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Biomedical subjects

D Zemer

Publications and source records attributed to D Zemer.

At least 37 records · Page 2Linked 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

Acute scrotal pain complicating familial Mediterranean fever in children.

Twenty-nine children with familial Mediterranean fever presented with 39 attacks of acute scrotal pain. Of these, 25 patients had an acute scrotum complicating familial Mediterranean fever and only four had testicular torsion. Scrotal pain was the only manifestation of a familial Mediterranean fever crisis in 36 episodes and in 15 boys scrotal involvement was the first manifestation of the condition. Fourteen patients were treated medically. Of 15 patients who underwent scrotal exploration there were no definite diagnostic findings in 11 and four had testicular torsion. Three cardinal features strongly suggest the diagnosis of acute scrotum in familial Mediterranean fever in a boy of Mediterranean origin with a relevant family history: recurrent scrotal pain or swelling; body temperature above 37.5 degrees C; and gradual onset of pain, usually of more than 12 h duration. Conservative management can safely be undertaken in these boys without fear of losing a salvageable testis.

Acute Disease↗
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

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↗
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

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

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

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↗
PubMed1992

Colchicine prevents kidney transplant amyloidosis in familial Mediterranean fever.

Twenty-one familial Mediterranean fever (FMF) patients who received a kidney transplant for terminal renal failure due to amyloidosis were studied retrospectively to evaluate the prophylactic effect of colchicine on graft amyloidosis. Proteinuria, highly suggestive of kidney transplant amyloidosis, developed in 11 patients within a median of 3 years after transplantation (range 0.5-10 years). In 10 patients, repeated urinalyses for protein were negative during a median of 5 years after transplantation (range 1-13). Patients who developed proteinuria or transplant amyloidosis received smaller colchicine doses than patients without proteinuria--mean 0.69 (range 0-1) versus 1.53 (range 1-2) milligrams per day (p = 0.0002), suggesting that colchicine prevents or delays development of transplant amyloidosis. This prophylactic effect of colchicine was complete at a dose of 1.5 mg/day or more and absent at a daily dose of 0.5 mg or less. In patients who received 1 mg/day, individual variability in the response to colchicine was observed. We conclude that the development of amyloidosis of the kidney transplant in FMF is inevitable at a colchicine dose lower than 1 mg/day, unpredictable at 1 mg/day and usually preventable with 1.5 mg/day or more.

Adolescent↗
PubMed1992

Treatment of resistant rheumatoid arthritis with minocycline: an open study.

In a 48-week open trial, 18 patients with active rheumatoid arthritis (RA), resistant to second line agents, received 200 mg minocycline daily. Twelve patients completed 48 weeks of therapy. Statistically significant improvement was noted in almost all variables of disease activity. Three patients discontinued therapy because of lack of improvement, 2 patients because of side effects and one patient was lost to followup. Cytofluorographic analysis revealed a significant decrease in expression of a T cell activation antigen (gp 26). Our data suggest that minocycline could be a useful therapeutic agent in RA.

Adult↗
PubMed1991

Long-term colchicine treatment in children with familial Mediterranean fever.

Three hundred fifty children (younger than age 16) who had familial Mediterranean fever (FMF) were given continuous prophylactic treatment with colchicine (1-2 mg/day) for 6-13 years. Complete remission of febrile attacks was achieved in 64% of the patients, and partial remission in 31%. Protracted attacks of arthritis virtually disappeared. None of the children developed amyloidosis while on the colchicine regimen. Side effects of colchicine were insignificant, and did not prompt permanent discontinuation of treatment in any of the children. Their growth, development, and subsequent fertility were normal. The efficacy of long-term colchicine treatment of children with FMF makes early diagnosis life saving.

Adolescent↗