ARPKD with Congenital Hepatic Fibrosis.
A rare, recessively-inherited disease that affects both kidneys and the liver. Diagnosis often happens in infancy or childhood, and a growing community of adults lives with it today. Leo is building a dedicated hub for ARPKD/CHF — for the adults, parents, and caregivers who deal with it day to day.
Two organs, one genetic root.
ARPKD — autosomal recessive polycystic kidney disease — is caused (in nearly all cases) by mutations in a single gene, PKHD1. A child inherits one mutated copy from each carrier parent. The disease affects the kidneys (with cysts) and the liver (with abnormal duct development called congenital hepatic fibrosis, or CHF) essentially together — they share the same genetic origin. — NIH NIDDK, 2025
Severity varies widely. Some children are diagnosed before birth or as newborns with severe kidney and breathing issues; others have a milder course and are diagnosed later in childhood, when liver involvement is more prominent. CHF can be the first manifestation noticed. — StatPearls (NIH Bookshelf), 2024
Because the disease is autosomal recessive, both parents must carry one mutated copy of PKHD1 for a child to be affected. The pattern below shows the inheritance math. — NIH NIDDK, 2025
The data that actually moves the conversation at appointments.
ARPKD/CHF is multi-system, so the day-to-day data picture spans several domains. Below is what tends to come up in routine nephrology and hepatology visits — for both children and adults living with the disease — drawn from NIDDK's ARPKD overview and the StatPearls clinical review. Leo doesn't tell you what to track; it just makes whatever your team asks you to track easier to keep.
Blood pressure
Hypertension is the most common complication — managing it well is a core long-term concern.
— NIH NIDDK, 2025Kidney function
Creatinine, eGFR, electrolytes, and urine output across labs and visits.
— StatPearls (NIH Bookshelf), 2024Liver labs
LFTs and signs of portal hypertension — splenomegaly, esophageal varices, GI bleeding.
— StatPearls (NIH Bookshelf), 2024Medications
Anti-hypertensives, ACE inhibitors or ARBs, and other meds — often complex regimens.
— NIH NIDDK, 2025Growth & nutrition
Pediatric patients often need closer growth and nutrition tracking; adults track maintenance, weight, and intake.
— NIH NIDDK, 2025Episodes & events
Hospitalizations, infections (cholangitis is a known risk), and any acute change worth logging.
— StatPearls (NIH Bookshelf), 2024The ARPKD/CHF hub, end to end.
Every condition pack in Leo has its own hub UI. The ARPKD/CHF hub is in active development — coming to Leo soon. Here's what it will include when it ships, alongside the general-purpose tools every Leo user gets today.
- Curated symptom pack covering both the kidney and liver presentations
- Episode templates for ED visits, BP spikes, and acute infections
- Blood pressure logging via HealthKit (Apple Watch / connected cuffs) or manual entry
- Medications tracked in Leo's general medication tools; hub-specific caregiver-administered dosing is on the roadmap
- A partner view exists app-wide today; dedicated role-based caregiver and provider sharing is coming
- PDF health reports — compile labs, vitals, and notes for specialist visits
Where to learn more.
The most useful first-line sources for families and clinicians researching ARPKD/CHF, in plain links — no affiliate, no tracking.
NIH NIDDK — Autosomal Recessive PKD
Government overview: causes, symptoms, treatment, and research.
StatPearls — ARPKD
Peer-reviewed clinical reference (free, NIH-hosted).
GARD — Congenital Hepatic Fibrosis
NIH Genetic and Rare Diseases information center page on CHF.
NORD — ARPKD
National Organization for Rare Disorders patient-facing summary.
One hub, coming soon.
The ARPKD/CHF hub is in active development. Leo is in private beta — join us to be among the first to use the hub when it ships and help us refine the pack along the way.