Cases reported "Chronic Disease"

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11/7622. Dermal vessels in acrodermatitis chronica atrophicans.

    An ultrastructural investigation of two patients suffering from acrodermatitis chronica atrophicans revealed in the small dermal vessels swelling of the endothelial cells, material of probably plasmatic origin accumulated in the subendothelial area, and sleeves of basement membrane-like material on a concentric perivascular layout.
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12/7622. Reactive lymphoid hyperplasia of liver coexisting with chronic thyroiditis: radiographical characteristics of the disorder.

    BACKGROUND: Reactive lymphoid hyperplasia of the liver is an extremely rare entity, with six cases reported so far. methods: We encountered a 47-year-old Japanese female with reactive lymphoid hyperplasia of the liver, which coexisted with chronic thyroiditis. The lesion was discovered incidentally as a hypo-echoic mass with a hyper-echoic rim at a routine ultrasonography examination. It increased from 12 to 17 mm diameter in 6 months. Radiological studies, such as contrast-enhanced computerized tomography (CT) and angiography demonstrated a hypervascular lesion. RESULTS: It was consequently diagnosed as a neoplasm with malignant potentiality and she underwent partial hepatectomy. The lesion was composed of small mature lymphocytes which formed prominent lymphoid follicles with germinal centres, scattered plasma cells and stromal fibrosis. Immunohistochemical study revealed polyclonal origins of the involved lymphocytes. dna analysis for the immunoglobulin heavy gene and the T cell receptor beta gene using Southern blot hybridization showed no monoclonality. The following features have characterized the images in past cases, as well as ours: hypo-echoic mass, occasionally with a rim, in ultrasonography and hypervascularity, shown by angiography and enhanced CT.
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13/7622. central nervous system T-cell lymphoproliferative disorder in a patient with chronic active Epstein-Barr virus infection.

    PURPOSE: central nervous system (CNS)-T cell lymphoproliferative disorder (T-LPD) developing during the course of chronic active Epstein-Barr virus (CAEBV) infection is reported. patients AND methods: CAEBV was diagnosed in a 14-month-old boy with fever, cytopenia, hepatosplenomegaly, and abnormal high titers of anti-Epstein-Barr virus (EBV) antibodies. At 8 years of age, he had a splenectomy because of progressive disease. RESULTS: After 27 months of clinical remission, muscle weakness and paresthesia developed. magnetic resonance imaging of his brain showed spotty T2 prolongation in left parietal, bilateral frontal, and temporal white matter with meningeal enhancement. brain biopsy revealed the cerebral infiltration of CD3 , CD4 , CD8-, CD45RO , CD56-, and EBV-encoded rna 1 cells. CONCLUSIONS: The CNS involvement of EBV-associated T-LPD is a rare but serious complication in CAEBV without known underlying immunodeficiency.
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14/7622. Use of transdermal amitriptyline gel in a patient with chronic pain and depression.

    A man with severe inflammatory bowel disease suffered from chronic abdominal pain and depression. A transdermal amitriptyline gel preparation was compounded since he was unable to take drugs orally serum concentrations of amitriptyline and its active metabolite nortriptyline were measured over 24 hours. Symptoms of depression were monitored before starting transdermal therapy and at the end of 6 weeks. Pain symptoms and amitriptyline adverse drug events were monitored daily Steady-state serum concentrations of drug and metabolite were within the therapeutic range over 24 hours. The patient reported that his mood was improved but his abdominal pain remained unchanged. Transdermal amitriptyline gel was well tolerated and is an alternative delivery system in patients unable to take drugs orally.
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15/7622. Chronic eosinophilic leukemia: successful treatment with an unrelated bone marrow transplantation.

    The optimal treatment of eosinophilic leukemia is still uncertain. We report the successful treatment of a 21-year-old patient with eosinophilic leukemia, without cytogenetic abnormalities, by bone marrow transplantation from an unrelated donor. The conditioning regimen for the transplantation consisted of fractionated total body irradiation and cyclophosphamide. Acute GVHD, grade I, post-transplantation was successfully treated. No other severe complications occured. The patient is alive in complete remission 21 months after unrelated bone marrow transplantation.
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16/7622. Renal granulomatous sarcoidosis in childhood: a report of 11 cases and a review of the literature.

    We analysed retrospectively 11 children with renal granulomatous sarcoidosis confirmed by renal histology in order to describe the course and prognosis of the disease. Symptomatic sarcoidosis was diagnosed at a mean age of 10.1 years. Nine children had renal involvement at the time of diagnosis. In the course of the disease, nine patients developed renal failure and mild proteinuria, seven had transient sterile leukocyturia, four showed microscopic haematuria, seven had a urinary concentrating defect, and enlarged kidneys were seen in three patients. One child had hypercalcaemia and hypercalciuria, none had hypertension. light microscopy of the kidney showed interstitial infiltration by mononuclear cells in all children, interstitial fibrosis in nine patients, epithelioid granulomas in seven, tubular involvement in eight, and mild glomerular involvement in seven patients. Renal immunofluorescence was negative. Ten children received prednisone for 1-11 years. After a mean follow up of 5.5 years, three patients had entered end-stage renal failure and one had chronic insufficiency after interruption of medical supervision and prednisone therapy. CONCLUSION: Renal failure, proteinuria, leukocyturia, haematuria, and concentration defect are the prominent features of renal granulomatous sarcoidosis in children. Steroid therapy, adjusted according to disease activity, may prevent end-stage renal failure.
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17/7622. Changing clinical, morphological and immunological patterns in chronic lymphocytic leukaemia.

    Two cases of chronic lymphocytic leukaemia are presented in which, in the terminal phase of the disease, a population of abnormal lymphocytes similar to those seen in lymphosarcoma cell leukaemia replaced the morphologically normal small lymphocytes observed previously. Immunologically, this change coincided with a striking alteration in the pattern of surface immunoglobulin markers. In both cases, most cells initially carried IgM and in both cases these were replaced by cells carrying IgG as the number of abnormal lymphocytes increased. In addition, the use of anti-delta antiserum in the second case revealed the coexistence of large numbers of IgD-bearing lymphocytes as well. Calculations showed that more than half the cells present at that time must have been carrying both IgD and IgG surface markers.
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18/7622. The effect of lidocaine on chronic tinnitus: a quantitative cerebral perfusion study.

    The cerebral perfusion of a 55-year-old female patient with chronic tinnitus was investigated quantitatively by single photon emission computed tomography (SPECT) with the xenon133 inhalation method. The first investigation was performed under standard conditions, the second during suppression of tinnitus with intravenous injection of lidocaine. As a reference a healthy volunteer was included under the same conditions. The global cortical perfusion was identical on both sides except for increased perfusion in the auditory cortex, especially on the right side when tinnitus lasted during the first measurement. The second investigation under treatment with lidocaine and during the tinnitus-free interval showed a decrease of the global cortical perfusion with a slightly higher value at the temporal cortex bilaterally. In comparison to the first investigation, a difference between right and left auditory cortex could no longer be demonstrated, and also the difference between regional and global perfusion decreased. A cerebral effect under treatment of tinnitus with lidocaine was shown by the quantitative measurement of brain perfusion. It is debatable whether this effect on cerebral activity results from the medication or as a reaction to the tinnitus-free interval.
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19/7622. Chronic axonal sensory and autonomic polyneuropathy without motor involvement: a new 'chronic inflammatory neuropathy?'.

    We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow-up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the 'chronic inflammatory neuropathies' are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area.
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20/7622. 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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