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H Amital

Publications and source records attributed to H Amital.

At least 19 recordsLinked to original sources

Hyperacute renal failure as the initial presentation of systemic lupus erythematosus.

SLE nephritis is usually a slow process that may lead to renal failure many years after its first presentation. Success of different therapeutic modalities in preventing renal failure is therefore evaluated and compared only after many years of treatment. Lately, this conservative philosophy has been challenged with the acknowledgment of collapsing glomerulopathy (CG), a recent recognized clinical-pathological entity, characterized by rapidly progressive renal failure. Despite this ominous description we present an unusual case of a patient who presented with systemic lupus erythematosus (SLE) and clinical and pathological findings of CG, who completely remitted several weeks after commencing immunosuppressive therapy with intravenous cyclophosphamide and prednisolone.

Acute Kidney Injury↗

SAPHO syndrome treated with pamidronate: an open-label study of 10 patients.

BACKGROUND: In recent years the SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) has been encountered more frequently. However, clinical evidence indicating superiority of a specific therapeutic modality is still absent. Pamidronate, a second-generation bisphosphonate, has a pronounced effect on bone metabolism by suppressing bone resorption. We report our clinical experience with intravenous pamidronate in SAPHO syndrome. METHODS: Between the years 1999 and 2003 we treated 10 patients with the SAPHO syndrome who did not respond to NSAIDs, oral corticosteroids, colchicine, methotrexate, sulphasalazine or infliximab. All patients were treated with 60 mg pamidronate, given intravenously within an hour. In cases of no response a subsequent dose was given within a month and if there was a partial response an additional infusion was given after 4 months. The primary endpoint was the disappearance of recurrent bouts of bone pain, osteitis or hyperostosis, or recurrent synovitis. Reduction of the frequency of attacks by 50% was regarded as a partial response. RESULTS: Seven of the patients were females and three were males. The age at diagnosis ranged from 26 to 68 yr. All patients had axial or peripheral arthritis and cutaneous involvement; three had severe acne, eight had pustulosis and two had concomitant psoriasis vulgaris. Hyperostosis of the anterior chest wall involving either sternocostal or sternoclavicular joints, as seen on technetium 99 bone scintigraphy, was detected in all patients. Complete remission was observed following therapy in six patients, three others partially responded and only one patient had no response. Two patients needed four cycles of pamidronate infusion, one patient needed three, six needed two infusions and one patient remitted following a single pamidronate infusion. In all but one patient pamidronate was effective in preventing recurrent bouts of pustulosis. CONCLUSION: Pamidronate seems to be a very effective mode of therapy for patients with the SAPHO syndrome, by promoting remission in all components of the disorder, such as bone, joint and skin involvement, and ceases the bouts that characterize this disorder.

Acquired Hyperostosis Syndrome↗

[Splenic infarction as an unusual cause of left pleural effusion].

Many etiologies lead to pleural effusion. The pathogenetic cause is usually located either in the lung parenchyma or in the pleura. Subphrenic causes that lead to pleural effusion are uncommon. Several reports elaborated on the role of splenic hemorrhages in the genesis of left-sided pleural effusion. Splenic infarction is a rare etiology of left-sided pleural effusion, and it has rarely been described in medical literature. We present a case study of an elderly female patient who suffered from polycythemia vera for more than a decade, and was hospitalized for left-sided pleural effusion that appeared following left upper abdominal pain.

Aged↗

Intravenous immunoglobulins in peripheral neuropathy associated with vasculitis.

BACKGROUND: Peripheral neuropathy is a prominent feature of the systemic and secondary vasculitides. Usually, it is responsive to corticosteroids, but in certain cases it may be resistant to corticosteroid or immunosuppressive treatment, or both. OBJECTIVE: To present patients who exhibited various inflammatory diseases accompanied with vasculitic peripheral neuropathies for which intravenous immunoglobulin (IVIg) was used for treatment. METHODS: Six patients with Sjögren's syndrome, systemic lupus erythematosus (SLE), vaccination induced vasculitis, Churg-Strauss vasculitis, mixed cryoglobulinaemia associated with hepatitis C infection, or sarcoidosis were included. All developed vasculitic peripheral neuropathy, and were treated with high dose IVIg (2 g/kg body weight). The patients were followed up for 1-5 years after this treatment. RESULTS: In four patients (Sjögren's syndrome, Churg-Strauss vasculitis, SLE, and vaccination induced vasculitis) the neuropathy resolved after IVIg treatment. CONCLUSION: IVIg may be beneficial in cases of resistant vasculitic peripheral neuropathy. IVIg should probably be considered as a sole or adjuvant treatment for patients with contraindications to conventional treatment, or alternatively, for patients in whom conventional treatment has failed.

Adult↗

Fibrosis regression induced by intravenous gammaglobulin treatment.

OBJECTIVES: To review case histories of patients in whom fibrosis played a significant role in the pathogenesis of their disease, and to determine whether intravenous gammaglobulin (IVIg) contributed to the regression of their fibrotic condition. METHODS: Eight patients with excess fibrotic reaction in the course of diverse diseases were analysed; a tendency that reverted with different IVIg treatment options. Myelofibrosis was predominant in three patients (a patient with a myeloproliferative syndrome, one with systemic lupus erythematosus, and one with Sjögren's syndrome). Three patients had scleroderma as their main feature, one patient had hepatitis C cirrhosis, and one had idiopathic thrombocytopenic purpura. RESULTS: Fibrotic excess was reduced in all the patients by IVIg treatment. In five patients the disease as a whole benefited from the infusion of immunoglobulins. CONCLUSION: IVIg may enhance resorption of fibrosis and promote healing in patients with fibrotic associated disorders.

Aged↗

Catastrophic antiphospholipid syndrome: remission following leg amputation in 2 cases.

OBJECTIVE: The antiphospholipid syndrome is characterized by venous and arterial thrombotic events that are often recurrent, thrombocytopenia, recurrent fetal loss, and elevated titers of antiphospholipid antibodies. A subtype of patients with a particularly overwhelming clinical picture has been termed catastrophic antiphospholipid syndrome (CAPS). In this report, we present 2 patients who exhibited a similar multisystem disorder associated with gangrenous changes in the lower extremities. METHODS: Two patients with CAPS are presented, highlighting the impact of this disorder on the patients and the response to various therapeutic modalities. RESULTS: Both patients had pulmonary, cardiac, cutaneous, and neurologic findings consistent with CAPS. In addition, they had large purulent leg ulcers associated with livedo reticularis. Amputation of the legs in each case induced remission of the systemic illness. CONCLUSIONS: We believe that infection plays a significant role in the pathogenesis and amplification of the antiphospholipid syndrome. In certain patients, this association probably is mediated via immune mechanisms, which also enhance the genesis of atherosclerosis. After the foci of infection (suppurative leg ulcers) were removed, the underlying illness improved. These case studies provide an opportunity to study the interrelationship between several confounding factors that converge and lead to the development of this autoimmune condition.

Amputation, Surgical↗

Diet and lupus.

The effect of dietary modifications has been extensively studied in lupus animal models. Calorie, protein, and especially fat restriction, caused a significant reduction in immune-complex deposition in the kidney, reduced proteinuria and prolongation of the mice's life span. The addition of polyunsaturated fatty acids (PUFAs), such as fish oil or linseed oil, was also related to decreased mice morbidity and mortality in animal models of lupus and of antiphospholipid syndrome. PUFAs such as eicosapetaenoic acid (EPA) and docosahexaenoic acid (DHA) competitively inhibit arachidonic acid with a resultant decrease in inflammatory eicosanoids and cytokines. Human studies support the effect of a PUFAs-enriched diet, both scrologically and clinically. Large scale clinical studies are needed to confirm the primary results.

Animals↗

Linseed oil suppresses the anti-beta-2-glycoprotein-I in experimental antiphospholipid syndrome.

The immunomodulatory potential of a diet enriched with n-3 polyunsaturated fatty acids was analysed in naïve mice with experimental antiphospholipid syndrome (APS) induced by active immunization with H-3, a human anti-beta-2-glycoprotein-I (anti-betaGPI) monoclonal antibody (mAb). Fetal loss and other clinical manifestations of APS were prevented in the group of APS mice upon exposure to the enriched n-3 diet compared to the control group. The titers of anti-betaGPI were significantly lower (in O.D. at 405 nm, 1.387+/-0.232 in comparison to non-treated mice 0.637+/-0. 111, P< 0.05). The reduced titer of anti-betaGPI antibodies in the sera of the treated mice was associated with a reduced number of anti-betaGPI forming cells in cultured splenocytes (84+/-14, antibody-forming cells (AFC)/10(5)cells in comparison to 37+/-4 AFC/10(5)cells (P< 0.02).In addition to the suppression of the humoral response in mice with experimental APS fed with linseed oil, we also observed an inhibitory effect on the cellular response. The T-cell response to anti-betaGPI was lower in comparison to mice immunized with H-3 anti-betaGPI mAB, which were kept on a normal diet. These results indicate that polyunsaturated fatty acids may improve clinical and laboratory parameters of APS. The beneficial effects of diets enriched with n-3 should be further examined as a potential mode of therapy for patients with APS.

Animals↗

Mycoplasma-pneumoniae-induced thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is a fatal disease characterized by widespread platelet aggregation, hemolytic anemia and fever with renal and neurological involvement. Different factors have been associated with the development of TTP, e.g. infections, pregnancy, chemotherapy, drug therapy and bone marrow transplantation. Recent data imply that all these different causes may induce the disease by decreasing the activity of the plasma von Willebrand factor-cleaving protease resulting in unusually large von Willebrand factor multimers that later on initiate the cascade of TTP. In this communication, we present a unique association between infection with Mycoplasma pneumoniae and TTP. We believe that the emergence of antibodies that cross-react with Mycoplasma and the protease might elucidate in this case the pathogenesis of TTP.

Adult↗

[Why do they leave? Clinical characteristics of patients who leave the emergency room against medical advice].

The object of this study was to characterize those who leave the emergency department against medical advice and to examine the short-term consequences. The study was conducted retrospectively during 1992 and prospectively during May-August 1993 by telephone interviews. About 0.5% of all the patients who applied to the emergency department left against medical advice. Their main characteristics was that they were mostly young, single men. A wide range of complaints had brought them to the emergency department, very similar to that of the control group, but with a higher tendency to abuse drugs and alcohol. Most of those who left against medical advice had less severe disease than the controls. However 10% of them had to be hospitalized within the subsequent 2 weeks, in comparison with 4% of the controls. This study suggests that improving the efficiency of the emergency department will decrease the number leaving against advice.

Adult↗

Clinical characteristics of ruptured chordae tendineae in hospitalized patients: primary tear versus infective endocarditis.

BACKGROUND: Several etiologies have been associated with the rupture of chordae tendineae. The leading causes are infective endocarditis, primary rupture, and the association with various connective tissue disorders. HYPOTHESIS: In order to define the attributes of these patients, a retrospective study was conducted that investigated the medical files of hospitalized patients in the Sheba Medical Center, Tel-Hashomer, Israel. METHODS: Twenty patients (17 men, 3 women) with ruptured chordae were detected. Primary rupture of the chordae tendineae had been diagnosed in 11 patients, while infectious endocarditis was the cause for the tear of the chordae tendineae in 9 patients. The patients who had primary rupture of the chordae were older than the patients with endocarditis (67.4 +/- 11.3 vs. 57 +/- 9.3 years, respectively, p < 0.05). RESULTS: The posterior mitral valve cusp was more commonly involved (15 patients). Six of the patients with posterior mitral valve cusp involvement manifested atrial fibrillation. Mitral valve prolapse (MVP) was detected among seven patients, six of whom belonged to the group with infective endocarditis. CONCLUSION: Primary tear and infective endocarditis are leading etiologies of ruptured chordae tendineae in hospitalized patients. Particularly among the patients with infective endocarditis, concomitant MVP was frequently detected. It is the authors' opinion that this coexistence implies that MVP may predispose to the rupture of chordae tendineae.

Aged↗

Primary biliary cirrhosis. Is (and how much of) the pathology preventible?

Primary biliary cirrhosis (PBC) is a chronic, progressive cholestatic liver disease, which is invariably fatal. Circumstantial and indirect evidence suggests that autoimmune mechanisms have a role in the pathogenesis of PBC. Antimitochondrial antibodies (AMA) are highly sensitive and specific markers that can predict the development of the disease in a healthy individual. Long-term administration of ursodeoxycholic acid (UDCA), a naturally occurring bile acid, safely slows the progression of PBC, delays the need for liver transplantation, and postpones death. An effort should be made to identify the patients with PBC in the asymptomatic stage by the presence of AMA and to conduct a clinical trial in order to assess the benefit of long-term administration of UDCA on the prevention of the overt disease in these individuals.

Antibodies↗