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弥漫性内生桥脑胶质瘤患者的诊断、治疗和护理趋势:来自巴基斯坦一家三级护理医院的观点。

The trends in diagnosis, management, and care of patients with diffuse intrinsic pontine gliomas: Perspectives from a tertiary care hospital of pakistan.

机构信息

Medical College, Aga Khan University, Karachi, 74800, Pakistan.

Department of Oncology, Aga Khan University Hospital, Karachi, 74800, Pakistan.

出版信息

Childs Nerv Syst. 2024 Nov;40(11):3537-3544. doi: 10.1007/s00381-024-06637-9. Epub 2024 Sep 30.

DOI:10.1007/s00381-024-06637-9
PMID:39349775
Abstract

BACKGROUND

Diffuse intrinsic pontine glioma (DIPG) primarily affects pediatric patients. Data on the global incidence of DIPG remain sparse, especially in South Asia and low-middle-income countries like Pakistan.

METHODS

After exemption from the Ethics Review Committee, a retrospective study was conducted. Records of patients with DIPG at the Aga Khan Hospital in Karachi, from January 2010 to December 2022, were reviewed.

RESULTS

A total of 35 pediatric patients were managed for DIPG. The median age of the patients was 9, with 19 (54.3%) males and 16 (45.7%) females. Cranial nerve palsies were the most common complaint and were present in 19 (54.3%) patients, followed by headaches in 18 (51.4%), long tract signs in 14 (40%), ataxia/cerebellar symptoms in 14 (40%), and seizures in 5 (14.3%). MRI was the primary diagnostic tool, used alone or with CT in 32 (94.1%) patients; CT alone was used in only 2 (5.7%) patients. Biopsy was performed in 10 (28.6%) patients. Primary radiation therapy was administered to 14 (40%) patients with 5400 cGy in 30 fractions. All these patients received steroids while none of them received reirradiation. VP shunt surgery for hydrocephalus was performed in 9 (25.7%) patients. Over half (54.3%) refused treatment post-diagnosis, and 71.4% were lost to follow-up.

CONCLUSION

Providing timely, quality multi-disciplinary care to DIPG patients within resource constraints remains challenging in Pakistan. However, recent developments show promise for improving DIPG care in the country.

摘要

背景

弥漫性内在脑桥胶质瘤(DIPG)主要影响儿科患者。关于 DIPG 的全球发病率数据仍然很少,特别是在南亚和巴基斯坦等中低收入国家。

方法

在获得伦理审查委员会豁免后,进行了一项回顾性研究。回顾了 2010 年 1 月至 2022 年 12 月在卡拉奇 Aga Khan 医院就诊的 DIPG 患者的记录。

结果

共管理了 35 名儿科患者的 DIPG。患者的中位年龄为 9 岁,其中 19 名(54.3%)为男性,16 名(45.7%)为女性。颅神经麻痹是最常见的主诉,19 例(54.3%)患者存在颅神经麻痹,其次是头痛 18 例(51.4%)、长束征 14 例(40%)、共济失调/小脑症状 14 例(40%)和癫痫发作 5 例(14.3%)。磁共振成像(MRI)是主要的诊断工具,单独使用或与 CT 一起使用 32 例(94.1%)患者;仅单独使用 CT 的患者有 2 例(5.7%)。10 例(28.6%)患者进行了活检。14 例(40%)患者接受了原发性放射治疗,剂量为 5400cGy,分 30 次进行。所有这些患者都接受了类固醇治疗,而没有人接受再放疗。9 例(25.7%)脑积水患者接受了脑室-心房分流术。超过一半(54.3%)患者在诊断后拒绝治疗,71.4%的患者失访。

结论

在资源有限的情况下,向 DIPG 患者提供及时、高质量的多学科护理仍然具有挑战性。然而,最近的发展表明,有希望改善该国的 DIPG 护理。

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本文引用的文献

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Capacity building for pediatric neuro-oncology in Pakistan- a project by my child matters program of Foundation S.巴基斯坦小儿神经肿瘤学的能力建设——S基金会“我的孩子至关重要”项目
Front Oncol. 2024 Feb 29;14:1325167. doi: 10.3389/fonc.2024.1325167. eCollection 2024.
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Hypofractionated re-irradiation for diffuse intrinsic pontine glioma.弥漫性内生桥脑胶质瘤的分割再放疗。
Pediatr Blood Cancer. 2024 May;71(5):e30929. doi: 10.1002/pbc.30929. Epub 2024 Mar 2.
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Pakistan National Guidelines for Pediatric High-Grade Gliomas.《巴基斯坦儿童高级别胶质瘤国家指南》
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Treatment Limitations for Pediatric Diffuse Intrinsic Pontine Gliomas in a Middle-Income Country.中等收入国家小儿弥漫性脑桥内在型胶质瘤的治疗局限性
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Building the ecosystem for pediatric neuro-oncology care in Pakistan: Results of a 7-year long twinning program between Canada and Pakistan.在巴基斯坦建立儿科神经肿瘤护理生态系统:加拿大和巴基斯坦长达 7 年的双胞胎项目的结果。
Pediatr Blood Cancer. 2022 Sep;69(9):e29726. doi: 10.1002/pbc.29726. Epub 2022 Apr 28.
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Development of Palliative Care Services at a Tertiary Care Teaching Hospital in Pakistan: Retrospective Analysis of Existing Palliative Care Program.巴基斯坦一所三级护理教学医院姑息治疗服务的发展:现有姑息治疗方案的回顾性分析。
J Pain Symptom Manage. 2022 Aug;64(2):178-185. doi: 10.1016/j.jpainsymman.2022.03.018. Epub 2022 Apr 18.
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Diffuse intrinsic pontine glioma: current insights and future directions.弥漫性脑桥内生型胶质瘤:当前见解与未来方向
Chin Neurosurg J. 2021 Jan 11;7(1):6. doi: 10.1186/s41016-020-00218-w.
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Clinical, imaging, and molecular analysis of pediatric pontine tumors lacking characteristic imaging features of DIPG.对缺乏 DIPG 典型影像学特征的儿童脑桥肿瘤的临床、影像学和分子分析。
Acta Neuropathol Commun. 2020 Apr 23;8(1):57. doi: 10.1186/s40478-020-00930-9.
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Biopsy in diffuse pontine gliomas: expert neurosurgeon opinion-a survey from the SIOPE brain tumor group.弥漫性脑桥胶质瘤的活检:神经外科专家意见——来自SIOPE脑肿瘤组的一项调查
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Childs Nerv Syst. 2020 Apr;36(4):697-704. doi: 10.1007/s00381-019-04463-y. Epub 2019 Dec 17.