ESPE2024 Free Communications Pituitary, Neuroendocrinology and Puberty 1 (6 abstracts)
The spectrum of Endocrinopathies in Children with Ectopic Posterior Pituitary (EPP) correlates with the severity of associated hypothalamo-pituitary abnormalities on imaging: a decade long experience from two tertiary centers.
Neha Malhotra 1 , Tiziana Camia 2 , Manuela Cerbone 3,4 , Natascia Di Iorgi 5,6 , Mohamad Maghnie 7,8 & Mehul Dattani 3,4,9
1Basildon University Hospital, Essex, United Kingdom. 2Operative Unit of Neonatology IRCCS Ospedale Policlinico San Martino, Genoa, Italy. 3Department of Paediatric Endocrinology, Great Ormond Street Hospital, London, United Kingdom. 4Genetics and Genomic Medicine Research and Teaching Programme, UCL GOS Institute of Child Health, London, United Kingdom. 5Paediatric Unit, Department of Precision and Regenerative Medicine and Ionian Area, University “A. Moro” of Bari, Bari, Italy. 6Division of Paediatric Endocrinology, Department of Public Health and Paediatrics, University of Turin, Regina Margherita Children’s Hospital, Turin, Italy. 7Department of Pediatrics, IRCCS Giannina Gaslini Institute, Department of Neuroscience, Rehabilitation, Ophtalmology, Genetics, Maternal and Child Health - University of Genova, Genova, Italy. 8Department of Pediatrics, IRCCS Istituto Giannina Gaslini, Genoa, Italy. 9Endocrinology Department, University College London Hospitals, London, United Kingdom.
Introduction: Ectopic posterior pituitary (EPP) is a neuroimaging diagnosis frequently identified together with other hypothalamo-pituitary (H-P) abnormalities [hypoplastic anterior pituitary and thin interrupted stalk -Pituitary Stalk Interruption Syndrome (PSIS)] and associated with variable endocrine phenotypes evolving over time. We aim to describe the spectrum of hormonal deficiencies and neuroimaging abnormalities in a large cohort of children with EPP.
Method: Longitudinal data collection at two tertiary centers (1993 -2023). 171 patients with EPP were identified (99 M, 58%). Patients were divided into 3 cohorts based on MRI 1) C1: PSIS [n = 121, 70.8%] 2) C2: PSIS-variant (PSIS-V;2/3 criteria met) [n = 37, 21.6%] 3) C3: Isolated EPP (i-EPP) [n = 13, 7.6 % ]. Long term growth data were also collected.
Results:
| Total | C1 | C2 | C3 | P | |
| Age at presentation (y), median (1st; 3rd quartile) | 1.9 (0.3; 5.1) | 3.1 (0.4; 7.1) | 7.4 (3.8; 9.9) | 0.003 | |
| Neonatal Hypoglycaemia | 57 | 49/109 (44.9 %) | 7/30 (23.3 %) | 1/9 (11.1 %) | 0.02 |
| Short stature | 79 | 53 (43.8 %) | 16 (43.2 %) | 10 (76.9 %) | 0.03 |
| Follow-Up length (y), median (1st; 3rd quartile) | 9.6 (5;14.2) [n = 112] | 7.2 (2.7;10.8) [n = 35] | 3.4 (1.1;7.8) [n = 24] | 0.003 | |
| GHD | |||||
| Age of presentation (y), Median (1st – 3rd quartile) | 2.83 (1.44;5.9) [n = 146] | 2.31 (1.36;4.7) [n = 113] | 3.56 (1.82;6.8) [n = 26] | 8.5 (7.38;9.66) [n = 7] | 0.008 |
| Reversal at Transition: Yes | 13/54 (24.1 %) | 6/36 (16.7 %) | 5/12 (41.7 %) | 2/3 (66.7 %) | 0.048 |
| No | 30/36 (83.3 %) | 7/12 (58.3 %) | 1/3 (58.3 %) | ||
| TSHD | |||||
| Age of presentation (y) Median (1st – 3rd quartile) | 1.12 (0.12;5.13) [n = 99] | 1.12 (0.14;4.5) [n = 82] | 0.45 (0.08;6.36) [n = 15] | 11.65 (10.7;12.6) [n = 2] | 0.13 |
| ACTHD | |||||
| Age of presentation (y), Median (1st – 3rd quartile) | 1.48 (0.12;6.36) [n = 94] | 1.07 (0.12;6.09) [n = 81] | 2.23 (0.13;6.36) [n = 11] | 14.37 (13.99;14.74) [n = 2] | 0.07 |
| ACTH DEFICIENCY: | |||||
| Complete | 87 (91.6 %) | 78 (95.1 %) | 8 (72.7 %) | 1 (50.0 %) | 0.017 |
| Partial | 8 (8.4 %) | 4 (4.9 %) | 3 (27.3 %) | 1 (50.0 %) | |
| Subjects in Pubertal age group HH: yes | 45 | 41 (55.4 %) | 4 (22.2 %) | 0 (0.0%) | < 0.0001 |
Conclusion: In EPP, presence of additional H-P abnormalities is associated with increased number and earlier onset endocrinopathies (GH, TSH, ACTH). Patients with the complete PSIS triad have more severe phenotypes, whilst patients with isolated EPP have the least severe phenotypes.
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