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Curr Opin Nephrol Hypertens 2019 Mar;28(2):183-194

Division of Nephrology, Department of Internal Medicine.

Purpose Of Review: The purpose of this review is to emphasize that single gene disorders are an important and sometimes unrecognized cause of progressive chronic kidney disease. We provide an overview of the benefits of making a genetic diagnosis, the currently available genetic testing methods and examples of diseases illustrating the impact of a genetic diagnosis.

Recent Findings: Although there are now a number of monogenic renal diseases, only a few, such as autosomal dominant polycystic kidney disease (ADPKD), are generally diagnosable without genetic testing. Read More

Radiology 2019 Mar 1;290(3):769-782. Epub 2019 Jan 1.

From the Department of General Pediatrics, Adolescent Medicine and Neonatology, Center for Pediatrics, Medical Center-University of Freiburg, Mathildenstr 1, 79106 Freiburg, Germany (C.G.); Department of Pediatric Radiology, Jeanne de Flandre Mother and Child Hospital, University of Lille, Lille, France (E.F.A.); Department of Pediatric Radiology, University Hospital of Leuven, Leuven, Belgium (L.B.); Department of Pediatrics, University Hospital of Cologne, Cologne, Germany (K.B.); Department of Bioengineering, IRCCS Mario Negri Institute for Pharmacological Research, Bergamo, Italy (A.C.); Department of Pediatrics II, University Hospital Essen, Essen, Germany (M.C.); Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany (D.H., D.F., L.P.); Division of Nephrology, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pa (E.A.H.); Department of General Pediatrics, University Children's Hospital, Münster, Germany (J.K., A.T.); Department of Pediatrics and Center for Molecular Medicine, University Hospital of Cologne, Cologne, Germany (M.C.L.); Department of Pediatric Nephrology, University Hospital of Leuven, Leuven, Belgium (D.M.); PKD Research Group, Laboratory of Pediatrics, Department of Development and Regeneration, GPURE, KU Leuven, Leuven, Belgium (D.M.); PKD Research Group, Department of Development and Regeneration, Catholic University Leuven (KU Leuven), Leuven, Belgium (D.M.); Academic Nephrology Unit, Department of Infection Immunity & Cardiovascular Disease, University of Sheffield, Sheffield, England (A.C.M.O.); Department of Nephrology, Fundació Puigvert, Autonomous University of Barcelona, IIB Sant Pau, REDINREN, Barcelona, Spain (R.T.); University College London Great Ormond Street, Institute of Child Health, London, England (P.J.D.W.); and Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany (F.S.).

Kidney cysts can manifest as focal disease (simple and complex kidney cysts), affect a whole kidney (eg, multicystic dysplastic kidney or cystic dysplasia), or manifest as bilateral cystic disease (eg, autosomal recessive polycystic kidney disease [ARPKD] or autosomal dominant polycystic kidney disease [ADPKD]). In children, as opposed to adults, a larger proportion of kidney cysts are due to genetic diseases (eg, HNF1B nephropathy, various ciliopathies, and tuberous sclerosis complex), and fewer patients have simple cysts or acquired cystic kidney disease. The purpose of this consensus statement is to provide clinical guidance on standardization of imaging tests to evaluate kidney cysts in children. Read More

JFMS Open Rep 2018 Jul-Dec;4(2):2055116918817631. Epub 2018 Dec 16.

Foundation Hospital Veterinary Clinic, Autonomous Foundation Hospital of Barcelona, Barcelona, Spain.

Case Summary: Congenital or acquired hepatic cystic lesions in cats are a rare condition. Congenital hepatic cysts are often present as part of a systemic polycystic disease involving several organs. Most cats with hepatic cysts remain clinically normal for their lives, although some patients may show abdominal distension, vomiting, abdominal pain and jaundice. Read More

Am J Surg Pathol 2019 Feb;43(2):277-287

Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN.

The aim of this work was to study small neoplasms of the epithelium of the renal tubules; kidneys from 402 unselected autopsies were sectioned at 1 to 2 mm intervals. All lesions were examined histologically. A total of 232 papillary adenomas were found in 76 patients (19%), ranging from 1 to 35 adenomas/patient (mean: 3, median: 2). Read More

Jpn J Radiol 2019 Feb 30;37(2):165-177. Epub 2018 Oct 30.

Department of Radiology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo, 663-8501, Japan.

Purpose: To evaluate renal cell carcinoma (RCC) findings in acquired cystic disease of the kidney (ACDK) shown by C-choline and FDG PET/CT, and contrast-enhanced CT.

Materials And Methods: Six ACDK patients with 7 RCCs underwent C-choline and FDG PET/CT, and contrast-enhanced CT before nephrectomy. Findings obtained with 3 imagings were evaluated and sensitivity detecting RCC was compared using 3-point grading scale (negative, equivocal, positive). Read More

J Nephrol 2018 Dec 6;31(6):863-879. Epub 2018 Sep 6.

Sant'Anna School of Advanced Studies, Department of Internal Medicine, University of Pisa, Pisa, Italy.

Chronic kidney disease (CKD) includes all clinical features and complications during the progression of various kidney conditions towards end-stage renal disease (ESRD). These conditions include immune and inflammatory disease such as: primary and hepatitis C virus (HCV)-related glomerulonephritis; infectious disease such as pyelonephritis with or without reflux and tuberculosis; vascular disease such as chronic ischemic nephropathy; hereditary and congenital disease such as polycystic disease and congenital cystic dysplasia; metabolic disease including diabetes and hyperuricemia; and systemic disease (collagen disease, vasculitis, myeloma). During the progression of CKD, ultrasound imaging and color Doppler imaging (US-CDI) can differentiate the etiology of the renal damage in only 50-70% of cases. Read More

Pathol Int 2018 Oct 6;68(10):543-549. Epub 2018 Sep 6.

Department of Pathology, Aichi Medical University, Aichi, Japan.

New pathological subtypes of renal cell carcinoma (RCC) were designated in the 2016 World Health Organization (WHO) classification corresponding to the features commonly seen in patients with end-stage renal disease (ESRD). To determine the clinicopathological findings of new subtypes, we reanalyzed all sections from 315 kidneys in 291 ESRD patients bearing RCC tumors surgically resected in three Japanese institutes by the central pathologist. Clear cell RCC was diagnosed in 144 kidneys (45. Read More

Nephrology (Carlton) 2018 07;23(7):704-705

School of Medicine, The University of Sydney, Camperdown, New South Wales, Australia.

Int J Urol 2018 09 31;25(9):780-786. Epub 2018 Jul 31.

Department of Surgical Pathology, Aichi Medical University Hospital, Nagakute, Aichi, Japan.

The incidence of end-stage renal disease has increased owing to the greater prevalence of patients with chronic kidney disease and diabetes mellitus. End-stage renal disease is usually accompanied by acquired cystic disease and is a risk factor for renal cell carcinoma. The present review discusses the etiology of renal cell carcinoma in end-stage renal disease patients, focusing on two unique renal cell carcinoma histological subtypes: acquired cystic disease-associated renal cell carcinoma and clear cell papillary renal cell carcinoma. Read More

Saudi J Kidney Dis Transpl 2018 May-Jun;29(3):732-734

Department of Nephrology, Salisbury VA Health Care System, Salisbury, NC, USA.

Immunosuppression is a well-known risk factor for malignancy. Renal transplant patients are at high risk for cancer in the native kidneys especially in the presence of acquired cystic disease. We report a case highlighting the importance of screening for renal malignancy in renal transplant patients. Read More

Indian J Surg Oncol 2018 Jun 23;9(2):247-250. Epub 2015 Jul 23.

Sir GangaRam Hospital, Rajinder Nagar, New Delhi, 110060 India.

Immunomarker α-methylacyl-coenzyme A racemase (AMACR), a protein involved in the metabolism of branched chain fatty acids was initially recognized in the evaluation of prostate cancer. AMACR is also a fairly sensitive marker for papillary renal cell carcinoma. Papillary adenoma associated with papillary renal cell carcinoma are AMACR positive and both represent a continuum of the same biological process. Read More

Am J Surg Pathol 2018 Sep;42(9):1156-1165

Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic.

The incidence of renal cell carcinoma (RCC) is known to be higher in patients with end-stage renal disease, including those with acquired cystic kidney disease due to dialysis. Acquired cystic disease (ACD)-associated RCC was recently incorporated into the 2016 WHO Classification of Tumors of the Urinary System and Male Genital Tract as a distinct entity and is reportedly the most common RCC arising in end-stage renal disease. In this study, we sought to further describe clinicopathologic findings in a large series of ACD-RCC, emphasizing histologic features, immunophenotype, clinical outcome, and patterns of disease spread. Read More

Zhonghua Bing Li Xue Za Zhi 2018 May;47(5):366-371

Department of Pathology, 401 Hospital of PLA, Qingdao 266071, China.

To study the clinicopathologic, immunohistochemical (IHC), histogenetic and prognostic features of acquired cystic kidney disease-associated renal cell carcinoma (ACKD-RCC). Three cases of ACKD-RCC, including two from 401 Hospital of PLA and one from the Affiliated Hospital of Qingdao University were studied by clinical, histological and IHC analysis with review of relevant literature. All the three patients were male, ranging from 46 to 78 years old. Read More

Am J Emerg Med 2019 Jan 21;37(1):45-47. Epub 2018 Apr 21.

Department of Urology, Korea University College of Medicine, Seoul, Republic of Korea. Electronic address:

Introduction: This study aimed to analyze the characteristics, etiology, and treatment of a series of patients with spontaneous perirenal hemorrhage (Wunderlich syndrome [WS]).

Methods: We retrospectively reviewed the records of 26 patients hospitalized for WS in a tertiary urological center between 2011 and 2018. All patients were evaluated for perirenal hemorrhage observed on computed tomography (CT) in the emergency department. Read More

J Pediatr 2018 08 9;199:22-28.e6. Epub 2018 May 9.

Department of Pediatrics, University Hospital of Cologne, Cologne, Germany; Center for Molecular Medicine, University Hospital of Cologne, Cologne, Germany.

Objective: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis.

Study Design: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life.

Results: Thirty-six out of 385 children (9. Read More

Kidney Int 2018 05 22;93(5):1052-1059. Epub 2018 Feb 22.

Emory University, Department of Medicine, Renal Division, Atlanta, Georgia, USA. Electronic address:

Sonography is increasingly being performed by clinicians and has applications throughout the spectrum of nephrology, including acute and chronic renal failure, urinary obstruction, cystic disease, pain, hematuria, transplantation, kidney biopsy, temporary and permanent vascular access, and assessment of fluid status. The skill is relatively easily acquired, expedites patient care, and enhances the practice of nephrology. However, the lack of exposure in most training programs remains a major obstacle. Read More

Tunis Med 2017 Feb;95(2):156-157

Acad Radiol 2018 07 10;25(7):850-855. Epub 2018 Jan 10.

Department of Electrical, Electronic, and Information Engineering "Guglielmo Marconi", University of Bologna, Bologna, Italy, Via Venezia 52, 47521 Cesena, Italy. Electronic address:

Rationale And Objectives: Total kidney volume is an important biomarker for the evaluation of autosomal dominant polycystic kidney disease progression. In this study, we present a novel approach for automated segmentation of polycystic kidneys from non-contrast-enhanced computed tomography (CT) images.

Materials And Methods: Non-contrast-enhanced CT images were acquired from 21 patients with a diagnosis of autosomal dominant polycystic kidney disease. Read More

Hemodial Int 2018 04 20;22(2):222-227. Epub 2017 Nov 20.

International Renal Research Institute of Vicenza (IRRIV), Vicenza, Italy.

Introduction: Spontaneous perirenal hemorrhage (SPH) or Wunderlich syndrome, is a rare but potentially life-threatening condition. It is characterized by an unexpected bleeding in the kidneys and usually presents as an abdominal pain. Angiography and more recently selective renal arterial embolization are emerging as effective modalities for the diagnosis and treatment of SPH. Read More

Acta Med Croatica 2016 12;70(4-5):295-300

Cystic kidney diseases is a heterogeneous group of developmental, hereditary and acquired disorders that result in the occurrence of one or more cysts on one or both kidneys, and are relatively common. Cystic kidneys can also occur as one of various syndromes of malformations. Kidney cysts can have several classifications, depending on the properties taken in consideration. Read More

J Bras Nefrol 2017 Jul-Sep;39(3):329-332

Universidade Federal de São Paulo.

Acquired Cystic Kidney Disease (ACKD) is regarded as a common late condition of end stage renal damage and expresses its most important features when associated with long term hemodialysis. ACKD is also widely known as a premalignant lesion. Its occurrence in chronically rejected renal allografts is rare and its frequency and behavior in this setting are not well known. Read More

Transplant Proc 2017 Oct;49(8):1783-1785

Department of Anatomic Pathology, School of Medical Sciences, State University of Campinas (Unicamp), Campinas, Brazil.

Background: The aim of this study was to revise the histopathologic types of neoplasias in the genitourinary tract and determine the frequency of 2 new entities included in the 2016 book of World Health Organization classification of renal tumors. It is not established so far whether these 2 recently described tumors are the most frequent in association with end-stage kidney disease.

Methods: In a retrospective analysis, we revised the histopathologic type of 37 genitourinary tumors from 21 patients in dialysis and/or submitted to renal transplantation from 2003 to 2016 aiming to find the frequency of acquired cystic disease-associated renal cell carcinoma and clear cell papillary (tubulopapillary) renal cell carcinoma. Read More

Arch Esp Urol 2017 Jul;70(6):612-617

Servicio de Anatomía Patológica. Hospital Universitario Virgen Macarena,Sevilla. España.

Objetive: We report a case of acquired renal cystic disease associated with renal dialysis and endstage renal disease. The patient suffered the two major complications related with acquired renal cystic disease; hemorrhage and renal carcinoma.

Methods: Our case is a patient with acquired renal cystic disease, single kidney after surgery for renal clear cell carcinoma four years earlier, who developed a Wünderlich syndrome (WS). Read More

BMJ Case Rep 2017 Jun 16;2017. Epub 2017 Jun 16.

Transplant Surgery, MedStar Washington Hospital Center, Washington, District of Columbia, USA.

Capillary haemangiomas are relatively common tumours, typically occurring in the subcutaneous tissue during childhood. However, visceral occurrence is very rare. These tumours make up a subset of vascular lesions that have previously, although rarely, been described in case reports in association with the kidney. Read More

Semin Dial 2017 07 21;30(4):373-379. Epub 2017 May 21.

Renal Division, Department of Medicine, Emory University School of Medicine, Atlanta, Georgia.

The incidence of renal malignancies is markedly increased in end-stage renal disease and appears to be related to the development of acquired cystic kidney disease. Identification and evaluation of simple cysts, complex cysts, and solid masses in these patients rely on imaging studies, including ultrasonography, computed tomography, and magnetic resonance imaging, each of which has advantages and disadvantages. While there are no published data to support screening of ESRD patients, this seems appropriate in at least some patients, based on patient characteristics and the presence or absence of acquired cystic kidney disease. Read More

J Diabetes Complications 2017 Jul 15;31(7):1243-1246. Epub 2016 Nov 15.

Division of Nephrology, West China Hospital of Sichuan University, Chengdu 610041, Sichuan, China. Electronic address:

Aims: Precise diagnosis of maturity-onset diabetes of the young (MODY) has proven valuable for understanding mechanism of diabetes and selecting optimal therapy. A proband and her mother with diabetic kidney disease (DKD) were studied to investigate potential genes responsible for diabetes and different severity of DKD between the parent and offspring.

Methods: The family with suspected MODY underwent mutational analyses by the whole exome sequencing (WES). Read More

Pediatr Nephrol 2018 Jan 25;33(1):41-51. Epub 2017 Apr 25.

Children's Mercy Hospital, 2401 Gillham Road, Kansas City, MO, 64108, USA.

Acquired cystic kidney disease (ACKD) is a condition that occurs predominantly in patients with end-stage renal disease (ESRD). In contrast to hereditary cystic kidney disease, ACKD is characterized by the presence of multiple small cysts in bilaterally small kidneys. Limited pediatric data suggest a high incidence (21. Read More

Arch Pathol Lab Med 2017 Apr;141(4):600-606

From the Department of Pathology, University of Texas Medical Branch, Galveston.

Acquired cystic disease-associated renal cell carcinoma (ACD-RCC) is a recently described subtype of RCC found in individuals with ACD of the kidney. Because of underrecognition, information regarding this lesion is sparse but continues to accumulate with each new report. Herein, a thorough literature review amassing the current understanding of this unique neoplasm is presented. Read More

J Investig Med 2017 04 8;65(4):784-786. Epub 2017 Feb 8.

Department of Internal Medicine, Antalya Training and Research Hospital, Antalya, Turkey.

CD200 is a novel immune-effective molecule, existing in a cell membrane-bound form, as well as in a soluble form in serum, which performs to modulate inflammatory and acquired immune responses. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of large renal cysts and progressive loss of renal function. As defects in cell cycle arrest and apoptosis of renal tubular epithelial cells occur in ADPKD, we asked whether serum soluble CD200 might underlie and effect on ADPKD. Read More

Pol J Pathol 2017;68(4):306-311

The disease entity of acquired cystic disease (ACD)-associated renal cell carcinoma (RCC) has been recently incorporated into the international renal tumor classification. However, there are a few descriptions on clinicopathologic features. We performed a clinicopathologic study of seven cases with ACD-RCC. Read More

Pathol Oncol Res 2017 Jul 28;23(3):689-698. Epub 2016 Dec 28.

Department of Pathology, University of Szeged, Állomás Street 1, Szeged, H-6725, Hungary.

The morphotype and grade of renal cell carcinoma (RCC) in 928 nephrectomies were reclassified according to the 2016 WHO classification in order to analyze the distribution and outcomes of RCC subtypes in Hungary, to assess whether microscopic tumor necrosis is an independent prognostic factor in clear cell RCC, and to study whether a two-tiered grading (low/high) for clear cell and papillary RCC provides similar prognostic information to that of the four-tiered ISUP grading system. 83.4% of the cohort were clear cell, 6. Read More

Ann Transplant 2016 Dec 6;21:745-754. Epub 2016 Dec 6.

Department of Diagnostics and Public Health, University and Hospital Trust of Verona, Verona, Italy.

BACKGROUND De novo renal neoplasia developing after kidney transplantation at Verona Kidney Transplant Center were reviewed according to new 2016 WHO Renal Tumor Classification. MATERIAL AND METHODS Primary renal tumors developed in native or transplanted kidneys de novo following renal transplantation were retrieved and histologically reviewed by three expert uropathologists. Immunoexpression of the diagnostic antigens CD13, CD10, CK7, CK34bE12, AMACR, CAIX, AE1/AE3, CK14, GATA-3, HMB-45, cathepsin-k, S100A1, and parvalbumin was assessed. Read More

Indian J Pathol Microbiol 2016 Oct-Dec;59(4):524-526

Department of Pathology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India.

Xanthogranulomatous pyelonephritis is an uncommon inflammatory condition accounting for 1% of chronic pyelonephritis cases. Clinically and radiologically it mimics other renal space occupying lesions. Hence, correct preoperative diagnosis is not possible in all cases and nephrectomy is done in most patients. Read More

Arch Pathol Lab Med 2016 Oct;140(10):1026-37

From the Department of Pathology (Drs Udager and Mehra) and the Comprehensive Cancer Center (Dr Mehra), University of Michigan Health System, Ann Arbor; and the Michigan Center for Translational Pathology, Ann Arbor (Dr Mehra).

Molecular and morphologic interrogation has driven a much-needed reexamination of renal cell carcinoma (RCC). Indeed, the recently released 2016 World Health Organization classification now recognizes 12 distinct RCC subtypes, as well as several other emerging/provisional RCC entities. From a clinical perspective, accurate RCC classification may have important implications for patients and their families, including prognostic risk stratification, targeted therapeutics selection, and identification for genetic testing. Read More

Hinyokika Kiyo 2016 Jul;62(7):349-53

The Department of Urology, Nishi-Kobe Medical Center.

A 59-year-old man who had undergone hemodialysis for 13 years was diagnosed with left renal cell carcinoma (RCC),cT1aN0M0,in 2010. He had a laparoscopic left nephrectomy,and the pathological diagnosis at that time was clear cell carcinoma,pT1a (multifocal). At 1 year after surgery,he was diagnosed with a left renal hilar lymph node metastasis and a new right RCC,cT1aN0M0. Read More

Contrib Nephrol 2016 12;188:131-43. Epub 2016 May 12.

Acquired cystic kidney disease (ACKD) and renal cell carcinoma (RCC) are the most important manifestations of end-stage kidneys' structural changes. ACKD is caused by kidney damage or scarring and it is characterized by the presence of small, multiple cortical and medullary cysts filled with a fluid similar to preurine. ACKD prevalence varies according to predialysis and dialysis age and its pathogenesis is unknown, although it is stated that progressive destruction of renal tissue induces hypertrophy/compensatory hyperplasia of residual nephrons and may trigger the degenerative process. Read More

Contrib Nephrol 2016 12;188:120-30. Epub 2016 May 12.

Cysts are frequently found in chronic kidney disease (CKD) and they have a different prognostic significance depending on the clinical context. Simple solitary parenchymal cysts and peripelvic cysts are very common and they have no clinical significance. At US, simple cyst appears as a round anechoic pouch with regular and thin profiles. Read More

Nephrology (Carlton) 2017 Jan;22(1):95

Pediatric Surgery Unit, Sapienza University of Rome, Rome, Italy.

EBioMedicine 2016 Mar 26;5:183-92. Epub 2016 Jan 26.

National Institute of Diabetes and Digestive and Kidney Disease, National Institutes of Health, Bethesda, MD, USA.

Background: The major gene mutated in autosomal dominant polycystic kidney disease was first identified over 20 years ago, yet its function remains poorly understood. We have used a systems-based approach to examine the effects of acquired loss of Pkd1 in adult mouse kidney as it transitions from normal to cystic state.

Methods: We performed transcriptional profiling of a large set of male and female kidneys, along with metabolomics and lipidomics analyses of a subset of male kidneys. Read More

Int J Nephrol Renovasc Dis 2016 7;9:45-52. Epub 2016 Mar 7.

Department of Surgery, Section of Urology, Augusta University, Augusta, GA, USA.

This review provides an overview of the incidence of renal cell carcinoma (RCC) and a summary of the most commonly associated risk factors. A literature review was performed with a focus on recent studies with a high level of evidence (large prospective cohort studies and meta-analyses). The incidence rate of RCC varies globally, with the rate rising rapidly in more developed regions, demonstrating the effects of increased use of diagnostic imaging and prevalence of modifiable risk factors. Read More

Int J Urol 2016 06 28;23(6):465-71. Epub 2016 Mar 28.

Department of Urology, Institute of Wonkwang Medical Science, Wonkwang University School of Medicine, Iksan, Korea.

Objectives: To describe the clinical features of renal cell carcinoma arising in end-stage renal disease and to compare survival outcomes after definitive treatment with non-end-stage renal disease renal cell carcinoma.

Methods: Data of 181 consecutive patients with end-stage renal disease renal cell carcinoma who had received surgical treatment between 1995 and 2011 at seven institutions were reviewed. Data of 362 non-end-stage renal disease renal cell carcinoma patients matched for clinicopathological parameters who received surgery at Asan Medical Center during the same study period were also reviewed. Read More

Am J Kidney Dis 2016 Jul 22;68(1):134-7. Epub 2016 Mar 22.

Department of Radiology, The Children's Hospital of Philadelphia, Philadelphia, PA.

Cystic kidney disease includes a wide range of hereditary, developmental, and acquired conditions of the kidneys. Some of the inherited causes of cystic kidney disease include autosomal dominant polycystic kidney diseases (caused by mutations in PKD1 or PKD2), autosomal recessive polycystic kidney disease, tuberous sclerosis complex, von Hippel-Lindau disease, oral-facial-digital syndrome type I, and Hadju-Cheney syndrome. Acquired cystic kidney disease has been reported in patients receiving long-term hemodialysis or peritoneal dialysis and in children after liver transplantation. Read More

Nephrology (Carlton) 2017 May;22(5):361-365

Izmir Bozyaka Education and Research Hospital, Department of General Surgery, Izmir, Turkey.

In patients with a solitary kidney; residual renal volume is an important prognostic factor for kidney survival. At present, the impact of renal cysts on solitary kidney survival is not clear. The aim of this study is to examine the association of cysts on progression of renal failure in patients with a solitary kidney. Read More

Eur Urol 2016 07 28;70(1):93-105. Epub 2016 Feb 28.

Department of Pathology and Laboratory Medicine, Indiana University Health Partners, Indiana University School of Medicine, Indianapolis, IN, USA.

Unlabelled: The fourth edition of the World Health Organization (WHO) classification of urogenital tumours (WHO "blue book"), published in 2016, contains significant revisions. These revisions were performed after consideration by a large international group of pathologists with special expertise in this area. A subgroup of these persons met at the WHO Consensus Conference in Zurich, Switzerland, in 2015 to finalize the revisions. Read More

Pediatr Blood Cancer 2016 07 29;63(7):1272-5. Epub 2016 Feb 29.

Department of Medical Genetics, Lady Davis Institute, Jewish General Hospital, McGill University, Montreal, Quebec, Canada.

Anaplastic sarcoma of kidney (ASK) is a rare neoplasm recently associated with DICER1 mutations. We report a child with germline DICER1 mutation who developed ASK in preexisting septated renal cysts, which were likely cystic nephroma. From age 2. Read More

Nat Rev Nephrol 2016 May 25;12(5):267-80. Epub 2016 Jan 25.

Clinical Genetics Unit, Department of Woman and Child Health, University of Padova, Via Giustiniani 3, 35128, Padova, Italy.

Mitochondria are increasingly recognized as key players in genetic and acquired renal diseases. Most mitochondrial cytopathies that cause renal symptoms are characterized by tubular defects, but glomerular, tubulointerstitial and cystic diseases have also been described. For example, defects in coenzyme Q10 (CoQ10) biosynthesis and the mitochondrial DNA 3243 A>G mutation are important causes of focal segmental glomerulosclerosis in children and in adults, respectively. Read More

Tumour Biol 2016 Jul 20;37(7):9511-9. Epub 2016 Jan 20.

Medical Faculty, University of Pecs, Pecs, Hungary.

End-stage renal disease (ESRD) and acquired cystic renal disease (ACRD) are associated with high risk of development of renal cell tumors (RCT) displaying unusual phenotype and genotype. The underlying molecular mechanism is not yet known. To explore the molecular microenvironment, we have established the expression profile of ESRD/ACRD kidneys. Read More

Internist (Berl) 2016 Jan;57(1):38-48

Klinik für Hämatologie, Onkologie und Palliativmedizin, Klinikum Ernst von Bergmann, Potsdam, Deutschland.

This article is concerned with the important topic of infections associated with organ transplantation and includes a discussion on four subtopics. The first section describes the current options in the prevention and therapy of viral hepatitis in association with liver transplantation. Infections with hepatitis B, C, D (delta) and E are discussed with special emphasis on the interferon-free treatment of hepatitis C with the new antiviral drugs. Read More