFarlane P, Pandeya S, Tam P, Bichet DG. Updated Canadian Expert Consensus on Assessing Risk of Disease Progression and Pharmacological Management of Autosomal Dominant Polycystic Kidney Disease. Can J Kidney Health Dis 2018; 5:2054358118801589. [PMID: 30345064 PMCID: PMC6187423 DOI: 10.1177/2054358118801589] [Citation(s) in RCA: 23] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/06/2018] [Accepted: 08/22/2018] [Indexed: 12/24/2022] Open
Abstract
PURPOSE The purpose of this article is to update the previously published consensus recommendations from March 2017 discussing the optimal management of adult patients with autosomal dominant polycystic kidney disease (ADPKD). This document focuses on recent developments in genetic testing, renal imaging, assessment of risk regarding disease progression, and pharmacological treatment options for ADPKD. SOURCES OF INFORMATION Published literature was searched in PubMed, the Cochrane Library, and Google Scholar to identify the latest evidence related to the treatment and management of ADPKD. METHODS All pertinent articles were reviewed by the authors to determine if a new recommendation was required, or if the previous recommendation needed updating. The consensus recommendations were developed by the authors based on discussion and review of the evidence. KEY FINDINGS The genetics of ADPKD are becoming more complex with the identification of new and rarer genetic variants such as GANAB. Magnetic resonance imaging (MRI) and computed tomography (CT) continue to be the main imaging modalities used to evaluate ADPKD. Total kidney volume (TKV) continues to be the most validated and most used measure to assess disease progression. Since the publication of the previous consensus recommendations, the use of the Mayo Clinic Classification for prognostication purposes has been validated in patients with class 1 ADPKD. Recent evidence supports the benefits of a low-osmolar diet and dietary sodium restriction in patients with ADPKD. Evidence from the Replicating Evidence of Preserved Renal Function: an Investigation of Tolvaptan Safety and Efficacy in ADPKD (REPRISE) trial supports the use of ADH (antidiuretic hormone) receptor antagonism in patients with ADPKD 18 to 55 years of age with eGFR (estimated glomerular filtration rate) of 25 to 65 mL/min/1.73 m2 or 56 to 65 years of age with eGFR of 25 to 44 mL/min/1.73 m2 with historical evidence of a decline in eGFR >2.0 mL/min/1.73 m2/year. LIMITATIONS Available literature was limited to English language publications and to publications indexed in PubMed, the Cochrane Library, and Google Scholar. IMPLICATIONS Advances in the assessment of the risk of disease progression include the validation of the Mayo Clinic Classification for patients with class 1 ADPKD. Advances in the pharmacological management of ADPKD include the expansion of the use of ADH receptor antagonism in patients 18 to 55 years of age with eGFR of 25 to 65 mL/min/1.73 m2 or 56 to 65 years of age with eGFR of 25 to 44 mL/min/1.73 m2 with historical evidence of a decline in eGFR >2.0 mL/min/1.73 m2/year, as per the results of the REPRISE study.
Collapse
Affiliation(s)
- Steven Soroka
- Division of Nephrology, Dalhousie University, Halifax, NS, Canada
| | - Ahsan Alam
- Division of Nephrology, Royal Victoria Hospital, McGill University, Montréal, QC, Canada
| | - Micheli Bevilacqua
- Division of Nephrology, The University of British Columbia, Vancouver, Canada
| | | | - Paul Komenda
- Division of Nephrology, Seven Oaks General Hospital, University of Manitoba, Winnipeg, Canada
| | - Rolf Loertscher
- Division of Nephrology, Lakeshore General Hospital, McGill University, Pointe-Claire, QC, Canada
| | - Philip McFarlane
- Division of Nephrology, St. Michael’s Hospital, University of Toronto, ON, Canada
| | - Sanjaya Pandeya
- Division of Nephrology, Halton Healthcare, Oakville, ON, Canada
| | - Paul Tam
- Division of Nephrology, Scarborough and Rouge Hospital, ON, Canada
| | - Daniel G. Bichet
- Division of Nephrology, Département de Médecine, Pharmacologie et Physiologie, Hôpital du Sacré-Cœur de Montréal, Université de Montréal, QC, Canada
| |
Collapse
|
|
24
|
Meng J, Xu Y, Li A, Fan S, Shen X, Ma D, Zhang L, Hao Z, Zhang X, Liang C. Clinical Features of 167 Inpatients with Autosomal Dominant Polycystic Kidney Disease at a Single Center in China. Med Sci Monit 2018; 24:6498-6505. [PMID: 30219820 PMCID: PMC6154125 DOI: 10.12659/msm.910127] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/22/2018] [Accepted: 06/01/2018] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The aim of this study was to describe the clinical characteristics of Chinese ADPKD inpatients and to identify the factors associated with disease severity. MATERIAL AND METHODS We included 167 hospitalized patients (inpatients) with ADPKD in this study. Multiple regression analyses were conducted to determine factors correlated with estimated glomerular filtration rate (eGFR). Patients were stratified into subgroups according to the presence of symptoms, in which clinical parameters were analyzed and compared. RESULTS The mean age of hospitalized ADPKD patients was 48.7 years old, lumbar and/or abdominal pain was seen in 40.12% of patients, following by nephrolithiasis (38.92%), hematuria (30.54%), and urinary tract infection (24.55%). Serum thrombocyte level and hemoglobin exhibited significant positive correlations with eGFR. Symptomatic patients accounted for 71.26% of the studied population. Patients with hypertension had increased risk of presence of symptoms (OR=2.794, 95%CI=1.341-5.822). Low thrombocyte and hemoglobin levels were observed in patients with hematuria. CONCLUSIONS Thrombocyte level was positively correlated with eGFR but was not associated with presence of PKD-related symptoms, suggesting thrombocyte level might be an independent serum biomarker for disease progression. Hypertension was associated with increased risk of symptom occurrence, indicating the relationship between hypertension and disease progression. This study reveals the clinical characteristics of inpatients with ADPKD in China and provides clinicians with useful insights into this intractable disease.
Collapse
|
|
25
|
Bhasin B, Alzubaidi M, Velez JCQ. Evaluation and Management of Gross Hematuria in Autosomal Dominant Polycystic Kidney Disease: A Point of Care Guide for Practicing Internists. Am J Med Sci 2018; 356:177-180. [DOI: 10.1016/j.amjms.2017.11.013] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2017] [Revised: 11/09/2017] [Accepted: 11/26/2017] [Indexed: 01/19/2023]
|
|
26
|
UPLC/MS-MS assay development for estimation of mozavaptan in plasma and its pharmacokinetic study in rats. Bioanalysis 2018; 10:1077-1086. [PMID: 29745750 DOI: 10.4155/bio-2018-0092] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
AIM Mozavaptan is a nonpeptide vasopressin receptor antagonist approved for the treatment of ectopic antidiuretic hormone secretion syndrome. METHODS & RESULTS A simple, rapid and fully validated UPLC/MS-MS method was developed for the quantitation of mozavaptan in rat plasma. The chromatographic separation was conducted on an Acquity UPLC BEH™ C18 column with an optimum mobile phase of 10 mM ammonium acetate buffer and 0.1% formic acid in acetonitrile (30:70 v/v) at a flow rate of 0.3 ml/min. The multiple reaction monitoring transitions were performed at m/z 428.16→119.03 for mozavaptan and m/z 237.06→179.10 for carbamazepine (internal standard). CONCLUSION The method was effectively applied for the determination of mozavaptan pharmacokinetic parameters after the oral administration of 3 mg/kg mozavaptan in rats.
Collapse
|
|
27
|
Primary Aldosteronism in a Patient with Autosomal Dominant Polycystic Kidney Disease Associated with Polycystic Liver Disease. ARS MEDICA TOMITANA 2018. [DOI: 10.2478/arsm-2018-0010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
Abstract
Polycystic kidney disease is an autosomal dominant genetic disorder (ADPKD) associated with arterial hypertension, as a common and early manifestation. However, the combination of hypertension and hypokalemia is very rare in these patients and may have another cause. We present a case of a 45 years old man with ADPKD associated with primary hyperaldosteronism. Unilateral suprarenal macroadenoma on abdominal CT scan, severe hypokalemia and low activity of plasmatic renin led to diagnosis.
Collapse
|
|
28
|
Montaña A, Patiño N, Larrate C, Zambrano FA, Martínez J, Lozano H, Lozano E. Actualización en enfermedad renal poliquística. REVISTA DE LA FACULTAD DE MEDICINA 2018. [DOI: 10.15446/revfacmed.v66n1.60760] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/01/2023] Open
Abstract
Introducción. La enfermedad renal poliquística (PKD, por su sigla en inglés) es una enfermedad genética frecuente en la que se desarrollan de forma progresiva lesiones quísticas que reemplazan el parénquima renal. Es una causa de insuficiencia renal terminal y una indicación común para diálisis y trasplante renal. Existen dos presentaciones de esta enfermedad que se distinguen por sus patrones de herencia: la enfermedad renal poliquística dominante (ADPKD, por su sigla en inglés) y la enfermedad renal poliquística recesiva (ARPKD, por su sigla en inglés).Objetivo. Resumir los aspectos más relevantes de la enfermedad renal: epidemiología, fisiopatología, diagnóstico, manifestaciones clínicas, tratamiento y pronóstico.Materiales y métodos. Revisión sistemática de la literatura en las bases de datos PubMed, Lilacs, UptoDate y Medline con los siguientes términos: enfermedades renales poliquísticas, riñón poliquístico autosómico dominante y riñón poliquístico autosómico recesivo.Resultados. Se encontraron 271 artículos y se escogieron 64 con base en su importancia.Conclusiones. Todo paciente con enfermedad renal poliquística en insuficiencia renal grado V debe ser estudiado para un trasplante renal; en la gran mayoría de los casos no se encontrará contraindicación para realizarlo.
Collapse
|
|
29
|
Jean RA, Alexandre M, Yoo PS. Kidney Transplantation With and Without Native Nephrectomy for Polycystic Kidney Disease: Results of the National Inpatient Sample and the Rationale for a 2-Staged Procedure. J Am Coll Surg 2017; 226:1079-1084. [PMID: 29224797 DOI: 10.1016/j.jamcollsurg.2017.11.021] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/02/2017] [Revised: 11/20/2017] [Accepted: 11/20/2017] [Indexed: 11/28/2022]
Abstract
BACKGROUND Polycystic kidney disease (PKD) is one of the most common causes of end-stage renal disease requiring hemodialysis or transplantation. In patients requiring transplantation, there are several indications for native nephrectomy, including recurrent cyst infection, bleeding, or to provide room for the graft. There is disagreement about whether it is advisable to perform kidney transplantation alone (KT), or to perform KT with simultaneous native nephrectomy (KTN). We compared postoperative outcomes of KTN and KT in a large national cohort. STUDY DESIGN The Nationwide Inpatient Sample (NIS) between 2000 and 2014 was examined for a diagnosis of PKD with evidence for KT or KTN. Logistic regression, adjusting for age, sex, comorbidity, and hospital region, was used to compare groups for the need for blood transfusion, need for critical care interventions, and development of postoperative complications. RESULTS A total of 4,003 hospitalizations were identified, which was representative of 19,302 weighted discharges nationally. In adjusted logistic regression models, KTN demonstrated significantly higher risk for blood transfusion (odds ratio [OR] 2.06; 95% CI 1.44 to 2.96; p < 0.0001), postoperative complications (OR 1.44; 95% CI 1.05 to 1.96; p = 0.02), and critical care interventions (OR 1.44; 95% CI 1.07 to 1.95; p = 0.02). Other significant predictors for blood transfusion included female sex (OR 1.76; 95% CI 1.45 to 2.13; p < 0.0001), age older than 61 years (OR 1.60; 95% CI 1.21 to 2.10; p = 0.001), and Charlson comorbidity score ≥2 (OR 1.52; 95% CI 1.10 to 2.09; p = 0.01). CONCLUSIONS Among patients with PKD, in comparison with KTN, KT alone represents a decreased risk for negative postoperative outcomes. A 2-staged procedure should be considered, when feasible, to minimize adverse patient outcomes.
Collapse
Affiliation(s)
- Raymond A Jean
- Department of Surgery, Yale School of Medicine, New Haven, CT; National Clinician Scholars Program, Department of Internal Medicine, Yale School of Medicine, New Haven, CT
| | | | - Peter S Yoo
- Section of Transplantation and Immunology, Department of Surgery, Yale School of Medicine, New Haven, CT.
| |
Collapse
|
|
30
|
Distinct oxylipin alterations in diverse models of cystic kidney diseases. Biochim Biophys Acta Mol Cell Biol Lipids 2017; 1862:1562-1574. [DOI: 10.1016/j.bbalip.2017.08.005] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2017] [Revised: 07/28/2017] [Accepted: 08/16/2017] [Indexed: 12/19/2022]
|
|
31
|
Dillman JR, Trout AT, Smith EA, Towbin AJ. Hereditary Renal Cystic Disorders: Imaging of the Kidneys and Beyond. Radiographics 2017; 37:924-946. [PMID: 28493804 DOI: 10.1148/rg.2017160148] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
The purpose of this article is to review the hereditary renal cystic diseases that can manifest in children and adults, with specific attention to pathogenesis and imaging features. Various common and uncommon hereditary renal cystic diseases are reviewed in terms of their underlying etiology, including the involved genetic mutations and the affected proteins and cellular structures. Focus is placed on the morphologic findings in each condition and the features that distinguish one disorder from another. The two most common categories of hereditary renal cystic disease are (a) the ciliopathic disorders, which are related to mutations affecting the primary cilia (called "ciliopathies"), and (b) the phakomatoses. Autosomal dominant polycystic kidney disease, autosomal recessive polycystic kidney disease, and the "medullary cystic disease complex" are all ciliopathies but have different phenotypes. Tuberous sclerosis complex and the associated "contiguous gene syndrome," as well as von Hippel-Lindau syndrome, are phakomatoses that can manifest with cystic renal lesions but have uniquely different extrarenal manifestations. Finally, DICER1 mutations can manifest with renal cystic lesions (typically, cystic nephromas) in patients predisposed to other malignancies in the chest, ovaries, and thyroid. Although some overlap exists in the appearance of the renal cysts associated with each of these diseases, there are clear morphologic differences (eg, cyst size, location, and complexity) that are emphasized in this review. To improve patient outcomes, it is important for the radiologist to recognize the various hereditary renal cystic diseases so that a correct diagnosis is assigned and so that the patient is adequately evaluated and followed up. ©RSNA, 2017.
Collapse
Affiliation(s)
- Jonathan R Dillman
- From the Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039 (J.R.D., A.T.T., A.J.T.); and the Section of Pediatric Radiology, Department of Radiology, University of Michigan Health System, Ann Arbor, Mich (E.A.S.)
| | - Andrew T Trout
- From the Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039 (J.R.D., A.T.T., A.J.T.); and the Section of Pediatric Radiology, Department of Radiology, University of Michigan Health System, Ann Arbor, Mich (E.A.S.)
| | - Ethan A Smith
- From the Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039 (J.R.D., A.T.T., A.J.T.); and the Section of Pediatric Radiology, Department of Radiology, University of Michigan Health System, Ann Arbor, Mich (E.A.S.)
| | - Alexander J Towbin
- From the Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039 (J.R.D., A.T.T., A.J.T.); and the Section of Pediatric Radiology, Department of Radiology, University of Michigan Health System, Ann Arbor, Mich (E.A.S.)
| |
Collapse
|
|
32
|
Wang R, Wang N, Tang J, Chen Y, Gao J. The safety and efficacy of MPR-CTU combined with precise intraoperative ultrasonography guided flexible ureteroscope in the treatment of renal cystic disease. Exp Ther Med 2017; 15:283-287. [PMID: 29375688 PMCID: PMC5763662 DOI: 10.3892/etm.2017.5379] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2017] [Accepted: 10/04/2017] [Indexed: 12/14/2022] Open
Abstract
The safety and efficacy of multi-planar reconstruction (MPR) image post-processing technique-computed tomography (CT) urography (CTU) combined with precise intraoperative ultrasonography guided flexible ureteroscope in renal cyst incision and drainage in the treatment of cystic diseases of kidney were evaluated. A total of 68 patients were randomly divided into control and observation group (n=34). All the patients were treated with renal cyst incision and drainage under flexible ureteroscope. The control group was under ultrasound guidance. The observation group was combined with MPR-CTU, the safety and efficacy was compared. There was no significant difference between the two groups in the success rate and the time of cyst treatment (P>0.05). The incidence of intraoperative and postoperative complications of the observation group was significantly lower than that of the control group. After 1 month follow-up, the total effective rate of the observation group was significantly higher than that of the control group, the difference was statistically significant (P<0.05). Conclusion MPR-CTU technique combined with intraoperative ultrasonography to guide cyst incision and drainage under flexible ureteroscope for renal cystic disease has a high safety and efficacy, and it is worthy of clinical application.
Collapse
Affiliation(s)
- Rongjiang Wang
- Department of Urology, The First Affiliated Hospital of Huzhou Teacher's College, Huzhou, Zhejiang 313000, P.R. China
| | - Ning Wang
- Department of Urology, The First Affiliated Hospital of Huzhou Teacher's College, Huzhou, Zhejiang 313000, P.R. China
| | - Jianer Tang
- Department of Urology, The First Affiliated Hospital of Huzhou Teacher's College, Huzhou, Zhejiang 313000, P.R. China
| | - Yu Chen
- Department of Urology, The First Affiliated Hospital of Huzhou Teacher's College, Huzhou, Zhejiang 313000, P.R. China
| | - Jianguo Gao
- Department of Urology, The First Affiliated Hospital of Huzhou Teacher's College, Huzhou, Zhejiang 313000, P.R. China
| |
Collapse
|
|
33
|
Argyrou C, Moris D, Vernadakis S. Tailoring the 'Perfect Fit' for Renal Transplant Recipients with End-stage Polycystic Kidney Disease: Indications and Timing of Native Nephrectomy. In Vivo 2017; 31:307-312. [PMID: 28438856 PMCID: PMC5461438 DOI: 10.21873/invivo.11060] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/15/2017] [Revised: 03/20/2017] [Accepted: 03/22/2017] [Indexed: 12/17/2022]
Abstract
BACKGROUND The ideal timing of native nephrectomy in relation to kidney transplantation in patients with autosomal-dominant polycystic kidney disease (ADPKD) can be a very puzzling decision for transplant surgeons and remains a matter of debate. This review article aims to present current literature regarding this highly controversial issue. MATERIALS AND METHODS The MEDLINE/PubMed database was searched using "polycystic kidney disease", "renal/kidney transplantation" and "native nephrectomy" as key words. Our search was focused on the optimal timing of and indications for native nephrectomy in renal transplant recipients with ADPKD. RESULTS In symptomatic cases, pre-transplant unilateral or bilateral native nephrectomy seems appropriate, in order to alleviate symptoms. In cases that are provided with the option of living-donor transplantation, the performance of the simultaneous procedure could be of benefit. When the principal indication of native nephrectomy is the creation of space for the renal allograft, various studies highlight the safety of the simultaneous approach of either unilateral or bilateral nephrectomy. CONCLUSION No consensus exists on the appropriate timing for native nephrectomy in patients with ADPKD. Several issues to be addressed in the decision-making process are the importance of residual diuresis, the longer operative time along with the associated prolonged ischemia time and higher complication rate of the combined procedure.
Collapse
Affiliation(s)
| | - Demetrios Moris
- Department of Surgery, The Ohio State University Comprehensive Cancer Center, The Ohio State University, Columbus, OH, U.S.A.
| | | |
Collapse
|
|
34
|
Soroka S, Alam A, Bevilacqua M, Girard LP, Komenda P, Loertscher R, McFarlane P, Pandeya S, Tam P, Bichet DG. Assessing Risk of Disease Progression and Pharmacological Management of Autosomal Dominant Polycystic Kidney Disease: A Canadian Expert Consensus. Can J Kidney Health Dis 2017; 4:2054358117695784. [PMID: 28321325 PMCID: PMC5347414 DOI: 10.1177/2054358117695784] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2016] [Accepted: 01/12/2017] [Indexed: 12/19/2022] Open
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder worldwide. The disease is characterized by renal cysts and progressive renal failure due to progressive enlargement of cysts and renal fibrosis. An estimated 45% to 70% of patients with ADPKD progress to end-stage renal disease by age 65 years. Although both targeted and nontargeted therapies have been tested in patients with ADPKD, tolvaptan is currently the only pharmacological therapy approved in Canada for the treatment of ADPKD. The purpose of this consensus recommendation is to develop an evidence-informed recommendation for the optimal management of adult patients with ADPKD. This document focuses on the role of genetic testing, the role of renal imaging, predicting the risk of disease progression, and pharmacological treatment options for ADPKD. These areas of focus were derived from 2 national surveys that were disseminated to nephrologists and patients with ADPKD with the aim of identifying unmet needs in the management of ADPKD in Canada. Specific recommendations are provided for the treatment of ADPKD with tolvaptan.
Collapse
Affiliation(s)
- Steven Soroka
- Division of Nephrology, Dalhousie University, Halifax, Nova Scotia, Canada
| | - Ahsan Alam
- Division of Nephrology, Royal Victoria Hospital, McGill University, Montreal, Québec, Canada
| | - Micheli Bevilacqua
- Division of Nephrology, St. Paul’s Hospital, University of British Columbia, Vancouver, Canada
| | - Louis-Philippe Girard
- Division of Nephrology, Foothills Medical Centre, University of Calgary, Alberta, Canada
| | - Paul Komenda
- Division of Nephrology, Seven Oaks General Hospital, University of Manitoba, Winnipeg, Canada
| | - Rolf Loertscher
- Division of Nephrology, Lakeshore General Hospital, McGill University, Pointe-Claire, Québec, Canada
| | - Philip McFarlane
- Division of Nephrology, St. Michael’s Hospital, University of Toronto, Ontario, Canada
| | - Sanjaya Pandeya
- Division of Nephrology, Halton Healthcare Services, Oakville, Ontario, Canada
| | - Paul Tam
- The Scarborough Hospital, Ontario, Canada
| | - Daniel G. Bichet
- Division of Nephrology, Département de Médecine et de Physiologie Moléculaire et Intégrative, Hôpital du Sacré-Cœur de Montréal, Université de Montréal, Québec, Canada
| |
Collapse
|
|
35
|
Rysz J, Gluba-Brzózka A, Franczyk B, Banach M, Bartnicki P. Combination drug versus monotherapy for the treatment of autosomal dominant polycystic kidney disease. Expert Opin Pharmacother 2016; 17:2049-56. [DOI: 10.1080/14656566.2016.1232394] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
|
|
36
|
Neuville M, Hustinx R, Jacques J, Krzesinski JM, Jouret F. Diagnostic Algorithm in the Management of Acute Febrile Abdomen in Patients with Autosomal Dominant Polycystic Kidney Disease. PLoS One 2016; 11:e0161277. [PMID: 27529555 PMCID: PMC4987061 DOI: 10.1371/journal.pone.0161277] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2016] [Accepted: 08/02/2016] [Indexed: 12/15/2022] Open
Abstract
Background Acute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Although criteria have been proposed for cyst infection (CyI) and hemorrhage (CyH), there is a lack of comparative assessments. Furthermore, distinguishing cystic from non-cystic complications remains problematic. Design ADPKD patients presenting with abdominal pain and/or fever between 01/2005 and 06/2015 were retrospectively identified in a systematic computerized billing database. CyH was defined as spontaneous intracystic density above 50 Hounsfield units on computed tomography (CT). CyI was definite if confirmed by cyst puncture, and probable if 4 criteria were met: 3-day fever, loin/liver tenderness, C-reactive protein (CRP) plasma levels >50mg/L and no CT evidence for CyH. Other episodes were grouped as inflammation of unknown origin (IUO). Results Among a cohort of 173 ADPKD patients, 101 presented with 205 episodes of abdominal pain (n = 172) and/or fever (n = 33). 20 patients experienced 30 CyH, whereas 16 presented 23 episodes of definite (n = 11) or probable (n = 12) CyI. 35 IUO were observed in 31 patients. Clinically, fever was observed in 7% vs. 100% vs. 66% of CyH, CyI and IUO, respectively. Biologically, CRP cut-off at 70 mg/dl showed 92% sensitivity and 81% specificity in CyI diagnosis. Urine or blood cultures remained sterile in >90% of CyH, but were contributive in 53.4% of CyI and IUO, with a 74.2% prevalence for E. coli. Radiologically, ultrasounds, CT and magnetic resonance diagnosed CyI in 2.6%, 20% and 16.7% of cases, respectively. 18F-FDG positron-emission tomography (PET)/CT was done within a median period of 7 days post antibiotics, and significantly changed patient management in 71.4%. Conclusions This retrospective single-center series underscores the usefulness of clinical–fever–and biological–CRP–parameters, but emphasizes the limitations of bacteriological and radiological investigations in cases of acute febrile abdomen in ADPKD patients. 18F-FDG-PET/CT imaging may be helpful in such condition.
Collapse
Affiliation(s)
- Marie Neuville
- Department of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium
| | - Roland Hustinx
- Department of Nuclear Medicine, University of Liège Hospital (ULg CHU), Liège, Belgium
| | - Jessica Jacques
- Department of Medico-Economic Information, University of Liège Hospital (ULg CHU), Liège, Belgium
| | - Jean-Marie Krzesinski
- Department of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium
- Groupe Interdisciplinaire de Génoprotéomique Appliquée (GIGA), Cardiovascular Sciences, University of Liège, Liège, Belgium
| | - François Jouret
- Department of Nephrology, University of Liège Hospital (ULg CHU), Liège, Belgium
- Groupe Interdisciplinaire de Génoprotéomique Appliquée (GIGA), Cardiovascular Sciences, University of Liège, Liège, Belgium
- * E-mail:
| |
Collapse
|
|
37
|
Simultaneous Native Nephrectomy and Kidney Transplantation in Patients With Autosomal Dominant Polycystic Kidney Disease. PLoS One 2016; 11:e0155481. [PMID: 27257690 PMCID: PMC4892472 DOI: 10.1371/journal.pone.0155481] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/26/2016] [Accepted: 04/29/2016] [Indexed: 12/17/2022] Open
Abstract
Introduction To evaluate the feasibility of simultaneous unilateral nephrectomy with kidney transplantation and to determine the effect of this procedure on perioperative morbidity and mortality and graft and patient survival. Methods Between January 2000 and May 2015, 145 patients with autosomal dominant polycystic kidney disease (ADPKD) underwent kidney transplantation. Of those, 40 (27.5%) underwent concurrent ipsilateral native nephrectomy (group NT). Patients in group NT were compared with patients with ADPKD not undergoing concurrent nephrectomy (group NT-) and asymptomatic patients undergoing pretransplant nephrectomy (group PNT). Results The average follow-up was 66 months. The graft survival rate at 1 and 5 years was 95% and 87.5% versus 93% and 76.2% in the NT and NT- groups, respectively (P = .903 and P = .544, respectively); 1-year patient survival was 100% for NT and 97% for NT- patients (P = .288), whereas 5-year patient survival was 100% and 92% for NT and NT- groups, respectively (P = .128). After propensity score matching (34 patients per group) no significant differences were observed in 1-year (97.1% in NT and 94.1%; P = 1) and 5-year (88.2% in NT and 91.2% in NT-; P = 1) graft survival, and in 1-year (100% for both groups; P = 1) and 5-year (100% in NT and 94.1% in NT-; P = 1) patient survival. Perioperative mortality was 0% among NT and 1.2% among NT- patients, whereas perioperative surgical complications were similar in both groups. One- and 5-year graft and patient survival were similar between the NT and PNT groups, but patients in the PNT group had significantly lower levels of hemoglobin and residual diuresis volumes at the time of transplant. Moreover, PNT patients had a longer pretransplant dialysis and a longer time on the waiting list. Conclusions Simultaneous unilateral nephrectomy does not have a negative effect on patient and graft survival in patients with ADPKD and is associated with low morbidity. Pretransplant nephrectomy should be restricted only to highly symptomatic patients, whereas unilateral nephrectomy in asymptomatic patients should be performed during kidney transplantation only if massive kidney size precludes graft positioning.
Collapse
|
|
38
|
Ramanathan S, Kumar D, Khanna M, Al Heidous M, Sheikh A, Virmani V, Palaniappan Y. Multi-modality imaging review of congenital abnormalities of kidney and upper urinary tract. World J Radiol 2016; 8:132-141. [PMID: 26981222 PMCID: PMC4770175 DOI: 10.4329/wjr.v8.i2.132] [Citation(s) in RCA: 47] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/26/2015] [Revised: 10/21/2015] [Accepted: 12/20/2015] [Indexed: 02/06/2023] Open
Abstract
Congenital abnormalities of the kidney and urinary tract (CAKUT) include a wide range of abnormalities ranging from asymptomatic ectopic kidneys to life threatening renal agenesis (bilateral). Many of them are detected in the antenatal or immediate postnatal with a significant proportion identified in the adult population with varying degree of severity. CAKUT can be classified on embryological basis in to abnormalities in the renal parenchymal development, aberrant embryonic migration and abnormalities of the collecting system. Renal parenchymal abnormalities include multi cystic dysplastic kidneys, renal hypoplasia, number (agenesis or supernumerary), shape and cystic renal diseases. Aberrant embryonic migration encompasses abnormal location and fusion anomalies. Collecting system abnormalities include duplex kidneys and Pelvi ureteric junction obstruction. Ultrasonography (US) is typically the first imaging performed as it is easily available, non-invasive and radiation free used both antenatally and postnatally. Computed tomography (CT) and magnetic resonance imaging (MRI) are useful to confirm the ultrasound detected abnormality, detection of complex malformations, demonstration of collecting system and vascular anatomy and more importantly for early detection of complications like renal calculi, infection and malignancies. As CAKUT are one of the leading causes of end stage renal disease, it is important for the radiologists to be familiar with the varying imaging appearances of CAKUT on US, CT and MRI, thereby helping in prompt diagnosis and optimal management.
Collapse
|