Đánh giá kết quả điều trị dài hạn sau 10 năm ở trẻ em cường insulin bẩm sinh tại Bệnh viện Nhi đồng 2

0 Người đánh giá. Xếp hạng trung bình 0

Tác giả: Tường Vy Dương, Thị Diễm Thúy Hoàng, Thị Vũ Quỳnh Huỳnh, Khoa Bình Minh Nguyễn, Văn Nhàn Nguyễn, Thị Mai Anh Phạm, Hữu Tùng Trịnh, Thị Kim Huệ Trịnh, Đình Khải Trương, Thị Phương Uyên Trương

Ngôn ngữ: vie

Ký hiệu phân loại: 616.462 Diabetes mellitus

Thông tin xuất bản: Tạp chí Y học Việt Nam (Tổng hội Y học Việt Nam), 2022

Mô tả vật lý: 46-53

Bộ sưu tập: Metadata

ID: 500517

Thêm vào giỏ

Đánh giá kết quả điều trị dài hạn sau 10 năm ở trẻ em cường insulin bẩm sinh tại bệnh viện Nhi Đồng 2 từ năm 2012 đến 2022. Phương pháp nghiên cứu: Nghiên cứu hồi cứu và tiến cứu mô tả hàng loạt ca. Kết quả: Từ tháng 01/2012 đến tháng 01/2022 tại bệnh viện Nhi đồng 2 có 36 bệnh nhân (BN) được chẩn đoán cường insulin bẩm sinh (CIBS). Theo dõi kết quả điều trị sau 4,4 ± 2,2 năm (1,4 - 8 năm), tỉ lệ BN khởi trị Diazoxide là 63,89%, trong đó có 39,13% BN đáp ứng, 62,5% có đột biến gen không liên quan kênh KATP. 88,89% BN ngưng thuốc sau 24,44 ± 14,01 tháng, 55,56% BN có tác dụng phụ của thuốc bao gồm phù, giữ muối nước, rậm lộng, chàm da, loét miệng, giảm bạch cầu hạt. Trong nhóm BN điều trị Octreotide, có 66,67% BN đáp ứng, 55,56% BN ngưng thuốc sau 24,08 ± 20,46 tháng, 22,22% BN có tác dụng phụ nôn ói. Ở nhóm BN được phẫu thuật cắt tụy, 22,22% BN còn hạ đường huyết, 66,67% BN đường huyết bình thường, 11,11% BN tiến triển thành đái tháo đường sau 8 năm. Về chức năng tụy ngoại tiết sau cắt tụy, 22,22% BN giảm men tụy (amylase, lipase) không triệu chứng. Tỷ lệ BN có chậm phát triển về tâm thần vận động trí tuệ là 60%. Có 24% BN động kinh, trong đó có 16,67% BN có bất thường não trên MRI. Kết luận: Việc đánh giá đáp ứng điều trị lâu dài là vô cùng cần thiết giúp ngăn ngừa tác dụng phụ của điều trị, những biến chứng của phẫu thuật, và những tổn thương thần kinh do tình trạng hạ đường huyết kéo dài.Evaluation of the long-term treatment outcomes over a 10-year period in patients with congenital hyperinsulinemia at Children's Hospital 2 from 2012 to 2022. Method: Retrospective and prospective case series. Results: From January 2012 to January 2022 at Children's Hospital 2 there were 36 cases diagnosed with congenital hyperinsulinemia (CHI). Follow-up after an average of 4.4 ± 2.2 years (1.4-8 years), 63.89% of patients was treated by Diazoxide for the first line, of which 39.13% responded, 62.5% had mutations unrelated to the KATP channel. 88.89% of patients discontinued after 24,44 ± 14,01 months, 55.56% of patients had side effects including edema, fluid retention, hyperemia, eczema, mouth ulcers, agranulocytosis. For Octreotide, there were 66.67% of patients responded to treatment, of which 55.56% discontinued after 24.08 ± 20.46 months, 22.22% had vomiting. In the group of patients undergoing surgery, 22.22% had hypoglycemia, 66.67% had normal blood glucose, 11.11% lead to diabetes after 8 years. For exocrine pancreatic function, 22.22 % of patients had pancreatic enzyme deficiency (amylase, lipase) without clinical symptoms. The rate of patients had neurodevelopmental problems is 60%. Epilepsy was seen in 24% of patients, of which one (16.67%) had abnormal brain MRI. Conclusion: Assessing the long-term response to treatment is essential to prevent medical side effects, surgical complications, and neurological damages from prolonged hypoglycemia.

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