Cases reported "Gastrointestinal Diseases"

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1/15. A systematic history for the patient with chronic pelvic pain.

    Chronic pelvic pain is a source of frustration to both the physician and the patient. physicians have been ill equipped by their training to confront the multifaceted nature of the complaints of patients with chronic pelvic pain. patients have experienced a repetitive dismissal of their complaints by physicians too busy in their practices to address their problems comprehensively. The approach to the patient with chronic pelvic pain must take into account six major sources of the origin of this pain: 1) gynecological, 2) psychological, 3) myofascial, 4) musculoskeletal, 5) urological, and 6) gastrointestinal. Only by addressing and evaluating each of these components by a very careful history and physical examination and by approaching the patient in a comprehensive manner can the source of the pain be determined and appropriate therapy be administered. This article was developed to provide the clinician with a set of tools and a methodology by which the patient with this complaint can be approached.
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2/15. Nasogastric hyperalimentation through a polyethylene catheter: an alternative to central venous hyperalimentation.

    We performed nasogastric hyperalimentation with polyethylene catheters and appropriate feeding solutions in 12 cachectic patients who had been referred as candidates for central venous hyperalimentation. Most patients had primary gastrointestinal disease. The duration of hyperalimentation averaged 31 days. Seven patients obtained rapid weight gain (average 0.3 kg/day) with the nasogastric hyperalimentation alone. An additional two were successfully repleted with the addition of parenteral fluids via peripheral veins. In the nine repleted patients, serum albumin rose by average 19%, 24-hr urine creatinine by average 21%, and triceps skinfold by average 46%. The nature of the weight gain in the nine successful cases was analyzed by the metabolic balance study technique. Average composition of the increment in weight was: 50% protoplasm, 48% extracellular fluid, 19% adipose tissue, and less than 1% bone. We conclude that nasogastric hyperalimentation can replace central venous hyperalimentation in a substantial proportion of patients now receiving the latter type of treatment.
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3/15. Acute disseminated encephalomyelitis in adults: a reappraisal of clinical, CSF, EEG, and MRI findings.

    OBJECTIVES: To establish an adequate definition of acute disseminated encephalomyelitis (ADEM) in adults, based on our clinical observations of a case-series. methods: Over a period of three years 10 adult patients with a para- or postinfectious disseminated (diffuse or multifocal) syndrome of the CNS fulfilling predefined strict criteria for the diagnosis of ADEM were encountered and systematically followed. RESULTS: The age ranged from 21 to 62 years, two were men. MRI was normal in 5 patients and only mildly abnormal in the remaining patients. CSF was normal in 5 patients and mildly abnormal in the remainder, EEG was abnormal in 7/8 patients. All patients survived and were followed over a period of 30 months (range: 8 to 48 months). Nine patients were left with some residual defects, consisting most often of a mild cognitive impairment. CONCLUSIONS: The EEG as an investigation of brain function can be crucial in establishing the organic nature of disease. MRI is important to exclude other diffuse or multifocal encephalopathies. However, in contrast to previous reports in the literature abnormal MRI should not be considered mandatory in adult ADEM. Difficulties in the diagnosis of ADEM are discussed and the importance of clinical and paraclinical findings for establishing the diagnosis is outlined.
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4/15. GI anthrax: report of one case confirmed with autopsy.

    BACKGROUND: bacillus Anthraces is a non-motile, rod like, gram-positive and aerobic bacillus that produces central oval-shaped spores and characterized by rough, irregular and often comma-shaped colonies in blood agar. About 95% of human anthrax is cutaneous and 5% respiratory. GI anthrax, a very rare type, has been reported in less than 1% of all cases. We thus report a case of GI anthrax with autopsy findings. CASE REPORT: A 15-year-old young adult man was admitted to our hospital with history of fever, abdominal pain and bloody diarrhea from 48 hours after ingestion of half-cooked sheep's meat. In endoscopic exam of upper GI tract, the esophagus had been filled with fresh blood without any varices, stomach showed evidence of hemorrhage and had multiple erosion but no ulceration of duodenum. penicillin g 24,000,000 U/day IV was started but unfortunately severe upper and lower GI hemorrhage developed, leading to hypotension and death. In autopsy, ascites, paraaortic and mesenteric lymphadenopathy was accident with some of them being hemorrhagic. The spleen was slightly enlarged with hemorrhagic congestion in some parts of it. In the blood sample drawn from the spleen, the filamentous bacillus was successfully found. CONCLUSIONS: Because of the fatal nature of the disease and the similarity of the signs and symptoms to the other gastrointestinal diseases, we should consider it as one of the probable differential diagnosis in the patients with upper and lower gastrointestinal bleeding with fever esp. in the endemic areas.
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5/15. A family with gastrointestinal amyloidosis associated with variant lysozyme.

    Hereditary nonneuropathic systemic lysozyme amyloidosis is a very rare form of amyloidosis, and only 4 families with this condition have been detailed until now in the literature. Clinical manifestations of lysozyme amyloidosis observed until now mainly concerned the kidneys, liver, and digestive tract. We report here a new family with hereditary lysozyme amyloidosis who presented predominantly with gastrointestinal involvement. The proband, a middle-aged woman, underwent partial gastrectomy for a hemorrhagic "gastric peptic ulcer" in 1984. Gastrointestinal amyloidosis was diagnosed in 1998 on biopsies performed on the gastroduodenal anastomosis, which appeared to be very congestive at presentation. Immunohistochemical stainings in tissue sections were positive for lysozyme. Amyloid was also observed in the colonic mucosa. The patient had a mutation in the lysozyme gene characterized by substitution of the amino acid at position 64 in the mature protein from tryptophan to arginine, previously described in only 1 French family with prominent nephropathy. It is interesting to note that her father had died many years before with an uncharacterized digestive amyloidosis. Our observation shows that a search for gastrointestinal amyloidosis is important, particularly when physicians are faced with congestive mucosa, unexplained abdominal hemorrhage, or abdominal symptoms. When gastrointestinal amyloidosis is diagnosed, it is important to determine with precision the nature of the amyloid fibril proteins, because various types of amyloidosis can involve the gastrointestinal tract.
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6/15. Lysozyme amyloidosis: report of 4 cases and a review of the literature.

    Autosomal dominant hereditary amyloidosis represents not 1 disease but a group of diseases, each the result of mutations in a specific protein. The most common form is transthyretin amyloidosis, which has been recognized clinically for over 50 years as a familial polyneuropathy. Nonneuropathic amyloidoses (Ostertag type amyloidosis) include those due to abnormalities in lysozyme, fibrinogen Aalpha-chain, and apolipoprotein a-i and A-II. The role of lysozyme in amyloid-related human disorders was first described in 1993; to date, there have been only 9 publications describing this disorder, which is a nonneuropathic form of hereditary amyloidosis. Reported cases have involved 7 unrelated families. We describe here our own experience with 4 families suffering from lysozyme amyloidosis: the first had prominent renal manifestations with sicca syndrome, the second and third had prominent gastrointestinal symptoms, and the fourth had a dramatic bleeding event due to rupture of abdominal lymph nodes. To our knowledge, this last symptom has not been reported previously, but is reminiscent of the hepatic hemorrhage seen in a previously reported case of a patient with lysozyme amyloidosis. To characterize the manifestations of this disorder, we performed an exhaustive literature review.Although hereditary amyloidosis is thought to be a rare disease, it is probably not as rare as we think and may well be underdiagnosed. Moreover, some cases of lysozyme amyloidosis are probably confused with acquired monoclonal immunoglobulin light-chain (AL) amyloidosis, formerly known as primary amyloidosis, which is the most frequent type of amyloidosis. Because treatment for each type of amyloidosis is different, and because therapy directed at 1 type may worsen symptoms of the other types, it is important to determine precisely the nature of the amyloid protein. Thus, hereditary lysozyme amyloidosis should be considered in all patients with systemic amyloidosis, particularly in patients who present with renal, gastrointestinal, or bleeding complications without evidence of AL or AA (secondary) amyloidoses.
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7/15. Unexpected gastrointestinal complications in severely burned children.

    The authors refer to the frequently difficult diagnosis of gastrointestinal complications of major burns, particularly in childhood, to weight the factors co-responsible for the development of those complications and to stress the importance of the patient's history. They report on the case of three and a half year-old child to demonstrate the fatal course of a late diagnosed complications -- perforating cholecystitis. autopsy showed the chronic nature of the disease with acute exacerbation in the course of treatment. No case similar to this one has yet been seen at the Prague burns Centre in any age category.
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8/15. Bilateral postinfectious optic neuritis and intravenous steroid therapy in children.

    Six patients presented with acute, simultaneous, bilateral optic neuritis. Five of the six patients admitted to a recent history of a brief upper respiratory or gastrointestinal illness, presumably viral in nature. visual acuity was severely reduced in all patients. Five of the six patients also demonstrated marked neurologic deficits, including seizure activity and cerebellar dysfunction. Three patients demonstrated enhancing intracranial lesions on magnetic resonance imaging (MRI) consistent with demyelinative plaques, whereas lumbar puncture was abnormal in three patients. HLA tissue typing was performed on five of the six patients. All patients were treated with intravenous methylprednisolone, followed by a 2-month tapering course of oral prednisone. Each patient experienced a rapid and nearly complete recovery of vision during treatment.
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9/15. 99mTc-DTPA uptake in malignant and inflammatory bowel disease: experience from gastrointestinal bleeding studies.

    During the evaluation of 99Tcm-DTPA for localization of acute gastrointestinal bleeding in 81 studies (78 patients), it was observed that there was an increased 99Tcm-DTPA at sites of inflammatory and malignant lesions of the GI tract in 18 studies. Active bleeding was detected only in 10 and no bleeding was detected in the remaining eight studies. In all the increased DTPA uptake helped to localize the site of bleeding. Image subtraction of early from delayed images according to a predefined acquisition and processing protocol helped to differentiate bleeding from non bleeding lesions. We conclude that increased 99Tcm-DTPA uptake in malignant and inflammatory lesions of the bowel is an additional advantage for its use in localizing the site of acute GI bleeding. technetium-99m diethylenetriamine pentaacetic acid (99Tcm-DTPA) has been widely used for imaging the brain and kidneys since it was introduced by Hauser in 1970. Kadir and Strauss in 1979 reported that 99Tcm-DTPA localizes in segments of bowel with inflammation due to ulcerative colitis, regional enteritis and other forms of enterocolitis. Recently we reported the advantages of 99Tcm-DTPA in localizing the site of acute gastrointestinal bleeding. inflammatory bowel diseases are among the causes of gastrointestinal bleeding. One of the problems in localizing the site of the bleeding by radionuclide procedures is the intermittent nature of the bleeding. We have reviewed all gastrointestinal bleeding studies in our department using 99Tcm-DTPA from January 1984 till September 1985 in order to find out whether increased uptake of 99Tcm-DTPA was helpful in localizing the bleeding lesion due to malignancy and/or inflammation.
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10/15. Acquired deficiency of the inhibitor of the first component of complement: report of five additional cases with commentary on the syndrome.

    The association of late onset recurrent angioedema with a deficiency of the inhibitor of the first component of complement (C1INH) and of the binding subunit of the first component, Clq, defines the syndrome of acquired C1INH deficiency. The description of five new cases, along with the original two and the 18 others in the literature, brings the total reported cases to 25 and highlights the associated B cell abnormalities that are present in 23 and are of a malignant nature in 19 cases. In three of the five newly reported cases, the occurrence of angioedema, which prompted recognition of the acquired deficiency of C1INH, C1q, and C4, preceded the delineation of the underlying B cell malignancy by 2 to 3 yr despite efforts to recognize neoplastic disease in two of these patients throughout the interval. Because the acquired C1INH deficiency reflects increased catabolism rather than impaired biosynthesis, only high-dose attenuated androgens elicit a measurable increment in serum C1INH. The occurrence of the syndrome with multiple myeloma is noted for the first time.
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