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Biomedical subjects

G Nesher

Publications and source records attributed to G Nesher.

At least 19 recordsLinked to original sources
PubMed2003

Decreased salivary output in patients with Gaucher disease.

BACKGROUND: Gaucher disease, the most common sphingolipid storage disease, results in accumulation of glucocerebroside in macrophages or "Gaucher cells". In a preliminary screening of 109 patients with type I disease, when asked specifically about dry mouth, approximately one quarter claimed to suffer from this symptom. AIM: To ascertain whether decreased salivary output is a feature of Gaucher disease. DESIGN: Prospective case-control study. METHODS: Salivary output was measured in 65 adult patients and 65 healthy controls using the Saxon test with Hochberg's modification. RESULTS: Mean salivary output was 1.91+/-1.19 g/min in the patient group vs. 2.74+/-1.17 g/min in the control group (p<0.001). This difference was greater among males. These results were not improved in the patients receiving enzyme replacement therapy, which is effective in ameliorating most Gaucher-related signs and symptoms. DISCUSSION: Recent studies have implicated an association between sicca syndrome and viral hepatitis C infection, which may imply an immunological trigger for these findings, but in this specific cohort, only three patients were reactive for hepatitis C. Follow-up of patients, both untreated and receiving enzyme therapy, is needed to delineate the association with salivary hypofunction, and ascertain whether enzyme therapy may induce sicca symptoms.

Adult↗
PubMed2001

Anti-nuclear envelope antibodies: Clinical associations.

OBJECTIVES: Characterization of the clinical associations and clinical implications of antibodies reacting with antigens of the nuclear envelope. METHODS: Description of an illustrative case and a MEDLINE search-assisted literature review of relevant cases. RESULTS: With indirect immunofluorescence, autoantibodies directed against various antigens of the nuclear envelope stain the nucleus in a ring-like (rim) pattern. Autoantibodies against 5 antigenic components of the nuclear envelope have been described: anti-gp210, p62, lamina, lamina-associated polypeptides, and lamin B receptor. Antibodies to antigens of the nuclear pore complex, such as gp210 and p62, are highly specific (> 95%) for primary biliary cirrhosis and may aid in the serologic diagnosis of this condition, especially in cases in which antimitochondrial antibodies are not detectable. In contrast, antilamin antibodies are not disease-specific but seem to be associated with lupus anticoagulant or anticardiolipin antibodies, antiphospholipid syndrome, thrombocytopenia, autoimmune liver diseases, and arthralgia. High-titered antilamin antibodies help to define a subset of lupus patients with antiphospholipid antibodies who are at a lower risk of developing thrombotic events. In addition, preliminary data suggest that the presence of antilamin antibodies may be helpful in the diagnosis of chronic fatigue syndrome. CONCLUSIONS: Each of the antibodies reacting with nuclear membrane antigens has its own spectrum of disease associations. RELEVANCE: Determination of anti-nuclear envelope antibody pattern by indirect immunofluorescence, with subsequent determination of the specific antibody, carries important diagnostic and prognostic implications in various autoimmune conditions.

Antibodies, Antinuclear↗
PubMed2001

Charles Bonnet syndrome in glaucoma patients with low vision.

PURPOSE: To characterize the nature and frequency of Charles Bonnet syndrome in glaucoma patients with low vision. PATIENTS AND METHODS: All patients attending the glaucoma clinic during a period of 10 months who had visual acuity of 20/80 or less in both eyes were included in this study. Each patient was questioned about the occurrence of visual hallucinations. Those who responded positively had a thorough interview relating to the characteristics of the hallucinations. Medical history and social history were taken, followed by a complete ocular examination. RESULTS: Eighty-nine patients met the inclusion criteria. Eleven patients (12.3%), eight men and three women, admitted to having experienced visual hallucinations. Except for one case, the patients did not disclose this experience previously. Eight patients had one repeatable hallucination, and three patients experienced more than one sight. The visions were usually sharp, and the figures were occasionally incomplete. Most hallucinations were chromatic. Frequency of hallucinations varied between daily and weekly, and duration was mostly a few minutes. In addition to glaucoma, nine of the eleven patients had other ocular findings that could have contributed to the reduction of vision. CONCLUSION: Visual hallucinations are not rare in glaucoma patients with low vision. Patients tend to conceal their experience of visual hallucinations, but a discussion of these phenomena with the patient and assurance of their harmless nature will reduce his or her anxiety and concerns.

Aged↗
PubMed2001

Visual hallucinations in giant cell arteritis: association with visual loss.

OBJECTIVE: To evaluate the frequency and characteristics of visual hallucinations (VH) in patients with giant cell arteritis (GCA) and to determine their relationship to other visual phenomena. METHODS: This prospective study included 31 consecutive patients with GCA. All were asked whether they had experienced recent visual phenomena. Patients with visual symptoms underwent a comprehensive ophthalmologic examination. When unusual visual phenomena were reported, patients were asked to describe their nature, duration, and frequency of occurrence. RESULTS: Visual symptoms occurred in 6 patients: permanent visual loss in 5 and amaurosis fugax in one. In 4 of the 5 patients with permanent visual loss, it was preceded by intermittent VH over a period of 1-10 days. Patients were aware of the unreal nature of the visions. Hallucinations disappeared within 2 weeks, but in one patient, recurred 6 months later in association with further visual deterioration. CONCLUSION: The occurrence of visual hallucinations in patients with GCA-associated visual loss is more common than previously appreciated. As hallucinations preceded permanent loss of vision, this phenomenon may serve as a harbinger of imminent visual loss.

Age Distribution↗
PubMed1999

Parvovirus infection mimicking systemic lupus erythematosus in a pediatric population.

OBJECTIVES: To assess the striking similarities of presentation in a pediatric population between human parvovirus B19 (HPV-B19) infection and systemic lupus erythematous (SLE). METHODS: Medical records of seven patients (ages 6 to 15) with HPV-B19 infection were reviewed retrospectively. RESULTS: Six of seven cases presented with a history of malar rash, and all seven had prolonged arthralgias and fatigue. Six of seven had a positive antinuclear antibody (ANA) titer ranging from 1:40 to greater than 1:640, with two patients having antibodies to Scl-70 and others to Sm, RNP, SS-A (Ro), or SS-B (La). The erythrocyte sedimentation rate (ESR) varied from 2 to 68 mm/h. Two patients presented with elevated rheumatoid factor (RF) titers of 24 and 271 IU/mL, respectively. All had elevated IgM antibody levels to parvovirus at the onset, and markedly elevated IgG levels when evaluated throughout their disease course. Over the course of 2 to 3 months, three improved, but the other four continued to have symptomatology for 14, 40, 78, and 120 weeks, respectively. Treatment was symptomatic, and no one developed classic SLE. CONCLUSIONS: HPV-B19 infection in a pediatric patient group may present with SLE-like symptomatology and positive serology suggestive of SLE. The course of the disease is usually self-limited, though it may be prolonged in some for up to 120 weeks.

Adolescent↗
PubMed1999

The changing clinical presentation of temporal arteritis in Israel: a multicenter study.

OBJECTIVES: To evaluate whether the increasing incidence of temporal arteritis in Israel is associated with a changing clinical presentation. METHODS: The demographic data and clinical manifestations of 144 TA patients in this large multicenter study were recorded and compared with data obtained in a previous study. RESULTS: The patient population was older, with 24% > or = 80 years compared to 6% in the previous study. There was an increase in the number of nonspecific presenting symptoms, and less patients presented with the "classical" manifestations of headache (81% vs. 71%), fever (83% vs. 40%), jaw claudication (21% vs. 13%), and visual symptoms (47% vs. 24%). The median time from presentation to diagnosis was significantly reduced, from 5 to 1.5 months. CONCLUSIONS: There were substantial changes in the clinical presentation of TA patients in Israel during 1980-95 compared to patients diagnosed prior to 1978. It is suggested that these changes may be attributed not only to the influence of aging of the population, but are due largely to increasing physician awareness to the spectrum of manifestations of TA, which leads to earlier diagnosis.

Age Distribution↗
PubMed1998

Alpha-interferon-induced arthritis: clinical presentation treatment, and prevention.

OBJECTIVE: The therapeutic applications of alpha-interferon (IFN) have expanded greatly to include chronic viral hepatitis and malignant disorders. Autoimmune phenomena occur frequently with IFN therapy, but arthritis is uncommon. We describe the clinical features and treatment of IFN-induced arthritis. METHODS: A patient with chronic myelogenous leukemia who developed arthritis secondary to IFN therapy is presented. The clinical features and treatment of this condition in 37 additional cases are reviewed. RESULTS: The most common clinical presentation was symmetric polyarthritis. This was associated with antinuclear antibodies in 72% of patients and rheumatoid factor in 34%. Cessation of IFN, with or without the addition of antiinflammatory or remittive agents, resulted in remission of arthritis in 89% and 71% of the cases, respectively. Restarting IFN therapy resulted in recurrence of arthritis in 63%. In the patient described in this report, recurrence of arthritis was prevented by coadministration of hydroxychloroquine (HCQ) and prednisone. CONCLUSION: Arthritis is an uncommon complication of IFN therapy; but it may lead to cessation of this treatment modality. In such cases, coadministration of a remittive agent such as HCQ may enable reinstitution of IFN therapy without recurrence of arthritis.

Anti-Inflammatory Agents↗
PubMed1997

Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features, and treatment.

Valvular abnormalities develop in 36% and 35% of patients with primary antiphospholipid syndrome (PAPS) and with systemic lupus erythematosus (SLE) respectively, and in 48% of patients with SLE and antiphospholipid antibodies (aPL). Valvulopathy includes leaflet thickening, vegetations, regurgitation, and stenosis. A literature survey shows that significant morbidity from valvular dysfunction, mostly mitral regurgitation leading to congestive heart failure, occurs in 4% and 6% of SLE and PAPS patients, respectively. The pathogenesis of valvulopathy may involve interaction of aPL with antigens on the valve surface, resulting in valvulitis. Current therapy includes symptomatic measures and valve replacement. A novel approach for symptomatic antiphospholipid syndrome (APS) related valvulopathy involves treatment with systemic corticosteroid. We describe four such patients and their dramatic clinical and hemodynamic response to treatment with prednisone when symptomatic measures failed.

Adrenal Cortex Hormones↗
PubMed1997

Giant cell arteritis and polymyalgia rheumatica: clues to early diagnosis.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely related disorders found predominantly in older patients. These disorders, which are being recognized more frequently, are more common in women, in Caucasians, and in various geographic locations. Early recognition and treatment may prevent possible catastrophic consequences of GCA, such as blindness, stroke, or dissection of the aorta. Although diagnosis is fairly easy with the classic presentation, it may be missed when the patient presents with nonspecific constitutional symptoms. An increased awareness among primary care physicians will aid in the prevention of much of the morbidity and mortality related to these diseases.

Aged↗
PubMed1996

Trends in the clinical presentation of temporal arteritis in Israel: reflection of increased physician awareness.

We have noticed significant changes in the clinical presentation in patients diagnosed with temporal arteritis in Israel between 1980-1992 compared to patients diagnosed prior to 1977. At the time of diagnosis 57% of the patients were older than 75 years, compared to only 23% within this age group in the previous period. There was an increase in the number of nonspecific and unusual presenting symptoms such as weakness, respiratory and neurological symptomatology, and a decreased proportion of patients presenting with the "classical" manifestation such as headaches, temporal tenderness and visual symptoms. The time from presentation to diagnosis was shortened significantly. It is suggested that these changes are largely due to the increasing awareness among physicians to the various manifestations of this conditions.

Aged↗
PubMed1995

Parvovirus infection mimicking systemic lupus erythematosus.

There are striking similarities between human parvovirus B19 (HPV-B19) infection and systemic lupus erythematosus (SLE): both may present with malar rash, fever, arthropathy, myalgia, cytopenia, hypocomplementemia, anti-DNA, and antinuclear antibodies (ANA). Therefore, it is difficult at times to differentiate HPV-B19 infection from SLE presentation or exacerbation. We report 4 cases of HPV-B19 infection mimicking SLE and review 10 other reported cases, all of whom were women. The similarity to a typical SLE presentation was indeed striking: most patients presented with rash, arthropathy, myalgia, fever, and positive ANA. In some cases, HPV-B19 infection seemed to exacerbate SLE rather then resemble it, and differentiation was difficult. Nearly all patients improved within several weeks. However, a few patients had symptoms and laboratory abnormalities lasting more than 6 months. The possibility of HPV-B19 infection should be entertained in patients presenting with SLE-like features.

Adult↗
PubMed1995

Rheumatologic complications of vitamin A and retinoids.

Retinoids are synthetic derivatives of vitamin A. They are administered primarily for dermatological conditions, such as psoriasis, acne, and disorders of keratinization. Toxicity has proven a significant problem with long-term administration of the retinoids. Bone abnormalities mimicking seronegative spondyloarthropathy or diffuse idiopathic skeletal hyperostosis have been described in many cases, as well as other rheumatologic manifestations such as arthritis, myopathy, and vasculitis. These retinoid-related adverse effects are reviewed.

Humans↗
PubMed1995

Charles Bonnet syndrome in temporal arteritis.

An 87-year-old woman presented with Charles Bonnet syndrome--the occurrence of formed visual hallucinations in sane aged individuals. This was followed by headaches and unilateral visual loss, and the diagnosis of temporal arteritis (TA) was confirmed by biopsy. Steroid therapy resulted in disappearance of hallucinations, which recurred 7 mo later, responding to an increase in steroid dosage. Charles Bonnet syndrome may be an early sign of decreasing visual acuity in aged individuals; thus, diagnosis of TA or exacerbation of established TA should be considered in such patients.

Aged↗