Abstract:
Lymphatic disorders presenting in the first year of life are difficult to identify and manage given the broad range of underlying etiologies. Neonatal lymphatic disease arising from congenital or acquired conditions results in the abnormal accumulation of lymph fluid in the pleura (chylothorax), peritoneum (chylous ascites) and skin (edema/anasarca). There is also increasing recognition of lymphatic losses through the intestine resulting in protein-losing enteropathy (PLE). While the incidence of lymphatic disorders in neonates is unclear, advances in genetic testing and lymphatic imaging are improving our understanding of the underlying pathophysiology. Despite these advancements, medical management of neonatal lymphatic disorders remains challenging and variable among clinicians.
摘要:
考虑到广泛的潜在病因,在生命的第一年出现的淋巴障碍很难识别和管理。由先天性或获得性疾病引起的新生儿淋巴疾病导致胸膜(乳糜胸)中淋巴液的异常积聚,腹膜(乳糜腹水)和皮肤(水肿/anasarca)。对通过肠道的淋巴管丢失也有越来越多的认识,从而导致蛋白质丢失性肠病(PLE)。虽然新生儿淋巴疾病的发生率尚不清楚,基因检测和淋巴成像的进展正在提高我们对潜在病理生理学的理解。尽管取得了这些进步,新生儿淋巴疾病的医疗管理在临床医生中仍然具有挑战性和可变性.