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Growth and development in patients operated on for islet cell dysplasia
Insights
Severe neonatal hypoglycemia due to islet cell dysplasia (ICD) can cause developmental issues. However, aggressive treatment and near-total pancreatectomy in infants resulted in normal psychomotor and neurologic development in a follow-up study.
Area of Science:
- Pediatrics
- Endocrinology
- Neonatology
Background:
- Neonatal hypoglycemia from islet cell dysplasia (ICD), or nesidioblastosis, can lead to significant psychomotor retardation and neurologic dysfunction.
- Early, aggressive treatment is crucial to prevent long-term adverse outcomes in affected infants.
Purpose of the Study:
- To evaluate the long-term neurodevelopmental and growth outcomes of infants treated with an aggressive protocol including near-total pancreatectomy for persistent neonatal hypoglycemia.
- To assess the efficacy of a comprehensive treatment strategy for severe neonatal hypoglycemia.
Main Methods:
- A cohort of 12 infants undergoing pancreatectomy for hypoglycemia between 1979-1984 were followed.
- Neurodevelopmental assessment included the Revised Yale Developmental Schedules, Peabody Picture Vocabulary, and Draw a Man Test.
- Growth, neurologic function, and psychomotor retardation were evaluated at follow-up (1.2-6.0 years).
Main Results:
- No significant growth abnormalities were observed in the patients.
- While some soft neurologic signs were present, no focal neurologic dysfunction was identified.
- The mean developmental quotient (DQ) was 99.2, indicating normal psychomotor function, with most patients showing average or above-average development.
- Psychomotor development correlated more strongly with socioeconomic factors than with the severity of neonatal hypoglycemia.
Conclusions:
- Infants treated aggressively for severe neonatal hypoglycemia, including pancreatectomy when necessary, can achieve normal neurodevelopmental and neurologic outcomes.
- The aggressive treatment protocol appears effective in mitigating the long-term risks associated with severe neonatal hypoglycemia.
- Long-term follow-up is recommended to monitor for any potential late-emerging sequelae.
Abstract:
Neonatal hypoglycemia caused by islet cell dysplasia (ICD), sometimes called nesidioblastosis, may lead to psychomotor retardation and neurologic dysfunction in up to 50% of patients who are not given early aggressive treatment. In 1979, we adopted a more aggressive protocol for treating this condition that consists of the following steps: immediate insertion of a silastic central venous line for reliable venous access; continuous intravenous infusion of glucose and glucagon to maintain euglycemia; oral diazoxide; and near total pancreatectomy if the first steps fail to overcome the hypoglycemia or the patient cannot be weaned off intravenous therapy. Twelve consecutive patients who underwent pancreatectomy for control of hypoglycemia between 1979 and 1984 were recalled and evaluated for growth delay, neurologic dysfunction, and psychomotor retardation using the Revised Yale Developmental Schedules, the Peabody Picture Vocabulary, and the Draw a Man Test. Follow-up ages ranged from 1.2 to 6.0 years with a median of 3.6 years. No significant growth abnormalities were identified. No patient exhibited focal neurologic dysfunction, although some demonstrated soft neurologic signs, which did not appear to be related to their earlier hypoglycemia. Psychomotor function for the group as a whole was normal, with a mean developmental quotient (DQ) of 99.2. The DQ was average for five patients and above average for four; no patient had a DQ in the frankly subnormal range. Psychomotor development correlated better with the family's socioeconomic and educational status than with the neonatal hypoglycemia. These children are developmentally and neurologically normal despite severe neonatal hypoglycemia. Continued follow-up will be necessary to detect any late sequelae.(ABSTRACT TRUNCATED AT 250 WORDS)