Cardiac and intestinal amyloidosis in a renal transplant recipient with familial Mediterranean fever.

In Turkey, familial Mediterranean fever (FMF) is an important cause of nephrotic syndrome and endstage renal disease due to renal deposition of AA type amyloid. We report a case of living-related donor renal transplant recipient with FMF and renal AA type amyloidosis, who died of progressive heart failure due to cardiac involvement. The patient also had intractable diarrhea caused by biopsy-proven intestinal amyloidosis. The patient was on 1 mg/day colchicine. Although he was attack-free throughout the post-transplant period, intestinal and clinically significant cardiac amyloidosis, which implied the presence of sustained inflammation and continuing amyloid deposition, appeared three years after renal transplantation. Cardiac deposition of AA amyloid may cause clinically significant heart disease, leading to cardiovascular mortality after renal transplantation for end-stage renal disease in FMF patients.

No association between deletion-type angiotensin-converting enzyme gene polymorphism and left-ventricular hypertrophy in hemodialysis patients.

Left-ventricular hypertrophy (LVH), a bad prognostic sign, is a common finding in hemodialysis patients. The aim of the study was to analyze factors, including angiotensin-converting enzyme (ACE) genotype that may have an effect on the development of LVH in hemodialysis patients. Seventy-nine hemodialysis patients (42 males, 37 females, mean age 37.7 +/- 13.1 years) and 82 age- and sex-matched normotensive healthy controls (40 males, 42 females, mean age 35.6 +/- 5.7 years) were included. Left-ventricular mass index (LVMI) was higher in the hemodialysis group compared to controls (170.1 +/- 69.3 versus 84.9 +/- 15.7 g/m(2), p < 0.001). Fourty-three hypertensive patients in the hemodialysis group had an increased LVMI compared to 36 normotensive hemodialysis patients (194.2 +/- 75.5 versus 141.2 +/- 48.0 g/m(2), p < 0.001). On univariate analysis, LVMI was found to be correlated with blood pressure (r = 0.38, p < 0.001), time spent on dialysis (r = 0.22, p = 0.02) and hemoglobin levels (r = -0.21, p = 0.03). No correlation was found between LVMI and age (r = 0.09, p = 0.22), predialytic creatinine (r = 0.09, p = 0.21) and albumin (r = -0.10, p = 0.18). On multivariate analysis for the predictors of LVMI, blood pressure, time spent on dialysis and hemoglobin levels were also found to be significant. LVMI in DD, ID and II genotypes were 155.0 +/- 71.2, 181.6 +/- 60.6, and 163.6 +/- 83.4 g/m(2), respectively (p > 0.05). No association between LVMI and DD genotype was found. ACE genotype distribution was similar in hemodialysis patients and healthy controls. It was concluded that LVH in hemodialysis patients was mainly related to hypertension, anemia and time spent on dialysis and the DD genotype had no effect on LVMI in hemodialysis patients.

Immune response to Haemophilus influenzae type B vaccination in renal transplant recipients with well-functioning allografts.

BACKGROUND: Haemophilus influenzae infection is a mild and self-limited disease in the healthy population. However, it may show an aggressive course in the immunocompromised state which underlines the importance of vaccination against this agent. On the other hand, posttranplant immunosuppression may impair immune responses and thus the efficacy of the vaccination. METHODS: Forty-three renal transplant recipients with well-functioning allografts were immunized with H. influenzae type b vaccine in order to investigate the immune response. The patients received a double or a triple immunosuppressive protocol. Seven healthy members of the dialysis unit served as controls. After obtaining basal serum samples, the patients and the control subjects were immunized with H. influenzae type b conjugate vaccine. After 6 and 12 weeks, serum samples obtained again to determine H. influenzae type b antibody titers. RESULTS: The antibody titers 6 and 12 weeks after vaccination were significantly higher as compared with the basal values, similar to those of the control subjects. These titers did not show statistically significant differences between the double and triple immunosuppressive therapy groups. After 12 weeks of vaccination, the antibody titers did not show a statistically significant difference as compared with those obtained after 6 weeks. CONCLUSION: H. influenzae type b vaccination is safe and effective in patients with well-functioning renal allografts and should be recommended to renal transplant recipients who may have the risk of invasive disease on the basis of the immunosuppressive state.

Transplantation in renal amyloidosis.

In this report, the results of renal transplantation in patients with renal amyloidosis were retrospectively analysed and compared with the control group. Fifteen (3.04%) of the 493 renal transplant recipients whom were followed up in Istanbul School of Medicine transplant outpatient clinic, between 1983 and 1997, were included in the study. The etiology of amyloidosis was familial Mediterranean fever in all patients. The mean follow-up period was 38.3 +/- 31.8 (range 7-65) months. Twelve of the patients were male and 3 female with the mean age 34.13 +/- 10.87 (range 21-60) years. Seven patients had living related, 4 living-unrelated and 4 cadaveric donors. Five patients were lost because of different complications: Three patients died from cardiac amyloidosis all with well functioning grafts, 2, 3 and 36 months after the operation. Sepsis and cardiovascular failure was the probable cause of death in 1 patient who also had chronic rejection. Another one patient with chronic rejection died from hepatic failure. Acute rejection developed in 2 patients. Renal functions of these patients improved by anti-rejection therapies. Chronic rejection developed in 3 patients. In the control group, acute rejection and chronic rejection were diagnosed in 5 and 1 patients, retrospectively. While 1 patients returned to hemodialysis in control group, the others are alive with satisfactory graft function. There was no death in control group. The 5-yr graft and patient survival rates in amyloidosis and the control groups were 75, 77, 95 and 100%, respectively. It was concluded that although transplantation is not a contraindication for the treatment of end stage renal failure in patients with renal amyloidosis, it carries high risk of cardiac complications in the postoperative period. Detailed preoperative cardiovascular evaluations are mandatory in these patients and this intervention should improve the prognosis by excluding the patients who have already been complicated with this problem.

Prevalence of ventricular premature complexes in healthy and untreated hypertensive elderly people.

Twenty-four-hour ambulatory electrocardiographic examination was performed in 130 elderly patients (49 healthy, active subjects; 40 hypertensive without left ventricular hypertrophy; and 41 hypertensive with left ventricular hypertrophy). In the study group, mean age was 72.4 +/- 7.1 (sixty-five to eighty-eight) years. A significantly high prevalence of ventricular ectopic complexes was found both in hypertensive and in normotensive groups. According to the Lown grades, in grade 1, VPCs had very high prevalence in healthy subjects, in grade 2, VPCs had a high prevalence in hypertensives with left ventricular hypertrophy (73.5% and 48.8%, respectively). The results of this study demonstrate that ventricular premature complexes were common in hypertensive patients and healthy elderly but did not cause high complexity in either group. Ventricular premature complexes detected by ambulatory ECG monitoring in healthy, active subjects and in untreated hypertensive patients are not an independent risk factor in elderly patients.

Left ventricular mass index and prevalence of heart disease in the population aged 80 years and over.

Clinical, electrocardiographic and echocardiographic findings of 69 subjects aged 80 years or over were analyzed in order to assess the prevalence of left ventricular mass, hyperlipidemia, hypertension and cigarette smoking. Of the 69 subjects studied, 41 had no symptoms or sign of cardiovascular disease, 28 had one or more cardiac symptoms (NYHA stage 2-4). 25 had electrocardiographic evidence of left ventricular hypertrophy and there were no differences between the asymptomatic and symptomatic groups. Echocardiographically, the left ventricular mass index ranged between 103 to 247 g/m2 in men and 170 to 251 g/m2 in women. In 36 subjects with high left ventricular mass index, the ventricular septal thicknesses ranged from 12 mm to 15 mm in 19 subjects, and posterior wall thicknesses ranged from 12 mm to 16 mm in 17 subjects. Of the 58 patients with an adequate echocardiogram, 47 had clinically diagnosed hypertension (81%). In our study population, a prevalence of left ventricular hypertrophy (62%), isolated systolic hypertension (26%), definite hypertension (33.3%), high LDL-cholesterol (63%), low HDL-cholesterol (26%), abnormal Q wave (16%), cigarette smoking (47.8%) and diabetes mellitus (1.4%) were found.