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https://www.readbyqxmd.com/read/29897930/loss-of-fnip1-alters-kidney-developmental-transcriptional-program-and-synergizes-with-tsc1-loss-to-promote-mtorc1-activation-and-renal-cyst-formation
#1
Ryan Centini, Mark Tsang, Terri Iwata, Heon Park, Jeffrey Delrow, Daciana Margineantu, Brandon M Iritani, Haiwei Gu, H Denny Liggitt, Janella Kang, Lim Kang, David M Hockenbery, Daniel Raftery, Brian M Iritani
Birt-Hogg-Dube' Syndrome (BHDS) is a rare genetic disorder in humans characterized by skin hamartomas, lung cysts, pneumothorax, and increased risk of renal tumors. BHDS is caused by mutations in the BHD gene, which encodes for Folliculin, a cytoplasmic adapter protein that binds to Folliculin interacting proteins-1 and -2 (Fnip1, Fnip2) as well as the master energy sensor AMP kinase (AMPK). Whereas kidney-specific deletion of the Bhd gene in mice is known to result in polycystic kidney disease (PKD) and renal cell carcinoma, the roles of Fnip1 in renal cell development and function are unclear...
2018: PloS One
https://www.readbyqxmd.com/read/29889867/tubular-cell-loss-in-early-inv-nphp2-mutant-kidneys-represents-a-possible-homeostatic-mechanism-in-cortical-tubular-formation
#2
Masaki Shigeta, Hirotaka Kanazawa, Takahiko Yokoyama
Inversion of embryonic turning (inv) cystic mice develop multiple renal cysts and are a model for type II nephronophthisis (NPHP2). The defect of planar cell polarity (PCP) by oriented cell division was proposed as the underlying cellular phenotype, while abnormal cell proliferation and apoptosis occur in some polycystic kidney disease models. However, how these cystogenic phenotypes are linked and what is most critical for cystogenesis remain largely unknown. In particular, in early cortical cytogenesis in the inv mutant cystic model, it remains uncertain whether the increased proliferation index results from changes in cell cycle length or cell fate determination...
2018: PloS One
https://www.readbyqxmd.com/read/29887830/a-novel-pkd1-mutation-associated-with-autosomal-dominant-kidney-disease-and-cerebral-cavernous-malformation
#3
Christian Thomas, Andrea Zühlsdorf, Konstanze Hörtnagel, Lejla Mulahasanovic, Oliver M Grauer, Philipp Kümpers, Heinz Wiendl, Sven G Meuth
Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder characterized by the presence of renal cysts and specific extrarenal abnormalities. ADPKD is caused by mutations in either PKD1 or PKD2 genes that encode for integral membrane proteins Polycystin-1 (PC1) and Polycystin-2 (PC2), respectively. Extrarenal involvement includes noncystic manifestations such as dilatation of the aortic root, artery dissection and intracranial aneurysms. Cerebral cavernous malformation (CCM) is a rare vascular malformation disorder characterized by closely clustered and irregularly dilated capillaries that can be asymptomatic or cause variable neurological manifestations, such as seizures, non-specific headaches, progressive or transient focal neurologic deficits, and cerebral hemorrhages...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29887714/type-1-papillary-renal-cell-carcinoma-presenting-as-an-infected-benign-renal-cyst-an-uncommon-presentation
#4
Chanchal Rana, Manoj Kumar, Suresh Babu, Atin Singhai, H Agarwal
We describe an unusual presentation of papillary renal cell carcinoma in a 40-year old male where radiological evaluation revealed a Bosniak type 2 cyst (probably benign), and clinical signs as well as symptoms also suggested an infected cyst l. However, on histopathological examination, an intra cystic type 1 papillary renal cell carcinoma was diagnosed. This is an extremely rare presentation of papillary renal cell carcinoma because it usually present as a heterogenous mass. Hence, even the radiologically benign cyst should be evaluated and managed cautiously...
June 2018: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/29885406/eosinophilic-solid-and-cystic-renal-cell-carcinoma-mimicking-epithelioid-angiomyolipoma-series-of-4-primary-tumors-and-2-metastases
#5
Maria S Tretiakova
Eosinophilic solid and cystic renal cell carcinoma (ESC-RCC) is a novel entity of rare tumors with rather unique morphology and immunohistochemical profile. Until recently these tumors were characterized by indolent behavior. Herein we describe a series of six primary and metastatic ESC-RCCs morphologically and immunophenotypically mimicking epithelioid angiomyolipoma (eAML). Retrospective review of unclassified RCCs with oncocytic phenotype yielded several candidate cases, 4 of which fulfilled diagnostic criteria after additional work-up...
June 6, 2018: Human Pathology
https://www.readbyqxmd.com/read/29880342/effect-of-sirolimus-on-native-total-kidney-volume-after-transplantation-in-patients-with-autosomal-dominant-polycystic-kidney-disease-a-randomized-controlled-pilot-study
#6
S Davis, J Gralla, L Chan, A Wiseman, C L Edelstein
BACKGROUND: The mammalian target of rapamycin (mTOR) pathway has been shown to be central to cyst formation and growth in patients with autosomal dominant polycystic kidney disease (ADPKD). Drugs that suppress mTOR signaling are frequently used as antiproliferative agents for maintenance immunosuppression in patients who have undergone kidney transplantation. The aim of this study was to determine the effect of sirolimus, an mTOR inhibitor, on cyst volume regression in patients with ADPKD who have undergone renal transplantation...
June 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29879710/trajectory-of-estimated-glomerular-filtration-rate-predicts-renal-injury-in-children-with-multicystic-dysplastic-kidney
#7
Kazuya Matsumura, Kyoko Sugii, Midori Awazu
BACKGROUND/AIMS: Children with a solitary functioning kidney have a risk of renal injury caused by hyperfiltration. Timely intervention with renin-angiotensin inhibitors may be beneficial. We examined whether trajectory of estimated glomerular filtration rate (eGFR) would predict renal injury, defined as microalbuminuria/proteinuria, hypertension, and/or a decline in eGFR. METHODS: Seventeen patients (male 7, female 10) with multicystic dysplastic kidney (MCDK; median age 13 years, range 6-19 years) followed in our clinic were examined retrospectively...
June 7, 2018: Nephron
https://www.readbyqxmd.com/read/29875161/a-potential-strategy-for-reducing-cysts-in-autosomal-dominant-polycystic-kidney-disease-with-a-cftr-corrector
#8
Murali Yanda, Qiangni Liu, Liudmila Cebotaru
Autosomal dominant polycystic kidney disease (ADPKD) is associated with progressive enlargement of cysts, leading to a decline in function and renal failure that cannot be prevented by current treatments. Mutations in pkd1 and pkd2, encoding the polycystin 1 and 2 proteins, induce growth-related pathways, including heat shock proteins, as occurs in some cancers raising the prospect that pharmacological interventions that target these pathways might alleviate or prevent ADPKD. Here, we demonstrate a role for VX-809, a corrector of cystic fibrosis transmembrane conductance regulator (CFTR) conventionally used to manage cystic fibrosis, in reducing renal cyst growth...
June 6, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29874928/annals-express-hypomagnesaemia-is-absent-in-children-with-autosomal-dominant-polycystic-kidney-disease
#9
Tomas Seeman, Magdalena Fortova, Bruno Sopko, Richard Prusa, Michael Pohl, Ulrike John
BACKGROUND: Hypomagnesaemia is present in 40-50% of children with autosomal dominant renal cysts and diabetes syndrome (RCAD). On contrary, the prevalence of hypomagnesaemia in children with autosomal dominant polycystic kidney disease (ADPKD) has never been examined. We aimed to investigate whether hypomagnesaemia is present in children with polycystic kidney diseases. METHODS: Children with cystic kidney diseases were investigated in a cross-sectional study. Serum levels of magnesium (S-Mg) and fractional excretion of magnesium (FE-Mg) were tested...
January 1, 2018: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/29871474/laparoscopic-treatment-of-symptomatic-simple-renal-cysts-in-children-single-center-experience
#10
Antonio Marte, Lucia Pintozzi
Simple renal cysts, although common in adults, are rare in children. They are usually discovered incidentally in the course of the study of other urinary tract symptoms, although they are not always asymptomatic. Renal cysts can be classified as being either simple or complex. The purpose of this review is to present our case series of simple symptomatic renal cysts treated with laparoscopy. Nineteen patients with symptomatic renal cysts (6 to 13.5 cm) were referred to our institution between January 2006 and January 2017...
May 24, 2018: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
https://www.readbyqxmd.com/read/29869165/polycystic-kidney-features-of-the-renal-pathology-in-glycogen-storage-disease-type-i-possible-evolution-to-renal-neoplasia
#11
Monika Gjorgjieva, Laure Monteillet, Julien Calderaro, Gilles Mithieux, Fabienne Rajas
Glycogen storage disease type I (GSDI) is a rare genetic pathology characterized by glucose-6 phosphatase (G6Pase) deficiency, translating in hypoglycemia during short fasts. Besides metabolic perturbations, GSDI patients develop long-term complications, especially chronic kidney disease (CKD). In GSDI patients, CKD is characterized by an accumulation of glycogen and lipids in kidneys, leading to a gradual decline in renal function. At a molecular level, the activation of the renin-angiotensin system is responsible for the development of renal fibrosis, eventually leading to renal failure...
June 4, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29862868/inhibition-of-mast-cell-degranulation-with-cromolyn-sodium-exhibits-organ-specific-effects-in-polycystic-kidney-pck-rats
#12
Lu Jiang, Pingping Fang, Seth Septer, Udayan Apte, Michele T Pritchard
Autosomal recessive polycystic kidney disease (ARPKD) is a monogenic disease characterized by development of hepatorenal cysts, pericystic fibrosis, and inflammation. Previous studies show that mast cell (MC) mediators such as histamine induce proliferation of cholangiocytes. We observed robust MC accumulation around liver cysts, but not kidney cysts, in polycystic kidney (PCK) rats (an animal model of ARPKD). Therefore, we hypothesized that MCs contribute to hepatic cyst growth in ARPKD. To test this hypothesis, we treated PCK rats with 1 of 2 different MC stabilizers, cromolyn sodium (CS) or ketotifen, or saline...
January 1, 2018: International Journal of Toxicology
https://www.readbyqxmd.com/read/29861570/autosomal-dominant-polycystic-kidney-disease-and-hypertension-masquerading-primary-aldosteronism
#13
P D Mital, K Abhijit, G Shishir
Hypertension is a frequent early manifestation of autosomal dominant polycystic kidney disease (ADPKD). Many mechanisms cause hypertension in ADPKD; however, primary aldosteronism (PA) as a possible manifestation of hypertension in ADPKD is extremely rare. The diagnosis of PA in ADPKD is extremely challenging because multiple renal cysts can mask the identification of adrenal adenomas, and ADPKD is associated with hypertension in majority of cases. Here, we report a unique case of a young lady with ADPKD with hypertension masquerading PA...
March 2018: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29860066/mutational-analysis-in-patients-with-autosomal-dominant-polycystic-kidney-disease-adpkd-identification-of-five-mutations-in-the-pkd1-gene
#14
Mayssa Abdelwahed, Pascale Hilbert, Asma Ahmed, Hichem Mahfoudh, Salem Bouomrani, Mouna Dey, Jamil Hachicha, Hassen Kamoun, Leila Keskes-Ammar, Neïla Belguith
Autosomal Dominant Polycystic Kidney Disease (ADPKD), the most frequent genetic disorder of the kidneys, is characterized by a typical presenting symptoms include cysts development in different organs and a non-cysts manifestations. ADPKD is caused by mutations in PKD1 or PKD2 genes. In this study, we aimed to search for molecular causative defects among PKD1 and PKD2 genes. Eighteen patients were diagnosed based on renal ultrasonography and renal/extra-renal manifestations. Then, Sanger sequencing was performed for PKD1 and PKD2 genes...
May 31, 2018: Gene
https://www.readbyqxmd.com/read/29851703/acquired-cystic-disease-associated-renal-cell-carcinoma-acd-rcc-a-multiinstitutional-study-of-40-cases-with-clinical-follow-up
#15
Christopher G Przybycin, Holly L Harper, Jordan P Reynolds, Cristina Magi-Galluzzi, Jane K Nguyen, Angela Wu, Ankur R Sangoi, Peter S Liu, Saleem Umar, Rohit Mehra, Xiaochun Zhang, Roni M Cox, Jesse K McKenney
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...
May 30, 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29850462/transcatheter-arterial-embolization-therapy-for-huge-renal-cysts-two-case-reports
#16
Naoya Toriu, Junichi Hoshino, Saeko Kobori, Sun Watanabe, Masahiko Oguro, Yoichi Oshima, Rikako Hiramatsu, Hiroki Mizuno, Daisuke Ikuma, Akinari Sekine, Noriko Hayami, Keiichi Sumida, Masayuki Yamanouchi, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Motoko Yanagita, Takuya Fujimaru, Eisei Sohara, Shinichi Uchida, Yoshifumi Ubara
We encountered 2 patients with symptomatic huge simple renal cysts. In case 1, 4,000 mL of cyst fluid was drained via a catheter, but intracystic bleeding occurred immediately afterwards. Transcatheter arterial embolization (TAE) was performed, after which the bleeding stopped, and cyst drainage was repeated successfully. After 2 years, the total cyst volume was reduced from 11,775 mL to 75.4 mL. In case 2, TAE was performed prophylactically before drainage. Subsequently, 9,400 mL of fluid was removed from multiple cysts...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29845879/proposal-for-a-simple-anatomical-classification-of-the-pelvicalyceal-system-for-endoscopic-surgery
#17
Ryoji Takazawa, Sachi Kitayama, Yusuke Uchida, Satoshi Yoshida, Yusuke Kohno, Toshihiko Tsujii
We herein propose a nomenclature for renal calyces and anatomical classification of the renal pelvis for endoscopic surgery. We prospectively collected data on a total of 270 patients (540 kidneys) who underwent CT-urography. We then constructed three-dimensional images of the renal pelvis and examined their morphological classification. Forty-eight kidneys were excluded from this study due to severe hydronephrosis or large renal cysts. The remaining 492 kidneys were included. We systematically categorized each minor calyx into five levels: Top, Upper, Middle, Lower, and Bottom...
May 30, 2018: Journal of Endourology
https://www.readbyqxmd.com/read/29796844/tubulocystic-renal-cell-carcinoma-a-review-of-literature-focused-on-radiological-findings-for-differential-diagnosis
#18
REVIEW
Yukiko Honda, Yuko Nakamura, Keisuke Goto, Hiroaki Terada, Kazuhiro Sentani, Wataru Yasui, Yohei Sekino, Tetsutaro Hayashi, Jun Teishima, Akio Matsubara, Motonori Akagi, Tomoyo Fuji, Yasutaka Baba, Makoto Iida, Kazuo Awai
Tubulocystic renal cell carcinoma (TC-RCC) has been classified as an independent subtype according to the 2016 World Health Organization (WHO) classification. It is a rare subtype that predominantly affects men. Although few in number, radiological imaging reports have suggested that TC-RCC is characterized by multilocular cystic lesions, which are categorized as the Bosniak classification II-IV, with signature pathological characteristics comprising numerous small cysts or a tubular structure. The Bosniak classification system facilitates patient management; however, the differentiation of cystic tumors exhibiting similar imaging findings remains impossible; in fact, the differentiation of multilocular cystic RCC, adult cystic nephroma, and mixed epithelial and stromal tumor remains challenging...
May 23, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29782307/cutaneous-manifestations-of-tuberous-sclerosis
#19
Mirjana Bakić, Marina Ratković, Branka Gledović, Balsa Vujović, Danilo Radunović, Vera Babić, Vladimir Prelević
Dear Editor, Tuberous sclerosis (TS) is an autosomal dominant multisystem disease, which occurs due to genetically determined hyperplasia of ectodermal and mesodermal cells. Clinical manifestations present on the skin and in the nervous system, kidneys, heart, and other organs. Recent studies estimate the incidence of TS at 1/6000 to 1/10,000 live births, and a prevalence in the general population of approximately 1 in 20,000 (1). There are two different genetic loci responsible for TS: 9q34 (TSC1-hamartin) and 16p13...
April 2018: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/29779678/spontaneous-perirenal-hemorrhage-wunderlich-syndrome-an-analysis-of-28-cases
#20
Jong Wook Kim, Jung Youn Kim, Sun Tae Ahn, Tae Yong Park, Mi Mi Oh, Du Geon Moon, Hong Seok Park
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. Clinical variables (age, underlying diseases, symptoms, shock, and hospitalization period), laboratory test results, and radiological and pathological results were reviewed...
April 21, 2018: American Journal of Emergency Medicine
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