The Role of Angiogenesis-related Pathways in the Development of Port Wine Stains

The Role of Angiogenesis-related Pathways in the Development of Refractory Port Wine Stains

1. Port wine stain (PWS) is a congenital, progressive vascular malformation of human skin involving the superficial vascular plexus that occurs in estimated 3-5 children per 1,000 live births. In childhood, PWS are flat red macules, but lesions tend to darken progressively to purple and, by middle age, often become raised as a result of the development of vascular nodules. Because most malformations occur on the face, PWS is a clinically significant problem in the majority of patients.
2. The late-stage cobblestoning appearance of PWS subjects is comprised by not only pronounced vascular ectasia with proliferation of thin and/or thick-walled vessels and their stroma, but also numerous epithelial, neural and mesenchymal hamartomatous abnormalities. Despite these histologic observations, the specific mechanisms involved in PWS nodular formation remains unclear.
3. In one nodular PWS subject, we found that phosphatidylinositol 3-kinases (PI3K)/protein kinase B (AKT) and phosphoinositide phospholipase C g subunit (PLC-g) were activated in both hypertrophic areas and nodules within the lesion. These observations led us to hypothesize that the PI3K pathway may play an important role in nodular formation.

Study Overview

• Status: Completed
• Conditions: Port Wine Stain
• Intervention / Treatment: Radiation: Pulsed dye laser (PDL)

• Study Type: Interventional
• Enrollment (Anticipated): 80
• Phase: Not Applicable

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 3 years to 66 years (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

Description

Inclusion Criteria:

• Age 1 month - 70 years old, male or female;
• The patient and the family of the child agreed to participate in the experiment and signed an informed consent form;
• Clinical diagnosis of refractory port wine stains with thickened nodular or PDL resistance; .There is no bleeding, ulceration, infection, etc. affecting the visual field of laser surgery operation.

Exclusion Criteria:

• Patients with severe infectious diseases;
• Heart disease patients;
• Epilepsy patient;
• Pregnant patient;
• Researchers believe that patients who are not suitable for this experiment

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Basic Science
• Allocation: Randomized
• Interventional Model: Single Group Assignment
• Masking: Double

Number of Arms

2

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: Treatment group; Patients with port wine stains receive PDL (1.5-10ms, 11-12.5J/cm2) with a treatment range of approximately 10*10cm2	Radiation: Pulsed dye laser (PDL); Pulsed dye laser (PDL, 595nm) is effective for vasodilatory diseases, especially for the superficial to middle layers of the dermis
No Intervention: No treatment group; Patients with port wine stains have not been treated with PDL treatment

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Exon mutation sequences and mutation frequencies	In patients with nodular port wine stains, the exon mutation sequence and mutation frequency were compared between normal skin tissue (or blood), erythema and nodular tissue of the same patient.	Baseline (before treatment)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Changes in the levels of cytokines in serum and skin tissues	Changes in the levels of VEGF, FGF, HGF, PDGF, TGF-beta and other factors in serum and skin tissues before and after laser treatment	baseline (before treatment) and 1, 3, 7 days after treatment

Collaborators and Investigators

• Sponsor: xjpfW
• Collaborators: Cancer Institute and Hospital, Chinese Academy of Medical Sciences; First Hospital of China Medical University; Air Force General Hospital of the PLA
• Investigators: Principal Investigator: Gang Wang, Prof, Dermatology Derpartment of Xijing Hospital

Study record dates

Study Major Dates

• Study Start (Actual): October 1, 2015
• Primary Completion (Actual): October 1, 2019
• Study Completion (Actual): December 1, 2019

Study Registration Dates

• First Submitted: May 10, 2019
• First Submitted That Met QC Criteria: May 13, 2019
• First Posted (Actual): May 14, 2019

Study Record Updates

• Last Update Posted (Actual): March 11, 2021
• Last Update Submitted That Met QC Criteria: March 10, 2021
• Last Verified: March 1, 2021

More Information

Terms related to this study

• Keywords: port wine stain, PI3K, vascular malformation,PDL
• Additional Relevant MeSH Terms: Skin Diseases; Neoplasms by Histologic Type; Neoplasms; Congenital Abnormalities; Skin Abnormalities; Hemangioma; Neoplasms, Vascular Tissue; Hemangioma, Capillary; Port-Wine Stain
• Other Study ID Numbers: XijingH-PF-20150902

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No