Cases reported "Chronic Disease"

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11/2420. Idiopathic chronic intestinal pseudo-obstruction. Use of central venous nutrition.

    patients with idiopathic chronic intestinal pseudo-obstruction suffer from malnutrition because of inability to maintain adequate oral intake without the development of obstructive symptoms. We have successfully used central venous nutrition in two patients with this syndrome, both on a short-term and long-term home-maintenance basis. Hyperalimentation can provide adequate nutrition in patients with intestinal pseudo-obstruction until normal bowel function returns or until definitive therapy for this chronic disease is found.
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12/2420. fibromuscular dysplasia involving coronary arteries--a case report.

    The authors report a young patient with fibromuscular dysplasia involving multivessels including coronary arteries. If young patients have chest pain on effort, fibromuscular dysplasia of coronary arteries must be considered. As fibromuscular dysplasia is a chronic progressive disease and some cases progress rapidly in a few months, careful follow-up and comprehensive medical management may be necessary in such patients.
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13/2420. Focal malakoplakia in chronic periapical periodontitis.

    AIMS: Three cases of chronic periapical periodontitis including focal areas with malakoplakia changes are reported. methods AND RESULTS: These areas included both von Hansemann-type macrophages and periodic acid-Schiff-positive, iron- and calcium-containing concretions. Some concretions corresponded to spherules with a targetoid configuration, thus fitting the morphological criteria for classical Michaelis-Gutmann bodies. CONCLUSION: The vast majority of the cases of malakoplakia that have been reported in the literature corresponded to a characteristic, fairly homogeneous lesion, but a few instances of focal malakoplakia have been described in various chronic conditions. These considerations support the opinion that the local conditions for the production of Michaelis-Gutmann bodies may occur focally in diseases characterized by macrophage accumulation.
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14/2420. celiac disease-associated alopecia in childhood.

    We report the association of celiac disease and alopecia in 3 children. In one, the alopecia developed after 4 years' nonadherence to a gluten-free diet; the other 2 patients presented with alopecia. Administration of a gluten-free diet resulted in partial regrowth of hair in the first child and complete hair growth in the others.
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15/2420. Internuclear ophthalmoplegia following minor head injury: a case report.

    Internuclear ophthalmoplegia (INO) is a common sign of multiple sclerosis in young patients and of vascular diseases in older people. Traumatic bilateral internuclear ophthalmoplegia following severe head injuries may occur. We present the unusual case of a young patient suffered from bilateral INO as an isolated finding after a minor head injury, without other signs of brain stem or cortical injury. The ophthalmoplegia has persisted for 22 months.
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16/2420. Increased whole blood viscosity on cooling in a patient with cold hemoagglutinin disease.

    The whole blood and plasma viscosities have been evaluated in a patient with cold hemoagglutinin disease at different temperatures and at different shear rates. At 37 and 42 degrees C, whole blood viscosity values, regardless of the shear rate applied, resulted to be correspondent to the hematocrit value (31%). The values observed were similar to those noted in a patient with chronic bleeding anemia and an approximately equivalent hematocrit (33%). The same was true for plasma viscosity. At 32 degrees C, whole blood viscosity, regardless of the shear rate, resulted to be higher than expected. The values observed were similar to those noted in a normal subject with a clearly higher hematocrit value (44%) and definitely higher than those noted in the chronic anemia patient. On the contrary, plasma viscosity remained unchanged. These studies indicate that in cold hemoagglutinin disease, red cell aggregation and piling are capable of increasing blood viscosity.
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17/2420. Chronic urticaria as a presenting sign of hairy cell leukemia.

    Chronic urticaria is a common clinical disorder that is idiopathic in over 75% of cases. Less commonly, urticaria may be the presenting manifestation of an allergic or infectious disease, endocrinopathy, inherited syndrome, or autoimmune disorder. Rarely, urticaria may be a sign of underlying malignancy, including leukemia. C.C. is a 48-year-old white female who was referred for evaluation of recurrent urticaria for 3 years. The pruritic, erythematous wheals were pinpoint, and appeared to be precipitated by heat, stress, and effort. Prick tests were negative except to D. pteronyssinus. CBCs over the past 5 years revealed WBCs of 2,300-5,000 cells/mm3. skin biopsy revealed interstitial edema with infiltration of eosinophils and mast cells consistent with urticaria. The impression was probable cholinergic urticaria, for which hydroxyzine was prescribed with fair symptomatic control. One year later, she presented with bright red blood per rectum. Repeat physical examination revealed lymphadenopathy and splenomegaly. Subsequent laboratory studies showed pancytopenia. endoscopy was normal except for small, nonbleeding hemorrhoids. Bone marrow biopsy revealed histologic evidence of hair, cell leukemia that was treated with 2-chlorodeoxyadenosine. Upon initiation of chemotherapy her pruritus and urticaria subsided. Recent CBC revealed Hgb 9.2 g/dL, platelets 290,000 cells/mm3, and WBC 4,100 cells/mm3. Peripheral blood smear showed no hairy cells.
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18/2420. Systemic lupus erythematosus with optical neuromyelitis (Devic's syndrome). A case with a 35-year follow-up.

    Optical neuromyelitis or Devic's syndrome is a very uncommon neurological manifestation of systemic lupus erythematosus. It is also associated with antiphospholipid antibodies, limited responsiveness to glucocorticoid treatment and a poor prognosis. We report the case of a female systemic lupus erythematosus patient who developed recurrent flares of optical neuritis and transverse myelitis. These flares consistently responded to glucocorticoid therapy. Despite the absence of overt anticardiolipin antibodies in the course of the disease, long-term anticoagulant therapy has been introduced with positive results. Treatments are usually of limited efficacy in Devic's syndrome. In our patient, however, aggressive glucocorticoid treatment resulted in prolonged survival.
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19/2420. Correction of bone marrow failure in dyskeratosis congenita by bone marrow transplantation.

    dyskeratosis congenita is recognized by its dermal lesions and constitutional aplastic anemia in some cases. We report successful allogeneic bone marrow transplantation in two siblings with this disease from their sister, and their long term follow-up. We used reduced doses of cyclophosphamide and busulfan for conditioning instead of total body irradiation. Also, we report late adverse effects of transplantation which are not distinguishable from the natural course of disease.
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20/2420. Double valve repair and maze procedure for degenerative valvular disease and chronic atrial fibrillation.

    A 61-year-old male with degenerative aortic valve regurgitation, mitral valve regurgitation and chronic atrial fibrillation underwent a combined reparative procedure consisting of aortic valve repair, mitral valve repair and maze procedure. Surgery was successful and postoperatively the patient is in NYHA class I, without anticoagulation. To the best of our knowledge, this is the first clinical report of this combined reparative surgery. As advances are made in valve repair surgery, it is expected that similar combined procedures will be performed more frequently in future. The benefits of avoiding valve replacement and anticoagulation after such combination treatment is discussed.
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