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CUONG NGUYEN, MSIV. 34 YO WHITE FEMALE WITH ABDOMINAL PAIN. OSUMC Diagnostic Radiology OCTOBER 15, 2010. HPI. 34 year old White female who presented to the hospital with a complaint of abdominal pain and fever x 2 days. (+) Decreased appetite (-) Nausea, vomiting, diarrhea.

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CUONG NGUYEN, MSIV

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Cuong nguyen msiv

CUONG NGUYEN, MSIV

34 YO WHITE FEMALE

WITH

ABDOMINAL PAIN

OSUMC Diagnostic Radiology

OCTOBER 15, 2010


Cuong nguyen msiv

HPI

  • 34 year old White female who presented to the hospital with a complaint of abdominal pain and fever x 2 days.

  • (+) Decreased appetite

  • (-) Nausea, vomiting, diarrhea


Cuong nguyen msiv

  • Internal medicine was consulted and initially started her on Rocephin 1g qd and did urine and blood cultures

  • Patient continued to have fever on Rocephin so a CT of the abdomen and pelvis was ordered


Cuong nguyen msiv

  • Dx: Autosomal dominant polycystic kidney disease(ADPK)

  • Renal cyst infection secondary to UTI was suspected

  • IR placed a catheter into a cyst and drained purulent and bloody fluid.

  • Fluid culture grew E. Coli sensitive to ciprofloxacin

  • Patient was then started on IV ciprofloxacin and her fever and abdominal pain resolved


Autosomal dominant polycystic kidney disease adpkd

Autosomal Dominant Polycystic Kidney Disease(ADPKD)

  • Most common inherited renal disorder

  • Characterized by the formation and progressive enlargement of renal cysts which which are destructive to renal parenchyma and often lead to renal failure (ESRF) in late middle age

  • Autosomal dominant!!!!!!!

  • Affects 1/400-1000 people in the US

  • Accountable for 10-12% all ESRD patients receiving hemodialysis

  • 200-400 thousand persons with ADPKD in US

  • ~ 600 new cases per year


Autosomal dominant polycystic kidney disease adpkd1

Autosomal Dominant Polycystic Kidney Disease(ADPKD)

  • Cyst formation probably begins in utero and symptoms may rarely be present in newborns

  • Cysts initially involve only portions of the nephrons

  • Asymptomatic and renal function is retained until 4th- 5th decade of life

  • Almost all patients experience renal failure by 80 years of age


Clinical presentation

Clinical Presentation

  • Palpable mass

  • Abdominal pain

  • Gross hematuria

  • Hypertension

  • Renal failure


Complications

Complications

  • Intracystic hemorrhage

  • Renal stones

  • Chronic pain symptoms

  • UTI

  • Cyst infection


Extrarenal manifestations of adpkd

Extrarenal Manifestations of ADPKD

  • Cerebral aneurysms (20%)

  • Hepatic cysts (60%)

    • From biliary epithelium

  • Pancreatic cysts (10%)

  • Cardiac

    • Mitral valve prolapse, bicuspid aortic valve, aortic aneurysms, aortic dissections

  • Colonic diverticula

  • Abdominal wall and inguinal hernia


Causes of death

Causes of death

The main causes of death in ADPKD are:

  • Uremia (ESRD)

  • Atherosclerosis affecting coronary, intracranial arteries

  • Sudden rupture of intracranial aneurysms

  • Sepsis


Gross appearance

Gross appearance


Pathophysiology

Pathophysiology

  • Caused by mutations of PKD1 and PKD2 genes on chromosomes 16 and 4 respectively

  • PKD1 codes for Polycystin-1 protein – function not known; thought to be involved in cell-cell, cell-matrix interactions.

  • PKD2 codes for Polycystin-2 protein – cation channel involved in regulation of intracellular Ca2+ level.


Pathophysiology1

Pathophysiology

  • These defects result in altered tubular epithelial growth and differentiation

    • Abnormal extracellular matrix

    • Increased cell proliferation

    • Increased fluid secretion

  • Formation of cysts

  • PKD1 mutations are more common and account for 90% of all ADPKD cases

  • PKD1 associated with earlier onset renal failure


Differentials

Differentials

  • Autosomal recessive polycystic kidney disease

  • Multiple simple cysts

  • Von Hippel-Lindau (VHL) disease

  • Tuberous sclerosis

  • Acquired uremic cystic kidney disease (hemodialysis)

  • Medullary sponge kidney

  • Multicystic dysplastic kidney


Diagnosis ultrasonography

Diagnosis: Ultrasonography

  • Ultrasonography is the most widely used technique to diagnose ADPKD

  • Findings: Diffuse hyperechogenicity, enlarged kidneys, and cysts, usually bilateral

  • Can detect cysts 1 – 1.5 cm

  • 99% sensitivity for at risk patients >20 yo

    • Higher false negatives in people < 20yo

  • Not recommended as a routine diagnostic procedure in patients < 14yo


Diagnosis ultrasonography1

Diagnosis: Ultrasonography

  • No exposure to radiation or contrast material

  • Inexpensive

  • Also useful for evaluating extra-renal cysts in the abdomen (liver, pancreas)


Cuong nguyen msiv

FIGURE 36.48. Autosomal Dominant Polycystic Disease. The kidney of a 57-year-old patient with a family history of cystic renal disease shows replacement of the renal parenchyma with innumerable cysts of varying size. Both kidneys were greatly enlarged.


Diagnosis computed tomography ct

Diagnosis: Computed tomography (CT)

  • More sensitive than US

  • Can detect smaller cysts (0.5cm)

  • Involves radiation exposure

  • More expensive

  • Not used routinely for diagnosis or follow up studies

  • More useful in unclear or more complicated cases in children


Diagnosis mri

Diagnosis: MRI

  • More sensitive than either US or CT

  • More useful in distinguishing renal cell carcinoma from cyst

  • Used to monitor kidney size after treatment to assess progress

  • Not routinely used due to its high cost


Diagnosis intravenous urography

Diagnosis: Intravenous Urography

  • Used to be a widely used technique to diagnosed ADPKD

  • Involves contrast (nephrotoxic)

  • Only helpful in the diagnosis of advanced ADPKD with distortion of renal calyces

  • No longer indicated


Diagnostic criteria

Diagnostic criteria

  • At risk patients <30 yo

    • at least 2 renal cysts (unilateral or bilateral)

  • 30-59 yo

    • At least 2 cysts in each kidney

  • 60+ yo

    • At least 4 cysts in each kidney


Treatment

Treatment

  • Treatment is primarily supportive care

  • Control of HTN is important to slow disease progression

    • ACE-I, ARB

  • Treating complications of renal failure

    • Hyperkalemia, hyperphosphatemia, hypocalcemia, acidosis

  • Treating UTI and renal cyst infections

  • Avoidings nephrotoxic agents (NSAIDS)


Cuong nguyen msiv

Treatment

  • Surgical drainage and decompression of large cysts is effective for pain relief

  • ESRD patients require hemodialysis or renal transplantation


References

References

  • E., William, and Clyde A. Fundamentals of diagnostic radiology. 3rd ed. Lippincott Williams & Wilkins, 2007. 949-950

  • D. Ravine, et al. Evaluation of ultrasonographic diagnostic criteria for autosomal dominant polycystic kidney disease 1. The Lancet. Volume 343, Issue 8901, 2 April 1994, Pages 824-827.

  • Alkis M. Pierides, et al. Autosomal dominant polycystic kidney disease—type 2. Ultrasound, genetic and clinical correlations. Nephrol. Dial. Transplant. (2000) 15 (2): 205-211.

  • Emedicine.com. (2010). Polycystic kidney disease. Retrieved: October 10, 2010 from http://emedicine.medscape.com/article/244907-overview

  • Emedicine.com. (2008). Autosomal Dominant Polycystic Kidney Disease. Retrived: October 9, 2010 from http://emedicine.medscape.com/article/376995-imaging

  • Torres VE, Harris PC, Pirson Y. Autosomal dominant polycystic kidney disease. Lancet. 2007;369(9569):1287-1301.


Thank you

THANK YOU !!!


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