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Query: UMLS:C0033774 (pruritus)
 14,546 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twelve of 43 patients with chronic active hepatitis (CAH) (28%) manifested clinical and laboratory features of cholestasis. The criteria for selection of these patients included at least two of the following: chronic or recurrent pruritus, serum alkaline phosphatase levels of 300 mU./ml. and cholesterol of 300 mg./dl. or more. When compared with 31 control cases these patients were found to have a preponderance of Ashkenazi Jews of Roumanian origin, a higher prevalence of joint and thyroid involvement and higher serum Ig-M Levels. Mortality was similar in both groups but patients with cholestatic features tended to die earlier in the course of the disease. Retrospectively, it was found that they had been treated more intensively, attained complete remissions less frequently and developed cirrhosis more readily. There were no significant differences in the frequency of HBsAg and anti-HBs, the mode of onset, the frequency of hepatosplenomegaly and jaundice, the hematologic findings and the prevalence of autoantibodies. Like acute cholangiolitic viral hepatitis, CAH with cholestatic features emerges as a more serious disease than the classical form of CAH.
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PMID:Chronic active hepatitis with cholestatic features. I. A clinical and immunological study. 53 97

Immunoblastic lymphadenopathy was first described in the german literature in 1975 by Lennert as "Lymphogranulomatosis X." The disease is characterized by generalized lymphadenopathy, hepatosplenomegaly, dysproteinemia, fever and hyperergic reactions such as pruritus, skin rush and eosinophilia. The first ORL occurrence of the disease in Waldeyer's ring and cervical lymph nodes is reported. The histologic picture is characterized by a vascular proliferation with immunoblasts, plasma cells and interstitial amorphous acidophilic material but without Sternberg cells. The origin of the disease from primary malignant neoplasia or hyperergic reactions has not yet been defined. Although prognosis is uncertain and therapy limited, the best management involves small doses of corticoids, supplemented by antibiotics.
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PMID:[Head and neck manifestations of lymphogranulomatosis X (author's transl)]. 70 Nov 5

The general clinical and pathological findings of angio-immunoblastic lymphadenopathy are reviewed and illustrated by a case-report with involvement of the tonsils. Our patient showed all the characteristic signs of this disease, including fever, pruritus, rash, generalized lymphadenopathy and hepatosplenomegaly. Histologically the wellknown triad of arborizing postcapillary vessels, proliferation of immunoblasts and plasma-cells, as well as deposition of PAS-positive interstitial material was found. Laboratory findings included a polyclonal hyperglobulinemia and a hemolytic anemia. Treatment consisted of corticosteroids and supportive medications. The prognosis is generally poor, with a median survival of 13 months. At present, the cause is unknown.
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PMID:[Angio-immunoblastic lymphadenopathy (author's transl)]. 75 11

To evaluate the consequences of receiving human immunodeficiency virus type 1 (HIV-1)-seropositive blood, 90 HIV-1-seronegative recipients of HIV-1-seropositive blood (case patients) and 90 HIV-1-seronegative recipients of HIV-1-seronegative blood, matched for age, sex, number of transfusions, diagnosis, and severity of illness (controls), were followed for 12 months after transfusion at Mama Yemo Hospital in Kinshasa, Zaire. Of case patients and controls, 72% were children transfused for anemia caused by malaria. Of the 46 case patients case patients alive 6 months after transfusion and for whom HIV-1 serologic results were obtained, 44 (96%) had seroconverted. Significantly more case patients (47%) than controls (16%) died within 1 year after transfusion (P less than .001). In the first 3 months after transfusion, fatigue, diarrhea, fever, cough, pruritus, pallor, oral candidiasis, polyadenopathy, hepatosplenomegaly, and rhinorrhea were observed more often among seroconverters than controls (P less than .04). Six percent of case patients and no controls had developed clinical AIDS after 12 months of follow-up. These findings underscore the urgent need for appropriate HIV screening facilities in transfusion centers worldwide.
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PMID:Seroconversion rate, mortality, and clinical manifestations associated with the receipt of a human immunodeficiency virus-infected blood transfusion in Kinshasa, Zaire. 186 35

A 5-year-old physically and mentally retarded female child born of non-consanguineous parents, who had had disseminated skin lesions for 4 1/2 years, is presented. She had persistent neonatal jaundice associated with clay-coloured stools and generalized pruritus which receded by the age of 2 years. Examination revealed characteristic facies, moderate hepatosplenomegaly, cardiac murmur and widespread smooth yellow papules and nodules on ears, trunk, bony prominences and palms. Ophthalmic examination revealed corneal opacities. Liver function tests and lipidogram were abnormal. A diagnosis of Watson-Alagille Syndrome was made on the basis of characteristic facies, xanthomatosis and cholestatic jaundice.
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PMID:Alagille syndrome. A case report. 198 31

The diagnosis of PSC in association with ulcerative colitis was delayed in this case by the interim development of papillary carcinoma of the thyroid that produced a rapidly enlarging neck mass. The presence of chronic cholestasis, pruritus, and hepatosplenomegaly, along with a liver biopsy with histologic changes consistent with PSC, were not immediately appreciated because of the concern for the patient's thyroid cancer. In all other respects, the clinical features of PSC in this case are characteristic of the disease, with the somewhat unusual feature of intra-abdominal lymphadenopathy. Whether or not the papillary thyroid carcinoma is a coincidental finding rather than an associated disease entity, such as ulcerative colitis, remains open to speculation. In any event, the finding of papillary thyroid carcinoma or any other extrahepatic malignancy in association with PSC is rare, but assumes an added dimension for patients with PSC because the presence of malignancy may affect their suitability for future liver transplant.
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PMID:A 22-year-old man with thyroid cancer and cholestatic liver disease. 204 92

Systemic mast cell disease (SMCD) is a rare disease often associated with symptoms of general malaise, pruritus, diarrhea, vomiting, fever, urticaria pigmentosa, hepatosplenomegaly and lymphadenopathy. We reported a case of SMCD associated with cutaneous xanthoma and serum hyper IgE. Skin biopsy revealed xanthomas and diffuse infiltration of mast cells in the dermis. The association of SMCD with xanthoma was reported in the literature for only one case. The hyper IgE could be due to the defect of IgE receptors on the cell membrane of mast cells of dysfunction of T and/or B cell. Any of the treatment using H1 and H2 receptor blockade, disodium cromoglycate, adrenocorticosteroid or chemotherapy (VEPA) were not effective. The patient died of pulmonary edema and multiple organ failure 7 months after the diagnosis was established. The crush method for the cytological examination of bone marrow was considered more useful than smear method for the diagnosis of SMCD.
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PMID:[Systemic mast cell disease associated with cutaneous xanthomas and markedly elevated serum IgE]. 224 20

Transient acantholytic dermatosis is a papulovesicular cutaneous eruption first described in 1970. There have been subsequent reports of similar disorders occurring in patients with malignancy. Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. The patient described had a papular acantholytic dermatosis associated with the development of angioimmunoblastic lymphadenopathy with dysproteinemia-like T-cell lymphoma. The cutaneous manifestations of angioimmunoblastic lymphadenopathy with dysproteinemia are discussed.
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PMID:Transient acantholytic dermatosis associated with lymphomatous angioimmunoblastic lymphadenopathy. 257 18

Twenty-five patients with primary biliary cirrhosis (PBC) in different stages were investigated with respect to pulmonary function abnormalities. The results were compared with a reference sample of 17 sex- and age-matched healthy subjects. A high prevalence of lung function impairment was found in the PBC patients (14/25 [56%]). Bronchial asthma was present in three patients, and severe lung emphysema in one. These four patients had an abnormal lung function, mainly of obstructive type. There was a statistically significant difference between the remaining 21 PBC patients without chronic obstructive lung disease and the reference subjects with respect to diffusion capacity. Almost all abnormal lung function data were found in the symptomatic PBC patients (i.e. symptoms of pruritus, xanthoma, xanthelasmata, jaundice, hyperpigmentation, hepatosplenomegaly), 13 out of 18 (72%), whereas only one out of seven asymptomatic patients was affected. Nine patients (36%) had reduced diffusion capacity compared with none of the reference subjects. The lung function abnormalities in PBC patients are similar to those found in sarcoidosis, another granulomatous disease.
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PMID:Lung function abnormalities in patients with primary biliary cirrhosis. 338 7

The results from the complex study on 26 patients with primary biliary cirrhosis (PBC), 20 females and 6 males, an average age of 46 years, are reported. The most frequent symptoms of PBC are itching, jaundice, hepatosplenomegaly; from the laboratory tests--most characteristic is the increase of serum 5'-nucleotidase, AP, LAP, gamma GTP, GOT, cholesterol, cholic acid and antimitochondrial antibodies and IgM (AP, 5'-nucleotidase and antimitochondrial antibodies, being most significant in making the early diagnosis). The laboratory results in PBC are compared with those of the chronic active hepatitis, cirrhosis of the liver, liver cancer, extrahepatic cholestasis, with outlining the characteristic differences, depending on the diagnosis. The diagnostic advantages of the various methods are discussed (mainly laparoscopy and liver biopsy) and the histologic and electron microscopic changes of percutaneous transhepatic cholangiography, via echography--81 per cent, laparoscopy--73 per cent, scintigraphy--61.53 per cent and liver biopsy--50 per cent. The results from the treatment with cholestrimine, corticosteroids and azathioprine and surgical treatment, observing a temporary improvement and progressing of PBC, are reported. With the follow-up care of 20 patients, it was established, that 9 had died 5 years, on the average, after making the diagnosis, 11 survived after the 5 years and they are still followed up. The longest survival was reported in two females--11 and 15 years after the onset of PBC.
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PMID:[Primary biliary cirrhosis]. 632 95


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