polycystic kidney by santosh
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Transcript of polycystic kidney by santosh
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DR.M.SANTOSH1ST YR PG
RADIODIAGNOSIS
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A 60 Yr old male patient reported with
c/o increased frequency of micturition
since 1 month.
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AUTOSOMAL DOMINANT POLYCYSTIC
KIDNEY DISEASE (ADPKD)
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Cystic Diseases of the Kidney
1)von Hippel-Lindau Disease
2)Acquired renal cystic disease3)Autosomal recessive polycystic kidney
disease
4)Medullary cystic disease
5)Renal dysplasia6)Simple renal cysts
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is a cystic genetic disorder of the kidneys.Thereare two types of PKD:
Autosomal Dominant Polycystic Kidney
Disease(ADPKD) and the Autosomal Recessive Polycystic Kidney
Disease(ARPKD).
The cysts are numerous and are fluid-filled
resulting in massive enlargement of the kidneys. The disease can also damage the liver, pancreas,
and in some rare cases, the heart and brain.
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ADPKD:
One of the most common genetic life-threatening
disease (1 in every 400 to 1000 people)
Often without symptoms (Only 1/2 will be diagnosed)
ARPKD
Rare (1 in 10,000 to 20,000 people)
Childhood polycystic kidney disease
Typically diagnosed in infancy,; less severe forms may
be diagnosed later in childhood or adolescence.
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10% of all transplant/dialysis patients
ADPKD-1 gene (polycystin) mutation
85%
Bilaterally enlarged kidneys (>3kg)
Symptoms appear in adult life
Kidney failure 5-10 years thereafter
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PKD1: Abnormality on chromosome 16:
(85% of families with ADPKD)
PKD2: Abnormality on chromosome 4:
(15% of families with ADPKD)
Cysts and kidney failure occur at an earlier
age in PKD1 disease
ESRD: 57 vs. 69 years of age
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In approximately 25-40% of cases, ADPKD
occurs in people without a family history of
the disease.
New mutation
More frequently, particularly in families
without PKD1, it is a disease that
progresses slowly and never causessymptoms
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Frequency increases with age: 4% of young adultsvs. 10% in older patients
Family history of aneurysm or SAH: the highest riskof forming an aneurysm.
xEarly diagnosis is recommended in peoplewho are at high-risk.x
Screening (CT scan or MRA)
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Only for high-risk patientsx Positive family history of a brain hemorrhagex Warning symptomsx Occupation (pilot), in whom loss of consciousness would
place patient or others at extreme risk
Screening of low-risk patients is not recommendedx Aneurysms are rare in this groupx Most aneurysms found have a low risk of rupturex There is a high risk of severe neurologic complications
associated with corrective surgeryx Most low-risk patients would not derive any benefit from
finding an aneurysm, since surgery for a small aneurysmwould not be recommended
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Management of aneurysms: > 7 mm: high risk of rupture (up to 2% /
year)xSurgeryxPlacing coil within aneurysm reduces risk
of rupture
Smaller aneurysms that do not causesymptoms are much less likely to ruptureand are not corrected, except in peoplewith history of bleeding aneurysm
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Common: 30 40% of people younger than 30; > 80% ofpeople over 60
More common in people with advanced kidney disease Incidence similar in men and women, but very large cystsoccur almost exclusively in women
More common in women who have had several pregnancies Most people have no symptoms and have normal or near-
normal liver function
Some develop pain: may require drainage if it ispersistent and severe
Cyst infection (which requires antibiotic therapy and,in some cases, drainage)
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Heart valve disease: (25 30%) Mostly without symptoms May progress over time (may require valve
replacement)
Colonic diverticula (outpocketing that form in wall ofcolon) Diverticulosis: diverticula are present
Diverticulitis: inflammation of the diverticula People with ADPKD have an increased likelihood ofcomplications from diverticula, especially aftertransplant
Symptoms: Abdominal pain, diarrhea, blood in the stool
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Usually easy to diagnose in people with: Flank or abdominal pain
Family history of ADPKD
Imaging study (Ultrasound, MRI, CT scan)x Cysts may be seen in the liver, pancreas, spleen
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Criteria are very sensitive in detecting PKD1
< 30 years: at least 2 cysts (on one or both kidneys)
30 59: at least 2 cysts in each kidney > 60: 4 or more cysts in each kidney
A negative study does not mean that a person doesnot have PKD1 unless the age older than 30 years
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A group of cysts with some connectivetissue, but no identifiable renal tissue
Most unilateral (left kidney)
Incidence 1:3000, boys>girls
Most common cause of abdominal mass in
newborn period Palpable flank mass in otherwise healthy infant
Reported in a variety of syndromes Beckwith-Wiedemann, Trisomy 18, VACTERL
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Role of nephrectomy controversial Recommended to treat or prevent pain, UTI,
hypertension, or renal malignancy
Lifetime follow up despite involution ornephrectomy secondary to complications Hypertension, UTI, proteinuria, renal, malignancy
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THANK
YOU