September 2026 Research Update

These studies add a clearer picture of what surgery can and cannot do for congenital hyperinsulinism when medicines are not enough. In focal CHI, minimally invasive surgery was often followed by stopping hypoglycemia medicines, while diffuse CHI had a wider range of results and sometimes trade-offs like postoperative high blood sugar. Imaging used to map where insulin overproduction is coming from matched surgical findings closely in the larger report, supporting its role in planning surgery. [1]

In a large single-center experience of minimally invasive surgery in 110 people, most focal cases were reported as cured without postoperative medication (91.1%). In diffuse CHI, near-total pancreatectomy had a higher reported cure rate than subtotal pancreatectomy (66.7% vs 51.2%), but diffuse cases also had higher risk of postoperative hyperglycemia and some surgical complications, and a few patients needed reoperation for ongoing hypoglycemia. [1]

A smaller series of 7 infants who had laparoscopic pancreatectomy reported that 6 were euglycemic after surgery with minimal short-term problems, although 1 later needed completion pancreatectomy for persistent hypoglycemia. Over an average follow-up of about 3.5 years, the report did not observe diabetes or pancreatic enzyme problems, but the authors noted that larger studies with longer follow-up are needed. [2]

For families facing surgery discussions, this month strengthens the idea that results depend strongly on whether CHI is focal or diffuse, with focal disease more often followed by stopping hypoglycemia medicines. It also makes the trade-offs easier to picture in diffuse disease: surgery may control low blood sugars, but some children can develop high blood sugar afterward or need more than one operation. These are reports from surgical centers rather than head-to-head trials, so they help set expectations and questions to ask, not predict what will happen for any one child. [1]

The smaller infant series is reassuring about what recovery and medium-term follow-up can look like in some settings, but it also shows that persistent hypoglycemia can still happen after a major pancreatectomy in a minority of children. The longer-term risks that matter most to families, like diabetes or digestive enzyme problems, were not seen in that group during the reported follow-up, but the follow-up time and number of children were limited. [2]

This month, the treatment stories were split between supportive care around established medicines and an early test of a newer drug. A breastfeeding study supported that day-to-day feeding can sometimes continue normally during diazoxide without obvious short-term safety problems, when glucose is monitored. In contrast, a single neonatal report found that pasireotide did not stabilize glucose and seemed to worsen swings between low and high blood sugars, leading to stopping the drug and proceeding to surgery. [3] [4]

In 17 infants treated with diazoxide, demand breastfeeding was continued without an increase in hypoglycemic episodes during transition to breastfeeding or during diazoxide reductions. The report also noted no fluid overload and no diazoxide-related adverse effects observed during the treatment period, suggesting breastfeeding progression was not disrupted in this group. [3]

In a neonate with severe CHI due to a homozygous ABCC8 mutation, off-label pasireotide was tried after diazoxide and octreotide with glucagon had not controlled glucose. The case report described no substantial improvement, more glycemic variability with more hypo- and hyperglycemic episodes, and the need to restart glucagon; pasireotide was stopped after 8 days and near-total pancreatectomy followed. No adverse effects were observed during the short pasireotide exposure. [4]

For caregivers, the breastfeeding report is practical: it describes a real-world group where breastfeeding on demand continued during diazoxide with stable sugars and no obvious short-term safety issues reported, which may be reassuring for families hoping to breastfeed while treatment is starting. At the same time, it does not prove that breastfeeding will be easy or stable for every infant, because there was no comparison group and details like severity varied. [3]

The pasireotide report mainly adds an early warning that this newer somatostatin-type medicine may not reliably improve glucose control in very severe neonatal CHI, at least in the situation described, and could increase glucose ups and downs. Because it is one patient with several therapies overlapping, it is not a final answer about pasireotide, but it helps set realistic expectations about how uncertain results can be when trying newer options. [4]

This month reinforced that ABCC8-related CHI is not one single pattern, even when the gene finding sounds similar. Across a pediatric series and two case reports, people with ABCC8 variants ranged from infants with focal or diffuse disease to a 41-year-old with late-presenting hyperinsulinemic hypoglycemia. These reports also showed that medicine response can be surprising, including diazoxide responsiveness in an infant with a homozygous ABCC8 mutation. [5] [6] [7]

In a 5-patient pediatric series with paternally inherited ABCC8 variants, the clinical picture varied: one child had a focal lesion and others were described as diffuse, and diazoxide responsiveness differed across cases. One child had an additional maternally inherited exon deletion (making two ABCC8 changes) and had diffuse disease unresponsive to diazoxide, while other children with a single paternal variant could still have diffuse disease. All were reported to have normal neurodevelopment at follow-up (ages 8 months to 13 years). [5]

One infant case from Nepal described a homozygous ABCC8 mutation with diffuse uptake on functional imaging, yet sustained glycemic control on diazoxide was achieved, which was noted as unexpected. The report also described a seizure when diazoxide was interrupted, and normal developmental milestones at 15 months. Practical constraints such as limited access to imaging and medication supply were part of the course described. [6]

An adult case report described a 41-year-old man with recurrent hyperinsulinemic hypoglycemia who carried a novel heterozygous ABCC8 variant classified as likely pathogenic. Glycemic control improved on diazoxide plus octreotide, and the authors emphasized considering CHI as a possible explanation even when hyperinsulinemic hypoglycemia is first recognized in adulthood. Follow-up ended after several months, and imaging did not definitively exclude other rare causes in the report. [7]

For families, these studies make the genetics story more realistic: an ABCC8 result does not automatically predict whether disease will be focal or diffuse, or whether diazoxide will work. They also show that CHI-like hyperinsulinemic hypoglycemia can sometimes be recognized later in life, which may matter for adults who have long had unexplained low blood sugars. This evidence is mostly small series and individual cases, so it helps explain what is possible, but it cannot forecast an individual child or adult's course. [5] [6] [7]

The pediatric series reporting normal neurodevelopment and the infant case reporting normal milestones both highlight that good developmental outcomes can occur when hypoglycemia is managed, but the reports do not prove which approaches guarantee this. What remains uncertain is why diazoxide response and disease pattern vary so much even within ABCC8-related CHI, and whether there are additional genetic factors that standard testing can miss. [5] [6]

One publication this month did not report new patient outcomes, but it pulled together how CHI care is commonly organized in children. It described a stepwise approach using blood and hormone testing, genetic testing, and functional imaging to tell focal from diffuse disease, because that distinction shapes treatment choices. It also summarized how diazoxide and octreotide are typically used and when surgery is considered, along with follow-up and rehabilitation planning. [8]

The guideline noted that diazoxide is generally used first, with octreotide as a next option, and that 18F-DOPA PET-CT is recommended to help separate focal from diffuse forms before surgery. It also described possible side effects to watch for with these medicines and discussed surgical risks and the need for ongoing developmental and nutritional support. The authors stated that some recommendations rely on moderate-to-low certainty evidence or expert consensus, and access to specialized imaging can vary. [8]

For patients and caregivers, this guideline is most useful as a map of what the care pathway can include, from diagnosis through long-term follow-up, rather than as new evidence that one approach works better than another. It may help families recognize why genetic testing and specialized imaging are often discussed, since treatment decisions can depend on whether disease is focal or diffuse. Uncertainty remains because the guideline acknowledges that parts of CHI care are based on limited evidence and local availability of tests and imaging. [8]