TREATMENT OF ACHONDROPLASIA WITH GROWTH HORMONE (r.h.G.H)

Zosi Paraskevi. Georgiadis Hercules, Karakaidos Dionisios, Tsilivakos Dimitrios, Vlahou Thomais, Karis Christos

Pediatric Department General Hospital of Nikea-Piraeus GREECE

Achondroplasia is one of the most common forms of skeletal dysplasia causing short-limbed short stature. Its treatment with growth hormone (GH) has recently been reported, but the effect of final height is not yet known.

In this study we report a case of a 7.5 year old girl, with achondroplasia and growth hormone deficiency in two provocative tests (L-Dopa, I.T.T or Clonidine) (partial deficiency). The patient's birth weight was 3200 gr and height 49.5 cm while short stature was noted at 5.5 years. She was born with cesarean section.

Treatment with r.h.GH 0.5 lU/Kg per week was commenced. Height velocity increased 7.3 cm during the first year and 5.6 cm in the second year from 2.8 cm/year pretreatment. There has been no change of body proportion and bone age. The r.h.GH therapy continues and the patient will be followed to final height.

IN CONCLUSION rhGH increased the height velocity of children with achondroplasia. It is impossible to predict whether this improvement will translate to a taller adult height. No significant side effects attributable to GH administration were observed.