MCID: EMP001
MIFTS: 48

Empty Sella Syndrome malady

Summaries for Empty Sella Syndrome

About this section
Sources:
42NIH Rare Diseases, 43NINDS, 63Wikipedia, 32MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
NINDS:43 Empty Sella Syndrome (ESS) is a disorder that involves the , a bony structure at the base of the brain that surrounds and protects the pituitary gland. ESS is a condition that is often discovered during tests for pituitary disorders, when radiological imaging of the pituitary gland reveals a sella turcica that appears to be empty. There are two types of ESS: primary and secondary. happens when a small anatomical defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten out along the interior walls of the sella turcica cavity. Primary ESS is associated with obesity and high blood pressure in women. The disorder sometimes results in a build-up of fluid pressure inside the skull and the pituitary gland may be smaller than usual. is the result of the pituitary gland regressing within the cavity after an injury, surgery, or radiation therapy. Individuals with secondary ESS due to destruction of the pituitary gland have symptoms that reflect the loss of pituitary functions, such as the ceasing of menstrual periods, infertility, fatigue, and intolerance to stress and infection. In children, ESS may be associated with early onset of puberty, growth hormone deficiency, pituitary tumors, or pituitary gland dysfunction. MRI scans are useful in evaluating ESS and differentiating it from other disorders that produce an enlarged sella.

MalaCards: Empty Sella Syndrome, also known as empty sella turcica, is related to panhypopituitarism and adenoma. An important gene associated with Empty Sella Syndrome is PRL (prolactin), and among its related pathways are Development Leptin signaling via JAK/STAT and MAPK cascades and Signaling events mediated by PTP1B. The compounds flesinoxan and acth 1-24 have been mentioned in the context of this disorder. Affiliated tissues include pituitary, testes and brain, and related mouse phenotypes are integument and liver/biliary system.

NIH Rare Diseases:42 Empty sella syndrome is a condition in which the pituitary gland shrinks or becomes flattened. there are two types of this syndrome, primary and secondary.the primary form occurs when a hole in the membrane covering the pituitary gland allows fluid in, which presses on the pituitary. the secondary form occurs when the pituitary gland is damaged by a tumor, radiation therapy, or surgery. empty sella syndrome can also be seen in a condition called pseudotumor cerebri.  often empty sella syndrome causes no symptoms, however some people experience symptoms of hypopituitarism due to a partial or complete loss of pituitary function. last updated: 1/28/2010

Wikipedia:63 Empty sella syndrome (abbreviated ESS) is a disorder that involves the sella turcica \"Turkish Saddle\",... more...

Aliases & Classifications for Empty Sella Syndrome

About this section
Sources:
8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 60UMLS, 44Novoseek, 39NCIt, 56SNOMED-CT, 34MeSH
See all sources

Aliases & Descriptions:

empty sella syndrome 8 42 43 10 60
empty sella turcica 42
empty sella 44


External Ids:

Disease Ontology8 DOID:3642
NCIt39 C84686
MeSH34 D004652

Related Diseases for Empty Sella Syndrome

About this section
Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Empty Sella Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 168)
idRelated DiseaseScoreTop Affiliating Genes
1panhypopituitarism30.7PRL, POMC
2adenoma30.7HSPG2, PRL, IGFBP3, TRH, POMC, INS
3hypertension30.7POMC, INS
4acromegaly30.7IGFBP3, PRL
5hyperprolactinemia30.7PRL, TRH, POMC
6diabetes insipidus30.7POMC, TRH, PRL
7pituitary adenoma30.7POMC, TRH, PRL
8thyroiditis30.6TRH, POMC
9pituitary apoplexy30.6POMC, TRH, PRL
10hypothyroidism30.6IGFBP3, TRH
11amenorrhea30.6PRL, TRH, POMC, INS
12hypopituitarism30.5PRL, IGFBP3, TRH, POMC, INS
13hypogonadism30.4PRL, IGFBP3, TRH, POMC, INS
14exophthalmos30.2TRH
15arthritis30.2POMC
16adrenal adenoma30.2POMC
17adenocarcinoma30.2HSPG2
18hypogonadotropism30.2PRL
19thyroid cancer30.2HSPG2, TRH
20prolactinoma30.2POMC, TRH, PRL
21cushing's syndrome30.2POMC, TRH, PRL
22obesity30.2IGFBP3, TRH, POMC, INS
23dwarfism30.1IGFBP3
24ovarian cancer30.1IGFBP3
25pancreatitis30.1HSPG2, INS
26premature ovarian failure30.1PRL, IGFBP3
27neuroendocrine tumor30.1POMC, TRH, PRL
28pseudotumor cerebri10.6
29pituitary deficiency due to empty sella turcica syndrome10.5
30intracranial hypertension10.5
31cysticercosis10.3
32neuropathy10.3
33adrenal carcinoma10.3
34leiomyoma10.3
35bardet-biedl syndrome10.2
36osteoporotic fracture10.2
37cardiac tamponade10.2
38neurofibromatosis10.2
39low tension glaucoma10.2
40mucopolysaccharidosis vi10.2
41allergic rhinitis10.2
42primary hyperparathyroidism10.2
43arachnoiditis10.2
44west syndrome10.2
45ischemic neuropathy10.2
46nevoid basal cell carcinoma syndrome10.2
47central retinal vein occlusion10.2
48adult syndrome10.2
49fibrous dysplasia10.2
50ischemic optic neuropathy10.2

Graphical network of the top 20 diseases related to Empty Sella Syndrome:



Diseases related to empty sella syndrome

Clinical Features for Empty Sella Syndrome

About this section

Drugs & Therapeutics for Empty Sella Syndrome

About this section
Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Empty Sella Syndrome

Drug clinical trials:

Search ClinicalTrials for Empty Sella Syndrome

Search NIH Clinical Center for Empty Sella Syndrome

Search CenterWatch for Empty Sella Syndrome

Genetic Tests for Empty Sella Syndrome

About this section

Anatomical Context for Empty Sella Syndrome

About this section
Sources:
32MalaCards
See all sources

MalaCards organs/tissues related to Empty Sella Syndrome:

32
Pituitary, Testes, Brain, Thyroid, Bone

Animal Models for Empty Sella Syndrome or affiliated genes

About this section
Sources:
36MGI
See all sources

MGI Mouse Phenotypes related to Empty Sella Syndrome:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00107718.3INS, POMC, PRL, HSPG2
2MP:00053707.8INS, POMC, IGFBP3, PRL, HSPG2
3MP:00053867.6HSPG2, PRL, IGFBP3, POMC, INS
4MP:00036317.6INS, POMC, TRH, PRL, HSPG2
5MP:00053787.2HSPG2, IGFBP3, TRH, POMC, INS
6MP:00053767.2HSPG2, PRL, IGFBP3, TRH, POMC, INS

Publications for Empty Sella Syndrome

About this section
Sources:
50PubMed
See all sources

Articles related to Empty Sella Syndrome:

(show top 50)    (show all 192)
idTitleAuthorsYear
1
Empty sella syndrome. (23304808)
2012
2
Primary pituitary abscess followed by empty sella syndrome in an adolescent girl. (18953655)
2010
3
Two cases of Kallmann syndrome associated with empty sella. (17530415)
2008
4
A new visual field test in empty sella syndrome: rarebit perimetry. (18609487)
2008
5
Visual vignette. Empty sella syndrome with central hypothyroidism and hypogonadism. (17599868)
2007
6
Profound vasodilatory hypotension in a patient with known empty sella syndrome following cardiac surgery. (17635438)
2007
7
Primary empty sella syndrome in a series of 142 patients. (16304986)
2005
8
Empty sella syndrome, panhypopituitarism, and diabetes insipidus. (16338880)
2005
9
Coexisting acromegaly and primary empty sella syndrome. (15361823)
2004
10
Reversible hypothyroidism in empty sella syndrome: a case report. (9790274)
1998
11
Primary empty sella syndrome and central retinal vein occlusion. (7746642)
1995
12
A case of Sheehan's syndrome associated with severe anemia and empty sella proved 48 years after postpartum hemorrhage. (8822323)
1995
13
43 cases of primary empty sella syndrome: a case series]. (7619655)
1995
14
Echographic and ophthalmodynamometric study in the empty sella syndrome. (8278157)
1993
15
The empty sella syndrome: much ado about nothing. (1581758)
1992
16
Visual disturbances associated with primary empty sella syndrome in patients with chronic renal failure. (1562126)
1992
17
A case of pregnancy associated osteoporosis and empty sella syndrome. (2045263)
1991
18
Continuous bromocriptine treatment of empty sella syndrome aggravating pregnancy. A case report. (1778518)
1991
19
Primary empty sella syndrome in childhood: association with precocious puberty. (1914345)
1991
20
Can empty sella syndrome be mistaken for a progressive form of nonarteritic ischemic optic neuropathy? (2383186)
1990
21
Relations between Sheehan's syndrome and empty sella turcica. A functional study apropos of 6 cases]. (2172669)
1990
22
Empty sella syndrome associated with diabetes insipidus: case report and review of the literature. (2671113)
1989
23
High frequency of empty sella syndrome in children with growth hormone deficiency. (2785096)
1989
24
Empty sella syndrome and pituitary apoplexy. (2896893)
1988
25
Antipituitary antibodies in patients with the primary empty sella syndrome. (3417843)
1988
26
Empty sella syndrome occurring with polyostotic fibrous dysplasia of the midface: report of a case. (3507319)
1987
27
Empty sella syndrome and growth deficiency in childhood. (3667334)
1987
28
The empty sella syndrome. A review of 14 cases. (6591594)
1983
29
Galactorrhea, amenorrhea, hyperprolactinemia and pseudotumor cerebri in a patient with primary empty sella syndrome: case report with review of the literature. (6984740)
1982
30
Primary empty sella syndrome, ACTH hypersecretion, and normal adrenocortical function. Report of two cases. (6289662)
1982
31
The pituitary: cerebrospinal fluid barrier in the primary empty sella syndrome. (6295076)
1982
32
The primary empty sella syndrome. Diagnosis with metrizamide cisternography. (6895391)
1981
33
Metrizamide cisternography in the investigation of the empty sella syndrome. (7212891)
1981
34
Pituitary function in the empty sella syndrome. (7207701)
1981
35
Diagnosis of empty sella syndrome using Amipaque cisternography combined with computerized tomography. (7350285)
1980
36
CSF rhinorrhea associated with the empty-sella syndrome. (7369927)
1980
37
Diurnal variation of prolactin secretion differentiates pituitary tumors from the primary empty sella syndrome. (7192490)
1980
38
A dynamic pressure study of spontaneous CSF rhinorrhea in the empty sella syndrome. Case report. (7350267)
1980
39
Primary empty sella syndrome with panhypopituitarism, diabetes insipidus, and visual field defects. (707005)
1978
40
The empty sella syndrome--intrasellar cisternal herniation--in "normal" patients and in patients with communicating hydrocephalus and intracranial tumors. (752126)
1978
41
Spontaneous fracture of the dorsum sellae associated with post-irradiation empty sella syndrome. (897994)
1977
42
Visual disturbances associated with primary empty sella syndrome. (890935)
1977
43
Pseudo-pseudohypoparathyroidism, empty sella syndrome, and hypopituitarism in a young woman. (831182)
1977
44
Empty sella syndrome. (615371)
1977
45
Empty sella syndrome secondary to intrasellar cyst in adolescence. (961646)
1976
46
Empty sella syndrome. Sequela of the spontaneous remission of acromegaly. (1190936)
1975
47
Empty sella syndrome in a boy with mucopolysaccharidosis type VI (Maroteaux-Lamy). (807539)
1975
48
Foster Kennedy syndrome, aqueductal stenosis and empty sella. (4546807)
1974
49
Empty-sella syndrome. (4125344)
1973
50
Endocrine function in nineteen patients with empty sella syndrome. (4334769)
1972

Genetic Variations for Empty Sella Syndrome

About this section

Expression for genes affiliated with Empty Sella Syndrome

About this section
Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Empty Sella Syndrome

Search GEO for disease gene expression data for Empty Sella Syndrome.

Pathways for genes affiliated with Empty Sella Syndrome

About this section
Sources:
12EMD Millipore, 37NCBI BioSystems Database, 53Reactome
See all sources

Pathways related to Empty Sella Syndrome according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Development Leptin signaling via JAK/STAT and MAPK cascades
Hide members
9.5POMC, TRH
29.2INS, PRL
3
Hide members
9.2INS, POMC
4
Hide members
8.7INS, PRL, HSPG2
5
Hide members
7.7HSPG2, PRL, TRH, POMC, INS

Compounds for genes affiliated with Empty Sella Syndrome

About this section
Sources:
44Novoseek, 28IUPHAR, 11DrugBank, 49PharmGKB, 2BitterDB, 24HMDB, 59Tocris Bioscience
See all sources

Compounds related to Empty Sella Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 85)
idCompoundScoreTop Affiliating Genes
1flesinoxan4410.0PRL, POMC
2acth 1-24449.9PRL, POMC
3ipsapirone44 2810.9PRL, POMC
411 deoxycortisol449.8PRL, POMC
5cycloheximide449.7HSPG2, TRH
6sulpiride44 28 1111.5TRH, PRL
7domperidone44 28 1111.5TRH, PRL
8megestrol acetate449.5POMC, IGFBP3
9naloxone44 49 28 1112.5PRL, TRH, POMC
10haloperidol44 28 2 49 1113.4POMC, TRH, PRL
11hexarelin44 2810.4PRL, IGFBP3, POMC
12naltrexone44 49 28 1112.4PRL, IGFBP3, POMC
13mecasermin44 1110.4IGFBP3, INS
14carbamazepine44 49 1111.4POMC, IGFBP3, PRL
15lanreotide44 2810.3IGFBP3, PRL
16oxandrolone44 1110.3IGFBP3, INS
17cocaine44 1110.3POMC, TRH, PRL
18ghrp449.3PRL, IGFBP3, TRH
19pyridostigmine44 1110.3PRL, IGFBP3, TRH
20progestin449.3POMC, IGFBP3, PRL
215-hydroxytryptamine449.3POMC, PRL, HSPG2
22gnrh449.3TRH, IGFBP3, HSPG2
23methimazole44 2 1111.3POMC, TRH, IGFBP3
24fructosamine44 2410.2IGFBP3, INS
25buserelin44 28 1111.1IGFBP3, PRL
262-deoxyglucose44 1110.0HSPG2, POMC, INS
27clomiphene citrate449.0INS, IGFBP3, PRL
28fenfluramine28 44 1110.9PRL, IGFBP3, TRH, POMC
29acth448.9PRL, IGFBP3, TRH, POMC
30bromocriptine28 44 1110.9POMC, TRH, IGFBP3, PRL
31clonidine44 28 1110.9POMC, TRH, IGFBP3, PRL
32triiodothyronine448.9PRL, IGFBP3, TRH, POMC
33ibmx44 28 5910.8INS, TRH, HSPG2
34estradiol44 11 2410.8POMC, TRH, IGFBP3, PRL
35progesterone44 59 28 11 2412.7PRL, IGFBP3, TRH, POMC
36hydrocortisone44 2 59 1111.7PRL, TRH, POMC, INS
37adenylate448.7POMC, TRH, IGFBP3, HSPG2
38glycerol44 11 2410.6IGFBP3, POMC, INS
39dehydroepiandrosterone sulfate448.6PRL, IGFBP3, POMC, INS
40dhea448.6PRL, IGFBP3, POMC, INS
41androstenedione44 249.6INS, POMC, IGFBP3, PRL
42epinephrine44 11 2410.5PRL, IGFBP3, POMC, INS
43octreotide44 59 28 1111.1PRL, IGFBP3, TRH, POMC, INS
44thyroxine44 249.1INS, POMC, TRH, IGFBP3, PRL
45norepinephrine44 11 2410.1INS, POMC, TRH, IGFBP3, PRL
46testosterone44 59 11 2411.0INS, POMC, TRH, IGFBP3, PRL
47arginine448.0PRL, IGFBP3, TRH, POMC, INS
48estrogen448.0PRL, IGFBP3, TRH, POMC, INS
49glucose448.0PRL, IGFBP3, TRH, POMC, INS
50dexamethasone44 49 28 1110.6HSPG2, PRL, IGFBP3, TRH, POMC, INS

GO Terms for genes affiliated with Empty Sella Syndrome

About this section
Sources:
16Gene Ontology
See all sources

Cellular components related to Empty Sella Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1secretory granuleGO:0301419.0PRL, TRH, POMC
2secretory granule lumenGO:0347748.9INS, POMC
3extracellular spaceGO:0056158.1INS, POMC, IGFBP3, HSPG2
4extracellular regionGO:0055767.1INS, POMC, TRH, IGFBP3, PRL, HSPG2

Biological processes related to Empty Sella Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1positive regulation of MAPK cascadeGO:0434109.0INS, IGFBP3
2cell-cell signalingGO:0072678.4INS, POMC, TRH

Molecular functions related to Empty Sella Syndrome according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1hormone activityGO:0051798.8INS, POMC, PRL

Products for genes affiliated with Empty Sella Syndrome

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Empty Sella Syndrome

About this section
3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet