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150篇 您的检索式:作者名="WEN DR"
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1Infant cholestasis patient with a novel missense mutation in the AKR1D1 gene successfully treated by early adequate supplementation with chenodeoxycholic acid: A case report and review of the literature显示文摘Steroid 5β-reductase [aldo-keto reductase family 1 member D1(AKR1D1)] is essential for bile acid biosynthesis. Bile acid deficiency caused by genetic defects in AKR1D1 leads to life-threatening neonatal hepatitis and cholestasis. There is still limited experience regarding the treatment of this disease. We describe an infant who presented with hyperbilirubinemia and coagulopathy but normal bile acid and γ-glutamyltransferase. Gene analysis was performed using genomic DNA from peripheral lymphocytes from the patient, his parents, and his elder brother. The patient was compound heterozygous for c.919C>T in exon 8 and exhibited a loss of heterozygosity of the AKR1D1 gene, which led to an amino acid substitution of arginine by cysteine at amino acid position 307(p.R307C). Based on these mutations, the patient was confirmed to have primary 5β-reductase deficiency. Ursodeoxycholic acid(UDCA) treatment did not have any effect on the patient. However, when we changed to chenodeoxycholic acid(CDCA) treatment, his symptoms and laboratory tests gradually improved. It is therefore crucial to supplement with an adequate dose of CDCA early to improve clinical symptoms and to normalize laboratory tests.Hui-Hui Wang Fei-Qiu Wen Dong-Ling Dai Jian-She Wang Jing Zhao Kenneth DR Setchell Li-Na Shi Shao-Ming Zhou Si-Xi Liu Qing-Hua Yang 2018World Journal of Gastroenterology2018,24,35:3
2Technical details of intraoperative lymphatic mapping for early stage melanoma 显示文摘Morton DL Wen DR Wong J H 1992Arch Surg1992,127,3:1
3Technical details of intraopenative lymphatic mapping for early stage melanoma显示文摘Morton DL Wen DR Wong J H 1992Arch Sura1992,127,4:1
4Technical details of intraoperation lymphatic mapping for early stage melanoma显示文摘Morton DL Wen DR Wong JH 1992Arch Surg1992,127,:1
5Arsenic trioxidereduces the invasive and metastatic properties of nasopharyngeal carcinoma cells in vitro显示文摘Du CW Wen BG Li DR 2006Braz J Med Biol Res2006,39,5:1
6Technical details of intraoperative lymphatic mapping for early stage melanoma 显示文摘Morton DI Wen DR Wong JH 1992Arch Surg1992,127,4:1
7Technical details of intraoperative lymphatic mapping for early stage melanoma显示文摘Morton DL Wen DR Wong JH 1992Arch Surg1992,127,4:1
8Updated response assessment criteria for high-grade gliomas: response assessment in neuro-oncology working group 显示文摘Wen PY Macdonald DR Reardon DA 2010J Clin Oncol2010,28,11:1
9Technical details of intraoperative lymphatic mapping for early stage melanoma 显示文摘Morton DL Wen DR Wang JH 1992Arch Surg1992,127,:1
10Technical details of intraoperative lymphatic mapping for early stage melanoma 显示文摘 Wen DR Wong JH 1992Arch Surg1992,127,4:1
11Technical details of intraoperative lymphatie mapping for early stage melanoma 显示文摘Morton DL Wen DR Wong JH 1992Arch Surg1992,127,4:1
12Management of the early stage melanoma by intraoperative lymphatic mapping and selective lymphadenectomy显示文摘Morton DL Wen DR Cochran 1992Surg Oncol Clin North Am1992,1,:1
13Arsenic trioxide reduces the invasive and metastatic properties of nasopharyngeal carcinoma cells in vitro 显示文摘Du Cw Wen BG Li DR 2006Braz J Med Biol Res2006,39,5:1
14Technical details of intraoperative lymphatic mapping for early stage melanoma 显示文摘Morton DL Wen DR Wong JH 1992Arch Surg1992,127,:1
15Updated response assessment criteria for high-grade gliomas: response assessment in neuro-oncology working group显示文摘Wen PY Macdonald DR Reardon DA 2010J Clin Oneol2010,28,:1
16Technical details of intraoperative lymphatic mapping for early-stage melanoma 显示文摘Morton DL Wen DR Wong JH 1992Arch Surg1992,127,4:1
17Paragangliomas:Assessment of prognosis by histologic,immunohistochemical,and ultrastructural techniques显示文摘Kliewer KE Wen DR Cancilla PA 1989Hum Pathol1989,20,1:1
18Technical details ofintraoperative lymphatic mapping for early stagemelanoma显示文摘Morton DL Wen DR’Wong JH 1992Arch Surg1992,127,4:1
19Proteomic identification of potential susceptibility factors in drug-induced liver disease 显示文摘Welch KD Wen B Goodlett DR 2005Chem Res Toxicol2005,18,6:1
20Technical details of intra- operative lymphatic mapping for early stage melanoma 显示文摘Morton DL Wen DR Wong JH L 1992Arch Surg1992,127,4:1
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