MCID: HYP835
MIFTS: 44

Hypothalamic Obesity

Categories: Endocrine diseases, Metabolic diseases, Rare diseases

Aliases & Classifications for Hypothalamic Obesity

MalaCards integrated aliases for Hypothalamic Obesity:

Name: Hypothalamic Obesity 54
Babinski-Froelich Syndrome 54
Adiposogenital Dystrophy 54
Froelich's Adiposity 54
Froelich's Syndrome 54
Froehlich Syndrome 54
Adiposodysgenesis 54

Classifications:



Summaries for Hypothalamic Obesity

NIH Rare Diseases : 54 Hypothalamic obesity refers to obesity that is caused by physical or inborn damage to the hypothalamus. The hypothalamus is part of the brain that makes hormones that control specific body functions such as sleep, body temperature, and hunger. It also makes hormones that control other organs in the body, especially the pituitary gland. The symptoms of hypothalamic obesity vary by the cause and include uncontrollable hunger, rapid, excessive weight gain, and a low metabolic rate. If the pituitary gland is involved, symptoms may include small underdeveloped testes in males and delayed puberty. This condition most often occurs because of injury to the hypothalamus due to a tumor, swelling in the brain, brain surgery, or head trauma. The diagnosis is made by physical examination and review of the symptoms. There is no cure for hypothalamic obesity.  Treatment involves a combination of surgery, medications, and nutritional and lifestyle counseling. The long-term outlook for people with this condition is dependent on weight loss and management.

MalaCards based summary : Hypothalamic Obesity, also known as babinski-froelich syndrome, is related to craniopharyngioma and hyperinsulinism. An important gene associated with Hypothalamic Obesity is INS (Insulin), and among its related pathways/superpathways are Prolactin Signaling Pathway and AMP-activated Protein Kinase (AMPK) Signaling. The drugs Octreotide and Antineoplastic Agents, Hormonal have been mentioned in the context of this disorder. Affiliated tissues include hypothalamus, brain and pituitary, and related phenotypes are endocrine/exocrine gland and homeostasis/metabolism

Related Diseases for Hypothalamic Obesity

Diseases related to Hypothalamic Obesity via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 101)
# Related Disease Score Top Affiliating Genes
1 craniopharyngioma 30.9 IGF1 INS
2 hyperinsulinism 30.0 IGF1 INS LEP
3 nonalcoholic fatty liver disease 29.5 INS LEP
4 fatty liver disease, nonalcoholic 1 29.4 INS LEP RETN
5 hypothyroidism 29.3 IGF1 LEP RETN
6 fatty liver disease 29.2 INS LEP LEPR
7 liver disease 29.2 INS LEP LEPR
8 diabetes mellitus 29.1 IGF1 INS LEP RETN
9 body mass index quantitative trait locus 11 28.6 GHRH IGF1 INS LEP LEPR RETN
10 prader-willi syndrome 28.4 IGF1 INS LEP LEPR RETN
11 froelich syndrome 11.5
12 secondary adrenal insufficiency 10.3 IGF1 INS
13 pineal hyperplasia, insulin-resistant diabetes mellitus, and somatic abnormalities 10.2 IGF1 INS
14 sheehan syndrome 10.2 IGF1 INS
15 donohue syndrome 10.2 IGF1 INS
16 body mass index quantitative trait locus 10 10.2
17 body mass index quantitative trait locus 14 10.2
18 body mass index quantitative trait locus 18 10.2
19 body mass index quantitative trait locus 19 10.2
20 rubeosis iridis 10.2 IGF1 INS
21 mammographic density 10.2 HSD17B1 IGF1
22 endocrine organ benign neoplasm 10.2 IGF1 INS
23 isolated growth hormone deficiency, type ib 10.1 GHRH IGF1
24 chromophobe adenoma 10.1 GHRH INS
25 isolated growth hormone deficiency 10.1 GHRH IGF1
26 growth hormone deficiency 10.1 GHRH IGF1
27 carcinoid syndrome 10.1 GHRH IGF1
28 hyperandrogenism 10.1 IGF1 INS
29 abdominal obesity-metabolic syndrome quantitative trait locus 2 10.1 INS LEP
30 lipodystrophy, familial partial, type 1 10.1 INS LEP
31 complete generalized lipodystrophy 10.1 INS LEP
32 pituitary-dependent cushing's disease 10.1 GHRH INS
33 abdominal obesity-metabolic syndrome 1 10.1 INS LEP
34 lipodystrophy, congenital generalized, type 1 10.1 INS LEP
35 lipodystrophy, familial partial, type 2 10.1 INS LEP
36 isolated growth hormone deficiency, type ii 10.1 GHRH IGF1
37 nonalcoholic steatohepatitis 10.1 INS LEP
38 prediabetes syndrome 10.1 INS LEP
39 cystadenoma 10.1
40 apnea, obstructive sleep 10.1 INS LEP
41 familial partial lipodystrophy 10.1 INS LEP
42 obesity-hypoventilation syndrome 10.0 LEP RETN
43 alstrom syndrome 10.0 INS LEP
44 idiopathic recurrent pericarditis 10.0 LEP RETN
45 sleep disorder 10.0 INS LEP
46 protein-energy malnutrition 10.0 IGF1 LEP
47 microvascular complications of diabetes 5 10.0 IGF1 INS
48 diabetic neuropathy 10.0 INS LEP
49 coronary artery aneurysm 10.0 LEP RETN
50 hyperpituitarism 10.0 GHRH IGF1 INS

Graphical network of the top 20 diseases related to Hypothalamic Obesity:



Diseases related to Hypothalamic Obesity

Symptoms & Phenotypes for Hypothalamic Obesity

MGI Mouse Phenotypes related to Hypothalamic Obesity:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.8 GHRH HSD17B1 IGF1 INS LEP LEPR
2 homeostasis/metabolism MP:0005376 9.8 GHRH HSD17B1 IGF1 INS LEP LEPR
3 adipose tissue MP:0005375 9.77 IGF1 INS LEP LEPR RETN
4 immune system MP:0005387 9.63 GHRH IGF1 INS LEP LEPR RETN
5 liver/biliary system MP:0005370 9.35 GHRH INS LEP LEPR RETN
6 reproductive system MP:0005389 9.1 GHRH HSD17B1 IGF1 INS LEP LEPR

Drugs & Therapeutics for Hypothalamic Obesity

Drugs for Hypothalamic Obesity (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 20)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Octreotide Approved, Investigational Phase 4 83150-76-9 6400441 383414
2 Antineoplastic Agents, Hormonal Phase 4
3 Gastrointestinal Agents Phase 4,Phase 3
4
Exenatide Approved, Investigational Phase 3,Phase 2,Phase 1,Not Applicable 141758-74-9 15991534
5
Glucagon Approved Phase 3 16941-32-5
6 Hormones Phase 3,Phase 2,Phase 1,Not Applicable
7 Anti-Obesity Agents Phase 3,Phase 2,Phase 1,Not Applicable
8 Incretins Phase 3,Phase 2,Phase 1,Not Applicable
9 Hypoglycemic Agents Phase 3,Phase 2,Phase 1,Not Applicable
10 Hormone Antagonists Phase 3,Phase 2,Phase 1,Not Applicable
11 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3,Phase 2,Phase 1,Not Applicable
12 Glucagon-Like Peptide 1 Phase 3
13
Diazoxide Approved Phase 2 364-98-7 3019
14
Metformin Approved Phase 2 657-24-9 14219 4091
15
Oxytocin Approved, Vet_approved Phase 2 50-56-6 439302 53477758
16 Vasodilator Agents Phase 2
17 Insulin, Globin Zinc Phase 2
18 insulin Phase 2
19 Antihypertensive Agents Phase 2
20 Oxytocics Phase 2

Interventional clinical trials:

(show all 12)
# Name Status NCT ID Phase Drugs
1 A Study of Octreotide Depot vs Saline Control in Pediatric Hypothalamic Obesity Patients Completed NCT00171613 Phase 4 Octreotide
2 Pediatric Hypothalamic Obesity Completed NCT00076362 Phase 4 Sandostatin LAR
3 Efficacy and Safety of Exenatide in the Treatment of Hypothalamic Obesity After Craniopharyngioma Therapy Completed NCT02860923 Phase 3 Exenatide;Placebo
4 Energy Balance & Weight Loss in Craniopharyngioma-related or Other Hypothalamic Tumors in Hypothalamic Obesity Active, not recruiting NCT02664441 Phase 3 Exenatide;placebo
5 Hypothalamic Obesity Following Craniopharyngioma Surgery: A Pilot Trial of Combined Metformin and Diazoxide Therapy Completed NCT00892073 Phase 2 Diazoxide;Metformin
6 Weight Loss Study for Patients With Obesity Due to Craniopharyngioma or Other Brain Tumor Completed NCT01484873 Phase 2 Exenatide
7 Intranasal Oxytocin in Hypothalamic Obesity Recruiting NCT02849743 Phase 2 Syntocinon;Placebo (for Syntocinon)
8 Effects of Exenatide on Hypothalamic Obesity Active, not recruiting NCT01061775 Phase 1, Phase 2 Exenatide
9 Neuromodulation for Hypothalamic Obesity Withdrawn NCT03708913 Phase 1
10 Effects of Exenatide on Obesity and/or Diabetes Mellitus Due to Hypothalamic Damage Unknown status NCT01783717 Not Applicable Exenatide
11 A Personalized Program of Physical Activity and Diet for Hypothalamic Obesity Completed NCT03673813
12 Evaluation of the SickKids Team Obesity Management Program Recruiting NCT01515904

Search NIH Clinical Center for Hypothalamic Obesity

Genetic Tests for Hypothalamic Obesity

Anatomical Context for Hypothalamic Obesity

MalaCards organs/tissues related to Hypothalamic Obesity:

42
Hypothalamus, Brain, Pituitary, Testes, Liver, Pancreas, Adipocyte

Publications for Hypothalamic Obesity

Articles related to Hypothalamic Obesity:

(show top 50) (show all 188)
# Title Authors Year
1
Hypothalamic Obesity Treatment Demands Thinking Outside the Box. ( 30474263 )
2019
2
Metabolic syndrome is a sequela of radiation exposure in hypothalamic obesity among survivors of childhood brain tumors. ( 30530528 )
2019
3
Treatment of Hypothalamic Obesity with Dextroamphetamine: A Case Series. ( 30844799 )
2019
4
Hypothalamic Obesity: Prologue and Promise. ( 30884480 )
2019
5
The development of hypothalamic obesity in craniopharyngioma patients: A risk factor analysis in a well-defined cohort. ( 29314661 )
2018
6
A Rare Cause of Hypothalamic Obesity, Rohhad Syndrome: 2 Cases. ( 29553042 )
2018
7
Interventions for the treatment of craniopharyngioma-related hypothalamic obesity: a systematic review. ( 29945001 )
2018
8
Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report. ( 29609450 )
2018
9
Pathophysiology and Individualized Treatment for Hypothalamic Obesity Following Craniopharyngioma and Other Suprasellar Tumors: A Systematic Review. ( 30247642 )
2018
10
Oxytocin and Naltrexone Successfully Treat Hypothalamic Obesity in a Boy Post-Craniopharyngioma Resection. ( 29220529 )
2018
11
Metabolic features and changes in glucose-induced serum glucagon-like peptide-1 levels in children with hypothalamic obesity. ( 29389666 )
2018
12
Management of Hypothalamic Obesity during Transition from Childhood to Adulthood. ( 29886502 )
2018
13
Hypothalamic Obesity: 4 Years of the International Registry of Hypothalamic Obesity Disorders. ( 30296362 )
2018
14
Hypothalamic obesity after craniopharyngioma surgery: Treatment with a long acting glucagon like peptide 1 derivated. ( 28440760 )
2017
15
Efficacy and safety of bariatric surgery for craniopharyngioma-related hypothalamic obesity: a matched case-control study with 2 years of follow-up. ( 27795552 )
2017
16
Robust Reductions of Excess Weight and Hyperphagia by Beloranib in Rat Models of Genetic and Hypothalamic Obesity. ( 27849360 )
2017
17
The effectiveness of interventions to treat hypothalamic obesity in survivors of childhood brain tumours: a systematic review. ( 28544764 )
2017
18
For Debate: Should Bariatric Surgery be Performed in Children and Adolescents with Hypothalamic Obesity? ( 28613044 )
2017
19
Risk factors for mortality caused by hypothalamic obesity in children with hypothalamic tumours. ( 26463004 )
2016
20
A 52-week pilot study of the effects of exenatide on body weight in patients with hypothalamic obesity. ( 27133664 )
2016
21
Effects of Physical Exercise on the Intestinal Mucosa of Rats Submitted to a Hypothalamic Obesity Condition. ( 27488821 )
2016
22
Hypothalamic Obesity in Craniopharyngioma Patients: Disturbed Energy Homeostasis Related to Extent of Hypothalamic Damage and Its Implication for Obesity Intervention. ( 26371051 )
2015
23
Survival, hypothalamic obesity, and neuropsychological/psychosocial status after childhood-onset craniopharyngioma: newly reported long-term outcomes. ( 25838139 )
2015
24
Effects of T3 treatment on brown adipose tissue and energy expenditure in a patient with craniopharyngioma and hypothalamic obesity. ( 25514327 )
2015
25
Hypothalamic obesity. ( 25370939 )
2015
26
Hypothalamic obesity in children: pathophysiology to clinical management. ( 25781673 )
2015
27
Effects of paternal hypothalamic obesity and taurine supplementation on adiposity and vascular reactivity in rat offspring. ( 25833542 )
2015
28
Semiquantitative analysis of hypothalamic damage on MRI predicts risk for hypothalamic obesity. ( 25884561 )
2015
29
Plasmodium berghei ANKA infection results in exacerbated immune responses from C57BL/6 mice displaying hypothalamic obesity. ( 26239414 )
2015
30
Hypothalamic obesity after treatment for craniopharyngioma: the importance of the home environment. ( 25381948 )
2014
31
A review of childhood and adolescent craniopharyngiomas with particular attention to hypothalamic obesity. ( 24188907 )
2014
32
Autonomic nervous system balance in children and adolescents with craniopharyngioma and hypothalamic obesity. ( 23482594 )
2013
33
Is bariatric surgery really inefficient in hypothalamic obesity? ( 22994633 )
2013
34
Hypothalamic obesity: prevalence, associations and longitudinal trends in weight in a specialist adult neuroendocrine clinic. ( 23293322 )
2013
35
GLP-1 analogues as a new treatment option for hypothalamic obesity in adults: report of nine cases. ( 23392214 )
2013
36
Effects of CB1 receptor blockade on monosodium glutamate induced hypometabolic and hypothalamic obesity in rats. ( 23620336 )
2013
37
Pathophysiology and clinical characteristics of hypothalamic obesity in children and adolescents. ( 24904871 )
2013
38
Functional Neuroimaging in Craniopharyngioma: A Useful Tool to Better Understand Hypothalamic Obesity? ( 22647305 )
2012
39
Glucagon-like peptide-1 agonist exendin-4 leads to reduction of weight and caloric intake in a rat model of hypothalamic obesity. ( 22831885 )
2012
40
Hypothalamic obesity syndrome: rare presentation of CNS+ B-cell lymphoblastic lymphoma. ( 22213612 )
2012
41
Hypothalamic obesity in patients with craniopharyngioma: treatment approaches and the emerging role of gastric bypass surgery. ( 21935755 )
2012
42
Decreased TNF-α gene expression in periodontal ligature in MSG-obese rats: a possible protective effect of hypothalamic obesity against periodontal disease? ( 22129469 )
2012
43
A glucagon-like peptide-1 (GLP-1) receptor agonist in the treatment for hypothalamic obesity complicated by type 2 diabetes mellitus. ( 22356092 )
2012
44
Hypothalamic obesity in children. ( 22577758 )
2012
45
Bariatric surgery in hypothalamic obesity. ( 22649412 )
2012
46
Treatment with glucagon-like Peptide-1 agonist exendin-4 in a patient with hypothalamic obesity secondary to intracranial tumor. ( 22831918 )
2012
47
Hypothalamic Obesity following Craniopharyngioma Surgery: Results of a Pilot Trial of Combined Diazoxide and Metformin Therapy. ( 21603206 )
2011
48
Hypothalamic obesity in patients with craniopharyngioma: profound changes of several weight regulatory circuits. ( 22654811 )
2011
49
Hypothalamic obesity after craniopharyngioma: mechanisms, diagnosis, and treatment. ( 22654817 )
2011
50
Primary hypothyroidism, precocious puberty and hypothalamic obesity in Langerhans cell histiocytosis. ( 21038089 )
2011

Variations for Hypothalamic Obesity

Expression for Hypothalamic Obesity

Search GEO for disease gene expression data for Hypothalamic Obesity.

Pathways for Hypothalamic Obesity

GO Terms for Hypothalamic Obesity

Cellular components related to Hypothalamic Obesity according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.35 GHRH IGF1 INS LEP RETN
2 extracellular region GO:0005576 9.1 GHRH IGF1 INS LEP LEPR RETN

Biological processes related to Hypothalamic Obesity according to GeneCards Suite gene sharing:

(show all 27)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.85 GHRH IGF1 INS LEP
2 positive regulation of MAPK cascade GO:0043410 9.67 IGF1 INS LEP
3 response to insulin GO:0032868 9.63 LEP RETN
4 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.63 IGF1 LEP
5 phagocytosis GO:0006909 9.62 LEP LEPR
6 glucose metabolic process GO:0006006 9.62 INS LEP
7 negative regulation of autophagy GO:0010507 9.61 LEP LEPR
8 T cell differentiation GO:0030217 9.61 LEP LEPR
9 glucose homeostasis GO:0042593 9.61 INS LEP LEPR
10 activation of protein kinase B activity GO:0032148 9.6 IGF1 INS
11 positive regulation of glucose import GO:0046326 9.59 IGF1 INS
12 positive regulation of mitotic nuclear division GO:0045840 9.58 IGF1 INS
13 positive regulation of glycolytic process GO:0045821 9.58 IGF1 INS
14 positive regulation of glycogen biosynthetic process GO:0045725 9.57 IGF1 INS
15 positive regulation of insulin receptor signaling pathway GO:0046628 9.56 INS LEP
16 energy reserve metabolic process GO:0006112 9.54 LEP LEPR
17 negative regulation of gluconeogenesis GO:0045721 9.52 INS LEPR
18 positive regulation of insulin-like growth factor receptor signaling pathway GO:0043568 9.51 GHRH IGF1
19 response to leptin GO:0044321 9.48 GHRH LEPR
20 sexual reproduction GO:0019953 9.46 LEP LEPR
21 leptin-mediated signaling pathway GO:0033210 9.4 LEP LEPR
22 negative regulation of feeding behavior GO:2000252 9.37 INS RETN
23 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.33 IGF1 INS LEP
24 regulation of bone remodeling GO:0046850 9.32 LEP LEPR
25 bone mineralization involved in bone maturation GO:0035630 9.26 IGF1 LEP
26 regulation of signaling receptor activity GO:0010469 9.02 GHRH IGF1 INS LEP RETN
27 bone growth GO:0098868 8.96 LEP LEPR

Molecular functions related to Hypothalamic Obesity according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 insulin receptor binding GO:0005158 9.26 IGF1 INS
2 insulin-like growth factor receptor binding GO:0005159 9.16 IGF1 INS
3 hormone activity GO:0005179 9.02 GHRH IGF1 INS LEP RETN
4 peptide hormone receptor binding GO:0051428 8.96 GHRH LEP

Sources for Hypothalamic Obesity

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....