Fabry disease; early diagnosis improves prognosis but diagnosis is often delayed.

Background: Fabry disease (FD) is a rare X-linked deficiency of lysosomal enzyme alpha-galactosidase (AGAL) resulting in accumulation of globotriaosylceramide (Gb-3) in the cells, with protean manifestations. Major organs affected are the kidneys, heart and nervous system. The diagnosis of FD is often delayed by many years.

Dietary iron enhances colonic inflammation and IL-6/IL-11-Stat3 signaling promoting colonic tumor development in mice.

Chronic intestinal inflammation and high dietary iron are associated with colorectal cancer development. The role of Stat3 activation in iron-induced colonic inflammation and tumorigenesis was investigated in a mouse model of inflammation-associated colorectal cancer. Mice, fed either an iron-supplemented or control diet, were treated with azoxymethane and dextran sodium sulfate (DSS).

Indomethacin and retinoic acid modify mouse intestinal inflammation and fibrosis: a role for SPARC.

The mouse model of 2,4,6-Trinitrobenzene Sulfonic Acid (TNBS)-induced intestinal fibrosis allows for detailed study of the extracellular matrix changes that complicate Crohn's disease. Indomethacin induces intestinal fibrosis, while retinoic acid (RA) reduces liver fibrosis. Secreted protein acidic and rich in cysteine (SPARC), an extracellular matrix-modifying agent, may potentially link these opposing effects.

Cytomegalovirus infection of a cutaneous ulcer in a patient with ANCA-positive vasculitis.

This case describes a patient in whom cytomegalovirus (CMV) infected a preexisting ulcer. The patient was immune-suppressed because of treatment for Wegener's granulomatosis. Specific antiviral therapy was delayed because of uncertainty as to the role of CMV, but the infection cleared and the ulcer improved promptly on institution of valganciclovir.

Gangliocytic paraganglioma of the bronchus: a case report with follow-up and ultrastructural assessment.

We report a case of gangliocytic paraganglioma of bronchus. A 54-year-old woman underwent bronchoscopy following two episodes of right lower lobe pneumonia over the previous 5 months with unresolved chest radiographic changes. A computerized tomographic scan showed a right lower lobe endobronchial lesion, and at bronchoscopy there was a mass partly occluding the lumen of the bronchus.