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Delayed adrenarche

MedGen UID:
699438
•Concept ID:
C1276238
•
Disease or Syndrome
SNOMED CT: Delayed adrenarche (124748007)
 
HPO: HP:0025453

Definition

Occurrence of adrenarche at a later than normal age. Adrenarche normally occurs between six and eight years of age with increased adrenal androgen secretion; its exact biologic role is not well understood. It is accompanied by changes in pilosebaceous units, a transient growth spurt and the appearance of axillary and pubic hair in some children, but no sexual development. [from HPO]

Term Hierarchy

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

Professional guidelines

PubMed

Cohort Expansion and Genotype-Phenotype Analysis of RAB11A-Associated Neurodevelopmental Disorder.
Borroto MC, Patel H, Srivastava S, Swanson LC, Keren B, Whalen S, Mignot C, Wang X, Chen Q, Rosenfeld JA, McLean S, Littlejohn RO; Undiagnosed Diseases Network, Emrick L, Burrage LC, Attali R, Lesca G, Acquaviva-Bourdain C, Sarret C, Seaver LH, Platzer K, Bartolomaeus T, Wünsch C, Fischer S, Rodriguez Barreto AM, Granadillo JL, Schreiner E, Brunet T, Schatz UA, Thiffault I, Mullegama SV, Michaud JL, Hamdan FF, Rossignol E, Campeau PM
Pediatr Neurol 2024 Nov;160:45-53. Epub 2024 Jul 20 doi: 10.1016/j.pediatrneurol.2024.07.010. PMID: 39181022
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
IGSF1 Deficiency: Lessons From an Extensive Case Series and Recommendations for Clinical Management.
Joustra SD, Heinen CA, Schoenmakers N, Bonomi M, Ballieux BE, Turgeon MO, Bernard DJ, Fliers E, van Trotsenburg AS, Losekoot M, Persani L, Wit JM, Biermasz NR, Pereira AM, Oostdijk W; IGSF1 Clinical Care Group
J Clin Endocrinol Metab 2016 Apr;101(4):1627-36. Epub 2016 Feb 3 doi: 10.1210/jc.2015-3880. PMID: 26840047Free PMC Article

Recent clinical studies

Etiology

Delayed Adrenarche may be an Additional Feature of Immunoglobulin Super Family Member 1 Deficiency Syndrome.
Van Hulle S, Craen M, Callewaert B, Joustra S, Oostdijk W, Losekoot M, Wit JM, Turgeon MO, Bernard DJ, De Schepper J
J Clin Res Pediatr Endocrinol 2016 Mar 5;8(1):86-91. Epub 2015 Dec 18 doi: 10.4274/jcrpe.2512. PMID: 26757742Free PMC Article
An unusual phenotype of Frasier syndrome due to IVS9 +4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis.
Melo KF, Martin RM, Costa EM, Carvalho FM, Jorge AA, Arnhold IJ, Mendonca BB
J Clin Endocrinol Metab 2002 Jun;87(6):2500-5. doi: 10.1210/jcem.87.6.8521. PMID: 12050205
Persistence of delayed adrenarche in boys with thalassemia.
Filosa A, Di Maio S, Esposito G, De Martinis F, De Terlizzi F
J Pediatr Endocrinol Metab 2001 Apr;14(4):407-14. doi: 10.1515/jpem.2001.14.4.407. PMID: 11327374
Can adrenarche influence the degree of osteopenia in thalassemic children?
Filosa A, Di Maio S, Saviano A, Vocca S, Esposito G
J Pediatr Endocrinol Metab 1996 May-Jun;9(3):401-6. doi: 10.1515/jpem.1996.9.3.401. PMID: 8887150

Diagnosis

Hypoprolactinemia as a Clue to Diagnosis of Mild Central Hypothyroidism due to IGSF1 Deficiency.
Papadimitriou A, Papadopoulou A, Kleanthous K, Papadimitriou DT, Papaevangelou V
J Clin Res Pediatr Endocrinol 2020 Jun 3;12(2):218-222. Epub 2019 Aug 26 doi: 10.4274/jcrpe.galenos.2019.2019.0085. PMID: 31448769Free PMC Article
An unusual phenotype of Frasier syndrome due to IVS9 +4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis.
Melo KF, Martin RM, Costa EM, Carvalho FM, Jorge AA, Arnhold IJ, Mendonca BB
J Clin Endocrinol Metab 2002 Jun;87(6):2500-5. doi: 10.1210/jcem.87.6.8521. PMID: 12050205

Therapy

Delayed Adrenarche may be an Additional Feature of Immunoglobulin Super Family Member 1 Deficiency Syndrome.
Van Hulle S, Craen M, Callewaert B, Joustra S, Oostdijk W, Losekoot M, Wit JM, Turgeon MO, Bernard DJ, De Schepper J
J Clin Res Pediatr Endocrinol 2016 Mar 5;8(1):86-91. Epub 2015 Dec 18 doi: 10.4274/jcrpe.2512. PMID: 26757742Free PMC Article
Persistence of delayed adrenarche in boys with thalassemia.
Filosa A, Di Maio S, Esposito G, De Martinis F, De Terlizzi F
J Pediatr Endocrinol Metab 2001 Apr;14(4):407-14. doi: 10.1515/jpem.2001.14.4.407. PMID: 11327374

Prognosis

Delayed Adrenarche may be an Additional Feature of Immunoglobulin Super Family Member 1 Deficiency Syndrome.
Van Hulle S, Craen M, Callewaert B, Joustra S, Oostdijk W, Losekoot M, Wit JM, Turgeon MO, Bernard DJ, De Schepper J
J Clin Res Pediatr Endocrinol 2016 Mar 5;8(1):86-91. Epub 2015 Dec 18 doi: 10.4274/jcrpe.2512. PMID: 26757742Free PMC Article
An unusual phenotype of Frasier syndrome due to IVS9 +4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis.
Melo KF, Martin RM, Costa EM, Carvalho FM, Jorge AA, Arnhold IJ, Mendonca BB
J Clin Endocrinol Metab 2002 Jun;87(6):2500-5. doi: 10.1210/jcem.87.6.8521. PMID: 12050205

Clinical prediction guides

Delayed Adrenarche may be an Additional Feature of Immunoglobulin Super Family Member 1 Deficiency Syndrome.
Van Hulle S, Craen M, Callewaert B, Joustra S, Oostdijk W, Losekoot M, Wit JM, Turgeon MO, Bernard DJ, De Schepper J
J Clin Res Pediatr Endocrinol 2016 Mar 5;8(1):86-91. Epub 2015 Dec 18 doi: 10.4274/jcrpe.2512. PMID: 26757742Free PMC Article
Absent or delayed adrenarche in Pit-1/POU1F1 deficiency.
Taha D, Mullis PE, Ibáñez L, de Zegher F
Horm Res 2005;64(4):175-9. Epub 2005 Oct 4 doi: 10.1159/000088793. PMID: 16210857
An unusual phenotype of Frasier syndrome due to IVS9 +4C>T mutation in the WT1 gene: predominantly male ambiguous genitalia and absence of gonadal dysgenesis.
Melo KF, Martin RM, Costa EM, Carvalho FM, Jorge AA, Arnhold IJ, Mendonca BB
J Clin Endocrinol Metab 2002 Jun;87(6):2500-5. doi: 10.1210/jcem.87.6.8521. PMID: 12050205
Persistence of delayed adrenarche in boys with thalassemia.
Filosa A, Di Maio S, Esposito G, De Martinis F, De Terlizzi F
J Pediatr Endocrinol Metab 2001 Apr;14(4):407-14. doi: 10.1515/jpem.2001.14.4.407. PMID: 11327374
Dissociation of adrenarche and gonadarche in diabetes mellitus.
Cohen HN, Paterson KR, Wallace AM, Beastall GH, Manderson WG, MacCuish AC
Clin Endocrinol (Oxf) 1984 Jun;20(6):717-24. doi: 10.1111/j.1365-2265.1984.tb00122.x. PMID: 6235985

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