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Disease Profile

Sheehan syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

Adult

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ICD-10

E23.0

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Postpartum hypopituitarism; Postpartum panhypopituitarism; Postpartum panhypopituitary syndrome;

Categories

Endocrine Diseases

Summary

Sheehan syndrome affects the function of the pituitary gland. The pituitary gland makes hormones and regulates other glands and many body processes, including reproduction. The cause of Sheehan syndrome is severe blood loss during or after childbirth (postpartum hemorrhage). This leads to lack of blood flow to the front part of the pituitary gland, causing it to gradually stop functioning. The symptoms of Sheehan syndrome occur as a result of the low hormone levels. These may include an inability to produce breast milk, irregular or absent periods, hot flashes, and a decreased sex drive. Other symptoms may include fatigue, headaches, low blood pressure, and hair loss. Symptoms usually do not appear from months to years after the hemorrhage. In some cases, the symptoms occur immediately and may be more severe and sometimes life threatening. Sheehan syndrome is diagnosed based on the symptoms, clinical history and exam, laboratory testing, and imaging studies. Treatment includes hormone replacement therapy, and steroids to help manage early symptoms.[1][2][3][4]

Symptoms

The following list includes the most common signs and symptoms in people with Sheehan syndrome. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition. 

Symptoms of Sheehan syndrome may include:[1][2][5]

  • Inability to produce breast milk
  • Fatigue
  • Dizziness
  • Irregular or absent menstrual periods (amenorrhea)
  • Hot flashes
  • Decreased libido
  • Loss of pubic and underarm hair
  • Low blood pressure (hypotension)
  • Slowed mental functioning
  • Weight gain or loss

In most cases, the symptoms of Sheehan syndrome do not appear until months or even years after severe blood loss after childbirth (postpartum hemorrhage). In rare cases, the symptoms of Sheehan syndrome occur within days and include an inability to produce breast milk, a drop in blood pressure, visual problems, and unconsciousness. These symptoms may be life threatening. In other cases, severe symptoms become apparent when the body is stressed by infection or surgery some period of time after delivery.[1][2]

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Medical Terms Other Names
Learn More:
HPO ID
80%-99% of people have these symptoms
Adrenocorticotropic hormone deficiency
0011748
Adrenocorticotropin deficient adrenal insufficiency
0011735
Chronic fatigue
Chronic extreme exhaustion
0012432
Decreased circulating cortisol level
Low blood cortisol level
0008163
Oligomenorrhea
Light or infrequent menstrual periods
0000876
Panhypopituitarism
0000871
Secondary growth hormone deficiency
0008240
30%-79% of people have these symptoms
Abnormal size of pituitary gland
0012504
Amenorrhea
Abnormal absence of menstruation
0000141
Blurred vision
0000622
Breast hypoplasia
Underdeveloped breasts
0003187
Decreased female libido
Decreased female sex drive
0030018
Decreased serum estradiol
0008214
Dry skin
0000958
Dyspareunia
0030016
Gonadotropin deficiency
0008213
Hypoglycemia
Low blood sugar
0001943
Hyposthenuria
0003158
Impotence
Difficulty getting a full erection
Difficulty getting an erection

[ more ]

0000802
Nausea
0002018
Normochromic anemia
0001895
Orthostatic hypotension
Decrease in blood pressure upon standing up
0001278
Pallor
0000980
Pituitary hypothyroidism
Low thyroid gland function due to abnormal pituitary gland
0008245
Progressive visual field defects
0007987
Reduced circulating prolactin concentration
0008202
Sparse axillary hair
Limited armpit hair
Little underarm hair

[ more ]

0002215
Sparse pubic hair
Decreased sexual hair
0002225
5%-29% of people have these symptoms
Antinuclear antibody positivity
0003493
Arthralgia
Joint pain
0002829
Bradycardia
Slow heartbeats
0001662
Chills
0025143
Chronic lymphocytic meningitis
0007041
Constipation
0002019
Diplopia
Double vision
0000651
Hashimoto thyroiditis
0000872
Hyponatremia
Low blood sodium levels
0002902
Muscle weakness
Muscular weakness
0001324
Obesity
Having too much body fat
0001513
Palpitations
Missed heart beat
Skipped heart beat

[ more ]

0001962
Poor appetite
Decreased appetite
0004396
Sensorineural hearing impairment
0000407
Thunderclap headache
0030907
Vertigo
Dizzy spell
0002321
1%-4% of people have these symptoms
Central diabetes insipidus
0000863
Coma
0001259
Psychosis
0000709

Cause

Sheehan syndrome is caused by severe blood loss or extremely low blood pressure during or after childbirth. This leads to lack of blood flow to the front part of the pituitary gland, which enlarges during pregnancy. This damages the hormone-producing tissue so that the gland cannot function properly.[1][4]

Diagnosis

Diagnosis of Sheehan syndrome is based on the symptoms, a clinical exam, blood tests, and imaging studies. Criteria for diagnosis include a history of significant blood loss during or after childbirth, inability to produce breast milk, irregular or absent menstrual periods, and low or absent levels of pituitary hormones.[1][3]

Because the symptoms of Sheehan syndrome may occur long after childbirth and are not specific to any disease, Sheehan syndrome is often difficult to diagnose.[1][3]

Treatment

Treatment of Sheehan syndrome involves replacement of the hormones produced by the pituitary gland. Hormone levels should be monitored on a regular basis and may need to be adjusted from time to time.[1][5]

Specialists involved in the care of someone with Sheehan syndrome may include:[1]

  • Obstetrician/gynecologist
  • Endocrinologist
  • Neurologist

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • Mayo Clinic has an information page on Sheehan syndrome.
    • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
    • The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

      In-Depth Information

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Sheehan syndrome. Click on the link to view a sample search on this topic.

        Selected Full-Text Journal Articles

          References

          1. Schury MP, Adigun R. Sheehan Syndrome. StatPearls. Updated Jul 17, 2020; https://pubmed.ncbi.nlm.nih.gov/29083621/.
          2. Diri H, Karaca Z, Tanriverdi F, Unluhizarci K, Kelestimur F. Sheehan's syndrome: new insights into an old disease. Endocrine. Jan 2016; 51(1):22-31. https://pubmed.ncbi.nlm.nih.gov/26323346/.
          3. Sheehan syndrome. Nat Rev Dis Primers. Dec 22, 2016; 2:16093. https://pubmed.ncbi.nlm.nih.gov/28004658/.
          4. Chanson P. Other Pituitary Conditions and Pregnancy. Endocrinol Metab Clin North Am. Sep 2019; 48(3):583-603. https://pubmed.ncbi.nlm.nih.gov/31345525/.
          5. Karaca Z, Laway BA, Dokmetas HS, Atmaca H, Kelestimur F. Sheehan syndrome. Nat Rev Dis Primers. Dec 22, 2016; 2:https://www.ncbi.nlm.nih.gov/pubmed/28004764.

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