Breatnach Colm R, Cleary Aoife, Prendiville Terence, Crumlish Kathleen, Murchan Helene, McMahon Colin J
Department of Paediatric Cardiology, Our Lady's Hospital for Sick Children, Crumlin, Dublin 4, Ireland.
University College Dublin, Dublin 4, Ireland.
Pediatr Cardiol. 2018 Jan;39(1):33-37. doi: 10.1007/s00246-017-1720-8. Epub 2017 Sep 6.
Protein-Losing Enteropathy post Fontan palliation is associated with significant morbidity and mortality. To date, very little research has been carried out to improve early identification of enteric protein loss in these patients. We hypothesise that subclinical enteric protein loss may occur in patients post Fontan surgery. A cross-sectional study was performed on 43 patients post Fontan surgery. We collected specimens of stool and blood from patients with no symptoms of protein-losing enteropathy post Fontan. Stool samples were assessed for alpha one antitrypsin. The stool samples of two patients were discarded, leaving 41 stool samples. Blood samples were also collected to review albumin, C-reactive protein, liver and renal function. Twenty-eight (65%) of those enrolled were male. The median (IQR) age between Fontan and collection of study specimens was 3.5 (2-7) years. Two (5%) patients had elevated levels of alpha-1-antitrypsin. There was no correlation between blood biochemistry and elevated stool alpha-1-antitrypsin. Subclinical protein loss is rare in asymptomatic children after Fontan procedure with only 5% of patients having elevated stool alpha-1-antitrypsin but no other symptoms. These findings may relate to our small cohort size and the time to testing post cardiac surgery. Future longitudinal follow-up studies should assess the ability of alpha-1-antitrypsin to provide earlier detection of protein-losing enteropathy in asymptomatic patients post Fontan. Given the serious prognosis of protein-losing enteropathy in this patient group, further work is warranted.
Fontan 姑息术后的蛋白丢失性肠病与显著的发病率和死亡率相关。迄今为止，针对改善这些患者肠道蛋白丢失的早期识别所开展的研究极少。我们推测 Fontan 手术后的患者可能会发生亚临床肠道蛋白丢失。对 43 例 Fontan 手术后的患者进行了一项横断面研究。我们从无 Fontan 术后蛋白丢失性肠病症状的患者中收集了粪便和血液样本。对粪便样本进行了α1抗胰蛋白酶评估。两名患者的粪便样本被丢弃，剩余 41 份粪便样本。还收集了血液样本以检测白蛋白、C 反应蛋白、肝功能和肾功能。纳入研究的患者中有 28 例（65%）为男性。从 Fontan 手术到采集研究样本的中位（四分位间距）年龄为 3.5（2 - 7）岁。两名（5%）患者的α1抗胰蛋白酶水平升高。血液生化指标与粪便α1抗胰蛋白酶升高之间无相关性。在 Fontan 手术后无症状的儿童中，亚临床蛋白丢失很少见，只有 5%的患者粪便α1抗胰蛋白酶升高但无其他症状。这些发现可能与我们的队列规模较小以及心脏手术后的检测时间有关。未来的纵向随访研究应评估α1抗胰蛋白酶在无症状的 Fontan 术后患者中早期检测蛋白丢失性肠病的能力。鉴于该患者群体中蛋白丢失性肠病的严重预后，有必要开展进一步的工作。
