Horseshoe Kidney
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Renal Agenesis, Renal Tubular Dysgenesis, and Polycystic Renal Diseases
Developmental & Structural Anomalies of the Genitourinary Tract DR. Alao MA Bowen University Teach Hosp Ogbomoso Picture test Introduction • Congenital Anomalies of the Kidney & Urinary Tract (CAKUT) Objectives • To review the embryogenesis of UGS and dysmorphogenesis of CAKUT • To describe the common CAKUT in children • To emphasize the role of imaging in the diagnosis of CAKUT Introduction •CAKUT refers to gross structural anomalies of the kidneys and or urinary tract present at birth. •Malformation of the renal parenchyma resulting in failure of normal nephron development as seen in renal dysplasia, renal agenesis, renal tubular dysgenesis, and polycystic renal diseases. Introduction •Abnormalities of embryonic migration of the kidneys as seen in renal ectopy (eg, pelvic kidney) and fusion anomalies, such as horseshoe kidney. •Abnormalities of the developing urinary collecting system as seen in duplicate collecting systems, posterior urethral valves, and ureteropelvic junction obstruction. Introduction •Prevalence is about 3-6 per 1000 births •CAKUT is one of the commonest anomalies found in human. •It constitute approximately 20 to 30 percent of all anomalies identified in the prenatal period •The presence of CAKUT in a child raises the chances of finding congenital anomalies of other organ-systems Why the interest in CAKUT? •Worldwide, CAKUT plays a causative role in 30 to 50 percent of cases of end-stage renal disease (ESRD), •The presence of CAKUT, especially ones affecting the bladder and lower tract adversely affects outcome of kidney graft after transplantation Why the interest in CAKUT? •They significantly predispose the children to UTI and urinary calculi •They may be the underlying basis for urinary incontinence Genes & Environment Interact to cause CAKUT? • Tens of different genes with role in nephrogenesis have been identified. -
A Study of Congenital Renal Anomalies in Adult Cadavers
Original Research Article DOI: 10.18231/2394-2126.2017.0045 A study of congenital renal anomalies in adult cadavers Pabbati Raji Reddy Associate Professor, RVM Institute of Medical Sciences & Research Center, Telangana Email: [email protected] Abstract Introduction: Congenital abnormalities of the kidneys and urinary tract play a major role in the morbidity and mortality. Many of these renal anomalies predispose to obstruction which lay lead to renal failure. We had in our study observed the different malformations in human kidneys among the adults human cadavers. Materials and Method: 50 cadavers who died of renal failure and were scheduled for post mortem were included in the study. The position of the suprarenal gland and the upper poles of the kidneys, the size, shape and the kidneys, the arrangement of the attached structures such as the hilum, ureter, bladder abdominal aorta and the inferior vena cava were noted and recorded. Results: Out of the 50 cadavers that were included into the study, 5 of them had congenital renal anomaly accounting for 10% of the deaths due to renal failure in adults. All the patients were between 40–60 years of age. There were two cases of lobulated kidney, one horse – shoe shaped kidney, one case of congenital hypoplasia and one 7 shaped left kidney. Conclusion: Renal anomalies are one of the common congenital anomalies which may remain unnoticed till adulthood. Of them, renal agenesis, horseshoe kidneys, renal hypoplasia and lobulated kidneys are relatively predominant. Keywords: Congenital renal anomalies, Cadavers, renal hypoplasia, Lobulated kidney, Horseshoe kidney. Introduction Hypoplasia usually occurs due to inadequate Congenital abnormalities of the kidneys and ureteral bud branching and results in a small kidney urinary tract play a major role in the morbidity and with histologically normal nephrons, though few in mortality. -
Ultrasound Appearance of Congenital Renal Disease: Pictorial Review
The Egyptian Journal of Radiology and Nuclear Medicine (2014) 45, 1255–1264 Egyptian Society of Radiology and Nuclear Medicine The Egyptian Journal of Radiology and Nuclear Medicine www.elsevier.com/locate/ejrnm www.sciencedirect.com REVIEW Ultrasound appearance of congenital renal disease: Pictorial review Narrotam A. Patel, Pokhraj P. Suthar * Department of Radiology, S.S.G. Hospital, Medical College, Vadodara, India Received 12 April 2014; accepted 27 June 2014 Available online 5 August 2014 KEYWORDS Abstract Congenital renal diseases consist of a variety of entities. The age of presentation and GUT; clinical examination narrow down the differential diagnosis; however, imaging is essential for accu- Renal disease; rate diagnosis and pretreatment planning. Ultrasound is often used for initial evaluation. Computed Congenital; tomography (CT) and MRI provide additional information. Ultrasonography continues to occupy Ultrasonography a central role in the evaluation and detection of congenital renal diseases due to its advantage of rapid scanning time, lack of radiation exposure, cost effective and easy feasibility. Ó 2014 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier B.V. All rights reserved. Contents 1. Technique. 1256 1.1. Anomalies related to ascent of kidney. 1256 1.1.1. Ectopia . 1256 1.1.2. Crossed renal ectopia . 1256 1.1.3. Horseshoe kidney. 1257 1.2. Anomalies related to the ureteric bud . 1258 1.2.1. Renal agenesis . 1258 1.2.2. Supernumerary kidney . 1258 1.2.3. Duplex collecting system and ureterocele . 1258 1.2.4. Uretero-pelvic junction obstruction . 1259 1.2.5. Congenital megacalyces . 1260 1.2.6. Congenital megaureter . -
Congenital Kidney and Urinary Tract Anomalies: a Review for Nephrologists
REVIEW ARTICLE Port J Nephrol Hypert 2018; 32(4): 385-391 • Advance Access publication 4 January 2019 Congenital kidney and urinary tract anomalies: a review for nephrologists Marina Vieira, Aníbal Ferreira, Fernando Nolasco Nephrology Department, Hospital Curry Cabral, Centro Hospitalar Lisboa Central, Lisboa, Portugal Received for publication: Sep 7, 2018 Accepted in revised form: Dec 7, 2018 ABSTRACT Kidney and urinary tract development disorder are two of the most prevalent congenital malformations and the main cause of chronic kidney disease in pediatric age patients. As such, it is very important that the neph‑ rologist understands these pathologies to improve transition and ensure a good continuity between pediatric and adult nephrological care. The purpose of this article is to present a brief review of congenital anomalies of the kidney and urinary tract (CAKUT). Kidney malformations are classified according to macroscopic and microscopic anatomic features, and are the result of the following abnormal renal developmental processes: malformations of the renal parenchyma, abnor‑ malities of the embryonic migration of the kidneys and abnormalities of the developing urinary collecting system. Keys words: congenital anomalies of the kidneys and urinary tract, dysplasia, ciliopathies, posterior urethral valves, vesicoureteral reflux. INTRODUCTION are more likely to require dialysis6. Kidney malforma‑ tions are classified according to macroscopic and micro‑ Kidney and urinary tract development disorders scopic anatomic features, and -
RENAL SCINTIGRAPHY in the 21St CENTURY
RENAL SCINTIGRAPHY IN THE 21st CENTURY 99m Tc- MAG3 with zero time injection of Furosemide (MAG3-F0) : A Fast and Easy Protocol, One for All Indications Clinical Experience Congenital Disorders PROTOCOL FOR MAG3 - F0 PATIENT PREPARATION Easy (only restriction, oral hydration, no bladder cath.) DYNAMIC STUDY (iv 1-10 mCi MAG3 + 40-80 mg LASIX) Simultaneous injection of Furosemide: MAG3-F0 Duration of the study 25 min TOMOGRAPHY-SPECT (20 mCi MAG3) No diuretic needed Duration of the study 4 min RENAL SCINTIGRAPHY AT UM/JMMC: (MAG3-F0) Method applied the last 18 years: FOR NATIVE KIDNEY STUDIES FOR RENAL TRANSPLANT STUDIES RENAL SCINTIGRAPHY AT UM/JMMC: (MAG3-F0) Method applied the last 18 years: A. DYNAMIC STUDY Injection iv 1-10 mCi MAG3 + 40-80 mg LASIX (Furosemide) Simultaneous Injection of MAG3 and Lasix = F0 ACQUISITION: FLOW: 1 min ( 1 frame per 1 sec) FUNCTION: 22 min ( 1 frame per 30 sec) POST VOID 2 min static image (at 25-30 min) DELAYED 2 min static images (at 1 hr) GROUPING IMAGES : -FLOW: in 3 sec images -FUNCTION: in 2 min images GRAPH GENERATION: -FLOW/FUNCTION, KIDNEY/CORTEX RENAL SCINTIGRAPHY AT UM/JMMC: (MAG3-F0) Method applied the last 18 years: This protocol was originally applied in the evaluation of drainage Soon it was realized that it allowed the evaluation of the parenchyma Then it was applied in all parenchymal indications (including APN) It was also utilized for the study of Renovascular Hypertension In children allowed the study of HIV and other Nephropathies In patients with renal colic unraveled the Stunned -
Renal Anomalies
We are IntechOpen, the world’s leading publisher of Open Access books Built by scientists, for scientists 4,800 122,000 135M Open access books available International authors and editors Downloads Our authors are among the 154 TOP 1% 12.2% Countries delivered to most cited scientists Contributors from top 500 universities Selection of our books indexed in the Book Citation Index in Web of Science™ Core Collection (BKCI) Interested in publishing with us? Contact [email protected] Numbers displayed above are based on latest data collected. For more information visit www.intechopen.com Chapter 13 Renal Anomalies Alexandru Cristian Comanescu, Florentina Tanase, AlexandruMaria Cristina Comanescu, Cristian Comanescu, Razvan Florentina Cosmin Pana, Tanase, MariaMadalina Barbu Cristina Comanescu, and Nicolae Cernea Razvan Cosmin Pana, MadalinaAdditional information Barbu and is available Nicolae at the Cernea end of the chapter Additional information is available at the end of the chapter http://dx.doi.org/10.5772/intechopen.71925 Abstract This chapter is dedicated to the main renal anomalies detectable by ultrasound. Anomalies of the lower urinary tract will be addressed in a separate chapter. The anomalies pre- sented are renal agenesis, renal development variants, autosomal recessive polycystic kidney disease, multicystic dysplastic kidney disease, autosomal dominant polycystic kidney disease, obstructive cystic dysplasia, pelvis dilatation, renal tumors, and non- chromosomal syndromes associated with renal anomalies. All chapters are structured similar into definition, incidence, pathology, ultrasound findings, differential diagnosis, and clinical facts. Keywords: kidney, anomaly, ultrasound 1. Introduction The present chapter addresses the main renal anomalies. It will be structured on nine sub- jects made to help the reader orientate easily when facing an anomaly in everyday practice. -
16 the Kidney J
16 The Kidney J. Charles Jennette FPO FPO FPO FPO CONGENITAL ANOMALIES IgA Nephropathy (Berger Disease) Renal Agenesis Anti-Glomerular Basement Membrane Ectopic Kidney Glomerulonephritis Horseshoe Kidney ANCA Glomerulonephritis Renal Dysplasia VASCULAR DISEASES CONGENITAL POLYCYSTIC KIDNEY DISEASES Renal Vasculitis Autosomal Dominant Polycystic Kidney Disease Hypertensive Nephrosclerosis (Benign Nephrosclerosis) (ADPKD) Malignant Hypertensive Nephropathy Autosomal Recessive Polycystic Kidney Disease Renovascular Hypertension (ARPKD) Thrombotic Microangiopathy Nephronophthisis–Medullary Cystic Disease Cortical Necrosis ACQUIRED CYSTIC KIDNEY DISEASE DISEASES OF TUBULES AND INTERSTITIUM GLOMERULAR DISEASES Acute Tubular Necrosis (ATN) Nephrotic Syndrome Pyelonephritis Nephritic Syndrome Analgesic Nephropathy Glomerular Inflammation and Immune Mechanisms Drug-Induced (Hypersensitivity) Acute Tubulointerstitial Minimal-Change Glomerulopathy Nephritis Focal Segmental Glomerulosclerosis (FSGS) Light-Chain Cast Nephropathy Membranous Glomerulopathy Urate Nephropathy Diabetic Glomerulosclerosis RENAL STONES (NEPHROLITHIASIS AND Amyloidosis UROLITHIASIS) Hereditary Nephritis (Alport Syndrome) OBSTRUCTIVE UROPATHY AND HYDRONEPHROSIS Thin Glomerular Basement Membrane Nephropathy RENAL TRANSPLANTATION Acute Postinfectious Glomerulonephritis MALIGNANT TUMORS OF THE KIDNEY Type I Membranoproliferative Glomerulonephritis Wilms’ Tumor (Nephroblastoma) Type II Membranoproliferative Glomerulonephritis Renal Cell Carcinoma (RCC) (Dense Deposit Disease) -
Congenital Anomalies of Kidney and Ureter
ogy: iol Cu ys r h re P n t & R y e s Anatomy & Physiology: Current m e o a t Mittal et al., Anat Physiol 2016, 6:1 r a c n h A Research DOI: 10.4172/2161-0940.1000190 ISSN: 2161-0940 Review Article Open Access Congenital Anomalies of Kidney and Ureter Mittal MK1, Sureka B1, Mittal A2, Sinha M1, Thukral BB1 and Mehta V3* 1Department of Radiodiagnosis, Safdarjung Hospital, India 2Department of Paediatrics, Safdarjung Hospital, India 3Department of Anatomy, Safdarjung Hospital, India Abstract The kidney is a common site for congenital anomalies which may be responsible for considerable morbidity among young patients. Radiological investigations play a central role in diagnosing these anomalies with the screening ultrasonography being commonly used as a preliminary diagnostic study. Intravenous urography can be used to specifically identify an area of obstruction and to determine the presence of duplex collecting systems and a ureterocele. Computed tomography and magnetic resonance (MR) imaging are unsuitable for general screening but provide superb anatomic detail and added diagnostic specificity. A sound knowledge of the anatomical details and familiarity with these anomalies is essential for correct diagnosis and appropriate management so as to avoid the high rate of morbidity associated with these malformations. Keywords: Kidney; Ureter; Intravenous urography; Duplex a separate ureter is seen then the supernumerary kidney is located cranially in relation to the normal kidney. In such a case the ureter Introduction enters the bladder ectopically and according to the Weigert-R Meyer Congenital anomalies of the kidney and ureter are a significant cause rule the ureter may insert medially and inferiorly into the bladder [2]. -
Congenital Renal Tract Abnormalities: a Neonatal Perspective
REVIEW © 2017 SNL All rights reserved Congenital renal tract abnormalities: a neonatal perspective Congenital renal and urinary system malformations are among the commonest congenital anomalies. They represent a wide spectrum of disorders with varying degrees of postnatal clinical significance. Many of these conditions can be detected on antenatal ultrasonography as part of the 18-20 week anomaly scan. This article will cover the postnatal management of renal dilatation and other commonly seen anomalies. 1 Pallavi Prasad he kidneys and urinary tract are the ■ Renal pelvic dilatation most common sites for congenital MBChB, MRCPCH, BMS BSc T ■ Congenital renal anomalies ST5 Paediatric Nephrology abnormalities and can account for 15-20% of all prenatally detected congenital • Renal agenesis 1 • Ectopic kidney Catherine Agar anomalies.1 Congenital renal and urinary - Pelvic kidney MBChB, MRCPCH system malformations are seen in 3-4% of - Crossed renal ectopia ST3 Community Paediatrics the population.2 - Horseshoe kidney Various conditions can result in renal Cath Harrison • Duplex kidney malformations seen on an antenatal BMedSci, BM BS, DTM&H, FRCPCH anomaly scan. Neonatal healthcare ■ Congenital cystic kidney disease Consultant Neonatologist1 and Consultant professionals should be aware of congenital • Multicystic dysplastic kidney (Neonatal Lead)2 renal abnormalities as early postnatal • Dysplastic kidney (renal dysplasia) ■ Posterior urethral valve (PUV) 1Leeds Teaching Hospitals NHS Trust intervention and appropriate long-term 2Embrace Transport Service follow-up will ensure prompt diagnosis FIGURE 1 Renal anomalies that are and management, avoid unnecessary commonly encountered in neonates. investigations and minimise renal damage. This article will review the postnatal management of the renal anomalies seen Kidney in FIGURE 1. -
UNILATERAL MEDULLARY SPONGE KIDNEY FIG. 1 Multiple Right
Case Report doi: 10.22374/jeleu.v1i1.12 UNILATERAL MEDULLARY SPONGE KIDNEY Rodrigo Neira S, Diego Barrera C, Gastón Astroza E Facultad de Medicina, Pontifi cia Universidad Católica de Chile, Santiago, Chile. Corresponding Author Gastón Astroza E, MD: gaeulufi @gmail.com Submitted: May 15, 2018. Accepted: July 5, 2018. Published: August 21, 2018. ABSTRACT A 40-year-old female with an allergy to medium contrast, presented with bilateral loin pain and renal colic type radiation. Image study with non-contrast computerized tomography and magnetic resonance imaging revealed multiple nephrolithiasis and a complex cyst at lower pole of the right kidney and no findings in the left one. The patient was treated with laser lithotripsy by flexible ureterorenoscopy revealing multiple lithiasis within cystic dilatations of the urothelium. The complex cyst was submitted to partial nephrec- tomy. Finally, biopsy revealed renal parenchyma with polycystic changes in the external corticomedullary area. All findings were compatible with unilateral medullary sponge kidney diagnosis. CLINICAL CASE A 40-year-old female patient presented with a his- tory of insulin resistance (treated with metformin), allergy to contrast medium, and maternal urolithia- sis. The patient consulted for bilateral loin pain and right renal colic pain. Non-contrast computerized tomography (NCCT) (Figures 1 and 2) demonstrated multiple right nephrolithiasis and a 3-cm hypodense right lower pole lesion with some calcifications. This was diagnosed as a complex cyst. Related to this cyst, a magnetic resonance imaging (MRI) of the abdo- men was requested which showed a 3.5 cm Bosniak 3 right renal cyst. Resolution of the lithiasis was decided by means of flexible ureterorenoscopy (fURS) with laser lithotripsy. -
Polycystic Horseshoe Kidney with Ruptured Stanford Type B Aortic Dissection
doi: 1053.446/actamednicomedia.944563 Cilt: 4 Sayı: 2 Haziran 2021 / Vol: 4 Issue: 2 June 2021 https://dergipark.org.tr/tr/pub/actamednicomedia Case Report | Olgu Sunumu POLYCYSTIC HORSESHOE KIDNEY WITH RUPTURED STANFORD TYPE B AORTIC DISSECTION POLİKİSTİK ATNALI BÖBREK İLE BİRLİKTE GÖRÜLEN RÜPTÜRE STANDFORD TİP B AORTİK DİSEKSİYON Özgür Çakır1 , Zeki Talas2, Demet Doğan3* 1Kocaeli University, School of Medicine, Departmant of Radiology, Kocaeli, Turkey. 2Kocaeli University, School of Medicine, Department of Cardiovascular Surgery, Kocaeli, Turkey. 3Tuzla State Hospital, Radiology, İstanbul, Turkey. ABSTRACT ÖZ 60-year-old male patient presented to the emergency clinic At nalı böbrek genellikle asemptomatiktir; ancak enfeksiyon, complaining of nausea, vomiting and abdominal pain with no üreter taşları ve malignite gibi nedenlerle semptomatik hale remarkable medical history except hypertension and active gelebilir. Literatürde, yetişkin polikistik böbrek hastalığı ile ilişkili smoking. The computed tomography (CT) revealed a Stanford B- eşzamanlı durumlar arasında karaciğerde yaygın kistik lezyonlar, type aortic dissection starting from the thoracic aorta to the iliac kolon divertikülü, kapak kalp hastalığı ve serebral arter anevrizması arteries and polycystic horseshoe kidneys with multiple cysts in the yer alır. Hipertansiyon ve aktif sigara içimi dışında kayda değer bir liver. Since polycystic horseshoe kidney and Stanford type B tıbbi öyküsü olmayan, 60 yaşında erkek hasta, bulantı, kusma ve dissection of the thoracic aorta is rare, and no study showing karın ağrısı şikayetleri ile acil polikliniğe başvurdu. Yapılan coexistence in the literature was found. This is the first case report Bilgisayarlı tomografi (BT) incelemede; torasik aorttan iliak that reveals the association of these clinical findings and CT images arterlere uzanım gösteren Standford tip B diseksiyon ve eşlik eden in literature. -
Congenital Anomalies of the Kidney Objectives
د.وليد نصار الجفال Lect.1 Congenital anomalies of the kidney Objectives: 1. To understand the presentations and management of congenital anomalies. Embryology of kidney and ureter The pronephros: derived from an intermediate plate of mesoderm. The mammalian pronephros is a transitory, nonfunctional kidney, analogous to that of primitive fish, the first evidence of pronephros is seen late in the third week, and it completely degenerates by the start of the fifth week. The mesonephros: functions from weeks 4and 8, and is associated with 2 duct systems of the mesonephric duct and, adjacent to this, the paramesonephric duct. The mesonephric (Wolffian) ducts develop laterally, and advance downwards to fuse with the primitive cloaca (hindgut).It is also transient, it serves as an excretory organ for the embryo while the definitive kidney, the metanephros, begins its development. The metanephros: The definitive kidney, or the metanephros, forms in the sacral region as a pair of new structures, called the ureteric buds, sprout from the distal portion of the mesonephric duct and come in contact with the condensing blastema of metanephric mesenchyme at about the 28th day. Branching of the ureteric bud forms the renal pelvis, calyces, and collecting ducts. Glomeruli and nephrons are created from metanephric mesenchyme. During weeks 6 and 10, the caudal end of the fetus grows rapidly and the fetal kidney effectively moves up the posterior abdominal wall to the lumbar region. Urine production starts at week 10 Thus, in both males and females, the mesonephric duct forms the ureters and renal collecting system. The paramesonephric essentially forms the female genital system (fallopian tubes, uterus, upper vagina); in males, it regresses.