Panhypopituitarism
Classification according to ICD-10 | |
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E23.0 | Hypopituitarism - Panhypopituitarism |
ICD-10 online (WHO version 2019) |
As Panhypopituitarism (syn. Crohn Simmonds , Simmondsche disease , Eng. Simmond's syndrome ) refers to a condition in which a deficiency or absence of all in the anterior pituitary educated hormones is. In a narrower sense, it describes the generalized form of anterior pituitary insufficiency.
The following hormones produced in the pituitary are affected:
- no TSH : hypothyroidism
- no ACTH : secondary adrenal insufficiency
- no MSH : paleness
- no FSH and LH : hypothyroidism, amenorrhea
- no prolactin : agalacty
- no growth hormone : short stature (in childhood), trunk obesity , CHD
The Hamburg pathologist Morris Simmonds (1855–1925) was the first to describe it in 1914 .
causes
- Pituitary tumor
- Autoimmune processes
- Sheehan's syndrome (postpartum pituitary necrosis)
- Hemochromatosis
Rare causes
An unusual cause of panhypopituitarism is an accidental tear off of the pituitary stalk. Complete transection of the pituitary stalk ( pituitary stalk disruption syndrome ) appears to be a rather rare disease. However, there could be a high number of unreported cases. There are approximately 1.5 to 2 million cases of traumatic brain injuries in the USA annually. In moderate craniocerebral trauma, about 30–40% of patients experience disorders of the pituitary function. For this reason, an endocrinological evaluation is always recommended for these people in suspected cases. The first report of hypopituitarism after head injury dates from 1918.
Symptoms
Clinical symptoms do not set in until 80% of the pituitary cells are destroyed. The symptoms of the disease are explained by the lack of the individual hormone effects, in particular disruption of growth, metabolism and fertility .
Note: The "7 A's": no armpit hair , no eyebrows , amenorrhea, agalactia, apathy, adynamia, alabaster-colored pallor as well as hypotension and reduced osmolality in the urine.
therapy
In addition to a causal therapy for the underlying disease (e.g. tumor), the reduced hormones are substituted: testosterone or estrogen / progestin , thyroxine , cortisol and growth hormone . The dosage is based on clinical findings and under regular control of the substituted hormones. The dosage of cortisol must be adjusted upwards in stressful situations, as cortisol is a so-called stress hormone.
Web links
- Network of Pituitary and Adrenal Diseases e. V.
- Craniopharyngioma group
- Children's pituitary group - Germany-wide self-help group for parents with children or adolescents with pituitary insufficiency. It offers information, exchange and support on all questions and topics related to the disease.
Individual evidence
- ↑ M. Simmonds: About hypophysis atrophy with fatal outcome. In: Dtsch. Med. Wochenschrift , 40, 1914, p. 322.
- ↑ Carlos Thomas (Ed.): Special Pathology. Schattauer Verlag, 1996, ISBN 3-7945-1713-X , p. 379; books.google.at
- ↑ K. Nakamura: Traumatic panhypopituitarism: case report. In: No Shinkei Geka , 34 (5), May 2006, pp. 491-495. PMID 16689392
- ^ RJ Urban: Hypopituitarism after acute brain injury. In: Growth Horm IGF Res. , 16 Suppl A, Jul 2006, pp. S25-S29. Epub 2006 May 12. PMID 16697673
- ↑ BL Herrmann u. a .: Hypopituitarism following severe traumatic brain injury. In: Exp Clin Endocrinol Diabetes , 114 (6), Jun 2006, pp. 316-321. PMID 16868891
- ^ S. Benvenga: Brain injury and hypopituitarism: the historical background. In: Pituitary , 8 (3-4), 2005, pp. 193-195. PMID 16508718