西罗莫司治疗儿童弥漫性肺淋巴管瘤病1例报告
收稿日期: 2024-04-28
录用日期: 2025-01-09
网络出版日期: 2025-08-27
A case report of sirolimus in the treatment of diffuse pulmonary lymphangiomatosis in children
Received date: 2024-04-28
Accepted date: 2025-01-09
Online published: 2025-08-27
叶泽慧 , 姜小丽 . 西罗莫司治疗儿童弥漫性肺淋巴管瘤病1例报告[J]. 临床儿科杂志, 2025 , 43(9) : 705 -709 . DOI: 10.12372/jcp.2025.24e0399
Objective To explore the clinical characteristics and treatment strategies of diffuse pulmonary lymphangiomatosis in children, in order to improve the early diagnosis and treatment level and the prognosis of children. Methods The clinical data of a child with diffuse pulmonary lymphangiomatosis admitted to the respiratory department of our hospital were retrospectively analyzed. The clinical manifestations, diagnostic process, and treatment outcomes were systematically summarized. Furthermore, current comprehensive treatment options and prognostic factors were discussed in light of relevant literature. Results The child received sirolimus treatment for 3 years, and the clinical symptoms were significantly relieved. Chest CT showed significant improvement. Conclusion Sirolimus demonstrates promising therapeutic efficacy in the management of diffuse pulmonary lymphangiomatosis in children, with a favorable safety profile and the potential to significantly enhance long-term prognosis.
Key words： diffuse pulmonary lymphangiomatosis; sirolimus; child
| [1] | 沈秋英, 农光民, 顾永耀. 儿童弥漫性肺淋巴管瘤病二例[J]. 中华儿科杂志, 2016, 54(10): 781-782. |
| Shen QY, Nong GM, Gu YY. Two cases of diffuse pulmonary lymphangioma in children[J]. Zhonghua Erke Zazhi, 2016, 54(10): 781-782. | |
| [2] | Ernotte C, Médart L, Collignon L. Diffuse pulmonary lymphangiomatosis[J]. J Belg Soc Radiol, 2018, 102(1): 64. |
| [3] | Gurskyt? V, Zeleckien? I, Maskoliūnait? V, et al. Successful treatment of diffuse pulmonary lymphangiomatosis with sirolimus[J]. Respir Med Case Rep, 2020, 29: 101014. |
| [4] | Triana P, Dore M, Cerezo VN, et al. Sirolimus in the treatment of vascular anomalies[J]. Eur J Pediatr Surg, 2017, 27(1): 86-90. |
| [5] | Luisi F, Torre O, Harari S. Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)[J]. Eur Respir Rev, 2016, 25(140): 170-177. |
| [6] | Zheng G, Tang H, Su R, et al. A gene missense mutation in diffuse pulmonary lymphangi- omatosis with thrombocytopenia: a case report[J]. Medicine (Baltimore), 2020, 99(39): e21941. |
| [7] | Faul JL, Berry GJ, Colby TV, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome[J]. Am J Respir Crit Care Med, 2000, 161(3Pt 1): 1037-1046. |
| [8] | Moreno RP, Hernández Y, Garrido P, et al. Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report[J]. Arch Argent Pediatr, 2021, 119(3): e264-e268. |
| [9] | Liu JR, Shen WB, Wen Z, et al. Clinical analysis of two cases with diffuse pulmonary lymphatic disease[J]. Zhonghua Erke Zazhi, 2016, 54(5): 360-364. |
| [10] | Ricci KW, Iacobas I. How we approach the diagnosis and management of complex lymphatic anomalies[J]. Pediatr Blood Cancer, 2022, 69(Suppl 3): e28985. |
| [11] | 曾祥妮, 吴爱民, 李岚, 等. 一例弥漫性肺淋巴管瘤病支气管镜下表现及分析[J]. 临床儿科杂志, 2024, 42(12): 1047-1050.. |
| Zeng XN, Wu AM, Li L, et al. A case report of a rare case of diffuse pulmonary lymphangiomatosis with bronchoscopic presentation[J]. Linchuang Erke Zazhi, 2024, 42(12): 1047-1050. | |
| [12] | Sun X, Shen W, Xia S, et al. Diffuse pulmonary lymphangiomatosis: MDCT findings after direct lymphangiography[J]. AJR Am J Roentgenol, 2017, 208(2): 300-305. |
| [13] | 潘登, 丛金鹏, 王芳芳, 等. 弥漫性肺淋巴管瘤病二例报[J]. 临床医学进展, 2020, 10(7): 1397-1404. |
| Pan D, Cong JP, Wang FF, et al. Diffuse pulmonary lymphangiomatosis: report of two cases[J]. Linchuang Yixue Jinzhan, 2020, 10(7): 1397-1404. | |
| [14] | Biscotto I, Rodrigues RS, Forny DN, et al. Diffuse pulmonary lymphangiomatosis[J]. J Bras Pneumol, 2019, 45(5): e20180412. |
| [15] | Lim HJ, Han J, Kim HK, et al. A rare case of diffuse pulmonary lymphangiomatosis in a middle-aged woman[J]. Korean J Radiol, 2014, 15(2): 295-299. |
| [16] | Ozeki M, Nozawa A, Yasue S, et al. The impact of sirolimus therapy on lesion size, clinical symptoms, and quality of life of patients with lymphatic anomalies[J]. Orphanet J Rare Dis, 2019, 14(1): 141. |
| [17] | Ricci KW, Hammill AM, Mobberley-Schuman P, et al. Efficacy of systemic sirolimus in the treatment of generalized lymphatic anomaly and Gorham-Stout disease[J]. Pediatr Blood Cancer, 2019, 66(5): e27614. |
| [18] | Rodriguez-Laguna L, Agra N, Iba?ez K, et al. Somatic activating mutations in PIK3CA cause generalized lymphatic anomaly[J]. J Exp Med, 2019, 216(2): 407-418. |
| [19] | Reinglas J, Ramphal R, Bromwich M. The successful management of diffuse lymphangio matosis using sirolimus: a case report[J]. Laryngoscope, 2011, 121(9): 1851-1854. |
| [20] | Sun X, Lu C, Huang Z, et al. Diagnosis and treatment of diffuse pulmonary lymphangioma in children: a case report[J]. Exp Ther Med, 2023, 25(4): 175. |
| [21] | Dimiene I, Bieksiene K, Zaveckiene J, et al. Effective initial treatment of diffuse pulmonary lymphangiomatosis with sirolimus and propranolol: a case report[J]. Medicina (Kaunas), 2021, 57(12): 1308. |
| [22] | Amodeo I, Colnaghi M, Raffaeli G, et al. The use of sirolimus in the treatment of giant cystic lymphangioma: four case reports and update of medical therapy[J]. Medicine (Baltimore), 2017, 96(51): e8871. |
| [23] | Ferrara N. Vascular endothelial growth factor: basic science and clinical progress[J]. Endocr Rev, 2004, 25(4): 581-611. |
| [24] | Onyeforo E, Barnett A, Zagami D, et al. Diffuse pulmonary lymphangiomatosis treated with bevacizumab[J]. Respirol Case Rep, 2018, 7(1): e00384. |
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