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奥文病患者需要胰腺手术:病例报告。

A patient with Owren disease requires pancreatic surgery: A case report.

机构信息

Department of Abdominal Surgery, Guiqian International Hospital, Guiyang City, China.

Department of Precise Medical Center, Guiqian International Hospital, Guiyang City, China.

出版信息

Medicine (Baltimore). 2023 Dec 15;102(50):e36562. doi: 10.1097/MD.0000000000036562.

Abstract

RATIONALE

Coagulation factor V deficiency is rare, and perioperative management of patients with this condition is particularly important, especially during major abdominal surgery. We present a case of a patient with pancreatic duct stones combined with coagulation factor V deficiency. We share our perioperative management experience.

PATIENT CONCERNS

A 31-year-old man presented with recurrent upper abdominal pain for 2 years.

DIAGNOSES

The diagnosis of pancreatic duct stones in the patient has been established through abdominal computed tomography and magnetic resonance imaging examinations. The diagnosis of factor V deficiency was initially identified through coagulation function tests, revealing significant prolongation of both aPTT and PT. Subsequent testing of coagulation factors and inhibitors demonstrated that the patient has a deficiency in coagulation factor V. Finally, genetic testing revealed that the factor V deficiency in this case is hereditary.

INTERVENTIONS

The patient underwent a partial resection of the pancreatic head, and FFP was infused 1 hour before surgery. 600 mL of FFP was instilled 1 hour before the start of surgery along with 10 U of cryoprecipitate. and 600 ml of FFP were added during surgery. Postoperative treatment included intermittent FFP supplemental infusion in the first 5 days after surgery while monitoring the coagulation function.

OUTCOMES

The patient underwent a successful surgery without any abnormal bleeding or oozing during the procedure. The postoperative recovery was smooth, with no abnormal bleeding.

LESSONS

Patients with a deficiency of coagulation factor V are not contraindicated for surgery. Appropriate Fresh Frozen Plasma (FFP) replacement therapy can ensure the safe conduct of the surgical procedure. For patients with abnormal blood coagulation function, we recommend testing for coagulation factors and inhibitors, as well as performing genetic testing for abnormal coagulation factors, which can provide guidance on marriage and childbirth.

摘要

背景

凝血因子 V 缺乏症较为罕见,此类患者的围手术期管理尤其重要,尤其是在进行大型腹部手术时。我们报告了一例胰腺管结石合并凝血因子 V 缺乏症的患者,并分享了我们的围手术期管理经验。

病例介绍

一名 31 岁男性,因反复发作性上腹痛 2 年来就诊。

诊断

腹部 CT 和磁共振成像检查明确了患者胰腺管结石的诊断。通过凝血功能检查初步诊断为凝血因子 V 缺乏症,发现 aPTT 和 PT 均显著延长。随后检测凝血因子和抑制剂,发现患者存在凝血因子 V 缺乏症。最终,基因检测显示该病例的凝血因子 V 缺乏症为遗传性的。

干预措施

患者接受了胰头部分切除术,在手术前 1 小时输注了 FFP。手术前 1 小时,共输注了 600mlFFP 和 10U 冷沉淀,手术期间还输注了 600mlFFP。术后治疗包括在术后前 5 天间歇性补充 FFP,并监测凝血功能。

结果

患者手术顺利,术中无异常出血或渗血。术后恢复顺利,无异常出血。

结论

凝血因子 V 缺乏症患者并非手术禁忌证。适当的新鲜冷冻血浆(FFP)替代治疗可以确保手术的安全进行。对于凝血功能异常的患者,建议检测凝血因子和抑制剂,并对异常凝血因子进行基因检测,这可以为婚姻和生育提供指导。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2b6e/10727639/aed0c00b51ee/medi-102-e36562-g001.jpg

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