Autosomal Dominant Polycystic Kidney Disease

What Adpkd Is

Hereditary

Managing Blood Pressure

Trials

The Importance of Working with with Your Healthcare Team

Predicting Renal Outcomes in Polycystic Kidney Disease

How Did You Find Out that You Had Pkd How Was It Diagnosed and What Are Your Suggestions to Viewers

Liver Injury

Diet

1 IN 2,000 in the US have been diagnosed with ADPKD

50% Chance of inheriting ADPKD

IF YOU HAVE A HISTORY OF ADPKD IN YOUR FAMILY

What is ADPKD?

ADPKD Pathogenesis

Mechanism of ADPKD

PKD1 and PKD2 mutations

Polycystin 2

Role of Vasopressin in ADPKD

Mechanisms of Cyst Formation

Conclusion

Autosomal Dominant Polycystic Kidney Disease

Risk factors

Causes of ADPKD

Pathophysiology of ADPKD

Causes of flank pain

Pathophysiology

Extra-renal manifestations of ADPKD

Diagnosis of ADPKD

Treatment

End-stage renal failure

What is ADPKD?

Therapeutic options

Therapeutic options for ADPKD

Autosomal Dominant Polycystic Kidney Disease

What Adpkd Is

Early Detection

Blood Pressure

Side Effects

The Importance of Working with with Your Health Care Team

Predicting Renal Outcomes in Polycystic Kidney Disease

How Did You Find Out that You Had Pkd How Was It Diagnosed and What Are Your Suggestions to Viewers about Getting the Best Care

Drug Drug Interactions

Diet

Autosomal Dominant Polycystic Kidney Disease (ADPKD)

TEMPO 3:4 and REPRISE Results

Effects of Tolvaptan On Total Kidney Volume

Comparison of ALT Elevations in 2 Pivotal Trials

What Are The Indications (label)?

Risk categories

When is Tolvaptan Available?

Risk Evaluation and Mitigation Strategy (REMS)

How Do I Get Tolvaptan?

This is only the beginning!

A few key genetic terms

Causes of genetic disease

Why autosomal "dominant" polycystic kidney disease?

Other rare genetic causes of ADPKD-like disease

Why should we do genetic testing?

Mosaicism can occur in ADPKD

Non-truncating variant types in PKD1

Renal survival for genotypic and imaging classes

Clinical genetic testing for ADPKD

ADPKD is mainly caused by pathogenic variants to PDI and PKD2

Symptoms of Polycystic Liver Disease

Liver Disease

Cholangitis

Ascites

Hepatic Fibrosis

Polycystic Liver Disease

Pivotal Clinical Trial

Mtor Inhibitors

Quality of Life Questionnaire

Summary

Intro

Pipeline of ADPKD Treatments in Clinical Development

How ADPKD Affects You Outside the Kidneys

When did you get a kidney transplant?

When should someone be referred to a nephrologist?

Are there special dietary considerations for patients with ADPKD?

What happened when you didn't have insurance?

What do you do now?

Conclusion

Intro

Prognostic biomarkers

Pro-PKD score

When to repeat ADPKD imaging

Reprise study

What is the starting point for monitoring patients?

Thoughts on how new disease modifying treatments might impact the treatment landscape once approved?

Recommendations for hydration

Monitoring liver function

Closing remarks

Blood Pressure for Children

Blood Pressure Measurement in Children

Early Onset Disease

Diagnosis

Repeated Scans

What Treatments Are Available and for Children and Young People

What Advice Would You Give for a Child with Stage 3 Ckd with Adpkd about Returning to School

What Cases of Adpkd Would Be Referred to Great Ormond Street and Which Would Be Managed by a Local Nephrology Team

What are the treatment options available?

Sodium restriction

Caffeine and ADPKD

Tolvaptan

What is the treatment for ADPKD?

Ketone bodies

Summary