Congenital Hyperinsulinism
New congenital hyperinsulinism research, summarized each month in plain language.
2026 so far: In the months covered (January 2026 to September 2026), reports clarified surgery outcomes. Focal CHI was often followed by stopping hypoglycemia medicines, while diffuse disease had mixed results. Reports also expanded genetic explanations and added practical information on feeding and monitoring. Read the 2026 review
We also cover clinical trials.
Yearly update
2026 so far
- Surgery outcomes. Evidence broadened from small series to larger surgical outcomes.
- Medicines over time. Reports added longer follow-up and gene-linked response patterns.
- Feeding and hospital care. More real-world detail emerged on feeding and perioperative needs.
- Genetic testing updates. Testing broadened to mosaic and non-coding causes, with limits.
- Long-term outcomes. Follow-up reports broadened focus to neurologic and metabolic outcomes.
New
September 2026
- After-surgery medicines. After focal CHI surgery, many stopped low-blood-sugar medicines.
- Breastfeeding during diazoxide. In one cohort, on-demand breastfeeding continued during diazoxide treatment.
- Pasireotide case report. In one newborn, pasireotide did not steady blood sugar levels.
- Limits of ABCC8 results. ABCC8 genetic results did not consistently match focal vs diffuse CHI.
August 2026
- Blood sugar control. Most diazoxide-unresponsive children had steadier blood sugar with long-acting octreotide.
- Key side effects. Main issues were gallstones and temporary increases in liver tests.
- Ghrelin-like signals. In a KCNJ11 lab model, ghrelin-like drugs increased fasting blood sugar.
July 2026
- Bile duct imaging. A glowing dye helped surgeons see the bile duct during surgery.
- After pancreas surgery. After large pancreas removal, many still had glucose swings and digestion issues.
- Blood clot risk. One small report found blood clots in persistent CHI.
June 2026
- Diazoxide and genes. Genetic cause often matched whether diazoxide helped raise blood sugar.
- Focal CHI surgery. In one center, surgery cured most infants with focal CHI.
- Infant feeding support. By 6 months, full mouth feeding was less common in CHI.
May 2026
- Low-level mosaic variants in blood. In some people with CHI, a genetic change was detected in only a small fraction of blood cells.
- Very low signals may not repeat. Some of the faintest sequencing signals in blood did not confirm when a second testing method was used.
- Blood may not match other tissues. Mosaic genetic findings seen in blood may be different in other tissues.
April 2026
- Surgery monitoring. During pancreatectomy, children often needed ICU-level monitoring and very close blood sugar control.
- Diabetes after surgery. Some children developed diabetes after near-total pancreatectomy.
- Repeat genetic testing. Repeat genetic testing found a cause in some cases that were previously unexplained.
- HI/HA follow-up. In HI/HA syndrome reports, longer-term follow-up commonly included epilepsy and developmental concerns.
March 2026
- After surgery outcomes. After pancreatectomy, some children later developed diabetes, and some had low blood sugar return.
- CGM during surgery. In the operating room, continuous glucose monitors tracked glucose trends, but the readings did not always match fingerstick values.
- HK1 non-coding variants. Changes in a non-coding region of HK1 (intron 2) accounted for some previously negative genetic test results.
February 2026
- CHI in genetic syndromes. Reports described CHI occurring in Kabuki syndrome and in one CHARGE syndrome case, with specific gene changes appearing to align with who developed low blood sugar.
- BWS subtype and CHI severity. In Beckwith-Wiedemann syndrome, the genetic subtype was linked to differences in CHI severity, including whether children more often needed surgery or responded to diazoxide.
- Medication risk signals. Diazoxide-related pulmonary hypertension was uncommon overall but was reported more often in trisomy 13, and one letter described early alpelisib use in severe, drug-resistant CHI.
January 2026
- Focal surgery results. In one small comparison, robotic and open surgery for focal CHI had broadly similar short-term recovery, and some children still needed a second operation after an incomplete first resection.
- Finding focal lesions. Reports described using rapid genetic results together with 18F-FDOPA PET scans to localize focal lesions and plan smaller, more targeted pancreas surgery when focal disease is likely.
- What pancreas tissue shows. Tissue studies in diffuse CHI described insulin-cell marker patterns that looked less typical, and very low-level mosaic genetic changes were sometimes only found when testing isolated islet tissue.
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