Pituitary Stalk Interruption Syndrome: Analysis of Response to Growth Hormone Therapy

Objective To analyse the clinical and radiological characteristics of pituitary stalk interruption syndrome (PSIS). Methods A retrospective analysis of confirmed cases of PSIS was performed. The development of new pituitary hormonal deficiencies and response to recombinant human growth hormone (rhGH...

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Bibliographic Details
Published in:Indian pediatrics 2024-02, Vol.61 (2), p.154-157
Main Authors: Ravichandran, Raghuraman, Saikia, Uma K., Bhuyan, Ashok K., Baro, Abhamoni
Format: Article
Language:English
Subjects:
Adolescent
Child
Child, Preschool
Dwarfism, Pituitary
Female
Growth Hormone - therapeutic use
Human Growth Hormone
Humans
Hypopituitarism
Infant
Male
Maternal and Child Health
Medicine
Medicine & Public Health
Original Article
Pediatric Surgery
Pediatrics
Pituitary Gland
Retrospective Studies
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Summary:Objective To analyse the clinical and radiological characteristics of pituitary stalk interruption syndrome (PSIS). Methods A retrospective analysis of confirmed cases of PSIS was performed. The development of new pituitary hormonal deficiencies and response to recombinant human growth hormone (rhGH) therapy were assessed during follow-up. Results This study included 14 children (10 boys) of PSIS with median (range) age of 12.15 years (2 months–18 years). Short stature was the most common presentation ( n = 13), and micropenis ( n = 4), cleft lip ( n = 1) and single central incisor ( n = 1) were other midline defects. Growth hormone (GH) deficiency was present in 14 children and 7 of them also had multiple pituitary hormone deficiencies at baseline. Central hypothyroidism ( n = 5), secondary adrenal deficiency ( n = 4) and gonadotropin deficiencies ( n = 2) were also seen. All children received rhGH. The mean height gain on follow-up was 12.78 cm in first year ( n = 14), 6.5 cm in second year ( n = 8) and 4.07 cm in third year ( n = 7) of rhGH therapy. Four children developed additional pituitary hormone deficiency on follow-up. Conclusion Short stature with isolated GH deficiency was the most common presentation of PSIS that showed good response to rhGH therapy.
ISSN:0019-6061
0974-7559
DOI:10.1007/s13312-024-3111-6