Panhypopituitarism Follow-up

Updated: Apr 21, 2022
  • Author: Robert P Hoffman, MD; Chief Editor: Sasigarn A Bowden, MD  more...
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Follow-up

Further Outpatient Care

Patients with hypopituitarism need close, ongoing, and regular follow-up by a pediatric endocrinologist. Closely monitor growth and measure free T4 levels on a regular basis to assess the adequacy of T4 replacement.

Body size and symptoms and signs of cortisol deficiency (eg, anorexia, recurrent abdominal pain, malaise) or cortisol excess (eg, excess weight gain, Cushingoid features, hypertension) determine the adequacy of cortisol replacement.

Close monitoring of pubertal status is also appropriate.

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Complications

Adrenal crisis, as mentioned, is the most acute complication that can arise in the treatment of patients with hypopituitarism and occurs when glucocorticoid replacement is not appropriately administered or, more likely, when the child develops a concurrent illness or medical treatment that increases the requirement for glucocorticoid and prevents oral replacement.

Growth hormone (GH) therapy is reported to cause some rare adverse effects. These include benign intracranial hypertension and slipped capital femoral epiphyses. Treatment also increases insulin resistance and, therefore, possibly increases the risk of diabetes. Although questions have been raised about malignancy, most data show little or no risk.

Appropriate monitoring should minimize any risks from thyroid or sex steroid treatment.

Women with panhypopituitarism may become pregnant with the help of reproductive endocrinology, and a study by Feferkorn et al indicated that the outcomes of these pregnancies are comparable to those of the general population. The risk of developing maternal infection and of congenital anomalies were found to be higher in the individuals with panhypopituitarism (odds ratios =  3.14 and 6.97, respectively), but the investigators stated that “due to the small number of cases these results should be interpreted with caution.” [39]

A study by Schönberger et al suggested that children with combined pituitary hormone deficiency (CPHD) may present with drug-resistant epilepsy and catastrophic outcomes from refractory seizures. The investigators found that 12 of 73 pediatric patients (16%) with CPHD had epilepsy, with 11 of these cases being drug resistant. Four of 12 patients with super-refractory status epilepticus (SRSE) were unexpected new-onset cases, with three of these experiencing a devastating clinical course; this included two who suffered major sequelae and one who died. [40]

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Prognosis

Morbidity and mortality due to hypopituitarism are caused by the individual hormone deficiencies or the underlying cause of hypopituitarism. Individual hormonal deficiencies are discussed in greater detail in the specific articles, and the underlying causes of death are not discussed here.

Acute mortality due to hormonal deficiencies is rare. When deaths occur due to hormonal deficiencies, they are usually caused by adrenal insufficiency secondary to ACTH deficiency. These deaths are most likely to occur when an accompanying illness prevents appropriate oral glucocorticoid replacement.

Growing, but not completely conclusive, evidence indicates that childhood hypopituitarism may be associated with a shortened adult lifespan, even with adequate hormonal replacement. [41] The increased mortality is due to cardiovascular abnormalities that are related to GH deficiency and past practices of not treating a GH deficiency when growth is complete. [42, 43] GH deficiency is associated with dyslipidemia that is not necessarily improved by GH therapy. [44] Children and adolescents with GH deficiency have been shown to have impaired vascular function. [45] Again, GH treatment may not restore function. Studies of adult growth treatment of GH deficiency have not conclusively demonstrated reductions of cardiovascular morbidity and mortality. [46, 47, 48, 49]

More recently, a French study reported increased adult mortality in childhood GH-treated patients. [50] The increased mortality was primarily in patients who had been small for gestational age at birth and who had received higher GH doses. Other studies have not confirmed the increased mortality. [51, 52]

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Patient Education

Educate parents about the dangers of adrenal insufficiency when the child is unable to take oral medication. Instruct parents to rapidly seek medical care. Many families can intramuscularly administer hydrocortisone at home if the child is unable to take oral medications. The home dose is generally 25 mg in children younger than 2 years, 50 mg in children younger than 5 years, and 100 mg in all other children. If children require intramuscular medication, they should be brought to the emergency department. If the family is unable to administer the intramuscular injection, they can take the parenteral hydrocortisone with them to the emergency department to avoid delays in administering appropriate treatment. [53]

Parents also need to be taught home stress coverage with triple the dose of glucocorticoid for less serious illnesses, such as fever greater than 38°C.

For patient education resources, see the Endocrine System Center and Growth Hormone Deficiency Center, as well as Hypopituitarism in Children, Growth Hormone Deficiency in Children, Growth Failure in Children, and Understanding Growth Hormone Deficiency Medications.

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