Duplication of the Pituitary Gland (DPG)-Plus Syndrome Associated With Midline Anomalies and Precocious Puberty: A Case Report and Review of the Literature

被引:4
|
作者
Prezioso, Giovanni [1 ]
Petraroli, Maddalena [1 ]
Bergonzani, Michela [2 ]
Davino, Giusy [1 ]
Labate, Marialuisa [1 ]
Ormitti, Francesca [3 ]
Anghinoni, Marilena [2 ]
Sesenna, Enrico [2 ]
Esposito, Susanna [1 ]
机构
[1] Univ Parma, Dept Med & Surg, Pediat Clin, Parma, Italy
[2] Univ Parma, Dept Med & Surg, Maxillofacial Surg Unit, Parma, Italy
[3] Univ Hosp Parma, Neuroradiol Unit, Parma, Italy
来源
关键词
pituitary; craniofacial abnormalities; midline malformations; pediatric endocrinology; precocious puberty; DEFECTS; WEIGHT; HEIGHT;
D O I
10.3389/fendo.2021.685888
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Duplication of the pituitary gland (DPG)-plus syndrome is a very rare developmental disorder with few cases described in the literature and characterized by multiple midline and central nervous system malformations. The hypothalamus and hypophysis involvement may be clinically associated with endocrine abnormalities. A 5.9-year-old female child was admitted to our Clinic for premature thelarche and acceleration of growth. DPG-plus syndrome with paired infundibula and pituitary glands was diagnosed after birth, when she appeared small for gestational age and she presented with lingual hypoplasia, cleft palate, right choanal stenosis, nasopharyngeal teratoma, and facial dysmorphisms. Neuroimaging revealed a duplication of the infundibula, the pituitary gland, and the dens of the epistropheus despite surgical removal of a rhino-pharyngeal mass performed at the age of two months. An array-CGH revealed a 2p12 deletion. At our evaluation, bone age assessment resulted advanced and initial pubertal activation was confirmed by Gonadotropin-Releasing Hormone stimulation test. Hormonal suppression treatment was started with satisfactory results. This case shows that DPG-plus syndrome must be considered in presence of midline and craniofacial malformations and endocrinological evaluations should be performed for the prompt and appropriate management of pubertal anomalies.
引用
收藏
页数:5
相关论文
共 50 条
  • [1] Perinatal management for fetal oral epignathus with duplication of the pituitary gland (DPG)-plus syndrome: A case report and literature review
    Khwankaew, Noppasin
    Sawaddisan, Rapphon
    Siripruekpong, Sirikarn
    Leelasawatsuk, Peesit
    Praditaukrit, Manapat
    Tanaanantarak, Pattama
    [J]. INTERNATIONAL JOURNAL OF GYNECOLOGY & OBSTETRICS, 2024,
  • [2] Central precocious puberty associated with pituitary duplication and midline defects
    Vieira, Teresa C.
    Chinen, Renata N.
    Ribeiro, Maria R. F.
    Nogueira, Roberto Gomes
    Abucham, Julio
    [J]. JOURNAL OF PEDIATRIC ENDOCRINOLOGY & METABOLISM, 2007, 20 (10): : 1141 - 1144
  • [3] Pituitary Duplication and Precocious Puberty Case Report
    Gabriela Rampi, Maria
    [J]. HORMONE RESEARCH IN PAEDIATRICS, 2018, 90 : 46 - 47
  • [4] Precocious puberty in Turner Syndrome: report of a case and review of the literature
    Improda, Nicola
    Rezzuto, Martina
    Alfano, Sara
    Parenti, Giancarlo
    Vajro, Pietro
    Pignata, Claudio
    Salerno, Mariacarolina
    [J]. ITALIAN JOURNAL OF PEDIATRICS, 2012, 38
  • [5] Precocious puberty in Turner Syndrome: report of a case and review of the literature
    Nicola Improda
    Martina Rezzuto
    Sara Alfano
    Giancarlo Parenti
    Pietro Vajro
    Claudio Pignata
    Mariacarolina Salerno
    [J]. Italian Journal of Pediatrics, 38
  • [6] A case report of precocious puberty related to Rett syndrome and a literature review
    Yang, Lianlian
    Jiang, Mingyan
    Yu, Ruixin
    Hu, Ruolan
    Xiong, Fei
    Li, Jinrong
    [J]. PHARMAZIE, 2021, 76 (11): : 559 - 561
  • [7] Double Pituitary gland-pulse syndrome ((DPG)-plus syndrome) and idiopathic infantile hypercalcemia: a combination of rare disorders- Case report and review of literature
    Alyahyawi, Naseem
    Stewart, Laura
    [J]. HORMONE RESEARCH IN PAEDIATRICS, 2022, 95 (SUPPL 2): : 544 - 544
  • [8] Precocious puberty and tumors of the hypothalamus - Report of a case wnd review of the literature, with a pathophysiologic explanation of the precocious sexual syndrome
    Weinberger, LM
    Grant, FC
    [J]. ARCHIVES OF INTERNAL MEDICINE, 1941, 67 (04) : 762 - 792
  • [9] PARANEOPLASIC PRECOCIOUS PUBERTY - REPORT OF A NEW CASE WITH HEPATOBLASTOMA AND REVIEW OF THE LITERATURE
    NAVARRO, C
    CORRETGER, JM
    SANCHO, A
    ROVIRA, J
    MORALES, L
    [J]. CANCER, 1985, 56 (07) : 1725 - 1729
  • [10] LAUGHING SEIZURES AND PRECOCIOUS PUBERTY - (CASE-REPORT AND REVIEW OF LITERATURE)
    PENFOLD, JL
    MANSON, JI
    CALDICOTT, WM
    [J]. AUSTRALIAN PAEDIATRIC JOURNAL, 1978, 14 (03): : 185 - 190