ESPN 54th Annual Meeting

ESPN 2022


 
Galectin-3 as a potential biomarker of renal and heart involvement in children with tuberous sclerosis complex
PIOTR SKRZYPCZYK 1 BEATA KUCINSKA 2 ANNA MARIA WABIK 1 MICHAL SZYSZKA 3 SERGIUSZ JOZWIAK 5 PRZEMYSLAW BOMBINSKI 4 ALEKSANDRA JAKIMOW-KOSTRZEWA 4 MICHAL BRZEWSKI 4 BOZENA WERNER 2 MALGORZATA PANCZYK-TOMASZEWSKA 1

1- DEPARTMENT OF PEDIATRICS AND NEPHROLOGY, MEDICAL UNIVERSITY OF WARSAW
2- DEPARTMENT OF PAEDIATRIC CARDIOLOGY, MEDICAL UNIVERSITY OF WARSAW
3- DEPARTMENT OF PEDIATRICS AND NEPHROLOGY, DOCTORAL SCHOOL, MEDICAL UNIVERSITY OF WARSAW
4- DEPARTMENT OF PEDIATRIC RADIOLOGY, MEDICAL UNIVERSITY OF WARSAW
5- DEPARTMENT OF PEDIATRIC NEUROLOGY, MEDICAL UNIVERSITY OF WARSAW
 
Introduction:

Elevated serum galectin-3 (Gal-3) is associated with numerous kidney and cardiovascular pathologies. Overexpression of Gal-3 was found in TSC (tuberous sclerosis complex)-related tumors and in serum of TSC adult patients with lymphangioleiomyomatosis. The study aimed to analyze serum Gal-3 in pediatric TSC patients and its relation with renal and cardiovascular manifestations of the disease. 

 

Material and methods:

In 38 children with TSC (age: 8.26±5.52 years, 19 boys, 19 girls), we evaluated serum Gal-3 levels [ng/mL], anthropometric parameters, renal lesion (by ultrasound and magnetic resonance imaging), heart lesions (by echocardiography), blood pressure, and biochemical parameters. The control group (CG) consisted of 20 healthy children (age: 8.83±4.68 years, 8 boys, 12 girls). 

Results:

Arterial hypertension was found in 4 (10.5%), renal angiomyolipomas (AML) in 24 (63.2%), and renal cysts in 30 (79.0%) TSC children; 24 (63.2%) had heart rhabdomyomas (not hemodynamically significant), only one child had GFR <90 mL/min./1.73m2 (77.1). TSC patients did not differ in Gal-3 compared to CG (2.80±1.59 vs. 2.72±1.54 [ng/mL], p=0.862). In children with TSC, Gal-3 correlated positively with systolic blood pressure Z-score (r=0.372, p=0.047) and negatively with left ventricular ejection fraction (r=-0.369, p=0.029). There was no difference in Gal-3 between patients with and without renal cysts, AMLs, and heart rhabdomyomas and no relation of Gal-3 with AML and cyst size. Gal-3 did not correlate with rhabdomyoma size, left ventricular mass index, or mitral and tricuspid annular plane systolic excursion. In CG, age was the strongest determinant of Gal-3 (r=-0.670, p=0.001) without relation of Gal-3 and blood pressure; in TSC patients, there was only trend towards negative association between Gal-3 and age (r=-0.294, p=0.073).

Conclusions:

      1. In TSC patients, increase in Gal-3 might reflect cardiovascular burden, but this requires further prospective studies.

      2Serum Gal-3 is not associated with presence or size of renal and heart tumors in pediatric patients with TSC.