Endocrine Abstracts

Congenital combined pituitary hormone deficiency (CCPHD) is a rare disease.

Although most of them are growth hormone deficient, growth retardation is not always the presenting symptom\. There is no normative data in children younger than 3 years regarding dose and frequency with rhGH treatment. The objective was to evaluate the timing of growth retardation and the first and second year growth response to rhGH compared to the mathematical growth prediction model in a large cohort of patients with CCPHD younger than 3 years of age. Twenty seven children were included. The occurrence of growth retardation analyzed as time to event showed that median age was 0.89±0.74 years with a mean height of −3.55 SDS (n=19). The remaining 8 grew well after a follow up of 2.29 years (range 0.42–3.64) despite low serum IGF1 in 6. rhGH treatment was started at a mean age of 1.76±1.0 years with a mean dose of 0.23±0.05 mg/kg per week(n=14).

rhGH induced an increment in height SDS of 1.81±0.98 and 0.88±0.81 at the first and second year respectively. Distance of target height from height at onset of treatment and GH doses were correlated with gain in height SDS throughout the first year of treatment (r=0.64, P0.01 and r=−0.66, P=0.01 respectively).

Predicted growth velocity was similar to observed growth velocity at the first year 15.1±4.1 vs 13.6±3.8 (P=NS) and second year 11.02±4.1 vs 10.09±2.3 (P=NS) respectively.

In summary most of the patients with CCPHD presented growth retardation before the first year of life. However, a normal growth pattern could be seen in face of abnormal IGF1 levels. All patients grew according to the mathematical model of growth prediction.

The significant correlation between GH doses and gain in height SDS represent an additional tool for those patients urged to attain a better catch-up growth.

Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.

Funding: This research did not receive any specific grant from any funding agency in the public, commercial or not-for-profit sector.