Whipple's disease was initially described in 1907. Over the next century, the clinical and pathological features of this disorder have been better appreciated. Most often, weight loss, diarrhea, abdominal and join...Whipple's disease was initially described in 1907. Over the next century, the clinical and pathological features of this disorder have been better appreciated. Most often, weight loss, diarrhea, abdominal and joint pain occur. Occasionally, other sites of involvement have been documented, including isolated neurological disease, changes in the eyes and culture-negative endocarditis. In the past decade, the responsible organism Tropheryma whipplei has been cultivated, its genome sequenced and its antibiotic susceptibility defined. Although rare, it is a systemic infection that may mimic a wide spectrum of clinical disorders and may have a fatal outcome. If recognized, prolonged antibiotic therapy may be a very successful form of treatment.展开更多
We report the first known case of both Noonan syndrome and Whipple's disease occurring in the same patient.A 36-year-old female with history of Noonan syndrome developed fatigue,anorexia,arthritis of the knees and...We report the first known case of both Noonan syndrome and Whipple's disease occurring in the same patient.A 36-year-old female with history of Noonan syndrome developed fatigue,anorexia,arthritis of the knees and hands with a diffuse hyperpigmented rash,night sweats,and an unintentional fifteen pound weight loss over 4 mo.Small bowel enteroscopy demonstrated mild edematous yellowish mucosa without friability.Random small bowel biopsies revealed extensive periodic acid-Schiff positive material within the foamy macrophages.She was treated with a 12 mo course of trimethoprim-sulfamethoxazole DS with clinical improvement to baseline status.展开更多
文摘Whipple's disease was initially described in 1907. Over the next century, the clinical and pathological features of this disorder have been better appreciated. Most often, weight loss, diarrhea, abdominal and joint pain occur. Occasionally, other sites of involvement have been documented, including isolated neurological disease, changes in the eyes and culture-negative endocarditis. In the past decade, the responsible organism Tropheryma whipplei has been cultivated, its genome sequenced and its antibiotic susceptibility defined. Although rare, it is a systemic infection that may mimic a wide spectrum of clinical disorders and may have a fatal outcome. If recognized, prolonged antibiotic therapy may be a very successful form of treatment.
文摘We report the first known case of both Noonan syndrome and Whipple's disease occurring in the same patient.A 36-year-old female with history of Noonan syndrome developed fatigue,anorexia,arthritis of the knees and hands with a diffuse hyperpigmented rash,night sweats,and an unintentional fifteen pound weight loss over 4 mo.Small bowel enteroscopy demonstrated mild edematous yellowish mucosa without friability.Random small bowel biopsies revealed extensive periodic acid-Schiff positive material within the foamy macrophages.She was treated with a 12 mo course of trimethoprim-sulfamethoxazole DS with clinical improvement to baseline status.