Overview
Hypoinsulinemic hypoglycemia and body hemihypertrophy is an extremely rare condition that combines two main features: episodes of low blood sugar (hypoglycemia) that occur despite low levels of insulin, and asymmetric overgrowth of one side of the body (hemihypertrophy or hemihyperplasia). In most forms of hypoglycemia, insulin levels are either too high or the body cannot respond to sugar properly. In this condition, however, blood sugar drops even though insulin levels are low, which makes it unusual and harder to diagnose. The overgrowth typically affects one side of the body, meaning one arm, leg, or half of the trunk may be noticeably larger than the other. This asymmetry can be present at birth or become more apparent as the child grows. Symptoms of low blood sugar can include shakiness, sweating, irritability, confusion, seizures, and in severe cases, loss of consciousness. The body asymmetry may cause difficulties with movement, posture, and self-image. Because hemihypertrophy is also associated with an increased risk of certain childhood tumors (such as Wilms tumor), regular screening with abdominal ultrasound is often recommended. Treatment focuses on managing blood sugar levels through frequent feeding, specialized diets, and sometimes medications, as well as monitoring and addressing the overgrowth and its complications. The exact cause of this syndrome is not fully understood, and management requires a team of specialists working together.
Key symptoms:
Low blood sugar episodesOne side of the body larger than the otherShakiness or tremblingExcessive sweatingIrritability or fussiness in infantsSeizures from low blood sugarPoor feeding in newbornsLethargy or excessive sleepinessConfusion or difficulty concentratingUnequal limb lengthAsymmetric facial featuresDifficulty with coordination or balanceFailure to thrive in infancy
Clinical phenotype terms (14)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Neonatal
Begins at or shortly after birth (first 4 weeks)
Treatments
No FDA-approved treatments are currently listed for Hypoinsulinemic hypoglycemia and body hemihypertrophy.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
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Questions for your doctor
Bring these to your next appointment
- Q1.What is causing my child's blood sugar to drop if insulin levels are low?,How often should we check blood sugar levels, and what numbers should concern us?,What should we do if my child has a severe low blood sugar episode at home?,How often does my child need tumor screening ultrasounds, and for how long?,Will the hypoglycemia improve as my child gets older?,Should we pursue genetic testing, and what might the results tell us?,What accommodations should we request at school for my child's safety?
Common questions about Hypoinsulinemic hypoglycemia and body hemihypertrophy
What is Hypoinsulinemic hypoglycemia and body hemihypertrophy?
Hypoinsulinemic hypoglycemia and body hemihypertrophy is an extremely rare condition that combines two main features: episodes of low blood sugar (hypoglycemia) that occur despite low levels of insulin, and asymmetric overgrowth of one side of the body (hemihypertrophy or hemihyperplasia). In most forms of hypoglycemia, insulin levels are either too high or the body cannot respond to sugar properly. In this condition, however, blood sugar drops even though insulin levels are low, which makes it unusual and harder to diagnose. The overgrowth typically affects one side of the body, meaning one a
How is Hypoinsulinemic hypoglycemia and body hemihypertrophy inherited?
Hypoinsulinemic hypoglycemia and body hemihypertrophy follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Hypoinsulinemic hypoglycemia and body hemihypertrophy typically begin?
Typical onset of Hypoinsulinemic hypoglycemia and body hemihypertrophy is neonatal. Age of onset can vary across affected individuals.