MCID: EMP001
MIFTS: 46

Empty Sella Syndrome malady

Summaries for Empty Sella Syndrome

Sources:
43NIH Rare Diseases, 44NINDS, 64Wikipedia, 33MalaCards
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NINDS:44 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 brain, thyroid and pituitary, and related mouse phenotypes are integument and liver/biliary system.

NIH Rare Diseases:43 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:64 Empty sella syndrome (abbreviated ESS) is a disorder that involves the sella turcica \"Turkish Saddle\",... more...

Aliases & Classifications for Empty Sella Syndrome

Sources:
8Disease Ontology, 43NIH Rare Diseases, 44NINDS, 10DISEASES, 61UMLS, 45Novoseek, 40NCIt, 57SNOMED-CT, 35MeSH
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Aliases & Descriptions:

empty sella syndrome 8 43 44 10 61
empty sella turcica 43
empty sella 45


External Ids:

Disease Ontology8 DOID:3642
NCIt40 C84686
MeSH35 D004652

Related Diseases for Empty Sella Syndrome

Sources:
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 163)
idRelated DiseaseScoreTop Affiliating Genes
1panhypopituitarism30.8PRL, POMC
2adenoma30.7HSPG2, PRL, IGFBP3, TRH, POMC, INS
3hyperprolactinemia30.7PRL, TRH, POMC
4diabetes insipidus30.7POMC, TRH, PRL
5acth deficiency30.7PRL, POMC
6acromegaly30.7INS, POMC, TRH, IGFBP3, PRL
7pituitary apoplexy30.6POMC, TRH, PRL
8precocious puberty30.6POMC, IGFBP3, PRL
9amenorrhea30.6PRL, TRH, POMC, INS
10sheehan syndrome30.5PRL, POMC
11growth hormone deficiency30.5PRL, IGFBP3, POMC, INS
12exophthalmos30.3TRH
13prader-willi syndrome30.3INS
14arthritis30.3POMC
15adrenal adenoma30.3POMC
16adenocarcinoma30.2HSPG2
17thyroiditis30.2TRH, POMC
18thyroid cancer30.2HSPG2, TRH
19prolactinoma30.2POMC, TRH, PRL
20pituitary tumors30.2POMC, TRH, PRL
21cushing's syndrome30.2POMC, TRH, PRL
22short stature30.2INS, IGFBP3, PRL
23dwarfism30.2IGFBP3
24premature ovarian failure30.1PRL, IGFBP3
25n syndrome10.8
26pseudotumor cerebri10.6
27pituitary deficiency due to empty sella turcica syndrome10.6
28cysticercosis10.3
29young syndrome10.3
30headache10.3
31adrenal carcinoma10.3
32isolated acth deficiency10.3
33testicular microlithiasis10.3
34bardet-biedl syndrome10.3
35isolated growth hormone deficiency10.3
36osteoporotic fracture10.2
37cardiac tamponade10.2
38neurofibromatosis10.2
39allergic rhinitis10.2
40primary hyperparathyroidism10.2
41arachnoiditis10.2
42west syndrome10.2
43ischemic neuropathy10.2
44nevoid basal cell carcinoma syndrome10.2
45central retinal vein occlusion10.2
46adult syndrome10.2
47fibrous dysplasia10.2
48ischemic optic neuropathy10.2
49renal tubular acidosis10.2
50midface dysplasia10.2

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



Diseases related to empty sella syndrome

Clinical Features for Empty Sella Syndrome

Drugs & Therapeutics for Empty Sella Syndrome

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
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Approved drugs:

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Drug clinical trials:

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Search NIH Clinical Center for Empty Sella Syndrome

Search CenterWatch for Empty Sella Syndrome

Genetic Tests for Empty Sella Syndrome

Anatomical Context for Empty Sella Syndrome

Sources:
33MalaCards
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MalaCards organs/tissues related to Empty Sella Syndrome:

33
Brain, Thyroid, Pituitary

Animal Models for Empty Sella Syndrome or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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Publications for Empty Sella Syndrome

Sources:
51PubMed
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Articles related to Empty Sella Syndrome:

(show top 50)    (show all 198)
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
Autoimmune polyglandular syndrome: an unusual presentation with empty sella, premature ovarian failure, and Hashimoto's thyroiditis associated with thyroid cancer. (22686234)
2012
3
Diagnostic dilemmas in a patient with anaemia. Empty sella syndrome--a case report. (20806186)
2010
4
Transsphenoidal treatment of secondary empty sella syndrome using low field strength intraoperative MRI: case report. (21132613)
2010
5
Rare case of Alstrom syndrome with empty sella and interfamilial presence of Bardet-Biedl phenotype. (20108498)
2009
6
Empty sella syndrome, hyperadrenocorticism and megaoesophagus in a dachshund. (17608665)
2007
7
Empty sella syndrome associated with a hyperfunctioning microadenoma invading the clivus. (18071980)
2007
8
Endonasal endoscopic transsphenoidal chiasmapexy with silicone plates for empty sella syndrome: technical note. (16127264)
2005
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: incidental findings at computerised tomography. (12789130)
2001
12
Two cases of empty sella syndrome with variable dexamethasone-suppressibility of ACTH and cortisol. (8930530)
1996
13
Transsphenoidal extradural chiasmapexy in the management of the symptomatic primary empty sella syndrome. (8237166)
1993
14
Primary empty sella syndrome and hypogonadotropic hypogonadism in young male patients. (1502422)
1992
15
Empty sella syndrome, diagnosed as allergic rhinitis. (1614692)
1992
16
Glaucomatous optic neuropathy and field loss in primary empty sella syndrome. (1764644)
1991
17
Co-existence of empty sella syndrome and low tension glaucoma. (2801037)
1989
18
Transsphenoidal chiasmopexy for long standing visual failure in the secondary empty sella syndrome. (3267311)
1988
19
Empty sella syndrome associated with central nervous system cysticercosis. (3154189)
1988
20
Empty sella syndrome occurring with polyostotic fibrous dysplasia of the midface: report of a case. (3507319)
1987
21
Empty sella syndrome. (3297893)
1987
22
A case of hyponatremia in panhypopituitarism caused by the primary empty sella syndrome. (3040385)
1987
23
Subnormal prolactin responsiveness to thyrotropin-releasing hormone (TRH) in women with primary empty sella syndrome. (3119699)
1987
24
Empty sella syndrome in an adolescent. (3700200)
1986
25
Hypothalamic-pituitary dysfunction in primary empty sella syndrome in childhood. (3958827)
1986
26
Primary empty sella syndrome with panhypopituitarism in a child. (6543857)
1984
27
Pituitary infarction and development of the empty sella syndrome after gastrointestinal haemorrhage. (6434032)
1984
28
Growth hormone secretion in empty sella syndrome. (6763056)
1982
29
Empty-sella syndrome. Occurrence in childhood. (7064951)
1982
30
Empty sella syndrome. (7338504)
1981
31
A case of empty sella syndrome with sufficient hypophyseal function and normal pregnancy. (7250078)
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
Physiopathological aspects of the empty sella syndrome combined with prolactin hypersecretion. (7193954)
1980
35
Familial pseudotumor cerebri and the empty sella syndrome. (6927213)
1980
36
Primary empty sella syndrome with CSF rhinorrhea. (7420665)
1980
37
Pseudotumor cerebri, empty sella syndrome, and adrenal adenoma. (7189029)
1980
38
Diagnosis of primary empty sella syndrome by computed tomography. (517991)
1979
39
Empty sella syndrome with neurofibromatosis. (411749)
1977
40
Amenorrhea-galactorrhea associated with the empty-sella syndrome. (560123)
1977
41
The empty sella syndrome analysis of 10 cases. (197801)
1977
42
Primary empty sella syndrome with visual field defects. (937363)
1976
43
The empty sella syndrome. (1013000)
1976
44
Secondary empty sella syndrome. (207113)
1976
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. (4124066)
1973
50
The empty sella syndrome. (5643927)
1968

Genetic Variations for Empty Sella Syndrome

Expression for genes affiliated with Empty Sella Syndrome

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
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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

Sources:
12EMD Millipore, 38NCBI BioSystems Database, 54Reactome
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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
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

Sources:
45Novoseek, 29IUPHAR, 11DrugBank, 50PharmGKB, 2BitterDB, 24HMDB, 60Tocris Bioscience
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Compounds related to Empty Sella Syndrome according to GeneCards/GeneDecks:

(show top 50)    (show all 85)
idCompoundScoreTop Affiliating Genes
1flesinoxan4510.0PRL, POMC
2acth 1-24459.9PRL, POMC
3ipsapirone45 2910.9PRL, POMC
411 deoxycortisol459.8PRL, POMC
5cycloheximide459.7HSPG2, TRH
6sulpiride45 29 1111.5TRH, PRL
7domperidone45 29 1111.5TRH, PRL
8megestrol acetate459.5POMC, IGFBP3
9naloxone45 50 29 1112.5PRL, TRH, POMC
10haloperidol45 29 2 50 1113.4POMC, TRH, PRL
11hexarelin45 2910.4PRL, IGFBP3, POMC
12naltrexone45 50 29 1112.4PRL, IGFBP3, POMC
13mecasermin45 1110.4IGFBP3, INS
14carbamazepine45 50 1111.4POMC, IGFBP3, PRL
15lanreotide45 2910.3IGFBP3, PRL
16oxandrolone45 1110.3IGFBP3, INS
17cocaine45 1110.3POMC, TRH, PRL
18ghrp459.3PRL, IGFBP3, TRH
19pyridostigmine45 1110.3PRL, IGFBP3, TRH
20progestin459.3POMC, IGFBP3, PRL
215-hydroxytryptamine459.3POMC, PRL, HSPG2
22gnrh459.3TRH, IGFBP3, HSPG2
23methimazole45 2 1111.3POMC, TRH, IGFBP3
24fructosamine45 2410.2IGFBP3, INS
25buserelin45 29 1111.1IGFBP3, PRL
262-deoxyglucose45 1110.0HSPG2, POMC, INS
27clomiphene citrate459.0INS, IGFBP3, PRL
28fenfluramine29 45 1110.9PRL, IGFBP3, TRH, POMC
29acth458.9PRL, IGFBP3, TRH, POMC
30bromocriptine29 45 1110.9POMC, TRH, IGFBP3, PRL
31clonidine45 29 1110.9POMC, TRH, IGFBP3, PRL
32triiodothyronine458.9PRL, IGFBP3, TRH, POMC
33ibmx45 29 6010.8INS, TRH, HSPG2
34estradiol45 11 2410.8POMC, TRH, IGFBP3, PRL
35progesterone45 60 29 11 2412.7PRL, IGFBP3, TRH, POMC
36hydrocortisone45 2 60 1111.7PRL, TRH, POMC, INS
37adenylate458.7POMC, TRH, IGFBP3, HSPG2
38glycerol45 11 2410.6IGFBP3, POMC, INS
39dehydroepiandrosterone sulfate458.6PRL, IGFBP3, POMC, INS
40dhea458.6PRL, IGFBP3, POMC, INS
41androstenedione45 249.6INS, POMC, IGFBP3, PRL
42epinephrine45 11 2410.5PRL, IGFBP3, POMC, INS
43octreotide45 60 29 1111.1PRL, IGFBP3, TRH, POMC, INS
44thyroxine45 249.1INS, POMC, TRH, IGFBP3, PRL
45norepinephrine45 11 2410.1INS, POMC, TRH, IGFBP3, PRL
46testosterone45 60 11 2411.0INS, POMC, TRH, IGFBP3, PRL
47arginine458.0PRL, IGFBP3, TRH, POMC, INS
48estrogen458.0PRL, IGFBP3, TRH, POMC, INS
49glucose458.0PRL, IGFBP3, TRH, POMC, INS
50dexamethasone45 50 29 1110.6HSPG2, PRL, IGFBP3, TRH, POMC, INS

GO Terms for genes affiliated with Empty Sella Syndrome

Sources:
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:0056158.1HSPG2, IGFBP3, INS, POMC
4extracellular regionGO:0055767.1INS, HSPG2, PRL, IGFBP3, TRH, POMC

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

3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet