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.

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.

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.

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.

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.

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.

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.

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.

A case of catastrophic antiphospholipid syndrome in an adolescent girl with parvovirus B19 infection.

Antiphospholipid syndrome is an autoimmune disease characterized by recurrent thrombosis and the presence of antiphospholipid antibodies. Clinical presentations are dependent on the affected vessels and organs. The most common presentation of antiphospholipid syndrome is arterial or venous thrombosis. An unusual presentation of the disease is characterized by microvascular thrombosis with multiorgan involvement, which is termed catastrophic antiphospholipid syndrome. The diagnosis of catastrophic antiphospholipid syndrome can be difficult because of the heterogeneity of the different clinical forms. Clinical manifestations of catastrophic antiphospholipid syndrome are complex with multiple organ involvement, resulting in renal insufficiency, heart failure, acute respiratory distress syndrome, and liver involvement. Early diagnosis and aggressive therapies are essential in this condition because of the extremely high mortality rate. Herein, the case of a 14-year-old girl with catastrophic antiphospholipid syndrome that was previously misdiagnosed as a vasculitis related to parvovirus B19 infection is presented.

Prevalence of the MEFV gene mutations in childhood polyarteritis nodosa.

OBJECTIVES: To test the hypothesis that alterations in the Mediterranean fever (MEFV) gene are a susceptibility factor for the development of polyarteritis nodosa (PAN) we investigated the prevalence of MEFV mutations in patients with PAN without any symptoms of familial Mediterranean fever (FMF). STUDY DESIGN: Pediatric patients with PAN (n = 29) were enrolled in this study. Six predominant mutations (p.M694V, p.M680I, p.M694I, p.V726A, p.K695R, p.E148Q) in the MEFV gene were studied. RESULTS: Fifteen MEFV mutations were identified in 58 chromosomes. Eleven of the 29 patients (38%) were found to carry MEFV mutations. Three (10.3%) of them had homozygous p.M694V mutation, and one of the patients (3.4%) had compound heterozygous mutation (p.V726A/p.E148Q). CONCLUSIONS: Our study confirms that alterations in the MEFV gene are important susceptibility factors for the development of PAN. We believe that mutations in MEFV gene provide a basis for the development of PAN both by forming a proinflammatory state and by possibly giving exaggerated response to streptococcal infections.

A child with primary Sjögren syndrome and a review of the literature.

Primary Sjögren syndrome (pSS) is an uncommon disease in childhood. Childhood pSS might have different clinical manifestations than adult pSS. We describe a 13-year-old girl with multiple episodes of bilateral parotid swelling lasting 2 years. Her history included severe arthralgia, local edema, and purpura episodes since 9 years of age. During her 3-week hospitalization, 2 episodes of parotid swelling occurred, which both resolved in 48 hours. Ultrasonography and magnetic resonance images of parotid glands showed parenchymal inhomogeneity related to adipose degeneration and nodular pattern. Investigations showed elevated erythrocyte sedimentation rate, the presence of hypergammaglobulinemia, positive antinuclear antibody, and elevated rheumatoid factor, anti-Sjögren syndrome antigen A, and anti-Sjögren syndrome antigen B. Histopathologic examination of labial minor salivary glands revealed focal periductal lymphocytic infiltrate and sialoduct ectasia. She was diagnosed as having pSS. Recurrent parotid swelling is a more characteristic feature of disease in children, and this finding should alert the clinician to the possible diagnosis of pSS.

Is there any relationship between Chlamydophila pneumoniae infection and juvenile idiopathic arthritis?

The role of Chlamydophila pneumoniae in the development and exacerbation of juvenile idiopathic arthritis (JIA) was investigated. Blood samples were taken from 60 JIA patients during an active disease period and for 4 weeks after. Synovial fluid samples were obtained from 20 of the 60 patients. In addition, 22 patients with familial Mediterranean fever (FMF) during the active period and 35 healthy children were included in the study as control groups. Synovial fluid samples were also obtained from three children with FMF. IgG, IgM and IgA levels against C. pneumoniae in serum samples were studied by immunofluorescence and IgG antibody and PCR studies were performed for C. pneumoniae DNA in synovial fluid samples. Twenty-nine (48.3 %) patients with JIA, 18 (81.8 %) patients with FMF and 22 (62.8 %) healthy children were found to be pre-infected with C. pneumoniae. Pre-infection with C. pneumoniae among FMF patients was found to be significantly higher than among those with JIA. We did not find a significant difference between JIA patients and healthy children. Chronic C. pneumoniae infection was observed only in six JIA patients, one FMF patient and two healthy children. Synovial fluid antibodies were found at higher than 1/512-fold dilution in one JIA patient and four times higher than normal serum in three JIA patients. C. pneumoniae DNA was not detected in any synovial fluid sample from FMF or JIA patients by PCR. In conclusion, C. pneumoniae infection does not have a triggering or a progressive effect on the clinical situation in JIA aetiopathogenesis, as a result of a multifactorial aetiology. New, extensive and serial studies (especially PCR studies of synovial tissue) are needed in order to confirm the indirect results.

Evaluation of bone with quantitative ultrasound in healthy Turkish children.

In this study bone status was assessed using a quantitative ultrasound (QUS) technique at the calcaneus in 141 healthy, prepubertal, Turkish schoolchildren (80 girls, 61 boys) aged 6-12 years. Broadband ultrasound attenuation (BUA, DB/MHz) was measured with a prototype pediatric contact bone analyzer (CUBA, McCue Ultrasonics Ltd). The relation of age, body weight and height to BUA was assessed. BUA increased linearly with age in boys and girls (R=0.448, p=0.0001 and R=0.382, p=0.002, respectively). BUA values in boys tended to be higher than in girls, reaching significance only at the age of seven years with a 95% confidence interval. In conclusion, the measurement of BUA in the calcaneus with QUS has important clinical implications in assessing bone mass in children. Further studies in not only healthy children but also in those with metabolic bone diseases would be helpful in order to evaluate its sensitivity and reproducibility.

Uveitis and anti nuclear antibody positivity in children with juvenile idiopathic arthritis.

This study was conducted to determine the frequency of antinuclear antibodies (ANA) positivity and uveitis in our newly diagnosed juvenile idiopathic arthritis (JIA) patients classified according to International League Against Rheumatology (ILAR) classification criteria. Ninety-two girls and 106 boys, totally 198 children were enrolled in the study. of them 36 (18.2 percent) were found to be ANA positive. Chronic anterior uveitis was detected in 20 (10.1 percent) patients. ANA positivity was determined in 4 of the systemic JIA patients, in whom no uveitis had been detected. Twenty-five of 37 patients with oligoarticular JIA were ANA positive, in 10 of them uveitis was also diagnosed. ANA were positive in 3 of 34 patients with RF positive polyarticulat JIA, only one patient had positive ANA, and another one had uveitis. Nine patients were extended JIA and in none of them, ANA positivity or uveitis were present. Of 43 patients classified as enthesitis related arthritis (ERA), uveitis was diagnosed in 6 and there was no evidence of ANA positivity, but one had uveitis. We conclude that the incidence of ANA positivity and uveitis is low in Turkish children with JIA.

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.