FREE TREATMENT REPORT
See ratings and reviews when you sign up for an account.
Clinical Investigations Into the Kidney and Liver Disease in Autosomal Recessive Polycystic Kidney Disease/Congenital Hepatic Fibrosis and Other Ciliopathies
This study is currently Recruiting
March 2011 By National Institutes of Health Clinical Center (CC)
First Recieved on September 10, 2003
Last Updated on April 22, 2011
This study will evaluate patients with autosomal recessive polycystic kidney disease (ARPKD)
and congenital hepatic fibrosis (CHF) and other related disorders (ciliopathies). People
with ARPKD develop kidney cysts and eventually kidney failure, symptoms may include
hypertension (high blood pressure), poor growth, and urinary infections. CHF is a specific
type of liver disease associated with ARPKD. It involves fibrosis, or scarring, of the
liver, which can lead to life-threatening complications, including internal bleeding of
enlarged blood vessels called varices in the esophagus (food pipe). The goal of the study is
to better understand the medical complications of these disorders and identify
characteristics that can help in the design of new treatments.
Resources/Links provided by NLM:
|Study Start Date:
|Estimated Primary Completion Date:
|Ages Eligible for Study:||6 Months|
|Genders Eligible for Study:||Both|
|Accepts Healthy Volunteers:||No|
- INCLUSION CRITERIA:
This protocol will enroll children and adults who carry a clinical diagnosis of ARPKD,
CHF, JSRD, BBS, OFD or AS and who has either PKD/NP spectrum of changes in the kidneys or
CHF/Caroli's syndrome of the liver. This might rarely include adults who are unable to
give informed consent.
Among patients who have received a kidney or liver allograft, those with stable graft
function and without severe transplant-related complications are eligible for enrollment.
Patients and their parents/legal guardians must be willing to come to the NIH Clinical
Center for admission annually.
Infants under 6 months of age.
Medically fragile patients who require frequent hospitalizations due to complications of
end-stage renal disease (uncontrolled hypertension, severe electrolyte imbalances) or
hepatic disease (current variceal bleeding, overt encephalopathy, intractable recurrent