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congenital renal

Rawan Al-Yousef, Ahmad Almarzouq, Saad Aldousari
INTRODUCTION AND OBJECTIVES: Ectopic pelvic kidneys with renal stones are challenging to treat. We report our experience in managing a case of ectopic pelvic kidney with a pelvic stone by robotic pyelolithotomy after failure of flexible ure¬teroscopy. MATERIALS AND METHODS: A 46-year old male with 2 months history of vague lower abdominal pain was found to have on Computed Tomography scan a left ectopic pelvic kidney with a 12mm stone in an anomalous renal pelvis...
October 20, 2016: International Braz J Urol: Official Journal of the Brazilian Society of Urology
Lily Marsden, Lawrence J Jennings, Samantha Gadd, Min Yu, Elizabeth J Perlman, Mariana M Cajaiba
Metanephric stromal tumors are rare renal stromal tumors that predominantly affect children. They belong to the metanephric family of tumors along with metanephric adenofibroma and metanephric adenoma. The previous documentation of BRAF exon 15 mutations in 88% of metanephric adenomas and in isolated cases of metanephric adenofibroma prompted us to investigate the prevalence of these mutations in metanephric stromal tumors and in other pediatric renal stromal tumors. In this study, 17 metanephric stromal tumors, 22 congenital mesoblastic nephromas and 6 ossifying renal tumors of infancy were selected for BRAF exon 15 testing...
October 18, 2016: Human Pathology
Tuula Hölttä, Marjolein Bonthuis, Karlijn J Van Stralen, Anna Bjerre, Rezan Topaloglu, Fatih Ozaltin, Christer Holmberg, Jerome Harambat, Kitty J Jager, Franz Schaefer, Jaap W Groothoff
BACKGROUND: Congenital nephrotic syndrome (CNS) of the Finnish type, NPHS1, is the most severe form of CNS. Outcomes of renal replacement therapy (RRT) in NPHS1 patients in Europe were analysed using data from the ESPN/ERA-EDTA Registry. As NPHS1 is most prevalent in Finland and the therapeutic approach differs from that in many other countries, we compared outcomes in Finnish and other European patients. METHODS: NPHS1 mutations were confirmed in 170 children with CNS who initiated RRT (dialysis or renal transplantation) between 1991 and 2012...
October 20, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Faris G Bakri, Ayman Wahbeh, Awni Abu Sneina, Ali Al Khader, Fatima Obeidat, Izzat AlAwwa, Maryam Buni, Chang-Seok Ki, Amira Masri
Patients with congenital insensitivity to pain and anhydrosis syndrome are at risk for renal amyloidosis and inflammatory bowel disease. Physicians caring for such patients should be aware of these complications.
October 2016: Clinical Case Reports
Jamshid Roozbeh, Leila Malekmakan, Mohammad Mostafa Harifi, Taraneh Tadayon
OBJECTIVES: Autosomal dominant polycystic kidney disease is the most common hereditary disorder resulting in end-stage renal disease that can affect other organs besides kidneys. Extrarenal involvement may increase mortality and morbidity. Approximately 50% of patients with this disorder require renal transplant. Posttransplant complications have been reported to be equal in these patients versus other recipients. We conducted this study to determine and compare characteristics and outcomes of transplanted patients with this disease versus other recipients at the only transplant center in southern Iran...
October 14, 2016: Experimental and Clinical Transplantation
Shabnam Bhandari Grover, Nishith Kumar, Hemal Grover, Dinesh Kumar Taneja, Amit Katyan
BACKGROUND: Common etiologies of scrotal swelling in neonates include hydrocoele, inguinal hernia and testicular torsion; less common is epididymo-orchitis. Congenital nephrotic syndrome (CNS), a rare entity, is known to present as progressive renal failure and its leading presentation with scrotal involvement has not been reported. MATERIAL/METHODS: We report a rare case of CNS with primary clinical presentation as scrotal cellulitis and epididymo-orchitis. In this neonate, scrotal and abdominal ultrasound examination was performed and the laboratory data were obtained...
2016: Polish Journal of Radiology
Tennille N Webb, Stuart L Goldstein
PURPOSE OF REVIEW: The incidence of cardiac surgery-associated acute kidney injury (CS-AKI) continues to increase and is associated with significant morbidity and mortality. Early diagnosis and identification of patients at risk are extremely important. Therefore, identifying associated risk factors, biomarkers for earlier detection, prevention and therapeutic options for CS-AKI warrant special attention. RECENT FINDINGS: The current diagnosis of acute kidney injury (AKI) largely depends upon the functional biomarkers serum creatinine and oliguria leading to delays in diagnosis and worsening outcomes...
September 29, 2016: Current Opinion in Anaesthesiology
Liat Lerner-Geva, Valentina Boyko, Shelley Ehrlich, Shlomo Mashiach, Ariel Hourvitz, Jigal Haas, Ehud Margalioth, David Levran, Ilan Calderon, Raoul Orvieto, Adrian Ellenbogen, Joseph Meyerovitch, Raphael Ron-El, Adel Farhi
BACKGROUND: Among children conceived by assisted reproductive technology (ART), increased risk of adverse birth outcomes has been observed, including multiple births, preterm births, and congenital malformations. Regarding cancer among ART-conceived children, findings are discrepant. METHODS: This is a historical cohort of 9,042 ART-conceived children and 211,763 spontaneously conceived (SC) children born from 1997 through 2004. The median duration of follow-up was 10...
October 17, 2016: Pediatric Blood & Cancer
Takaaki Saito, Hiroki Tanaka, Naoto Yamamoto, Kazunori Inuzuka, Masaki Sano, Naoki Unno
We report the rare case of a 54-year-old man with uncontrolled renovascular hypertension, who was found to have an abdominal aortic aneurysm with congenital solitary pelvic kidney and superior mesenteric artery stenosis. A single renal artery branched from aneurysmal aortic bifurcation, and both the renal artery and the superior mesenteric artery (SMA) had severe stenosis at their origins. The aneurysm was repaired with a bifurcated Dacron graft, to which the renal artery was anastomosed. SMA bypass was created between the graft's left limb and the SMA using another Dacron graft...
2016: Annals of Vascular Diseases
Jason Misurac
Neonatal chronic kidney disease (CKD) occurs with an estimated incidence of 1 in 10,000 live births, whereas the incidence of neonatal end-stage renal disease (ESRD) is about 7.1 per million age-related population. The most frequent etiologies are renal hypoplasia/dysplasia, posterior urethral valves, and other congenital anomalies of the kidney and urinary tract. Other etiologies include polycystic kidney disease, cortical necrosis, and renal vascular thrombosis. Management of CKD focuses primarily on replacing renal functions such as erythropoietin, 1,25-hydroxylation of vitamin D, electrolyte homeostasis/excretion, and, in ESRD, waste product removal...
October 9, 2016: Seminars in Fetal & Neonatal Medicine
Priya S Verghese
Successful renal transplantation is the optimal treatment for chronic kidney failure, but this was not always so for children. Beginning with the first kidney transplants in the 1950s, children experienced poorer patient and graft survival rates than adult patients. But over the last 6 decades, an improved understanding of the immune system which has steered pediatric multi-center clinical / pharmacokinetic and mechanistic studies that have sculpted our immunosuppression with markedly better patient and graft survivals...
October 12, 2016: Pediatric Research
Phayvanh P Sjogren, Richard K Gurgel, Albert H Park
OBJECTIVES: To determine whether a canal wall down mastoidectomy can provide long-term benefit for children with aural stenosis. METHODS: Retrospective case series of children with congenital aural stenosis having undergone a canal wall down mastoidectomy over a twelve-year period at a tertiary children's hospital. RESULTS: Data from thirteen children who underwent a total of twenty canal wall down mastoidectomies for aural stenosis were reviewed...
November 2016: International Journal of Pediatric Otorhinolaryngology
Connie Y Chang, Daniel I Rosenthal, Deborah M Mitchell, Atsuhiko Handa, Susan V Kattapuram, Ambrose J Huang
Metabolic bone diseases are a diverse group of diseases that result in abnormalities of (a) bone mass, (b) structure mineral homeostasis, (c) bone turnover, or (d) growth. Osteoporosis, the most common metabolic bone disease, results in generalized loss of bone mass and deterioration in the bone microarchitecture. Impaired chondrocyte development and failure to mineralize growth plate cartilage in rickets lead to widened growth plates and frayed metaphyses at sites of greatest growth. Osteomalacia is the result of impaired mineralization of newly formed osteoid, which leads to characteristic Looser zones...
October 2016: Radiographics: a Review Publication of the Radiological Society of North America, Inc
R Thuret, F Kleinclauss, N Terrier, G Karam, M O Timsit
OBJECTIVES: To describe kidney transplantation surgical techniques and to propose strategies in high-risk recipients. MATERIAL AND METHODS: Relevant publications were identified through Medline ( and Embase ( database using the following keywords, alone or in association, "renal transplantation; peripheral arterial disease; obesity; third and fourth transplantation; robotic-assisted kidney transplant; anticoagulant therapy; dual kidney transplant"...
October 6, 2016: Progrès en Urologie
Laura Fontana, Barbara Gentilin, Luigi Fedele, Cristina Gervasini, Monica Miozzo
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also referred to as Müllerian agenesis, is the second most common cause of primary amenorrhea. It is characterized by congenital absence of the uterus, cervix, and the upper part of the vagina in otherwise phenotypically normal 46,XX females. MRKH syndrome has an incidence of about 1 in 4,500-5,000 newborn females and it is generally divided into two subtypes: MRKH type 1, in which only the upper vagina, cervix and the uterus are affected, and MRKH type 2, which is associated with additional malformations generally affecting the renal and skeletal systems, and also includes MURCS (MÜllerian Renal Cervical Somite) characterized by cervico-thoracic defects...
October 7, 2016: Clinical Genetics
Tuncer Ahmet Ali, Karavelioğlu Afra, Baskin Embleton Didem, Elmas Muhsin
Holt-Oram syndrome (HOS) is a rare autosomal dominant disorder, characterized by upper limb dysplasia and congenital cardiac defect. We report two cases with HOS, first associated with renal agenesis, coronal hypospadias, urethral duplication and second associated with duodenal atresia and horseshoe kidney that have not been reported in English literature.
October 2016: Journal of Indian Association of Pediatric Surgeons
Jake A Nieto, Michael A Yamin, Itzhak D Goldberg, Prakash Narayan
Autosomal recessive polycystic kidney disease (ARPKD) is associated with progressive enlargement of the kidneys fuelled by the formation and expansion of fluid-filled cysts. The disease is congenital and children that do not succumb to it during the neonatal period will, by age 10 years, more often than not, require nephrectomy+renal replacement therapy for management of both pain and renal insufficiency. Since increasing cystic index (CI; percent of kidney occupied by cysts) drives both renal expansion and organ dysfunction, management of these patients, including decisions such as elective nephrectomy and prioritization on the transplant waitlist, could clearly benefit from serial determination of CI...
2016: PloS One
Farouk M Nasser, Ahmed M Shouman, Mohammed S ElSheemy, Mohammed A Lotfi, Waseem Aboulela, Mohamed El Ghoneimy, Mohammed Abdelwahab, Ahmed I Shoukry, Waleed Ghoneima, Hany Morsi, Hesham Badawy
OBJECTIVES: To compare outcome of dismembered-pyeloplasty in infants with and without external nephro-ureteric stent (ENUS) for treatment of congenital ureteropelvic junction obstruction (UPJ-O). METHODS: This is a parallel, randomized comparative study between October 2013 and September 2014. 30 infants ≤6 months old with UPJ-O indicated for dismembered-pyeloplasty were randomly assigned (block randomization, closed envelope method) into two groups; group-A (stentless) and group-B (ENUS)...
September 28, 2016: Urology
Ahmed R El-Nahas, Mohamed M Elsaadany, Diaa-Eldin Taha, Ahmed M Elshal, Mohamed Abo El-Ghar, Amani M Ismail, Essam A Elsawy, Hazem H Saleh, Ehab W Wafa, Amira Awadalla, Tamer S Barakat, Khaled Z Sheir
OBJECTIVE: To evaluate the protective effects of selenium with vitamins A, C and E (selenium ACE, i.e. antioxidants), verapamil (calcium channel blocker), and losartan (angiotensin receptor blocker) against extracorporeal shockwave lithotripsy (ESWL)-induced renal injury. PATIENTS AND METHODS: A randomised controlled trial was conducted between August 2012 and February 2015. Inclusion criteria were adult patients with a single renal stone (<2 cm) suitable for ESWL...
September 30, 2016: BJU International
Amine Slaoui, Souhail Regragui, Abdelouahad Lasri, Tarik Karmouni, Khalid El Khader, Abdellatif Koutani, Ahmed Ibn Attya
BACKGROUND: Congenital malformations of the seminal vesicle are uncommon, and most of them are cystic malformations. If an insult occurs between the 4th and the 13 h gestational week, the embryogenesis of the kidney, ureter, seminal vesicle, and vas deferens could be altered. Cysts of the seminal vesicle may appear with a mass effect, dysuria, epididymitis, or obstruction of the gastrointestinal and genitourinary tracts. Approximately two thirds of them are associated with ipsilateral renal agenesis, because both the ureteral buds and seminal vesicles originate from the mesonephric (Wolffian) duct...
2016: Basic and Clinical Andrology
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