Pathogenesis and Treatment of Kaposiform Lymphangiomatosis
Funded at Cincinnati Childrens Hosp Med Ctr · NIH-11445264 · Data updated 2026-11-12
Aims to understand what drives a rare childhood disease, Kaposiform lymphangiomatosis (KLA), and to improve its diagnosis and treatment so affected children and young adults can live longer and healthier lives.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Cincinnati Childrens Hosp Med Ctr NIH-funded |
| Lab location | 1 site (Cincinnati, United States) |
| Project start | January 1, 2022 |
| Project end | May 31, 2030 |
| Funding year | 2026 |
| Award (this year) | $808,579 |
| NIH project number | 2R01HL156866-05A1 |
| Project ID | NIH-11445264 on NIH RePORTER |
Who could benefit from this research
✓Could help
Children and young adults with Kaposiform lymphangiomatosis or related vascular anomalies may be candidates, especially those who would benefit from biomarker-based diagnosis or targeted therapies.
✕Less likely to help
Patients without KLA or without access to specialized healthcare centers may not experience direct benefit.
What this research studies
Kaposiform lymphangiomatosis is a chronic, serious disease that causes harmful lymphatic growths and bleeding problems, often in multiple organs. This project studies a somatic NRAS mutation (NRASQ61R) linked to KLA by creating human endothelial cells and mouse models to mirror the disease and understand how this mutation leads to the characteristic spindled cells and vessel changes. The work builds on a blood biomarker discovery (ANG-2) and a diagnostic test, aiming to connect molecular mechanisms to better therapies beyond current rapamycin treatment. Patients and families could benefit from earlier diagnosis, clearer prognosis, and targeted treatment approaches as the science translates toward new options.
Why it matters
Potential benefit: If successful, this research could lead to earlier diagnosis and more effective, targeted therapies for KLA, potentially improving survival and quality of life.
How similar studies have gone: Early genetic models and biomarker-driven approaches in related vascular disorders have advanced understanding and informed therapy development, suggesting potential for meaningful progress in KLA.
Where this research is happening
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Cincinnati Childrens Hosp Med Ctr
Cincinnati, United States Active
Researchers
- Principal investigator: Le Cras, Timothy David — Cincinnati Childrens Hosp Med Ctr
- Study coordinator: Le Cras, Timothy David
About this research
- This is an active NIH-funded research project — typically early-stage science, not a clinical trial accepting patient enrollment.
- Some NIH-funded labs run parallel clinical studies or seek volunteers for related work. To check, contact the principal investigator or institution listed above.
- For full project details, budget, and progress reports, visit the official NIH RePORTER page.