Obesity due to congenital leptin deficiency

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ORPHA:66628OMIM:614962E66.8
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Overview

Obesity due to congenital leptin deficiency is a very rare genetic condition where the body cannot make enough of a hormone called leptin. Leptin is sometimes called the 'satiety hormone' because it tells your brain when you have eaten enough and helps control how much energy your body stores as fat. Without leptin, the brain never receives the signal to stop eating, which leads to severe, uncontrollable hunger starting in early infancy and rapid, extreme weight gain. This condition is also sometimes called congenital leptin deficiency or CLD. Children with this condition feel constantly hungry no matter how much they eat. This is not a matter of willpower — the brain is simply not receiving the chemical message it needs. Beyond obesity, leptin deficiency also affects the immune system, making infections harder to fight off, and it delays puberty and normal sexual development. Some children may also have problems with how their body handles blood sugar. The good news is that this condition has a specific, highly effective treatment. A medication called metreleptin (a man-made form of leptin) can be given by injection and dramatically reduces hunger, leads to significant weight loss, and improves many of the other health problems caused by leptin deficiency. With early diagnosis and treatment, people with this condition can live much healthier lives.

Key symptoms:

Severe, constant hunger that cannot be satisfiedRapid and extreme weight gain starting in the first few months of lifeSevere obesity in early childhoodDelayed or absent pubertyUnderdeveloped reproductive organsFrequent infections due to a weakened immune systemHigh levels of insulin in the blood (hyperinsulinemia)Abnormal blood fat levelsLow levels of leptin detectable in blood testsBehavioral problems related to food-seekingReduced thyroid hormone levels in some cases

Clinical phenotype terms (20)— hover any for plain English
Decreased CD4+ T cell proportionHP:0032218Decreased serum leptinHP:0003292Decreased T cell activationHP:0005419Absence of secondary sex characteristicsHP:0008187Decreased serum estradiolHP:0008214Hypoplasia of the ovaryHP:0008724Decreased serum testosterone concentrationHP:0040171Orthostatic hypotension due to autonomic dysfunctionHP:0004926
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Obesity due to congenital leptin deficiency.

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No actively recruiting trials found for Obesity due to congenital leptin deficiency at this time.

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No specialists are currently listed for Obesity due to congenital leptin deficiency.

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Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Obesity due to congenital leptin deficiency.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.Is metreleptin available and covered by insurance or our healthcare system, and how do we access it?,How soon after starting metreleptin can we expect to see changes in hunger and weight?,What monitoring tests will my child need regularly, and how often?,Should other family members, including siblings and parents, be tested for the LEP gene change?,What should we do if my child gets a serious infection?,Will my child be able to go through puberty normally with treatment, and what should we watch for?,Are there any clinical trials or research studies we should know about?

Common questions about Obesity due to congenital leptin deficiency

What is Obesity due to congenital leptin deficiency?

Obesity due to congenital leptin deficiency is a very rare genetic condition where the body cannot make enough of a hormone called leptin. Leptin is sometimes called the 'satiety hormone' because it tells your brain when you have eaten enough and helps control how much energy your body stores as fat. Without leptin, the brain never receives the signal to stop eating, which leads to severe, uncontrollable hunger starting in early infancy and rapid, extreme weight gain. This condition is also sometimes called congenital leptin deficiency or CLD. Children with this condition feel constantly hung

How is Obesity due to congenital leptin deficiency inherited?

Obesity due to congenital leptin deficiency follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Obesity due to congenital leptin deficiency typically begin?

Typical onset of Obesity due to congenital leptin deficiency is infantile. Age of onset can vary across affected individuals.