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Renal manifestations of ADPKD 신장내과 R1 최경진
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Introduction - ADPKD ADPKD Continued enlargement of the cysts Leads to progressive renal failure Total kidney volume is the strongest predictor for the development of renal insufficiency Renal manifestation of ADPKD Hypertension / Urinary tract infection / Hematuria Concentrating defect / Nephrolithiasis Acute or chronic flank & Abdominal pain All complications relate directly to the extent of renal cyst involvement : be assessed by total kidney volume measurements
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Hematuria Occurs in 35-40% of Pt. with ADPKD Usually occurs prior to loss of kidney function May be presenting symptom of the disease Gross hematuria is associated with more rapid progression of kidney disease in ADPKD Gross hematuria is more likely in Larger kidneys ( > 15cm in length) Hypertension Higher plasma creatinine concentrations Association with worse renal outcomes
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Hematuria Rupture of a cyst into the collecting system Be responsible for the development of hematuria Although hemorrhage into a cyst is common, typical presentation is pain d/t many cysts don’t communicate with the collecting system Hematuria resolves within 2-7 days with conservative therapy : Bed rest, Hydration, Analgesics not NSAID Unusual & severe bleeding Percutaneous arterial embolization Nephrectomy
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Concentrating defect Complain of thirst, polyuria, nocturia & urinary frequency a Decrease in urinary concentrating ability is one of the earliest manifestations of ADPKD Worsens with increasing age & declining kidney function Severity of anatomical deformities induced by the cysts Underlying cause is not known Disruption of tubular architecture Defect in principal cell function Early tubulointerstitial disease
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Concentrating defect Elevation of Vasopression level > Central cause is excluded occurs early in the course of the disease in order to preserve water balance Urine diluting capacity appears to be intact in ADPKD Increased vasopressin concentration may play a role in disease progression Strategies to inhibit vasopressin action are potential therapeutic modalities in ADPKD Vasopressin Rc antagonists reduce vasopressin levels with increased free water intake
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Nephrolithiasis Occurs in -25% of Pt. with ADPKD Composition of uric acid > ½ of stones in ADPKD Risk factor for nephrolithiasis Increased renal volume : controversial In one study, renal volume determined by CT was greater in 35 pts with ADPKD and nephrolithiasis, compared with those who had ADPKD but no nephrolithiasis In the CRISP study of 241 individuals, no association between nephrolithiasis and kidney volume could be established Low urinary volume / Low urinary citrate Hyperuricosuria / Hypercalciuria
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Nephrolithiasis Diagnosis of stone by USG is more difficult than in idiopathic stone formers, the large cysts obscuring the view of the collecting system calcifications that may be present in the cyst walls Most stones can be detected by Intravenous pyelography By CT scanning is more sensitive for small or radiolucent stones Treatment of obstructing stones More difficult than idiopathic stone disease Cystoscopy : be complicated by an infected cyst Percutaneous nephrostomy or ESWL is hard d/t large cysts small stones( < 2cm in diameter) is successful Percutaneous nephrolithotomy in a limited patients
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Flank & Abdominal pain Common problem Can be due to renal or extrarenal etiologies Acute kidney pain Infections (cystic or parenchymal) Nephrolithiasis Cyst hemorrhage Cysts in the liver Chronic kidney pain more common in advanced disease who have enlarged kidneys Dull, persistent Stretching of the capsule or traction on the renal pedicle
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Renal cell carcinoma (RCC) an infrequent complication of ADPKD not occur with increased frequency than general population Differentiating characteristics of RCC in ADPKD Patients frequently present with fever Bilateral at presentation Multicentric Sarcomatoid type
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Renal cell carcinoma (RCC) Diagnosis of RCC is difficult in ADPKD Hematuria / a Flank mass / Bleeding into cysts / a Complex cyst on USG, CT or MRI > common in ADPKD with out malignancy Some clinical clues may be helpful systemic signs & symptoms( fever, anorexia, fatigue, Wt.loss) rapid growth of a complex cyst Percutaneous aspiration & cytologic examination
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Urinary tract infection Approximately 30–50 % of patients with ADPKD An infected cyst & APN = m/c kidney infections Cyst infection incidence = 0.01 episode/patient /year Source of infection Women > Men Typically caused by Gram(-) enteric organisms the causes of cyst infection are often more difficult to document the cysts may not be in communication with the collecting system the urine culture is often negative 20 % of patients with ADPKD develop nephrolithiasis, a source of recurrent infections
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Urinary tract infection Clinical features Fever / Flank pain / Nausea / Vomiting A more insidious presentation Location of the cyst infection > specific area of tenderness Diagnosis Pyelonephritis : Diffuse flank pain / urine culture(+) / blood culture(+) Infected cyst : Discrete Td / urine culture(-) / blood culture(+)
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Urinary tract infection – Antibiotics whether the patient has PN or a cyst infection, it is frequently difficult to initially distinguish between the two the Choice of initial empiric therapy : to successfully treat both types of infection Therapeutic concentrations within cysts + against Gram(-) enteric organisms Ciprofloxacin Levofloxacin Trimethoprim-sulfamethoxazole Chloramphenicol No penicillins (do not penetrate the cyst)
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Urinary tract infection – Antibiotics Initially with intravenous Ciprofloxacin Initially with Cefotaxime or Ampicillin + Gentamycin, because Quinolone resistance is an increasing problem Streptococcal or staphylococcal infection Vancomycin or erythromycin Resistant group A streptococcal infection Levofloxacin Anaerobic organism Metronidazole or Clindamycin
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Urinary tract infection – Antibiotics The duration of therapy APN : a minimum of 10~14 days Optimal duration of therapy for infected cysts is unclear for at least 4 weeks and sometimes for up to 6 weeks If the infection recurs after withdrawal of antimicrobials, therapy may be reinstituted and continued for 2~3 months or longer
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Urinary tract infection Large infected cysts(>3~5cm) are more likely to fail medical therapy Percutaneous or surgical drainage of the cyst Infrequently necessary & hard to perform Difficult to ascertain radiologically which of the many cysts is infected Perinephric abscess Drainage may be indicated Recurrent UTIs Stone removal procedures may be required if nephrolithiasis is contributing to recurrent UTIs
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Nephrectomy in ADPKD though Cyst formation occur < 5-10% of nephrons Cyst and total renal size increase progressively over time Unilateral or Bilateral nephrectomy indication Recurrent infection Limitation of daily activities, fatigue, & anorexia (signs of malnutrition) Suspected malignancy Extension of the native polycystic kidney into the potential pelvic surgical site. Uncontrollable renal hemorrhage in CIx to or failure of intraarterial embolization Development of ventral hernia d/t massive renomegaly
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