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MCID: EMP001
MIFTS: 44

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 74 52 53 43 15 17 71
Empty Sella Turcica 52
Empty Sella 54

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 43 D004652
NCIt 49 C84686
UMLS 71 C0014008
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Summaries for Empty Sella Syndrome

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NINDS : 53 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 hypogonadotropism and lymphocytic hypophysitis. An important gene associated with Empty Sella Syndrome is PRL (Prolactin), and among its related pathways/superpathways are Peptide ligand-binding receptors and Regulation of lipid metabolism Insulin signaling-generic cascades. The drugs Acetaminophen and Acetazolamide have been mentioned in the context of this disorder. Affiliated tissues include pituitary, brain and thyroid, and related phenotypes are endocrine/exocrine gland and growth/size/body region

NIH Rare Diseases : 52 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 : 74 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 404)
# Related Disease Score Top Affiliating Genes
1 hypogonadotropism 31.2 PRL GNRH1
2 lymphocytic hypophysitis 31.1 POMC GH1
3 precocious puberty 31.0 GNRH1 GH1
4 galactorrhea 30.9 PRL IGF1
5 diabetes insipidus 30.7 PRL POMC INS GH1
6 adenoma 30.6 PRL POMC IGF1 GH1
7 hypoadrenalism 30.5 POMC GH1
8 intracranial hypertension, idiopathic 30.5 POMC IGF1 GH1
9 acth-secreting pituitary adenoma 30.4 PRL POMC
10 growth hormone secreting pituitary adenoma 30.3 PRL POMC IGF1
11 pituitary hypoplasia 30.3 PRL LHX3 GH1
12 thyroiditis 30.3 TRH PRL INS
13 chromophobe adenoma 30.1 TRH PRL POMC GH1
14 traumatic brain injury 30.0 IGF1 GH1
15 central precocious puberty 30.0 IGF1 GNRH1 GH1
16 multiple endocrine neoplasia, type i 30.0 PRL POMC INS
17 pseudohypoparathyroidism 29.9 TRH PRL IGF1
18 fibrous dysplasia 29.9 PRL IGF1 GH1
19 meningioma, familial 29.8 PRL POMC IGF1
20 migraine with or without aura 1 29.8 PRL POMC NPY INS
21 pituitary adenoma 29.7 TRH PRL POMC IGF1 GNRH1 GH1
22 impotence 29.7 PRL POMC INS GNRH1
23 primary hyperparathyroidism 29.7 PRL POMC INS IGF1
24 islet cell tumor 29.7 INS IGF1 GH1
25 hypothyroidism, congenital, nongoitrous, 4 29.7 PROP1 PRL POMC GH1
26 hydrocephalus 29.6 PRL NPY IGFBP3 IGF1
27 secondary adrenal insufficiency 29.6 INS IGFBP3 IGF1
28 goiter 29.6 TRH PRL INS IGF1
29 microvascular complications of diabetes 1 29.6 INS IGFBP3 IGF1
30 pituitary adenoma, prolactin-secreting 29.5 TRH PRL POMC IGF1 GNRH1 GH1
31 sheehan syndrome 29.5 PRL POMC INS IGF1
32 pituitary tumors 29.4 PROP1 PRL POMC IGF1 GH1
33 hypogonadotropic hypogonadism 29.3 PROP1 PRL INS GNRH1
34 kallmann syndrome 29.3 PROP1 PRL LHX3 GNRH1
35 hypoglycemia 29.3 PRL POMC INS IGFBP3 IGF1 GH1
36 pituitary apoplexy 29.2 PRL POMC INS IGF1 GNRH1 GH1
37 turner syndrome 29.2 PRL INS IGFBP3 IGF1 GH1
38 isolated growth hormone deficiency, type ii 29.2 PROP1 IGF1 GH1
39 hyperprolactinemia 29.2 TRH PRL POMC INS IGF1 GNRH1
40 anovulation 29.2 PRL INS IGFBP3 IGF1 GNRH1
41 insulin-like growth factor i 29.1 PRL INS IGFBP3 IGF1 GH1
42 acth deficiency, isolated 29.1 TRH PROP1 PRL POMC LHX3
43 hypogonadism 28.9 PROP1 PRL INS IGF1 GNRH1 GH1
44 amenorrhea 28.8 TRH PRL POMC NPY INS IGF1
45 conn's syndrome 28.8 TRH PRL POMC IGF1 GNRH1 GH1
46 hyperthyroidism 28.7 TRH PRL POMC INS IGF1 GH1
47 congenital hypopituitarism 28.7 PROP1 PRL LHX3 IGF1
48 osteoporosis 28.7 PRL INS IGFBP3 IGF1 GNRH1 GH1
49 pituitary infarct 28.7 TRH PRL POMC INS IGF1 GNRH1
50 isolated growth hormone deficiency 28.6 PROP1 POMC LHX3 IGF1 GH1

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:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 10.06 GNRH1 IGF1 IGFBP3 INS LHX3 POMC
2 growth/size/body region MP:0005378 10.02 GNRH1 IGF1 IGFBP3 INS LHX3 NPY
3 homeostasis/metabolism MP:0005376 10.02 GNRH1 IGF1 IGFBP3 INS LHX3 NPY
4 adipose tissue MP:0005375 9.83 IGF1 IGFBP3 INS NPY POMC
5 integument MP:0010771 9.73 GNRH1 IGF1 IGFBP3 INS POMC PRL
6 liver/biliary system MP:0005370 9.63 GNRH1 IGFBP3 INS NPY POMC PRL
7 nervous system MP:0003631 9.61 GNRH1 IGF1 INS LHX3 NPY POMC
8 renal/urinary system MP:0005367 9.1 GNRH1 IGF1 INS NPY POMC TRH
Sources

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

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:

40
Pituitary, Brain, Thyroid, Ovary, Testes, Bone, Lung
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Publications for Empty Sella Syndrome

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Articles related to Empty Sella Syndrome:

(show top 50) (show all 661)
# Title Authors PMID Year
1
SAPHO syndrome with adrenal deficiency: a case report. 54 61
20184675 2009
2
[Serum levels of growth hormone and insulin-like growth factor in women with empty sella]. 54 61
9695643 1998
3
TSH and prolactin responses to thyrotropin releasing hormone (TRH) and domperidone in patients with empty sella syndrome. 54 61
8796337 1996
4
Cerebrospinal fluid pressure and prolactin in empty sella syndrome. 54 61
2107018 1990
5
Bitemporal Hemianopsia Associated With Empty Sella Syndrome. 61
31449212 2019
6
[Ectopic pituitary adenoma associated with empty sella turcica]. 61
31530410 2019
7
Clinicoradiological Discrepancy in Multisystem Langerhans Cell Histiocytosis with Central Nervous System Involvement. 61
31591994 2019
8
Sinus Bradycardia as a Rare and Unusual Presentation of Partial Empty Sella Syndrome: A Case Report. 61
31327866 2019
9
Hypopituitarism other than sellar and parasellar tumors or traumatic brain injury assessed in a tertiary hospital. 61
31372159 2019
10
Osseous Remodeling Technique of the Sella Turcica: A New Surgical Option for Primary Empty Sella Syndrome. 61
30877013 2019
11
Rare empty sella syndrome found after postoperative hypotension and respiratory failure: A case report. 61
30863767 2019
12
Nivolumab-induced hypophysitis leading to hypopituitarism and secondary empty sella syndrome in a patient with non-small cell lung cancer. 61
30850565 2019
13
[Infertility Due to Hyperprolactinemia in Empty Sella : A Case Report]. 61
31067844 2019
14
A Recurrent Suprapituitary Ependymal Cyst Managed by Endoscopy-Assisted Transsphenoidal Surgery in a Canine: A Case Report. 61
31041316 2019
15
[Empty sella syndrome: a case report]. 61
31692756 2019
16
Endonasal Chiasmapexy Using Autologous Cartilage/Bone for Empty Sella Syndrome After Cabergoline Therapy for Prolactinoma. 61
30315973 2019
17
[Comparison and analysis of spontaneous cerebrospinal fluid rhinorrhea with and without empty sella patients]. 61
30716800 2018
18
Surgical Management of Sphenoid Sinus Lateral Recess Cerebrospinal Fluid Leaks: A Single Neurosurgical Center Analysis of Endoscopic Endonasal Minimal Transpterygoid Approach. 61
29981913 2018
19
[Clinical and genetic analysis of Verheij syndrome caused by PUF60 de novo mutation in a Chinese boy and literature review]. 61
30078240 2018
20
Prognosis of Hormonal Deficits in Empty Sella Syndrome Using Neuroimaging. 61
30283536 2018
21
An Interesting Case of Polyuria in a Child. 61
30072839 2018
22
Refractory Hypoglycemia and Seizures as the Initial Presenting Manifestation of Empty Sella Syndrome. 61
30116682 2018
23
Transnasal Transsphenoidal Elevation of Optic Chiasm in Secondary Empty Sella Syndrome Following Prolactinoma Treatment. 61
29421446 2018
24
Primary Empty Sella Syndrome and the Prevalence of Hormonal Dysregulation. 61
29510819 2018
25
Resolution of symptomatic secondary empty sella syndrome following lumbar-peritoneal shunt. 61
29721351 2018
26
Empty sella associated with growth hormone deficiency and polydactyly. 61
29940653 2018
27
A case of Kallmann syndrome associated with a non-functional pituitary microadenoma. 61
29692900 2018
28
New Technique for Chiasmapexy Using Iliac Crest Bone Graft: 2 Cases of Visual Impairment Caused by Empty Sella Syndrome. 61
28843758 2017
29
MRI features of growth hormone deficiency in children with short stature caused by pituitary lesions. 61
28587427 2017
30
Delayed Cerebrospinal Fluid Rhinorrhea After Gamma Knife Radiosurgery with or without Preceding Transsphenoidal Resection for Pituitary Pathology. 61
28089836 2017
31
Macroprolactinemia and Empty Sella Syndrome. 61
29187947 2017
32
Polyglandular Autoimmune Syndrome III with Hypoglycemia and Association with Empty Sella and Hypopituitarism. 61
30787757 2017
33
Update on the evaluation of pediatric idiopathic intracranial hypertension. 61
27585209 2016
34
Ectopic Acromegaly Arising from a Pituitary Adenoma within the Bony Intersphenoid Septum of a Patient with Empty Sella Syndrome. 61
27468406 2016
35
Primary Empty Sella Syndrome Presenting With Severe Hyponatremia And Minimal Salt Wasting. 61
28712246 2016
36
LHX4 Gene Alterations: Patient Report and Review of the Literature. 61
27464418 2016
37
Visual Deterioration and Herniation of the Anterior Cerebral Artery: Unusual Presentation of an Empty Sella Syndrome Complicating Decompression of a Rathke Cleft Cyst. 61
26828843 2016
38
Psychosis in Secondary Empty Sella Syndrome following a Russell's Viper Bite. 61
27335524 2016
39
Case Report: The Specter of Untreated Congenital Hypothyroidism in Immigrant Families. 61
27244801 2016
40
Endonasal endoscopic transsphenoidal chiasmapexy using a clival cranial base cranioplasty for visual loss from massive empty sella following macroprolactinoma treatment with bromocriptine: case report. 61
26339851 2016
41
A Case Report of Late Onset Mania Caused by Hyponatremia in a Patient With Empty Sella Syndrome. 61
26871784 2016
42
Idiopathic intracranial hypertension: ongoing clinical challenges and future prospects. 61
26929666 2016
43
Sellar reconstruction without intrasellar packing after endoscopic surgery of pituitary macroadenomas is better than its reputation. 61
27408609 2016
44
Idiopathic intracranial hypertension in children: Diagnostic and management approach. 61
28096561 2016
45
Unusual association of turner syndrome and hypopituitarism in a Tunisian family. 61
27140594 2016
46
Anterior Hypopituitarism and Treatment Response in Hunter Syndrome: A Comparison of Two Patients. 61
28018694 2016
47
[Etiological diagnosis of central diabetes insipidus: about 41 cases]. 61
27642481 2016
48
Extra-Pontine Myelinolysis in a Case of Pan-hypopituitarism Due to Empty Sella Syndrome. 61
27608705 2015
49
A case of hypopituitarism and porphyria cutanea tarda in relation to estrogen therapy in a patient with empty sella syndrome. 61
25572312 2015
50
Symptomatic empty sella syndrome: an unusual manifestation of Erdheim-Chester disease. 61
25810917 2015
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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:

# Super pathways Score Top Affiliating Genes
1
Peptide ligand-binding receptors 65
Show member pathways
 13.16 TRH PRL POMC NPY GNRH1 GH1
2
Regulation of lipid metabolism Insulin signaling-generic cascades 22
Show member pathways
 12.53 INS IGFBP3 IGF1 GH1
3
Relaxin signaling pathway 36
Show member pathways
 12.15 POMC IGFBP3 IGF1 GH1
4
Adipogenesis 1
11.62 INS IGF1 GH1
5
Peptide hormone metabolism 65
Show member pathways
 11.52 POMC INS IGF1 GH1
6
Senescence and Autophagy in Cancer 1
11.36 INS IGFBP3 IGF1
7
Development_Leptin signaling via JAK/STAT and MAPK cascades 22
Show member pathways
 10.91 TRH POMC
8
Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs) 65
10.69 IGFBP3 IGF1
9
Antipsychotics Pathway (Metabolic Side Effects), Pharmacodynamics 60
10.23 PRL POMC NPY INS
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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 9.76 PRL POMC NPY INS IGFBP3 IGF1
2 insulin-like growth factor ternary complex GO:0042567 9.32 IGFBP3 IGF1
3 extracellular region GO:0005576 9.28 TRH PRL POMC NPY INS IGFBP3
4 insulin-like growth factor binding protein complex GO:0016942 9.26 IGFBP3 IGF1
5 endosome lumen GO:0031904 9.13 PRL INS GH1

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

(show all 17)
# Name GO ID Score Top Affiliating Genes
1 G protein-coupled receptor signaling pathway GO:0007186 9.96 TRH POMC NPY INS GNRH1
2 signal transduction GO:0007165 9.87 TRH PRL POMC INS IGF1 GNRH1
3 negative regulation of apoptotic process GO:0043066 9.81 PROP1 LHX3 IGF1 GNRH1
4 positive regulation of MAPK cascade GO:0043410 9.58 INS IGFBP3 IGF1
5 activation of protein kinase B activity GO:0032148 9.57 INS IGF1
6 positive regulation of JAK-STAT cascade GO:0046427 9.55 PRL GH1
7 pituitary gland development GO:0021983 9.54 PROP1 LHX3
8 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.54 INS IGF1 GH1
9 positive regulation of mitotic nuclear division GO:0045840 9.52 INS IGF1
10 regulation of multicellular organism growth GO:0040014 9.49 PRL IGF1
11 positive regulation of glycolytic process GO:0045821 9.48 INS IGF1
12 positive regulation of glycogen biosynthetic process GO:0045725 9.46 INS IGF1
13 cellular protein metabolic process GO:0044267 9.46 PRL INS IGFBP3 IGF1
14 JAK-STAT cascade involved in growth hormone signaling pathway GO:0060397 9.43 PRL GH1
15 negative regulation of feeding behavior GO:2000252 9.26 TRH INS
16 cell-cell signaling GO:0007267 9.26 TRH POMC INS GNRH1
17 positive regulation of insulin-like growth factor receptor signaling pathway GO:0043568 8.8 IGFBP3 IGF1 GH1

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

# Name GO ID Score Top Affiliating Genes
1 insulin receptor binding GO:0005158 9.26 INS IGF1
2 hormone activity GO:0005179 9.23 TRH PRL POMC NPY INS IGF1
3 insulin-like growth factor receptor binding GO:0005159 9.16 INS IGF1
4 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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 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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