Staging

Diagnostic studies should evaluate tissues along the presumed route of infection, which starts with bacterial invasion in the upper small intestine (Table 53-1). Beyond endoscopy, the draining mesenteric nodes and other retroperitoneal lymph nodes should be investigated by

TABLE 53-1. Proposal for Staging of Whipple's Disease

Stage Location of Documented Manifestation

Ia Small intestine

Ib Abdomincal lymph nodes

II Extraabdominal lymph nodes

III Extraabdominal orans (brain, heart, others)

abdominal sonography. Enlarged nodes with lipid deposits (which result in high echogenicity) are a special sign of intestinal WD. Physical examination should focus on the presence of peripheral lymphadenopathy, on evidence of cardiac involvement (pericardial effusion, valvular murmur), and also look for skin hyperpigmentation and evaluate body weight and temperature. Chest radiography should be performed, looking at heart diameter, possible pericardial calcifications, and mediastinal lymphadeno-pathy. Echocardiography is generally not helpful in patients without a murmur and normal chest radiography.

An essential part of staging is cerebrospinal fluid (CSF) analysis, even in the absence of neurological or psychiatric symptoms. One of the new insights gained during the 1990s was the recognition that, at the time of first diagnosis, approximately 70% of untreated patients with intestinal WD have asymptomatic ("silent") CNS infection with T. whippelii. These patients are considered to be at high risk of progressing from silent CNS infection to symptomatic CNS WD (or cerebral WD) unless the bacteria are eradicated. CSF analysis should be performed in parallel by means of polymerase chain reaction (PCR), which detects bacterial DNA, and by cytology of a large CSF sample which detects highly characteristic sickle-form particle containing cells. In practice, we recommand obtaining a 10 mL sample of CSF where possible; 8 mL of fresh CSF should be immediately prepared by cell concentration techniques for cytological study with PAS stain, while an aliquot of 2 mL should be fresh frozen and forwarded for PCR analysis. In our personal experience, the diagnostic yield of CSF cytology is roughly similiar with PCR analysis, but it is much less effective with samples < 5 mL (Von Herbay et al, 1997; Von Herbay, 2003). Imaging studies of the brain (computed tomography, magnetic resonance imaging) are generally not helpful in WD patients without neurological symptoms.

Constipation Prescription

Constipation Prescription

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