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MCID: EMP001
MIFTS: 43

Empty Sella Syndrome

Categories: Endocrine diseases, Neuronal diseases, Rare diseases
Genes Tissues Related diseases Publications Pathways Drugs
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Aliases & Classifications for Empty Sella Syndrome

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MalaCards integrated aliases for Empty Sella Syndrome:

Name: Empty Sella Syndrome 12 75 53 54 44 15 17 72
Empty Sella Turcica 53
Empty Sella 55

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Endocrine diseases Neuronal diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:3642
MeSH 44 D004652
NCIt 50 C84686
UMLS 72 C0014008
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Summaries for Empty Sella Syndrome

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NINDS : 54 Empty Sella Syndrome (ESS) is a disorder that involves the sella turcica, a bony structure at the base of the brain that surrounds and protects the pituitary gland. ESS is often discovered during radiological imaging tests for pituitary disorders. ESS occurs in up to 25 percent of the population. An individual with ESS may have no symptoms or may have symptoms resulting from partial or complete loss of pituitary function (including headaches, low sex drive, and impotence). There are two types of ESS: primary and secondary. Primary ESS happens when a small anatomical defect above the pituitary gland allows spinal fluid to partially or completely fill the sella turcica. This causes the gland to flatten out along the interior walls of the sella turcica cavity. Individuals with primary ESS may have high levels of the hormone prolactin, which can interfere with the normal function of the testicles and ovaries. Primary ESS is most common in adults and women, and is often associated with obesity and high blood pressure. In some instances the pituitary gland may be smaller than usual; this may be due to a condition called pseudotumor cerebri (which means "false brain tumor," brought on by high pressure within the skull).  In rare instances this high fluid pressure can be associated with drainage of spinal fluid through the nose. Secondary ESS is the result of the pituitary gland regressing within the cavity after an injury, surgery, or radiation therapy. Individuals with secondary ESS can sometimes 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. Magnetic resonance imaging (MRI) scans are useful in evaluating ESS and for identifying underlying disorders that may be the cause of high fluid pressure.

MalaCards based summary : Empty Sella Syndrome, also known as empty sella turcica, is related to diabetes insipidus and galactorrhea. An important gene associated with Empty Sella Syndrome is PRL (Prolactin), and among its related pathways/superpathways are Peptide ligand-binding receptors and NF-KappaB Family Pathway. The drugs Guaifenesin and Acetaminophen have been mentioned in the context of this disorder. Affiliated tissues include pituitary, brain and testes, and related phenotypes are endocrine/exocrine gland and neoplasm

NIH Rare Diseases : 53 Empty sella syndrome (ESS) is a condition that involves the sella turcica, a bony structure at the base of the brain that protects the pituitary gland. There is a primary and secondary form of the condition. The primary form occurs when a structural defect above the pituitary gland increases pressure in the sella turcica and causes the gland to flatten. The secondary form occurs when the pituitary gland is damaged due to injury, a tumor, surgery or radiation therapy. Some people with ESS have no symptoms. People with secondary ESS may have symptoms of decreased pituitary function such as absence of menstruation, 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. Treatment focuses on the symptoms present in each person.

Wikipedia : 75 Empty sella syndrome (ESS) is the condition when the pituitary gland shrinks or becomes flattened,... more...

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Related Diseases for Empty Sella Syndrome

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

(show top 50) (show all 329)
# Related Disease Score Top Affiliating Genes
1 diabetes insipidus 31.3 PRL POMC
2 galactorrhea 31.0 PRL IGF1
3 lymphocytic hypophysitis 30.9 POMC GH1
4 hypogonadotropism 30.7 PRL GNRH1
5 growth hormone deficiency 30.7 IGF1 GH1
6 acth-secreting pituitary adenoma 30.4 PRL POMC
7 precocious puberty 30.4 GNRH1 GH1
8 isolated growth hormone deficiency 30.4 IGF1 GH1
9 hypoadrenalism 30.3 POMC GH1
10 turner syndrome 30.2 IGF1 GH1
11 adenoma 30.1 PRL POMC IGF1 GH1
12 traumatic brain injury 30.1 IGF1 GH1
13 isolated growth hormone deficiency, type ii 30.0 IGF1 GH1
14 pituitary apoplexy 29.9 PRL POMC GNRH1
15 pituitary hormone deficiency, combined, 2 29.8 PRL POMC IGF1 GH1
16 insulin-like growth factor i 29.8 PRL IGF1 GH1
17 pituitary tumors 29.8 PRL POMC IGF1 GH1
18 sheehan syndrome 29.8 PRL POMC IGF1
19 conn's syndrome 29.7 PRL POMC GH1
20 fibrous dysplasia 29.7 PRL IGF1 GH1
21 anovulation 29.5 PRL IGF1 GNRH1
22 hyperthyroidism 29.5 TRH PRL GH1
23 central precocious puberty 29.3 IGF1 GNRH1 GH1
24 polycystic ovary syndrome 29.2 PRL IGF1 GNRH1
25 amenorrhea 29.2 TRH PRL POMC GNRH1
26 premature ovarian failure 1 29.1 PRL GNRH1
27 hypothyroidism 29.1 TRH PRL POMC IGF1 GH1
28 hypopituitarism 29.0 PRL POMC IGF1 GNRH1 GH1
29 pituitary adenoma, prolactin-secreting 28.9 TRH PRL POMC IGF1 GH1
30 acromegaly 28.8 TRH PRL POMC IGF1 GH1
31 pituitary infarct 28.7 PRL POMC IGF1 GNRH1
32 hyperandrogenism 28.6 PRL POMC IGF1 GNRH1
33 hypothalamic disease 28.6 PRL POMC GNRH1 GH1
34 hypothyroidism, congenital, nongoitrous, 4 28.5 TRH PRL POMC IGF1 GH1
35 prader-willi syndrome 28.5 POMC IGF1 GNRH1 GH1
36 chromophobe adenoma 28.0 TRH PRL POMC GNRH1 GH1
37 hyperprolactinemia 27.9 TRH PRL POMC IGF1 GNRH1 GH1
38 pituitary gland disease 27.7 TRH PRL POMC IGF1 GNRH1 GH1
39 craniopharyngioma 27.7 TRH PRL IGF1 GNRH1 GH1
40 pituitary deficiency due to empty sella turcica syndrome 12.7
41 craniofacial anomalies and anterior segment dysgenesis syndrome 11.7
42 pituitary adenoma 10.6
43 intracranial hypertension 10.6
44 headache 10.5
45 hypogonadism 10.5
46 hypoglycemia 10.4
47 intracranial hypertension, idiopathic 10.4
48 papilledema 10.4
49 pituitary carcinoma 10.3 PRL POMC
50 tuberculum sellae meningioma 10.3 PRL POMC

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



Diseases related to Empty Sella Syndrome
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Symptoms & Phenotypes for Empty Sella Syndrome

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MGI Mouse Phenotypes related to Empty Sella Syndrome:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.35 GNRH1 IGF1 POMC PRL TRH
2 neoplasm MP:0002006 8.92 GNRH1 IGF1 POMC PRL
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Drugs & Therapeutics for Empty Sella Syndrome

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Drugs for Empty Sella Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 30)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Guaifenesin Approved, Investigational, Vet_approved Phase 2, Phase 3 93-14-1 3516
2
Acetaminophen Approved Phase 2, Phase 3 103-90-2 1983
3
Acetazolamide Approved, Vet_approved Phase 2, Phase 3 59-66-5 1986
4
Codeine Approved, Illicit Phase 2, Phase 3 76-57-3 5284371
5
Naproxen Approved, Vet_approved Phase 2, Phase 3 22204-53-1 1302 156391
6
Ibuprofen Approved Phase 2, Phase 3 15687-27-1 3672
7
Aspirin Approved, Vet_approved Phase 2, Phase 3 50-78-2 2244
8 Narcotics Phase 2, Phase 3
9 Analgesics, Non-Narcotic Phase 2, Phase 3
10 Cyclooxygenase Inhibitors Phase 2, Phase 3
11 Central Nervous System Depressants Phase 2, Phase 3
12 Fibrinolytic Agents Phase 2, Phase 3
13 diuretics Phase 2, Phase 3
14 Natriuretic Agents Phase 2, Phase 3
15 Anti-Inflammatory Agents, Non-Steroidal Phase 2, Phase 3
16 Analgesics Phase 2, Phase 3
17 Peripheral Nervous System Agents Phase 2, Phase 3
18 Respiratory System Agents Phase 2, Phase 3
19 Chlorpheniramine, phenylpropanolamine drug combination Phase 2, Phase 3
20 Analgesics, Opioid Phase 2, Phase 3
21 Anti-Inflammatory Agents Phase 2, Phase 3
22 Platelet Aggregation Inhibitors Phase 2, Phase 3
23 Anticonvulsants Phase 2, Phase 3
24 Antitussive Agents Phase 2, Phase 3
25 Antipyretics Phase 2, Phase 3
26 Antirheumatic Agents Phase 2, Phase 3
27 Carbonic Anhydrase Inhibitors Phase 2, Phase 3
28 Hormones
29 Hormone Antagonists
30 Hormones, Hormone Substitutes, and Hormone Antagonists

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Multicenter, Double-blind, Randomized, Placebo-controlled Study of Weight-Reduction and/or Low Sodium Diet Plus Acetazolamide vs Diet Plus Placebo in Subjects With Idiopathic Intracranial Hypertension With Mild Visual Loss Completed NCT01003639 Phase 2, Phase 3 Acetazolamide;Placebo
2 Growth Hormone Deficiency, Empty Sella and Cardio-Metabolic Risk Factors in Obesity: a Cross-Sectional Study. Completed NCT02092779

Search NIH Clinical Center for Empty Sella Syndrome

Cochrane evidence based reviews: empty sella syndrome

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Genetic Tests for Empty Sella Syndrome

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Anatomical Context for Empty Sella Syndrome

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MalaCards organs/tissues related to Empty Sella Syndrome:

41
Pituitary, Brain, Testes, Ovary, Thyroid, Bone, Eye
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Publications for Empty Sella Syndrome

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Articles related to Empty Sella Syndrome:

(show top 50) (show all 657)
# Title Authors PMID Year
1
SAPHO syndrome with adrenal deficiency: a case report. 9 38
20184675 2009
2
[Serum levels of growth hormone and insulin-like growth factor in women with empty sella]. 9 38
9695643 1998
3
TSH and prolactin responses to thyrotropin releasing hormone (TRH) and domperidone in patients with empty sella syndrome. 9 38
8796337 1996
4
Cerebrospinal fluid pressure and prolactin in empty sella syndrome. 9 38
2107018 1990
5
Hypopituitarism other than sellar and parasellar tumors or traumatic brain injury assessed in a tertiary hospital. 38
31372159 2019
6
Sinus Bradycardia as a Rare and Unusual Presentation of Partial Empty Sella Syndrome: A Case Report. 38
31327866 2019
7
Rare empty sella syndrome found after postoperative hypotension and respiratory failure: A case report. 38
30863767 2019
8
Nivolumab-induced hypophysitis leading to hypopituitarism and secondary empty sella syndrome in a patient with non-small cell lung cancer. 38
30850565 2019
9
Osseous Remodeling Technique of the Sella Turcica: A New Surgical Option for Primary Empty Sella Syndrome. 38
30877013 2019
10
[Infertility Due to Hyperprolactinemia in Empty Sella : A Case Report]. 38
31067844 2019
11
A Recurrent Suprapituitary Ependymal Cyst Managed by Endoscopy-Assisted Transsphenoidal Surgery in a Canine: A Case Report. 38
31041316 2019
12
Endonasal Chiasmapexy Using Autologous Cartilage/Bone for Empty Sella Syndrome After Cabergoline Therapy for Prolactinoma. 38
30315973 2019
13
[Comparison and analysis of spontaneous cerebrospinal fluid rhinorrhea with and without empty sella patients]. 38
30716800 2018
14
Surgical Management of Sphenoid Sinus Lateral Recess Cerebrospinal Fluid Leaks: A Single Neurosurgical Center Analysis of Endoscopic Endonasal Minimal Transpterygoid Approach. 38
29981913 2018
15
[Clinical and genetic analysis of Verheij syndrome caused by PUF60 de novo mutation in a Chinese boy and literature review]. 38
30078240 2018
16
Prognosis of Hormonal Deficits in Empty Sella Syndrome Using Neuroimaging. 38
30283536 2018
17
An Interesting Case of Polyuria in a Child. 38
30072839 2018
18
Refractory Hypoglycemia and Seizures as the Initial Presenting Manifestation of Empty Sella Syndrome. 38
30116682 2018
19
Transnasal Transsphenoidal Elevation of Optic Chiasm in Secondary Empty Sella Syndrome Following Prolactinoma Treatment. 38
29421446 2018
20
Primary Empty Sella Syndrome and the Prevalence of Hormonal Dysregulation. 38
29510819 2018
21
Resolution of symptomatic secondary empty sella syndrome following lumbar-peritoneal shunt. 38
29721351 2018
22
Empty sella associated with growth hormone deficiency and polydactyly. 38
29940653 2018
23
A case of Kallmann syndrome associated with a non-functional pituitary microadenoma. 38
29692900 2018
24
New Technique for Chiasmapexy Using Iliac Crest Bone Graft: 2 Cases of Visual Impairment Caused by Empty Sella Syndrome. 38
28843758 2017
25
MRI features of growth hormone deficiency in children with short stature caused by pituitary lesions. 38
28587427 2017
26
Delayed Cerebrospinal Fluid Rhinorrhea After Gamma Knife Radiosurgery with or without Preceding Transsphenoidal Resection for Pituitary Pathology. 38
28089836 2017
27
Macroprolactinemia and Empty Sella Syndrome. 38
29187947 2017
28
Polyglandular Autoimmune Syndrome III with Hypoglycemia and Association with Empty Sella and Hypopituitarism. 38
30787757 2017
29
Update on the evaluation of pediatric idiopathic intracranial hypertension. 38
27585209 2016
30
Primary Empty Sella Syndrome Presenting With Severe Hyponatremia And Minimal Salt Wasting. 38
28712246 2016
31
Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome. 38
27468406 2016
32
Visual Deterioration and Herniation of the Anterior Cerebral Artery: Unusual Presentation of an Empty Sella Syndrome Complicating Decompression of a Rathke Cleft Cyst. 38
26828843 2016
33
LHX4 Gene Alterations: Patient Report and Review of the Literature. 38
27464418 2016
34
Case Report: The Specter of Untreated Congenital Hypothyroidism in Immigrant Families. 38
27244801 2016
35
Psychosis in Secondary Empty Sella Syndrome following a Russell's Viper Bite. 38
27335524 2016
36
Endonasal endoscopic transsphenoidal chiasmapexy using a clival cranial base cranioplasty for visual loss from massive empty sella following macroprolactinoma treatment with bromocriptine: case report. 38
26339851 2016
37
A Case Report of Late Onset Mania Caused by Hyponatremia in a Patient With Empty Sella Syndrome. 38
26871784 2016
38
Idiopathic intracranial hypertension: ongoing clinical challenges and future prospects. 38
26929666 2016
39
Sellar reconstruction without intrasellar packing after endoscopic surgery of pituitary macroadenomas is better than its reputation. 38
27408609 2016
40
Unusual association of turner syndrome and hypopituitarism in a Tunisian family. 38
27140594 2016
41
Idiopathic intracranial hypertension in children: Diagnostic and management approach. 38
28096561 2016
42
Anterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients. 38
28018694 2016
43
[Etiological diagnosis of central diabetes insipidus: about 41 cases]. 38
27642481 2016
44
Extra-Pontine Myelinolysis in a Case of Pan-hypopituitarism Due to Empty Sella Syndrome. 38
27608705 2015
45
A case of hypopituitarism and porphyria cutanea tarda in relation to estrogen therapy in a patient with empty sella syndrome. 38
25572312 2015
46
Incidental findings of skull-base abnormalities in cone-beam computed tomography scans with consultation by maxillofacial radiologists. 38
25533079 2015
47
Symptomatic empty sella syndrome: an unusual manifestation of Erdheim-Chester disease. 38
25810917 2015
48
Empty sella syndrome in a male child with failure to thrive. 38
25878744 2015
49
Effect of primary empty sella syndrome on pituitary surgery for Cushing's disease. 38
24857241 2014
50
Pituitary mass and subsequent involution causing fluctuations of serum follicle-stimulating hormone levels in a Turner syndrome patient with premature ovarian failure: a case report. 38
25330695 2014
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Variations for Empty Sella Syndrome

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Expression for Empty Sella Syndrome

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

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Pathways related to Empty Sella Syndrome according to GeneCards Suite gene sharing:

(show all 12)
# Super pathways Score Top Affiliating Genes
1
Peptide ligand-binding receptors 66
Show member pathways
 12.64 TRH PRL POMC GNRH1 GH1
2
NF-KappaB Family Pathway 64
Show member pathways
 12.23 IGF1 GNRH1 GH1
3
G-protein signaling RAC1 in cellular process 22
Show member pathways
 11.95 IGF1 GNRH1 GH1
4
Immune response IFN alpha/beta signaling pathway 22
Show member pathways
 11.75 IGF1 GNRH1 GH1
5
Immune response IFN gamma signaling pathway 22
Show member pathways
 11.59 IGF1 GNRH1 GH1
6
Glucocorticoid receptor regulatory network 1
11.41 PRL POMC
7
Endochondral Ossification 1
11.27 IGF1 GH1
8
G-protein signaling_RhoA regulation pathway 22
11.12 IGF1 GNRH1 GH1
9
Peptide hormone metabolism 66
Show member pathways
 11.06 POMC IGF1 GH1
10
Growth hormone receptor signaling 66
Show member pathways
 10.93 PRL GH1
11
Development_Leptin signaling via JAK/STAT and MAPK cascades 22
Show member pathways
 10.63 TRH POMC
12
G-protein signaling_Rap2B regulation pathway 22
10.59 IGF1 GNRH1 GH1
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GO Terms for Empty Sella Syndrome

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Cellular components related to Empty Sella Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.65 PRL POMC IGF1 GNRH1 GH1
2 endosome lumen GO:0031904 9.16 PRL GH1
3 extracellular region GO:0005576 9.1 TRH PRL POMC IGF1 GNRH1 GH1
4 secretory granule GO:0030141 8.96 TRH POMC

Biological processes related to Empty Sella Syndrome according to GeneCards Suite gene sharing:

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 female pregnancy GO:0007565 9.54 PRL GNRH1
2 cell-cell signaling GO:0007267 9.54 TRH POMC GNRH1
3 positive regulation of peptidyl-tyrosine phosphorylation GO:0050731 9.52 IGF1 GH1
4 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.51 IGF1 GH1
5 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.49 IGF1 GH1
6 response to nutrient levels GO:0031667 9.46 PRL GH1
7 regulation of multicellular organism growth GO:0040014 9.43 PRL IGF1
8 positive regulation of JAK-STAT cascade GO:0046427 9.4 PRL GH1
9 positive regulation of insulin-like growth factor receptor signaling pathway GO:0043568 9.32 IGF1 GH1
10 JAK-STAT cascade involved in growth hormone signaling pathway GO:0060397 9.26 PRL GH1
11 positive regulation of epithelial cell proliferation GO:0050679 9.18 IGF1
12 response to organic cyclic compound GO:0014070 9.16 TRH GNRH1
13 ovulation cycle GO:0042698 8.65 GNRH1
14 response to ethanol GO:0045471 8.62 TRH GNRH1

Molecular functions related to Empty Sella Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.1 TRH PRL POMC IGF1 GNRH1 GH1
2 prolactin receptor binding GO:0005148 8.96 PRL GH1
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Sources for Empty Sella Syndrome

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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