Esther Lamoureux

Meta-analysis of Outcome of Cytomegalovirus Colitis in Immunocompetent Hosts

There are only a few anecdotal reports of cytomegalovirus (CMV) colitis in immunocompetent hosts. The impact of the disease in this patient population remains poorly understood. The aim of this study was to perform a meta-analysis using individual patient data to determine outcomes of CMV colitis in immunocompetent patients and identify risk factors that might influence prognosis. A literature search was performed from 1980 to 2003 looking for immunocompetent patients with CMV colitis. Immunocompetence was defined as absence of congenital or acquired immune deficiency, transplant, or immunosuppressive medication. Patients were divided by age (< 55 versus ≥55) and grouped according to coexisting illnesses. Kaplan–Meier curves were plotted to assess survival. Variables included age, sex, site of acquisition of infection, extent of disease, coexisting illnesses, and treatment modality. A total of 44 patients were identified, with an average age of 61.1. Only 10 were free of any comorbidity. The mean follow-up was 13.4 months. Spontaneous remission occurred in 31.8%, mostly individuals < 55 years old. Fourteen deaths occurred, all of which were in patients ≥55. There was a higher mortality rate among male patients ≥55 (56.9%; P = 0.08), patients with immune-modulating diseases (75.2%; P = 0.10), and those having a colectomy (68.9%; P = 0.09). This analysis underlines the rarity of CMV colitis in patients with an intact immune system. Advanced age, male gender, presence of immune-modulating comorbidities, and need for surgical intervention are factors negatively influencing survival. Conversely, young healthy patients have a good prognosis with no intervention.

Lung scintigraphy in pulmonary capillary hemangiomatosis. A rare disorder causing primary pulmonary hypertension.

The authors report the results of lung scintigraphy in two patients with primary pulmonary hypertension caused by pulmonary capillary hemangiomatosis, a rare disorder resulting from the proliferation of histologically normal capillaries in the lung. Perfusion studies revealed a non-homogeneous pattern with some focal defects, similar to that seen with some other histologic types of primary pulmonary hypertension. The mechanisms underlying this perfusion pattern are unknown.

Colonic obstruction secondary to sarcoidosis: nonsurgical diagnosis and management.

A 57-year-old black man presented with a 2-week history of abdominal pain, weight loss, anorexia, and constipation. His history was significant for remote Hodgkins disease and systemic sarcoidosis. Physical examination showed abdominal distention and hyperactive bowel sounds, periorbital swelling, and mandibular lymphadenopathy. A barium enema examination showed two high-grade obstructive lesions in the rectum and splenic flexure. Colonoscopy confirmed the presence of the two areas of colonic obstruction. The mucosa showed diffuse fine ulcerations in the areas of obstruction as well as in the intervening region. Endoscopic biopsy specimens showed numerous mucosal noncaseating granulomas but no acid-fast bacilli or foreign bodies. The patient was treated with oral prednisone and improved symptomatically within 3 days. The ocular lesions and lymphadenopathy also responded promptly. Findings of follow-up barium enema and colonoscopy performed after 1 month of steroid treatment were essentially normal. Mucosal biopsy specimens showed only mild nonspecific chronic inflammation of the lamina propria and no granulomas. Colonic involvement is rarely reported with systemic sarcoidosis. We believe that this is the first report of colonic obstruction due to sarcoid diagnosed endoscopically and managed nonsurgically.

Case Report: Fatal Fulminant Hepatic Failure due to Cyproterone Acetate

Cyproterone acetate is a normally well-tolerated drug that is used widely for the treatment of prostatic carcinoma. Liver toxicity due to its use is not well known. We describe two cases of fatal fulminant hepatitis related to the use of cyproterone acetate.

Occult dysplasia in a localized giant pseudopolyp in Crohn's colitis: A case report

Localized giant pseudopolyposis of the colon (pseudopolyp larger than 1.5 cm in size) is a rare complication of inflammatory bowel disease. There is one report of an occult carcinoma within such a lesion, and no reports of sole dysplasia. A case of a 42-year-old man with longstanding Crohns colitis who underwent a colonoscopy revealing a large, multilobulated mass at the splenic flexure that was not amenable to endoscopic removal, is described. Multiple biopsies showed no dysplasia and histology was consistent with an inflammatory pseudopolyp. Computed tomographic colonography demonstrated a mass resembling a large villous tumour. A decision for surgery was made. The surgical specimen was a complex anastomosing inflammatory pseudopolyp 5 cm x 4 cm x 3 cm in size, with a focus of low-grade dysplasia in an area free of inflammation. The present case is the first reported occult dysplasia in a giant pseudopolyp. Occult dysplasia without superficial dysplasia may exist in these lesions and further studies are needed to examine risk factors that make a giant pseudopolyp more likely to harbour dysplasia and/or carcinoma.

Autistic enterocolitis: fact or fiction?

Autism spectrum disorder refers to syndromes of varying severity, typified by impaired social interactions, communicative delays and restricted, repetitive behaviours and interests. The prevalence of autism spectrum disorders has been on the rise, while the etiology remains unclear and most likely multifactorial. There have been several reports of a link between autism and chronic gastrointestinal symptoms. Endoscopy trials have demonstrated a higher prevalence of nonspecific colitis, lymphoid hyperplasia and focally enhanced gastritis compared with controls. Postulated mechanisms include aberrant immune responses to some dietary proteins, abnormal intestinal permeability and unfavourable gut microflora. Two autism spectrum disorder patients with chronic intestinal symptoms and abnormal endoscopic findings are described, followed by a review of this controversial topic.

Effects of vascular endothelial growth factor on endothelin-1 production by human lung microvascular endothelial cells in vitro.

AIMS Increased endothelin-1 (ET-1) is a hallmark of pulmonary arterial hypertension (PAH), and contributes to its pathogenesis. The factors controlling ET-1 in PAH are poorly understood. Combined with other stimuli, vascular endothelial growth factor (VEGF) blockade results in PAH-like lesions in animal models, and has been associated with PAH in humans. The effects of VEGF on ET-1 production by human lung blood microvascular endothelial cells (HMVEC-LBl) are unknown. MAIN METHODS We exposed HMVEC-LBl in-vitro to human VEGF-121 (40 ng/mL) in serum-free medium for 7h, in the absence or presence of the VEGF receptor antagonist, SU5416 (3 and 10 μM). ET-1 production was measured in the supernatant. Phosphorylation of VEGF receptor 2 (VEGFR2) was measured by Western blotting after exposure to VEGF without or with SU5416 for 5 and 10 min. KEY FINDINGS VEGF effectively caused VEGFR2 phosphorylation, which was blocked by SU5416. VEGF decreased ET-1 production by at least 29%. In the absence of VEGF, SU5416 increased ET-1 production, by 16% at 10 μM, and SU5416 was able to completely abolish the VEGF effect on ET-1 production. SIGNIFICANCE VEGF may promote vascular health by decreasing ET-1 production in HVMEC-LBl. Blockade of VEGF signaling by SU5416 increases ET-1 levels. The role of VEGF in modulating endothelin production in PAH deserves further study.

Nodular regenerative hyperplasia of the liver and focal global glomerulosclerosis associated with sickle cell anemia.

Nodular regenerative hyperplasia (NRH) of the liver is a lesion of uncertain pathogenesis that has been found to be associated with a variety of systemic and hematological disorders, particularly with rheumatoid arthritis, Felty’s syndrome, other connective tissue disorders, and lympho- and myeloproliferative disorders (1–3). It is characterized by nodules of regenerative hepatocytes distributed diffusely through the liver and atrophy of the intervening parenchyma without significant fibrous septae between the nodules (4). We describe a case that links NRH to sickle cell disease. Incidently, we also found that this patient had focal global glomerulosclerosis (FGGS) of the kidney. The coexistence of NRH and FGGS in our patient with sickle cell disease is suggestive of a common mechanism involved in the pathogenesis of these two conditions. Since vasoocclusive crises are characteristic of sickle cell disease, it is possible that this recurrent and persistent microvascular compromise may occur in both the kidney and the liver, leading to the development of FGGS and NRH, respectively. CASE REPORT

Cholestasis in Crohn's Disease: A Diagnostic Challenge

A 24-year-old male with Crohns disease who developed three independent episodes of cholestatic liver disease over an eight-year period is described. The first episode was related to an idiosyncratic drug reaction while on sulfasalazine. The second episode, at the time of an exacerbation of his colitis, was characterized by moderate portal inflammation on liver biopsy and resolved quickly while he was on corticosteroid therapy. The most recent episode, occurring when the bowel disease was quiescent, was due to granulomatous hepatitis and resolved clinically with no specific therapy. Because numerous potentially serious hepatobiliary complications have been associated with inflammatory bowel disease, prompt and aggressive investigation in these instances is recommended.

Alagille syndrome and Wilson disease in siblings: a diagnostic conundrum.

The authors describe two siblings, each with a different, rare genetic condition that affects liver function. The index case, the 18-year-old asymptomatic brother of a young man recently diagnosed with Wilson disease, presented for Wilson disease screening and was also found to have abnormal liver function suggestive of cholestasis. However, ceruloplasmin level, 24 h urine copper concentration and liver synthetic function were normal. Further hepatic investigations and genetic mutation analysis were performed, ultimately leading to a diagnosis of Alagille syndrome. He was treated with ursodiol, which resulted in normalization of his liver function tests. Subsequently, he was found to be a carrier for a mutation in the Wilson disease gene, ATP7B. In the present report, the potential implications of being a heterozygote for Wilson disease in the context of Alagille syndrome are discussed. Also stressed is that care must be exercised by the clinician when diagnosing family members who may present with two different disorders closely mimicking one another.