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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 due to USP7 mutation
MedGen UID:
1853151
•Concept ID:
C5816734
•
Disease or Syndrome
See: Condition Record
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).
See: Condition Record
Hao-Fountain syndrome due to USP7 mutation
Intellectual developmental disorder, autosomal dominant 73

Professional guidelines

PubMed

Abnormal linear growth in paediatric adrenal diseases: Pathogenesis, prevalence and management.
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
Diagnosis and management of Silver-Russell syndrome: first international consensus statement.
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
Increasing incidence of type 2 diabetes in children and adolescents: treatment considerations.
Rosenbloom AL
Paediatr Drugs 2002;4(4):209-21. doi: 10.2165/00128072-200204040-00001. PMID: 11960510

Recent clinical studies

Therapy

Congenital Adrenal Hyperplasia.
Fraga NR, Minaeian N, Kim MS
Pediatr Rev 2024 Feb 1;45(2):74-84. doi: 10.1542/pir.2022-005617. PMID: 38296783
Adrenarche in Silver-Russell Syndrome: Timing and Consequences.
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
The Association between Premature Adrenarche and Cardiovascular Risk May Be Greater than Expected.
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
Diagnosis and management of Silver-Russell syndrome: first international consensus statement.
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
Pathophysiology, genetics, and treatment of hyperandrogenism.
Miller WL
Pediatr Clin North Am 1997 Apr;44(2):375-95. doi: 10.1016/s0031-3955(05)70482-x. PMID: 9130926

Prognosis

Evaluation of metabolic parameters and aortic elasticity in normotensive children with premature adrenarche.
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
Premature Adrenarche.
Novello L, Speiser PW
Pediatr Ann 2018 Jan 1;47(1):e7-e11. doi: 10.3928/19382359-20171214-04. PMID: 29323690
Adrenarche in Silver-Russell Syndrome: Timing and Consequences.
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
The Association between Premature Adrenarche and Cardiovascular Risk May Be Greater than Expected.
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
Isosexual precocious puberty.
Shulman DI
Pediatrician 1987;14(4):261-9. PMID: 3331207

Clinical prediction guides

The Association of Accelerated Early Growth, Timing of Puberty, and Metabolic Consequences in Children.
Jee YH, Jumani S, Mericq V
J Clin Endocrinol Metab 2023 Aug 18;108(9):e663-e670. doi: 10.1210/clinem/dgad202. PMID: 37029976Free PMC Article
History of Polycystic Ovary Syndrome, Premature Adrenarche, and Hyperandrogenism in Pediatric Endocrinology.
Witchel SF, Azziz R, Oberfield SE
Horm Res Paediatr 2022;95(6):557-567. Epub 2022 Nov 29 doi: 10.1159/000526722. PMID: 36446329
Endocrine disorders in Prader-Willi syndrome: a model to understand and treat hypothalamic dysfunction.
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
Premature Adrenarche.
Novello L, Speiser PW
Pediatr Ann 2018 Jan 1;47(1):e7-e11. doi: 10.3928/19382359-20171214-04. PMID: 29323690
Premature adrenarche.
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

Dose inhibin B or anti-Müllerian hormone relate to precocious puberty in girls? result of a systematic review and meta-analysis.
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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