Edit concept Question Editor Create issue ticket

Bilateral Multicystic Dysplastic Kidney


Presentation

  • […] the kidney caused by abnormal metanephric differentiation, occurring 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[pathologyoutlines.com]
  • The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.[orpha.net]
  • Weiner, Bernard Gonik, Caroline Crowther, and Stephen Robson present an evidence-based approach to the available management options, equipping you with the most appropriate strategy for each patient.[books.google.com]
  • Complications Occasionally an MCDK may become infected 2-3 or present with persistent mass effect or secondary hypertension 3.[radiopaedia.org]
  • Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery[icd10data.com]
Pneumonia
  • However, the chest radiograph showed normal lung expansion and there were few in homogenous radio-opaque shadows, suggesting congenital pneumonia. The respiratory distress subsided after four days and the neonate was weaned from ventilator.[ijcasereportsandimages.com]
Hydrops Fetalis
  • Stay at the forefront of your field thanks to new and completely revised chapters covering topics such as: Principles and Practice l Immune and Non-immune Hydrops Fetalis l Amniotic Fluid Volume l Enhancing Safe Prescribing in the Neonatal Intensive Care[books.google.com]
Waardenburg Syndrome
  • MCDK has been reported in association with Alagille syndrome, Beckwith-Wiedemann syndrome, Branchio-oto-renal (BOR) syndrome, Joubert syndrome, Trisomy 18, Waardenburg syndrome type 1, and Renal coloboma syndrome.[ijcasereportsandimages.com]
  • Multicystic dysplastic kidney associated with Waardenburg syndrome type 1. Pediatr Nephrol. 1997 Dec. 11(6):744-5. [Medline]. Javadpour N, Chelouhy E, Moncada L, et al. Hypertension in a child caused by a multicystic kidney.[emedicine.medscape.com]
  • Multicystic dysplastic kidney associated with Waardenburg syndrome type 1. Pediatr Nephrol . 1997 Dec. 11(6):744-5. [Medline] . Javadpour N, Chelouhy E, Moncada L, et al. Hypertension in a child caused by a multicystic kidney.[emedicine.medscape.com]
Headache
  • Later, symptoms include Pain in the back and lower sides Headaches Blood in the urine Doctors diagnose PKD with imaging tests and family history. There is no cure. Treatments can help with symptoms and complications.[icdlist.com]

Workup

Karyotype Abnormal
  • No karyotype abnormalities were found in fetuses with unilateral MCDK if an associated non-renal abnormality was not found. The ratio of male to female fetuses was 2.4:1. Female fetuses were twice as likely to have bilateral MCDK as males.[fetalsono.com]

Treatment

  • […] which the hospitals are located, and check the cost of treatment.[vaidam.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]
  • Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout.[books.google.com]
  • Prepare for clinical challenges and save time in addressing them thanks to expert advice on treatment options from international contributors.[books.google.com]

Prognosis

  • Postnatal/Prognosis? The majority of MCDK are unilateral and have a good prognosis. Cases with bilateral MCDK that survive the pregnancy pass away shortly after birth.[prenatalpediatrics.org]
  • This information is of value in determining fetal prognosis, in deciding the method of delivery, and in alerting the pediatricians to supportive and corrective measures which may need to be taken in the postnatal period.[link.springer.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]
  • 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]
  • Treatment and prognosis A normal life expectancy can be expected as long as the contralateral kidney is normal. Bilateral MCDK is fatal.[radiopaedia.org]

Etiology

  • 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]
  • Etiology • Atresia of the proximal ureter or PUJ during the metanephric phase of intrauterine development. 4.[slideshare.net]
  • Review paper about unilateral renal agenesis Ongoing controversies regarding etiology, diagnosis, treatment Long-term follow-up studies of patients with renal hypoplasia/dysplasia/agenesis are needed to provide insight into prognostic factors identified[clinicaladvisor.com]
  • A population study of the VACTERL association: evidence for its etiologic heterogeneity. Pediatrics . 1983 May. 71(5):815-20. [Medline] . Kiprov DD, Colvin RB, McCluskey RT.[emedicine.medscape.com]

Epidemiology

  • 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]
  • 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]
  • […] 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]
  • 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.[rarediseases.info.nih.gov]
  • External ear malformations: epidemiology, genetics, and natural history. Birth Defects Orig Artic Ser . 1979. DA - 19800417(9):i-ix, 1-140. [Medline] . Mesrobian HG, Rushton HG, Bulas D.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • Chapters about glomerulonephritis, nephrotic syndrome, and tubular disorders provide an orientation in the pathophysiology, differential diagnosis, and treatment of these heterogeneous disease entities.[books.google.it]
  • Pathophysiology A brief overview of nephrogenesis is provided to highlight the developmental defects that lead to multicystic dysplastic kidney.[emedicine.medscape.com]
  • Improved understanding of pathophysiologic mechanisms has led to development and testing of novel drugs for some of these diseases.[appliedradiology.com]

Prevention

  • 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]
  • Treatment is focused on preventing progression to stage 5, end-stage renal disease.[clinicaladvisor.com]
  • Prevention: There is no way to prevent MCDK from occurring early in a baby's development, and there is no known way to prevent it from happening later in life. Outlook: A baby with MCDK and no other birth defects has a normal life expectancy.[healthychildren.org]
  • The role of nephrectomy is controversial and is indicated to treat or prevent complications like abdominal or flank pain, UTI, hypertension, or renal malignancy.[ijcasereportsandimages.com]
  • Noe et al came to the same conclusion quite independently with a number needed to treat as 2000 to prevent the occurrence of one Wilms tumor (11).[pediatricurologybook.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!