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Renal Dysplasia, Bilateral


Presentation

  • Most of the patients were adults, and the most common clinical presentation in them was loin pain. The younger patients presented with occasional loin pain, pyrexia and hematuria.[cags.org.ae]
  • The major clinical presentations are marked protruberances and abdominal cysts.[bmcurol.biomedcentral.com]
  • In addition, the histological, immunofluorescent, immunohistochemical and ultrastructural features of the major glomerular diseases and renal transplantation pathology are presented.[books.google.de]
  • Both kidneys presented primitive appearance featuring dysplasia. Dysontogenic metaplasia in urinary tract feline with renal dysplasia, has not been described. Keywords Congenital disease; Malformation; Nephropathy. DOI: Semina: Ciênc. Agrár.[uel.br]
  • In severe cases microcysts or macrocysts can be present. Dys- plastic changes are often focal, may be unilateral or bilateral, and can appear in a solitary kidney.[link.springer.com]
Pain
  • While our pain is almost unbearable, knowing her was more joyous than anything this world could ever offer.”[academicdepartments.musc.edu]
  • Most of the patients were adults, and the most common clinical presentation in them was loin pain. The younger patients presented with occasional loin pain, pyrexia and hematuria.[cags.org.ae]
  • More than half of patients with ADPK experience pain, which can be intense, sudden and last a long time. The pain can be from kidney stones, blood clots, or an infection. Treating the pain depends on its cause.[urologyhealth.org]
  • DYSPLASIA, BILATERAL; MCRD, PELVIURETERIC JUNCTION OBSTRUCTION; PUJO, URETEROPELVIC JUNCTION OBSTRUCTION; UPJO Classification developmental, genetic, renal Phenotypes Autosomal dominant inheritance ; Congenital megaureter ; Cystic renal dysplasia ; Flank pain[mousephenotype.org]
  • Perform nephrectomy if the mass is causing symptoms (flank pain, infections, hypertension) or if the mass is suspicious for malignancy. Index: 1–9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z References Hains, D. S.; Bates, C.[urology-textbook.com]
Fever
  • He presented with fever, failure to thrive, abdominal distension, and loose stools with non bilious vomiting. He was found to be dehydrated and febrile with the left kidney palpable, and mild hepatomegaly was felt.[cags.org.ae]
  • Babies and children with UTIs may become irritable, have a fever, have pain on weeing, feel sick or be sick. If your child has a UTI, he or she will need to take antibiotics, medicines that kill the germs.[infokid.org.uk]
Recurrent Infection
  • Obstruction and severe reflux may be corrected surgically when indicated (if causing hypertension, recurrent infections, or renal growth retardation).[msdmanuals.com]
Vomiting
  • He presented with fever, failure to thrive, abdominal distension, and loose stools with non bilious vomiting. He was found to be dehydrated and febrile with the left kidney palpable, and mild hepatomegaly was felt.[cags.org.ae]
  • Some are big enough and can push on the stomach and diaphragm and cause vomiting and difficulty breathing, respectively. In these cases removal of the kidney surgically may be required.[urologyhealth.org]
  • In children, it normally presents with pain and vomiting. A significant number will require intervention - some do not present with functional obstruction until adulthood.[patient.info]
Abdominal Distension
  • He presented with fever, failure to thrive, abdominal distension, and loose stools with non bilious vomiting. He was found to be dehydrated and febrile with the left kidney palpable, and mild hepatomegaly was felt.[cags.org.ae]
  • Indications for Surgery Large MCDK causing abdominal distension, discomfort, pain or respiratory compromise in the first few months of life Tumors that continue to increase in size Hypertension Failure to regress is considered by some as a relative indication[pediatricurologybook.com]
Bilious Vomiting
  • He presented with fever, failure to thrive, abdominal distension, and loose stools with non bilious vomiting. He was found to be dehydrated and febrile with the left kidney palpable, and mild hepatomegaly was felt.[cags.org.ae]
Diarrhea
  • So, if the child has diarrhea or poor fluid and food intake or both, the kidney is not able to reabsorb most of the salt and water filtered and to reduce the amount of urine, as is a normal renal response.[cybernephrology.ualberta.ca]
Abdominal Pain
  • It frequently presents with haematuria, with symptoms of infection and abdominal pain. Abnormalities of the bladder [ 14 ] Bladder exstrophy This is absence of the anterior wall of the bladder, with ureters delivering urine into the lower abdomen.[patient.info]
Hepatomegaly
  • He was found to be dehydrated and febrile with the left kidney palpable, and mild hepatomegaly was felt.[cags.org.ae]
Vascular Disease
  • This is followed by a comprehensive survey of developmental and cystic kidney diseases, vascular diseases and tubulointerstitial diseases. An emphasis is placed on gross diagnostic findings with detailed histological correlates.[books.google.de]
Narrow Face
  • Other abnormalities that have been found in association with BOR syndrome are narrowing of the tear duct in the eyes interfering with the normal flow of tears; a long narrow face; cleft palate; paralysis of certain muscles in the face; and/or a deep overbite[rarediseases.org]
Kidney Failure
  • Failure 1619 Pathophysiology of Progressive Renal Disease 1631 Management of Chronic Kidney Disease 1661 Handling of Drugs in Children with Abnormal Renal Function 1693 Endocrine and Growth Disorders in Chronic Kidney Disease 1713 Chronic Kidney Disease[books.google.de]
  • About half of people with the most common type of PKD end up with kidney failure. PKD also causes cysts in other parts of the body, such as the liver. Often, there are no symptoms at first.[icdlist.com]
  • It is very rare for a baby to be born with kidney failure. Occasionally, a young baby or child has kidney failure.[infokid.org.uk]
  • Signs in babies are kidney failure and breathing problems. Signs in children older than 1 year are high blood pressure or enlarged kidneys.[urologyhealth.org]
  • Over time, if these problems are not corrected, they can damage the one working kidney and lead to total kidney failure.[medicinenet.com]
Flank Pain
  • DYSPLASIA, BILATERAL; MCRD, PELVIURETERIC JUNCTION OBSTRUCTION; PUJO, URETEROPELVIC JUNCTION OBSTRUCTION; UPJO Classification developmental, genetic, renal Phenotypes Autosomal dominant inheritance ; Congenital megaureter ; Cystic renal dysplasia ; Flank[mousephenotype.org]
  • Perform nephrectomy if the mass is causing symptoms (flank pain, infections, hypertension) or if the mass is suspicious for malignancy. Index: 1–9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z References Hains, D. S.; Bates, C.[urology-textbook.com]
  • He had no history of urinary tract infection or flank pain. The abdominal examination showed a defined cystic abdominal mass with a smooth surface measuring 15   10 cm.[bmcurol.biomedcentral.com]
  • Signs of cystic kidney disease getting worse are: Flank pain Blood in the urine Urinary tract infections Kidney disease may also progress with no clear signs. Your urologist will recommend regular follow-up tests based on your kidney status.[urologyhealth.org]
  • The brother (Subject III-2) of the index patient (Subject III-3) had a poorly functioning left kidney with a large cyst of the renal pelvis, discovered during an investigation of flank pain.[nejm.org]
Renal Impairment
  • Also, a physician specializing in kidney problems (nephrologist) should closely monitor any renal impairment. Surgical repair may be undertaken for correctable defects. Severe kidney problems may warrant dialysis or kidney transplantation.[rarediseases.org]
  • Signs and symptoms of bilateral renal hypoplasia/dysplasia depend on the severity of renal impairment. Commonly, these patients present with various stages of chronic renal disease.[clinicaladvisor.com]
Dysuria
  • The animal had history of abdominal enlargement since birth and dysuria, eliminating urine only dropwise. Due to the poor prognosis we opted for euthanasia.[uel.br]

Workup

Small Kidney
  • Other phenotypes include renal duplication, small kidneys, ureteropelvic junction obstruction, hydronephrosis, and renal dysplasia. These abnormalities can result in kidney damage, and possibly renal failure (summary by {19:Vivante et al., 2015}).[monarchinitiative.org]
  • Variants renal adysplasia (association unilateral renal agenesis and controlateral renal dysplasia) hypoplastic renal dysplasia (small kidneys with extensive renal dysplasia) aplastic renal dysplasia (association of a renal dysplasia and unilateral renal[humpath.com]
  • If a child's kidneys are very small, kidney function may decrease as the child enters teenage years. Kidney failure may occur, which would call for supportive measures.[urologyhealth.org]
  • Hypoplasia usually occurs because inadequate ureteral bud branching causes an underdeveloped, small kidney with histologically normal nephrons. If hypoplasia is segmental, hypertension can occur, and ablative surgery may be needed.[msdmanuals.com]
Hyponatremia
  • Investigations revealed hyponatremia (125mmol/l), hyperkalemia (6.5mmol/l), urea of 9.4mmol/l, creatinine of 53mmol/l, and bicarbonate of 15mmol/l.[cags.org.ae]
  • Hyponatremia is frequent in the presence of polyuria. Prompt sodium chloride supplementation is essential. · Hyperkalemia due to reduced glomerular filtration, reduced tubular secretion, and associated metabolic acidosis can be life-threatening.[clinicaladvisor.com]
Abnormal Renal Function
  • Renal Function 1693 Endocrine and Growth Disorders in Chronic Kidney Disease 1713 Chronic Kidney Disease Mineral and Bone Disorder 1755 Peritoneal Dialysis 1785 Hemodialysis 1817 Transplantation Immunobiology 1835 Pediatric Kidney Transplantation 1867[books.google.de]

Treatment

  • Compared with congenital megaureter, the treatment of CGM is specific.[bmcurol.biomedcentral.com]
  • Usually, the cysts are harmless and do not need treatment. If they do cause complications, treatments include medicines, draining the cysts, or surgery.[icdlist.com]
  • Treatments for CKD There's no cure for CKD, but treatment can help relieve the symptoms and stop it getting worse. Your treatment will depend on how severe your kidney disease is.[nhs.uk]
  • Dialysis is a treatment that filters and purifies the blood using a machine. This helps keep your body in balance when the kidneys can’t do their job. Factors such as lung development and overall health determine the success of this treatment.[healthline.com]
  • Treatment In most cases, there is no treatment needed before birth. Options Very rarely, there is concern that the problems with the baby’s kidneys and/or lungs are very severe, and may mean that he or she will not be able to live a normal life.[infokid.org.uk]

Prognosis

  • Due to the poor prognosis we opted for euthanasia. At necropsy was observed enlarged and distended bladder, reduced kidneys and dilated and tortuous ureters.[uel.br]
  • Therefore, the clinical features and long-term prognosis of eight patients with bilateral renal dysplasia have been evaluated.[karger.com]
  • Prognosis is highly unpredictable due to an inability to quantify residual functional parenchyma. Treatment is surgical correction of any associated GU abnormalities and, if renal insufficiency or renal failure is present, renal replacement therapy.[merckmanuals.com]
  • In almost half of the cases of unilateral MCDK, the contralateral kidney will have associated malformations, the severity of which determines the overall prognosis.[mhmedical.com]
  • The multicystic dysplastic kidney has an excellent long-term prognosis, and an affected patient will most likely have one normally functioning kidney for the remainder of their life.[nationwidechildrens.org]

Etiology

  • Etiology The etiology is unclear, there are several theories leading to a multicystic dysplastic kidney: Fetal ureteral atresia Abnormal induction of the metanephritic blastema by the ureteral bud.[urology-textbook.com]
  • Etiology Clinical features Diagnostics Ultrasound imaging of the kidneys and bladder First-line imaging modality Detects anatomical abnormalities Renal ultrasound is warranted for all children with recurrent UTIs or UTI in children 24 months of age.[amboss.com]
  • Etiology MCDK results from disrupted nephrogenesis but the exact pathogenic mechanism is still unknown. Disturbed formation of nephrons could result from impaired fetal urine flow early in development.[orpha.net]
  • […] concerning dermatopathology and pathology Atlas of fetal pathology Congenital malformations Physiological developement of an embryo and fetus, growth and changes of the external shape Congenital malformations and congenital anomalies Basic terminology Etiology[atlases.muni.cz]

Epidemiology

  • Nephrotoxins 1275 Urinary Tract Infections 1299 Vesicoureteral Reflux and Renal Scarring 1311 Obstructive Uropathy 1337 Bladder Dysfunction in Children 1379 Urolithiasis 1405 Pediatric Renal Tumors 1431 Epidemiology of Hypertension 1459 Pathophysiology[books.google.de]
  • In severe cases MCDK can lead to neonatal fatality (in bilateral cases), however in unilateral cases the prognosis might be better (it would be dependent on associated anomalies). [11] [12] Epidemiology [ edit ] In regard to the epidemiology of multicystic[en.wikipedia.org]
  • Epidemiology Multicystic renal dysplasia is the most common abdominal tumor in the neonatal period and the most common cystic lesion of the kidney in childhood. Incidence 1 of 4300 live birth.[urology-textbook.com]
  • […] by week 20 in utero Enlarged, cystic kidneys with disorganized parenchyma and numerous cysts Immature tubules surrounded by mesenchymal collars and islands of immature mesenchyme are present between the cysts Also known as multicystic renal dysplasia Epidemiology[pathologyoutlines.com]
  • Summary Epidemiology The prevalence and incidence of bilateral MCDK are unknown. Clinical description Bilateral MCDK may be suspected antenatally at routine ultrasound scans, with the majority detected around the 20th week of gestation.[orpha.net]
Sex distribution
Age distribution

Pathophysiology

  • Nephrotoxins 1275 Urinary Tract Infections 1299 Vesicoureteral Reflux and Renal Scarring 1311 Obstructive Uropathy 1337 Bladder Dysfunction in Children 1379 Urolithiasis 1405 Pediatric Renal Tumors 1431 Epidemiology of Hypertension 1459 Pathophysiology[books.google.de]
  • If the mother had been taking certain prescription drugs such as those for hypertension, this may be a precipitating factor as well. [8] Ultrasound Scan -Vesicoureteral reflux Pathophysiology [ edit ] The mechanism of multicystic dysplastic kidney is[en.wikipedia.org]
  • Pathophysiology A brief overview of nephrogenesis is provided to highlight the developmental defects that lead to multicystic dysplastic kidney.[emedicine.medscape.com]

Prevention

  • How can kidney dysplasia be prevented? Researchers have not found a way to prevent kidney dysplasia caused by genetic factors or certain genetic syndromes.[niddk.nih.gov]
  • There is no way to prevent multicystic dysplastic kidney from occurring early in a baby's development, and there is no known way to prevent it from happening.[nationwidechildrens.org]
  • For this reason, early identification of these malformations is essential in order to prevent renal damage.[amboss.com]
  • Treatment is focused on preventing progression to stage 5, end-stage renal disease.[clinicaladvisor.com]
  • […] cysts develop in the kidneys blockages in the flow of urine – for example, from recurrent kidney stones or an enlarged prostate long-term, regular use of certain medicines – such as lithium and non-steroidal anti-inflammatory drugs (NSAIDs) You can help prevent[nhs.uk]

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