Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu, China.
Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, Chengdu, China.
Medicine (Baltimore). 2024 Mar 15;103(11):e37341. doi: 10.1097/MD.0000000000037341.
Hepatic sinusoidal obstruction syndrome (HSOS), which includes hepatic stasis and portal hypertension, is a rare vascular disorder of the liver. It is often associated with hematopoietic stem cell transplantation. It is also possible to treat this disease using Chinese herbal medicines that contain pyrrolizidine alkaloids (PAs). This disease is extremely rare in children and poses a serious threat to their health. To our knowledge, this is the first case of HSOS in a child with PAs.
We report a 4-year-old boy suffering from abdominal pain, hepatomegaly, massive ascites, elevated liver enzyme level, and severe portal hypertension as a result of the consumption of Gynura segetum (also known as Tusanqi in Chinese, a traditional herbal medicine containing PAs).
The child was finally diagnosed with PA-HSOS based on pathological diagnosis and imaging examination.
With active symptomatic and supportive care and sequential anticoagulation therapy, the abdominal distension and liver function improved in the patient.
The patient was eventually recovered. The levels of liver enzymes, hemoglobin, and bilirubin were normal, and the international normalized ratio fluctuated between 2.0 and 3.0 during 1-year follow-up after discharge.
This case report emphasizes the prevention of Chinese herb-induced liver injury in children and the importance of active long-term sequential anticoagulant therapy to reduce the progressive damage of PA-HSOS in the liver.
肝窦阻塞综合征(HSOS)包括肝淤血和门静脉高压,是一种罕见的肝脏血管疾病。它常与造血干细胞移植有关。也可以使用含有吡咯里西啶生物碱(PAs)的中草药来治疗这种疾病。这种疾病在儿童中极为罕见,对其健康构成严重威胁。据我们所知,这是首例儿童 PAs 相关 HSOS 病例。
我们报告了一例 4 岁男孩,因食用三叶鬼针草(又称图山齐,一种含有 PAs 的传统草药)而出现腹痛、肝肿大、大量腹水、肝酶水平升高和严重门静脉高压。
根据病理诊断和影像学检查,患儿最终被诊断为 PAs-HSOS。
通过积极的对症支持治疗和序贯抗凝治疗,患者腹胀和肝功能改善。
患儿最终康复。出院后 1 年随访期间,肝酶、血红蛋白和胆红素水平正常,国际标准化比值在 2.0 至 3.0 之间波动。
本病例报告强调了预防儿童中药肝损伤的重要性,以及积极进行长期序贯抗凝治疗以减少 PA-HSOS 在肝脏进行性损害的重要性。