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Premature adrenarche

MedGen UID:
137972
Concept ID:
C0342546
Disease or Syndrome
Synonym: Premature Adrenarche
SNOMED CT: Premature adrenarche (103021001)
 
HPO: HP:0012412

Definition

Onset of adrenarche at an earlier age than usual. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPremature adrenarche

Conditions with this feature

Hao-Fountain syndrome
MedGen UID:
1719035
Concept ID:
C5393908
Disease or Syndrome
Hao-Fountain syndrome (HAFOUS) is a neurodevelopmental disorder characterized by global developmental delay, variably impaired intellectual development with significant speech delay, behavioral abnormalities, such as autism, and mild dysmorphic facies. Additional features are variable, but may include hypotonia, feeding problems, delayed walking with unsteady gait, hypogonadism in males, and ocular anomalies, such as strabismus. Some patients develop seizures and some have mild white matter abnormalities on brain imaging (summary by Fountain et al., 2019).
Intellectual developmental disorder, autosomal dominant 73
MedGen UID:
1841272
Concept ID:
C5830636
Mental or Behavioral Dysfunction
Autosomal dominant intellectual developmental disorder-73 (MRD73) is a highly variable neurodevelopmental disorder characterized by impaired intellectual development that ranges from mild to severe, speech delay, behavioral abnormalities, and nonspecific dysmorphic facial features (Janssen et al., 2022).

Professional guidelines

PubMed

Minnetti M, Caiulo S, Ferrigno R, Baldini-Ferroli B, Bottaro G, Gianfrilli D, Sbardella E, De Martino MC, Savage MO
Clin Endocrinol (Oxf) 2020 Feb;92(2):98-108. Epub 2019 Dec 5 doi: 10.1111/cen.14131. PMID: 31747461
Wakeling EL, Brioude F, Lokulo-Sodipe O, O'Connell SM, Salem J, Bliek J, Canton AP, Chrzanowska KH, Davies JH, Dias RP, Dubern B, Elbracht M, Giabicani E, Grimberg A, Grønskov K, Hokken-Koelega AC, Jorge AA, Kagami M, Linglart A, Maghnie M, Mohnike K, Monk D, Moore GE, Murray PG, Ogata T, Petit IO, Russo S, Said E, Toumba M, Tümer Z, Binder G, Eggermann T, Harbison MD, Temple IK, Mackay DJ, Netchine I
Nat Rev Endocrinol 2017 Feb;13(2):105-124. Epub 2016 Sep 2 doi: 10.1038/nrendo.2016.138. PMID: 27585961
Rosenbloom AL
Paediatr Drugs 2002;4(4):209-21. doi: 10.2165/00128072-200204040-00001. PMID: 11960510

Recent clinical studies

Etiology

Fraga NR, Minaeian N, Kim MS
Pediatr Rev 2024 Feb 1;45(2):74-84. doi: 10.1542/pir.2022-005617. PMID: 38296783
Augsburger P, Liimatta J, Flück CE
J Clin Endocrinol Metab 2024 May 17;109(6):1403-1422. doi: 10.1210/clinem/dgae008. PMID: 38181424
Rosenfield RL
Endocr Rev 2021 Nov 16;42(6):783-814. doi: 10.1210/endrev/bnab009. PMID: 33788946Free PMC Article
Wakeling EL, Brioude F, Lokulo-Sodipe O, O'Connell SM, Salem J, Bliek J, Canton AP, Chrzanowska KH, Davies JH, Dias RP, Dubern B, Elbracht M, Giabicani E, Grimberg A, Grønskov K, Hokken-Koelega AC, Jorge AA, Kagami M, Linglart A, Maghnie M, Mohnike K, Monk D, Moore GE, Murray PG, Ogata T, Petit IO, Russo S, Said E, Toumba M, Tümer Z, Binder G, Eggermann T, Harbison MD, Temple IK, Mackay DJ, Netchine I
Nat Rev Endocrinol 2017 Feb;13(2):105-124. Epub 2016 Sep 2 doi: 10.1038/nrendo.2016.138. PMID: 27585961
Williams RM, Ward CE, Hughes IA
Arch Dis Child 2012 Mar;97(3):250-4. Epub 2011 Aug 11 doi: 10.1136/archdischild-2011-300011. PMID: 21835833

Diagnosis

Rosenfield RL
Endocr Rev 2021 Nov 16;42(6):783-814. doi: 10.1210/endrev/bnab009. PMID: 33788946Free PMC Article
Witchel SF, Pinto B, Burghard AC, Oberfield SE
Curr Opin Pediatr 2020 Aug;32(4):574-581. doi: 10.1097/MOP.0000000000000928. PMID: 32692055Free PMC Article
Novello L, Speiser PW
Pediatr Ann 2018 Jan 1;47(1):e7-e11. doi: 10.3928/19382359-20171214-04. PMID: 29323690
Witchel SF
J Pediatr Adolesc Gynecol 2017 Oct;30(5):520-534. Epub 2017 Apr 24 doi: 10.1016/j.jpag.2017.04.001. PMID: 28450075Free PMC Article
Wakeling EL, Brioude F, Lokulo-Sodipe O, O'Connell SM, Salem J, Bliek J, Canton AP, Chrzanowska KH, Davies JH, Dias RP, Dubern B, Elbracht M, Giabicani E, Grimberg A, Grønskov K, Hokken-Koelega AC, Jorge AA, Kagami M, Linglart A, Maghnie M, Mohnike K, Monk D, Moore GE, Murray PG, Ogata T, Petit IO, Russo S, Said E, Toumba M, Tümer Z, Binder G, Eggermann T, Harbison MD, Temple IK, Mackay DJ, Netchine I
Nat Rev Endocrinol 2017 Feb;13(2):105-124. Epub 2016 Sep 2 doi: 10.1038/nrendo.2016.138. PMID: 27585961

Therapy

Fraga NR, Minaeian N, Kim MS
Pediatr Rev 2024 Feb 1;45(2):74-84. doi: 10.1542/pir.2022-005617. PMID: 38296783
Binder G, Schweizer R, Blumenstock G, Ferrand N
J Clin Endocrinol Metab 2017 Nov 1;102(11):4100-4108. doi: 10.1210/jc.2017-00874. PMID: 28945864
Çelik N, Alp H, Çamtosun E, Alp E, Çelik S, Berk E
Horm Res Paediatr 2017;87(1):7-14. Epub 2016 Nov 22 doi: 10.1159/000452445. PMID: 27871080
Wakeling EL, Brioude F, Lokulo-Sodipe O, O'Connell SM, Salem J, Bliek J, Canton AP, Chrzanowska KH, Davies JH, Dias RP, Dubern B, Elbracht M, Giabicani E, Grimberg A, Grønskov K, Hokken-Koelega AC, Jorge AA, Kagami M, Linglart A, Maghnie M, Mohnike K, Monk D, Moore GE, Murray PG, Ogata T, Petit IO, Russo S, Said E, Toumba M, Tümer Z, Binder G, Eggermann T, Harbison MD, Temple IK, Mackay DJ, Netchine I
Nat Rev Endocrinol 2017 Feb;13(2):105-124. Epub 2016 Sep 2 doi: 10.1038/nrendo.2016.138. PMID: 27585961
Bridges NA, Cooke A, Healy MJ, Hindmarsh PC, Brook CG
Clin Endocrinol (Oxf) 1995 Feb;42(2):135-40. doi: 10.1111/j.1365-2265.1995.tb01853.x. PMID: 7704957

Prognosis

Topaktaş E, Erolu E, Dursun F, Kırmızıbekmez H
J Pediatr Endocrinol Metab 2021 Aug 26;34(8):1009-1015. Epub 2021 Jun 24 doi: 10.1515/jpem-2021-0160. PMID: 34167179
Santos-Silva R, Costa C, Castro-Correia C, Fontoura M
J Pediatr Endocrinol Metab 2019 Nov 26;32(11):1247-1252. doi: 10.1515/jpem-2019-0185. PMID: 31472065
Novello L, Speiser PW
Pediatr Ann 2018 Jan 1;47(1):e7-e11. doi: 10.3928/19382359-20171214-04. PMID: 29323690
Binder G, Schweizer R, Blumenstock G, Ferrand N
J Clin Endocrinol Metab 2017 Nov 1;102(11):4100-4108. doi: 10.1210/jc.2017-00874. PMID: 28945864
Çelik N, Alp H, Çamtosun E, Alp E, Çelik S, Berk E
Horm Res Paediatr 2017;87(1):7-14. Epub 2016 Nov 22 doi: 10.1159/000452445. PMID: 27871080

Clinical prediction guides

Witchel SF, Azziz R, Oberfield SE
Horm Res Paediatr 2022;95(6):557-567. Epub 2022 Nov 29 doi: 10.1159/000526722. PMID: 36446329
Tauber M, Hoybye C
Lancet Diabetes Endocrinol 2021 Apr;9(4):235-246. Epub 2021 Feb 26 doi: 10.1016/S2213-8587(21)00002-4. PMID: 33647242
Akın O, Bideci A, Döğer E, Demet Akbaş E, Kilinç Uğurlu A, Yavuz ST, Elbeğ Ş, Çamurdan O, Cinaz P
Pediatr Int 2018 Oct;60(10):938-942. Epub 2018 Oct 8 doi: 10.1111/ped.13682. PMID: 30129969
Novello L, Speiser PW
Pediatr Ann 2018 Jan 1;47(1):e7-e11. doi: 10.3928/19382359-20171214-04. PMID: 29323690
Leung AK, Robson WL
J Pediatr Health Care 2008 Jul-Aug;22(4):230-3. Epub 2008 Mar 5 doi: 10.1016/j.pedhc.2007.07.002. PMID: 18590867

Recent systematic reviews

Jiang M, Gao Y, Qu T, Ji Y, Niu Y, Zhang J, Huang L
J Ovarian Res 2023 Nov 23;16(1):227. doi: 10.1186/s13048-023-01302-2. PMID: 37996919Free PMC Article

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