Juvenile Rheumatoid Arthritis Patients Research Articles

Обеспеченность витамином D и минеральная плотность костной ткани у больных ювенильным ревматоидным артритом

Актуальность. Мониторирование минеральной плотности костной ткани (МПКТ) у больных ювенильным ревматоидным артритом (ЮРА), дополненное определением сывороточного уровня кальцидиола (25(ОН)D) является индикатором системности процессов катаболизма и критерием успешности терапии. Цель. Оценка показателей денситометрии, уровня кальцидиола у больных ЮРА для определения остеопенического синдрома, целесообразности назначения дополнительных доз витамина D. Материалы и методы. Оценено значение 25(ОН)D и МПКТ у 65 больных ЮРА. Данные обработаны с применением xи-квадрата Пирсона и рангового корреляционного анализа по Спирмену. Результаты. У 92 % больных ЮРА определяется нарушение обеспеченности витамином D. При проведении двухэнергетической рентгеновской абсорбциометрии (DXA) выявлено, что 60 % больных имеют вторичный остеопенический синдром (Z-score ≤ –2 SD). У пациентов, которые получали иммунобиологические препараты в комплексном лечении, наблюдаются достоверно более высокие показатели МПКТ (р < 0,01). Показатель МПКТ имеет сильную негативную корреляционную зависимость от степени активности болезни (коэффициент корреляции (r Spearman’s) равен –0,72), умеренную зависимость от уровня 25(ОН)D (r Spearman’s равный 0,39). Выводы. В процессе наблюдения и мониторинга лечения больных ЮРА необходимо контролировать уровень 25(ОН)D и МПКТ. 68 % больных ЮРА, которые в качестве базисной терапии получают плановое лечение иммунобиологическими препаратами, имеют нормальную МПКТ.

Relationship of C-Reactive Proteins and Erythrocyte Sedimentation Rate with Active Disease in Patients with Juvenile Rheumatoid Arthritis

Objective: To determine the relationship between serum C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) with active disease in patients with juvenile rheumatoid arthritis (JRA). Methods: This is a cross-sectional analytical study conducted at the Department of Paediatrics, Abbasi Shaheed Hospital, Karachi for one-year duration from April 2020 to April 2021. All patients who met the MJA’s American College of Rheumatology (ACR) criteria were enrolled in the study. The ACR 20 improvement criteria were used to define the pardon of the disease, and those who met the ACR20 recovery criteria were included in the remission group. Laboratory tests such as PCR and ESR. Statistical analysis was performed using SAS software (version 10.3). Results: The study involved 90 patients, including 28 in the remission group (31.2%) and 62(68.88%) in the active disease group. There were 61.11% (n = 55) of females, and the ratio of males to females was 3: 4. The mean age of the subjects was 10.12 ± 3.39 years (4–17 years). Distribution of the age of admission by different subgroups, 13 patients (14.44%) aged 1-5 years, 31 patients (34.44%) aged 5-10 years, 40 patients (44.44%) aged 10-15 years and 6 patients (6.66%) were over 15 years old. The mean duration of the disease was 2.40 + 2.11 years (range = 0.3–7 years). The onset of the disease in 21 patients (23.33%) lasting one year (22.9%) from the onset of the disease, 48 patients (53.33%) presented one to five years from the onset of the disease, and 21 patients (23.33%) over five years. The most common type of arthritis was polyarthritis in 43 patients (47.77%), followed by oligo-arthritis in 31 patients (34.44%) and systemic onset in 12 patients (13.33%). The mean ESR was 41.03 + 27.80 mm / hour 1. (Range = 07-128 mm / hour 1) And mean CRP 16.1 + 13.80 mg / L (range = 6-47 mg / L). While the ESR was> 30 mm / 1 hour in 50 of the 90 patients (55.55%), 43 of these 50 patients (86%) were in the active disease group. Similarly, positive CRP was found in 58 patients (64.44%), of whom 52 (89.7%) belonged to the group with active disease. Compared with the remission and active disease groups, 33 patients of active diseases were female. In the active disease group, the mean age was 11.01 + 3.30 years, and the duration of the disease began at one year in patients. Polyarthritis was detected in 26/62 (41.9%) of patients in the active disease group. Conclusion: High CRP and ESR parameters are good for predicting active disease in JRA patients. Keywords: C-reactive protein, Juvenile rheumatoid arthritis, JRA and Erythrocyte mentation rate.

Dissomic disorders associated with juvenile rheumatoid arthritis: impact on quality of life

Dyssomnic disorders (DD) associated with juvenile rheumatoid arthritis (JRA) are some of the most common conditions that are difficult to endure and that lead to deconditioning. This study aimed to assess prevalence and structure of DD, their relationship with clinical picture peculiarities and contribution to deterioration of the quality of lives of JRA patients. At the 1st stage, we assessed prevalence of DD in a continuous sample of JRA patients and healthy children aged 8–16 years. At the 2nd stage, we assessed DD structure, features associated with gender and age, connections to the key clinical characteristics of JRA and quality of life of the patients. In the context of the study, we used the SDSC sleep quality scale, the PedsQL 4.0 quality of life model, and the Ritchie index. DD develop in JRA patients 3.3 times more often than in healthy children (in 178 (72.3%) and 93 (22.2%) children, respectively). The DD registered were sleep initiation and maintenance disorders (54 cases, 22.0%), respiratory disorders (32 cases, 13.0%), sleep-to-wakefulness transition disorders (31 cases, 12.6%), excessive sleepiness disorders (38 cases, 15.4%), combinations thereof (23, 9.3%). Girls had sleep initiation and maintenance disorders more pronounced (p = 0.003), boys were more prone to excessive sleepiness (p = 0.008). The severity of DD increases with patients' age (r = 0.69; p = 0.001) and JRA onset age (r = 0.71; p = 0.001); they are also more severe in polyarticular JRA patients (r = 0.32; p = 0.048). We have clarified the relationship between DD and indicators of inflammatory (r = 0.56; p = 0.001) and Ritchie indices (r = 0.44; p = 0.005), duration of morning stiffness (r = 0.49; p = 0.029). The proven connection between DD and JRA entails the need for routine checks for DD in such patients, and, when discovered, DD should call for personalized therapeutic and diagnostic approach rather than be regarded as one of the JRA syndromes.

Ilizarov technique for severe knee flexion contracture in juvenile rheumatoid arthritis

Severe flexion contracture deformity of the knee joint is often found in patients with rheumatoid arthritis (RA), which severly limiting the walking ability. Total knee arthroplasty (TKA) is a good option, but it is difficult and complicated to perform TKA, especially for juvenile patients with severe flexion deformity. There are limited reports about management of severe knee flexion contracture (KFC) of RA with Ilizarov technique. The aim of this study was to retrospectively analyzed the results of Ilizarov technique for the juvenile rheumatoid arthritis (JRA) patients with severe knee flexion contracture (KFC). From March 2000 to October 2018, nine patients (15 knees) with severe knee flexion contracture (KFC) of JRA who underwent Ilizarov knee joint distraction technique were reviewed retrospectively. There were 4 male (6 knees), 5 female (9 knees) with an average age of 15 years old (12–19years old). However, 6 cases also had equinus deformity, whose foot and ankle joint distraction device were installed simultaneously. The mean preoperative KFC and ROM angle of the knee were 48.8 ​± ​16.7° (mean ​± ​standard deviation) and 41.6 ​± ​15.3°, respectively. Preoperative VAS score (Visual analogue score) and HSS score (hospital for special surgery knee score) were 7.7 ​± ​2.5 and 37.4 ​± ​11.2, respectively. The average time of follow-up was 47.6 ​± ​21.3 months. All the patients achieved full correction of flexion contracture at the end of distraction. At the last follow-up, the mean flexion degree and ROM were 12.1 ​± ​11.7° and 23.5 ​± ​19.3° respectively, which were significantly different compared with preoperative condition. The total duration of ring fixation application was 10–21 weeks, with an average time of 14.3 weeks. The mean VAS and HSS score at the last follow-up were 1.4 ​± ​1.2 and 71.5 ​± ​7.3, which were significantly improvement to the preoperative condition. There were no severe complications during treatment in this study. Pin tract infection occurred in 7 cases during the knee joint distraction and the infection were controlled after slowing down the distraction speed and wrapping with alcohol gauze at the interface between the fixing pin and the skin. Ilizarov method is an effective and safe procedure to treat severe KFC in JRA which can correct deformities, relieve pain and improve the function of weight bearing. Pin tract infection and knee joint stiffness and even fusion are common problems, but correction of deformity of the knee using Ilizarov technique has possible positive outcome for possible further surgery if necessary. Our study shows that Ilizarov knee joint distraction for treatment of severe KFC in JRA is effective and safe. It is concluded that this method offers a new option for juvenile rheumatoid arthritis.

VITAMIN D AND ITS ROLE IN COURSE OF INFLAMMATORY DISEASES OF JOINTS IN CHILDREN

Studies of recent decades have found that vitamin D implements immunoregulatory effects by influence on mechanisms of nonspecific protection from infectious agents, as well as on a system of a specific immune response. A number of studies indicate a relationschip between vitamin D deficiency and the autoimmune diseases onset.Objective. To improve diagnosis of juvenile rheumatoid arthritis and reactive arthritis in children by studying of the serum 25-hydroxyvitamin D impact on disease activity. Materials and methods. 80 children aged 2 to 16 years were observed. I group included 20 children with articular form of juvenile rheumatoid arthritis (JRA), II group - 40 children with reactive arthritis (RA). The control group consisted of 20 healthy children. To all children level of serum 25-hydroxyvitamin D was measured by enzyme immunoassay.Results. A significant 25-hydroxyvitamin D decrease was found among children with JRA 19.53 [12.34;27.50] mmol/L and RA 22.77 [12.39;36.33] in comparison with the children of control group 27.08 [19.87;50.90] mmol/l (p<0,05). Inverse correlation was found between serum 25-hydroxyvitamin D and number of affected joints in children with JRA (p<0,02), as well as between 25-hydroxyvitamin D and indicators of laboratory activity in RA and JRA patients (p<0,05).Conclusion. This study indicates a reliable relationship between the disease аctivity and serum 25-hydroxyvitamin in reactive arthritis and juvenile rheumatoid arthritis patients. Thus, serum 25-hydroxyvitamin D can be used as a marker of disease severity, as well as for predicting the course of inflammatory joint disease in children.

Lack of Association between STAT4 Single Nucleotide Polymorphisms and Iranian Juvenile Rheumatoid Arthritis Patients

ABSTRACTJuvenile rheumatoid arthritis (JRA) is a common chronic systemic autoimmune disease in children. Single nucleotide polymorphisms (SNPs) of signal transducer and activator of transcription 4 (STAT4) gene are suspected to have association with the risk of autoimmune diseases. Previous investigations have indicated that the STAT4 rs7574865 T allele was significantly associated with rheumatoid arthritis. In this study, we aimed to evaluate the association of STAT4 SNPs with JRA in Iranian population. T allele of STAT4 rs7574865 SNP was less frequent in patients than in controls, and the difference was not significant (p = 0.19, OR = 0.72, 95% CI: 0.44 −1.17). In addition, G allele of this SNP was frequent but not significant in JRA patients (p = 0.19, OR = 1.38, 95% CI: 0.85–2.25). Neither alleles nor genotypes of rs7601754 SNP of STAT4 gene demonstrated associations with JRA. We recognize that gene variants of STAT4 did not affect JRA susceptibility in Iranian population.

OPTIMIZATION OF PHARMACEUTICAL CARE FOR JUVENILE RHEUMATOID ARTHRITIS AT THE REGIONAL LEVEL

Clinical practice and content analysis of scientifi c literature indicate that for treatment of the immune-infl amatory disease called juvenile rheumatoid arthritis (JRA), the use of only basic medicines (BM) is not suffi cient. The aim of the study is the organization of pharmacotherapy of JRA patients by genetically engineered biological preparations (GEBP), affecting the immune processes of the body directly. Materials and methods : the theory of marketing, content analysis, the ABC-analysis, sociological survey, pharmacoeconomic methods. Results and discussion . Depending on the individual characteristics of patients, the clinical picture of the disease can manifest itself in different ways, which requires justifi cation of the necessary schemes of GEBP pharmacotherapy at each stage of the carried out treatment. In the course of studying the organization of pharmaceutical assistance at the inpatient stage, we carried out a content analysis of 175 medical records of the patients (aged 2–17 years old) of the cardiologic department of Irkutsk State Children’s Clinical Hospital, in the dynamics of a few years (2015–2017), and the expert assessment of the degree of GEBP demand was carried out by the doctors. The analysis of the pharmacotherapy has shown that GEBP is used as monotherapy in patients with intolerance to basic medicines or to combinations with basic medicines. It is irrational to start the GIBP treatment immediately because of possible side effects and their high cost. According to the content analysis of the reports of the regional Ministry of Health on preferential drug provision of handicapped children, both in hospital and at outpatient treatment stage, the pharmacotherapy schemes including Adalimumab, Abatacept, Infl iximab, Tocilizumab, Etanercept are most in demand. Conclusion. The study of the organization of GEBP pharmacotherapy, the expert assessment carried out by the doctors, the results of the marketing analysis of the regional pharmaceutical market made it possible to substantiate and propose a rational assortment portfolio of this group of medicines for treatment of various JRA options, taking into account the price component in the Irkutsk region. The obtained results can be used for the formation of the medicinal budget of medical organizations in the region.

Juvenile Idiopathic Arthritis in Olmsted County, Minnesota, 1960-2013.

To evaluate the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Olmsted County, Minnesota in 1994-2013 and trends in juvenile rheumatoid arthritis (JRA) in 1960-2013. Cases of arthritis in 1994-2013 were identified by diagnosis code with medical chart review to confirm diagnosis separately for JIA and JRA. Overall incidence rates with 95% confidence intervals (95% CIs) were age and sex adjusted to the 2010 US white population. Comparisons were made with an earlier (1960-1993) cohort from this same population. Seventy-one incident cases of JIA in 1994-2013 were identified, with an overall age- and sex-adjusted incidence rate of 10.3 per 100,000 (95% CI 7.9-12.7). Forty-two (59%) were female, with an incidence of 12.4 per 100,000 (95% CI 8.6-16.2), as compared to 8.3 per 100,000 (95% CI 5.2-11.3) in males. The most common subtype was oligoarthritis (63%). The mean ± SD age at diagnosis was 8.2 ± 5.3 years. The prevalence of JIA on January 1, 2000 and January 1, 2010 was 51.0 per 100,000 (95% CI 25.2-76.8) and 57.6 per 100,000 (95% CI 31.0-94.5), respectively. When the annual incidence of JRA was compared over time from 1960 to 2013, there was no significant change in incidence overall; however, the incidence decreased among females (P = 0.003). A cyclic pattern of incidence was observed, with peaks approximately every 10 years. Similar to the findings with regard to incidence, prevalence did not change overall, but decreased among females (P = 0.048). There were 4 deaths in the cohort of JRA patients diagnosed in 1960-2013; the standardized mortality ratio was 1.50 (95% CI 0.41-3.83). Incidence of juvenile arthritis overall in Olmsted County, Minnesota has not changed significantly in the past 53 years. A consistent cyclic pattern was noted.

Relation between functional ability and health-related quality of life of children with juvenile rheumatoid arthritis.

[Purpose] The aim of this study was to assess patients’ health-related quality of life, compare it with a healthy age-matched population, and examine associations between functional ability and quality of life among juvenile rheumatoid arthritis (JRA) patients. [Subjects and Methods] The study participants were 26 JRA patients and 25 controls. The Childhood Health Assessment Questionnaire and the Pediatric Quality of Life Inventory 4.0 Generic Core Scales were used to evaluate functional ability and health-related quality of life, respectively. [Results] Functional ability scores averaged 0.37 in the JRA group and 0.08 in the control group. There were significant between-group differences in functional ability scores in the overall cohort and in the subgroup of participants aged 14–16 years. Health-related quality of life scores were significantly lower in the JRA group than in the control group (68.39 vs. 85.17). In the JRA group, functional ability was statistically positively correlated with health-related quality of life. [Conclusion] We conclude that the mental state of adolescents with JRA affects their particular functional abilities. Subjects in the 14–16 age group who had a longer disease duration and higher difficulty scores showed a lower health-related quality of life than children in the other age groups.

Assessing the likelihood of new-onset inflammatory bowel disease following tumor necrosis factor-alpha inhibitor therapy for rheumatoid arthritis and juvenile rheumatoid arthritis.

The association between inhibition of tumor necrosis factor-alpha (TNF-α) in patients with rheumatoid arthritis (RA) and juvenile rheumatoid arthritis (JRA) and the onset of inflammatory bowel disease (IBD) is unclear. We sought to evaluate this association by analyzing adverse events (AEs) reported to the Food and Drug Administration Adverse Event Reporting System (FAERS) with a standardized scoring tool for drug-induced AEs. A search of the FAERS for RA or JRA (January 2003-December 2011) reported with adalimumab, certolizumab pegol, etanercept, golimumab, or infliximab was performed. This dataset was then queried for cases indicating IBD. Full-length reports were accessed using the Freedom of Information Act and organized by age, sex, concomitant medications, co-morbidities, type of TNF-α inhibitor used, and diagnosis/treatment details. The Naranjo score was used to determine whether the drug-induced AEs were definite, probable, possible, or doubtful. There were 158 cases of IBD after TNF-α inhibitor exposure in RA or JRA patients. Use of the Naranjo score revealed that, in a majority of the cases (71.5 %), TNF-α inhibitor exposure was considered a 'possible' cause. A majority of the 'probable cases' in JRA were reported with etanercept (40 patients, 90.91 %). There were no 'definite' cases of anti-TNF-induced IBD. After applying the Naranjo scale, a weak association between new-onset IBD and TNF-α inhibitor therapy in RA patients and a moderately strong association especially with etanercept exposure in JRA patients was observed. However, causality cannot be determined due to limitations of the FAERS and the Naranjo score.

The clinical significance of anti-CCP antibodies in Korean JRA patients

To determine the clinical significance of anti-CCP (anti-citrullinated cyclic peptide) in Korean patients with JRA. Rheumatoid Factor (RF) is a non-specific serologic test in juvenile rheumatoid arthritis (JRA) but the anti-perinuclear autoantibodies are found in adult rheumatoid arthritis (RA) recently. One of these autoantibodies can detect epitopes called citrulline. The authors want to know that these autoantibodies can be applied in Korean JRA.

AB0477 Comparative Analysis of the Faults, Complications, Follow up in Patients with Wide Spectrum for Rheumatic Diseases Attached to Total Hip Replacement

ObjectivesThe feasible of the total hip replacement (THR) in the wide spectrum rheumatic diseases with hip involvements, analysis of the faults, complications, preliminary follow up.MethodsThe are 1069 patients, 773 women...

Repair of radiographic hip joint in juvenile rheumatoid arthritis patients treated with etanercept plus methotrexate

For patients suffering from rheumatoid arthritis (RA), structural damage, i.e. bone erosion and joint space narrowing, is a major factor leading to functional disability. Negative radiographic progression has been shown in joints, especially in RA patients treated with tumor necrosis factor alpha (TNFα) inhibitors in combination with methotrexate. Bone erosion repair in small joints have been observed but only one study selected large weight-bearing joints. We reported 2 cases of patients with severe seropositive juvenile RA who shown improvement of joint space narrowing and subchondral erosion in hip joint when treated with etanercept in combination with methotrexate for at least 1year. Two Japanese cases were also published but with different TNF inhibitors. The mechanisms of bone erosion or joint space narrowing repair are unclear. One study investigated whether bone erosions in rheumatoid arthritis patients show evidence of repair in metacarpophalangeal joints when treated with TNF inhibitors and MTX. These results suggested that repair in RA emerged from the bone marrow and the endosteal lining rather than the periosteal compartment. No study investigated joint space narrowing repair in hip joint in rheumatoid arthritis patients. Larger studies needed to confirm joint space narrowing improvement in hip joint in patients treated with TNF inhibitors and to explain the mechanisms of repair.

A95: The Risk of Dyslipidemia in Pediatric Arthritis Patients Is Similar to That of Adult Patients With Arthritis

Background/Purpose:Patients with autoimmune diseases run a higher risk for dyslipidemia due to chronic inflammatory processes and immune dysregulation. This phenomenon has been recognized to a certain extent in adult arthritis patients but has not been previously explored in pediatric arthritis patients. The objective of this study is to compare the frequency of dyslipidemia in children with Juvenile Rheumatoid Arthritis (JRA), children with Non‐Specific Arthritis (NOSA), adults with Rheumatoid Arthritis (RA), and adults with Osteoarthritis (OA). Determining differences amongst these groups is important in understanding the connection between arthritis and dyslipidemia risk, especially in the pediatric population.Methods:After IRB approval, patient's in this retrospective data base study were gathered from chart review of consecutive patient's with JRA, NOSA, RA, and OA who presented to the pediatric rheumatology and adult rheumatology clinics at an academic center. Outcomes of interest that were compared included total cholesterol, high density lipoprotein (HDL), low density lipoprotein (LDL), triglycerides (TG), cholesterol: HDL ratio, non‐HDL, and very low density lipoprotein (VLDL). Other demographic information compared included patient age, sex, and use of biologic DMARD. Comparisons between groups were made using the Fisher's Exact Test and multiple testing adjustments were made by controlling for the False Discovery Rate.Results:There were 32 pediatric patients (40.6% male) ranging from age 2–17 (median 11 years). Out of 32 pediatric patients, 22 had JRA and 10 had non‐ specific arthritis (NOSA). Furthermore, there were 82 adult patient's (18.3% male) ranging from age 30–85 (median 63 years). Out of these, 49 had diagnosis of RA while 33 had diagnosis of OA. In the pediatric cohort, more JRA patients than NOSA had abnormal LDL values (68.2% and 0.0%, p value = 0.005) and non‐HDL values (50.0% and 0.0%, p value = 0.05). Other lipid parameters were not significantly different. Between the pediatric cohort and adult cohort, the pediatric cohort had greater percentages of abnormal LDL (46.9% and 19.5%, p value = 0.05), TG (93.8% and 24.4%, p value <0.001), and VLDL (62.5% and 15.9%, p value <0.001). No significant difference in lipid parameters between patients with RA and OA. There was also no significant difference in lipid parameters between those with or without RF/CCP positivity, male sex, or biologic DMARD use.Conclusion:Pediatric JRA patients were more likely to have an abnormal lipid panel, specifically LDL and non–HDL, as compared to NOSA patients. Interestingly, pediatric patients had higher rates of abnormal LDL, triglycerides, and VLDL. This suggests that the concern for dyslipidemia in the adult population should also be shown to pediatric patients with JRA. Additional studies are needed to determine whether modification of cardiovascular risk in pediatric patients with chronic inflammatory disease is beneficial in preventing clinically significant premature atherosclerosis.

Human Leukocyte Antigen-DRB1 Associations in Korean Juvenile Rheumatoid Arthritis

Objective. The aim of this study is to investigate the association of different subgroups of juvenile rheumatoid arthritis (JRA) with human leukocyte antigen (HLA) class II DR alleles. Methods. One hundred and nineteen Korean juvenile rheumatoid arthritis patients were classified as HLA-DRB1 allele. To assess the frequency, phenotype frequencies of all JRA cases and each subtypes were compared to those of 485 adult controls. Results. HLA-DRB1*01 was associated with increased risk of JRA. Furthermore, DRB1*01 was associated with polyarticular JRA and pauciarticular JRA. The frequencies of DRB1*14 and DRB1*15 were higher in systemic JRA patients than the controls. Conclusion. The data of this study on Korean children with JRA suggests that HLA-DRB1*01 was associated with the susceptibility of JRA. The study should be extended to include larger numbers of patients.

Sexual maturation in Egyptian boys and girls with juvenile rheumatoid arthritis

Children with juvenile rheumatoid arthritis (JRA) exhibit a compromised growth status while information concerning the pattern of their sexual maturity is scant. The aim of the current work was to study sexual maturity in boys and girls with JRA. The study included eighty JRA patients they were 45 male and 35 female and eighty age- and sex-matched normal children served as controls. Development of genitalia was evaluated as per sexual maturity rating criteria given by Tanner score. Development of hair (pubic, axillary and facial) and age of monarch to JRA females were noted The mean (±SD) age of appearance of genitalia stage G-2 in boys with systemic onset JRA (12.0 ± 0.3 years) was earlier when compared with pauciarticular (12.60 ± 0.93 years) and polyarticular (13.39 ± 0.93 years) JRA but delayed for all types of JRA when compared with controls (10.06 ± 0.63 years). In comparison with female groups, the mean (±SD) age of appearance of genitalia stage G-2 with systemic onset JRA (12.0 ± 0.4 years) was also earlier when compared with pauciarticular (12.68 ± 1.09 years) and polyarticular (13.72 ± 0.39 years). Age of menarche delayed in all JRA female patients. None of the study group reach stage G-5 of genitalia development. The timing of initiation of sexual maturity in boys and girls with JRA delayed and this delay variable according to disease subtype.

A case of fat embolism syndrome in juvenile rheumatoid arthritis patient

Fat embolism syndrome is a serious complication that can occur after trauma or operation of the limbs. Clinical criteria are used for the diagnosis of fat embolism syndrome and sometimes radiologic findings are helpful. Fat embolism syndrome is known to occur less frequently in children than in adults, but there is an increased risk in children with connective tissue disease. However, there are only a few reported cases of fat embolism syndrome in juvenile rheumatoid arthritis which is the most common connective tissue disease in children. We report a case of fat embolism syndrome diagnosed in a 13-year-old boy with juvenile rheumatoid arthritis, which was treated with corticosteroid. (�������� ������ ������ ����(���������������

New-Onset Inflammatory Bowel Disease During Tumor Necrosis Factor-Alpha Inhibitor Therapy for Rheumatoid Arthritis and Juvenile Rheumatoid Arthritis: A Review of the Food and Drug Administration Adverse Event Reporting System and Literature

Purpose: New-onset inflammatory bowel disease (IBD) has been reported as a paradoxical adverse event (PAE) in patients receiving tumor necrosis factor-alpha (TNF-α) inhibitors. We conducted a review of the Food and Drug Administration Adverse Event Reporting System (AERS) and published literature for cases of IBD reported in rheumatoid arthritis (RA) and juvenile rheumatoid arthritis (JRA) with TNF-α inhibitor therapy. Methods: The FDA AERS is a publicly available database of voluntary reports for post marketing surveillance of all approved drugs. 2,562,390 files from 2003 - June 2011 were downloaded and analyzed using SSPS 20 (IBM Co. Armonk, NY). Files were queried for reaction terms from the Dictionary for Regulatory Activities - inflammatory bowel disease, Crohn's disease (CD), ulcerative colitis (UC), and ulcerative proctitis reported with infliximab, etanercept, adalimumab, golimumab, and certolizumab and their trade names. “Primary suspect” reports were selected where indication for use was RA or JRA, (diseases are not associated with increased risk of IBD). A MEDLINE search was performed for published English language literature using MESH terms “inflammatory bowel disease/chemically induce”, “Crohn's disease/chemically induced”, and “ulcerative colitis/chemically induced” combined with the Boolean operator “OR” between terms. Additional articles were found in bibliographies of selected articles. Results: There were 183 cases of IBD after TNF-α inhibitor therapy (Table 1). The majority (95; 51.91%) were cases of CD, 61 (33.33%) were UC, and 27 (14.75%) were unspecified IBD. IBD was most commonly (105 cases; 57.37%) reported after etanercept and during therapy for RA. 77.4% of cases were females. The MEDLINE search revealed 30 previously described cases, 2 in RA patients (1 on infliximab and 1 on adalimumab) and 28 in JRA patients (26 on etanercept, 2 on infliximab). 26 JRA cases were histologically verified; 18 cases were CD, 3 were UC, and 5 were indeterminate IBD.Table 1: Inflammatory bowel disease with TNF-α inhibitor therapy reported to FDA AERSConclusion: TNF-α inhibitor therapy may be associated with new-onset of IBD and appears to occur most commonly in RA patients.

Successful ICU Care of a 14-Year-Old With Total Artificial Circulation Using Two Ventricular Assist Devices for 118 Days

Within the first year following orthotopic heart transplantation, the patient is at risk of graft failure, rejection, and infection. Late mortality is often due to allograph vasculopathy. Most of the patients with primary graft failure will usually recover with temporary mechanical ventricular assist support; however, on occasion the patient must be retransplanted. For the patient who suffers primary graft failure necessitating explanation and bridging until a new suitable graft is found, there are several total artificial heart options. However, in the pediatric population these options are limited. The authors’ surgical colleagues were able to fashion a paracorporeal total artificial heart constructed with 2 ventricular assist devices. In this case report, the authors discuss a pediatric patient who had to be explanted and supported for an extended amount of time. This article describes the incidence of cardiomyopathy in juvenile rheumatoid arthritis patients, the choice of extracorporeal membrane oxygenation/extracorporeal complete life support versus ventricular assist devices for support, the major causes of primary graft failure, and the authors’ experience in caring for an adolescent with such a device for a protracted length of time (118 days).

Dosing Celecoxib in Pediatric Patients With Juvenile Rheumatoid Arthritis

The objective was to derive dosing recommendations for the use of celecoxib in patients with juvenile rheumatoid arthritis (JRA) using pharmacokinetic (PK) and exposure-response data. PK and efficacy data from a randomized, double-blind, 12-week study of celecoxib dosed at 3 and 6 mg/kg twice a day (bid) as an investigational suspension formulation in 152 JRA patients aged 2 to 17 years, PK data from 36 adult RA patients, and relative bioavailability data in healthy adults comparing suspension or capsule sprinkles with the commercial capsule were analyzed. Typical oral clearance (L/h) values were 40% and 24% lower in patients weighing 10 and 25 kg, respectively, compared with a 70-kg patient. Longitudinal, logistic pharmacodynamic models incorporating linear effects of dose/area under the plasma concentration-time curve (AUC) over 0 to 12 hours (AUC(0-12)) suggested that the percentage of responders increased with celecoxib exposure. Systemic exposures (AUC) were similar for the suspension, capsule sprinkles, and intact capsule. Administration of a 50-mg bid capsule (or sprinkles) for patients weighing 10 to 25 kg and 100 mg bid for patients >25 kg was predicted to yield similar exposures and response rates as those observed in the JRA trial. Doses and dosage forms not studied in the JRA trial were approved based on the results of this analysis.