INTERNATIONAL RENAL MEETING AND MAYO CLINIC DAY IN SARDINIA AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE 1. THERAPEUTIC ROLE OF mTOR INHIBITORS 2. TESTING OF RELATIVES AT RISK: ASYMPTOMATIC CHILDREN 3. HYPERTENSION: WHICH IS THE BEST METHOD OF MONITORING BP?
mTOR INHIBITORS IN ADPKD Everolimus slowed the increase in TKV but Sirolimus at a daily target dose of 2 mg did not slow the progression of renal did not halt polycystic kidney growth impairment
mTOR INHIBITORS IN ADPKD
mTOR INHIBITORS IN ADPKD Do mTOR inhibitors still have a future in ADPKD? (Perico and Remuzzi, 2010)
ADPKD IN CHILDREN TESTING OF RELATIVES AT RISK � At present, there is no indication for testing of asymptomatic children. This may change in the future, if and when effective therapies are found � . Harris PC and Torres VE, GeneReviews, 2011
ADPKD and HYPERTENSION IN CHILDREN � HYPERTENSION: 10-20% of ADPKD children � AMBULATORY BP MEASUREMENT (ABPM): 34% of hypertension � OFFICE BP MEASUREMENT: 16% � NON-DIPPERS: 25% of ADPKD boy Chapman, 2010; Cadnapaphornchai, 2009
HYPERTENSIVE ADPKD CHILDREN ARE AT HIGH RISK FOR DECREASED RENAL FUNCTION Cadnapaphornchai M A et al. CJASN 2009;4:820-829
HYPERTENSION IN ADPKD CHILDREN
ADPKD IN CHILDREN TESTING OF RELATIVES AT RISK Should we change the traditional approach? Should we check children of ADPKD families for blood pressure?
HYPERTENSION: A MODIFIABLE RISK FACTOR IN ADPKD HYPERTENSION IS ASSOCIATED WITH A FASTER PROGRESSION TO ESRD. Gabow, 1992
HYPERTENSION IN ADPKD ADULTS WAITING FOR THE RESULTS OF HALT PKD CLINICAL TRIALS (2013) ��� CURRENT APPROACHES TO THE ANTI-HYPERTENSIVE TREATMENT 1)early recognition of hypertension 2)aggressive control of BP (< 130/80 mmHg ) 3) inhibitors of the RAAS: first line 4)appropriate lifestyle modifications
WHICH IS THE BEST NON-INVASIVE METHODS OF BP MONITORING? 1. OFFICE BLOOD PRESSURE MONITORING (OBPM) White Coat Hypertension (30%) 7 � 17mmHg higher for SBP 5 � 14mmHg higher for DBP vs. ABPM and HBPM 2. HOME BLOOD PRESSURE MONITORING (HBPM) Frequency of hypertension measured by HBPM not determined Predict End-organ Damage more Reliably than OBPM More Reproducible than OBPM 3. 24-H AMBULATORY BLOOD PRESSURE MONITORING (ABPM) Circadian Differences in Blood Pressure Nondipping Associated with End-organ Damage
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