PubMed HealthSearch

Biomedical subjects

N Harats

Publications and source records attributed to N Harats.

16 recordsLinked to original sources

Localized primary sclerosing cholangitis mimicking a cholecystectomy stricture relieved by an endoprosthesis.

Primary sclerosing cholangitis presenting as a localized stricture affecting a segment of the extrahepatic biliary tree is rarely found. We describe the case of a 39 year old woman with obstructive jaundice, in whom this diagnosis was proven by endoscopic retrograde cholangiography. An endoprosthesis was endoscopically introduced through the stenotic area which led to a dramatic improvement enabling a successful liver transplantation after 2 years.

Adult

Lack of association of a restriction fragment length polymorphism for serum amyloid P gene with reactive amyloidosis.

The prevalence of a recently described restriction fragment length polymorphism using Msp I for the serum amyloid P gene was determined in 5 groups of patients. Patients with reactive (secondary) amyloidosis, juvenile rheumatoid arthritis, related inflammatory conditions, or juvenile rheumatoid arthritis with reactive amyloidosis, and healthy control subjects were found to be polymorphic for 8.8-kb and 5.6-kb gene fragments; they either had one or the other or both fragments. No significant differences were seen between these groups with relation to this polymorphism, and no correlation with the presence of reactive amyloidosis was observed.

Amyloidosis

Hereditary amyloidosis: evidence against early amyloid deposition.

Twelve members of the Indiana hereditary amyloidosis type II kindred were tested for the presence of amyloid deposits. All were young adults (age 26-37), with no evidence of disease and with 1 affected parent. Six were found to be carriers of the variant gene, by DNA testing and/or reduced serum retinol-binding protein levels. Nevertheless, no amyloid could be found in any skin, rectal, or carpal tunnel biopsy specimens. Our results suggest that hereditary amyloidosis type II is a true late-onset disease, in which accumulation of amyloid does not start until late in life--perhaps only a short time before symptoms appear.

Adult

IgG rheumatoid factor in purified IgG fractions and whole sera from patients with rheumatoid arthritis.

There are inherent technical difficulties in measuring IgG rheumatoid factor (IgG-RF) in the serum of patients with rheumatoid arthritis (RA). These arise from measuring a reaction between two IgG molecules and the interference of IgM-RF in the reaction. We compared the prevalence of IgG-RF in whole sera and purified IgG fractions from 58 RA patients (43 of whom were latex or sheep cell agglutination positive). Methods of purification were: ammonium sulphate precipitation and DEAE cellulose or protein A-Sepharose chromatography. IgG-RF was measured by two methods: (1) radioimmunoassay and ELISA with a monoclonal myeloma IgG (IgG4,K) as the antigen and radiolabelled rabbit anti-human IgG (previously absorbed on a column with IgG4,K) as the second antibody; (2) ELISA using rabbit IgG as the antigen and a peroxidase conjugated goat anti-human IgG as the second antibody. When whole sera were assayed, 18 (31%) contained IgG-RF. In contrast, only three of the IgG fractions (5%) were positive for IgG-RF by all methods, while the remainder were uniformly negative. These results suggest that IgG-RF determination in whole sera does not accurately reflect IgG-RF activity.

Antigens

Spinal claudication in systemic amyloidosis.

We describe a patient with hereditary amyloidosis who developed the syndrome of spinal claudication. Myelography and computerized tomography of the lumbar spine demonstrated stenosis of the spinal canal and surgical exploration confirmed dense amyloid infiltration of ligamentous structures compressing the nerve roots. Two cousins of our patient and 2 patients with immunoglobulin amyloidosis had similar clinical syndromes. Spinal stenosis can be demonstrated by myelography or by computerized tomography. Diagnosis of this syndrome is important since wide decompression may be needed to relieve symptoms. Patients who had only disc extraction did not improve after the operation; their symptoms persisted and even worsened.

Amyloidosis

Concurrence of ovarian cancer and dermatomyositis. A report of two cases and literature review.

The concurrence of dermatomyositis and ovarian carcinoma deserves special attention owing to the gravity of its prognosis. Until now, only scattered case reports on women suffering from this disease combination had been published. In this paper the information available in the literature on 28 women afflicted with these diseases is collated. In addition, two such patients treated in our hospital are described in detail. The review highlights the various aspects of this disease combination, emphasizing the quadrupled incidence, advanced stage and uniform epithelial origin of the malignancy as compared with that in the general female population with ovarian carcinoma. The value of a meticulous gynecologic evaluation was proven in one of our patients with dermatomyositis; in her, ovarian carcinoma was detected in its early phase.

Adult

Paravertebral extramedullary hematopoiesis associated with improvement of anemia in congenital dyserythropoietic anemia type II.

Thoracic masses resulting from extramedullary hematopoiesis developed in two sisters of Moroccan origin with congenital dyserythropoietic anemia type II (HEMPAS). In one patient, the diagnosis of extramedullary hematopoiesis was confirmed histologically. The appearance of extramedullary foci of hematopoiesis mimicking mediastinal tumors has not been previously described in HEMPAS. These masses result from persistent erythropoietic stimulation associated with chronic hemolytic anemia. In both patients, detection of the asymptomatic masses was preceded by normalization of hemoglobin levels. Thus unexpected correction of a chronic refractory anemia associated with the appearance of mediastinal masses might be the heralding manifestation of an effective extramedullary hemopoiesis.

Anemia, Dyserythropoietic, Congenital

Acute poststreptococcal polymyalgia.

Three patients developed severe incapacitating diffuse pain and tenderness of the skeletal muscles after acute streptococcal infection. There was no evidence of concomitant arthritis, glomerulonephritis, or inflammatory muscle disease in any of the cases. All patients responded promptly to anti-inflammatory therapy. Severe myalgia should be considered an additional complication of immunologically mediated poststreptococcal response.

Acute Disease

A simple prognostic index for hospitalized geriatric patients. A prospective study of 70 patients.

A simple prognostic index for hospitalized geriatric patients is reported herein. Seven parameters including: mobility, sphincter control, mental competence, feeding ability, presence of pressure sores, medical condition and family state were recorded in 70 patients. The sum of the scores of all parameters constituted the prognostic index. At the end of 3 months the patients were divided into two groups: 18 patients who were discharged (group 1) and 52 patients who were still hospitalized (35 cases) or had died (17 cases) (group 2). The mean index for group 1 was 14.9 +/- 3.2 (mean +/- SD), while that for group 2 was 23.5 +/- 4.3 (p less than 0.00001). Eighty-three percent of the patients in group 1 and only 8% of those in group 2 had scored lower than 17. Thus, the score of 17 or more had a prognostic sensitivity of 92% and a specificity of 83%. The predictive value of indices of 17 or more was 94%. The index described offers therefore a simple and relatively accurate tool for the assessment of the prognosis of elderly patients.

Aged

Cholestatic jaundice associated with D-penicillamine therapy.

Cholestatic jaundice is a rare complication of penicillamine therapy. We report here a 35-year-old patient who developed fever, a rash and cholestatic jaundice 16 days after commencing treatment with penicillamine for cystinuria. The jaundice subsided slowly after discontinuation of the drug and with prolonged therapy with prednisone. The literature on penicillamine-induced liver injury is reviewed.

Adult

Quinidine-induced vasculitis.

Four patients developed nonthrombocytopenic purpura two to three weeks after initiation of quinidine therapy. The skin lesions disappeared and did not recur after cessation of quinidine therapy. Histologic examination revealed leukocytoclastic vasculitis with deposition of C3, IgA, and/or IgM in the small dermal vessels. Since quinidine purpura is usually associated with thrombocytopenia, the possibility of leukocytoclastic vasculitis as an additional cause of purpura is stressed.

Aged