CHOP has established a research database of data and biological samples of people who have autosomal polycystic kidney disease (ARPKD) or another hepato/renal fibrocystic disease (HRFD). The purpose is to try to learn more about the causes of these diseases. We will collect data from participants' medical records to contribute to the database. Optional blood/saliva or tissue sample may also be collected. Visit arpkdb.org to download the consent or call the CHOP Research Coordinator, Jasmine Jaber at jaberj2@chop.edu.

CKiD is an important research study looking at the health of children, teenagers, and young adults who have kidney problems. This research is being done to identify what factors help kidneys stay healthy, and also to see how kidney problems affect health and well-being. Teenagers and young adults 16-22 years old who have been diagnosed with chronic kidney disease (CKD) may be eligible to join the study. The CKiD study has enrolled approximately 1100 children and teenagers across North America since the study began in 2003. Our goal now is to enroll teenagers and young adults in the study. By doing this we can have a better idea of what happens to kidney function and overall health as someone with childhood chronic kidney disease grows up. This will help provide us with a more complete picture of CKD from childhood through adulthood.

Cure Glomerulonephropathy Network (CureGN) is a multi-year study of 2,400 children and adults with four rare glomerular (kidney) diseases at over 50 centers in the US, Canada, and Italy. The purpose of CureGN is to create a patient research network to learn about four major causes of glomerular disease : 1. Minimal Change Disease (MCD) 2. Focal Segmental Glomerulosclerosis (FSGS) 3. Membranous Nephropathy (MN) 4. IgA Nephropathy (IgAN)

For this study, we are studying the relationship between variations in the genes of the immune system called HLA and how they relate to:

1. The risk of developing nephrotic syndrome
2. The response to treatment of nephrotic syndrome and
3. The risk of recurrence of nephrotic syndrome following kidney transplantation.

1. Clinical diagnosis of nephrotic syndrome
2. Biopsy proven diagnosis of FSGS or MCD and,
3. History of living or deceased donor kidney transplantation

In this study, we are developing new magnetic resonance imaging (MRI) and ultrasound methods to determine if it is effective in diagnosing complication in kidney transplant patients. Participants in this study will involve patients that have had a kidney transplant and are scheduled for a kidney biopsy. The study will also involve a comparison group of healthy individuals that have no history of kidney conditions. Study procedures will include a review of medical history and medications, MRI scan and ultrasound of the abdomen and blood/ urine test.

In this study, we are developing new ultrasound and magnetic resonance imaging (MRI) methods to measure kidney and liver disease severity in autosomal recessive polycystic kidney disease (ARPKD). Participants in this study will include individuals with ARPKD, and a comparison group of healthy individuals without liver or kidney disease. Individuals of any age may take part in the study.

Kidney stones are one of the fastest growing health conditions among children, adolescents, and young adults. The rapid increase over a short period of time has resulted in a large number of pediatric patients who require surgery to remove kidney stones with very little information available to guide selection of treatment options. There are three alternative approaches to remove kidney stones:

• Ureteroscopy (an endoscopic outpatient procedure)
• Shockwave lithotripsy (a noninvasive outpatient procedure)
• Percutaneous nephrolithotomy (a minimally invasive surgery with a short hospital stay)

The purpose of this study is to determine how what you eat and drink (your diet), antibiotics you take, and the bacteria that live in your gut (microbiome) contribute to developing kidney stones. Kidney stone disease, known as nephrolithiasis, is also influenced by products of metabolism (metabolites) that are found in your urine. These factors will be examined in participants at least 4 years of age. Each participant will complete three 24-hour dietary recalls and will provide one stool sample and up to two urine samples. The information from this study will help doctors find new metabolic pathways that can be used for treatment of kidney stones.

Urinary stone disease (USD) is a common, chronic disorder that is seen more frequently in adolescence today than in the past. Current understanding of its impact on bone development and structure as well as dental health is not known. We will be evaluating bone and dental quality in 90 children/adolescents with USD and 90 healthy children/adolescents. The study requires: two to three visits for a total of three to four hours, that the participant be 5-21 years old, and compensation will be provided. If interested in participating contact the research coordinator at 267-226-6995.