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(Albany, New York) – October 08, 2026 – The market dynamics for Congenital Hyperinsulinism (CHI) are witnessing significant growth, driven by increasing disease awareness, advancements in genetic testing, improvements in neonatal diagnosis, and growing demand for effective treatments for persistent hypoglycemia. The therapeutic landscape is gradually evolving from conventional glucose management and insulin-suppressing medications toward targeted approaches designed to address abnormal insulin signaling. Additionally, the development of emerging therapies such as ersodetug (RZ358), ZEGALOGUE (dasiglucagon), and efpegerglucagon is expected to create new treatment opportunities and influence the congenital hyperinsulinism market during the forecast period.

DelveInsight, a leading market research firm, announces the release of its latest report, “Congenital Hyperinsulinism Market Insights, Epidemiology, and Market Forecast – 2036.” The report provides an in-depth assessment of congenital hyperinsulinism, including historical and forecasted epidemiology, current treatment practices, emerging therapies, market dynamics, competitive landscape, and growth opportunities across the United States, EU4 (Germany, Spain, Italy, and France), the United Kingdom, and Japan.

Key Takeaways from the Congenital Hyperinsulinism Market Report

• The Congenital Hyperinsulinism market across the 7MM was valued at approximately USD 110 million in 2025 and is expected to grow at a CAGR of 12% during 2026–2036.

• The United States accounted for approximately USD 45 million in 2025, representing the largest individual country market among the 7MM.

• The total diagnosed prevalent population of congenital hyperinsulinism across the 7MM was approximately 15,700 cases in 2025, with the patient population expected to increase during the forecast period.

• The United States recorded the highest diagnosed prevalence among the seven major markets.

• Among the EU4 and the UK, Germany accounted for the highest number of congenital hyperinsulinism cases, with approximately 2,800 cases in 2025.

• In France, approximately 1,750 diffuse cases, 360 focal cases, and 165 atypical cases were reported in 2025.

• Congenital hyperinsulinism is primarily associated with mutations in genes such as ABCC8 and KCNJ11, which regulate pancreatic insulin secretion.

• Current treatment primarily involves diazoxide, somatostatin analogs, glucagon, nutritional management, and surgical intervention in selected severe cases.

• Key emerging therapies include ersodetug (RZ358), ZEGALOGUE (dasiglucagon), and efpegerglucagon.

• Major companies involved in the congenital hyperinsulinism therapeutic landscape include Zealand Pharma, Rezolute, and Hanmi Pharm, among others.

• The development of targeted therapies capable of addressing severe and treatment-resistant hypoglycemia is expected to create opportunities for market expansion.

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Key Factors Driving the Congenital Hyperinsulinism MarketIncreasing Awareness and Improvements in Early Diagnosis

Growing awareness of congenital hyperinsulinism among pediatricians, neonatologists, endocrinologists, and other healthcare professionals is contributing to earlier identification of affected infants.

Congenital hyperinsulinism is characterized by excessive insulin secretion despite low blood glucose levels. Because symptoms such as seizures, lethargy, feeding difficulties, and irritability may overlap with other neonatal conditions, diagnosis can be delayed.

Greater recognition of persistent neonatal hypoglycemia and improvements in diagnostic protocols are helping clinicians distinguish congenital hyperinsulinism from other causes of low blood glucose.

Earlier diagnosis enables timely treatment initiation, potentially reducing the risk of permanent neurological injury and increasing the number of patients receiving appropriate medical care.

Advancements in Genetic Testing and Precision Diagnostics

The increasing adoption of molecular diagnostic technologies represents another important factor supporting the congenital hyperinsulinism market.

Mutations in genes such as ABCC8 and KCNJ11 are among the most common genetic causes of congenital hyperinsulinism. These genes encode components of ATP-sensitive potassium channels involved in regulating insulin secretion.

Advances in next-generation sequencing and genetic testing are improving the identification of causative mutations and helping clinicians distinguish between different genetic and histological forms of the disease.

Genetic characterization is particularly important because treatment response can vary considerably depending on the underlying mutation.

Improved understanding of disease biology is also supporting the development of therapies targeting specific molecular pathways involved in abnormal insulin regulation.

Increasing Demand for Effective Treatments in Diazoxide-Unresponsive Patients

Although diazoxide remains an established first-line pharmacological treatment, a proportion of congenital hyperinsulinism patients do not respond adequately.

Patients with severe genetic forms of the disease may experience persistent or recurrent hypoglycemia despite conventional treatment, requiring additional medications, continuous glucose administration, or surgical intervention.

The limited effectiveness of existing therapies in these patients has created a substantial need for treatments capable of providing more consistent glycemic control.

Investigational therapies such as ersodetug (RZ358) are designed to modulate insulin receptor activity rather than directly suppress insulin secretion, representing a differentiated approach to managing congenital hyperinsulinism.

Growing Development of Targeted and Mechanism-Based Therapies

The congenital hyperinsulinism treatment landscape is gradually expanding beyond traditional therapies toward agents targeting abnormal insulin signaling and glucose regulation.

Research efforts are focused on developing therapies that can reduce the frequency and severity of hypoglycemic episodes while minimizing treatment burden.

New approaches involving insulin receptor modulation, glucagon-based therapies, and other molecular mechanisms could potentially improve disease management, particularly in patients with severe or refractory congenital hyperinsulinism.

However, the commercial potential of these therapies remains dependent on successful clinical development and regulatory approval.

Increasing Focus on Preventing Neurological Complications

Persistent hypoglycemia during infancy can result in serious neurological complications, including seizures, developmental delays, cognitive impairment, and permanent brain injury.

Maintaining stable blood glucose levels is therefore essential to reducing long-term disease burden.

The potential consequences of inadequately controlled hypoglycemia are encouraging investment in earlier diagnosis, improved monitoring technologies, and more effective therapeutic interventions.

Congenital Hyperinsulinism Competitive Landscape

The congenital hyperinsulinism competitive landscape is evolving as pharmaceutical companies pursue therapies targeting different aspects of glucose regulation and insulin signaling.

Major companies identified in DelveInsight’s report include Zealand Pharma, Rezolute, and Hanmi Pharm, among others.

Zealand Pharma has been developing ZEGALOGUE (dasiglucagon) for hypoglycemia associated with congenital hyperinsulinism.

Dasiglucagon is a glucagon analog that increases blood glucose concentrations by stimulating hepatic glucose release. Its mechanism provides a potential therapeutic approach for patients experiencing persistent hypoglycemia due to excessive insulin secretion.

Although ZEGALOGUE is approved for severe hypoglycemia in people with diabetes, its congenital hyperinsulinism indication remains subject to regulatory review. The US FDA issued a second Complete Response Letter in October 2024, requesting additional analyses of clinical trial data. The report indicates that a new drug application resubmission was anticipated during the second half of 2026.

Rezolute is developing ersodetug (RZ358), an investigational monoclonal antibody that functions as a negative allosteric modulator of the insulin receptor.

Unlike conventional treatments that primarily suppress insulin secretion, ersodetug is designed to reduce the biological effects of excessive insulin signaling.

The therapy has received regulatory designations, including Breakthrough Therapy Designation in the United States, Orphan Drug Designations in the United States and Europe, and inclusion in the United Kingdom’s Innovative Licensing and Access Pathway.

However, Rezolute reported in December 2025 that its Phase III sunRIZE trial did not meet its primary endpoint of reducing weekly hypoglycemic events. The company subsequently continued evaluating the regulatory pathway for the program.

Hanmi Pharm is also identified among the companies involved in the congenital hyperinsulinism therapeutic landscape, including development activity associated with glucagon-based approaches such as efpegerglucagon.

The future competitive environment will depend on clinical efficacy, safety, treatment convenience, regulatory outcomes, and the ability of emerging therapies to address patients who remain inadequately controlled with existing medications.

Discover more about therapies set to impact the Congenital Hyperinsulinism market @

Congenital Hyperinsulinism Treatment Landscape

Recent Developments in the Congenital Hyperinsulinism Market

• August 2026: Rezolute continued evaluating the regulatory pathway for ersodetug (RZ358) following results from the Phase III sunRIZE study. The company anticipated discussions with the US FDA during the second half of 2026 to determine the next steps for the congenital hyperinsulinism program.

• August 2026: Rhythm Pharmaceuticals continued advancing preclinical research programs related to congenital hyperinsulinism as part of its broader rare neuroendocrine disease pipeline.

• December 2025: Rezolute announced that its Phase III sunRIZE study evaluating ersodetug in congenital hyperinsulinism did not achieve its primary endpoint of reducing weekly hypoglycemic events.

• October 2024: The US FDA issued a second Complete Response Letter for ZEGALOGUE (dasiglucagon) for the treatment of hypoglycemia in infants with congenital hyperinsulinism. The agency requested additional analyses involving continuous glucose monitoring data from the Phase III program.

These developments demonstrate the continued interest in developing targeted therapies for congenital hyperinsulinism while highlighting the clinical and regulatory challenges associated with bringing new treatments to market.

What is Congenital Hyperinsulinism?

Congenital Hyperinsulinism (CHI) is a rare inherited metabolic disorder characterized by inappropriate and excessive insulin secretion from pancreatic beta cells, resulting in persistent or recurrent hypoglycemia.

The disease typically presents during the neonatal period or early infancy and can vary considerably in severity.

Under normal physiological conditions, insulin secretion decreases when blood glucose concentrations fall. However, in congenital hyperinsulinism, abnormalities in pancreatic beta-cell regulation cause insulin to continue being released despite hypoglycemia.

This excessive insulin activity suppresses hepatic glucose production and ketone formation, reducing the availability of essential energy substrates for the brain.

Consequently, affected infants may experience seizures, lethargy, feeding difficulties, irritability, developmental delays, and neurological injury.

Congenital hyperinsulinism is frequently associated with genetic mutations affecting pancreatic beta-cell function, particularly ABCC8 and KCNJ11.

The condition is commonly classified into diffuse, focal, and atypical forms according to the distribution and characteristics of abnormal insulin-secreting pancreatic tissue.

Diffuse congenital hyperinsulinism affects beta cells throughout the pancreas, whereas focal disease involves a localized area of abnormal insulin secretion.

Accurate classification is important because treatment approaches and surgical outcomes differ considerably between these forms.

Diagnosis generally involves biochemical evaluation during hypoglycemic episodes, genetic testing, and specialized imaging such as 18F-DOPA PET/CT to distinguish focal from diffuse disease.

Congenital Hyperinsulinism Epidemiology Segmentation

DelveInsight’s congenital hyperinsulinism epidemiological assessment includes:

• Total diagnosed prevalent cases of congenital hyperinsulinism

• Type-specific diagnosed prevalent cases based on histological presentation

• Mutation-specific diagnosed prevalent cases

• Total treated cases of congenital hyperinsulinism

According to DelveInsight estimates, approximately 15,700 diagnosed prevalent congenital hyperinsulinism cases were recorded across the 7MM in 2025, with the patient population expected to increase during the forecast period.

The United States accounted for the highest diagnosed prevalence among the seven major markets.

Among the EU4 and the United Kingdom, Germany recorded the highest number of cases, with approximately 2,800 diagnosed prevalent cases in 2025.

In France, the histological distribution of congenital hyperinsulinism included approximately 1,750 diffuse cases, 360 focal cases, and 165 atypical cases in 2025.

The report also highlights the importance of mutation-specific epidemiology, particularly mutations involving ABCC8 and KCNJ11, which contribute substantially to the genetic burden of congenital hyperinsulinism.

In Japan, ABCC8 and KCNJ11 mutations accounted for the largest contribution among identified genetic categories.

Understanding these epidemiological patterns is important for evaluating treatment eligibility, identifying potential patient populations for emerging therapies, and forecasting future market demand.

Current Congenital Hyperinsulinism Treatment Landscape

The current treatment paradigm for congenital hyperinsulinism focuses on maintaining stable blood glucose concentrations, preventing recurrent hypoglycemia, and reducing the risk of neurological injury.

Treatment selection depends on disease severity, genetic background, histological presentation, and response to pharmacological interventions.

Diazoxide

Diazoxide is generally considered the first-line pharmacological treatment for congenital hyperinsulinism.

It acts by opening ATP-sensitive potassium channels in pancreatic beta cells, reducing insulin secretion and helping maintain blood glucose levels.

However, patients with certain genetic mutations, particularly those affecting the KATP channel, may demonstrate inadequate response.

Somatostatin Analogs

Somatostatin analogs such as octreotide and lanreotide are used in patients who do not respond adequately to diazoxide.

These therapies suppress insulin secretion by activating somatostatin receptors.

Octreotide may be administered subcutaneously or intravenously, while lanreotide provides a longer-acting treatment option.

Potential adverse effects include gastrointestinal complications, growth suppression, and gallstones.

Glucagon Therapy

Glucagon is used to increase blood glucose concentrations by stimulating hepatic glucose release.

It can be administered during acute hypoglycemic episodes or through continuous infusion in selected patients requiring ongoing glucose support.

Nutritional Management

Frequent feeding, continuous enteral nutrition, and intravenous glucose administration remain important components of congenital hyperinsulinism management.

Continuous glucose monitoring may also help clinicians and caregivers identify fluctuations in blood glucose levels and reduce the risk of severe hypoglycemic episodes.

Surgical Intervention

Surgical intervention may be necessary in severe or treatment-resistant congenital hyperinsulinism.

For patients with focal disease, targeted pancreatic resection can potentially provide a cure.

However, diffuse disease may require more extensive pancreatic surgery, which can increase the risk of subsequent diabetes and pancreatic insufficiency.

The limitations of existing treatment options reinforce the need for therapies capable of delivering effective and sustained glucose control without invasive procedures.

Unmet Needs in the Congenital Hyperinsulinism Market

Despite available treatment options, several important unmet medical needs remain.

The absence of broadly effective disease-modifying therapies represents one of the most significant challenges. Current treatments primarily focus on suppressing insulin secretion or correcting hypoglycemia rather than directly addressing the underlying genetic abnormalities.

Another major challenge is inadequate treatment response among patients with severe or genetically driven disease. Diazoxide resistance can substantially complicate disease management, while second-line therapies may not provide consistent glucose control.

The continued dependence on invasive surgical procedures in selected patients represents another important unmet need. Although focal pancreatic resection can be curative, extensive surgery in diffuse disease may create long-term metabolic complications.

The risk of permanent neurological injury remains a central concern, particularly when hypoglycemia is prolonged, recurrent, or inadequately recognized.

Additional challenges include delayed diagnosis, limited availability of specialized genetic testing, variability in clinical presentation, treatment-related adverse effects, and the burden of frequent glucose monitoring and nutritional interventions.

Emerging therapies capable of providing more durable glucose control, reducing treatment burden, and improving outcomes in diazoxide-unresponsive patients could address substantial gaps in the existing therapeutic landscape.

Scope of the Congenital Hyperinsulinism Market Report

The report covers the 2022–2036 study period, with 2022–2025 representing the historical period, 2026–2036 the forecast period, and 2026 as the base year.

Geographic coverage includes the United States, Germany, France, Italy, Spain, the United Kingdom, and Japan.

The report provides comprehensive analysis of:

• Congenital hyperinsulinism epidemiology and patient burden

• Historical and forecasted market size

• Diagnosed prevalent cases

• Histological type-specific patient distribution

• Mutation-specific epidemiology

• Total treated patient population

• Current treatment practices and guidelines

• Marketed and emerging therapies

• Clinical development pipeline

• Competitive landscape

• Drug uptake and patient-share analysis

• Market access and reimbursement

• Pricing and analogue assessment

• Key opinion leader insights

• SWOT and conjoint analysis

• Market drivers and barriers

• Unmet medical needs

• Future market opportunities

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Table of Contents

1. Key Insights

2. Report Introduction

3. Executive Summary of Congenital Hyperinsulinism

4. Key Events

5. Epidemiology and Market Forecast Methodology

6. Congenital Hyperinsulinism Market Overview at a Glance

7. Congenital Hyperinsulinism Disease Background and Overview

8. Congenital Hyperinsulinism Treatment and Management

9. Congenital Hyperinsulinism Treatment Guidelines

10. Congenital Hyperinsulinism Epidemiology and Patient Population

11. Total Diagnosed Prevalent Cases Across the 7MM

12. Histological Type-specific Diagnosed Prevalent Cases

13. Mutation-specific Diagnosed Prevalent Cases

14. Total Treated Cases

15. Congenital Hyperinsulinism Marketed Therapies

16. Congenital Hyperinsulinism Emerging Therapies

17. Drug Analysis and Competitive Landscape

18. Congenital Hyperinsulinism Market Outlook

19. Congenital Hyperinsulinism Market Size and Forecast

20. Drug Uptake Analysis

21. Market Access and Reimbursement

22. Pricing and Analogue Assessment

23. Congenital Hyperinsulinism Unmet Needs

24. KOL Views and Expert Insights

25. SWOT and Conjoint Analysis

26. Market Drivers and Barriers

27. Appendix

About DelveInsight

DelveInsight is a leading market research and consulting firm specializing in the life sciences and healthcare industries. Founded in 2014, the company provides comprehensive market intelligence, epidemiology, and insights across pharmaceuticals, biotechnology, medical devices, and emerging therapies. DelveInsight helps healthcare organizations make informed strategic decisions through data-driven research and industry expertise.

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