Children with isolated growth hormone deficiency: Empty sella versus normal sella.

BACKGROUND: Empty sella (ES) may be associated with variable clinical conditions ranging from the occasional discovery of a clinically asymptomatic pouch within the sella turcica to severe intracranial hypertension and rhinorrhea. The need for replacement hormone therapy in ES, as in other syndromes that may cause hypopituitarism, must be assessed for every single hormone, including growth hormone (GH). AIM: To determine whether or not the presence of ES could allow some changes in the GH responses of the isolated growth hormone deficiency (GHD) patients. MATERIALS AND METHODS: We included a cohort of 59 short stature children and adolescents with isolated GHD. According to computed tomography finding, they were classified into 2 groups: Group 1 included 40 children with normal sella and 19 children with ES in Group 2. All patients received recombinant human growth hormone (rhGH) with a standard dose of 20 IU/m(2)/week. RESULTS: The baseline results were not significantly different for all variables except weight standard deviation was smaller with statistical significant difference (P = 0.02). We identified no significant differences when comparing both groups, except for height standard deviation (HTSD) after the first year of therapy which revealed significant difference in favor of group 1. When comparing pre- and the two post-treatments HTSD results of the studied cases, all showed significant changes after GH therapy. The results of related variables pre-and post-treatment in both the groups showed significant improvement in all variables of the two groups of the study. CONCLUSION: Our study showed a similar stature outcome in the two treatment groups.

Amino acid and acylcarnitine profiles in premature neonates: a pilot study.

OBJECTIVE: To investigate the longitudinal changes in amino acid (AA) and acylcarnitine (AC) profiles of preterm neonates over the first 2 wk of life, and to detect any significant deviation from full term values that requires change of cut-off values used for detection of metabolic disorders in preterm neonates. METHODS: This observational analytical longitudinal study was conducted on 131 premature neonates (gestational age ranged from 27 to 36 wk) and 143 healthy full-term neonates. Dried blood spots were taken on the 5th and 14th postnatal day from the premature neonates and on day 5 from full term neonates for neonatal screening. Samples were analyzed for AA and AC using tandem mass spectrometer. RESULTS: Most AA significantly decreased on day 14 compared to day 5 among preterm neonates (p < 0.05). The combined values of total carnitine (TC), total acylcarnitine (tAC) and short-chain acylcarnitines on day 5 among preterm neonates were statistically significantly higher compared to the day 14 sample (p 0.0001), whereas no statistically significant difference was found regarding the values of medium-, long-chain acylcarnitines, tAC/FC, and FC/TC (p > 0.05). The levels of AA of preterm neonates were statistically significantly higher than that of the controls (p < 0.05). The values of TC, tAC, short-, medium- and long-chain acylcarnitines, were significantly higher than those of the controls (p < 0.05). The reference ranges for preterm neonates were determined using the 1st and 99.9th percentiles. CONCLUSIONS: AA and AC showed an age-related distribution of their concentrations. This underlines the importance of using appropriate reference values when working with a prematurely born population.

Growth response of Egyptian children with idiopathic short stature during four years of growth hormone therapy.

BACKGROUND: Multiple factors affect the growth response to recombinant human growth hormone (rhGH) in children with idiopathic short stature (ISS). AIM: To evaluate the growth responses of children with ISS treated with rhGH, aiming to identify the predictors of growth response. MATERIALS AND METHODS: We studied 120 cases, 90 males (75%), with a mean age of 13.8±2.7 years and 30 females (25%), with a mean age of 12.3±2.5 years. All patients received rhGH with a standard dose of 20 IU/m(2)/week. The calculated dose per week was divided into six days and given subcutaneous at night. RESULTS: A significant positive trend was detected in the delta changes of all anthropometric data. For the first year, the growth response was positively correlated to CA and BA delay and negatively correlated to height, weight and IGF-1 SDSs. For the second year, the growth response was correlated positively to first year growth velocity, BA, triceps skin fold thickness SDS and deviation from target height, and negatively correlated to weight, IGFBP3 SDS and target height SDS. For the third year, the growth response was positively correlated to five variables namely target height, 2(nd) year growth velocity, IGF-1 SDS, weight SDS and triceps skin fold thickness SDS. For the fourth year, growth response was positively correlated to 2(nd) and 3(rd) year growth velocity, BA, deviation from target height and weight/ height SDS. CONCLUSION: Our study showed multiplicity of predictors that is responsible for response in ISS children treated with rhGH, and BA was an important predictor.

Bone age is the best predictor of growth response to recombinant human growth hormone in Turner's syndrome.

BACKGROUND AND OBJECTIVES: Recombinant human growth hormone (rhGH) is approved for use in children with Turner's syndrome (TS) in most industrialized countries and is recommended in the recently issued guidelines. We determined the growth responses of girls who are treated with rhGH for TS, with an aim to identify the predictors of growth response. MATERIALS AND METHODS: Fifty-six prepubertal girls with TS, documented by peripheral blood karyotype, were enrolled. All the patients received biosynthetic growth hormone therapy with a standard dose of 30 IU/m(2)/week. The calculated dose per week was divided for 6 days and given subcutaneously at night. RESULTS: This study showed that rhGH therapy provides satisfactory auxological results. Bone age delay is to be considered as a predictive factor which may negatively influence the effect of rhGH therapy on final height. The growth velocity in the preceding year is the most important predictor of rhGH therapy response. CONCLUSION: These observations help us to guide rhGH prescription, to reduce the risks and costs.