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Postpartum Hypopituitarism

Disease Details

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Description: Postpartum hypopituitarism, also known as Sheehan's syndrome, is a rare condition characterized by the underperformance of the pituitary gland due to severe blood loss or low blood pressure during or after childbirth.
Type: Postpartum hypopituitarism, also known as Sheehan's syndrome, is typically not a genetic disorder. It most commonly occurs due to severe blood loss or extremely low blood pressure during or after childbirth, leading to the damage of the pituitary gland. Therefore, it does not have a type of genetic transmission.
Signs And Symptoms: The various signs and symptoms in Sheehan's syndrome are caused by damage to the pituitary, thereby causing a decrease in one or more of the hormones it normally secretes. Since the pituitary controls many glands in the endocrine system, partial or complete loss of a variety of functions may result. Many of the signs and symptoms of Sheehan's are considered "nonspecific" in the medical community; in other words these signs and symptoms are seen in a number of different disease processes, and are not specific to a singular disease or syndrome.In some cases, a woman with Sheehan syndrome may be relatively asymptomatic initially; therefore, the diagnosis would not be made until years later when features of hypopituitarism become evident. In rare instances this syndrome can present acutely with unstable vital signs, dangerously low blood glucose levels, heart failure, or even psychosis. Hypopituitarism can lead to an interruption in any of the following hormone pathways: thyroid disorder (secondary hypothyroidism), adrenal gland (adrenal insufficiency due to glucocorticoid deficiency), sex hormone (gonadotropin deficiency), prolactin (a hormone responsible for lactation), growth hormone, or rarely anti-diuretic hormone deficiency (central diabetes insipidus). Since damage to the pituitary can cause a deficiency in more than one of these hormone pathways simultaneously, it is possible to have a mix of any of the signs or symptoms listed below.Sheehan's syndrome's most common initial symptoms are difficulties with or total absence of lactation (agalactorrhea). Another common sign is infrequent menstrual cycles (oligomenorrhea) or absent menstrual cycles (amenorrhea) following delivery. In addition to menstrual irregularities other signs of sex hormone deficiency are hot flashes, decreased libido, and breast involution. Symptoms and signs of thyroid disorder are tiredness, intolerance to cold, constipation, weight gain, hair loss, slowed thinking, as well as a slowed heart rate and low blood pressure. Adrenal gland malfunction can present acutely or chronically. In a more chronic case, it is similar to Addison's disease with symptoms including fatigue, weight loss, hypoglycemia (low blood sugar levels), low hemoglobin levels (anemia) and hyponatremia (low sodium levels) that develop over several months or years. Acute adrenal insufficiency is referred to as an adrenal crisis, which can be life-threatening, and occurs very shortly after the inciting event i.e. significant blood loss post-partum in the context of Sheehan's syndrome. Adrenal crisis signs and symptoms include hypoglycemia, hypotension, weakness, fatigue, and seizures from severe hyponatremia.Growth hormone deficiency is one of the most common hormone deficiencies of hypopituitarism seen in Sheehan's syndrome. Low levels of growth hormone may present with low energy, body aches, or subtle wrinkling of the skin around the eyes or mouth. The symptoms of anti-diuretic hormone deficiency are increased thirst, excessive urination, headache, and fatigue. Hematological changes might be seen as well such as anemia or low platelets (thrombocytopenia). Hyponatremia is seen in many cases of Sheehan's syndrome because it can result from multiple etiologies. Drops in thyroid hormones and glucocorticoid/adrenal hormones can indirectly lead to hyponatremia through water retention, while blood loss can trigger hyponatremia through ADH secretion. The development of Syndrome of Inappropriate Anti-Diuretic Hormone in patients with Sheehan's syndrome has been documented in the literature, although the mechanism is not well understood.
Prognosis: Prognosis for postpartum hypopituitarism, also known as Sheehan's syndrome, can vary depending on the severity of pituitary damage and the effectiveness of treatment. With appropriate hormone replacement therapy, many women can manage symptoms and lead relatively normal lives. However, lifelong medical supervision is often required to adjust hormone dosages and monitor for potential complications. Early diagnosis and intervention are crucial for improving long-term outcomes.
Onset: Postpartum hypopituitarism, also known as Sheehan's syndrome, typically has an onset that occurs after childbirth, particularly following severe postpartum hemorrhage. This condition results from ischemic necrosis of the pituitary gland due to significant blood loss during or after delivery, leading to varying degrees of pituitary hormone deficiencies.
Prevalence: The prevalence of postpartum hypopituitarism is not well-documented, making it difficult to provide precise statistics. However, this condition is relatively rare. Commonly known as Sheehan's syndrome, it typically occurs due to significant blood loss or severe low blood pressure during or after childbirth, which results in damage to the pituitary gland.
Epidemiology: The exact prevalence of this syndrome is difficult to define because the incidence varies so much from country to country. Sheehan syndrome is more prevalent in developing countries than developed countries. In a study from the United Kingdom in 2001 only 1.4% of patients with hypopituitarism were diagnosed with Sheehan's syndrome. Just a few years earlier in 1996 the World Health Organization estimated that 3 million women were effected by Sheehan's syndrome. In a study of 1,034 symptomatic adults, Sheehan's syndrome was found to be the sixth-most frequent etiology of growth hormone deficiency, being responsible for 3.1% of cases (versus 53.9% due to a pituitary tumor). Additionally, it was found that the majority of women who experienced Sheehan syndrome gave birth at home rather than in a hospital.
Intractability: Postpartum hypopituitarism, also known as Sheehan's syndrome, is typically not considered intractable. Management involves lifelong hormone replacement therapy to compensate for the deficiencies in pituitary hormones. With appropriate treatment, patients can manage symptoms effectively and lead a normal life, although the condition itself cannot be cured. Regular follow-ups with an endocrinologist are essential to adjust hormone therapy and monitor for any complications.
Disease Severity: The severity of postpartum hypopituitarism, also known as Sheehan's syndrome, can vary from mild to severe, depending on the extent of the pituitary gland damage. In severe cases, it can be life-threatening if not properly managed, as it can lead to adrenal crisis, severe hypotension, and other critical complications. Early diagnosis and hormone replacement therapy are crucial for reducing morbidity and managing symptoms effectively.
Healthcare Professionals: Disease Ontology ID - DOID:9476
Pathophysiology: This syndrome seems to arise when certain factors compound each other to cause pituitary injury. The physiologic enlargement of the pituitary gland in conjunction with an interference in its blood supply ultimately result in pituitary ischemia and necrosis. One cause of pituitary growth associated with the risk of Sheehan's syndrome is the hyperplasia of lactotrophs which produce prolactin, the hormone responsible for milk production. Other hormone-secreting cells of the pituitary undergo rapid growth in pregnant women as well, which contribute to the gland's enlargement.The anterior pituitary is supplied by a low pressure portal venous system. The anterior pituitary is more commonly affected in Sheehan's syndrome because of the structure of the portal venous system. Posterior pituitary involvement leading to central diabetes insipidus is much rarer, and typically reflects more extensive damage to the organ and more severe disease. It has been suggested that the arrangement of the pituitary's blood supply contribute to its susceptibility for injury. "The highly vascularized pituitary tissue involves one of the most rapid blood flow in the human body and probably, therefore, has a tendency to infarction because even small degrees of change in the pituitary intravascular pressure cause an arrest of blood flow". Ischemia may occur as a result of vasospasm from shock, hypotension, thrombosis, or direct vascular compression of the hypophyseal artery from the enlarged pituitary gland itself. The presence of disseminated intravascular coagulation (i.e., in amniotic fluid embolism or HELLP syndrome) also appears to be a factor in its development.
Carrier Status: Postpartum hypopituitarism, also known as Sheehan's syndrome, is not a genetic or inheritable condition and therefore does not have a carrier status. It occurs due to severe blood loss or extremely low blood pressure during or after childbirth, which can lead to the pituitary gland being deprived of blood and oxygen, causing damage and impaired function.
Mechanism: Postpartum hypopituitarism, also known as Sheehan's syndrome, occurs due to ischemic necrosis of the pituitary gland resulting from severe postpartum hemorrhage and hypovolemic shock.

**Mechanism:**
- During childbirth, excessive bleeding can significantly reduce blood flow to the pituitary gland.
- The anterior pituitary gland, which is highly vascular, is especially susceptible to ischemia.
- Decreased blood flow leads to necrosis (tissue death) of the gland, impairing its ability to produce essential hormones.

**Molecular Mechanisms:**
- Hypoperfusion (reduced blood flow) leads to oxygen deprivation (hypoxia) of pituitary cells.
- This triggers apoptosis (programmed cell death) or necrosis due to the lack of necessary nutrients and oxygen.
- Inflammatory responses may follow, exacerbating tissue damage.
- Subsequent fibrosis in the gland further disrupts its function, leading to long-term hormone deficiencies.

Reduced hormone production can affect multiple endocrine pathways, as the pituitary gland influences the thyroid, adrenal glands, and gonads, among others. Understanding the molecular underpinnings helps in developing targeted interventions to mitigate or manage the syndrome.
Treatment: The mainstay of treatment is hormone replacement therapy for the hormones that are missing. Treatment plans and dosages should be individualized by an endocrinologist. Glucocorticoids may be administered to address or prevent an adrenal crisis, a potential serious complication of Sheehan's syndrome. Hormone replacement is vital in reducing the morbidity and mortality of this syndrome.
Compassionate Use Treatment: Postpartum hypopituitarism, also known as Sheehan's syndrome, occurs due to necrosis of the pituitary gland following severe postpartum hemorrhage. For compassionate use or experimental treatments, there is no widely recognized compassionate use program specifically for this condition. However, hormone replacement therapy (HRT) is the mainstay of treatment and may include:

1. **Corticosteroids**: For adrenal insufficiency, hydrocortisone or prednisone may be used.
2. **Thyroid Hormone Replacement**: Levothyroxine for hypothyroidism.
3. **Sex Hormones**: Estrogen and progesterone therapy for women with ovarian insufficiency.

Experimental treatments are not well-documented, but research into regenerative medicine and stem cell therapy is ongoing and might offer future potential. Always consult with a healthcare provider for the most current and individualized treatment options.
Lifestyle Recommendations: For postpartum hypopituitarism (Sheehan's syndrome), here are some lifestyle recommendations that may support better health management:

1. **Medication Adherence**: Ensure strict adherence to hormone replacement therapies as prescribed by your healthcare provider. This typically includes cortisol, thyroid hormone, and sometimes sex hormones.

2. **Regular Monitoring**: Frequent follow-up visits with your endocrinologist to monitor hormone levels and adjust treatments as necessary.

3. **Balanced Diet**: Maintain a healthy, balanced diet rich in essential vitamins and minerals to support overall health.

4. **Stress Management**: Utilize stress-reducing techniques such as yoga, meditation, or deep-breathing exercises to manage cortisol levels effectively.

5. **Physical Activity**: Engage in regular, moderate exercise to maintain overall well-being, improve mood, and boost energy levels.

6. **Hydration**: Stay well-hydrated. Hormonal imbalances can sometimes affect fluid balance in the body.

7. **Educate Yourself**: Understanding your condition helps you to recognize symptoms that might require medical attention more promptly.

8. **Support System**: Establish a strong support system of family and friends to help manage stress and assist with daily activities if needed.

9. **Medical Identification**: Consider wearing a medical alert bracelet that indicates you have hypopituitarism, in case of emergencies where you cannot communicate your condition.

10. **Sleep Hygiene**: Ensure adequate and consistent sleep patterns to support overall health and hormonal balance.

Consult with your healthcare provider to tailor these recommendations to your specific needs and medical history.
Medication: For postpartum hypopituitarism, also known as Sheehan's syndrome, treatment typically involves lifelong hormone replacement therapy to address the deficiencies caused by the pituitary gland's impaired function. These hormones may include:

1. **Corticosteroids**: To replace adrenal hormones.
2. **Levothyroxine**: To replace thyroid hormones.
3. **Estrogen and Progesterone**: For menstrual cycle regulation and other functions.
4. **Growth Hormone**: If there's a deficiency in growth hormone.
5. **Desmopressin**: To manage diabetes insipidus if present.

The exact medication regimen should be tailored to the individual's specific hormone deficiencies and monitored regularly by a healthcare provider.
Repurposable Drugs: For postpartum hypopituitarism, also known as Sheehan's syndrome, treatment primarily involves hormone replacement therapy to address the deficiencies caused by the pituitary damage. While specific repurposed drugs for this condition are not well-documented, managing the individual hormone deficiencies with commonly available medications is standard. These include:

1. **Levothyroxine** - to replace deficient thyroid hormone levels.
2. **Hydrocortisone or prednisone** - to replace adrenal hormones.
3. **Desmopressin** - for diabetes insipidus if there is a deficiency in vasopressin.
4. **Estrogen/progesterone** - replacement therapy if ovarian function is impaired and the patient is premenopausal.
5. **Human growth hormone (somatropin)** - in cases of growth hormone deficiency.

These treatments are based on the hormone deficiencies and symptoms presented by the patient. The management is typically lifelong and requires regular monitoring and dose adjustments.
Metabolites: Metabolites that can be affected in postpartum hypopituitarism (Sheehan's syndrome) may include:

- Cortisol
- Thyroid hormones (T3, T4)
- Prolactin
- Estrogen
- Growth hormone (GH)

Nan refers to "Not applicable" or is used for indicating no relevant data.
Nutraceuticals: Nutraceuticals are non-specific compounds found in foods that provide medical or health benefits, including the prevention and treatment of disease. However, for postpartum hypopituitarism, also known as Sheehan's syndrome, no specific nutraceuticals are clinically recommended as treatment. Management typically involves hormone replacement therapy tailored to the deficient hormones (such as cortisol, thyroid hormones, estrogen, and growth hormone). It's essential for patients to work closely with their healthcare provider to manage this condition effectively.
Peptides: For postpartum hypopituitarism, also known as Sheehan's syndrome, peptide hormone deficiencies are a significant aspect. These deficiencies include:

1. **Adrenocorticotropic hormone (ACTH)** - leading to adrenal insufficiency.
2. **Thyroid-stimulating hormone (TSH)** - causing hypothyroidism.
3. **Luteinizing hormone (LH) and Follicle-stimulating hormone (FSH)** - resulting in issues with reproductive functions.
4. **Growth hormone (GH)** - leading to decreased growth and metabolic issues.
5. **Prolactin (PRL)** - causing lactation failure.

For postpartum hypopituitarism, neuroimaging such as MRI is typically used to rule out other causes of pituitary damage, but specific peptides like IGF-1 (a marker for GH) are often tested to assess various hormone deficiencies.