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Growth progression and 24-hour hormone profile in an infant treated chronically with a long-acting somatostatin
A Rodriguez1, E del Pozo, M D Rodriguez-Arnao
1Department of Pediatrics, Hospital General G. Marañón, Madrid, Spain.
Insights
This study shows Sandostatin (Octreotide) effectively managed hypoglycemia and seizures in a child with nesidioblastosis. Normal growth was maintained despite low growth hormone (GH) levels, suggesting alternative growth mechanisms.
Area of Science:
- Pediatric Endocrinology
- Pharmacology
Background:
- Nesidioblastosis is a rare cause of persistent hypoglycemia in infants.
- Recurrent seizures and hypoglycemia necessitate effective treatment strategies.
Observation:
- A 9-month-old male with nesidioblastosis, seizures, and hypoglycemia was treated with Sandostatin (Octreotide) after pancreatectomy failed.
- Treatment involved Sandostatin 25 mcg t.i.d. between carbohydrate-enriched meals.
- A 30-month follow-up assessed growth, weight, and hormonal profiles.
Findings:
- Sandostatin maintained blood glucose in the low normal range, resolving seizures and ensuring normal growth.
- Hormonal analysis revealed reactive hyperinsulinemia, a hyperglycemic effect of Sandostatin due to reduced insulin, and low mean growth hormone (GH) with normal somatomedin C.
- Normal growth progression occurred despite reduced GH secretion, indicating potential alternative growth regulation pathways.
Implications:
- Somatostatin analogs like Sandostatin can be effective in managing nesidioblastosis-related hypoglycemia and seizures.
- Normal growth in the presence of low GH secretion highlights complex postnatal growth mechanisms.
- Further research on somatostatin derivatives in non-GH-dependent conditions is needed to understand postnatal growth regulation.
Abstract:
The diagnosis of nesidioblastosis was established in a 9-month-old male child with a history of recurrent convulsive seizures and hypoglycemia. After unsuccessful subtotal pancreatectomy, treatment was started with the long-acting somatostatin derivative Sandostatin (Octreotide, Sandoz) at a dosage of 25 micrograms t.i.d. spaced between carbohydrate-enriched meals. With this regime, blood glucose was maintained at the low normal range and seizures ceased. During a 30-month observation period, growth velocity and weight progression were well within the predicted limits. A 24-hour hormone profile recorded at the end of the observation period revealed the following: (1) failure to improve blood glucose with carbohydrate-enriched food due to reactive hyperinsulinemia; (2) hyperglycemic reaction after administration of Sandostatin caused by a reduction of plasma insulin; this effect was particularly marked during sleep; (3) low mean GH, decreased spiking frequency and reduced area covered by the nocturnal peaks by recognized standards, and (4) normal somatomedin C levels for age. Interpretation of growth hormone (GH) data is hindered by the lack of pertinent information from the patient's age group. Recording of normal growth progression in the case illustrated here can only be explained by the capability of a reduced GH secretory rate to maintain full biological activity as shown by the normal plasma level of somatomedin C. Indeed, recent evidence has been provided elsewhere for normal growth progression in the presence of low GH secretion, although other factors unrelated to this hormone may also be operative at this early age. Further reports concerning the treatment of non-GH-dependent conditions with somatostatin derivatives will certainly contribute to the better understanding of the mechanisms governing growth in the postnatal period.