BSPED2024 Poster Presentations Pituitary and Growth (8 abstracts)
Spectrum of endocrinopathies in children with ectopic posterior pituitary correlates with severity of associated hypothalamo-pituitary abnormalities on imaging: decade long experience from two tertiary centres
Neha Malhotra 1 , Tiziana Camia 2 , Manuela Cerbone 3,4 , Natascia Di. Iorgi 5,6 , Mohamad Maghnie 5,6 & Mehul Dattani 3,4,7
1Basildon University Hospital, Basildon, 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; 5Department of Pediatrics, IRCCS Giannina Gaslini Institute, Department of Neuroscience, Rehabilitation, Ophtalmology, Genetics, Maternal and Child Health - University of Genova, Genova, Italy; 6Department of Pediatrics, IRCCS Istituto Giannina Gaslini, Genova, Italy; 7Endocrinology 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%). Three 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 (years), median (1st; 3rd quartile) | 1.9 | 3.1 | 7.4 | 0.003 | |
| Neonatal Hypoglycaemia | 57 | 49/109 | 7/30 | 1/9 | 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 | 7.2 | 3.4 | 0.003 | |
| GHD | |||||
| Age of presentation (y), Median (1st – 3rd quartile) | 2.83 | 2.31 | 3.56 | 8.5 | 0.008 |
| Reversal at Transition: Yes | 13/54 | 6/36 | 5/12 | 2/3 | 0.048 |
| No | 30/36 | 7/12 | 1/3 | ||
| TSHD | |||||
| Age of presentation (y) Median (1st – 3rd quartile) | 1.12 | 1.12 | 0.45 | 11.65 | 0.13 |
| ACTHD | |||||
| Age of presentation (y), Median (1st – 3rd quartile) | 1.48 | 1.07 | 2.23 | 14.37 | 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. Patients with complete PSIS triad have severe phenotypes.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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