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Pre-pubertal growth in the hyperprostaglandin E syndrome
C Seidel1, S Reinalter, H W Seyberth
1University Children's Hospital, Heidelberg, Germany.
Insights
Indomethacin treatment promotes normal growth in children with hyperprostaglandin E syndrome (neonatal Bartter syndrome). Long-term skeletal growth catches up to that of healthy preterm infants, with weight and bone maturation normalizing.
Area of Science:
- Pediatric Endocrinology
- Nephrology
Background:
- Hyperprostaglandin E syndrome, also known as neonatal Bartter syndrome, is a condition affecting pre-pubertal children.
- Growth patterns in these children are often impacted, necessitating effective therapeutic interventions.
Purpose of the Study:
- To evaluate the long-term effects of indomethacin on the pre-pubertal body growth of children diagnosed with hyperprostaglandin E syndrome.
- To assess catch-up growth and final height achieved during extended indomethacin therapy.
Main Methods:
- Longitudinal follow-up of eight children with hyperprostaglandin E syndrome over 5-12 years.
- Monitoring of height standard deviation scores (SDS), corrected for prematurity, before and during indomethacin treatment.
- Assessment of weight, body mass index, and bone maturation, with correlation analysis against serum potassium and calcium excretion.
Main Results:
- Indomethacin therapy led to significant catch-up growth in the first two years, normalizing height SDS from -2.8 to -0.5.
- Weight, body mass index, and bone maturation reached normal ranges.
- Final height SDS was comparable to target height SDS and similar to other preterm children, irrespective of potassium or calcium levels.
Conclusions:
- Indomethacin treatment facilitates normal long-term skeletal growth in children with hyperprostaglandin E syndrome.
- The growth pattern under indomethacin resembles that of healthy preterm children, indicating therapeutic efficacy.
- Early intervention is crucial for optimal growth outcomes in this condition.
Abstract:
Pre-pubertal body growth was followed in eight children with the hyperprostaglandin E syndrome (neonatal Bartter syndrome) treated with indomethacin over a period of 5-12 years. When corrected for prematurity, the general growth pattern was normal, with the exception of a child with delayed therapy. From the first observation (usually at birth) to the start of indomethacin, the mean height standard deviation score (SDS) corrected for prematurity changed from -0.2 to -2.8. During the first 2 years of therapy rapid catch-up growth occurred, followed by a slow adaptation of the growth pattern to that of healthy children born at term. At last observation the mean corrected height SDS was -0.5 (range -1.9 to +0.9) and the mean target height -0.9 SDS (range -1.8 to +0.1). Weight, body mass index and bone maturation also reached the normal range. No correlation was found between height SDS per year and serum potassium levels or calcium excretion. We conclude that under indomethacin treatment long-term skeletal growth of children with the hyperprostaglandin E syndrome is similar to that of other preterm children.