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Published on: September 19, 2019
A CHILD WITH LARON SYNDROME ASSOCIATED WITH VASCULITIS
R Unsal Sac1, M A Tasar1, U Tiras2
1Ministry of Health Ankara Training and Research Hospital, Dept. of Pediatrics, Ankara, Turkey.
Insights
Laron syndrome, characterized by low insulin-like growth factor-I, is linked to vasculitis in a pediatric case. This suggests a potential role for insulin-like growth factor-I in vascular integrity and vasculitis development.
Area of Science:
- Endocrinology
- Vascular Biology
- Pediatric Rheumatology
Background:
- Laron syndrome is a rare genetic disorder characterized by severe insulin-like growth factor-I (IGF-I) deficiency.
- IGF-I plays a crucial role in vascular health, development, and protection against oxidative stress.
- Previous studies have linked congenital IGF-I deficiency to various vascular disorders, but not vasculitis.
Observation:
- A two-and-a-half-year-old Turkish girl diagnosed with Laron syndrome presented with acute vasculitis lesions.
- Clinical and laboratory evaluations excluded specific infectious agents and autoimmune diseases.
- The vasculitis lesions resolved spontaneously during hospitalization without complications.
Findings:
- This case represents the first reported association between Laron syndrome and vasculitis in the English literature.
- The findings suggest a potential underlying mechanism connecting IGF-I deficiency and vasculitis development.
- IGF-I's known protective effects on endothelial cells may be relevant to the observed vasculitis.
Implications:
- The study highlights a potential novel association between Laron syndrome and vasculitis.
- Further research is warranted to elucidate the shared pathophysiological mechanisms.
- The therapeutic potential of IGF-I or recombinant human IGF-I in managing vasculitis requires investigation.
Background And Objectives:
Levels of insulin-like growth factor-I are characteristically low in Laron syndrome which is a factor that has important roles on vascular health and development. Congenital insulin-like growth factor-I deficiency was reported to be associated with some vascular disorders. However, vasculitis diseases and Laron Syndrome association has not been reported in English literature up to date.
Patient:
We report the case of a two and a half years old Turkish girl, who was diagnosed as Laron syndrome when she was 12 months old. She presented with acute vasculitis lesions. Her physical examination and laboratory studies did not reveal a specific infectious agent or also an autoimmune disease was not detected. Her lesions disappeared during hospitalization without a complication.
Conclusion:
Since insulin-like growth factor-I reduces endothelial cell oxidative stress and maintains the structural integrity of vessels, some common mechanisms might be responsible for the occurrence of vasculitis in this patient with Laron syndome. The role of insulin-like growth factor-I and recombinant human insulin-like growth factor-I treatment choice in vasculitis diseases is a matter of investigation.
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