ESPE2024 Poster Category 1 Growth and Syndromes 3 (10 abstracts)
Polysomnography Findings and Respiratory Problems in Prader-Willi Syndrome: The Effect of Growth Hormone Treatment
Elif Kelestemur 1 , Eda Esra Baysal 2 , Tugba Seven Menevse 1 , Serpil Bas 1 , Ilknur Kurt 1 , Ahmet Kahveci 1 , Busra Gurpinar Tosun 1 , Didem Helvacioglu 1 , Zehra Yavas Abali 1 , Ali Cemal Yumusakhuylu 3 , Yasemin Gokdemir 2 , Ela Erdem Eralp 2 , Almala Pinar Ergenekon 2 , Bulent Karadag 2 , Belma Haliloglu 1 , Tulay Guran 1 , Abdullah Bereket 1 & Serap Turan 1
1Marmara University School of Medicine, Department of Pediatric Endocrinology and Diabetes, Istanbul, Turkey. 2Marmara University School of Medicine, Department of Pediatric Pulmonology, Istanbul, Turkey. 3Marmara University, Department of Otorhinolaryngology-Head and Neck Surgery, Istanbul, Turkey
Introduction: Prader-Willi syndrome (PWS) is a genetic condition characterized by a complex hypothalamic dysregulation of the growth hormone/insulin-like growth factor (GH/IGF) axis, short stature, obesity, reduced muscle mass and impaired quality of life. The risk of obstructive sleep apnea (OSA), central hypoventilation (CHV) and sleep-disordered breathing (SDB) are the major causes of morbidity and mortality in these patients.
Objective: To evaluate the clinical and polysomnographic (PSG) characteristics of patients with PWS, and the changes in PSG following GH therapy (GHT).
Results: Forty-four patients with PWS were admitted to our centre. Eleven of these were excluded from the study due to short follow-up periods. Upon evaluation of the initial PSG results of the remaining 33 patients, 5 patients exhibited normal PSG findings. Conversely, 11 patients had mild SA, 7 patients had moderate SA, 9 patients had severe SA, and 1 patient had very severe SA. Among these, 18 had OSA, 3 had CHV and 4 had mixed SA. Adenoidectomy was performed in 14 of 18 patients with OSA and non-invasive ventilatory support was initiated in 14 patients. The mean peak values in GH stimulation test was 4.4 mcg/L, and 65.6% of the patients had complete GH deficiency (<5 mcg/L). GHT was commenced in 31 patients, while 2 patients could not receive GHT due to scoliosis and SA. The mean age at presentation was 2.64 years (0.24-15.8), the mean age at first PSG was 3.38 (0.33-13.3), and the mean age at initiation of GHT was 3.52 (0.44-13.19). The mean follow-up periods was 5.36 years (0.45-11.1) and the mean duration of GHT was 3.42 years (0.43-6.93). When the basal PSG findings were evaluated, no significant difference were detected among the five different age groups. After at least 6 months of GHT, PSG findings remained same in 7 patients, improved in 11 patients, and worsened in 2 patients. During the follow-up period, GHT was discontinued in 5 patients due to increased scoliosis (n:1), family request (n = 1), allergic reaction (n = 1), and drug supply problems (n = 2). When we compare PSG finding between patients who received GHT (n = 20) and those did not (n = 6), the changes in severity of apnea scores were significantly better in the group receiving GHT.
Conclusion: There were no serious respiratory complications or apnea-related mortality in PWS patients who were followed up with PSG and monitoried and treated respiratory support when required.A multidisciplinary approach in managing PWS is effective in preventing disease and/or treatment-related complications.
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