Autosomal Dominant Polycystic Kidney disease (ADPKD) & Pyelonephritis

8/10/2019 Autosomal Dominant Polycystic Kidney disease (ADPKD) & Pyelonephritis

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Tuesday urology conference

2/12/2014

By Dr. Anas HindawiMGH PGY 3 Urology Resident


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Vitals T: 38 ,P 90 ,BP 150/90

P.E :

Chest : bilat. basal crackles

Abdomen : soft ,enlarged

Palpable bilat. Mildly tender masses

Bilateral CVA tenderness /more on the left/

DRE normal

Lower extremeties +2 pitting oedema

Labs :

Bun41 ,Cr3 ,electrolytes140 ,3.8 ,104 ,21

Hg/Hct11.5/35.6 ,MCV 82

U/Aturbid amber ,+1 LE ,6-8 rbc ,numerous wbc

U/CxEcoli /ESBL/ > 100.000 CFU ,Sensitive on Amikacin & Imipinem


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Next ?


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Next ?

IV Antibiotics

percutaneous drainage /unroofing/

partial nephrectomy

simple unilateral nephrectomy

radical unilateral nephrectomy

bilateral nephrectomy


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ADULT POLYCYSTIC KIDNEY

DISEASE

Adult polycystic kidney disease is an autosomal dominant hereditarycondition /bilateral in 95% /

5% to 10% of ADPKD cases are due to spontaneous mutations without apositive family history

ADPKD 3rdleading cause for ESRD

Accounting for 5% to 10% of all dialysis cases

Affecting chromosomes 16 and 4

Do not appear until after age 40 ,no preference of sex or race

May be associated with cysts in other organs

Risk of renal cell carcinoma is the same of general population

Smith and Tanagho's General Urology, 18th EditionAdult Renal Cystic Disease: Current Update on Pathogenesis, Cross sectional Imaging Findings and Management

AUA update series 2013 ,volume 32 ,lesson 2


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Adult Renal Cystic Disease: Current Update on Pathogenesis, Cross sectional Imaging Findings and

Management


Pathogenesis

It is now established that hereditary renal cysticdiseases, so-called ciliopathies, are secondary togenetic abnormalitiesthat influence the formationandfunctionof primary ciliaof the renal tubular epithelialcells resulting in inappropriate epithelial proliferation andsubsequent development of renal cysts.


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Clinical findings

Symptoms:

Pain over one or both kidneys

Gross or microscopic total hematuria

Colic may occur if blood clots or stones arepassed

Abdominal mass Infection

Vesical irretability

Smith and Tanagho's General Urology, 18th Edition


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Clinical findings

Signs:

Palpable kidney/s with or without nodularity

Tenderness justified by infection

HTN in 60-70 % Cardiomegaly

Fever with pyelonephritis or infected cyst

Uremia ,anemia and weight loss

Laboratory Findings hematopoieticdepression accompanying uremia,proteinuriaandmicroscopic (if not gross) hematuriawith developping renalimpairment



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Clinical findings

X-Ray Findings

Enlarged on a plain film of the abdomen, even as much as five times normal The calyces are broadened and flattened, enlarged, and often curved, as theytend to hug the periphery of adjacent cysts.

Ultrasonography 1smethod of investigation non expensive ,safe and effective

CT scanning ADPKD is characterized by bilateral enlarged kidneys with innumerable cysts of

various sizes ,in addition complications related to ADPKD, such as cyst infection,hemorrhage or rarely cyst rupture

Extrarenal manifestations of ADPKD including hepatic, pancreatic, seminal

vesicle and splenic cysts; intracranial arterial aneurysms; aortic aneurysms;abdominal wall hernias; colonic diverticulosis; and aortic/mitral valve abnormalitiescan be diagnosed

Adult Renal Cystic Disease: Current Update on Pathogenesis, Cross

sectional Imaging Findings and Management




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Complications of ADPKD

Refractory HTN

Chronic pain

Stones

Tumour

Cysts rupture Pyelonephritis /most common complication/

Intracranial Berry aneurysms rupture

Campbell walsh urology 10th edition


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General mesaures

the new drugs target fluid secretion, whereasothers target cellular growth and proliferation of

the cysts

ACEI's & ARB's

NSAID's has to be avoided

Narcotics has to be limited



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Surgical managment of ADPKD

There is no evidence that excision or decompression of cystsimproves renal function

In patients infected with gas forming organisms

Recurrent, recent or refractory infections who are about to undergorenal transplantation ,In this setting, nephrectomy will minimize therisk of post-transplant infection when immunosuppressive agentsare administered to prevent rejection .

Patients with a staghorn calculus causing recurrent UTIs in a

relatively nonfunctioning kidney.

Hemodialysis and ultimately renal transplantation is the treatment ofchoice for patients with ADPKD and renal failure

Uptodate :Urinary tract infection in autosomal dominant polycystic

kidney disease ,literature review by Mar 2013


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Pyelonephritis & ADPKD

30 % to 50 % of ADPKD Distinguishing between infections of the bladder, renal parenchyma, and cystsis important

Often leads to serious complications, including perinephric abscess,septicemia, and death.

Predisposing factors include age, female sex, and recent instrumentation ofthe urinary tract.

Most commonly caused by gram-negative enteric organisms.

Diagnosis of these infections may be difficult since some patients do not havebacteriuria.

Eradication of cyst infections with conventional antibiotic therapy can be difficultdespite in vitro sensitivity of responsible organisms to the agents administered.

Where fever persists beyond 1-2 weeks of appropriate antibiotic therapy,percutaneous or surgical drainageof infected cysts should be considered.Where there is end-stage disease, nephrectomy may be indicated

American Journal of Kidney Diseases ,Volume 10, Issue 2, August 1987, Pages 8188

Polycystic Kidney Disease Treatment & Managemen ,Author: Roser Torra, MD, PhD; Chief

Editor: Vecihi Batuman, MD, FACP, FASN


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References



Urology Board review 4th edition

Adult Renal Cystic Disease: Current Update on Pathogenesis, Cross sectional Imaging Findingsand Management ,AUA update series 2013 ,volume 32 ,lesson 2

Uptodate :Urinary tract infection in autosomal dominant polycystic kidney disease ,literature

review by Mar 2013

American Journal of Kidney Diseases ,Volume 10, Issue 2, August 1987, Pages 8188

Pubmed : Polycystic Kidney Disease Treatment & Managemen ,Author: Roser Torra,

MD, PhD; Chief Editor: Vecihi Batuman, MD, FACP, FASN


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Thank you

Documents

Autosomal Dominant Polycystic Kidney disease (ADPKD) & Pyelonephritis