March 2026 Research Update

These studies add a clearer picture of what pancreatic surgery can look like for children with hyperinsulinism, both in the long run and in the operating room. One report followed children for years after pancreatectomy and found a mix of outcomes, including later diabetes in some and recurrent low blood sugar in others. Another study focused on monitoring during surgery and found that continuous glucose monitoring (CGM) was considered helpful and could track glucose changes, though accuracy was not perfect and larger studies are still needed. [1] [2]

In a single-center review of 14 children who had pancreatectomy, the authors reported that long-term results were not uniform: some children remained stable without major glucose problems, while others developed diabetes or had hypoglycemia come back over time. Neurodevelopmental delay was also reported in some children during follow-up, and one child died due to sepsis in the surgical course described. This study helps show why surgery is often discussed as a tradeoff between controlling hypoglycemia and the possibility of later complications. [1]

During surgery, one small observational study of 13 infants using Dexcom G6 CGM reported that high blood sugar was seen in 8 of 13 children, and staff often found CGM useful for tracking changes and reducing the need for repeated blood samples. The CGM readings were compared with fingerstick glucose and showed a mean absolute relative difference of 19.3%, meaning the sensor did not match point-of-care testing closely in all moments. This adds an early signal that CGM can be workable around surgery, but the measurement differences matter when decisions depend on precise numbers. [2]

A broader pediatric report on robot-assisted pancreatic surgery included only one child with congenital hyperinsulinism, so it does not say much about CHI specifically. Still, it reported that robotic surgery was feasible across pediatric pancreatic conditions, with conversions to open surgery and postoperative complications occurring in some patients. For CHI families, this mainly adds context that newer surgical approaches are being used in children, but CHI-specific results were not detailed. [3]

For patients and caregivers, this month adds practical context: pancreatectomy can lead to different long-term paths, including stable glucose for some children and diabetes or recurring hypoglycemia for others. The CGM study is early evidence that real-time sensor tracking during surgery may be helpful for managing glucose changes in the operating room, but it also showed that sensor readings can differ from fingerstick tests. None of these studies establish a single expected outcome for an individual child, but they help clarify the range of experiences that have been reported and what uncertainties remain around long-term results and monitoring methods. This information may matter most for understanding what researchers are measuring and trying to improve, rather than changing anything immediately. [1] [2] [3]

This month strengthens the idea that some congenital hyperinsulinism can be explained by genetic changes outside the more familiar CHI genes, especially in a non-coding region of HK1. A larger sequencing effort found HK1 intron 2 variants in a notable minority of previously genetics-negative patients, with a wide range of onset ages and mixed response to diazoxide. A separate family report adds lab evidence that at least one HK1 intron 2 variant can change how a gene-regulating protein (FOXA2) affects HK1 activity, although how this leads to hyperinsulinism in beta cells is still not fully known. [4] [5]

In 281 people with CHI who previously had negative genetic testing, sequencing focused on HK1 intron 2 found 16 unique non-coding variants in 18 patients (6.4%). The reported cases started anywhere from the first day of life up to 21 months, and both diazoxide-responsive and diazoxide-unresponsive patterns were seen. Some children in this group ultimately underwent pancreatectomy, showing that these variants were found in patients with more persistent disease as well as those who responded to medicine. [4]

In one family, a boy with CHI and his father carried a new HK1 intron 2 variant located at a FOX-binding site, which is a DNA spot where certain proteins attach to help control gene activity. In lab testing, the normal binding site supported activation by FOXA2, while the variant reduced this activation. This helps connect an HK1 intron 2 change to a measurable shift in gene regulation, even though the study also notes that the exact chain of events leading to CHI in pancreatic beta cells remains unclear. [5]

For families whose earlier genetic testing did not find an answer, these studies show that researchers are identifying additional genetic causes, including changes in regions that standard tests may not always cover. The HK1 finding may matter in the future because it could increase the chance of getting a genetic explanation and could clarify whether a variant happened new in a child (de novo) or was inherited, which can affect how families understand recurrence risk. At the same time, the mechanism is not fully worked out and the studies do not show that knowing about HK1 variants automatically predicts how severe CHI will be or which treatment will work. For now, this is mostly an advance in understanding and testing possibilities rather than a change to day-to-day care. [4] [5]

This month included small patient reports showing that CHI does not always fit neatly into simple categories like "medicine-responsive" or "one lesion that can be removed." One case series described newborns with diffuse disease who did not respond to diazoxide but did stabilize on octreotide after genetic testing and specialized imaging. Another series described patients who appeared to have both focal and diffuse features, where removing a focal lesion did not fully stop hyperinsulinism, but diazoxide could help after surgery for some. [6] [7]

In two newborns with mutations in KCNJ11 and ABCC8, diazoxide did not control low blood sugar, and both were reported to stabilize after starting octreotide. Genetic testing and 18F-DOPA PET/MRI imaging supported diffuse disease in both infants, and one infant also used continuous glucose monitoring. These cases underline that confirming the genetic cause and disease pattern can coincide with changes in which therapies appear to control glucose in an individual patient. [6]

In three patients described as diazoxide-unresponsive at presentation, 18F-DOPA PET identified focal pancreatic lesions that were surgically removed, but fasting studies after surgery still showed ongoing hyperinsulinism. Biopsies outside the lesions suggested diffuse involvement as well, and genetic results were described as supporting a mechanism for both focal and diffuse disease. After surgery, diazoxide was able to manage ongoing hyperinsulinism in some patients, showing that treatment response can change after part of the pancreas is removed. [7]

For patients and caregivers, these case series mainly add a more realistic picture of variability: some infants with diffuse CHI may not respond to diazoxide but may stabilize with octreotide, and some children can have a mixture of focal and diffuse features that complicates expectations around surgery. Because these reports involve only a few individuals, they cannot predict what will happen for most children, but they help clarify why genetic testing and imaging are often used to better explain what type of CHI a child has. They also highlight uncertainty that families often face, such as whether surgery will fully resolve hypoglycemia and whether medication response might shift over time. This information is best viewed as descriptive and hypothesis-building, rather than proof of which approach is best. [6] [7]