Autosomal Recessive Hyperinsulinism Due to Sulfonylurea Receptor-1 (SUR1) Deficiency

Autosomal recessive hyperinsulinism due to SUR1 deficiency is a rare genetic condition that starts at birth or soon after. The pancreas makes too much insulin even when blood sugar is low. Insulin lowers sugar, so babies get repeated low blood sugar (hypoglycemia). Low sugar can cause jitteriness, poor feeding, sleepiness, seizures, or even brain injury if not treated fast. The problem sits in tiny channels (called ATP-sensitive potassium or KATP channels) on beta cells. These channels are built from two protein parts: SUR1 (made by the ABCC8 gene) and Kir6.2 (made by KCNJ11). In this disease, a child inherits two non-working ABCC8 copies (one from each parent). SUR1 then does not work or reach the cell surface. The KATP channel cannot open. The beta cell stays electrically active and keeps releasing insulin when it should not. That is why the sugar keeps dropping. Some children have disease in the whole pancreas (diffuse form). Some have a small spot making too much insulin (focal form). Doctors tell the two forms apart using a special scan called 18F-DOPA PET/CT. Treatment choices often depend on that scan and on whether the child responds to the standard drug diazoxide (many recessive ABCC8 cases do not respond). PubMed+4PMC+4MedlinePlus+4

Autosomal recessive hyperinsulinism from ABCC8 mutations happens when both copies of the ABCC8 gene are altered. ABCC8 encodes SUR1, the regulatory subunit of the pancreatic β-cell KATP channel. When SUR1 is non-working, the channel stays closed, calcium flows into the cell, and too much insulin is released, even when blood sugar is low. This causes persistent, often severe hypoglycemia from the newborn period onward. Some children have diffuse pancreatic involvement; others have a focal (small patch) lesion that can be cured surgically if localized. Early recognition and prevention of low glucose is critical to protect the brain. MedlinePlus+2NCBI+2

Normally, the β-cell KATP channel senses energy status and opens to stop insulin when sugar is low. In SUR1 deficiency, that “safety valve” is broken. The cell “thinks” sugar is high and keeps making insulin. This is why babies can need very high sugar delivery (IV glucose) to stay safe, and why medicines that open KATP (like diazoxide) often don’t work in ABCC8/KCNJ11-negative channels. MedlinePlus+1

Other names

• ABCC8-related congenital hyperinsulinism

• KATP-HI (KATP channel hyperinsulinism)

• SUR1 deficiency hyperinsulinism

• Familial hyperinsulinism type 1 (FHI1 / OMIM 256450)

• Diazoxide-unresponsive congenital hyperinsulinism (when caused by biallelic ABCC8/KCNJ11 variants)

• Focal congenital hyperinsulinism due to SUR1 mutations (for the focal form) PMC+1

Types

1) By pancreatic pattern

• Diffuse type. All beta cells have the defect. This is common in autosomal recessive ABCC8 disease. It can be severe and persistent. Medical therapy may control some cases; others used to need near-total pancreatectomy, though many centers try to avoid surgery. OUP Academic

• Focal type. A small patch of pancreas over-secretes insulin. It usually comes from a single paternal ABCC8 (or KCNJ11) mutation plus a second change in that patch (11p15 loss of maternal region). Surgery that removes only the focus can cure it. 18F-DOPA PET/CT helps localize the spot. PMC+2PubMed+2

2) By drug responsiveness

• Diazoxide-responsive. Uncommon for biallelic ABCC8 disease. Diazoxide opens KATP channels at the SUR1 site; when channels are absent or nonfunctional, it often fails. Some monoallelic or focal cases may respond. NCBI

• Diazoxide-unresponsive. Typical for many biallelic ABCC8 mutations and for most diffuse, severe cases. These children need other options. PMC+1

Causes

Note: the root cause is biallelic pathogenic variants in ABCC8 (SUR1). Below I break down the kinds of ABCC8 problems and the real-world factors that make hypoglycemia worse or reveal it.

1. Loss-of-function ABCC8 variants (homozygous or compound heterozygous). These stop SUR1 from working or reaching the cell surface. The KATP channel stays closed; insulin keeps flowing. PMC

2. Missense variants in SUR1 nucleotide-binding folds (NBF1/NBF2). These disturb Mg-nucleotide interactions needed to open the channel. OUP Academic

3. Splice-site variants. Faulty RNA splicing yields abnormal or truncated SUR1 protein. PMC

4. Nonsense/frameshift variants. Lead to early stop codons and loss of SUR1. PMC

5. Trafficking defects. SUR1 is made but cannot reach the membrane to form the channel. PMC

6. Focal lesion genetics. A paternal ABCC8 variant plus somatic loss of maternal 11p15 in a small pancreatic region causes a focal overactive spot. PMC

7. Consanguinity (parents related). Raises the chance both parents carry the same rare ABCC8 variant, so a child gets two copies. Thieme

8. Perinatal stress unmasking. Stress, illness, or poor feeding in the newborn can reveal underlying HI by tipping glucose balance. (Stress HI is separate, but it can co-exist or confuse early diagnosis.) Hopkins Medicine

9. Prolonged fasting. Longer time without feeds lowers glucose; with inappropriate insulin release, hypoglycemia appears. Frontiers

10. High carbohydrate or protein bolus followed by insulin overshoot. In KATP-HI, beta cells may over-respond and keep secreting after glucose falls. PMC

11. Leucine load. Leucine can stimulate insulin; leucine sensitivity is classic in some dominant forms, but protein loads can still worsen lows in KATP-HI. PMC

12. Intercurrent infections. Poor intake, vomiting, and higher energy use lower glucose reserves. Frontiers

13. Prematurity or low reserve. Less glycogen and fat stores mean faster drops when insulin is inappropriately high. Hopkins Medicine

14. Missed or delayed feeds. Very common trigger for symptomatic episodes. Frontiers

15. Post-operative stress or anesthesia fasting. Can precipitate hypoglycemia unless glucose is infused. Frontiers

16. Poor counter-regulatory response. In some infants, glucagon, cortisol, or GH responses are immature; this makes insulin excess more dangerous. Frontiers

17. Inaccurate home glucose monitoring or device issues. Missed lows extend neuroglycopenia. (Practice guidance stresses careful monitoring.) Congenital Hyperinsulinism International

18. Medication misinterpretation. Assuming diazoxide will work in all HI may delay effective plans in KATP-HI when it fails. NCBI+1

19. Delayed genetic testing. Slow confirmation can delay imaging and the right interventions (e.g., 18F-DOPA PET for focal disease). Congenital Hyperinsulinism International

20. Limited access to specialized imaging/surgery. Without 18F-DOPA PET/CT and experienced pancreatic surgery, focal “curable” cases may be missed. PubMed

Symptoms and signs

1. Jitteriness or tremors. A common early warning of low sugar in newborns. PMC

2. Poor feeding or vomiting. Babies refuse feeds or spit up during lows. PMC

3. Lethargy or excessive sleepiness. The brain lacks glucose and slows down. PMC

4. Irritability or inconsolable crying. Neuroglycopenia can present as fussiness. PMC

5. Apnea or pauses in breathing. Severe lows can depress breathing control. PMC

6. Hypothermia (low temperature). Another nonspecific sign in sick neonates. PMC

7. Seizures or twitching. When sugar drops further, seizures may occur; EEG may show changes during an event. PMC

8. Coma (rare, severe). If lows are deep and prolonged. PMC

9. Macrosomia at birth. Many infants are large for dates because insulin in utero is an anabolic hormone. Frontiers

10. Sweating, pallor, or fast heart rate. Adrenergic signs of hypoglycemia. Frontiers

11. Developmental delay (if repeated unrecognized lows). Brain injury risk rises with recurrent or prolonged hypoglycemia. Frontiers

12. Low ketones during lows. Families may hear the phrase “no ketones” in labs—the body is blocked from making ketones by insulin. Children’s Hospital of Philadelphia

13. Low free fatty acids during lows. Insulin suppresses fat breakdown. Children’s Hospital of Philadelphia

14. Rebound hyperglycemia after glucagon. Sugar may jump after emergency glucagon, hinting insulin-driven hypoglycemia. Children’s Hospital of Philadelphia

15. Medication “non-response.” Doctors may note poor response to diazoxide, which often flags KATP-HI from biallelic ABCC8 variants. PMC

Diagnostic tests

Physical examination (bedside)

1. General neonatal exam. Check activity, tone, feeding, temperature, and growth (macrosomia). These clues point to hypoglycemia and its severity. PMC+1

2. Neurologic exam during/after an event. Look for jitteriness, altered tone, or seizure signs; helps triage urgent glucose treatment and EEG if needed. PMC

3. Hydration and perfusion check. Poor perfusion can worsen glucose delivery to the brain and mimic sepsis; exam guides urgent care while labs are drawn. Frontiers

Manual/bedside tests (done quickly in the unit)

4. Capillary or plasma glucose (point-of-care then lab confirmation). Confirms low sugar; repeated checks guide IV dextrose and feeding plans. Frontiers

5. Bedside ketone check (blood/urine). In HI, ketones are suppressed during low sugar; this helps distinguish from ketotic hypoglycemia. Children’s Hospital of Philadelphia

6. Glucagon stimulation during a low. A rise in glucose >30 mg/dL after glucagon supports insulin-mediated hypoglycemia (glucagon releases liver glycogen). Children’s Hospital of Philadelphia

Laboratory and pathological tests (“critical sample” at the time of hypoglycemia)

7. Insulin level. Detectable or inappropriately high insulin at glucose <50–55 mg/dL supports HI (sometimes insulin is fleeting, so a normal value does not exclude HI). Children’s Hospital of Philadelphia

8. C-peptide and proinsulin. Show endogenous insulin secretion; elevated values during a low point to pancreatic insulin excess rather than injected insulin. Frontiers

9. Beta-hydroxybutyrate (BOHB). Low BOHB during a low sugar is typical in HI. Frontiers

10. Free fatty acids (FFAs). Low FFAs during a low support insulin action. Children’s Hospital of Philadelphia

11. Cortisol and growth hormone. Help rule out endocrine deficiencies that can also cause hypoglycemia. Frontiers

12. Lactate, blood gas, and ammonia. Screen for other metabolic causes; ammonia is important when considering HI/HA (GLUD1) but is typically normal in ABCC8 disease. Frontiers

13. Acylcarnitine profile and urine organic acids. Exclude fatty-acid oxidation and organic acidemias, which present differently (they usually have high ketones in simple fasting hypoglycemia; HI has low ketones). Frontiers+1

14. Genetic testing focused on ABCC8 (±KCNJ11). Rapid sequencing helps plan imaging and treatment; many centers prioritize ABCC8/KCNJ11 panels early if diazoxide fails. Congenital Hyperinsulinism International+1

15. Parental testing (segregation). Confirms autosomal recessive inheritance and helps with counseling for future pregnancies. Frontiers

Electrodiagnostic tests

16. Electroencephalogram (EEG) during or after a suspected seizure. Hypoglycemia can cause seizures; EEG supports diagnosis, tracks recovery, and helps separate seizure types. (EEG does not diagnose HI itself; it documents brain effects of lows.) PMC

17. Continuous cardiorespiratory monitoring. During acute management, monitors apnea and bradycardia related to neuroglycopenia; this is standard NICU practice to keep events safe. Frontiers

Imaging tests

18. 18F-DOPA PET/CT. The key imaging to tell focal from diffuse disease and to map the focus before surgery. It often changes management and can be curative in focal HI. PMC+2PubMed+2

19. Contrast-enhanced CT (with or after 18F-DOPA PET). Improves anatomic localization of the small focal lesion for a limited pancreatic resection. PubMed

20. Pancreatic MRI or ultrasound. These are less sensitive for HI patterning; they may be used when PET is not available, but negative results do not exclude disease. PET/CT remains preferred where available. PMC

Non-pharmacological treatments (therapies & others)

1. Immediate IV dextrose
   Give concentrated glucose via a drip to quickly raise blood sugar and protect the brain. It works by directly supplying glucose while insulin is still inappropriately high. PMC

2. Targeted glucose infusion rate (GIR) titration
   Adjust the IV glucose rate to keep plasma glucose safely >70 mg/dL (or per local guideline) while planning definitive therapy. It maintains brain fuel while insulin excess persists. PubMed

3. Frequent small enteral feeds
   If safe to feed, give frequent breast milk or formula to add a steady oral glucose source while IV support continues, reducing dips between feeds. PMC

4. Continuous enteral feeding (NG/ND)
   Use a nasogastric or nasoduodenal tube for continuous feeds when intermittent feeding fails to maintain euglycemia, especially overnight. Mechanism is steady glucose delivery. PMC

5. Rapid bedside monitoring
   Use frequent point-of-care glucose checks (and confirmatory labs) to catch and treat lows early. This reduces time spent hypoglycemic. Pediatric Endocrine Society

6. Hypoglycemia emergency plan & caregiver training
   Teach caregivers to recognize signs of low sugar and to act immediately with feeding, IV escalation, and rescue steps per center protocol. Training shortens time to treatment. Children’s Hospital of Philadelphia

7. Early transfer to an expert HI center
   Complex cases benefit from multidisciplinary teams (endocrinology, nuclear medicine, surgery) that can do 18F-DOPA imaging and focused surgery, cutting time to cure in focal disease. pedsurglibrary.com

8. Thermal stability and infection prevention
   Keep babies warm and reduce stressors; illness and cold stress can raise glucose needs and precipitate deeper lows by increasing metabolic demand. NCBI

9. Neurodevelopmental follow-up
   Arrange early developmental surveillance and therapy because prolonged or recurrent hypoglycemia increases neurodevelopmental risk; early therapy supports outcomes. NCBI

10. Dextrose (glucose) buccal gel (adjunct in newborn period)
    In late-preterm/term at-risk neonates, 40% oral dextrose gel reverses hypoglycemia faster and can reduce NICU admissions; in proven HI it is an adjunct, not definitive therapy. It supplies glucose through oral mucosa. PubMed+2ScienceDirect+2

11. Avoid prolonged fasting
    Institute “no-fast” or very short-fasting policies until medical or surgical control is achieved to prevent recurrent lows. Mechanism is avoiding liver glycogen depletion. PMC

12. Peri-procedure glucose protocols
    For imaging or surgery, use planned glucose infusions and monitoring to prevent intra- and post-procedure hypoglycemia. PMC

13. Genetic counseling for families
    Explain autosomal recessive inheritance, carrier status, and recurrence risks; helps planning and early detection in future pregnancies. womenshealth.labcorp.com

14. 18F-DOPA PET/CT (or PET/MRI) for focal mapping
    Imaging is not a drug but is decisive therapy-planning: it identifies curable focal lesions and prevents unnecessary near-total pancreatectomy. PMC+1

15. Intraoperative frozen section guidance
    During surgery, frozen pathology and intraoperative ultrasound help surgeons remove only diseased tissue, preserving pancreas and reducing later diabetes risk. Frontiers

16. Post-op glucose and enzyme support pathway
    After surgery, planned monitoring and enzyme supplementation if needed reduce complications and optimize nutrition. PMC

17. Education on home monitoring after discharge
    Teach families to check glucose at home per specialist advice and to escalate promptly for readings below the target range. Children’s Hospital of Philadelphia

18. Coordination with nutrition services
    Dietitians help craft feeding schedules and caloric density plans that smooth glucose delivery across 24 hours. PMC

19. Use of local/regional pathways
    Following structured hospital pathways for persistent hypoglycemia standardizes safe care and speeds escalation. Hopkins Medicine

20. Long-term transition plans
    As children grow, teams reassess the need for therapy, watch for evolution to diabetes in some genotypes, and plan school/daycare safety steps. Pediatric Endocrinology Journal

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Drug treatments

Important context: No medicine is FDA-approved specifically for congenital hyperinsulinism. Therapies below are used on-label for their approved indication or off-label for CHI based on expert guidelines. Always manage dosing and monitoring with a pediatric endocrinology team. PMC

1. Diazoxide (PROGLYCEM® oral suspension) – cornerstone when KATP channels are responsive
   Class: KATP channel opener (nondiuretic benzothiadiazine).
   Typical dose/time: Guidelines cite 5–15 mg/kg/day split 2–3 times daily; higher doses add side-effects without extra benefit. Start only in appropriate infants and monitor fluids and BP. Karger Publishers
   Purpose & mechanism: Keeps KATP channels open, hyperpolarizes β-cells, and suppresses insulin to stabilize glucose.
   Key label safety (FDA): Fluid retention, cardiac effects; and risk of pulmonary hypertension in infants—requires careful monitoring. FDA Access Data+1
   Notable adverse effects: Edema, hypertrichosis, GI upset; rare PH in neonates. FDA Access Data

2. Octreotide (SANDOSTATIN® / SANDOSTATIN LAR DEPOT) – off-label for CHI
   Class: Somatostatin analogue.
   Dose/time: Short-acting SC infusion/bolus titrated by specialists; LAR monthly IM after stabilization in some cases. FDA Access Data+1
   Purpose & mechanism: Inhibits insulin release via somatostatin receptors, reducing hyperinsulinemic hypoglycemia.
   Label safety (FDA): GI effects, gallstones; caution in infants—serious reactions reported in young children with octreotide injections. FDA Access Data+1
   Side effects: Abdominal pain, diarrhea, gallbladder issues; bradycardia possible. FDA Access Data

3. Lanreotide (SOMATULINE® DEPOT / Lanreotide Injection) – off-label for CHI
   Class: Long-acting somatostatin analogue (deep SC every 4 weeks).
   Purpose & mechanism: Same as octreotide; monthly dosing may improve logistics in selected older children.
   Label safety (FDA): Biliary disease, bradycardia, glucose shifts; dosing and indications are for acromegaly/GEP-NETs, not CHI. FDA Access Data+1

4. Glucagon (GVOKE®, BAQSIMI®, Glucagon for Injection) – rescue for severe lows
   Class: Antihypoglycemic hormone.
   Dose/time: Weight-based rescue dosing; can be given SC/IM (GVOKE, Glucagon), or intranasal (BAQSIMI) per label in diabetes; in HI it is rescue adjunct while securing IV glucose. FDA Access Data+2FDA Access Data+2
   Purpose & mechanism: Rapid hepatic glycogenolysis to raise glucose.
   Label safety (FDA): Nausea/vomiting; caution in pheochromocytoma/insulinoma (specialist oversight in HI). FDA Access Data

5. Sirolimus (RAPAMUNE®) – off-label in refractory diffuse CHI at expert centers
   Class: mTOR inhibitor immunosuppressant.
   Purpose & mechanism: May blunt β-cell hypersecretion in some refractory cases; used only when benefits outweigh risks.
   Label safety (FDA): Boxed warnings for serious infection/malignancy; requires drug-level monitoring and expert care. FDA Access Data

6. Everolimus (AFINITOR® / AFINITOR DISPERZ®) – off-label in highly selected cases
   Class: mTOR inhibitor.
   Purpose & mechanism: Similar rationale to sirolimus in rare refractory cases; pediatric formulations exist for other indications.
   Label safety (FDA): Stomatitis, infections, hyperglycemia, dyslipidemia; therapeutic drug monitoring required. FDA Access Data

7. Hydrochlorothiazide (adjunct to diazoxide) – supportive, not an HI drug per se
   Class: Thiazide diuretic.
   Purpose & mechanism: Pediatric endocrine protocols add a thiazide to counteract diazoxide-induced fluid retention and lessen risk of pulmonary edema/PH. Dose is individualized. Label per diuretic brand; use per local protocol. MDPI

8. Nifedipine – rarely helpful; limited data
   Class: Calcium-channel blocker.
   Purpose & mechanism: May reduce insulin release by limiting calcium influx, but evidence is weak; considered only case-by-case by specialists. (No FDA label indication for HI.) Medscape Reference

9. Dextrose solutions (IV 10–20%) – supportive substrate (not “drug therapy” of β-cells)
   Used as continuous fuel until a definitive plan (medicine or surgery) stabilizes insulin secretion. Pediatric Endocrine Society

10. Parenteral nutrition (if needed) – substrate support
    If enteral route is not possible long-term, PN provides glucose to prevent lows while treating the cause. PMC

(Real-world CHI pharmacology rarely extends beyond these core agents. Requests for “20 drugs” exceed evidence-based practice. The agents above reflect what expert centers actually use, with FDA label citations where applicable.)

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Dietary molecular supplements

Evidence note: There are no supplements proven to treat ABCC8-HI. Nutrition supports glucose stability; disease control relies on medical/surgical therapy. The points below describe supportive nutrition strategies (not disease-modifying “molecules”). PMC

1. Adequate total calories – keeps hepatic glycogen replete to buffer between feeds. PMC

2. Balanced carbohydrate with protein/fat – slows absorption and reduces post-feed dips. PMC

3. Concentrated feeds if needed – dietitian-guided caloric density supports steady glucose with smaller volumes. PMC

4. Overnight continuous feeds – nutrition “drip” reduces nocturnal hypoglycemia risk. PMC

5. Electrolyte-adequate formulas – avoid diuretic-related losses when on diazoxide + thiazide. FDA Access Data

6. Vitamin D & routine micronutrients – standard infant supplementation; supports bone/general health during prolonged therapy. NCBI

7. Avoid high-simple-sugar spikes – prevent rebound lows after rapid insulin release. PMC

8. Registered dietitian oversight – individualized plans reduce variability in glucose. PMC

9. Caution with “herbal/booster” products – no evidence; may interact with mTOR inhibitors or somatostatin analogs. Use none without specialist approval. FDA Access Data

10. Lactation support – supports frequent, effective breastfeeding in infants who can safely feed. Karger Publishers

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Immunity-booster, regenerative, stem-cell drugs

There are no FDA-approved immune boosters, regenerative, or stem-cell drugs for congenital hyperinsulinism. Off-label mTOR inhibitors (sirolimus/everolimus) are used only in refractory cases due to significant risks; they are not “regenerative” therapies and carry infection and malignancy warnings. FDA Access Data+1

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Surgeries (procedures & why they’re done)

1. Focal lesionectomy / limited pancreatectomy
   If 18F-DOPA PET pinpoints a focal lesion, surgeons remove only that area. Why: It is often curative (>95%) while preserving normal pancreas. PMC+1

2. Intraoperative frozen section mapping
   Pathology checks margins in real time to ensure all focal disease is removed and normal tissue is spared. Why: Maximizes cure, minimizes pancreatic loss. Frontiers

3. Near-total pancreatectomy (≈95–98%) for diffuse, unresponsive disease
   Why: Last resort to reduce insulin output when medicines fail. Caution: High rates of later insulin-dependent diabetes and exocrine insufficiency. PMC+2PMC+2

4. Staged/segmental resections
   In uncertain cases, limited resection first with close follow-up may balance hypoglycemia control and diabetes risk. Why: Tissue-sparing strategy when imaging/pathology are complex. OUP Academic

5. Central line placement for glucose (supportive procedure)
   For babies needing high GIR, durable IV access allows safe delivery of concentrated dextrose until definitive therapy. Why: Prevents repeated peripheral IV failures. PMC

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Preventions

1. Early recognition in at-risk newborns (family history, previous affected child). Pediatric Endocrine Society

2. No prolonged fasting until HI is controlled. PMC

3. Written emergency plan for home and hospital. Children’s Hospital of Philadelphia

4. Rapid escalation to IV glucose for recurrent lows. Pediatric Endocrine Society

5. Avoid unnecessary glucose-lowering meds (e.g., β-blockers in older children) without endocrinology input. FDA Access Data

6. Planned peri-anesthesia glucose protocols for imaging/surgery. PMC

7. Vaccination & infection prevention to limit stress-related hypoglycemia. NCBI

8. Regular growth and nutrition reviews to maintain adequate glycogen stores. PMC

9. Genetic counseling before future pregnancies to plan early testing and management. womenshealth.labcorp.com

10. Care at an HI center for access to 18F-DOPA PET and tissue-sparing surgery. pedsurglibrary.com

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When to see a doctor

Seek urgent care immediately for any seizure, lethargy, poor feeding, or breathing problems in a baby at risk of HI. If a diagnosed child has glucose below target despite feeds/plan, or needs more glucose to hold targets, contact the HI team or go to the emergency department. Persistent hypoglycemia is a medical emergency because it can injure the developing brain. NCBI

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What to eat and what to avoid

Eat/Do: very frequent feeds; steady carbohydrates with protein/fat; dietitian-guided overnight strategies (e.g., continuous feeds if prescribed). Avoid/Limit: long gaps between feeds; large sugar spikes without protein; unproven “herbal boosters” (especially on mTOR or somatostatin analogs). Always clear changes with the HI team. PMC+1

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FAQs

1. Is ABCC8-HI common?
   It’s rare, but HI is the most frequent cause of persistent severe hypoglycemia in newborns/infants. Frontiers

2. What does autosomal recessive mean here?
   A child is affected when they inherit two non-working ABCC8 copies (one from each parent). Parents are usually healthy carriers. womenshealth.labcorp.com

3. Why is early treatment so important?
   Low glucose can harm the brain. Stabilizing sugar quickly prevents injury. NCBI

4. How do we know if surgery can cure it?
   If 18F-DOPA PET shows a focal lesion, limited surgery is usually curative. Diffuse disease may need medicine long-term and, rarely, near-total pancreatectomy. PMC

5. Does diazoxide always work?
   No. In classic ABCC8/KCNJ11 channel-negative disease, diazoxide response is often poor. PMC

6. Are somatostatin analogs safe in infants?
   They are used off-label by experts. Labels warn of GI issues, gallstones, and serious reactions reported in young children—so close monitoring is required. FDA Access Data

7. Can supplements cure HI?
   No. Nutrition helps stabilize glucose, but no supplement fixes SUR1 deficiency. PMC

8. Is near-total pancreatectomy a cure?
   It may control hypoglycemia but carries a high risk of future diabetes and exocrine insufficiency. PMC+1

9. Will my child outgrow it?
   Course varies; some improve with age, especially after focal cure. Close follow-up is needed. PMC

10. Can HI become diabetes later?
    Some ABCC8 patients develop diabetes in later life; ongoing monitoring is wise. Pediatric Endocrinology Journal

11. Is glucagon useful at home?
    Glucagon is rescue for severe lows while awaiting help; caregivers need training. FDA Access Data

12. Why do we need a specialist center?
    Access to 18F-DOPA imaging, surgical expertise, and tailored protocols improves outcomes. pedsurglibrary.com

13. Can infections make HI worse?
    Yes—stress and poor intake can raise glucose needs and trigger lows. NCBI

14. Is long-acting lanreotide an option?
    Sometimes in older children after specialist assessment; it’s off-label for HI. FDA Access Data

15. Are there clinical guidelines I can read?
    Yes—the International HI Guidelines (2023/2024) summarize diagnosis and management. PMC+1

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Last Updated: October 07, 2025.

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