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IGF-I replacement therapy in children with congenital IGF-I deficiency (Laron syndrome) maintains heart dimension and

Mickey Scheinowitz1, Micha S Feinberg, Zvi Laron

  • 1Neufeld Cardiac Research Institute, Petah Tikva, Tel Aviv University, Israel. mickey.scheinowitz@medstar.net

Growth Hormone & IGF Research : Official Journal of the Growth Hormone Research Society and the International IGF Research Society
|January 2, 2009
PubMed

Insights

Insulin-like Growth Factor-I (IGF-I) therapy helps young patients with Laron syndrome maintain normal heart size and function. This treatment ensures cardiac dimensions and function remain within the expected range for their age group.

Area of Science:

  • Pediatric Endocrinology
  • Cardiology
  • Genetics

Background:

  • Congenital growth hormone deficiency (GHD) and IGF-I deficiency lead to dwarfism, acromicria, and organomicria, including cardiac underdevelopment.
  • Laron syndrome (LS) is a form of GHD characterized by IGF-I deficiency.

Purpose of the Study:

  • To assess cardiac dimensions and function in very young patients with Laron syndrome undergoing IGF-I replacement therapy.
  • To compare cardiac parameters of treated LS patients with age- and gender-matched healthy controls.

Main Methods:

  • Echocardiographic measurements were performed on 8 young LS patients (mean age 7.1 years) during IGF-I therapy.
  • LS patient data were normalized to body surface area and compared to 44 healthy controls and standard nomograms.
  • Cardiac dimensions (LVDD, LVSD, LV mass) and function (shortening fraction, septal thickness) were evaluated.

Main Results:

  • Left ventricular diastolic and systolic dimensions and LV mass were significantly smaller in IGF-I treated LS patients compared to controls.
  • Shortening fraction and intraventricular septum thickness were similar between LS patients and controls.
  • All treated LS patients' cardiac parameters were within 1 standard deviation of the mean for their age group.

Conclusions:

  • IGF-I therapy effectively maintains normal left ventricular dimensions and function in young Laron syndrome patients.
  • Cardiac dimensions and function in treated LS patients fall within the normal range compared to age-matched controls.
  • IGF-I replacement therapy is crucial for normalizing cardiac development in Laron syndrome.
Abstract

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