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multicystic dysplastic kidney

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https://www.readbyqxmd.com/read/29879710/trajectory-of-estimated-glomerular-filtration-rate-predicts-renal-injury-in-children-with-multicystic-dysplastic-kidney
#1
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/29796733/a-refractory-hypertensive-patient-with-fibromuscular-dysplasia-and-multicystic-dysplastic-kidney
#2
Shin Takahashi, Yurie Takizawa, Satoshi Nakano, Kotaro Oyama
The case of a 16-year-old male patient with left renal artery stenosis due to fibromuscular dysplasia (FMD) and an atrophic kidney due to a right multicystic dysplastic kidney (MCDK) who presented with refractory hypertension is reported. On continuous Doppler imaging, the peak systolic velocity (PSV) at the stenotic site of the left renal artery was 404 cm/s. The FMD formed a "string-of-beads" appearance on computed tomographic angiography (CTA) and renal artery angiography. Percutaneous transluminal renal angioplasty (PTRA) with a balloon catheter was performed, after which the left renal artery returned to near normal, and his blood pressure decreased to within the normal range...
May 23, 2018: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/29730988/pelvic-dystopia-of-right-rudimentary-multicystic-dysplastic-kidney-as-a-rare-cause-of-bedwetting-in-a-patient-with-a-single-pelvic-ectopic-left-kidney-and-agenesis-of-the-uterus-and-vagina-mayer-rokitansky-k%C3%A3-ster-hauser-syndrome-a-case-report
#3
Kidirali Karimbayev, Nazarbek Dzumanazarov, Mukhtar Akhaibekov, Nurzhan Berdikulov, Abay Karimbayev, Assanaly Mustafayev
BACKGROUND: Pelvic dystopia of rudimentary multicystic dysplastic kidney as a rare cause of bedwetting in children. CASE PRESENTATION: We report the case of a 14-year-old Kazakh girl who presented with difficulty in starting the stream of urine and intermittent interruption of the urinary stream while voiding as well as bedwetting, caused by a rare congenital disease (pelvic dystopia of rudimentary multicystic dysplastic kidney). The diagnostic workup, differential diagnosis, and management, and a review of the literature are presented...
May 7, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29572458/predictors-of-poor-neonatal-outcomes-in-prenatally-diagnosed-multicystic-dysplastic-kidney-disease
#4
Malathi Balasundaram, Valerie Y Chock, Hsi Yang Wu, Yair J Blumenfeld, Susan R Hintz
OBJECTIVE: Multicystic dysplastic kidney (MCDK) is one of the most common anomalies detected by prenatal ultrasound. Our objective was to identify factors associated with severe adverse neonatal outcomes of prenatally diagnosed MCDK STUDY DESIGN: A retrospective review of prenatally diagnosed MCDK (1 January 2009 to 30 December 2014) from a single academic center was conducted. The primary outcome was death or need for dialysis among live-born infants. Associations between prenatal characteristics and outcome were analyzed by Fisher's exact test and Mann-Whitney test...
March 23, 2018: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29515943/applicability-of-laparoscopic-nephrectomy-in-the-treatment-of-multicystic-dysplastic-kidney-sorting-out-surgical-indication
#5
Carlos Augusto F Molina, Jose Bessa Junior, Andrey G Estevanato, Gustavo S Viana, Inalda Facincani, Jose Murillo Bastos Netto, Silvio Tucci Junior
Introduction We evaluated the applicability of laparoscopic nephrectomy in the treatment of multicystic dysplastic kidney (MCDK) in children, including procedures performed by resident physicians or trainees in surgical urology. Methods We retrospectively evaluated the medical records of 20 children with MCDK who underwent laparoscopic nephrectomy over a six-year period. Data collected included gender, laterality of the affected kidney, age at the time of surgery, the largest diameter of the multicystic kidney and associated urological diagnoses, surgical, and postoperative data...
January 1, 2018: Curēus
https://www.readbyqxmd.com/read/29459133/screening-for-mullerian-anomalies-in-patients-with-unilateral-renal-agenesis-leveraging-early-detection-to-prevent-complications
#6
M Alexandra Friedman, Liza Aguilar, Quetrell Heyward, Carol Wheeler, Anthony Caldamone
BACKGROUND: Mullerian anomalies have a known association with renal agenesis yet, to date, there are no formal recommendations for screening women with certain renal anomalies for associated genital tract disorders. OBJECTIVE: The objective of this study is to review current data regarding the association between renal and Mullerian anomalies, and propose screening recommendations. STUDY DESIGN: A comprehensive review of the literature was performed to identify relevant articles using the keywords "unilateral renal agenesis," "renal anomalies," and "Mullerian anomalies...
February 9, 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/29146704/prospective-evaluation-of-kidney-disease-in-joubert-syndrome
#7
Leah R Fleming, Daniel A Doherty, Melissa A Parisi, Ian A Glass, Joy Bryant, Roxanne Fischer, Baris Turkbey, Peter Choyke, Kailash Daryanani, Meghana Vemulapalli, James C Mullikin, May Christine Malicdan, Thierry Vilboux, John A Sayer, William A Gahl, Meral Gunay-Aygun
BACKGROUND AND OBJECTIVES: Joubert syndrome is a genetically heterogeneous ciliopathy associated with >30 genes. The characteristics of kidney disease and genotype-phenotype correlations have not been evaluated in a large cohort at a single center. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We evaluated 97 individuals with Joubert syndrome at the National Institutes of Health Clinical Center using abdominal ultrasonography, blood and urine chemistries, and DNA sequencing...
December 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29072675/outcomes-of-prenatally-diagnosed-solitary-functioning-kidney-during-early-life
#8
M Davidovits, R Cleper, N Eizenberg, O Hocherman, R Mashiach
OBJECTIVE: To evaluate outcomes of congenital solitary functioning kidney (SFK) in early childhood. STUDY DESIGN: A retrospective study of 32 children diagnosed in utero with SFK owing to unilateral renal agenesis or multicystic dysplastic kidney and followed for 1 to 11.5 years. RESULTS: SFK length was in the compensatory hypertrophy range in 45% of fetal sonographic evaluations from mid-pregnancy, and in 85% on postnatal follow-up. Glomerular filtration rate was below normal range in 44...
December 2017: Journal of Perinatology: Official Journal of the California Perinatal Association
https://www.readbyqxmd.com/read/29046082/hydronephrotic-kidney-with-multiple-extra-renal-calyces
#9
S Rajendran, A Cho, P Mishra, A Cherian
Extra-renal calyces are a rare anomaly of the renal collecting system, characterised by the presence of calyces and pelvis outside the renal parenchyma. It may also be associated with other anomalies, such as renal ectopia, fusion and malrotation. We describe an unusual case of extra-renal calyces with six long calyces arising from a suspected multicystic dysplastic kidney that was successfully managed laparoscopically.
November 2017: Annals of the Royal College of Surgeons of England
https://www.readbyqxmd.com/read/29023893/analysis-of-renal-blood-flow-and-renal-volume-in-normal-fetuses-and-in-fetuses-with-a-solitary-functioning-kidney
#10
An Hindryckx, Anke Raaijmakers, Elena Levtchenko, Karel Allegaert, Luc De Catte
OBJECTIVE: To evaluate renal blood flow and renal volume for the prediction of postnatal renal function in fetuses with solitary functioning kidney (SFK). METHODS: Seventy-four SFK fetuses (unilateral renal agenesis [12], multicystic dysplastic kidney [36], and severe renal dysplasia [26]) were compared with 58 healthy fetuses. Peak systolic velocity (PSV), pulsatility index (PI), and resistance index (RI) of the renal artery (RA) were measured; 2D and 3D (VOCAL) volumes were calculated...
December 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28955442/multicystic-dysplastic-kidney-a-new-association-of-wolcott-rallison-syndrome
#11
Asma Deeb, Faisal Al-Zidgali, Bibian N Ofoegbu
Wolcott-Rallison syndrome (WRS) is a rare autosomal recessive disorder due to mutations in the EIF2AK3 gene. It is characterized by permanent neonatal diabetes mellitus, skeletal dysplasia, liver impairment, neutropenia and renal dysfunction. Liver is the most commonly affected organ and liver failure is the commonest cause of death in this syndrome. The EIF2AK3 gene encodes a transmembrane protein PERK, which is important for the cellular response to endoplasmic reticulum (ER) stress. The absence of PERK activity reduces the ER's abilities to deal with stress, leading to cell death by apoptosis...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28941593/laparoendoscopic-single-site-nephrectomy-in-children-is-it-a-good-alternative-to-conventional-laparoscopic-approach
#12
Belén Aneiros Castro, Daniel Cabezalí Barbancho, Cristina Tordable Ojeda, Isabel Carrillo Arroyo, Jesús Redondo Sedano, Andrés Gómez Fraile
BACKGROUND: Minimally invasive surgery is considered to be the gold standard treatment for nephrectomy in children. In recent decades it has been proposed that laparoendoscopic single-site (LESS) surgery is a feasible alternative to perform laparoscopic nephrectomies. OBJECTIVE: The aim of our study was to compare the safety and efficacy of LESS against conventional laparoscopic (CL) nephrectomy. STUDY DESIGN: From March 2010 to November 2012 charts of pediatric patients who underwent laparoscopic nephrectomy at our tertiary center were revised...
February 2018: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28764564/neonatal-renal-cystic-diseases
#13
Anshika Khare, Vinod Krishnappa, Deepak Kumar, Rupesh Raina
PURPOSE: Neonatal renal cystic diseases have a great impact on the morbidity and mortality of the affected neonates and infants. A good insight into the pathophysiology, diagnosis and treatment options of various neonatal renal cystic diseases aid in early diagnosis and intervention, thereby preventing complications. METHODS: PubMed search was done for articles on "neonatal renal cystic diseases" and relevant publications including reviews were considered for our article...
August 2, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28658845/large-multicystic-dysplastic-kidney-mimicking-a-large-cystic-renal-neoplasm
#14
Priyank Yadav, Sanjoy Kumar Sureka, M S Ansari, Rahul Soni, Hira Lal
Multicystic Dysplastic Kidney (MCDK) is one of the most common renal conditions seen in paediatric population. The natural history typically involves involution and many of the patients with unilateral disease may actually never become symptomatic. The initial evaluation is usually done on Ultrasonography (USG) while cross-sectional imaging and nuclear scan are reserved for diagnostic dilemmas. Management is conservative and surgery is done for selected patients with symptomatic cysts or suspicion of neoplasm...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28645868/natural-history-of-contralateral-hypertrophy-in-patients-with-multicystic-dysplastic-kidneys
#15
Thomas W Gaither, Ankur Patel, Chandni Patel, Kai-Wen Chuang, Ronald A Cohen, Laurence S Baskin
PURPOSE: We evaluated predictive factors for compensatory hypertrophy and renal outcomes in a large cohort of patients with multicystic dysplastic kidneys. MATERIALS AND METHODS: We conducted a retrospective review from 1997 to 2016. Contralateral kidney and multicystic dysplastic kidney length were recorded from all ultrasounds as well as creatinine when available. We used generalized estimating equations to determine predictors of contralateral kidney length. RESULTS: A total of 443 children with multicystic dysplastic kidneys were identified based on sonographic findings and lack of function on nuclear scan...
January 2018: Journal of Urology
https://www.readbyqxmd.com/read/28440349/cyst-to-kidney-volume-ratio-in-the-sonographic-diagnosis-of-unilateral-multicystic-dysplastic-kidney-in-children
#16
Sevin Ayaz, Alper Dilli, Salih Sinan Gültekin, Ümit Yaşar Ayaz
AIMS: To evaluate the usefulness of the cyst-to-kidney volume ratio determined by ultrasonography (US) in unilateral multicystic dysplastic kidney (MCDK) in children. MATERIAL AND METHODS: Our study group included 21 children (average age: 431 days) with unilateral MCDK and 22 children (average age: 440 days) with unilateral grade IV hydronephrosis due to ureteropelvic junction obstruction as the control group. All the children underwent transabdominal US. In children with MCDK, we calculated cyst-to-kidney volume ratios (volume of the largest cyst/volume of the MCDK) and in the control group the volume ratios of the renal pelvis and the largest calyx (volume of the pelvis or largest calyx/volume of the ipsilateral hydronephrotic kidney)...
April 22, 2017: Medical Ultrasonography
https://www.readbyqxmd.com/read/28346956/early-nephrectomy-in-unilateral-multicystic-dysplastic-kidney-in-children-cures-hypertension-early-an-observation
#17
Basant Kumar, Vijai Datta Upadhyaya, Manish Kumar Gupta, Laxmi Kant Bharti, Ram Nawal Rao, Sheo Kumar
Objective  Surgery is still indicated as the treatment of choice in subset of patients with unilateral multicystic dysplastic kidney (UMCDK) because of its potential complications and malignant change. The purpose of this study is to present our observation that early nephrectomy may cure hypertension early in children with UMCDK and review the literature. Materials and Methods  We report here four children (two males and two females) with antenatally diagnosed UMCDK with hypertension, treated in the past 4 years...
December 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28249589/diagnostic-and-therapeutic-challenges-of-an-ambiguous-cystic-kidney-disease-in-a-resource-limited-setting-a-case-report
#18
Christian Akem Dimala, Ndemazie Nkafu Bechem, Benjamin Momo Kadia, Vitalis Fambombi Feteh, Simeon Pierre Choukem
BACKGROUND: Unilateral renal cystic disease is a rare condition that shares morphological similarities with multicystic dysplastic kidney, the former often distinguished from the latter on some clinical and histopathological grounds. However serious diagnostic and therapeutic dilemmas set in when there is a considerable overlap in the distinguishing features between these entities. CASE PRESENTATION: A 19-year-old African female presented with a chronic severe debilitating right lower quadrant abdominal pain refractory to analgesics...
March 1, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28164035/proximal-ureteral-atresia-a-rare-congenital-anomaly-incidental-finding-a-case-report
#19
Cosimo Bleve, Maria Luisa Conighi, Lorella Fasoli, Valeria Bucci, Francesco Battaglino, Salvatore Fabio Chiarenza
Ureteral atresia is a rare disease usually associated with a non-functioning dysplastic kidney. The condition may be unilateral or bilateral; focal, short or long and may involve any part of the ureter. Association with other urinary anomalies is rare. We report the case of a 10-month-old boy with prenatal diagnosis of multicystic right kidney. This suspicion was confirmed after birth by ultrasound and static scintigraphy; a right vesicoureteral reflux (VUR) was recorded at cystography. The boy presented a regular renal function but was hospitalized twice for suspected pyelonephritis between the 8th and the 10th month of life and were recorded occasional mild changes in blood pressure...
January 2017: Translational Pediatrics
https://www.readbyqxmd.com/read/28101154/minimally-invasive-open-nephrectomy-on-children-with-multicystic-dysplastic-kidney
#20
Dongchuan Feng, Xiaoyu Zhu, Fang Sun, Tongsheng Ma, Yuan Li, Shujing Chen
The aim of the study was to summarize the preliminary experience of minimally invasive open nephrectomy operation on children with multicystic dysplastic kidney (MCDK). A retrospective review was performed on the clinical materials of the 15 children that had accepted consecutive minimally invasive open nephrectomies during the previous 2 years. The enrolled children were diagnosed with unilateral MCDK under computed tomography, emission computerized tomography and ultrasound and no anomaly in the contralateral functioning kidney was found...
December 2016: Experimental and Therapeutic Medicine
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