from Part VII - Clinical syndromes: gastrointestinal tract, liver, and abdomen
Published online by Cambridge University Press: 05 April 2015
In 1907, Dr. George H. Whipple, a pathologist at Johns Hopkins Hospital, described the first case of “intestinal lipodystrophy” in a 36-year-old male physician with symptoms of chronic diarrhea, abdominal pain, weight loss, and chronic cough. The patient succumbed to his death after 5 years of his disease. Universally fatal prior to the advent of antibiotics, the condition now known as Whipple’s disease has accumulated case reports and case series with a recent prospective study in the management of the disease. This rare disease masquerades as a multisystem condition of symptoms that are nonspecific, rendering the diagnosis inaccessible unless included in the differential diagnosis. Based on the most recent prospective study, the disease is well managed with appropriate attention to diagnosis and antibiotic therapy.
After the initial case report by Dr. Whipple, progress was made towards establishing the means of diagnosis and treatment. In 1949, periodic acid–Schiff (PAS) staining helped identify the red appearance of glycoproteins within intestinal macrophages. Shortly afterwards, the first microscopic identification of a bacteria-like organism led to the first successful treatment of Whipple’s disease with chloramphenicol. Electron microscopy and the advancement of histologic staining helped further characterize the infectious entity as a gram-positive bacterium. In 1992, polymerase chain reaction (PCR) was used to identify the ribosomal RNA of the organism, classifying it as an actinomycete. Genomic sequencing of the organism in 2003 has brought us to the current classification of this rod-shaped, gram-positive actinomycete as Tropheryma whipplei.
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