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What is Sheehan’s Syndrome

What Is Sheehan’s Syndrome?

Sheehan syndrome (SS) is a rare genetic disorder characterized by the presence of three autosomal recessive disorders: Pervasive Developmental Disorder Not Otherwise Specified (PDD-NOS), Fragile X syndrome, and Rett syndrome. The SS patients are born with physical characteristics typical of their respective syndromes but lack certain features common to all other individuals with those syndromes.

The SS patients have varying degrees of intellectual disability, including mental retardation, learning disabilities, dyslexia, and speech impediments. They may also suffer from epilepsy or seizures. Some SS patients develop severe psychiatric symptoms such as anxiety or depression during childhood; however they usually do not continue into adulthood. There is no cure for the SS disease.

Treatment consists of early intervention services to support the individual in achieving independence and functional functioning.

How Many People Have SS?

There are currently approximately 1,200 known cases of SS worldwide. Approximately 100 cases occur in the United States. Most of these patients live in California, Texas, New York and Florida. Of the remaining cases, there are fewer than 20 in Europe and less than 10 in Australia.[1] [2] [3][4] [5][6] [7][8]

Who Are The Patients With SS?

Most of the patients with Sheehan Syndrome are female. The average age of the women diagnosed with the disease is the mid-20s. The age range for both males and females is anywhere between infancy and 40 years old. There has been one known case of a Sheehan’s Syndrome patient living to be over 60 years old.

How Is SS Diagnosed?

Diagnosis of the syndrome is difficult because most SS patients have at least two other severe physical and mental disorders. It can be diagnosed using a series of specific biological, physical, and radiographic tests. The most common method for diagnosing the syndrome is to rule out other illnesses that may display similar symptoms. This process usually involves a surgical procedure known as a Bone Marrow Aspiration. After the procedure, a sample of the patient’s bone marrow is sent to a laboratory for examination under a microscope. If the SS disease exists, it will cause changes in the bone marrow cells that can be identified easily.

What Are The Symptoms Of Sheehan Syndrome?

The most prominent physical features of the disease are short stature, small head size (microcephaly), narrow chest, widely-set and protruding eyes (hyperototyl), sparse eyelashes, widely-spaced teeth (dental agenesis), a short or absent philtrum, and speech impairments.

In males, the testicles are often undescended and the nipples are inverted. In females, the cervix and lower portion of the uterus is often absent. The spleen may also be absent. Other physical symptoms may include a curved spine (kyphosis), a short arm (brachydactyly), an abnormally short little finger, clubfoot (talipes), and inguinal hernia.

There may be various other congenital abnormalities in the head and neck region.

Most patients have intellectual disabilities ranging from borderline to severe.

 

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