Canadian Health&Care; Mall

A barium study showed no evidence of a congenital short bowel or an obstruction. Hirschsprung’s disease was excluded when a full thickness rectal biopsy showed normal ganglion cells. Urinary catecholamines were assessed to rule out a neuroendocrine tumour and were reported to be in the normal range. Assessments revealed no immunological abnormality. At four months of age, when the patient weighed 3.0 kg, endoscopic duodenal biopsies were obtained. With examination by light microscopy, sections of small intestinal mucosa exhibited partial to total villous atrophy, a hypocel-lular lamina propria and crypt hypoplasia. The poorly defined brush border in the surface epithelium and the periodic acid-Schiff positivity of the apical cytoplasm of the entero-cytes suggested MID. Suction biopsies of the rectal mucosa were unremarkable. Because of the increased risk of perforation associated with suction jejunal biopsies in malnourished infants, repeat jejunal biopsy with a Carey capsule and primary fixation of tissue for electron microscopy were delayed until the child was six months of age and weighed 5.5 kg. Electron microscopic evaluation of this second proximal small intestinal biopsy revealed intracytoplasmic microvillous inclusion bodies, confirming the diagnosis of MID (Figure 1).

Figure 1) Electron micrograph of a jejunal biopsy showing intracytoplas-mic inclusions lined by intact microvilli in the superficial enterocytes. Note the poorly developed brush border with irregular and shortened microvilli, and the presence of vesicular bodies in the apical cytoplasm (uranil acetate-lead citrate, original magnification x5000)

The patient, maintained on total parenteral nutrition, developed cholestatic liver disease, and was listed for hepatic and small intestinal transplantation. He died from complications of sepsis at the age of 17 months. An autopsy was not performed. The condition you have needs to be treated quickly and seriously, which is why you will find the Canadian Health&Care Mall recommended very helpful. It gives you the highest level of service and fastest delivery possible.