Department of Paediatric Endocrinology, Royal Manchester Children's Hospital, Manchester, United Kingdom.
Therapy and Dietetic Department, Royal Manchester Children's Hospital, Manchester, United Kingdom.
Front Endocrinol (Lausanne). 2020 Mar 18;11:143. doi: 10.3389/fendo.2020.00143. eCollection 2020.
Congenital Hyperinsulinism (CHI) is the most common cause of recurrent and severe hypoglycaemia in childhood. Feeding problems occur frequently in severe CHI but long-term persistence and rates of resolution have not been described. All patients with CHI admitted to a specialist center during 2015-2016 were assessed for feeding problems at hospital admission and for three years following discharge, through a combination of specialist speech and language therapy review and parent-report at clinical contact. Twenty-five patients (18% of all patients admitted) with CHI were prospectively identified to have feeding problems related to sucking ( = 6), swallowing ( = 2), vomiting ( = 20), and feed aversion ( = 17) at the time of diagnosis. Sixteen (64%) patients required feeding support by nasogastric/gastrostomy tubes at diagnosis; tube feeding reduced to 4 (16%) patients by one year and 3 (12%) patients by three years. Feed aversion resolved slowly with mean time to resolution of 240 days after discharge; in 15 patients followed up for three years, 6 (24%) continued to report aversion. The mean time (days) to resolution of feeding problems was lower in those who underwent lesionectomy ( = 4) than in those who did not (30 vs. 590, = 0.009) and significance persisted after adjustment for associated factors ( = 0.015). Feeding problems, particularly feed aversion, are frequent in patients with CHI and require support over several years. By contrast, feeding problems resolve rapidly in patients with focal CHI undergoing curative lesionectomy, suggesting the association of feeding problems with hyperinsulinism.
先天性高胰岛素血症(CHI)是儿童反复严重低血糖的最常见原因。严重 CHI 常伴有喂养问题,但长期持续存在和解决率尚未描述。2015-2016 年期间,所有在专科中心住院的 CHI 患者在入院时和出院后三年内,通过专科言语治疗师的评估和临床接触时家长的报告,对喂养问题进行了评估。25 例(所有住院患者的 18%)CHI 患者在诊断时存在与吸吮(=6)、吞咽(=2)、呕吐(=20)和厌恶喂养(=17)相关的喂养问题。16 例(64%)患者在诊断时需要经鼻胃/胃造口管喂养支持;1 年后降至 4 例(16%),3 年后降至 3 例(12%)。厌恶喂养的解决速度较慢,出院后平均解决时间为 240 天;在随访 3 年的 15 例患者中,有 6 例(24%)继续报告有厌恶感。行病变切除术的患者(=4)的喂养问题解决时间(天)短于未行病变切除术的患者(=30 比=590,=0.009),调整相关因素后仍有统计学意义(=0.015)。CHI 患者常伴有喂养问题,特别是厌恶喂养,需要数年的支持。相比之下,行根治性病变切除术的局灶性 CHI 患者的喂养问题能迅速解决,这提示喂养问题与高胰岛素血症有关。