The hypohyseal system can also be disturbed by Fabry. Since many important processes are controlled by it, we have dealt with it in more detail:

Part 1 Vasopressin

Pituitary gland (hypophysis).

Together with the hypothalamus – a part of the diencephalon – the pituitary gland controls the involuntary (autonomic) nervous system. This monitors and regulates energy, heat and water balance – and thus body temperature, heartbeat and urine output, as well as sleep, hunger and thirst. The pituitary gland also produces a number of hormones that regulate most of the endocrine glands in the body or act directly on specific organs.

The role of vasopressin

Vasopressin is a hormone produced by the pituitary gland. Vaopressin helps regulate the amount of water in the body by controlling how much water is excreted by the kidneys. Vasopressin is also called antidiuretic hormone (ADH), which is a substance that has an antidiuretic effect (stops urine formation). Vasopressin is a hormone that causes the kidney to recover more water from the urine. It is secreted more at night and thus ensures that less urine is produced.

Diagnosis: Copeptin and serum osmolality are determined for diagnosis, fasting in the morning after 8 hours of thirst.

Too much or too little vasopressin

Central diabetes insipidus

Vasopressin deficiency causes central diabetes insipidus. Excessive urine production occurs. In order not to become dehydrated, one feels a strong sense of thirst and has to drink a lot.

Syndrome of inadequate ADH secretion (SIADH).

When too much vasopressin is produced, it decreases the excretion of water by the kidneys. As a result, more water, which decreases the concentration of sodium in the body, is retained. Too low a level of sodium in the blood is called hyponatremia. Low excretion of urine is an important symptom.

Other symptoms include:

• Loss of appetite, nausea, vomiting.
• headache
• muscle cramps
• Irritability
• No edema

Hyponatremia can lead to fluid accumulation in the brain (cerebral edema), which can result in severe symptoms, such as:

• Unsteadiness of gait and stance
• Impaired memory and concentration
• seizures
• respiratory insufficiency
• Rhabdomyolysis (The medical term rhabdomyolysis describes a breakdown of muscle fibers).

The severity of the symptomatology depends on the severity of the hyponatremia and the speed of its development.

Empty Sella Syndrome

The pituitary gland is a pea-sized gland located within a bony structure (turk’s saddle) at the base of the skull. The turkensattel protects the pituitary gland, but leaves it little room to enlarge. If the pressure in the cerebrospinal fluid is too high, this can displace the pituitary gland from its proper position, resulting in what is known as “Empty Sella Syndrome”. Empty sella syndrome is diagnosed by computed tomography (CT).

Symptoms: Empty Sella syndrome can be asymptomatic, sometimes causing severe symptoms such as headaches and high blood pressure. Cerebrospinal fluid may also leak through the nose and visual disturbances may occur.

Therapy: Treatment is needed when the pituitary gland produces too many or too few hormones, depending on which hormones are affected. For treatment, the missing hormone can be replaced or medication can be administered to reduce excess hormones.