Hepatitis B virus vaccination in children with steroid sensitive nephrotic syndrome: immunogenicity and safety?

We investigated the efficacy and safety of Hepatitis B vaccine (HBVac) in steroid sensitive nephrotic syndrome (SSNS) children. 41 patients with SSNS and 30 controls were vaccinated with HBVac(Engerix B(®)). Patients were divided into 3 subgroups:full dose steroid users, alternate-day steroid users and steroid non-users. Seroconversion rate was lower in steroid users than non-users at the 6th(p=0.015) and 12th(p=0.036) months. Antibody to Hepatitis B surface antigen(HBsAb) titers were significantly different between subgroups and controls at the 15th month. However, HBsAb and response rates were not different between subgroups at the 12th and 15th months (p>0.05). Five patients were unresponsive to HBVac. Relapse rates after the vaccination were higher than those in the prevaccination period (p=0.002). HBVac is less effective in producing immune response in SSNS children with steroid therapy. HBVac may trigger relapse in some patients. We recommend HBVac to SSNS children with low dose steroid therapy or after steroids are discontinued.

Ambulatory blood pressure and subclinical cardiovascular disease in patients with juvenile-onset systemic lupus erythematosus.

BACKGROUND: The aim of this study was to evaluate the presence of subclinical cardiovascular disease (CVD) and its relation to risk factors, particularly hypertension in juvenile-onset systemic lupus erythematosus (SLE). METHODS: A total of 24 patients with normal renal function were examined for subclinical CVD by using non-invasive methods, including the measurement of carotid intima-media thickness (IMT), carotid distensibility, aortic pulse wave velocity (PWV) and left ventricular mass (LVM). Blood pressure (BP) pattern and the presence of hypertension were assessed by 24-h ambulatory blood pressure monitoring (ABPM). RESULTS: The patients had higher aortic PWV than the controls (p = 0.011). Increased carotid IMT was present in 48 % of the patients and reduced carotid distensibility in 17 %. Left ventricular hypertrophy (LVH) was present in 22 % of the patients. Eight patients were hypertensive; hypertensive patients had a significantly lower distensibility coefficient (DC)-SDS, higher aortic PWV and higher LVM index than the normotensive patients (p = 0.008, p = 0.023 and p = 0.001, respectively). Higher carotid IMT-standard deviation score (SDS) significantly correlated with higher nighttime diastolic BP-SDS (R (2) = 0.204, p = 0.046); lower DC-SDS correlated with higher nighttime systolic BP-SDS (R (2) = 0.290, p = 0.014). Increased aortic PWV was significantly associated with higher daytime systolic BP-SDS and elevated erythrocyte sedimentation rate (R (2) = 0.607, p = 0.003 and p = 0.010, respectively). PWV was the only independent predictor of LVM index (R (2) = 0.396, p = 0.004). CONCLUSION: These results provide additional evidence for the presence of subclinical CVD and its relation to hypertension in juvenile-onset SLE. They also indicate a significant relation between LVH and increased arterial stiffness. It is also important to note that our findings reveal significant relationships between ambulatory BP and cardiovascular changes and underline the importance of ABPM to predict CVD.

Glucose intolerance: is it a risk factor for cardiovascular disease in children with chronic kidney disease?

A total of 66 children and adolescents with chronic kidney disease (CKD) (20 pre-dialysis patients and 46 chronic dialysis patients) were evaluated to address the prevalence of abnormalities in glucose and insulin metabolism and their association with cardiovascular disease. Glucose intolerance was assessed using an oral glucose tolerance test; insulin resistance was estimated by the homeostasis model assessment of insulin resistance (HOMA-IR). Carotid artery intima-media thickness (IMT) and left ventricular hypertrophy (LVH) were examined as early markers of cardiovascular disease. Thirty-four patients (7 pre-dialysis, 27 dialysis) exhibited an abnormal glucose tolerance; however, ten patients (7 pre-dialysis, 3 dialysis) were insulin-resistant. Height-specific standard deviation scores of carotid artery IMT were above normal in three of the pre-dialysis patients (15%) and in 34 of the dialysis patients (74%). LVH was present in seven pre-dialysis (35%) and 34 dialysis patients (74%). In addition, two of the pre-dialysis patients (10%) and 18 of the dialysis patients (39%) had severe LVH. The prevalence of both increased carotid artery IMT and LVH were similar in patients with or without glucose intolerance in both groups, but severe LVH was more prevalent in pre-dialysis patients with glucose intolerance (p = 0.042). The multivariate analyses showed that neither carotid artery IMT nor LVM index was predicted by serum glucose levels or HOMA-IR. In conclusion, children with CKD are at a high risk of glucose intolerance and also have a greater risk of subclinical cardiovascular disease (CVD). However, the presence of glucose intolerance does not appear to be an independent risk factor for increased carotid artery IMT or LVH.

Is it safe to use anti-TNF-α agents for tuberculosis in children suffering with chronic rheumatic disease?

To determine the incidence of latent tuberculosis infection and evaluate the follow-up protocol of the patients diagnosed with juvenile idiopathic arthritis (JIA) and other chronic rheumatologic diseases treated with anti-TNF-α treatment (etanercept, infliximab, adalimumab) in Turkey, 144 patients were evaluated retrospectively for the development of tuberculosis. Patients were evaluated every 6 months for tuberculosis using history, physical examination, tuberculin skin test (TST), chest radiographs, and, when required, examination of sputum/early morning gastric aspirates for acid-fast bacilli and chest tomography. A tuberculin skin test over 10 mm induration was interpreted as positive. Patients were diagnosed with JIA (n = 132), enthesitis-related arthritis (ERA; n = 14), juvenile psoriatic arthritis (JPsA; n = 4), chronic idiopathic uveitis (n = 4), and chronic arthritis related to FMF (n = 8). Mean age was 12.25 ± 3.96 years (4.08-19.41 years), mean duration of illness was 5.86 ± 3.77 years (0.66-15 years), and the mean duration of anti-TNF-α treatment was 2.41 ± 1.47 years (0.6-7 years). Anti-TNF-α agents prescribed were etanercept (n = 133), infliximab (n = 30), and adalimumab (n = 6). When unresponsive to one anti-TNF-α therapy, patients were switched to another. There was no history of contact with individuals having tuberculosis. During follow-up, seven patients (4.8%) with positive TST were given INH prophylaxis. One oligoarticular JIA patient (0.69%) diagnosed with secondary uveitis who had been followed for 5 years and had been using infliximab for 2 years, developed a positive Quantiferon-TB test while on INH prophylaxis. He was started on an anti-tuberculosis drug regimen. In conclusion, anti-TNF-α treatment in children with chronic inflammatory disease is safe. Follow-up every 6 months of children on anti-TNF-α treatment with respect to tuberculosis by the pediatric infectious disease department is important to prevent possible complications.

Economic impact of juvenile idiopathic arthritis and familial Mediterranean fever.

The aim of the study was to determine the economical impact of juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF) in Turkey. A total of 100 patients (69 F/31 M) with JIA and 100 with FMF (68 F/32 F) who were consecutively seen in the outpatient clinic of the pediatric rheumatology department at Cerrahpasa Medical School between August 2008 and January 2009 were studied. Cost data were collected through a questionnaire filled out by the parents. The mean age (JIA: 11 ± 5 years; FMF:12 ± 4 years) and mean disease duration (JIA:5 ± 3 years; FMF: 4 ± 3 years) of the patients were similar. JIA patients were assigned to 5 subtypes (polyarticular: n = 45, oligoarticular: n = 30, systemic onset: n = 13, psoriatic: n = 6, and enthesopathy-related JIA: n = 6). Forty-nine percent of the patients with JIA were treated with anti-TNF drugs and 61% with DMARDs. All patients with FMF were using colchicine. The total annual cost of JIA (3,994 ± 4,101) was considerably higher than that of FMF (162 ± 77) (P < 0.001). Medication fee was the major determinant of total costs in both diseases constituting 85% in JIA and 39% in FMF. Among the subtypes of JIA, total annual costs were the highest among patients with polyarticular type (6,045 ± 4,078). Medications especially anti-TNF drugs were the major contributor among all determinants of costs in JIA. The low costs of health care system and prominent changes in the health care policies for the last 5 years in Turkey might have played role in our findings.

Hepatitis B vaccination in juvenile systemic lupus erythematosus.

OBJECTIVES: In this study, we examined the antibody responses after recombinant hepatitis B vaccine in juvenile SLE patients and whether antibody levels were affected by immunosuppressive therapy. METHODS: This study consisted of 64 juvenile SLE patients and 24 healthy controls. We evaluated HBsAg, Anti-HBs and Anti-HbcIgG titers in SLE patients. 24 patients (37%) were non-immunised, 39 patients were immunised (61%) and 1 patient (1.5%) was chronic hepatitis B carrier. Of the 24 non-immunised patients, 3 had active disease (SLEDAI>10) and 1 was being treated for tuberculosis infection so they were not included in the vaccination program. Twenty non-immunised SLE patients were given 3 dose recombinant hepatitis B vaccine doses at 0,1,6 months. AntiHBs antibody titer >10 IU/ml one month after the last dose of vaccine was accepted as seroconversion. RESULTS: After 3 doses of vaccination, 16 (80%) of SLE patients and all of the healthy controls had seroconversion. Since two patients had SLEDAI score >10 after the first 2 doses of vaccine and one patient had SLEDAI score >10 after the first dose, these patients were given only two doses of hepatitis B vaccine. These patients had already seroconverted. One patient had exacerbation of the disease one month after the third dose of the vaccine. Protective antibody responses were statistically insignificant between the two groups (p=0.49). Geometric mean antibody titers of SLE patients were lower than those of the healthy controls. Adequate antibody response was not affected by immunosuppressive treatment as prednisone, azathioprine, and hydroxychloroquine. CONCLUSIONS: Juvenile SLE patients could reach an adequate antibody response after recombinant hepatitis B vaccination and this response is not affected by immunosuppressive treatment.

Analysis of MEFV exon methylation and expression patterns in familial Mediterranean fever.

BACKGROUND: MEFV mutations and decreased expression level of the gene are related to FMF pathology. DNA methylation at CpG islands is a well-known mechanism for transcriptional silencing. MEFV has a CpG island, spanning a part of the first intron and the whole of the second exon of the gene covering 998 bp region. Here, we tested the hypothesis that the MEFV transcript level in FMF patients correlates with its methylation level, and methylation, by allowing transcription silencing, has a role in FMF ethiopathogenesis. METHODS: The study group was composed of pediatric FMF patients (N = 51) and age-gender matched healthy controls (N = 21). The relative expression level of MEFV was assessed via quantitative real-time PCR (qRT-PCR) and bisulfite sequencing (BS) was performed to analyse the methylation level quantitatively. RESULTS: MEFV expression in FMF patients were decreased compared to healthy controls (P = 0.031). Methylation level of exon 2 of MEFV was found to be slightly higher in FMF patients compared to healthy controls (76% versus 74%) (P = 0.049). The expression level of the MEFV was negatively correlated with the methylation level of the CpG island in both FMF and healthy controls groups (cor = -0.29, P = 0.041) but more so in the FMF only group (cor = -0.36, P = 0.035). CONCLUSIONS: In this study, the relation between reduced MEFV expression level and FMF was confirmed. Observed slight increase in methylation in FMF patients, and correlation of methylation with expression might be indicative of its role in FMF, however a larger dataset is needed to confirm our preliminary findings.

Dialysate CA125 levels after 5 years on continuous peritoneal dialysis.

The aim of this study was to evaluate longitudinal changes in dialysate cancer antigen 125 (dCA125) levels over time and to analyze relationships between dCA125 and peritoneal glucose exposure (PGE) in children undergoing long-term peritoneal dialysis (PD). The study group included seven boys and four girls (mean age 13 ± 5.1 years) with a mean PD duration of 84.0 ± 1.1 months. A peritoneal equilibration test (PET) was performed, and dCA125 levels were measured in all patients. Peritoneal appearance rates (AR) of dCA125, the velocity of the decrease in dCA125AR values, and annual PGE levels were also calculated. The final tests were performed at a mean of 63.3 ± 3.5 months after the initial ones. Both dCA125 and dCA125AR levels showed statistically significant decrements during the follow-up period (p = 0.003), with the velocity of decrease in dCA125AR found to be 52.6 ± 19.4%. There were no significant differences in peritoneal transport parameters between the beginning and end of the study period. PGE values were significantly higher in the last year of the study than in the first year (p = 0.014), but the velocity of the decrease in dCA125AR levels was not related to total PGE. In conclusion, a significant decline was found in dCA125 and CA125 AR levels, reflecting mesothelial cell mass, in children undergoing long-term PD (>5 years), but these were not related to PGE.

The distribution of juvenile idiopathic arthritis in the eastern Mediterranean: results from the registry of the Turkish Paediatric Rheumatology Association.

OBJECTIVES: To analyse the demographics, main clinical and laboratory features and subtype distribution of juvenile idiopathic arthritis (JIA) in an eastern Mediterranean country, based on a multicentre registry. METHODS: Between March 2008 and February 2009 with this cross-sectional study, consecutive patients seen with JIA in selected centres were registered through a web-based registry. All patients were classified according to the International League of Associations for Rheumatology (ILAR) criteria. RESULTS: There were 634 patients with a mean age of 11.84 ± 4.66 years and the female/male ratio was 1.2. The distributions of JIA patients according to onset of disease were as follows: systemic 92 (14.5%), oligoarticular extended 26 (4.1%), oligoarticular persistent 234 (36.9%), rheumatoid factor (RF) positive polyarthritis 20 (3.2%), RF negative polyarthritis 129 (20.3%), enthesitis-related 120 (18.9%), psoriatic 13(2.1%). The frequency of uveitis was 15.7% among all of the oligoarthritis patients. Anti-nuclear antibody (ANA) was positive mainly among the oligoarticular onset patients. Twenty-one patients also had Familial Mediterranean fever (FMF). Among systemic JIA patients, the frequency of macrophage activation syndrome (MAS) was 15.2% (n=14). At the end of the mean follow-up of 7.6 ± 4.4 years, 305 (48.1%) patients were defined to have inactive disease on medication, and 106 (16.7%) were completely free of any disease symptoms without medication. CONCLUSIONS: Enthesitis related arthritis had a high frequency whereas psoriatic arthritis was very rare compared to other series. We suggest that there are certain differences in the characteristics of JIA in our eastern Mediterranean population. Thus, genetic studies need to be assessed in these populations separately and findings of genome wide association studies need to be confirmed in different populations.

Do infections trigger juvenile idiopathic arthritis?

Juvenile idiopathic arthritis (JIA) is a disease that was prominent with increased inflammation response in immune system, appeared mostly with peripheral arthritis and endogenous and exogenous antigens play a role in the pathogenesis of disease. Two major reasons were thinking to be considerably important. First of them is immunological predisposition and the second one is environmental factors. Infections are considered to be the most important between environmental factors but also stress and trauma are also important in the etiology of the disease. However, the relation between JIA and infections is not clearly defined but the relation between adult chronic arthritis and infections was well-defined. A total of 70 patients, 26 with primer JIA, 20 with recurrent JIA, 24 healthy control were included in this study. Mycoplasma pneumoniae, Chlamydophila pneumoniae and C. Jejuni were detected in 4, 1 and 1 of 10 (38.46%) patients with primer JIA, respectively. Salmonella enteritidis, EBV, M. pneumoniae, C. jejuni and Borrelia burgdorferi were detected in 1, 2, 2, 2, and 1 of the 8(40%) patients with recurrent JIA, respectively. S. enteritidis were isolated in feces culture and also identified by agglutination method. Infection was detected in total 18 (39.13%) of patient groups. C. pneumoniae and C. jejuni were detected in 1 and 1 of 2(8.33) healthy control groups, respectively. Throat culture positivity was not detected in any of the patient and healthy control groups. In conclusion, etiopathogenesis of JIA is not clearly understood and suggested that various factors can trigger the disease and it is the most common rheumatoid disease of childhood. However, there are some studies focusing especially on one infectious agent but this is the first study including such a big range of infectious agents in the literature for the microorganisms that can be suggested to have a role in the etiopathogenesis of JIA. We have a conclusion in the light of our results and suggest that some microorganisms can trigger and increase the intensity of clinical situation according to the case. When we evaluate the primer and recurrent JIA groups; M. pneumoniae and C. jejuni come forward and seen common in JIA cases. We also suggest that the pre-diagnosis of microorganisms, which can play a role as primarily or by intervening in the etiopathogenesis of JIA and adding specific antimicrobial therapy to the standard JIA therapy, it is possible to perform new, extended, especially molecular based serial case studies.

Abdominal manifestations of polyarteritis nodosa demonstrated with CT.

We report a rare case of polyarteritis nodosa (PAN) presenting in childhood. The child had multiple visceral aneurysms and later developed ascending colitis and jejunitis. The diagnosis was established with multidetector CT and CT angiography.

Left ventricular function by 'conventional' and 'tissue Doppler' echocardiography in paediatric dialysis patients.

AIM: Cardiovascular abnormalities are common in children with chronic kidney disease (CKD). Left ventricular (LV) structure and functions have been extensively studied by conventional pulse-wave Doppler echocardiography (cPWD), however, tissue Doppler imaging (TDI) is a relatively new echocardiography method. The aims of this study were to evaluate LV diastolic function in paediatric dialysis patients using cPWD and TDI methods, and to compare the findings obtained with two modalities. METHODS: This study included 38 children and adolescents on dialysis (14 haemodialysis and 24 peritoneal dialysis, duration of dialysis 58.0 +/- 32.8 months) and 16 age- and sex-matched healthy subjects. RESULTS: The mean left ventricular mass index (LVMI) was significantly higher in the patient group (P < 0.001) and the most common cardiac geometry was concentric LV hypertrophy (55%). There was no significant difference in LV systolic function between patient and control groups. However, dialysis patients had worse LV diastolic function both according to cPWD (lower E/A ratio) and TDI (lower Em/Am ratio) than the healthy subjects (P < 0.001 and P = 0.001, respectively). Also, the index of LV filling pressure (E/Em ratio) obtained by the combination of cPWD and TDI was significantly higher in the patients (P < 0.001). Cumulative dose of calcium-based phosphate binder (CBPB), diastolic blood pressure and LVMI were the independent predictors of E/Em ratio. CONCLUSION: Our study shows that LV diastolic dysfunction is common in paediatric dialysis patients and TDI findings correlate well with cPWD findings. Similarly, higher dose intake of CBPB, hypertension and LV hypertrophy have a negative effect on LV filling pressure suggesting diastolic function.

Purified protein derivative response in juvenile idiopathic arthritis.

OBJECTIVE: To examine the purified protein derivative (PPD) response that develops depending upon Th1 immune response in children with juvenile idiopathic arthritis (JIA). METHODS: PPD skin test was performed in 115 children with JIA who were vaccinated with bacillus Calmette-Guerin (BCG), and then they were compared to the PPD response of 45 healthy children of the same age who were vaccinated with BCG. Children with a PPD induration > or = 5 mm were accepted as PPD-positive. PPD induration > or = 10 mm was accepted as a limit for suspecting tuberculosis. RESULTS: PPD induration size and PPD positivity rates (PPD > or = 5 mm) of children with JIA were significantly lower than those of healthy children. The mean of PPD induration size was significantly lower (p < 0.0001) in patients with either 1 BCG vaccine (3.7 +/- 3.6) or more than 1 BCG vaccine than controls with either 1 BCG vaccine (7.10 +/- 3.2) or more than 1 BCG vaccine (10.05 +/- 4.1). PPD was positive in 35.9% of patients with JIA vaccinated once (n = 32), in 50% of patients with JIA vaccinated more than once (n = 13), in 82.1% of controls vaccinated once (n = 23), and in 88.2% of controls vaccinated more than once. This result was statistically significant (patients, p = 0.03; controls, p = 0.039). It was determined that neither the activity of the disease nor the use of corticosteroid and methotrexate affected the PPD response. CONCLUSION: The response to PPD, which is one of the Th1 cell-type responses, was significantly lower in BCG-vaccinated children with JIA compared to healthy children.

Blood volume monitoring to adjust dry weight in hypertensive pediatric hemodialysis patients.

The aim of this study was to adjust dry weight by short-term blood volume monitoring (BVM)-guided ultrafiltration and evaluate the effects of optimizing dry weight on blood pressure (BP) control and intradialytic symptoms (IDS) in a group of hypertensive hemodialysis (HD) patients. The study was performed in four sequential phases, each of which lasted for 1 week, on nine hypertensive HD patients (six girls, age 16.9 +/- 3.1 years). In phase I, patients were observed by BVM. In phase II, BVM was used to guide ultrafiltration to adjust dry weight. Antihypertensive drugs were gradually tapered or withheld in phase III, when the patients were hypotensive and/or their IDS increased. In phase IV, this particular weight was maintained without any intervention. Pre- and post-HD body weight, pre-HD, post-HD, 30 min after HD casual BP values, and IDS in each HD session were recorded. The BP was also assessed by 44-h ambulatory BP monitoring (ABPM), which is an ideal method to determine BP changes throughout the interdialytic period at the beginning of phase I and at the end of phase IV. There was a decrease in mean dry weight, all casual systolic BPs, and systolic/diastolic ABPM at the end of the study (all p < or = 0.05). Antihypertensive drugs were stopped in five patients and reduced in two during phase III of the study. The IDS was more frequent (36%) in phase IV than in phase I (16%); however, this increase did not reach statistical significance. The results of this study suggest that short-term BVM guided-ultrafiltration may be a useful tool to diagnose volume overload and to adjust dry weight and, consequently, to achieve a better control of BP in pediatric HD patients.

Progression of coronary calcification in pediatric chronic kidney disease stage 5.

Coronary artery calcification (CAC) is common in adults with chronic kidney disease (CKD) and progresses with time. However, data are limited for younger patients. We have previously reported CAC in eight of 53 children with CKD. After 2 years, CAC evaluation was repeated in 48 patients. The median CAC score (CACS) increased from 101.3 (1473.6 +/- 1978.6, range 8.5-4332) to 1759.2 (2236.4 +/- 2463.3, range 0-5858) Agatston units (AU). When the individual changes in CACS were evaluated one by one, we showed a mild decrease in two patients on hemodialysis (HD) and in one transplant (Tx) recipient, a moderate increase in one patient on HD, one on peritoneal dialysis (PD) and one Tx recipient, and a large increase in one HD patient. Also, CAC disappeared in one HD patient. All patients with no calcification at baseline remained calcification-free at follow-up. To obtain the individual cumulative exposure, we calculated time-averaged mean values, using the laboratory values from the beginning of dialysis to the first and second multidetector spiral computed tomography (MDCT) scans (baseline and final values, respectively). Final CACS was positively related to final calcium-phosphorus (CaxP) product, while CAC progression was inversely associated with final serum albumin level. This report is the first study with the largest number and the youngest cohort to document the natural history of coronary calcification.

Left ventricular systolic and diastolic function and carotid intima-media thickness in pediatric dialysis patients.

BACKGROUND: Cardiovascular complications are common in patients with end-stage renal disease (ESRD). We aimed to investigate left ventricular (LV) function and carotid intima-media thickness (cIMT) in children and adolescents with ESRD. METHODS: This study included 38 ESRD patients (15 hemodialysis and 23 peritoneal dialysis) and 17 age- and sex-matched healthy subjects. RESULTS: The ESRD patients had significantly lower mean mitral E/A ratio, and higher left ventricular mass index (LVMI) and cIMT than the control group. Compared with PD patients, HD patients had worse LV diastolic function. In stepwise linear regression analysis, LVMI (P = 0.043) and hemoglobin (P = 0.015) turned out to be independent variables for predicting diastolic dysfunction (reduced E/A ratio), and the only significant predictor of cIMT was indexed diastolic blood pressure (DBP) (P = 0.035). CONCLUSION: Cardiovascular structure and function abnormalities are also common in pediatric dialysis patients, as in adults. Furthermore our data indicated that hemodialysis was disadvantageous for preserving LV diastolic function as compared with peritoneal dialysis.

MEFV mutations modify the clinical presentation of Henoch-Schönlein purpura.

OBJECTIVE: To investigate the prevalence of MEFV gene mutations in Turkish patients with Henoch-Schönlein purpura (HSP) but with no symptoms of familial Mediterranean fever (FMF). In addition, we assessed the clinical and laboratory characteristics of HSP patients with and without MEFV mutations. METHODS: Eighty pediatric patients with HSP (44 boys and 36 girls) were enrolled. Blood for mutation analysis was obtained either at the time of the diagnosis of HSP or during followup visits in previously diagnosed patients. No patient had the diagnosis of FMF in their history and in the followup period. Exon 10 of the MEFV gene was screened, together with p.E148Q mutation analysis. RESULTS: Twenty-seven (34%) patients were found to be heterozygous for one of the screened MEFV mutations; p.M694V in 16, p.M680I in 5, p.V726A in 3, and p.E148Q in 3 patients. Patients with MEFV mutations were younger than those without mutations and they had edema and arthritis more frequently. Also, the frequencies of elevated erythrocyte sedimentation rate and C-reactive protein values were found to be significantly higher in patients who had MEFV mutations. CONCLUSION: Alterations in the MEFV gene are important susceptibility factors for the development of HSP and also affect the clinical presentation of it.