MCID: EMP001
MIFTS: 48

Empty Sella Syndrome malady

Summaries for Empty Sella Syndrome

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42NIH Rare Diseases, 43NINDS, 63Wikipedia, 32MalaCards
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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

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 60UMLS, 44Novoseek, 39NCIt, 56SNOMED-CT, 34MeSH
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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

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17GeneCards, 18GeneDecks
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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

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Drugs & Therapeutics for Empty Sella Syndrome

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Empty Sella Syndrome

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Anatomical Context for Empty Sella Syndrome

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32MalaCards
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MalaCards organs/tissues related to Empty Sella Syndrome:

32
Pituitary, Testes, Brain, Thyroid, Bone

Animal Models for Empty Sella Syndrome or affiliated genes

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36MGI
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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

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50PubMed
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Articles related to Empty Sella Syndrome:

(show top 50)    (show all 192)
idTitleAuthorsYear
1
Osteoporotic fractures and persistent non-fusion of the hand epiphyses caused by empty sella syndrome in an adult: a case report. (24029021)
2013
2
Empty sella syndrome - beyond being an incidental finding. (23565413)
2012
3
Precocious puberty and empty sella syndrome in a girl cured of acute lymphoblastic leukemia. (22308868)
2011
4
Rare case of Alstrom syndrome with empty sella and interfamilial presence of Bardet-Biedl phenotype. (20108498)
2009
5
Empty sella syndrome associated with a hyperfunctioning microadenoma invading the clivus. (18071980)
2007
6
Endocrine disturbances in empty sella syndrome: case reports and review of literature. (15901528)
2005
7
Endonasal endoscopic transsphenoidal chiasmapexy with silicone plates for empty sella syndrome: technical note. (16127264)
2005
8
Primary empty sella syndrome. (12900138)
2003
9
Spontaneous nasal cerebrospinal fluid leaks and empty sella syndrome: a clinical association. (12751703)
2003
10
Treating hyponatremia in an empty sella syndrome patient complicated with possible myelinolysis. (12635841)
2002
11
Empty sella syndrome in nevoid basal cell carcinoma syndrome. (10838118)
2000
12
Empty sella syndrome. (23119635)
1999
13
Two cases of empty sella syndrome with variable dexamethasone-suppressibility of ACTH and cortisol. (8930530)
1996
14
Glaucomatous optic neuropathy and field loss in primary empty sella syndrome. (1764644)
1991
15
Cerebrospinal fluid pressure and prolactin in empty sella syndrome. (2107018)
1990
16
Co-existence of empty sella syndrome and low tension glaucoma. (2801037)
1989
17
Empty sella syndrome associated with central nervous system cysticercosis. (3154189)
1988
18
Empty sella syndrome and pituitary apoplexy. (2895306)
1988
19
Empty sella syndrome and growth failure. (3443554)
1987
20
Empty sella syndrome. (3297893)
1987
21
A case of hyponatremia in panhypopituitarism caused by the primary empty sella syndrome. (3040385)
1987
22
Subnormal prolactin responsiveness to thyrotropin-releasing hormone (TRH) in women with primary empty sella syndrome. (3119699)
1987
23
Empty sella syndrome caused by syphilitic arachnoiditis. (3951113)
1986
24
Hypothalamic-pituitary dysfunction in primary empty sella syndrome in childhood. (3958827)
1986
25
Empty sella syndrome. (6723249)
1984
26
Dissociation of serum prolactin response to sequential thyrotropin-releasing hormone and chlorpromazine stimulation in patients with primary empty sella syndrome. (6436062)
1984
27
Pituitary infarction and development of the empty sella syndrome after gastrointestinal haemorrhage. (6434032)
1984
28
Coexistent primary empty sella syndrome and hyperprolactinemia. Report of 11 cases. (6870411)
1983
29
Growth hormone secretion in empty sella syndrome. (6763056)
1982
30
Empty-sella syndrome. Occurrence in childhood. (7064951)
1982
31
Primary empty sella syndrome and hypopituitarism associated with primary hypothyroidism. (6792264)
1981
32
Neurosurgical aspects of the empty sella syndrome. (7051219)
1981
33
Pregnancy-induced reversible visual field changes in primary empty sella syndrome. (6942254)
1981
34
Pituitary function in patients with enlarged sella turcica and primary empty sella syndrome. (7211487)
1981
35
Empty sella syndrome, pulsating exophthalmos, and calvarial bone defects in a case of neurofibroma. (6782020)
1980
36
The clinical and radiological features of the empty sella syndrome. (7471624)
1980
37
Primary empty sella syndrome with CSF rhinorrhea. (7420665)
1980
38
Pseudotumor cerebri, empty sella syndrome, and adrenal adenoma. (7189029)
1980
39
Primary empty sella syndrome with elevated serum prolactin. (561354)
1977
40
The empty sella syndrome. (904521)
1977
41
Coexisting primary empty sella syndrome and acromegally. (908157)
1977
42
The empty sella syndrome analysis of 10 cases. (197801)
1977
43
Secondary empty sella syndrome. (207113)
1976
44
Does pseudotumor cerebri cause the empty sella syndrome? (1168876)
1975
45
Primary empty sella syndrome and amenorrhea. (1196556)
1975
46
Empty sella syndrome as complication of benign intracranial hypertension. (1185249)
1975
47
Primary empty sella syndrome. (165637)
1975
48
Intrasellar arachnoidocele and the so-called empty sella syndrome. (4852546)
1974
49
The empty sella syndrome. Case report. (5457646)
1970
50
The empty sella syndrome. (5643927)
1968

Genetic Variations for Empty Sella Syndrome

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Expression for genes affiliated with Empty Sella Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Empty Sella Syndrome

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Pathways for genes affiliated with Empty Sella Syndrome

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12EMD Millipore, 37NCBI BioSystems Database, 53Reactome
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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
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9.5POMC, TRH
29.2INS, PRL
3
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9.2INS, POMC
4
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8.7INS, PRL, HSPG2
5
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7.7HSPG2, PRL, TRH, POMC, INS

Compounds for genes affiliated with Empty Sella Syndrome

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44Novoseek, 28IUPHAR, 11DrugBank, 49PharmGKB, 2BitterDB, 24HMDB, 59Tocris Bioscience
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Compounds related to Empty Sella Syndrome according to GeneCards/GeneDecks:

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

GO Terms for genes affiliated with Empty Sella Syndrome

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16Gene Ontology
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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

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Sources for Empty Sella Syndrome

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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