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Systemic Hypertension and Renal Transplantation In Arpkd PowerPoint Presentation

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Description : Check out this medical PowerPoint presentation titled "Systemic hypertension and renal transplantation in ARPKD” by KEVIN E. C. MEYERS.

This medical PowerPoint presentation is about systemic hypertension and renal transplantation in ARPKD (Autosomal Recessive Polycystic Kidney Disease), two important issues that need to be considered in the management of patients with ARPKD.

ARPKD is a genetic disorder that affects the development of the kidneys and liver, leading to the formation of cysts in these organs. Hypertension is a common complication of ARPKD, which can lead to further damage to the kidneys and other organs.

Renal transplantation is often considered as a treatment option for patients with end-stage renal disease due to ARPKD. However, the presence of hypertension can complicate the transplantation process and affect the long-term outcomes of the transplant.

Before a patient undergoes renal transplantation, it is important to manage their hypertension effectively. This may involve the use of medications such as ACE inhibitors, angiotensin receptor blockers, and beta-blockers to control blood pressure. In addition, lifestyle modifications such as weight loss, regular exercise, and a low-sodium diet may also be recommended.

After transplantation, it is important to continue monitoring blood pressure closely and adjusting medications as needed to prevent further damage to the transplanted kidney. Regular follow-up visits with a healthcare provider are also important to ensure proper management of hypertension and overall transplant health.

In conclusion, hypertension is a common complication of ARPKD that needs to be effectively managed before and after renal transplantation to improve the long-term outcomes of the transplant.

Tags : Systemic hypertension, renal transplantation, ARPKD, Autosomal Recessive Polycystic Kidney Disease, genetic disorder, Nephrology, Nephrology PowerPoint presentation

Published on : Jun 07, 2023 | Views : 1172 | Downloads : 1

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SYSTEMIC HYPERTENSION AND RENAL TRANSPLANTATION IN ARPKD KEVIN E. C. MEYERS PROFESSOR OF PEDIATRICS THE CHILDREN’S HOSPITAL OF PHILADELPHIA AND THE UNIVERSITY OF PENNSYLVANIA
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HYPERTENSION Gidding S S et al. Pediatrics 2012; 129:e1311-e1319 CARDIOVASCULAR (CV) RISK FROM THE FETUS TO ADULT
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HYPERTENSION In ARPKD occurs pre and post transplant
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HYPERTENSION Neonates with large echogenic kidneys have ectasia of 60–90 % of the renal collecting tubules and generally have hypertension and decreased kidney function Neonates with hypertension are more likely to require mechanical ventilation Guay-Woodford LM, Desomond RA (2003) ARPKD: the clinical experience in North America. Pediatrics 111:1072–1080 Sweeney WE Jr, Avner ED (2011) Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol 26: 675–692
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HYPERTENSION Beyond the neonatal period, hypertension occurs in two-thirds (2/3) of children Studies show that activation of the intra-renal renin-angiotensin-aldosterone system, without concurrent elevation in systemic angiotensin I and II levels, may play a role Sweeney WE Jr, Avner ED (2011) Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol 26: 675–692 Loghman-Adham M, Soto CE, Inagami T, Sotelo-Avila C. Expression of components of the renin-angiotensin system in ARPKD. The Journal of Histochemistry and Cytochemistry. 2005; 53:979–988 Goto M, Hoxha N, Osman R, Dell KM. The renin-angiotensin system and hypertension in ARPKD. Pediatric Nephrology. 2010; 25:2449–2457
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HYPERTENSION
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HYPERTENSION Impaired urinary dilution with associated fluid retention Dysregulation of the collecting duct epithelial sodium channel may be contributing factors to ARPKD-related systemic HTN Rohatgi R, Greenberg A, Burrow CR, Wilson PD, Satlin LM. Na transport in autosomal recessive polycystic kidney disease (ARPKD) cyst lining epithelial cells. J Am Soc Nephrol. 2003; 14:827–836. Veizis IE, Cotton CU. Abnormal EGF-dependent regulation of sodium absorption in ARPKD collecting duct cells. American journal of physiology Renal physiology. 2005; 288:F474–F482.
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HYPERTENSION ACEi or ARB not both (aim - optimize blood pressure control, while minimizing further reduction in GFR in the context of chronic kidney disease (CKD)) Avoid excess fluid intake (water) [Diuretics – Lasix] Rohatgi R, Greenberg A, Burrow CR, Wilson PD, Satlin LM. Na transport in autosomal recessive polycystic kidney disease (ARPKD) cyst lining epithelial cells. J Am Soc Nephrol. 2003; 14:827–836. Veizis IE, Cotton CU. Abnormal EGF-dependent regulation of sodium absorption in ARPKD collecting duct cells. American journal of physiology Renal physiology. 2005; 288:F474–F482 The recent multicenter ESCAPE trial in children with Chronic Kidney Disease stages 2–4 indicated that aggressive blood pressure control (target 24-hour mean arterial blood pressure below the 50th percentile for age, height and sex) may slow progression to end-stage renal disease; the specific target for ARPKD has not been established, however the same principles apply MANAGEMENT
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STEPPED-CARE THERAPY HYPERTENSION
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ARPKD CASE Prenatal diagnosis of enlarged polycystic kidneys NICU for 2 months Renal ultrasound revealed massive echogenic cystic kidneys measuring 10 cm and 10.7 cm, and diffuse hepatic biliary duct ectasia
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ARPKD CASE Transferred to CHOP for uncontrolled hypertension and poor weight gain Discharged from CHOP at 3 months of age on multiple antihypertensive medications, including diuretics and NG feeds Unable to maintain normal electrolyte balance and stopped gaining weight. A gastro-jejunostomy tube was placed for feeding without improvement in growth
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ARPKD CASE She underwent a left nephrectomy on 1/15/03 in an attempt to improve gastric emptying and weight gain Her post-operative course was uncomplicated, however, she gradually became more edematous and hyperkalemic She started hemodialysis
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ARPKD CASE She received a LRD kidney transplant from her Father with a right native nephrectomy over 15 years ago and has done well. She is now a 16 year old but has CKD with hypertension and will require a second transplant in the future.
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OBJECTIVES 1. To describe which persons with ARPKD might need a kidney transplant 2. To outline the preparatory process for kidney transplant in ARPKD 3. To review ARPKD kidney and kidney/liver transplants
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WHICH CHILDREN WITH ARPKD NEED A KIDNEY TRANSPLANT
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COMPLICATIONS & TREATMENT IN 50 PTS WITH FOLLOW-UP INFORMATION Adeva et al. Medicine 2006;85:1–21
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RENAL REPLACEMENT THERAPY At the age of 10 years: 60% of patients require kidney transplantation About 10% need liver transplantation Mainly sequential liver–kidney transplantation
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TRANSPLANT Wen JW, Furth SL, Ruebner RL. Kidney and liver transplantation in children with fibrocystic liver–kidney disease: data from the U.S. Scientific Registry of Transplant Recipients: Pediatr Transplant 2014; 18:726–732 In this cohort, the reason for liver replacement was Caroli syndrome in 29% and CHF in 71% Mortality after LTx was 23%, after KTx 10% and after SLKTx 12% It is of note that the overall mortality has improved in the recent decade
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THE PREPARATION PROCESS FOR KIDNEY TRANSPLANT IN ARPKD
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STAGES OF CHRONIC KIDNEY DISEASE (CKD)
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EVALUATION FOR TRANSPLANT RECIPIENT Medical management of CKD Nutrition Ca/PO4/Fluid/Calories/Acidosis NGT/GT/GJT Liver/GI Hypersplenism/Portal HTN/Infection Cardiovascular HTN Dialysis Psychosocial evaluation
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EVALUATION FOR TRANSPLANT DONOR LRD or DD Medical assessment Psychosocial evaluation
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CHOP ARPKD CHILDREN WITH KIDNEY TRANSPLANTS AND REVIEW OF PUBLISHED LITERATURE
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CHOP: KIDNEY TRANSPLANTS IN CHILDREN WITH ARPKD 2002 LF – liver fibrosis; PHTN – portal hypertension; HS - hypersplenism
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CHOP: KIDNEY TRANSPLANTS IN CHILDREN WITH ARPKD 2018
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OUTCOMES OF ARPKD Clinical course of 164 neonatal survivors Bergmann et al Kidney Int. 2005 67:829-48 Chronic renal failure first detected at a mean age of 4 years Patient survival
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ACTUARIAL RENAL SURVIVAL Bergmann et al Kidney Int. 2005 67:829-48 Clinical course of 164 neonatal survivors Percent
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From birth: 46% at 15 years [Kaplan et al 1989] From 1 month: 42% at 20 years [Bergman et al. 2005] From 1 month: 67% at 15 years [Roy et al 1993] From 1 year: 79% at 15 years [Kaplan et al 1989] ACTUARIAL RENAL SURVIVAL TIME TO ESRD FROM DIAGNOSIS
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LIKELIHOOD OF BEING ALIVE WITHOUT ESRD 20 YEARS AFTER THE DIAGNOSIS PERCENT AGE AT DIAGNOSIS Adeva et al. Medicine 2006;85:1–21
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Likelihood of being alive without ESRD 20 years after diagnosis Adeva et al. Medicine 2006;85:1–21
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Survival and renal survival for the ARPKD cohort Adeva et al. Medicine 2006;85:1–21
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NEONATAL NEPHRECTOMY DIALYSIS & KIDNEY TRANSPLANT Sarawal et al. Pediatric Transplantation 2007
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PATIENT SURVIVAL RATE North American Pediatric Renal Collaborative TrialS (NAPRCTS) registry data Davis et al. Pediatric Transplantation 2003
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GRAFT SURVIVAL RATE Davis et al. Pediatric Transplantation 2003 North American Pediatric Renal Collaborative TrialS (NAPRCTS) registry data
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COMPLICATONS GRAFT THROMBOSIS REJECTION GROWTH PTLD CARDIOVASCULAR (HTN) INFECTION* LIVER*
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MORBIDITY AND MORTALITY FROM CHF AFTER RENAL TRANSPLANTATION FOR ARPKD 14 patients 6 males 8 females Mean age: 8.3 yr, range 1-22.3 yr Follow-up: 14.5 years (3.1-33.6 yr) Am J Transplant. April 2002 Survival
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MORBIDITY AND MORTALITY FROM CHF AFTER RENAL TRANSPLANTATION FOR ARPKD Complications of CHF occurred in 79% with kidney transplants for ARPKD Mortality related to CHF occurred in 29% (5 pts) Accounted for 80% deaths (4/5 pts) Am J Transplant. April 2002
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Current guidelines regarding listing for combined kidney-liver transplantation (CKLT) are in the context of end-stage liver disease due to hepatocellular dysfunction, for which listing for CKLT is recommended when estimated glomerular ﬁltration rate is <30 mL/min/1.73 m2 . DUAL KIDNEY–LIVER TRANSPLANT IN ARPKD Khan K, Schwarzenberg SJ, Sharp HL, Matas AJ, Chavers BM. Morbidity from congenital hepatic ﬁbrosis after renal transplantation for autosomal recessive polycystic kidney disease. Am J Transplant. 2002;2(4):360–365 Charlton MR, Wall WJ, Ojo AO, et al; International Liver Transplantation Society Expert Panel. Report of the ﬁrst international liver transplantation society expert panel consensus conference on renal insufﬁciency in liver transplantation. Liver Transpl. 2009;15(11):S1– S34 doi:10.1002/lt.21877
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Whereas survival after KTx in ARPKD is in the order of other underlying kidney diseases, the mortality after KTx is in 60–80% related to ascending cholangitis. The immunosuppression after KTx may increase the risk for cholangitis. Therefore, SLKTx should be considered for patients with ESRD and liver disease, especially with Caroli syndrome or a history with cholangitis. A recent report from Hamburg [Brinkert] and duplicated in Essen [Hoyer] show excellent survival rates after SLKTx, justifying this strategy in a subgroup at risk. Telega G, Cronin D, Avner ED. New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney–liver complications. Pediatr Transplant 2013; 17:328–335 Brinkert F, Lehnhardt A, Montoya C, et al. Combined liver–kidney transplantation for children with autosomal recessive polycystic kidney disease (ARPKD): indication and outcome. Transpl Int 2013; 26:640–650 DUAL KIDNEY–LIVER TRANSPLANT IN ARPKD
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HEALTH RELATED QOL AFTER COMBINED KL TRANSPLANT Child self- report PedsQL 4.0 of all four (CLKT, LTx, KTx, and healthy) groups. Mean scores for each subscale and each sample for the child self- report; P<.01*, P<.05+ ; significant difference indicates the comparison between the CLKT sample and the selected group Parent proxy- report PedsQL 4.0 of all four (CLKT, LTx, KTx, and healthy) groups. Mean scores for each subscale and each sample for the parent proxy- report; P<.01*, P<.05+ ; significant difference indicates the comparison between the CLKT sample and the selected group Schmaeschke et al. Pediatric Transplantation 2107