Secondary Amyloidosis Presenting as Ischemic Proctitis.

A 49-year-old man presented with abdominal pain and rectal bleeding for two days associated with a 50-pound unintentional weight loss. History was notable for hypertension, chronic kidney disease, obesity, gout, and acute cholecystitis status post cholecystectomy. Computed tomography (CT) of the abdomen and pelvis showed rectal wall thickening. Colonoscopy showed proctitis with superficial ulcerations. In the setting of renal insufficiency, malabsorption, and low-voltage QRS complexes on electrocardiogram (ECG), amyloidosis was considered in the differential diagnosis. Rectal and renal biopsies with subsequent retrospective staining of gallbladder tissue confirmed amyloid deposition. Gastrointestinal involvement of amyloidosis is relatively uncommon. Particularly, amyloid deposition in the gallbladder and rectum is very rare. The development of AA amyloidosis in our patient may have been related to gout, obesity, and the presence of a heterozygous complex variant for the MEFV (familial Mediterranean fever) gene. Awareness of this atypical presentation of amyloidosis is important, as additional staining of biopsy samples is necessary, and diagnosis allows for directed treatment.

26-Year-Old Survivor of Kasai Procedure With Native Liver.

A 26-year old woman with history of congenital biliary atresia and Kasai procedure as a newborn presented with recurrent nausea and abdominal pain. Physical examination showed anicteric sclera and tender right upper quadrant. Elevated liver function tests suggested obstruction and hepatitis. Percutaneous transhepatic cholangiogram showed generalized ductal dilation, innumerable intrahepatic bile duct stones, and multiple filling defects. No anastomotic stenosis was noted. The patient was treated for acute symptoms and referred to a liver transplant center. Long-term survivors of the Kasai procedure with a native liver are rare, and this represents the third known U.S. case in the literature.

Gastroesophageal reflux disease: the case for improving patient education in primary care.

PURPOSE: Gastroesophageal reflux disease (GERD) affects up to 25% of the western population, and the annual expenditure for managing GERD is estimated to be more than $14 billion. Most GERD patients do not consult a specialist, but rather rely on their primary care physician for symptom management. Research has shown that many patients--regardless of diagnosis--do not fully understand what their doctors tell them and remain uncertain as to what they are supposed to do to take care of themselves. To determine if patients are adequately educated in the management of GERD, we conducted a survey. METHOD: We administered a survey to patients with GERD in an outpatient setting and explored their knowledge of such management practices as modification of behavior and diet and use of medication. RESULTS: Of 333 patients enrolled, 66% reported having an in-depth discussion with their primary care physician. Among patients taking a proton pump inhibitor, 85% of those who'd had an in-depth discussion were aware of the best time to take their medication, compared with only 18% of those who did not have an in-depth discussion. In addition, patients who'd had in-depth conversations were significantly more likely than those who didn't to know some of the behavior modification measures that might improve their symptoms. CONCLUSION: Our study underscores the need for primary care providers to fully discuss GERD with their patients to improve overall management of the disease.

Successful wireless capsule endoscopy for a 2.5-year-old child: obscure gastrointestinal bleeding from mixed, juvenile, capillary hemangioma-angiomatosis of the jejunum.

The lesion responsible for obscure gastrointestinal bleeding in the pediatric population may not be determined with standard primary endoscopic methods. Wireless capsule endoscopy, now a first-line modality for evaluation of the small bowel in the adult population, is a tool that may be useful among children. We report a case of a 2.5-year-old girl who presented with melenic stools. Upper and lower endoscopy, Meckel scans, and mesenteric angiography yielded negative results. Wireless capsule endoscopy identified numerous abnormal, dilated, blood vessels in the proximal jejunum, with associated fresh blood. The patient underwent surgical exploration, with resection of the affected portion of the jejunum. Pathologically, the dilated blood vessels were consistent with mixed, juvenile, capillary hemangioma-angiomatosis of developmental or congenital origin. The patient fared well postoperatively, with no additional bleeding in 9 months of follow-up monitoring. This case report highlights the use of capsule endoscopy in the diagnosis and successful treatment of gastrointestinal bleeding in a young infant. This is the youngest reported patient treated with the use of wireless capsule endoscopy in the pediatric population.