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Rare endocrine diseases are complex conditions that require lifelong specialized care due to their chronic nature and associated long-term complications. In Korea, a lack of nationwide data on clinical practice and outcomes has limited progress in patient care. Therefore, the Multicenter Networks for Ideal Outcomes of Pediatric Rare Endocrine and Metabolic Disease (OUTSPREAD) study was initiated.

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Pituitary gigantism (PG) is a rare endocrine disorder that may present with multiple pituitary hormone abnormalities in pediatric patients. A hallmark presentation is accelerated growth due to growth hormone (GH) excess. Current treatment modalities include surgery, radiation, and medical therapy.

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Article Synopsis
  • The study investigates sleep health in children and adolescents with congenital adrenal hyperplasia (CAH) using wrist actigraphy and sleep questionnaires.
  • It found that these children had poorer sleep duration, efficiency, and more awakenings compared to healthy peers, particularly related to hydrocortisone medication timing.
  • The results indicate that sleep issues are significant for children with CAH, highlighting the need for attention in clinical practice and further research.
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Pituitary Acrogigantism: From the Past to the Future.

Front Horm Res

November 2024

Department of Endocrinology, Centre Hospitalier Universitaire de Liège, University of Liège, Liège, Belgium.

Pituitary acrogigantism is a very rare disease that is caused by chronic growth hormone (GH) axis excess that begins during childhood and adolescence. As such, it represents one of the most severe manifestations of acromegaly. In most cases, acrogigantism is caused by a pituitary adenoma, but hyperplasia can also accompany the adenoma or rarely occur alone.

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Article Synopsis
  • The study investigates the glycemic patterns in children with congenital hyperinsulinism (HI) using Continuous Glucose Monitoring (CGM) over a 12-month period to provide better insights for future treatments.
  • It involved 45 patients, revealing a consistent risk of hypoglycemia, especially early in the morning, but showed that CGM did not effectively reduce these episodes and the device's accuracy was found to be poor.
  • Despite high dissatisfaction among patients and families regarding CGM usage, qualitative feedback indicated that it helped them understand glycemic patterns and make behavior adjustments to reduce hypoglycemia.
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