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[Panhypopituitarism in one identical twin: the effect of hormone replacement]
Harrij Del Canho1, Janiëlle van Alfen-van der Velden, Riwka del Canho
1Medisch Spectrum Twente, afd. Kindergeneeskunde, Enschede, the Netherlands.
Insights
Panhypopituitarism in childhood is rare, especially in identical twins. Hormone replacement therapy can effectively manage growth delays and allow catch-up growth in affected children.
Area of Science:
- Pediatric Endocrinology
- Genetics
- Hormone Therapy
Background:
- Childhood panhypopituitarism is a rare endocrine disorder.
- A unique case involves a boy with an identical twin brother, where only one twin is affected.
- Studying this scenario offers insights into hormone deficiency consequences and treatment efficacy.
Observation:
- A 6-year-old boy presented with short stature, increased abdominal fat, and a smaller penis compared to his healthy identical twin.
- Diagnostic tests revealed central hypothyroidism, hypocortisolism, and growth hormone deficiency.
- Hormone replacement initiated at age 6 improved growth rate.
Findings:
- Testosterone therapy was introduced at age 15 due to lack of puberty and continued slow growth.
- The patient ultimately achieved a height exceeding that of his twin brother.
- Early childhood growth (first year) was not negatively impacted, but significant delays occurred thereafter.
Implications:
- Panhypopituitarism requires timely and comprehensive hormone replacement for optimal outcomes.
- Effective hormone therapy can lead to complete catch-up growth, even in severe pediatric cases.
- This case highlights the critical role of endocrine management in childhood development and achieving adult height potential.
Background:
Panhypopituitarism in childhood is rare. It is even rarer if the disorder appears in a boy with an identical but healthy twin brother. In such a patient it is useful to study the consequences of the hormone disorder and the effect of hormone replacement.
Case Description:
A 6-year-old boy saw a paediatrician because of short stature. He was much shorter than his identical twin brother and he had more abdominal fat mass and a smaller penis. Laboratory tests identified hypothyroidism of central origin, in combination with hypocortisolism and growth hormone deficiency. Hormonal replacement resulted in an improvement in growth rate. At the age of 15 years, testosterone therapy was introduced because puberty had not occurred and his growth rate was low. Finally the patient grew a few centimetres taller than his twin brother.
Conclusion:
In the first year of life, panhypopituitarism has no negative consequences for growth. After this point, growth is clearly delayed. With sufficient replacement growth can completely catch up.
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