Hypopituitarism-micropenis-cleft lip/palate syndrome

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Overview

Hypopituitarism-micropenis-cleft lip/palate syndrome is a very rare condition that affects the pituitary gland, the development of the genitals, and the formation of the face before birth. The pituitary gland is a small but important gland at the base of the brain that controls many hormones in the body. When it does not work properly (a condition called hypopituitarism), the body may not produce enough growth hormone, thyroid hormone, cortisol, or other important hormones. This can affect growth, energy, metabolism, and sexual development. In males, one of the most noticeable signs is a smaller-than-typical penis at birth, called micropenis. Many affected individuals are also born with a cleft lip, a cleft palate, or both — meaning there is a gap or split in the upper lip or the roof of the mouth that forms during pregnancy. This syndrome is sometimes referred to by its combination of features rather than a single widely used name. Because the pituitary gland controls so many body systems, children with this condition may face a wide range of health challenges, including slow growth, low blood sugar in newborns, problems with puberty, and difficulties with feeding due to the cleft palate. Treatment focuses on replacing the hormones the body is not making enough of and surgically repairing the cleft lip and palate. With early diagnosis and careful management, many of these challenges can be addressed effectively.

Key symptoms:

Smaller-than-typical penis at birth (micropenis) in malesCleft lip — a gap or split in the upper lipCleft palate — a gap in the roof of the mouthLow levels of pituitary hormones (hypopituitarism)Slow or poor growth in childhoodLow blood sugar (hypoglycemia) in newbornsDelayed or absent pubertyUndescended testicles in malesFatigue and low energy due to hormone deficienciesFeeding difficulties in infancy due to cleft palateLow thyroid hormone levels (hypothyroidism)Low cortisol levels, which can cause serious illness during stress or infection

Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hypopituitarism-micropenis-cleft lip/palate syndrome.

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No actively recruiting trials found for Hypopituitarism-micropenis-cleft lip/palate syndrome at this time.

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No specialists are currently listed for Hypopituitarism-micropenis-cleft lip/palate syndrome.

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

Travel Grants

No travel grants are currently matched to Hypopituitarism-micropenis-cleft lip/palate syndrome.

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Community

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Latest news about Hypopituitarism-micropenis-cleft lip/palate syndrome

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.Which specific hormones are deficient, and what medications will my child need to take?,What is the sick-day plan if my child gets a fever or needs surgery — how do we adjust the cortisol dose?,Should we do genetic testing, and what genes should be looked at?,What is the plan for cleft lip and palate surgery, and what specialists will be involved?,How will we monitor my child's growth and development over time?,Will my child go through puberty normally, or will they need hormone support for that?,Are there any support groups or patient organizations that can connect us with other families dealing with this condition?

Common questions about Hypopituitarism-micropenis-cleft lip/palate syndrome

What is Hypopituitarism-micropenis-cleft lip/palate syndrome?

Hypopituitarism-micropenis-cleft lip/palate syndrome is a very rare condition that affects the pituitary gland, the development of the genitals, and the formation of the face before birth. The pituitary gland is a small but important gland at the base of the brain that controls many hormones in the body. When it does not work properly (a condition called hypopituitarism), the body may not produce enough growth hormone, thyroid hormone, cortisol, or other important hormones. This can affect growth, energy, metabolism, and sexual development. In males, one of the most noticeable signs is a small

At what age does Hypopituitarism-micropenis-cleft lip/palate syndrome typically begin?

Typical onset of Hypopituitarism-micropenis-cleft lip/palate syndrome is neonatal. Age of onset can vary across affected individuals.