گزارش یک مورد سندرم بودکیاری با تظاهر سندرم هلپ

نوع مقاله : گزارش مورد

نویسندگان

1 استادیار گروه زنان و مامایی، مرکز تحقیقات زایمان، دانشکده پزشکی، دانشگاه علوم پزشکی کرمانشاه، کرمانشاه، ایران.

2 کارشناس ارشد بیوشیمی، دانشکده داروسازی، دانشگاه علوم پزشکی کرمانشاه، کرمانشاه، ایران.

3 دانشیار گروه داخلی، دانشکده پزشکی، دانشگاه علوم پزشکی کرمانشاه، کرمانشاه، ایران.

چکیده

مقدمه: سندرم بودکیاری، بیمارى نادرى است که ناشى از انسداد وریدهاى خروجى کبد است. علت اصلى در اکثر موارد، ترومبوز خودبه‌خودى وریدهاى کبدى است که غالباً با پلى ‌سیتمى حقیقى یا سابقه مصرف قرص‌هاى ضد باردارى خوراکى نیز همراه است. برخی بیماران با تنگی غشایى ایدیوپاتیک در ورید اجوف تحتانی، در فاصله بین وریدهاى کبدى و دهلیز راست مراجعه مى‌کنند که معمولاً با ترومبوز یک یا هر دو ورید کبدى همراه است. ابتدا تصور مى‌شد که این ضایعات مادرزادى هستند ولى امروزه مشخص شده که این ضایعات از تنگى‌ ناشى از ترومبوز و تشکیل اسکار ناشى مى‌شوند. انسداد پس از کبد، فشار سینوزوئیدها را بالا مى‌برد و نهایتاً باعث پرفشارى ورید پورت مى‌شود. علائم معمولاً در ابتدا خفیف و عمومى هستند و شامل درد شکمى مبهم در ربع فوقانى راست، نفخ بعد از غذا و بى‌اشتهایی می باشند. پس از چند هفته یا چند ماه، تصویر بالینى واضح‌ترى شامل تب شدید، آسیت بالینی، بزرگی کبد و نارسایى کبد بروز مى‌کند. تشخیص بالینى با ونوگرافى تأیید می شود.
گزارش مورد:در این گزارش، یک مورد سندرم بودکیاری به دنبال زایمان در یک خانم 22 ساله معرفی می شود. این بیمار به دنبال عمل سزارین با علائم آسیت، تب شدید و فشار خون بالا، به دلیل مشکلات و تأخیر در تشخیص، مورد توجه قرار گرفت. به دنبال انجام همزمان ونوگرافی و بیوپسی کبد، ترمبوز ورید کبدی گزارش و سندرم بودکیاری مطرح شد.
نتیجه گیری: متخصصین زنان و داخلی بهتر است که به سندرم بودکیاری بعنوان یکی از علل نادر استمرار تب و آسیت پس از ختم بارداری توجه ویژه داشته باشند..

کلیدواژه‌ها

عنوان مقاله [English]

A Case Report of Budd-Chiari Syndrome with HELLP Syndrome Symptoms

نویسندگان [English]

  • Nasrin Jalilian 1
  • Elaheh Alizadeh 2
  • Mahdi Zobeiri 3
1 Assistant Professor, Department of Obstetrics and Gynecology, Health Services Research Center of Obstetrics, School of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran.
2 M.Sc. of Biochemistry, School of Pharmacy, Kermanshah University of Medical Sciences, Kermanshah, Iran.
3 Associate Professor, Department of Internal Medicine, School of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran.
چکیده [English]

Introduction: Budd-Chiari Syndrome is a rare disease primarily determined by the hepatic veins thrombosis. Spontaneous thrombosis of the hepatic veins is the main reason, but polycythemia vera and a history of using oral contraceptive pills can be associated with thrombosis. Some patients refer with idiopathic membranous obstruction of the inferior vena cava at the distance between hepatic veins and right atrium which is usually associated with hepatic vein thrombosis of one or both hepatic veins. Initially it was thought that these lesions are congenital, but it is now clear that these lesions result from stenosis due to thrombosis and scar formation. Obstruction after the liver increases sinusoids pressure and eventually portal vein hypertension occurs. Symptoms are usually mild and general at first and include abdominal pain of the right upper quadrant, bloating and anorexia. After a few weeks or months, clinical manifestations presented as sever fever, clinical ascites, hepatomegaly and hepatic necrosis. Venography is one of the ways of diagnosis.
Case Report: A 22 years old woman is reported with Budd-Chiari syndrome which started after delivery. The patient was considered following the cesarean section because of ascites symptoms, high fever and high blood pressure due to delays in diagnosis.
After conducting ultrasonography and liver biopsy simultaneously, hepatic vein thrombosis was reported and Budd-Chiari syndrome was diagnosed.

کلیدواژه‌ها [English]

  • Ascites
  • Budd-Chiari Syndrome
  • Hepatic Veins
  • pregnancy

مراجع

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مجله زنان، مامایی و نازایی ایران
دوره 17، شماره 107 - شماره پیاپی 107
هفته اول مرداد ماه 1393
مرداد 1393
صفحه 15-20

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