کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
4309804 1289324 2008 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
The influence of surgery in MEN-1 syndrome: Observations over 150 years
موضوعات مرتبط
علوم پزشکی و سلامت پزشکی و دندانپزشکی عمل جراحی
پیش نمایش صفحه اول مقاله
The influence of surgery in MEN-1 syndrome: Observations over 150 years
چکیده انگلیسی

BackgroundEfficacy and timing of operative intervention in patients with multiple endocrine neoplasia type 1 (MEN-1) syndrome remains controversial. This report utilizes a novel approach to evaluate the influence of evolving operative interventions for patients with MEN-1 syndrome.MethodsSix generations from a large MEN-1 family pedigree were studied. The number of operations for MEN-1 related pathology was recorded according to birth eras over 150 years. Length of life was a primary outcome measurement.ResultsInheritance of the MEN-1 trait was near 50%. There were no instances of a skipped generation. Affected individuals born before 1900 died from gastrointestinal hemorrhage and without any surgical intervention. After 1900, there were increasing numbers of gastric, parathyroid, and pancreatic operations in successive eras. Death occurred >20 years earlier in MEN-1 individuals than unaffected family members in eras 1 and 2. Family members with MEN-1 lived longer in succeeding eras with increasing number of operative and pharmacologic interventions.ConclusionMEN-1 family members invariably have pathologic changes in pituitary, parathyroid, and pancreatic islets when long lived, the “all-or-none” phenomenon. Patients are not cured with operative interventions, although they may live longer and without symptoms with a good quality of life. This model may allow better comparisons with other MEN-1 patients when evaluating outcomes of new medical and operative management schemes and long-term follow-up.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Surgery - Volume 144, Issue 4, October 2008, Pages 695–702
نویسندگان
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