Rafiq A. Sheikh

Spontaneous intrahepatic hemorrhage and hepatic rupture in the HELLP syndrome: four cases and a review.

Subcapsular hemorrhage and hepatic rupture are unusual catastrophic complications of the HELLP (hemolysis, elevated liver enzymes, and low platelets) syndrome. A high index of suspicion and prompt recognition are keys to proper diagnosis and management of affected patients. The optimal management of these patients is evolving. An aggressive multidisciplinary approach has considerably improved the morbidity and mortality associated with these complications. We present our experience with four cases of hepatic hemorrhage occurring in association with the HELLP syndrome and review the literature on this subject. All of our patients were multiparous, and three had a history of eclampsia/preeclampsia in a previous pregnancy. All four patients developed intrahepatic hemorrhage; two developed hepatic rupture requiring surgical intervention. Three patients developed disseminated intravascular coagulation and acute renal failure. Two patients developed pericardial effusion, pleural effusions, and ascites. One patient died of septic complications after multiple surgical interventions.

Capsule endoscopy's impact on clinical management and outcomes: a single-center experience with 145 patients.

BACKGROUND:Capsule endoscopy (CE) is a new technology that has been shown to have superior diagnostic yield compared with other methods of evaluating the small bowel. However, there have not been many studies supporting capsule endoscopys impact on clinical outcomes. This study is a chart review evaluating the diagnostic yield and the impact of CE on management and clinical outcomes.METHODS:Retrospective chart review was performed on 145 patients who had undergone capsule endoscopy. Demographic characteristics, indication, prior diagnostic tests, capsule findings, interventions, and clinical outcomes up to 8 months following CE were evaluated. Indications included five main categories that were overt gastrointestinal (GI) bleed, occult GI bleed, abdominal pain, Crohns disease, and iron deficiency anemia. Findings on capsule endoscopy were classified into angiodysplasias, ulcers, gastritis and/or duodenitis, ulcers suggestive of Crohns and normal findings. Interventions performed based on capsule findings were recorded, which included the discontinuation of nonsteroidal anti-inflammatory drugs (NSAIDS), further diagnostic or therapeutic studies, increase in medications, and surgery. Positive outcomes including stabilization or improvement of hemoglobin, decreased need for transfusions, improved symptoms of pain, and a decrease in medications based on interventions were assessed.RESULTS:There were 145 patients who underwent CE. The indications for CE were overt GI bleed (38%), occult GI bleed (22%), abdominal pain (20%), Crohns (12%), iron deficiency anemia (2.7%), and miscellaneous (4%). Eighty percent achieved completion and 6 patients had complications of capsule retention with 4 requiring surgery. The overall diagnostic yield was 69% and included findings of angiodysplasias (24%), intestinal ulcers (13%) gastritis or duodenitis (13.8%), ulcers suggestive of Crohns disease (8.9%), and mass or polyp (3.4%). Based on capsule findings, 35.8% of patients had an intervention. Of the patients who received intervention, 71.7% had a positive clinical outcome (P = 0.032).CONCLUSIONS:The high diagnostic yield of CE influences clinical management leading to improved outcomes. However, the utility of CE may be greater in patients who are referred for certain indications or have specific findings. Additional studies are needed to clarify the role of capsule endoscopy in the evaluation of various indications as well as identify factors associated with positive outcomes.

Sclerosing mesenteritis seen clinically as pancreatic pseudotumor: two cases and a review.

Sclerosing mesenteritis is an uncommon nonneoplastic inflammatory process in the mesentery that is seen as a pseudotumor, usually involving the small bowel mesentery, the mesenteric fat, and less commonly, the mesentery of the large bowel. We report two cases of sclerosing mesenteritis and review the literature on this rare disease. Both patients had pain, profound weight loss, and a mass on computed tomography (CT) scan of the abdomen. The provisional diagnosis was pancreatic neoplasm on the basis of clinical presentation and imaging studies. The diagnosis of sclerosing mesenteritis was established by histologic findings in biopsy material obtained at laparotomy in both cases. Interval histologic studies in one patient who had a high CA 19-9 level, progressive biliary ductal and partial duodenal compression, revealed a transitional histologic pattern from predominant inflammation and fat necrosis to predominant fibrosis. This may explain the varied descriptive terms used in the literature to describe this entity.

Microsporidial AIDS cholangiopathy due to Encephalitozoon intestinalis: case report and review.

Microsporidia are increasingly recognized as opportunistic infections in immunodeficient patients, predominantly patients with AIDS. The two microsporidia most commonly associated with disease in AIDS patients are Enterocytozoon bieneusi and Encephalitozoon intestinalis (previously known as Septata intestinalis). The most common clinical presentation of microsporidiosis in AIDS patients is diarrhea, most commonly caused by the Enterocytozoon bieneusi species. Encephalitozoon intestinalis is a recently described species that has been reported to cause disseminated human infection including cholangitis. We report a case of AIDS cholangiopathy that presented with abdominal pain and cholestatic liver tests. Ultrasound examination and ERCP revealed a picture of sclerosing cholangitis. Bile samples obtained at ERCP were negative for microsporidia; stool studies for microsporidia and cryptosporidia were also negative. No organisms were identified on routine light microscopy of the biopsy specimens from the duodenum, ampulla, and bile duct. E. intestinalis spores were demonstrated in the bile duct biopsies, by methylene blue and azure 11 staining and confirmed by electron microscopy. Albendazole therapy was successful in eradicating E. intestinalis with clinical improvement and improvement in CD4 count. However, the cholangiographic picture did not improve and repeat cholangiography revealed progressive bile duct injury. Albendazole therapy was delayed and may have been too late to prevent bile duct damage; the drug had to be approved by the US Food and Drug Administration for compassionate use. This is an unusual case of sclerosing cholangitis caused by an unusual organism and requiring biliary sphincterotomy and stent placement for progressive stricturing despite eradication of the infection.

Correlation of Ki-67, p53, and Adnab-9 immunohistochemical staining and ploidy with clinical and histopathologic features of severely dysplastic colorectal adenomas.

Variations of Ki-67, p53, and Adnab-9 monoclonal antibody reactions in colonic adenomas may be associated with colonic cancer risk. We studied the predictive value of these markers for adverse behavior in severely dysplastic colorectal adenomas, such as an associated carcinoma, multiplicity of adenomas, and subsequent development of adenomas. For this purpose we compared the clinical, gross, and histologic characteristics of highly dysplastic index polyps in 42 patients with Ki 67, p53, and Adnab-9 immunostaining and other molecular markers. Polyps were removed endoscopically, and severely dysplastic polyps were stained immunohistochemically with Ki-67, Adnab-9, and p53 protein by the avidin biotin conjugate (ABC) technique. Quantitative DNA (QDNA) was analyzed by computer-assisted image analysis. Ki-67 immunohistochemistry showed reversal of normal distribution of nuclear staining from the normal basal position to the upper third of the colonic crypts. This abnormality of immunostaining in dysplastic adenomas was the earliest detected by the panel we used. A statistically significant correlation was seen between invasiveness of carcinoma in the index polyp and polyp size (P = 0.003), sessile morphology (P = 0.037), and villous or tubulovillous histology (P = 0.019). In the index adenoma, p53 positivity was correlated with multiplicity at initial examination (P = 0.053), villous histology (P = 0.053), invasiveness of carcinoma (P < 0.003), and recurrence of colorectal adenomas (P = 0.025). Although p53 positivity and aneuploidy were correlated with invasiveness of carcinoma in the index polyp (P = 0.025), Adnab-9 positivity was not. However, Adnab-9 positivity in the index polyp was associated with multiplicity of adenomas (P = 0.04) as well as recurrence of adenomas (P < 0.024). In conclusion, in addition to the morphologic and histologic markers already known, Ki-67, Adnab-9 antibody, and p53 protein may be prognostic indicators useful in follow-up of patients with severely dysplastic colorectal adenomas. Adnab-9 antibody may identify a field defect in above-average-risk adenoma-bearing patients.

Prognostic significance of the labeling of Adnab-9 in pancreatic intraductal papillary mucinous neoplasms.

SummaryBackground. Pancreatic intraductal papillary mucinous neoplasms (IPMN), morphologically resembling colonic adenomas, often have an indefinable malignant potential. We used a monoclonal antibody (MAb) raised against colonic adenomas, Adnab-9, to identify patients with a better prognosis.Methods. We assessed Adnab-9-labeled sections of these neoplasms from 50 patients, 13 pancreatic adenocarcinomas, and 32 colonic adenomas using standard immunohistochemical techniques.Results. 26% of the IPMNs labeled with Adnab-9 as compared to 0% of pancreatic ductal cancers or surrounding benign tissues, (p<0.001) and 53% of adenomas (p<0.025). Labeling in IPMNs was usually seen in the noninvasive epithelium suggesting that Adnab-9 is a premalignant marker in these lesions. Labeling of invasive IPMN’s identified a group of patients with a superior overall survival (p=0.027). Conclusion. Adnab-9 labeling-characteristics appear similar for both IPMNs and adenomatous polyps, suggesting that they are analogous lesions. Adnab-9 labeling may also be a useful prognostic marker for invasive intraductal papillary mucinous neoplasms.

AIDS-related myopathy.

Infection with the human immunodeficiency virus (HIV) is often associated with the acquired immunodeficiency syndrome (AIDS), and wasting is one of the defining clinical features of AIDS. Muscular weakness due to myopathy may develop at any stage of HIV infection. We report two illustrative cases of HIV-associated myopathies. One was due to inflammatory myosits most likely directly related to the HIV infection, and the other was most likely the result of mitochondrial damage due to zidovudine, a nucleoside analogue commonly used in treating HIV infection. Biopsies from both patients showed alterations of myofiber structures, of varying severity, culminating in necrosis, lipid droplets, and lymphoplasmocytic inflammatory response. The zidovudine-treated patient also showed distinctive mitochondrial changes, predominantly enlargement, variation in shape and size, and disorganization of the cristae. These two types of HIV-associated inflammatory myopathies are reviewed, along with other HIV-associated myopathies, including HIV wasting syndrome, nemaline rod myopathy, pyomyositis, rhabdomyolysis, cardiomyopathy, and other miscellaneous myopathies associated with HIV infection.

Gastric outlet obstruction caused by microperforation of Helicobacter pylori-related antral ulcer.

We report a Helicobacter pylori-related antral gastric ulcer that continued to cause symptoms despite H. pylori eradication. The patient had progressive gastric outlet obstruction, raising the possibility of a malignancy. On exploratory laparotomy, he was found to have a large inflammatory mass surrounding a contained microperforation.