ESPE2022 Poster Category 1 Pituitary, Neuroendocrinology and Puberty (77 abstracts)
Spectrum of Endocrinopathy in Children with Ectopic Posterior Pituitary (EPP)
Neha Malhotra 1 , Arliena Amin 2 , Manuela Cerbone 1 , Laura Bosch 3 , Sally Tollerfield 1 , Abigail Atterbury 1 , Harshini Katugampola 1,3 & Mehul Dattani 1,3,4
1Great Ormond Street Hospital for Children, London, United Kingdom; 2Putrajaya Hospital, Malaysia, Malaysia; 3University College London, London, United Kingdom; 4Genetics and Genomic Medicine Research and Teaching Programme, UCl, London, United Kingdom
Introduction: Ectopic posterior pituitary (EPP) is a radiological diagnosis likely caused by a migration defect that occurs during pituitary development. We aim to describe the clinical characteristics and hormonal deficiencies seen in patients with EPP.
Method: Longitudinal observational study performed in a single [HK1] [NM2] centre from 1993-2020. As per MRI findings, cases were divided into three cohorts 1) C1: EPP only [n=8 (9%)] 2) C2: EPP + PSIS (Pituitary Stalk interruption Syndrome) [n=44 (49.4%)] and 3) C3: EPP + Cortical abnormalities (CA) [n=37 (41.6%)].
Results: 89 patients were identified with EPP (55 male (61.8%)). The median duration of follow up was 9.85 years (IQR 3.96).
| C1 | C2 | C3 | |
| Median Age at presentation (in years) | 2.73 | 2.36 | 1.2 |
| Symptoms at presentation | |||
| Short Stature | 4 (50%) | 19 (43.2%) | 7 (18.9%) |
| Hypoglycaemia | 2 (25%) | 21 (46.7%) | 22 (48.9%) |
| Incidental MRI finding | 2 (25%) | 4 (9%) | 0 |
| Ophthalmology Referral | 0 | 0 | 8 (21.6%) |
| C1 | C2 | C3 | p- value | |
| Isolated GHD | 5 (62.5%) | 8 (18.2%) | 5 (13.5%) | 0.009 |
| GHD+ACTHD+TSHD | 0 | 30 (68.2%) | 29 (78.4%) | <0.005 |
| GHD+ACTHD | 0 | 2 (4.5%) | 1 (2.7%) | 1.00 |
| GHD+TSHD | 1(12.5%) | 3 (6.8%) | 1 (2.7%) | 0.489 |
| ACTHD+TSHD | 0 | 1 (2.3%) | 0 | |
| Isolated TSHD | 0 | 0 | 1 (2.7%) | |
| Hypogonadotropic Hypogonadism | 0 | 20 (45%) | 12 (32.4%) | 0.04 |
| Age of GHD (mean ± SD) | 6.02± 3.40 | 3.68 ± 3.12 | 2.96 ± 3.06 | |
| Age of ACTHD (mean ± SD) | 0 | 3.26 ± 4.53 | 1.68 ± 2.64 | |
| Age of TSHD (mean ± SD) | 3.86 | 3.29 ± 4.05 | 1.97 ± 2.84 | |
| No hormone deficiency | 2 (25%) | 0 | 0 | |
| GHD: growth hormone deficiency ACTHD: Adrenocorticotrophic hormone deficiency TSHD: Thyroid stimulating hormone deficiency |
||||
GHD was the single most frequent endocrinopathy across all cohorts (n=83, 93.2%). C2 and C3 both had evidence of MPHD with 72.8% within these groups having GHD, ACTHD and TSHD. ACTHD was the earliest endocrinopathy to present at 1.68 years in C3 and 3.26 years in C2. This was followed by TSHD which presented at 1.97 years in C3 and 3.29 years in C2. GHD developed before 5 years in C2 and C3 and after 5 years in C1.
Discussion and conclusion: The presence of other abnormalities in addition to EPP were associated with progression to multiple pituitary hormone deficiency (MPHD). EPP with normal pituitary stalk and no CA is a strong predictor for isolated GHD rather than MPHD.
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