Short stature due to growth hormone qualitative anomaly

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ORPHA:629OMIM:262650E23.0
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Overview

Short stature due to growth hormone qualitative anomaly is a rare endocrine condition where the body produces growth hormone (GH), but the hormone does not work properly. Unlike typical growth hormone deficiency where the body makes too little GH, in this condition the growth hormone molecule itself is abnormal or has a changed structure. This means that even though blood tests may show normal or even high levels of growth hormone, the hormone cannot do its job of stimulating growth effectively. The condition is sometimes called bioinactive growth hormone or Kowarski syndrome. Children with this condition typically show significant short stature that becomes noticeable during early childhood. Their growth rate is slower than expected, and they may fall further behind their peers over time. Other features can include delayed bone maturation and a younger-looking appearance compared to their actual age. Because standard growth hormone blood tests may come back normal, this condition can be tricky to diagnose and may be missed or confused with other causes of short stature. Treatment usually involves replacement therapy with standard recombinant growth hormone, which can bypass the problem of the faulty natural hormone. When started early, growth hormone therapy can significantly improve final adult height. Regular monitoring by a pediatric endocrinologist is essential to track growth response and adjust treatment as needed.

Also known as:

Key symptoms:

Short stature or being much shorter than expected for ageSlow growth rate during childhoodDelayed bone age (bones appear younger on X-ray than actual age)Younger-looking facial features compared to peersNormal or high growth hormone levels on blood tests despite poor growthLow levels of IGF-1 (a growth factor normally stimulated by growth hormone)Delayed puberty in some casesSmall body proportions that are otherwise normalPossible mild increase in body fatReduced muscle mass compared to peers

Inheritance

Autosomal recessive

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

1 available

Increlex

Mecasermin· Eton Pharmaceuticals, Inc.
INCRELEX (mecasermin) injection is indicated for the treatment of growth failure in pediatric patients 2 years of age and older with growth hormone (GH) gene deletion who have developed neutralizing a

INCRELEX (mecasermin) injection is indicated for the treatment of growth failure in pediatric patients 2 years of age and older with growth hormone (GH) gene deletion who have developed neutralizing antibodies to GH

No actively recruiting trials found for Short stature due to growth hormone qualitative anomaly at this time.

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No specialists are currently listed for Short stature due to growth hormone qualitative anomaly.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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

Financial Resources

1 resources
Increlex(Mecasermin)Eton Pharmaceuticals, Inc.

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.How was this diagnosis confirmed, and could other causes of short stature be ruled out?,What is the expected improvement in growth with growth hormone therapy?,How long will my child need to take growth hormone injections?,What side effects should I watch for with growth hormone treatment?,How often will my child need follow-up visits and blood tests?,Should other family members be tested for this genetic condition?,Are there any newer or longer-acting growth hormone treatments available?

Common questions about Short stature due to growth hormone qualitative anomaly

What is Short stature due to growth hormone qualitative anomaly?

Short stature due to growth hormone qualitative anomaly is a rare endocrine condition where the body produces growth hormone (GH), but the hormone does not work properly. Unlike typical growth hormone deficiency where the body makes too little GH, in this condition the growth hormone molecule itself is abnormal or has a changed structure. This means that even though blood tests may show normal or even high levels of growth hormone, the hormone cannot do its job of stimulating growth effectively. The condition is sometimes called bioinactive growth hormone or Kowarski syndrome. Children with t

How is Short stature due to growth hormone qualitative anomaly inherited?

Short stature due to growth hormone qualitative anomaly follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Short stature due to growth hormone qualitative anomaly typically begin?

Typical onset of Short stature due to growth hormone qualitative anomaly is childhood. Age of onset can vary across affected individuals.