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Biomedical subjects

A Livneh

Publications and source records attributed to A Livneh.

At least 91 records · Page 5Linked to original sources
PubMed1994

Colchicine treatment of AA amyloidosis of familial Mediterranean fever. An analysis of factors affecting outcome.

OBJECTIVE: To elucidate factors possibly influencing the outcome of colchicine therapy in patients with amyloidosis of familial Mediterranean fever (FMF). METHODS: Retrospective analysis of data abstracted from the charts of all 68 FMF patients with amyloidosis who presented during the study period (1974-1992) with proteinuria (> or = 0.5 gm/24 hours) and creatinine values < or = 2.5 mg/dl, received colchicine, and were followed up for > or = 5 years. RESULTS: At the end of the study period, kidney disease had worsened in 31 patients and remained stable in 22. Proteinuria had regressed in 15 patients. Deterioration was related to initial serum creatinine values > or = 1.5 mg/dl (P < 0.01) and to mean colchicine dosage < or = 1.5 mg/day (P < 0.001). The 3 groups were comparable in terms of initial urinary protein levels, duration of proteinuria, presence of hypertension, occurrence of febrile attacks, sex distribution, and proportion of non-compliant patients. CONCLUSION: The therapeutic dosage of colchicine for amyloidosis of FMF is > 1.5 mg/day. This dosage is effective only in patients with initial serum creatinine levels < 1.5 mg/dl.

Adult↗
PubMed1994

Cytokines modulate interleukin-6 production by human salivary gland cell line.

The ability of HrTNF-alpha, HrIL-1 beta, and HrIFN-gamma to modulate IL-6 production by cultured human salivary cell line (HSG) was examined. IL-6 activity was measured by the hybridoma growth factor biologic activity. HrTNF-alpha had a significant dose-dependent effect, whereas HrIL-1 beta and HrIFN-gamma had moderate and minor effects, respectively. Maximal effect (920 units) was noted when cytokines were applied in combination (1000 units HrIFN-gamma and 20 units HrTNF-alpha) and incubated with human salivary gland cells for 72 hr. The same combination of cytokines had also a marked inhibitory effect (70% of control) on human salivary gland cell growth. This is the first report of these concomitant phenomena in a human cell line originating from salivary tissue.

Cell Division↗
PubMed1994

Recurrent episodes of acute scrotum with ischemic testicular necrosis in a patient with familial Mediterranean fever.

The tunica vaginalis is 1 of the sites involved in the recurrent febrile attacks of serositis, which are the hallmark of familial Mediterranean fever. The attacks present clinically as "orchitis." We report on a patient with familial Mediterranean fever in whom recurrent episodes of scrotal attacks were complicated by testicular necrosis requiring orchiectomy. The case emphasizes the challenge of recognizing and differentiating these attacks from other causes of acute scrotum.

Acute Disease↗
PubMed1994

Prevention of recurrent aphthous stomatitis with colchicine: an open trial.

BACKGROUND: Recurrent aphthous stomatitis (RAS) is a common disorder with hitherto unsatisfactory drug therapy. OBJECTIVE: Our purpose was to evaluate the prophylactic effect of colchicine in the treatment of RAS. METHODS: An open, prospective, 4-month study was conducted in 20 patients with RAS who served as their own controls. During the first 2 months of the study no medications were given and in the last 2 months colchicine, 1.5 mg/day, was prescribed. RESULTS: The mean number of aphthae per week and the subjective daily pain scores were reduced by 71% and 77%, respectively, during colchicine treatment as compared with the previous period (p < 0.001 for both). No serious side effects of colchicine were noted. CONCLUSION: These findings suggest a role for continuous colchicine therapy in the prevention of RAS.

Adolescent↗
PubMed1994

Direct evidence for SAA deposition in tissues during murine amyloidogenesis.

To study the mechanism of amyloid deposition, the nature of amyloid proteins formed in experimental murine amyloidosis, was examined. Spleen specimens, 15-60 mg, were homogenized and extracted using aqueous acidic acetonitrile, in a recently developed procedure, making it possible to obtain amyloid proteins from minute amounts of tissue. The extracted material, 1.5-4 mg, was analysed by Western blotting and ELISA using antibodies recognizing differentially proteins AA and SAA. Two immunoreactive proteins of 8 and 12 KDa were isolated and subjected to amino acid analysis and N-terminal sequence determination. The results of immunochemical and chemical examination showed that the 8 and 12 KDa proteins represented proteins AA and SAA, respectively. The data obtained provide new direct evidence for SAA in tissues during murine amyloidogenesis.

Amino Acid Sequence↗
PubMed1994

The preferential expression of the anti-DNA associated 8.12 idiotype in lupus is not genetically controlled.

Anti-DNA antibodies are autoantibodies unique to systemic lupus erythematosus. Studies of their structure have demonstrated cross reactive idiotypes present in genetically unrelated individuals. Despite much research, it is still not clear what triggers their production and what governs the presence of particular idiotypic determinants in their structure. To study the role of genetic and environmental factors in the expression of idiotype, we analyzed sera of SLE patients, their family members and nonautoimmune individuals vaccinated with pneumococcal polysaccharide, for the presence of the 8.12 idiotype, which is present on lambda light chains of anti-DNA antibodies. Elevated titers of the 8.12 idiotype was found in the serum of 57% of SLE patients. Elevated titers were present in only 9% of family members, and always associated with the presence of high levels of IgG anti-DNA antibodies. Following vaccination with pneumococcal polysaccharide, 8.12 reactive anti-pneumococcal antibodies were produced by 7 of 10 non-autoimmune individuals and 8.12 reactive anti-DNA antibodies by one. These results suggest that 8.12 reactive antibodies are antigen driven and bind structurally related antigens, but there is no evidence that expression of this idiotype is genetically controlled.

Antibodies, Antinuclear↗
PubMed1994

Protracted febrile myalgia in patients with familial Mediterranean fever.

OBJECTIVE: We describe a newly defined syndrome of protracted febrile myalgia in patients with familial Mediterranean fever (FMF). METHODS: Fourteen patients with FMF were admitted with an attack of severe disabling myalgia accompanied by fever, high erythrocyte sedimentation rate, and hyperglobulinemia, lasting up to 6 weeks. RESULTS: Unlike in the classical manifestations of FMF response to corticosteroids therapy was prompt. CONCLUSION: Protracted febrile myalgia is an uncommon dramatic manifestation of FMF that may occur despite colchicine therapy and requires treatment with corticosteroids.

Adolescent↗
PubMed1994

An outbreak of foodborne streptococcal throat infection.

Streptococcal pharyngitis is a common disease of epidemic nature, usually transmitted by saliva droplets. We present an epidemiologic analysis of an outbreak of streptococcal pharyngitis in a military unit involving 75 soldiers. The causing organism was Streptococcus pyogenes (group A beta hemolytic Streptococcus, T28 M56), which was isolated from 53 affected individuals. The source of the epidemic was probably an army cook. The infection was transferred by a cabbage salad to individuals who became symptomatic within 24-48 h. This epidemic is a reminder of an exceptional route of streptococcal dissemination and one of the largest outbreaks of foodborne streptococcal pharyngitis documented.

Disease Outbreaks↗
PubMed1993

Familial Mediterranean fever in the colchicine era: the fate of one family.

In order to demonstrate the effect of prophylactic colchicine treatment on the natural history of familial Mediterranean fever (FMF), a family is presented with 6 out of 9 siblings affected by FMF. Each patient represents a different stage of the amyloidotic kidney disease of FMF and the effect of continuous colchicine treatment on its course. Considered together, the members of this family present an almost complete clinical, genetic, and behavioral picture of the disease.

Adult↗
PubMed1993

Anti-DNA antibodies secreted by peripheral B cells of lupus patients have both normal and lupus-specific features.

Autoantibodies present in sera of patients with systemic lupus erythematosus (SLE) are found in low titer in sera of healthy individuals. Hence, it is possible that B cell populations in SLE patients and in normal individuals are homologous and in the absence of regulatory forces would secrete similar antibodies. We therefore studied antibody secretion of Epstein-Barr virus-transformed B cells of 20 SLE patients and 20 healthy subjects. Normal and lupus antibody repertoires did not differ significantly with respect to antigenic specificities, isotype, avidity, and titer. However, anti-DNA antibodies bearing the anti-DNA-associated idiotype 8.12 were found only in SLE. These findings suggest that the B cell repertoire of SLE and normal individuals is more similar than the serum antibody profile. However, part of the anti-DNA response in SLE probably reflects affinity (antigen driven) maturation as it differs in idiotype profile from anti-DNA response of normal individuals.

Antibodies, Antinuclear↗
PubMed1993

Colchicine in the treatment of AA and AL amyloidosis.

Colchicine is an effective medication in the prevention and treatment of amyloidosis of familial Mediterranean fever. Its therapeutic effect depends on the stage of renal disease and the drug dose. To evaluate colchicine effect in AA amyloidosis of other diseases and in primary AL amyloidosis, the literature was reviewed. Findings were that (1) the effect of colchicine in reactive amyloidosis has not been methodically studied, but anecdotal reports suggest it may be beneficial; and (2) the results of studies and case reports on the effect of colchicine in primary amyloidosis are conflicting. Because a therapeutic effect of colchicine in primary and reactive amyloidosis has been shown in sporadic cases, a prospective, controlled, multicenter study assessing the effect of colchicine in all types of amyloidosis appears to be justified. Until such a study is available, the addition of colchicine in an appropriate dose to any therapeutic regimen of patients with AA or AL amyloidosis should be considered.

Adult↗
PubMed1993

Differential suppression activity induced by paternal leukocyte immunization in habitual abortion.

In order to investigate the possible role of suppressor cells in paternal leukocyte immunization to prevent recurrent miscarriages, peripheral blood lymphocytes from habitually aborting women before and after immunization were assessed. Immunization-induced suppressor activity as shown by (1) rise in CD8-positive cells and a decline in the CD4/CD8 ratio, (2) failure of cyclosporine A to inhibit the proliferation of phytohemagglutinin- and alloantigen-stimulated cells and (3) unresponsiveness to the immunizing spouses' antigen. These findings resembled those in normal pregnant women. Hence, paternal leukocyte immunization may induce specific and nonspecific T cell suppression which may induce the immune tolerance necessary to maintain pregnancy.

Abortion, Habitual↗
PubMed1993

Effect of pregnancy on renal function in amyloidosis of familial Mediterranean fever.

OBJECTIVE: To study the effect of pregnancy on kidney function in patients with familial Mediterranean fever (FMF) with amyloidosis. METHODS: A retrospective analysis relating kidney function at term to kidney function at conception and to blood pressure and colchicine treatment before and during pregnancy in 17 patients with 29 pregnancies found among more than 3000 patient files in our FMF clinic. RESULTS: Following pregnancy, 7 patients (24% of pregnancies) experienced a decline in renal function. Urine protein > or = 2 g/24 h at conception was present in all pregnancies which sustained deterioration in contrast to 6 of 22 which did not (p < 0.001). Serum creatinine > or = 1.5 mg/dl at conception was present in 3 patients, all of whom experienced deterioration of renal function during pregnancy (p < 0.01). Neither colchicine dose nor elevated blood pressure correlated with status of renal function at term. CONCLUSION: Our findings suggest a possible deleterious effect of pregnancy on amyloid nephropathy and that this effect may be associated with more advanced renal disease at conception.

Adolescent↗
PubMed1992

The anti-DNA-associated idiotype 8.12 is encoded by the V lambda II gene family and maps to the vicinity of L chain CDR1.

The 8.12 idiotype is an anti-DNA-associated Id present on lambda L chains that are expressed at high titers in 50% of patients with systemic lupus erythematosus. Since this Id can be present on as much as a third of a patient's anti-DNA antibodies and is found in renal glomeruli, 8.12 is thought to be a marker for a subset of pathogenic anti-DNA auto-antibodies. A molecular analysis of the 8.12 positive antibodies was designed to explore the genetic basis of this Id. Monoclonal human B cell lines were generated by transformation with EBV and lambda L chain-secreting lines were analyzed for Id expression and V region gene usage. In this panel of Ig lambda cell lines, the 8.12 idiotype is encoded exclusively by members of the V lambda II gene family. The sequences of several 8.12+ and 8.12- V lambda II genes are reported here and are used to map the 8.12 Id to the vicinity of CDR1, as well as to further characterize the large and polymorphic V lambda II gene family.

Amino Acid Sequence↗
PubMed1992

Twin studies in familial Mediterranean fever.

Familial Mediterranean fever (FMF) is a genetic disease characterized by recurrent short episodes of fever, accompanied by peritonitis, pleuritis, or arthritis. The disease is almost completely ethnically restricted to patients of Mediterranean descent--Sephardic Jews, Armenians, Anatolian Turks, and Arabs. Although many family studies have been performed, no twin study has been reported as yet. We studied 21 di- and monozygotic twin sets, identified among the 1,943 FMF patients in our registry. Full concordance was observed in all the 10 monozygotic twin sets. In the 11 dizygotic twins, concordance for FMF disease was found in only 3 pairs. Variability in the clinical manifestations and degree of severity have been noted within twins. These findings provide definitive evidence for the genetic cause of FMF. They also support the single gene autosomal recessive model, and provide support for the contention that the lower observed than expected incidence found in FMF is due to genetically affected but clinically undiagnosed patients.

Adolescent↗