Kang Yu-Lin, Cui Yun, Wu Ying, Hao Shen, Kuang Xin-Yu, Zhang Yu-Cai, Huang Wen-Yan, Zhu Guang-Hua
Department of Nephrology and Rheumatology, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China.
Pediatric Intensive Care Unit, Shanghai Children's Hospital, Shanghai Jiao Tong University, Shanghai, China.
BMC Pediatr. 2018 Apr 5;18(1):127. doi: 10.1186/s12887-018-1101-3.
Chylopericardium effusion is characterized by the accumulation of milky effusion in the pericardium. It is often idiopathic but it can be secondary to trauma, chest radiation, tuberculosis and malignancy. If cardiac tamponade ensues, it becomes life-threatening. Herein we describe chylopericardium tamponade in a child with IgA nephropathy. To the best of our knowledge, this is the first reported case of chylopericardium tamponade in IgA nephropathy.
A 6 years old boy with IgA nephropathy presented with dyspnea, orthopnea, pretibial pitting edema, ascites and fever. Muffled heart sounds and hepatomegaly were also noted. Echocardiography and thoracic CT revealed that there was a large volume of hydropericardium. Moreover, the pericardial milky fluid by pericardiocentesis was analyzed and chylopericardium effusion was eventually confirmed. Pericardial drainage was continued and his diet was modified to low fat, rich MCT and high protein. Complete remission was achieved after 3 weeks of this combined treatment.
Chylopericardial tamponade could be a rare and life-threatening complication of IgA nephropathy. Etiological analysis is critical for determining the therapeutic approach in patients with pericardial effusion.
乳糜性心包积液的特征是心包内积聚乳状积液。它通常是特发性的,但也可能继发于创伤、胸部放疗、结核病和恶性肿瘤。如果发生心脏压塞,则会危及生命。在此,我们描述了一名患有IgA肾病的儿童发生乳糜性心包压塞的情况。据我们所知,这是首例关于IgA肾病合并乳糜性心包压塞的报道病例。
一名6岁患有IgA肾病的男孩出现呼吸困难、端坐呼吸、胫前凹陷性水肿、腹水和发热症状。还发现心音减弱和肝肿大。超声心动图和胸部CT显示有大量心包积液。此外,对心包穿刺抽出的心包乳状液进行分析,最终确诊为乳糜性心包积液。持续进行心包引流,并将其饮食调整为低脂、富含中链甘油三酯和高蛋白。联合治疗3周后实现完全缓解。
乳糜性心包压塞可能是IgA肾病一种罕见且危及生命的并发症。病因分析对于确定心包积液患者的治疗方法至关重要。